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Movement Disorders
Vol. 22, Suppl. 16, 2007, pp. S1S295
2007 Movement Disorder Society

ORAL PLATFORM
PRESENTATION 4601
Tuesday, June 5, 2007
2:30 PM 4:30 PM
Location: Marmara Room
RESTLESS LEGS SYNDROME AND
OTHER MOVEMENT DISORDERS
1
Evidence for linkage of Restless legs syndrome to chromosome 9p:
Are there two distinct loci?
K. Lohmann-Hedrich, A. Ziegler, A. Neumann, A. Kleensang,
T. Lohnau, H. Muhle, A. Djarmati, I.R. Konig, P.L. Kramer,
U. Stephani, C. Klein (Luebeck, Germany)
Objective: To investigate the genetic cause of Restless legs syndrome (RLS) in a large German family.
Background: RLS is a common sensory-motor disorder characterized by paresthesias and an intense urge to move the legs with a
considerable familial aggregation. To date, no gene mutation has been
found but ve gene loci have been mapped in primary RLS to chromosomes 12q, 14q, 9p, 2q, and 20p (RLS1-5).
Methods: We identied a four-generational German RLS family
with 37 family members including 15 affected cases. We performed
linkage analysis using microsatellite markers at the ve known loci.
Prompted by the identication of a potentially shared haplotype near
the RLS3 locus, we expanded the investigated linkage region on chromosome 9p using additional DNA markers.
Results: Mode of inheritance in our RLS family was compatible with
an autosomal dominant pattern and disease onset was mainly in childhood or adolescence. We excluded linkage to the RLS1, RLS2, RLS4,
and RLS5 loci. However, we identied a likely new RLS gene locus
(RLS3*) on chromosome 9p with a maximum LOD score of 3.60
generated by model-based multipoint linkage analysis. A haplotype
centromeric to RLS3, anked by D9S974 and D9S1118, was shared by
all twelve investigated patients. In addition, eleven of them carried a
common haplotype extending telomeric to D9S2189 that is located
within RLS3. Among the unaffected family members, only a 7-year old
child carried the disease-associated haplotype. The RLS critical region
shared by all affected family members covers 20cM or 9.94Mb on
chromosome 9p21 and is separated by 5cM or 2.31Mb from the RLS3
locus. The extended RLS critical region that is common to all but one
affected comprises 26cM or 13.19Mb with an overlap with RLS3 of
2cM or 0.93Mb.
Conclusions: We demonstrate linkage to a locus on chromosome 9p
that is probably distinct from RLS3. Our family with a rather homogeneous phenotype and very early disease onset represents a unique
opportunity to further elucidate the genetic causes of the frequent RLS.
2
RLS patients can also develop compulsions on dopaminergic agonists
E. Pourcher, H. Cohen (Quebec, Canada)
Objective: The aim of this study was to assess the frequency of
self-reported compulsive behaviors, depressive and stress symptoms
and sleep disorders in a population of Restless legs syndrome (RLS)
patients treated with DA agonists.
Background: Recent reports by Tippmann-Peikert et al (2007) and
Ritz et al (2006) extend the risk of developing DA agonists related
impulse control disorders such as gambling, heightened sexual drive, or
unreasonably overinvested repetitive activities to RLS patients.
Methods: A questionnaire covering RLS severity (IRLS-Walters et
al 2003), mood variables, The Beck Depression Inventory II (Beck et

al 2003), a visual Analog Scale of current level of stress and sleep

parameters (The MOS Sleepscale, Hays & Stewart 1992) was sent at 3
points in time over twelve months in 2005 (February, June, October) to
150 RLS patients cared for at the Quebec Memory & Motor Skills
Disorders Clinic. A last section exploring changes in hobbies, development of new habits and compulsions was added at the third mailing.
Results: Data derived from a population of 97 patients having
answered all three mailings identied 17 positive responses to compulsions. After re-evaluation only 12 (8 women and 4 men) were found
to have truly compulsive behaviours. In two women, simple motor
stereotyped sequences in response to sensory urge evoked the phenomenology of Gilles de La Tourette Syndrome. In two other, one male,
one female, a reactivation of trichottillomania previously exhibited in
childhood was observed. For the other eight patients, abnormal behavioural display was similar to complex compulsory behaviours with
short-term rewarding properties such as buying clothes, buying food,
eating or gambling. In addition BDI and stress scores signicantly
contrasted the compulsion () vs compulsion (-) patients. Their MOS
sub-scale sleep problem Index I and II showed signicantly higher
scores and nally, the augmentation phenomenon was of higher prevalence in the C group at the time of the 3rd questionnaire.
Conclusions: In summary, 12 out of 97 RLS patients on stable DA
agonist therapy presented with progressive dishinibition of behaviour
possibly shaped by pre treatment gender and individual specicities.
None had a previous diagnosis of anxious generalized disorder or
obsessive compulsive disorder. They showed more dysphoric and recognized themselves as more stressed than the compulsion (-) group of
RLS patients.
3
Transcranial sonography in Restless legs syndrome
J. Godau, A.-K. Wevers, A. Gaenslen, A. Di Santo, E. Caliskan-Erle,
T. Gasser, D. Berg (Tuebingen, Germany)
Objective: To investigate morphological abnormalities of patients
with restless legs syndrome (RLS) using transcranial sonography
(TCS).
Background: Sonographic hypoechogenicity of the substantia nigra
(SN) has been demonstrated as a typical nding in RLS, which is
thought to be correlated with decreased SN iron content. Other sonographic features have not yet been examined. Especially common
comorbidities such as depression, which is known to be correlated with
hypoechogenicity of the raphe in primary depressive disorders, and
periodic limb movements, which have been shown to be correlated with
red nucleus activation on fMRI, may be associated with sonographic
abnormalities.
Methods: 103 RLS patients underwent TCS according to a standardized protocol by an experienced and independent rater, who was
blinded for the clinical presentation. Diagnosis of RLS and depression
was made according to current diagnostic criteria after thorough clinical and neurological examination by a second experienced rater
blinded for the TCS results.
Results: 71.8% of the patients were diagnosed with idiopathic RLS,
17.4% with secondary RLS and in 10.6% RLS origin was uncertain.
49.5% of the patients had depression or were on antidepressant therapy.
64% of the patients reported PLM. 86.4% of all patients (93.1% in
idiopathic RLS) showed SN hypoechogenicity, 61.1% hypoechogenicity of the raphe, 28.7% hyperechogenicity of the basal ganglia and
64.2% hyperechogenicity of the red nucleus (NR). Raphe hypoechogenicity correlated with depression (p0.03), NR hyperechogenicity
correlated with reported PLM (p0.002). Hyperechogenicity of the
basal ganglia did not correlate with any assessed clinical feature. 27.7%
showed 2 of the 4 features, 46.6% 3 of 4 and 6.8% all four features.
Conclusions: SN hypoechogenicity is a characteristic feature of RLS.
Also, NR hyperechogenicity and raphe hypoechogenicity are typical
and independent ndings in RLS. Since they are associated with typical
clinical features, they may represent pathophysiologic changes of RLS
related disorders. Further studies are needed to evaluate a potential
diagnostic, differential diagnostic and therapeutic value of TCS for
RLS.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S2

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

4
Original clinical and biological ndings in 3 new mutations of the
senataxin gene
M. Anheim, M.C. Fleury, J. Franques, J.-P. Delaunoy, M. Moreira,
M. Koenig, C. Tranchant (Strasbourg, France)
Objective: To report clinical and genetical pictures of 7 patients,
issued from 4 unrelated families, affected with ataxia with oculomotor
apraxia (AOA2).
Background: AOA2 is one of the most recently described nonFriedreich autosomal recessive cerebellar ataxia (ARCA), and is genetically dened by mutations of SETX localized in 9q34. AOA2 is
characterized by cerebellar ataxia, sensory-motor axonal neuropathy,
cerebellar atrophy on imaging and elevation of serum foeto-protein
(AFP) level.
Methods: The four families affected with AOA2 were genotyped
with microsatellite markers present in the AOA2 region on chromosome 9q34. We sequenced all exons of SETX of the patients linked to
the AOA2 locus and we tested for the presence or absence of the
missense mutation by DHPLC analysis of 100 control DNA samples of
the same geographic origin.
Results: Mutations (including 3 new mutations) of the senataxin
gene were found for the seven patients : nonsense mutations E343X
and K2209X, frameshift mutation 5264delC and missense mutation
R2444H. Cerebellar ataxia, sensory-motor axonal neuropathy, cerebellar atrophy on imaging and elevation of serum foeto-protein (AFP)
level were present in all patients. However oculomotor apraxia was
present in only one case. First signs occured between 13 and 18 years.
One patient presented with lower limb distal amyotrophy and fasciculations with elevated creatine kinase ranged from 500 to 20.000 UI/L
confounding with lower motor neuron involvement. In the consanguineous family with the E343X mutation, an elevated AFP level was found
in 3 asymptomatic heterozygous patients.
Conclusions: These new cases underline the phenotypic homogeneity of AOA2 despite the heterogeneity of SETX mutations, with 4
mutations described. Our results suggests also that AFP level could be
the sign of heterozygosity in asymptomatic carriers and could be useful
for genetic counselling.
5
Silver syndrome variant of hereditary spastic paraplegia: Identication of a novel locus
A. Orlacchio, C. Patrono, F. Gaudiello, V. Moschella, A. Borreca,
A. Orlacchio, R. Floris, G. Bernardi, T. Kawarai (Rome, Italy)
Objective: To assess genotype-phenotype associations, we carried
out a clinical and genetic study of two large Italian families with Silver
Syndrome, SS (RM-36 and RM-51).
Background: SS is a complicated form of hereditary spastic paraplegia (HSP) associated with amyotrophy and weakness of the intrinsic
hand muscle. A recent genetic study has shown that SS can be caused
by mutations in the BSCL2 gene (seipin) on chromosome 11q12-q14
(SPG17). In this context, investigating the genotype-phenotype correlations is essential to understand the mechanism(s) of the disease.
Methods: Neurological evaluations, neurophysiological and neuropsychological assessments, neuroimaging studies, linkage study, and
sequencing.
Results: Linkage analyses demonstrated that the two families are
excluded for linkage to the BSCL2 locus. Sequence of the seipin gene
revealed no disease-causing nucleotide substitution. Having excluded
the multiple known HSP loci in the pedigree RM-36, we performed a
genome-wide search for linkage of SS, which showed a novel SS locus
on chromosome 4p. The clinical features of this family are comparable
to those in SS. Linkage analyses at the currently-known ADHSP loci
showed evidence of linkage to SPG4 in the pedigree RM-51. SPG4
sequence analysis revealed a novel frameshift mutation in exon 6
(c.961insG) resulting in premature termination at the codon 326
(p.N326X). PCR-RFLP analysis showed that the mutation segregates
with the disease and the mutation was not found in 200 control
chromosomes. Clinical investigations in the family carrying the frame-

Movement Disorders, Vol. 22, Suppl. 16, 2007

shift mutation in SPG4 demonstrated decreased vibration sense in

lower limbs, pes cavus, cognitive dysfunctions, and temporal lobe
epilepsy (TLE), in addition to SS typical clinical manifestations. Analysis of age at onset and degree of disease severity among generations
revealed a general impression of genetic anticipation. Electrophysiological studies revealed the involvement of lower motor neurons as
well as upper motor neurons.
Conclusions: The data presented in this study identify a second locus
for the SS variant of HSP. Our study also demonstrates that SPG4-HSP
may include clinical features observed in SS and widen the spectrum of
genetic abnormalities of the disorder.
6
Spectrum of gait impairments in presymptomatic and symptomatic
Huntingtons disease: Cross sectional data
A.K. Rao, L.M. Muratori, E.D. Louis, C.B. Moskowitz, K.S. Marder
(New York, New York, USA)
Objective: To quantify gait impairments in presymptomatic and
symptomatic Huntingtons disease (HD) subjects using a cross-sectional design, and examine the sensitivity of gait measures.
Background: Gait impairments are important because they may lead
to an increased risk for falls. Little is known about gait impairments in
the presymptomatic stage. We thus quantied gait impairments in HD
using a cross-sectional analysis of presymptomatic carriers and symptomatic individuals across stages of the disease.
Methods: Our sample (n65) included presymptomatic gene carriers
(PSGC) (n 15), symptomatic HD subjects (n 30) and healthy
controls (n 20). Gait data were collected with the GaitRITE (CIR
Systems, Inc.: Havertown, PA). For PSGC and symptomatic HD subjects, we collected CAG repeat length (if available), UHDRS total
motor score and TFC scale score, and number of years since symptom
onset (for symptomatic HD subjects). Subjects were requested to walk
at a preferred speed on the GAITRite mat and completed three trials for
data analysis.
Results: PSGC demonstrated decreased gait velocity (p0.01),
stride length (p0.022), and increased time in double support (p0.04)
compared with controls; and demonstrated higher variability in stride
length (p0.01)and step time variability (p0.05) compared with
controls. These impairments worsened with increasing disease severity
for symptomatic HD subjects. Gait impairments were correlated with
predicted years to onset in PSGC (gait velocity-.65; cadence-.70,
step time-.71) and demonstrated high sensitivity (velocity 0.72;
stride length 0.66; percent double support 0.66) and specicity
(velocity 0.93; stride length 0.82; percent double support 1) in
distinguishing between controls and those with a trinucleotide expansion. In contrast, UHDRS scores did not reveal impairments in gait and
balance.
Conclusions: Gait bradykinesia and dynamic balance impairments
are present in the presymptomatic stage of HD and continue to worsen
following onset of clinically evident motor symptoms. Quantitative gait
measures were sensitive in differentiating between individuals with and
without an expanded trinucleotide repeat sequence even when these
impairments are not detected by clinical neurological examination.
7
Frequency of dementia in FMR1 premutation carriers
M. Sevin, Z. Kutalik, P. Damier, M. Vercelletto, P. Renou,
P. Boisseau, S. Bergmann, J.-M. Rival, S. Jacquemont (Nantes,
France)
Objective: To study the frequency of dementia in older male carriers
of the FMR1 premutation.
Background: The Fragile Xassociated tremor ataxia syndrome (FXTAS) is a neurodegenerative disorder caused by the premutation (a
CGG repeat expansion) of the FMR1 gene. Only a subgroup of premutation carriers develop symptoms of FXTAS and primarily present
with cerebellar ataxia and intention tremor. Cognitive impairment has
also been reported in patients affected with FXTAS. This is the rst

ORAL PLATFORM PRESENTATION 4602, TUESDAY, JUNE 5, 2007

study addressing the frequency of cognitive decline and dementia in
premutation carriers.
Methods: 67 males aged 50 years or older were recruited from fragile
X families, regardless of their medical history or genetic status. The
Mattis Dementia Rating Scale (MDRS) was used to quantify global
cognitive capacities. Other tests assessed executive functioning, verbal
and non-verbal working memory, visuospatial skills, and reasoning
capacities. The study was double blinded in that genetic status and
CGG repeat length were obtained after evaluation.
Results: Molecular testing revealed 30 premutation carriers and 37
intrafamilial controls. Age and education level distributions were similar in both groups. Based on a cut-off score of 129 for the MDRS and
an education level corrected at 12 years, the frequency of dementia at
age 70 among individuals with large premutation (90 CGG), small
premutation (60 CGG) and controls was 40%, 15%, and 4% respectively. At age 60 these frequencies were 5%, 0%, and 0% respectively.
All of these differences were highly signicantly (p0.001). Multiregression analysis accounting for age and education, found a signicant
correlation between the CGG repeat length and the MDRS, tests of
executive functions (Wisconsin Card Sorting Test, verbal uency),
visuospatial performances, and reasoning capacities (p0.001). Working memory tasks were not affected by the premutation size.
Conclusions: The frequency of signicant cognitive decline and
dementia among carriers of large premutation is particularly high. It is
less frequent for smaller premutation alleles, however there is still a
measurable effect. This is of importance since smaller alleles are much
more frequent in the general population.
8
Intrafusal effects of botulinum toxin injection in patients with
upper motor neuron syndrome
C. Trompetto, G. Francavilla, C. Ogliastro, L. Avanzino, M. Bove,
A. Berardelli, G. Abbruzzese (Genova, Italy)
Objective: To evaluate the role of intrafusal chemodenervation in the
treatment of post-stroke upper limb spasticity with botulinum toxin
type A (BoNT-A).
Background: Evidences from animal and human studies suggest that
BoNT-A, besides its effect on extrafusal motor bres, can act also on
gamma motor endings thus modifying spindle afferent discharges from
the injected muscles. This effect can be studied in humans by investigating BoNT-A induced changes of the tonic vibration reex (TVR).
Methods: In 7 patients (4 men, aged 6417 years) with upper motor
neuron syndrome due to stroke and never treated with BoNT-A, we
measured the maximal M-wave (Mmax), the H/M ratio, the maximal
voluntary contraction (MVC) and the TVR in the injected wrist and
nger exor muscles before (T0), 1 month (T1) and 4 months (T2) after
injection. The ratio between pre- and post-injection values was calculated. Muscle tone was clinically evaluated using the Modied Ashworth Score (MAS). In two subjects, the physiological assessment was
performed also 8 months after the treatment.
Results: The TVR was signicantly more inhibited than Mmax and
MVC at T1 (p 0.05). At T2, Mmax and MVC reached the preinjection values, while the TVR remained depressed. The H/M ratio,
slightly decreased at T1, regained the pre-injection value at T2. After
BoNT-A injection muscle tone was reduced both at T1 and T2. In the
two subjects studied 8 months after treatment, the TVR resulted still
depressed, while the MAS value matched that at T0 and the H/M ratio
was found to be increased compared to pre-injection values.
Conclusions: The special sensitivity of TVR to suppression by
BoNT-A depends on the chemodenervation of intrafusal muscle bers,
leading to a reduced spindle inow to the central nervous system during
vibration. This effect possibly explains the residual clinical benet at
T2. The lack of clinical evidence of increased spasticity at T3, in spite
of the raised H/M ratio, suggests that the chemodenervation of intrafusal bers could counteract the progressive increase of spinal excitability, thus preventing further development of spasticity.

S3

ORAL PLATFORM
PRESENTATION 4602
Tuesday, June 5, 2007
2:30 PM 4:30 PM
Location: Halic Room
ATYPICAL PARKINSONISM AND
DYSTONIA
9
Effect of disease duration on the pattern of cerebral glucose metabolism in patients with multiple system atrophy
J.H. Lee, C.H. Lyoo, S.H. Oh, M.S. Lee (Seoul, Republic of Korea)
Objective: To investigate the effect of disease duration on the 18[F]deoxyglucose positron emission tomography (FDG PET) ndings in
patients with early multiple system atrophy (MSA).
Background: Using a region of interest method, a previous FDG PET
study of the patients with early MSA showed reduced glucose uptake
at the putamen, cerebellum and brainstem. However, no voxel based
FDG PET study has been reported about the effect of disease duration
on the pattern of metabolic changes in the patients with early MSA.
Methods: We included 38 patients who had a shorter than 3 years
history of motor or cerebellar symptoms compatible with probable
MSA. We also included 16 age-matched controls. According to the
disease duration, we classied the patients into three groups (1YR
group: patients with disease duration between 1 and 12 months; 2YR
group: patients with disease duration between 13 and 24 months; 3YR
group: patients with disease between 25 and 36 months). The quantitative FDG PET scan studies with arterial blood samplings were
performed, according to the method reported previously. Using the
group comparison statistics in the SPM2 (statistical parametric mapping) software, we compared each group of MSA with normal controls.
We considered the cerebral regions signicant when the cluster-level
corrected p value was less than 0.05.
Results: In the 1YR group, the glucose metabolism was reduced in the
cerebellum, brainstem, medial frontal (BA10) and lateral frontal (BA8)
cortices. The 2YR group showed reduced metabolism at the temporoparieto-occipital association cortices, thalamus, caudate and posterior part
of the putamen, in addition to the areas involved in 1YR group. In 3YR
group, the pattern of hypometabolism was similar to that of 2YR group,
but the striatal hypometabolism spread to involve its anterior part.
Conclusions: The hypometabolism of cerebellum, brainstem and
frontal motor association cortices occurs within one year after the onset
of motor or cerebllar symptoms. Hypometabolism of the putamen and
temporo-parieto-occipital cortices occurs in patients with disease duration longer than one year. Hypometabolism of the anterior part of the
striatum occurs in patients with duration longer than two years.
10
Neuroprotection and Natural History in Parkinson Plus Syndromes (NNIPPS): Results of a randomized placebo-controlled
trial of riluzole in PSP and MSA
P.N. Leigh, A. Ludolph, Y. Agid, G. Bensimon, The NNIPPS
Consortium (London, United Kingdom)
Objective: To test the efcacy of riluzole in progressive supranuclear
palsy (PSP) and multiple system atrophy (MSA) and to investigate the
natural history of PSP and MSA presenting as parkinsons plus
syndromes.
Background: PSP and MSA often present as parkinson plus syndromes. The natural history is poorly understood, and diagnosis is
problematic. Excitotoxicity may contribute to neuronal damage. We
investigated the effect of riluzole, a glutamate release inhibitor, on
survival and function and acquired prospective data on diagnostic
criteria, natural history, MRI changes, and pathology.
Methods: NNIPPS is an European multicentre, stratied, randomized
(PSP or MSA) parallel-group trial of riluzole (50mg-200mg daily)

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

versus placebo in patients with PSP and MSA. The primary outcome
measure was survival at 36 months. Secondary outcome measures
included functional status, cognition, quality of life, service costs, and
MRI abnormalities. Power calculations were based on published estimates of survival. The primary analysis was intent-to-treat (ITT).
Treatment effect was analyzed by the Kaplan-Meier method using a
stratied Log-Rank test, and the Cox proportional hazards model.
Results: 766 subjects (363 PSP, 403 PSP) were recuited in UK,
France, and Germany, randomized over 3 years and followed doubleblind for up to 36 months. Pathological diagnosis in 112 cases showed
90% sensitivity and specicity for PSP and MSA using NNIPPS
diagnostic criteria. Median survival from onset was 7.8 years for PSP
and 8.7 for MSA. There was no signicant difference in survival
between PSP and MSA from randomization. PSP subjects were more
impaired on measures of clinical and cognitive function. Analysis of
the ITT population showed no evidence of a signicant treatment effect
of riluzole in the population as a whole, or in the PSP or MSA strata.
Conclusions: Riluzole has no neuroprotective effect in PSP or MSA,
but survival can be used as a primary outcome measure for trials in
early-stage PSP and MSA (parkinsons plus syndromes). NNIPPS
provides a unique resource for understanding motor, cognitive, neuroimaging, and genetic factors in MSA and PSP.
11
Degeneration of cardiac sympathetic nerve can occur in multiple
system atrophy
S. Orimo, T. Kanazawa, A. Nakamura, T. Uchihara, F. Mori,
A. Kakita, K. Wakabayashi, H. Takahashi (Tokyo, Japan)
Objective: The pathophysiological mechanism accounting for the
slight decrease in meta-iodobenzylguanidine (MIBG) uptake in some
patients with multiple system atrophy (MSA) remains to be elucidated.
For conrmation, we examined cardiac tissue and sympathetic ganglia
from patients with MSA.
Background: Decreased cardiac uptake of MIBG on MIBG myocardial scintigraphy, a sensitive biological marker for Parkinsons disease
(PD), is related to cardiac sympathetic denervation in patients with PD.
A slight decrease in cardiac uptake of MIBG has also been reported in
some patients with MSA.
Methods: We immunohistochemically examined each specimen of
15 patients with MSA together with 10 control subjects using antibodies against tyrosine hydroxylase (TH) and neurolament (NF).
Results: The number of TH-immunoreactive nerve bers in the
epicardium was preserved in 8 of 15 patients with MSA, as well as in
10 control subjects. The number of TH-immunoreactive, but not of
NF-immunoreactive nerve bers in the epicardium, was mildly or
moderately decreased in six patients with MSA, of whom four showed
a decrease of TH immunoreactivity in the neuronal somata in the
sympathetic ganglia. Moreover, TH- and NF-immunoreactive nerve
bers almost entirely disappeared in the heart in one patient with MSA,
in whom Lewy body pathology was present in the sympathetic ganglia.
Conclusions: These ndings suggest that mild degeneration of the
cardiac sympathetic nerve can occur in MSA, which is closely related
to the pathological change of neurons in the sympathetic ganglia,
accounting for the slight decrease in cardiac uptake of MIBG. Moreover, concurrent Lewy body pathology in the sympathetic ganglia
might accelerate cardiac sympathetic denervation even in MSA.
12
Correlates of side-to-side symmetry of motor manifestations in
Parkinsonian disorders
R.P. Munhoz, H.A. Teive, L.C. Werneck (Curitiba, PR, Brazil)
Objective: To study the clinical prole of Parkinsons disease (PD)
patients with symmetric motor symptoms in comparison with those
presenting more typical asymmetric parkinsonism.
Background: Although symptoms of PD are initially unilateral, both
body sides will eventually become affected with some degree of asymmetry remaining evident throughout the disease course. In cases of symmetric
symptoms, the possibility of atypical forms of parkinsonism should be

Movement Disorders, Vol. 22, Suppl. 16, 2007

suspected. A minority of cases, however, will not fulll criteria for diagnoses other than PD despite exhibiting symmetric symptoms.
Methods: A total of 909 patients with parkinsonism were assessed
after a 12 hour drug withdrawal following a standardized protocol
including clinical data, UPDRS-III, Hoehn & Yahr staging, assessment
of cognition, and psychosis. Asymmetry was dened subjectively according to history of side of symptom onset and most affected side on
examination concordant with objective side to side comparison of
UPDRS items scores for motor signs.
Results: Symmetric symptoms were detected in 240 (26.4%) subjects: 69 (28.7%) had PD, 56 (23.3%) drug induced parkinsonism
(DIP), 40 (16.6%) vascular parkinsonism (VP), 25 (10.4%) Lewy body
dementia (LBD), 22 (9.1%) multiple system atrophy (MSA), 20 (8.3%)
progressive supranuclear palsy (PSP), 8 (3.3%) normal pressure hydrocephalus. Among the 669 subjects with asymmetrical symptoms, 24
(3.5%) had non-PD diagnoses: 7 MSA, 4 each with VP, LBD and
corticobasal degeneration, 3 with DIP and 2 with PSP. Patients with
symmetrical PD (SPD) were signicantly older than those with asymmetrical symptoms (APD), age of onset was signicantly later for SPD
patients. Psychosis but not dementia was more frequent in the SPD
group. Rigid akinetic parkinsonism with worse postural instability was
signicantly more common among SPD patients.
Conclusions: SPD is an unusual presentation of PD and usually
inidicates alternative diagnoses. We hypothesize that some of the
aspects that differentiate SPD and APD may reect a more widespread,
extra nigral neurodegeneration.
13
The dystonia-associated protein torsinA modulates synaptic vesicle
recycling
A. Granata, G. Schiavo, T.T. Warner (London, United Kingdom)
Objective: Investigate the role of torsinA in dystonia by identifying
new protein binding partner.
Background: Most of cases of early-onset torsion dystonia are
caused by a dominantly inherited in-frame GAG deletion in the DYT1
(TOR1A) gene, which results in the loss of a glutamic acid in the
C-terminal region of the encoded protein, torsinA. There is no evidence
for neurodegeneration, implying that there is a functional neuronal
defect. TorsinA is a member of the AAA ATPase superfamily of
chaperone-like proteins, which is present in the cytoplasm and in the
lumen of endoplasmic reticulum (ER). Mutant torsinA (E-torsinA) is
redistributed from ER to the nuclear envelope (NE) and it is accumulated in large perinuclear membranous inclusions. These inclusions
have been detected in DYT1 brain, supporting their role in the pathogenesis of dystonia.
Methods: We performed a yeast two-hybrid screening using fulllength wt-torsinA and E-torsinA as baits to identify interacting partners. We then analyzed vesicle recycling in human neuroblastoma
SH-SY5Y cell lines, stably transfected with the wt and E-torsinA.
Two methods were used to measure synaptic vesicle (SV) trafc: 1.
labelling with the antibody raised against the intravesicular epitope of
synaptotagminI (Syt-I); 2. analysis of loading and unloading of the
lipophilic dye FM1-43.
Results: The two-hybrid screen identied Snapin, a SNAP25 (synaptosomal associated protein of 25 kDa) binding protein and its interaction with both wild-type and mutant torsinA was conrmed by in
vitro and in vivo assays. Snapin is required to enhance the interaction
between the SNAREs and the synaptic calcium sensor, SynaptotagminI. This is a crucial step in the mechanism of fusion of the SV
triggered by calcium inux. Both experiments to monitor SV recycling
showed that wt-torsinA negatively affected the endocytic mechanism,
whereas the mutant E-torsinA had the opposite effect of increasing
vesicle loading.
Conclusions: These ndings suggest that torsinA has a role in
controlling SV turnover, which may be mediated by its interaction with
snapin. This may affect neuronal trafc of neurotransmitters, such us
dopamine, leading to abnormal signalling in the pathway involved in
the control of movement.

ORAL PLATFORM PRESENTATION 4602, TUESDAY, JUNE 5, 2007

14
Pallidal deep brain stimulation improves quality of life in segmental and generalized dystonia: Results from a prospective, randomized sham-controlled trial
J. Mueller, I.-M. Skogseid, R. Benecke, W. Poewe, G. Deuschl,
A. Kupsch, T. Trottenberg, J. Volkmann (Innsbruck, Austria)
Objective: To evaluate the impact of deep brain stimulation (DBS)
on health-related quality of life (HRQoL) and symptoms of depression
in a randomized, sham-stimulation-controlled trial of DBS in primary
segmental and generalized dystonia.
Background: Trials of pallidal DBS have shown promising results in
patients with medically refractory dystonia, but little is known about
the effects of this treatment on HRQoL and symptoms of psychological
distress.
Methods: 40 pts. with medically refractory primary generalized
(n24) or segmental dystonia (n16) were implanted with electrodes
into GPI and randomized to either active or sham-stimulation for 3
months, followed by either 3 or 6 months of open-label treatment, for
a total of 6 months of neurostimulation in each group. HRQoL was
assessed by SF-36, depression with BDI. Severity of dystonia was
measured with BFMDRS Movement score, and disability with BFMDRS Disability score.
Results: At baseline, all SF-36 domain scores were signicantly
reduced in the patient group compared to data from a normal population sample. Randomized study period: Active stimulation as compared
to sham-stimulation resulted in signicant reductions of BFMDRS
Movement and Disability scores, and of pain scores after 3 months.
Signicant improvement of HRQoL was observed in the active stimulation group only. Open-label study extension: After 6 months of
continuous DBS, the average BFMDRS Movement score reduction was
signicant both in generalized (-42%) and segmental dystonia (-53%).
Comparisons of baseline and 6-month data for the entire group showed
signicant improvements in all SF-36 domains and the BDI score.
Conclusions: This rst sham-controlled DBS trial in dystonia proves
a positive effect on HRQoL in patients with medically refractory
segmental and generalized dystonia. Three months of active stimulation
resulted in signicant improvement of the severity of dystonia, and
physical aspects of HRQoL, compared to sham stimulation. Notably,
none of the SF-36 dimensions improved with sham-stimulation. The
clinical benet was sustained after 6 months of continuous DBS, and
the symptomatic benet translated into a signicant improvement of all
SF-36 domains, as well as of depression scores.
15
Patients with writers cramp show a reduced activation of the left
subthalamic region during handwriting with the affected hand
M. Peller, K. Zeuner, M. Weiss, A. Knutzen, M. Hallett, G. Deuschl,
H.R. Siebner (Kiel, Schleswig-Holstein, Germany)
Objective: To use functional MRI (fMRI) at 3 T to investigate
changes in regional neuronal activity during handwriting and scribbling
in patients with writers cramp (WC). We hypothesized that patients
with writers cramp would show an abnormal activation of the contralateral substantia nigra during handwriting, but not during scribbling.
Background: Writers cramp is a task-specic hand dystonia affecting handwriting. Though current concepts assume a dopaminergic
dysfunction in the basal ganglia, motor activation studies during handwriting have mainly revealed changes in cortical activity.
Methods: 22 patients with WC and 18 healthy controls were studied
with BOLD-sensitive fMRI at 3 T. We used an epoch-related fMRI
design with alternating periods of rest and task. There were two
experimental tasks which were performed in blocks. Participants repeatedly wrote the german German word alle or produced consecutive up- and down strokes with their right dominant hand. Each block
consisted of ve trials which were repeated every 5s. Participants were
familiarized with the task prior to fMRI and the written trace of
handwriting and scribbling was recorded outside the MR scanner. To
minimize dystonia during writing, a foam grip was attached to the shaft
of the pen. fMRI data were analyzed with SPM2 (Wellcome Dept. of

S5

Imaging Neuroscience, UCL, UK). We performed a small volume

correction using spherical regions-of-interest tha covered the right and
left substantia nigra (30 mm diameter). Signicance threshold was set
at P0.05 (FDR; cluster level).
Results: Dystonic symptoms were very mild during scribbling and
handwriting. The comparison of regional BOLD signal changes during
handwriting and scribbling revealed a between-group difference in
task-related activity in the left substantia nigra. Patients with WC
showed a relative decrease in activation of the substantia nigra during
handwriting as opposed to scribbling (T 4.06; x,y,z -9,-18,-9).
Conclusions: This is the rst fMRI study that shows a task-specic
underactivation of the substantia nigra contralateral to the affected
hand. The relative underactivition of the substantia nigra during handwriting indicates a context-specic functional abnormality of the basal
ganglia in writers cramp.
16
Clinical and electrophysiological phenotype of myoclonus dystonia
due to epsilon sarcoglycan gene mutations
E. Apartis, E. Roze, F. Clot, I. Guyon-Marechal, S. Thobois,
C. Tranchant, P. Damier, Y. Beaugendre, A. Durr, M. Vidailhet
(Paris, France)
Objective: To describe the clinical and electrophysiological picture
of myoclonus, in myoclonus dystonia (M-D) or essential myoclonus
(E-M) syndromes, caused by mutations in the epsilon-sarcoglycan gene
(SGSE).
Background: SGSE gene mutations are present in up to 30 % of M-D
or E-M syndromes. Myoclonus in M-D or E-M is the predominant
feature, with alcohol sensitive brisk jerks involving mainly the face,
neck and upper limbs. Myoclonus is documented as sub cortical, but a
genetic signature lacks in electrophysiological studies.
Methods: We conducted a multi centric prospective clinical and
electrophysiological study of myoclonus in 35 patients - from 21
families - carrying a SGSE mutation with M-D or E-M syndromes.
Electrophysiological analysis included polymyography (n35), C-reex recording (n27) at rest and during mild contraction and EEG
jerk-locked-back-averaging (JLBA) (n15).
Results: Myoclonus involved the face (31%), voice (34%), four
limbs (50%) with upper limbs predominance, two upper limbs (23%) or
lower limbs (4%) and almost always the axis. It was symmetrical in
60% of the cases or asymmetrical/unilateral. Generally, myoclonus
occurred at rest and was increased by action. Nevertheless, it was
absent at rest in 7 cases. It was never responsive to somesthetic stimuli
and rarely (5 %) induced by loud sounds. Brief shock-like myoclonus
occurred without any temporo-spatial organization. The duration of
myoclonic bursts recorded at rest and during action was 95.7/-37.4
ms (m/-SD; range 32-256 ms; n287 bursts) and there was no
difference in both conditions. Rhythmic myoclonus occurred in 8 cases
(frequency: 3-10 Hz). Negative myoclonus was an exception. C-reex
was negative and JLBA failed to detect any premyoclonic cortical
potential. Moreover, we describe in detail the associated dystonia, if
present.
Conclusions: We provide a straightforward and comprehensive electro clinical description of myoclonus in a large series of patients
carrying diverse mutations in the SGSE gene. We conrm that brief
shock-like myoclonus observed in this condition is of sub-cortical
origin.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S6

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

ORAL PLATFORM
PRESENTATION 5601
Wednesday, June 6, 2007
2:30 PM 4:30 PM
Location: Marmara Room
PARKINSONS DISEASE
17
The physiological effects of pedunculopontine stimulation in patients with Parkinsons disease
S. Tisch, J.C. Rothwell, V. Di Lazzaro, M. Dileone, F. Capone,
P. Proce, A. Insola, P. Mazzone (London, United Kingdom)
Objective: To study the effects of pedunculopontine (PPN) stimulation on motor cortex and spinal excitability in patients with Parkinsons
disease (PD).
Background: The PPN is a brainstem nucleus involved in motor
control and locomotion and has emerged as a promising new target for
DBS in PD, although little is known about the physiological effects of
PPN stimulation in humans.
Methods: Four patients with PD implanted in PPN were studied with
the DBS electrode externalized. EMG was recorded from rst dorsal
interosseous muscle, TMS was applied to the motor cortex at an
intensity to elicit a 1 mV motor evoked potential (MEP) unconditioned
and with prior single stimuli to PPN at interstimulus intervals (ISI) of
2,4,6,8 and 10ms. PPN stimuli were bipolar, 2-4 V, 60-90 sec duration. H-reexes were recorded from soleus and exor carpi radialis
with single stimuli, 1s trains and continuous PPN stimulation at 25Hz.
In one patient we recorded scalp EEG during 0.2 Hz PPN stimulation.
Results: Single PPN stimuli inhibited the MEP from the ipsilateral
motor cortex by a mean of 37% 9 maximal for ISI 4-6 ms. No
inhibition was observed in the contralateral hemisphere. Single and
trains of PPN stimuli did not alter H-reex amplitude. Continuous
stimulation resulted in progressive increase in H-reex amplitude over
minutes (mean 67% 21) which persisted for several minutes after
PPN stimulation was stopped, independent of changes in M-wave
amplitude. PPN stimulation produced a distinct central cortical evoked
potential with an early negative waveform (onset latency 4.6 ms, peak
8 ms, amplitude 0.61 V) and a later larger positive waveform (onset
latency 33 ms, peak 47ms, amplitude 2.2 V).
Conclusions: We have demonstrated ipsilateral short latency motor
cortex inhibition after PPN stimulation and an evoked potential early
component at a similar latency. These effects may be due to antidromic
stimulation of cortico-PPN bres or activation of adjacent medial
lemniscus or cerebellar bres. The progressive facilitation of H-reexes
during and after 25 Hz PPN stimulation suggests short-term plasticity
within descending reticulospinal control of spinal excitability, and may
explain why patients experience progressive benet, over minutes, after
PPN DBS is commenced.
18
Clinimetric testing of the new UPDRS (MDS-UPDRS) vs. original
version
C.G. Goetz, B.C. Tilley, S. Shaftman, G.T. Stebbins, S. Fahn,
P. Martinez-Martin, W. Poewe, C. Sampaio, M.B. Stern, R. Dodel,
B. Dubois, R. Holloway, J. Jankovic, J. Kulisevsky, A.E. Lang,
A.J. Lees, S. Leurgans, P.A. LeWitt, D. Nyenhuis, C.W. Olanow,
O.O. Rascol, A. Schrag, J. Teresi, J.J. van Hilten (Chicago, Illinois,
USA)
Objective: Compare the original Unied Parkinsons Disease Rating
Scale (UPDRS) and the new version of the scale (MDS-UPDRS) for
internal consistency, measurement characteristics, and score equivalency.
Background: The standard rating scale for Parkinsons disease (PD)
patient monitoring is the UPDRS. In 2001, after a Movement Disorder

Movement Disorders, Vol. 22, Suppl. 16, 2007

Society-sponsored critique of the scale, the society ofcially endorsed

the development of a new version of the UPDRS to address weaknesses
and to expand the assessment of PD to include more non-motor aspects.
The new scale (MDS-UPDRS) was adapted in response to cognitive
pretesting, more data have been gathered, and this report is an initial
comparison of the original and new versions in a large PD patient
sample.
Methods: Movement disorder physicians and experienced study coordinators examined PD patients with both scales. Scores were submitted to a central data base, and all queries were resolved before the
database was locked. An emphasis was placed on assessing patients
from diverse racial/ethnic backgrounds and across all Hoehn and Yahr
stages. Patients receiving levodopa or other drug treatments, untreated
patients, and those who had undergone neurosurgical procedures were
permitted. All patients and raters spoke uent English. Sample size
calculations to assess equivalency between the total scores on the two
scales, as well as item-by-item evaluations required a sample of 700
patients. Data are reported as means and standard deviations (SDs);
associations are measured with spearman correlation coefcients. The
MDS-UPDRS and UPDRS total scores were compared using a paired
t-test (new version minus old version).
Results: Data entry closed on January 31, 2007 with 809 patients
enrolled, approximately two-thirds men. The majority was Caucasian
(675), but minority representation (total 134, 16.6%) included 8 Native
Americans, 41 Asians, 47 African-Americans, and 42 participants of
other ethnicities. Hoehn and Yahr stage at the time of UPDRS and
MDS-UPDRS scoring was: Stage I72, Stage II459, Stage III173,
Stage IV74, Stage V27. The number of patients receiving levodopa
alone or in combination with another symptomatic treatment for PD
was 741, while 66 were untreated. Internal consistency was high for
both instruments (Cronbachs alpha UPDRS 0.945; MDS-UPDRS
0.940). The distributions of scores were similar: Mean, SD, UPDRS
56.7, 27.0, covering 55 items; MDS-UPDRS 63.6, 27.1, covering 65
items. The mean difference between the MDS-UPDRS and the UPDRS
was 8.41 (SD8.77, p0.0001, paired t-test). The correlation between
the total scores (r 0.95), combined Parts I and II (Mentation and
Activities of Daily Living from UPDRS and Non-motor and Motor
Experiences of Daily Living from MDS-UPDRS) (r 0.90), Part III
(Motor examination for both UPDRS and MDS-UPDRS) (r 0.95),
and Part IV (items 32-39 on dyskinesias and motor uctuations on
UPDRS and Motor Complications on MDS-UPDRS) (r 0.88) were
high.
Conclusions: In the beginning phases of the clinimetric analysis, the
MDS-UPDRS demonstrates good validity for total and sub-section
scores. As expected, a positive difference was detected between the two
total scores as they were not designed to be in exact agreement. The
MDS-UPDRS was designed to be an expanded version of the UPDRS
and this is consistent with the positive mean difference when old is
subtracted from new. A full Item Response Theory analysis will be
important to assess the similarities between the two scales, to understand the psychometric properties of the newly added items, and to
eliminate duplicative items. The additional analyses will also include
developing an algorithm to interpret UPDRS scores when a comparison
to the MDS-UPDRS is required.
19
The 2 adrenergic antagonist pamezole prolongs the anti-parkinsonian actions of L-DOPA in the MPTP-lesioned macaque
T.H. Johnston, J.-M. Savola, S.H. Fox, J.M. Brotchie (Toronto,
Ontario, Canada)
Objective: To assess the quality of the extended on-time observed
when L-DOPA is combined with pamezole in the MPTP-lesioned
macaque.
Background: We have previously shown that the 2 adrenergic
antagonist pamezole is effective at reducing peak-dose L-DOPAinduced dyskinesia and increases the duration of anti-parkinsonian
action of L-DOPA in both MPTP-lesioned primates and PD patients.
Methods: Six cynomolgus monkeys (Macaca fascicularis) were rendered parkinsonian by repeated administration of MPTP and motor

ORAL PLATFORM PRESENTATION 5601, WEDNESDAY, JUNE 6, 2007

S7

complications were induced by twice daily L-DOPA treatment

(16.9mg/kg) for 6 months. For each animal, two doses of L-DOPA, low
and high were dened such that the lower dose (20-30mg/kg) provided
an anti-parkinsonian benet that lasted only 2h but was not compromised by disabling dyskinesia while the high dose (40-60mg/kg) provided a longer, 3-4h, antiparkinsonian benet but was compromised by
signicant levels of disabling dyskinesia. These doses of L-DOPA were
co-administered, orally, with either vehicle or pamezole (10mg/kg).
Results: Fipamezole signicantly increased the duration of action,
i.e. total on-time, of both the low and the high dose of L-DOPA, by
76% and 43% respectively. Fipamezole also decreased the severity of
peak dose chorea, but not dystonia, seen following administration of
the high dose L-DOPA. These actions were associated with an increase
in the amount of good on time, i.e. on-time during which dyskinesia
was not disabling, or was absent, by 74% and 98%, after combination
of pamezole with the low and high doses of L-DOPA respectively.
Conclusions: These data conrm that pamezole can reduce peakdose dyskinesia and increase the time for which L-DOPA provides
anti-parkinsonian action. Moreover, the increase in on-time is not
associated with disabling dyskinesia thus pamezole provides a means
to increase good on-time after each administration of L-DOPA.
These data further support to the concept of developing pamezole to
reduce motor complications in PD.

21

20

22

Topography of -synuclein pathology in Parkinsons disease

M.E. Kalaitzakis, M.B. Graeber, S.M. Gentleman, R.K.B. Pearce
(London, United Kingdom)

Staging of lewy-related pathology in a community-based sample of

dementia: Evidence for disease-dependent anatomic distribution
J.B. Leverenz, E.B. Larson, D. Vavrek, E.R. Peskind, J.D. Bowen,
W.C. McCormick, L. Teri, W.A. Kukull, T.J. Montine, D.W. Tsuang
(Seattle, Washington, USA)

Objective: Assessment of the topographical distribution of

-synuclein (aSN) pathology in the Parkinsons disease (PD) brain.
Background: It has been proposed that the development of aSN
pathology in PD follows an ascending stereotypical pattern starting in
the lower brainstem to then involve what are considered less vulnerable
cell groups in more rostral brainstem areas. Involvement of higher brain
centres and the cerebral cortex would be secondary. However, the
reliability of this staging of aSN pathology originally proposed by
Braak has been questioned.
Methods: 75 brains (57 PD and 18 with other neurodegenerative
disorder; mean age at death 77.5 years) were examined. Alphasynuclein immunohistochemistry was carried out for the dorsal motor
nucleus of the vagus (DMV), locus coeruleus (LC), substantia nigra
(SN), Nucleus Basalis of Meynert (NBM), amygdaloid complex (AC),
CA2 sector of the hippocampus, entorhinal cortex (EC) and superior
frontal gyrus (SFG) followed by semi-quantitative assessment. In 14
PD cases the spinal cord (SC) was also examined.
Results: Among PD subjects, the SN was affected in 100% of the
analyzed cases as expected. The NBM was affected in 98.2% of the
cases, making it the second most vulnerable nucleus. The neurons of
the DMV were aSN immunopositive in 92.9% and LC in 91.2% of
cases. The involvement of AC was seen in 85.9% followed by the EC
84.2%. The CA2 sector of the hippocampus was affected in 78.9% of
the cases whereas the least affected region was the SFG (77.1%). The
DMV was unaffected in 4 PD cases in whom aSN pathology was
observed in rostral brainstem structures (including the SN) and the
cerebral cortex. In addition, 15 of the analyzed PD cases did not seem
to t the predicted caudo-rostral spread of aSN pathology. The SC was
affected in all (n14) PD cases analyzed with accompanied affection
of the DMV.
Conclusions: Our results demonstrate a predominant involvement of
the SN and NBM but do not support the existence of a medullary
induction site of aSN pathology in the PD brain.

Prospective comparison of weight gain and energy intake after

subthalamic (STN), pallidal (GPi) and thalamic (VIM) deep brain
stimulation (DBS) in Parkinsons disease
S. Blanchard, G. Drillet, P. Sauleau, S. Drapier, A.-S. Gillioz,
T. Rouaud, J. Peron, M. Verin (Rennes, France)
Objective: To study both quality and specicity of changes in food
intake after STN DBS.
Background: Little is known about the mechanism of weight gain
observed after STN DBS.
Methods: We assessed prospectively the weight, body mass index
(BMI) and daily energy intake (DEI) recorded by a dietary interview in
48 parkinsonian patients three months before and six months after STN
(n24), GPi (n12) or VIM (n12) DBS. In addition, motor and
neuropsychological evaluations were performed in the same sessions
according to the Core Assessment Program for Intracerebral Transplantations protocol.
Results: Weight and BMI increased signicantly in STN group in
comparison with both GPi and VIM groups. DEI increased in STN
group, mainly because of increase of glucides intake. This increase was
not correlated with the motor improvement. DEI dramatically decreased in GPi group, correlated with motor improvement, and remained unchanged in VIM group.
Conclusions: STN DBS induced food intake and energy balance
dysregulation, mainly because of increase of glucides intake, neither
observed after GPi nor VIM DBS. These results are consistent with the
involvement of human STN in motivation for food, already demonstrated in animal models.

Objective: To examine the anatomic distribution of LRP in a community-based autopsy sample of dementia.
Background: Published criteria for the staging of Lewy-related pathology (LRP) in normal elderly, Parkinsons disease (PD), and dementia with Lewy bodies suggest a caudal (medulla) to rostral (neocortex) progression of this pathologic change. However, there is
evidence that in some patients, particularly those with dementia, the
anatomic distribution of LRP is not always consistent with this staging
scheme.
Methods: Cases from this autopsy sample were enrolled in a longitudinal study of dementia in a community-based setting. All cases were
examined for LRP using alpha-synuclein immunohistochemistry in the
medulla, substantia nigra, amygdala, cingulate gyrus/parahippocampal
gyrus, and frontal cortex. Severity of LRP in each region was rated, and
cases were assigned a LRP classication based on a modication of
published criteria.
Results: 260 autopsy cases from the sample were available for
analysis. LRP was observed in 126 of these cases (49%). In the LRP
positive cases 16% had brainstem predominant LRP, 19% were amygdala predominant, 18% were limbic, and 44% were neocortical. Coexistent Alzheimers disease (AD) was associated with an atypical anatomic distribution of LRP. Thus, 96% of amygdala predominant LRP
positive cases (limited or no brainstem LRP) had AD, while only 40%
of the brainstem predominant LRP cases also had AD.
Conclusions: LRP is common in dementia, as evidenced by the
presence of this pathology in nearly half of the cases. Of note, nearly
half of these had neocortical LRP. The distribution of LRP in a subset
of this sample, those with amygdala predominant LRP, was inconsistent with published staging criteria for LRP in PD and dementia with
Lewy bodies. In addition, nearly all of these cases had coexistent AD.
The results suggest that the progression of LRP in AD may not follow
the pattern observed in more pure LRP-associated diseases such as
PD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S8

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

23
Neuropsychological and psychiatric sequelae of deep-brain stimulation for Parkinsons disease a randomized controlled multicenter study
C. Daniels, K. Witt, J. Reiff, P. Krack, M. Krause, K. Boetzel,
A. Schnitzler, L. Wojtecki, R. Hilker, E. Kalbe, G.H. Schneider,
A. Kupsch, G. Deuschl, for the German Parkinson Study Group,
Neurostimulation Section (Kiel, Germany)
Objective: To evaluate the neuropsychological and psychiatric effects of bilateral deep brain stimulation of the subthalamic nucleus(STN-DBS) compared to optimized medical treatment in advanced
Parkinsons disease (PD) in a randomized controlled study.
Background: STN-DBS markedly reduces parkinsonian symptoms
in patients with advanced Parkinsons disease and leads to a signicant
improvement of quality of life. Two controlled studies on the neuropsychological effects of STN-DBS reported a mild deterioration of
attention and executive functioning, but in both studies no randomization between surgery and best medical treatment was performed.
Methods: 134 pairwise randomized patients from the Randomized
trial of deep brain stimulation for Parkinsons disease1 underwent
neuropsychological and psychiatric examinations to evaluate the
changes from baseline to six month after STN-DBS or optimized
medical treatment.
Results: The STN-DBS group showed a signicant decline in verbal
uency and the Stroop test. All other neuropsychological tests and the
psychiatric scales did not reveal signicant differences between the
STN-DBS group and the medication group. However, adverse events
associated to neuropsychological or psychiatric symptoms were more
frequent in the STN-DBS group.
Conclusions: This is the rst randomized controlled study evaluating
neuropsychological and psychiatric sequelae of STN-DBS. STN-DBS
seems to be relatively safe concerning neuropsychological and psychiatric side effects in carefully selected patients. The decline of executive
functioning is in line with the ndings of previous studies. However,
the implications of the decline in executive functioning after STN-DBS
for daily life still need to be investigated in detail. The evaluation of
mood and overall psychiatric functioning did not reveal signicant
changes on group level, though patients with STN-DBS more frequently developed neuropsychological or psychiatric symptoms.
1
Deuschl et al. A randomized trial of deep-brain stimulation for
Parkinsons disease. N Engl J Med. 2006 Aug 31;355(9):896-908.
24
Neuropathological characteristics of Parkinsons disease associated
with LRRK2 I2020T (Sagamihara family)
S. Ujiie, Y. Ogino, M. Ogino, T. Uchihara, S. Yagishita,
K. Hasegawa, H. Kowa, F. Sakai (Sagamihara, Kanagawa, Japan)
Objective: To evaluate neuropathological characteristics of Parkinsons disease associated with LRRK2 I2020T (Sagamihara family).
Background: In spite of the high prevalence of LRRK2 mutation in
familial Parkinsons disease, neuropathological studies have been performed only on limited number of cases and the results so far have
shown a pleomorphic picture. This pathological heterogeneity is
thought to be characteristics of the disease with LRRK2 mutations.
Here we report pathological ndings of this family in detail.
Methods: Six autopsied cases (ve women, one man) with Parkinsons disease associated with LRRK2 I2020T (Sagamihara family)
were studied. Age at onset was 52.34.9, disease duration was
19.34.9 (years) and age at death was 71.79.9. The initial symptom
was clumsiness of unilateral leg movement in ve cases and difculty
in walking in one case. Response to L-Dopa was good for long time.
Autonomic symptom was absent or mild and there was no cognitive
dysfunction. Brain sections of six autopsy cases were studied. Brain
sections were stained with hematoxylin-eosin, anti phosphorylated
alfa-synyclein and anti-PHF tau(AT8) antibodies.
Results: Our results showed marked neuronal loss and gliosis in Substantia Nigra (SN). In comparison with SN, the changes of Locus Ceruleus
(LC) were very mild. Lewy Bodies (LBs) were found in only one case in

Movement Disorders, Vol. 22, Suppl. 16, 2007

SN, LC, Dorsal Vagal Nucleus (DVN) and Amygdara. In contrast, no LBs
were found in other cases. Anti-tau antibodies (AT8) immunostaining
performed in four cases revealed tau positive structure in LC in all cases
and in mid-brain Central gray matter in two cases. Tau positive structures
in Hippocampus were not found in three out of four cases.
Conclusions: The core pathologic ndings of this family were
marked neuronal loss and gliosis in SN with preservation of LC. LBs
were not found in most cases but denitely positive in one case. Despite
similarity in clinical features and distribution of neuronal loss, existence of pathological aggregates is heterogeneous. And we suppose that
tau positive deposits mainly found in LC and mid-brain central gray
matter are unique to this family.

ORAL PLATFORM
PRESENTATION 5602
Wednesday, June 6, 2007
2:30 PM 4:30 PM
Location: Halic Room
CLINICAL ELECTROPHYSIOLOGY
AND IMAGING
25
Probing a heterosynaptic manifestation of homeostatic plasticity in
the intact human motor cortex
M. Poetter, S. Fischer, G. Deuschl, A. Quartarone, H. Siebner (Kiel,
Germany)
Objective: The aim of our study was to investigate homeostatic
mechanisms of primary motor hand area (M1-Hand) excitability
changes induced by paired associative stimulation (PAS).
Background: Homeostatic control mechanisms represent an important tool to stabilize cortical excitability changes within a physiological
scope. We tried to establish a valid human model to investigate in vivo
homeostatic mechanisms of heterosynaptic plasticity of the corticospinal output projections in the M1-Hand.
Methods: In 10 healthy subjects, we performed two experimental
sessions separated by at least 1 week. In one session we applied
facilitatory premotor rTMS (5Hz, 5300stimuli, I90%active MT,
biphasic pulse) prior to facilitatory PAS. The PAS protocol consisted of
200 paired stimuli (0.25Hz) of electrical conditoning stimuli applied to
the right median nerve at the wrist (I300%sensory threshold) preceding single monophasic TMS-pulses at left M1-Hand (IMEP1mV) in
interstimulus interval (ISI) adjusted to the N20 latency of the cortical
SSEP of the median nerve (ISI: N20 latency2ms). In the second
session we used inhibitory premotor TMS (1Hz, 2750stimuli) before
application of inhibitory PAS (ISI: N20 latency-5ms). To probe corticospinal excitability, we recorded 4 blocks of MEP before and after
rTMS and PAS from the rst dorsal interosseus (FDI, target muscle),
the right abductor pollicis brevis (APB) and abductor digiti minimi
(ADM) muscle (80-2500Hz, sampling rate 5000Hz).
Results: With preceding 5Hz rTMS, the facilitatory PAS protocol
inhibited the MEP amplitudes at rest (FDI: pre PAS 1.650.09 mV;
post PAS 1.080.17mV). Conversely, when 1Hz rTMS was applied
prior to inhibitory PAS, MEP were facilitated by the inhibitory Pas
protocol (FDI: pre PAS 0.830.09 mV; post PAS 1.420.15mV).
3factorial ANOVA with the factors session (1Hz premotorTMS-iPAS
vs. 5Hz premotor rTMS-fPAS), muscle (FDI, APB, ADM) and time
(t1-4) revealed a signicant interaction of intervention x time (p0.26,
F3.9, Greenhouse-Geisser).
Conclusions: The impact of PAS on M1-HAND critically depends
on the preexisting state of excitability of the conditioned M1-HAND.
Our ndings suggest the existence of heterosynaptic homeostatic metaplasticity in the M1-Hand that keeps corticospinal excitability within a
physiological range.

ORAL PLATFORM PRESENTATION 5602, WEDNESDAY, JUNE 6, 2007

26
Impaired temporal preparation in Parkinsons disease: Slow brain
potential and oscillatory manifestations
P. Praamstra, P. Pope (Birmingham, United Kingdom)
Objective: To investigate temporal preparation in Parkinsons disease (PD) under conditions where temporal preparation is incidental to
the primary task.
Background: One of the functions attributed to the basal ganglia is
the processing of temporal information. Performance on time perception and production tasks in PD provide support for such a role.
However, evidence from explicit timing tasks is not necessarily relevant to timing in motor performance. Here we use EEG measures of
implicit timing to investigate preparation under conditions where preparation is optional.
Methods: PD patients (n10) and age-matched control subjects (CS;
n12) conducted a choice reaction task with two temporally regular
stimulus presentation regimes, both including occasional deviant interstimulus intervals. EEG was recorded continuously from 128 scalp
electrodes and analyzed to extract slow brain potentials and eventrelated spectral perturbations during the interstimulus intervals.
Results: Control subjects, but not patients, demonstrated temporal
preparation in the form of an adjustment in time course of slow brain
potentials to the duration of the interstimulus interval. Deviant intervals
were accompanied by a slow brain potential amplitude drop at the time
of expected stimulus occurrence, demonstrating intact representation of
time in patients. Oscillatory activity in beta and alpha bands showed
reduced post-movement event-related synchronisation (ERS) and attenuated preparatory desynchronisation (ERD) in patients. Although
the modulation of beta activity was abnormal, beta activity was not
increased compared to controls.
Conclusions: PD patients demonstrate impaired temporal preparation
but intact encoding of temporal intervals. This dissociation, ie. the
failure to translate the registration of temporal regularity into preparatory activity, may be explained by impaired dopamine dependent
striatal learning mechanisms.

S9

Conclusions: Results of residual EMG-contrasts differed from the

block contrasts. In healthy controls, the variability in exion-extension
movement (EMG) correlated with the cerebellum, whereas during
sustained posture no specic area was identied with EMG. The
FCMTE patients, with high EMG variability during posture, showed
bilateral activity of the somatosensory area, compatible with cortical
hyperexcitability. Continuous EMG recording during fMRI-scanning
gives, additionally to information concerning task execution, insight in
brain activation related to (superuous) movement.

27
Simultaneous EMG-fMRI; relating movement and brain activity in
tremor
A.-F. van Rootselaar, N.M. Maurits, R. Renken, J.H.T.M. Koelman,
J.M. Hoogduin, K.L. Leenders, M.A.J. Tijssen (Amsterdam,
Netherlands)
Objective: 1) To investigate the additional value of electromyography
(EMG) during functional magnetic resonance imaging (fMRI) in Movement Disorders patients, and 2) To gain insight in the origin of tremor.
Background: Simultaneous EMG-fMRI is a new method proven to
be feasible in healthy controls. It potentially permits to investigate the
functional abnormalities of involuntary movements. Familial cortical
myoclonic tremor with epilepsy (FCMTE) and essential tremor (ET)
are both clinically characterized by tremulous movements of the limbs
and both show cerebellar pathology. However, in FCMTE functional
abnormalities of the cerebral cortex are likely.
Methods: Eight FCMTE patients, eight ET patients and nine healthy
controls performed wrist exion-extension movement and posture (extended wrist and ngers). Surface EMG was recorded from wrist extensor
muscles. The design matrix incorporated both conventional block design
and residual EMG power regressors, after Gram-Schmidt orthogonalization of the EMG-regressors with respect to the block-design regressors for
posture and movement. For the rst-level analyses SPM2 and for the
second-level random effects analysis SnPM was used.
Results: Major ndings: 1) In controls block movement contrast
correlated with the known motor areas whereas the residual EMG
movement contrast correlated with the ipsilateral cerebellum; 2) In ET
patients the block contrasts showed less involvement of motor areas
and more (contra)lateral cerebellar activity; 3) In FCMTE patients all
contrasts showed less cerebellar activity. Residual-EMG posture contrast (tremor-activity) corrrelated with bilateral sensory areas wereas
block posture contrast did not (Figure).

FIG. 1 (27).
28
Change in water diffusion MRI following repetitive transcranial
magnetic stimulation
M. Abe, T. Mima, N. Sawamoto, S. Urayama, T. Aso, H. Fukuyama
(Kyoto, Japan)
Objective: To examine whether diffusion-weighted imaging (DWI)
technique can detect cortical changes elicited by repetitive transcranial
magnetic stimulation (rTMS).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S10

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: rTMS can modulate the cortical excitability, which can

last beyond the end of stimulation. Recent neuroimaging studies have
suggested lasting synaptic activity at the stimulated site and distant
areas after the end of rTMS. DWI is a useful tool for measuring
microscopic states of the brain tissue by probing water diffusion. We
hypothesized that DWI technique can detect the cellular changes elicited by rTMS. Previous reports examining alternations of water diffusion in the stimulated site were controversial. In the present study, we
explored brain regions showing rTMS-induced cortical alternations of
water diffusion in the stimulated site and remote areas.
Methods: Ten healthy volunteers received rTMS at 0.9 Hz (10min;
subthreshold) applied over the left primary hand motor area (M1HAND)
within a 3-T MR scanner. We scanned 5 sets of DWI data (before
(twice), and 0min, 10min and 20 min after rTMS), and measured motor
evoked potentials (MEPs) of the hand intrinsic muscle for estimating
the motor cortical excitability. Images were analyzed with the statistical
parametric mapping software (SPM2). Signal changes between data
sets before and just after the intervention were statistically determined
by a voxel-by-voxel one-way ANOVA. The intervention-induced alterations of MEP size were also assessed by using paired two-tailed t
tests.
Results: MEP size tended to have a lasting reduction within 10 min
after end of rTMS, consistent with previous reports. Following the
rTMS, water diffusion was signicantly increased in bilateral sensorimotor cortex (SMC), premotor cortex (PMC) and prefrontal cortex
(PFC). Among these areas, the alterations in right PMC and bilateral
PFC lasted for longer than 10 min after cessation of the intervention:
the former recovered within 20 min. the latter kept for 50 min.
Conclusions: We found the increased water diffusion elicited by
rTMS in SMC, PMC, PFC bilaterally. It was possible that the cellular
water diffusion changes might reect trans-synaptic neural plasticity
elicited by rTMS.
29
White matter changes in the diagnosis of presymptomatic neurodegenerative diseases: The example of Huntingtons disease
S. Kloppel, B. Draganski, S.J. Tabrizi, R.S.J. Frackowiak (London,
Germany)
Objective: 1.) Can diffusion tensor MRI-data (DTI) from patients in
the presymptomatic stage of a neurodegenerative disease like Huntingtons disease (HD) be used as a diagnostic tool. 2.) Can mapping of
white matter bre tracts identify region-specic neuropathology.
Background: HD is a neurodegenerative disorder with primary pathology in the striatum and characteristic abnormalities of movement,
cognition and behaviour. It is caused by a known genetic mutation and
can therefore be diagnosed in patients many years before the onset of
symptoms. The disease constitutes an important model for investigating other neurodegenerative disorders for which no genetic test is
available.
Methods: We present diffusion tensor MRI-data (DTI) from 22
pre-symptomatic gene carriers (PSC) and 20 matched controls. The
signal obtained depends on the diffusion of water along white matter
bre tracts and is used to compute the local fractional anisotropy (FA),
a marker of white matter integrity. The principal direction of diffusion
in each white matter voxel can be used to generate streamlines that
identify anatomical connections.
Results: Using a multivariate support-vector machine we classied
80% of subjects correctly as either PSC or controls based on their
subject-specic FA-map alone. Comparing fronto-striatal connections
between PSC and controls we found a region specic degeneration of
bre tracts from superior frontal gyrus, compatible with neuropathological studies suggesting a loss of pyramidal neurons in that area.
Conclusions: Taken together our study indicates that classication
by DTI is a sensitive diagnostic tool for this pre-symptomatic neurodegenerative disorder. The region-specic pattern of bre degeneration
may provide differential information for clinically similar disorders and
suggests further studies of selective neuronal vulnerability in the pathophysiology of HD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

30
Hyposmia, midbrain hyperechogenity and Parkinsons disease:
Findings in a population-based study
K. Seppi, H. Stockner, S. Kiechl, J. Schwaiger, M. Sawires,
J. Willeit, W. Poewe (Innsbruck, Austria)
Objective: Assessment of the association of nigral hyperechognicity
and hyposmia with Parkinsons disease PD in a population-based
cohort.
Background: In recent years a number of potential biomarkers for
Parkinsons disease (PD) have been identied including transcranial
sonography (TCS) of the substantia nigra (SN) and the assessment of
olfaction by smell tests.
Methods: Subjects: Population-based cohort of 562 subjects aged
55-94 years. Methods: TCS from both sides, a thorough neurological
assessment and assessment of olfactory dysfunction (odor identication
of the 12 items of the Snifn Sticks Screening test). For the purpose
of the present study, hyperechogenicity was dened as an area of
echogenic signal of 0.16cm2 or greater at least on one side (i.e. 75th
percentile of controls), hyposmia was dened as a correct identication
of less than or equal to 8 of the 12 items of the smell test (i.e. 25th
percentile of controls).
Results: 15% of the individuals had an insufcient bone window,
therefore analysis was performed in 480 participants. About one quarter
of total population were found to have SN hyperechogenicity at least on
one side and hyposmia. 16 subjects out of the sample were diagnosed
with PD. Of these, 94% had hyperechogenic areas and 63% had
hyposmia compared to 22% and 24% of healthy participants. Patients
with PD had signicantly increased mean echogenic areas (0.19cm2
vs0.10cm2) and signicantly decreased olfactory function scores (6
vs10) compared to controls. Among normosmic subjects with normoechogenic SN, none of the subjects had PD, PD was present in 3% of
the hyposmic subjects with normal SN echogenity; among the normosmic subjects with SN hyperechogenicity the proportion of subjects with
PD was 7%; the proportion of PD rose to over 30% in the subcohort of
hyposmic subjects with SN hyperechogenicity.
Conclusions: This is the rst population-based study assessing associations of two potential biomarkers for PD with the prevalence of
PD. The prevalence of PD was highest in the subcohort of hyposmic
subjects with hyperechogenic SN and second highest in the subcohort
of subjects with SN hyperechogenity and normosmia. This data suggests that hyperechogenicity is a risk marker for PD and that PD risk
further increases in individuals with both SN hyperechogenity and
hyposmia.
31
Firing patterns of STN in early stage PD patients implanted with
DBS
C.H. Harrison, N.D. Manus, C.E. Gill, C.C. Kao, M.S. Remple,
T.L. Davis, J.S. Neimat, P.E. Konrad, P.D. Charles (Nashville,
Tennessee, USA)
Objective: To compare the size and ring patterns of the subthalamic
nucleus (STN) in early Parkinsons disease (EPD) patients to those
with more advanced disease (APD).
Background: Recent literature has begun to explore the use of deep
brain stimulation (DBS) as a treatment of PD before patients develop
disabling motor uctuations. Intraoperative microelectrode recordings
(MER) facilitate optimal lead placement and provide valuable data
about cell ring patterns. Excessive glutaminergic outow from STN
may contribute to neuronal loss in the substantia nigra pars compacta
(SNc). Animal data suggest stimulation of STN slows the rate of SNc
dopaminergic cell loss.
Methods: We collected MER in the rst three subjects randomized to
DBS as part of a prospective investigation of DBS in EPD (Hoehn and
Yahr II). Two to four recording electrodes were advanced in 500
micrometer increments along a trajectory determined by preoperative
neuroimaging. Electrical potentials were recorded at each point and
analyzed for multi-unit ring rate and background noise. Firing frequencies of individual neurons in the STN were determined by ofine

POSTER SESSION I, TUESDAY, JUNE 5, 2007

analysis. The length of STN in each recording tract was determined by
the relative hyperactivity characteristic of the nucleus. These ndings
were compared with MER using a similar trajectory from three age and
gender matched patients with H&Y IV PD previously implanted as
standard of care.
Results: There was no signicant difference in the length of STN
(4.760.24mm for EPD vs. 4.710.94mm for APD [n12]). The ratio
of background noise in STN to substantia nigra pars reticulata (SNr)
was signicantly lower in EPD (2.120.63 versus 3.190.41 for APD,
p0.01). For ring frequency, a total of 119 STN units were isolated
across patients. Average ring was signicantly lower in EPD (23.09
Hz, n61) relative to APD (26.99 Hz, n58, p0.02).
Conclusions: STN mapping of three EPD patients shows that hyperactive neuronal activity similar to that observed in APD can be used to
guide DBS lead placement. Relative increases in background noise and
ring frequency of STN in APD indicate increased activity with disease
progression. Given the potential toxicity of STN output on SNc, these
data may support the argument for earlier initiation of DBS therapy.
32
Connections between premotor and motor cortex in healthy subjects and in patients with Parkinsons disease
A. Suppa, M. Bologna, C. Lorenzano, F. Gilio, M. Napoletani,
A. Berardelli (Rome, Italy)
Objective: To investigate whether subthreshold rTMS over premotor
cortex (PM) lead to changes in the MEP facilitation elicited by rTMS
of M1.
Background: Suprathreshold 5 Hz repetitive transcranial magnetic
stimulation (rTMS) over primary motor cortex (M1) evokes motor
potentials (MEPs) in hand muscles that progressively increase in amplitude (MEP facilitation).
Methods: 15 right handed normal subjects were studied in three
separate experimental sessions using a conditioningtest rTMS paradigm. In all experimental sessions test-rTMS consisted of trains of 10
stimuli delivered at 5 Hz over the left M1. Conditioning-rTMS consisted of trains of 1500 subthreshold stimuli (90% active motor threshold, AMT) delivered randomly in two separate sessions at 1 Hz or 5 Hz
over PM. The changes in MEP facilitation were measured and analyzed
before and after 1 Hz conditioning (real and sham) or 5 Hz PM-rTMS
stimulation. We also conducted a preliminary study on the connection
between the premotor area and the hand area of the primary motor
cortex in 5 parkinsonian (PD) patients, while off and on therapy.
Results: In normal subjects 1 Hz rTMS over PM signicantly decreased the degree of MEP facilitation. The inhibitory effect lasted for
about 30 minutes after conditioning stimulation ended. There was no
effect on MEP facilitation after 5 Hz PM-rTMS. Test-rTMS delivered
in trains at 5-Hz frequency over the primary motor cortex, had no
facilitatory effect on the size of MEPs in PD patients off therapy, and
only a non signicant facilitatory effect in patients on therapy. Conditioning-rTMS with 5Hz suprathreshold trains over the premotor cortex
had no facilitatory effects on MEP in the off state, whereas there was
an increase of the MEP facilitation during the train in the on state.
Conclusions: In normal subjects 1 Hz PM-rTMS reduces the mechanism of short-term cortical plasticity underlying MEP facilitation
induced by 5 Hz M1-rTMS, probably by acting through PM-to-M1
cortico-cortical connections. In Parkinsons disease suprathreshold 5
Hz rTMS of the left premotor cortex in PD patients facilitates short
term cortical plasticity, only when PD patients are in the on state.

S11

POSTER SESSION I
Tuesday, June 5, 2007
9:00 AM 4:00 PM
Rumeli Hall, Lower Level
Poster Numbers 33345 and Poster
Number 683
Authors Present: 12:30 PM2:30 PM
ATAXIA
33
High eld proton MR spectroscopy of sporadic and hereditary
spinocerebellar ataxias
G. Oz, I. Iltis, D. Hutter, C.M. Gomez (Minneapolis, Minnesota, USA)
Objective: To compare neurochemical alterations in cerebella and pons
of patients with cerebellar multiple system atrophy (MSA/C) to two
prototypic hereditary ataxias, SCA2, a cerebellar-predominant multiple
system atrophy and SCA6, a pure cerebellar ataxia, as well as healthy
controls.
Background: SCAs are characterized by loss of cerebellar Purkinje
cells in combination with neuronal loss in other regions, such as the
pons. Most MRS studies of SCAs reported differences in ratios of
NAA, creatine and choline between patients and controls. Utilizing a 4
tesla (T) magnet allows measurement of concentrations of numerous
neurochemicals, such as neurotransmitters (glutamate, GABA) and
gliosis markers (myo-inositol, glutamine) in the human brain (Oz et al,
Neurology, 2005, 64:434).
Methods: Neurochemical proles of the vermis, cerebellar white
matter and pons of healthy controls (N16) and patients with MSA/C,
SCA2 and SCA6 (N5 in each group) were obtained by ultra-short
echo time (5 ms) 1H MR spectroscopy at 4 T using previously described methods (Oz et al, Neurology, 2005, 64:434). Concentrations of
9-12 metabolites were measured reliably in the three volumes-ofinterest and corrected for atrophy.
Results: Signicant neuronal loss in the vermis was indicated in both
MSA/C and SCA2 by reduced NAA and glutamate (p0.05). Vermian
gliosis was indicated in all three diseases by increased myo-inositol
(p0.001), but was most prominent in MSA/C, where glutamine was
also increased (p0.05). Creatine was increased in the vermis in SCA2
and MSA/C (p0.001), which may also result from gliosis. Metabolic
disturbances in vermis in MSA/C and SCA6 were implied by increased
lactate (p0.05). Involvement of cerebellar white matter was more
pronounced in SCA2 and MSA/C than SCA6 based on alterations in
NAA, glutamate, myo-inositol, glutamine and creatine, while increased
choline, likely due to myelin loss, was only signicant in SCA2 and
SCA6. Involvement of pons was restricted to SCA2 and MSA/C, and
more prominent in MSA/C based on more drastic changes in NAA and
myo-inositol (p0.0002).
Conclusions: Alterations in MSA/C were more similar to SCA2 than
SCA6, consistent with known pathology. Proton MRS at 4 T can be
used to non-invasively evaluate disease- and brain region-specic
cellular and biochemical events in sporadic and hereditary SCAs.
34
Frequency of the MCP sign in FMR1 premutation carriers and
FXTAS
M.A. Leehey, D. Rubinstein, A.G. Brega, D. Hall, F. Tassone,
L. Zhang, R. Hagerman, P.J. Hagerman, J. Grigsby (Denver,
Colorado, USA)
Objective: To dene the frequency of increased signal intensity in the
middle cerebellar peduncles on MR imaging, the MCP sign among: 1)
carriers of fragile X mental retardation 1 (FMR1) premutation, 2) noncarriers of FMR1 premutation, 3) carriers with fragile X-associated tremor/
ataxia syndrome (FXTAS) and 4) carriers without FXTAS.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S12

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: FXTAS is a recently described late-onset disorder characterized by progressive action tremor, gait ataxia and cognitive decline;
other signs include parkinsonism, autonomic and possibly neuropathic
dysfunction. The disorder typically affects male carriers with CGG repeat
expansions in the premutation range (55-200) of FMR1. Larger expansions
(200 repeats) cause fragile X syndrome, the most common heritable
form of mental retardation. MR imaging may reveal the unusual and
distinctive MCP sign, but it is unclear how often affected and non-affected
carriers have this sign. To date the MCP sign has been described in only
two persons shown to have a normal FMR1 repeat size.
Methods: All males over age 50 with FMR1 repeat size in the control
or premutation range that participated in a genotype-phenotype study of
FXTAS at two academic centers and that underwent standardized MR
scanning were included. Carriers were considered affected if they had
action tremor or cerebellar ataxia. Images were scored by a neuroradiologist (D.R.) blinded to FMR1 repeat size for the presence, symmetry and intensity of MCP signs.
Results: Among the 106 men that participated, 67 were premutation
carriers, of which 39 were affected. Average age (SD) of affected
carriers was 68.18.5, unaffected carriers 63.811.1, and non-carriers
59.19.7. The MCP sign was present in 23 of the 67 carriers (34%) and
in none of the non-carriers. Twenty of 39 (51%) affected carriers and three
of 28 unaffected carriers (11%) had the MCP sign. MCP signs varied in
intensity; all were bilateral and frequently extended into the cerebellum.
Conclusions: While the MCP sign is present in only about half of
persons with FXTAS and a third of older male premutation carriers, it
seems specic for the FMR1 premutation status.
35
NARP-MILS syndrome caused by 8993T>G mitochondrial DNA
mutation showing ragged-red bers
J. Youn, J.Y. Kim, W.Y. Lee, E.J. Chung, W.T. Yoon, Y.-L. Suh,
C.S. Ki (Seoul, Republic of Korea)
Objective: To report a 32-year-old NARP-MILS patient with
8993TG mutation in mitochondrial DNA, who presented atypical
dyskinesia and ragged-red bers in muscle biopsy.
Background: The 8993TG mutation in mitochondrial DNA is
related with variable clinical ndings of neuropathy, ataxia, retinitis
pigmentosa (NARP) and inherited Leighs syndrome (MILS). NARPMILS syndrome has been reported to seldom have ragged-red bers or
dyskinetic symptoms.

Methods: In this patient, we examined neurologically and ophthalmologically, and performed nerve conduction study (NCS), muscle biopsy,
brain MRI and direct sequencing analysis of the mitochondrial DNA.
Results: In the neurological, she had truncal ataxia and dyskinetic
movement affecting face (Video). Fundoscopic examination revealed
retinitis pigmentosa in both eyes. NCS demonstrated sensory dominant
sensorimotor polyneuropathy in all extremities. Her muscle biopsy
showed ragged-red bers and neuropathic myopathy under light level
histopathology and mitochondriopathy associated cytoarchitectural
changes under electron microscopy (Figure 1). Brain MRI showed
signicant cerebellar atrophy. Direct sequencing analysis of the mitochondrial DNA revealed the m.8993TG mutation in the patient
(Figure 2).
Conclusions: It is rare case showing abnormal dyskinetic movement
and ragged-red bers on muscle biopsy in genetically conrmed
NARP-MILS syndrome.
36
Gordon Holmes spinocerebellar ataxia with retinal dystrophy
S.-J. Kim, E.-J. Chung, J.-H. Joo (Busan, Korea)
Objective: To report a case of Gordon Holmes spinocerebellar ataxia
with retinal dystrophy.
Background: Gordon Holmes spinocerebellar ataxia is characterized
by co-occurance of progressvie spinocerebellar ataxia and hypogonadism. It is rare disease which was rst described by Gordon Holmes in
1907. This disease is highly heterogeneous syndrome with insidious
onset in second decade of life. Gordon Holmes spinocerebellar ataxia
may be associated with cognitive impairment, choreoathetosis, retinopathy, deafness, hypercalcemia or pyramidal signs.
Methods: We present a case with ataxia, hypogonadism and retinopathy.
Results: An 18-year-old male patient visited our clinic with progressive ataxia. On past history, he was diagnosed as retinal dystrophy at
12-years-old. On physical examination, he had no axillary and pubic
hair. The genital examination showed small penis and testis. Neurological examination revealed cerebellar ataxia and gaze evoked nystagmus. Routine and specic laboratory analyses focusing on metabolic
or inammatory disease gave no evidence for abnormalities. Endocrinological assessment revealed a low serum levels of testosterone 0.23
pg/ml (2.8-8.0 pg/ml), follicle stimulating hormone 0.86 mIU (1.4-18.1
mIU) and luteinizing hormone 0.51 mIU (1.5-9.3 mIU). In summary,
these results were consistent with hypogonadotrophic hypogonadism.
Brain MRI showed diffuse cerebellar atrophy and normal pituitary
gland.
Conclusions: We report clinical and laboratory features of a patient
with ataxia and hypogonadism, typical phenotype of Gordon Holmes
spinocerebellar ataxia and retinal dystrophy.
37
Ataxin-2 localizes at the Endoplasmic reticulum and co-sediments
with polysomes
S. van de Loo, J. Nowock, R. Hilker, G. Auburger (Frankfurt/Main,
Germany)

FIG. 1 (35).

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To understand the physiological functions of ataxin-2, we

have analyzed its subcellular distribution by immunocytochemistry and
subcellular fractionation.
Background: Spinocerebellar ataxia type 2 (SCA2) is a neurodegenerative disorder with pronounced atrophy of cerebellar Purkinje, pontoolivary, nigral and motoneurons, caused by the unstable expansion of a
polyglutamine domain in the disease protein ataxin-2.
Methods: Immunocytochemical analyses were performed on COS-7
cells and on primary cultures of murine hippocampal neurons. Endoplasmic reticulum and polysomal fractions were generated using subcellular fractionantion and ultracentrifugation experiments of mouse
brain homogenates.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

S13

Results: Ataxin-2 immunostaining was exclusively observed in the

cytoplasm, particularly at a prominent structure adjacent to the nucleus,
and in punctae towards the cell periphery. The pathogenic form of
ataxin-2 with an expanded polyQ domain showed the same distribution
pattern. Double-labeling and confocal microscopy identied the juxtanuclear structure as endoplasmic reticulum (ER). Further, ataxin-2
was found to colocalize in part with endosomes. Ultracentrifugation
experiments found ataxin-2 to co-sediment with the polysomal fraction.
Conclusions: These results are in agreement with recent ndings that
ataxin-2 is recruited to stress granules which represent transient structures of stalled mRNA synthesis under environmental stress. For the
ataxin-2 homologue of drosophila, an association with polyribosomes
has also been shown. These data in conjunction with the protein
architecture of ataxin-2 suggest that ataxin-2 is involved in mRNA
processing and/or regulation of translation.

onset to wheelchair use and bedridden living was 3.8 and 5.7 years
respectively. Twenty eight received regular outpatient treatment at the
time of surveillance (average duration 8.2 years, 18 with PEG, 12 with
tracheotomy, average BI 15.4). Time to wheelchair and bedridden
living of this group was 4.4 and 5.4 years respectively. There were no
statistically signicant differences about background factors (age, sex,
type of MSA, duration and BI), numbers of PEG and tracheotomy
between two groups.
Conclusions: The risk of death in MSA may not be inuenced by
PEG or tracheotomy. However, it is conceivable that the procedures
would extend the life span to the natural point, since these are usually
done at the unendurable points.

38

Clinical relevance of bulging eyes for the differential diagnosis of

spinocerebellar ataxias
H.A.G. Teive, R.P. Munhoz, S. Raskin, W.O. Arruda, L.C. Werneck
(Curitiba, PR, Brazil)

A phase III double-blind, randomised, placebo-controlled study of

the efcacy, safety and tolerability of idebenone in the treatment of
Friedreichs ataxia patients
P. Giunti, J. Gray, N.W. Wood (London, United Kingdom)
Objective: The study aims to conrm the positive effect on neurological function, assessed using the International Cooperative Ataxia
Rating Scale (ICARS) and the Friedreichs Ataxia Rating Scale
(FARS). Effects on cardiac mass and function will be also assessed
using magnetic resonance imaging and detailed echocardiography including strain rate imaging. Safety and tolerability will be carefully
monitored. 160 patients will enroll in a few European countries. The
UK will contribute with 50 patients.
Background: Idebenone is a short-chain benzoquinone derivative
which has positive effects on mitochondrial bioenergetics as well as
antioxidant properties, mechanisms which may relevant to the treatment of Friedreichs ataxia (FRDA). Positive effects of idebenone have
been reported on the cardiomyopathy associated with FRDA, although
older studies on its effect on neurological function have yielded conicting results. A recent study at the NIH indicated positive effects of
higher doses of idebenone on neurological function, suggesting that
adequate dosing may be needed to observe effects in the central
nervous system.
Methods: Dosage: Patients are randomised in a 1:1:1:1 ratio to
blinded administration of 1 of 3 treatment arms of oral Idebenone (low,
medium or high dose), or placebo. UK Investigators: Prof N Wood,
Prof R Surtees, Prof W McKenna, London and Prof P Chinnery,
Newcastle.
39
The natural history of multiple system atrophy
K. Arai, Y. Yoshiyama, K. Ogawara, C. Ishikawa, K. Ito (Chiba,
Japan)
Objective: To evaluate the effects of intervention for nutrition and
respiration on the natural history of multiple system atrophy (MSA).
Background: On the natural history of MSA, there are some reports
focusing attention on activities of daily living (ADL) and mortality.
However, the effects of intervention for nutrition and respiration have
not been fully illuminated.
Methods: Forty-seven patients with MSA (MSA-C 34, MSA-P 13,
M:F22:25, average age 65.36.6, mean duration of illness 8.74.0
years) were surveyed retrospectively. All patients belonged to probable
MSA of the diagnostic categories in consensus statement. We inquired
into the inuences of percutaneous endoscopic gastrostomy (PEG) and
tracheotomy on mortality, Barthel Index (BI) for ADL (activity of daily
living). Students t-test, ANOVA and a Cox proportional hazards
model were used for statistical analysis.
Results: Seventeen patients were dead (average duration 9.5 years,
13 with PEG, 9 with tracheotomy, average BI 5.6). The time from the

40

Objective: To investigate the relevance of the nding of bulging

eyes (BE) in a large Brazilian cohort of spinocerebelar ataxias (SCAs)
and to assess its importance in clinical differential diagnosis among
various forms of SCAs.
Background: To date 30 loci have been described for SCAs and
molecular tests are available for SCA 1,2,3,5,6,7,8,10,12,14,17 and
DRPLA. The phenotypic spectrum of SCAs is pleomorphic and the
presence of BE is a distinctive feature, classically described for SCA 3
(Machado-Joseph disease - MJD). This sign, however, has been described in other SCAs.
Methods: 335 patients from 136 Brazilian families with SCAs were
assessed with neurological examination and molecular genetic testing
for SCA types 1, 2, 3, 6, 7, 8, 10 12, 14, 17 and DRPLA. BE was
characterized by the presence of eyelid retraction or eyelid lag. Genetically ascertained SCA 3 detected in 116 patients, SCA 10 in 34, SCA
2 in 12, SCA 1 in 7, SCA7 in 5, and SCA 6 in 3. The remaining 158
patients remained undiagnosed genetically.
Results: BE was detected in 113 patients with SCAs (33.73%),
namely 97 (83.62 %) of the 116 SCA 3 patients and in 12 (19.67 %) of
the others SCA patients (3 SCA 10 patients, 4 SCA 2 patients, 3 SCA
1 patients, 2 SCA 7 patients). There was no correlation between the
presence of BE and the repeat length in the different genes. In the group
of 158 patients without detected mutation BE was found in 4 patients
(2.53%).
Conclusions: In this large Brazilian cohort of SCA patients BE was
detected in the majority of patients with SCA 3 and could be used with
a clinical tool for the differential diagnosis of SCAs.
Disclosure: The authors have nothing to disclose.

41
Involuntary movements in ataxia-telangiectasia: Natural history
and quantitative characteristics
A.G. Shaikh, D.S. Zee, A.E. Meyer, H.M. Lederman, T.O. Crawford
(Baltimore, Maryland, USA)
Objective: We characterized tremor and non-tremor involuntary
movements in A-T.
Background: Ataxia-telangiectasia is an autosomal recessive multisystem disorder affecting the immune and nervous systems. Although
ataxia-telangiectasia (A-T) causes degeneration of cerebellar Purkinje
neurons, the abnormalities of tone, posture and movement suggest
additional extra-pyramidal dysfunction.
Methods: Three-axis accelerometer recordings were used to quantify
the tremor and non-tremor involuntary movements in 79 patients with
A-T.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Results: All patients had some pathological tremor during action,

posture, or the resting conditions. The amplitude-weighted mean frequency of the tremor was in the range of 2-6 Hz in the 80% of the
patients. Thirty-one patients had only action and postural tremors (of
putative cerebellar origin), whereas 48 patients also had resting tremor,
suggesting a broader range of motor systems impairment. The amplitudes of action and postural tremors were signicantly larger than
resting tremor. The amplitude of action tremor correlated with the
patients age, consistent with the progressive cerebellar degeneration.
Conversely, the amplitudes of resting tremor and non-tremor involuntary movements were not associated with age, pointing to a more static
abnormality, probably within the extra-pyramidal system. Head tremors, in the 3-5 Hz frequency range, were also common. Classic clinicalpathologic correlations suggest that the head tremors might be a consequence of the pathology in the anterior cerebellar vermis. The
measures of tremor were consistent over both short (10 minutes) and
long (6 months) intervals.
Conclusions: The test/re-test stability of these objective quantitative
measures indicates they assess a trait with low biologic variability and
measurement error, suggesting potential value as an instrumented objective measure for therapeutic clinical trials. These quantitative characteristics were simulated with a computational model that potentially
provides insight into pathophysiology and potential therapeutic strategies.
42
Different metabolic pattern in SCA 1,2,3 and 6 in FDG-PET and
correlation with clinical parameters
M. Minnerop, E. Rota Kops, H. Herzog, E. Brunt, K.L. Leenders,
T. Klockgether, U. Wullner (Bonn, Germany)
Objective: To examine (1) hypo- and hypermetabolism with dynamic FDG-PET images especially of extracerebellar structures in the
most frequent spinocerebellar ataxias (SCAs) type 1,2,3 and 6, and (2)
to correlate the detected metabolic changes with clinical and genetic
parameters.
Background: SCAs are a clinically and genetically heterogeneous
group of autosomal dominantly inherited neurodegenerative disorders,
characterized by prominent ataxia and cerebellar atrophy. FDG-PET
studies in small patient groups showed controversial results regarding
the involvement of extracerebellar structures. A reduced glucose metabolism is often associated with only mild or no atrophy in affected
regions suggesting that the tissue atrophy has little effect on the PET
ndings.
Methods: We analyzed the regional metabolic rate of glucose (rMRGlu) using 18F-FDG and PET in 43 patients with SCA1,2,3 and 6 (9
SCA1, 6 SCA2, 18 SCA3, 10 SCA6) compared to 13 normal human
volunteers (m/f: 6/7, age 48 12 years). Correlations with ataxia score,
disease duration, age and CAG repeat length were evaluated. The
statistical comparisons between two groups were calculated as 2-sample t tests with an uncorrected threshold of 0.001.
Results: A cerebellar hypometabolism was found for all subtypes
(Figure 1, column a). Further, a reduced rMRGlu was depicted in the
brainstem of SCA 1 and SCA 3 and additionally in both thalami and
left caudate nucleus in SCA1. Cortical hypermetabolic areas (Figure 1,
column b) were found in a subtype-specic pattern, involving especially temporal regions. In SCA2, an extensive cortical hypermetabolism was present. Only for SCA3, negative correlations between rMRGlu and disease duration/ataxia score were detectable.
Conclusions: Hypometabolism was mainly conned to areas with
neuropathological changes like cerebellum, brainstem, thalamus and
basal ganglia. The cortical hypermetabolism without widespread cortical neuronal degeneration could simply reect (1) subclinical neuropa-thological features or metabolic dysfunction, (2) cerebellar diachisis with deafferentation of the cerebral cortex or (3) compensatory
mechanism.

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (42).

43
SCA 12 with the identication of novel intermediate allele
A.K. Srivastava, M. Mukerji, M.V. Padma, K. Prasad, M. Behari
(New Delhi, India)
Objective: To report the smallest allele at SCA 12 locus.
Background: Spinocerebellar ataxia 12 (SCA12) is autosomal dominant cerebellar ataxia (ADCA) associated with an expanded unstable
CAG repeat on chromosome 5q31-33. SCA 12 has basically been
reported from India except for its rst description from USA and it
accounts for about 8% of ataxia families as 3rd/2nd common cause of
ADCA in India and occur in one particular community. Normal allele

POSTER SESSION I, TUESDAY, JUNE 5, 2007

ranges from 7-31 repeats and the lowest pathological range reported
being 53 repeats.
Methods: CAG repeat size at the SCA 12 locus was estimated using
previously published primers and size was conrmed by directly sequencing the PCR products containing the expanded CAG repeats.
Results: We identied a 3-generation pedigree with the affected
proband having 51 CAG repeats. The proband was a 54-year-old man
belonging to same community with progressive tremor in hands, pyramidal, extrapyramidal features and eye movement abnormalities. His
nerve conduction studies revealed evidence of motor sensory neuropathy and MRI of brain showed both cerebellar and cerebral atrophy. He
was investigated for other causes of tremor.
Conclusions: This is the smallest pathological allele reported at the
SCA 12 locus.
44
SCA 7 with late retinal degeneration from India
A.K. Srivastava, M. Mukerji, M.B. Singh, M. Tripathi, R. Bhatia,
M.V. Padma, K. Prasad, M. Behari (New Delhi, India)
Objective: To report the phenotype of genotypically conrmed SCA
7 patient who did not show retinal degeneration till 2 years of disease
onset.
Background: Retinal degeneration constitutes an important component in early adult onset patients with Spinocerebellar Ataxia type 7
(SCA7), a neurodegenerative disorder caused by expansion of an
unstable CAG repeat on chromosome 3p12-21.1.
Methods: Size of the (CAG) array of expansion of SCA 7 locus was
detected by gene scan analysis in ABI prism 377 automated DNA
sequencer and it was 50 whereas normal alleles had 7 17 repeats (200
chromosomes).
Results: The patient was a 29-year-old man who had progressive gait
ataxia since the age of 27 years. His father suffered from similar
problem since the age of 47 years. On examination he had visual acuity
of 6/36 bilaterally, normal visual eld and color vision. Fundoscopic
examination revealed neither macular pigmentation nor disc pallor.
There was no nystagmus or ophthalmoparesis but saccades were mildly
slow. He had gait ataxia and mild incoordination in the upper limbs. All
deep tendon reexes were brisk and plantars were exor. Visual evoked
responses were normal in latency and had reduced amplitude bilaterally. MRI of brain revealed cerebellar and pontine atrophy. Nerve
conduction studies in limbs were normal.
Conclusions: Phenotypically ADCA II or SCA 7 diagnosis was not
thought. This case emphasizes the need for genetic conrmation of type
of SCA. It also reveals that some cases of early adult onset SCA 7 may
have late appearance of visual symptoms and signs.
45
Cognitive dysfunctions in spinocerebellar ataxia type 1 and 2
E. Pastorello, S. Lombardi, F. Cappa, M. Clementi, P. Bisiacchi,
D. Paganini, C.P. Trevisan (Padova, Italy)
Objective: To assess cognitive functions in a series of 16 patients
with genetically conrmed diagnosis of autosomal dominant SpinoCerebellar Ataxia (SCA) type 1 or 2.
Background: It seems that cerebellum may indirectly interfere with
cognition. Despite many reports on the subject, it is still controversial
how frequently, and to what extent, cognitive impairment or overt
dementia may affect patients with hereditary ataxia.
Methods: We evaluated 16 patients diagnosed by molecular analysis
as affected by SCA1 (12 cases, three males and nine females, mean age
52 years) and SCA2 (four cases, two males and two females, mean age
41 years), the two most frequent autosomal dominant cerebellar disorders in the Italian population. Patients underwent clinical neurological
examination, MRI investigation and a comprehensive test battery designed to evaluate cognitive abilities, including tests for attention,
verbal and visuo-spatial memory, as well as executive functions.
Results: None of our patients showed clear-cut dementia, while
frontal-executive dysfunctions and/or verbal memory alterations were
found in three out 12 SCA1 and in one out of four SCA2 cases. Disease

S15

duration, differently from cerebellar MRI abnormalities, seemed to be

correlated with the cognitive alterations.
Conclusions: On the whole, although limited to small series of
patients, our data indicated that possible cognitive dysfunctions in
SCA1 and SCA2 are rather of sectorial type.
46
Characteristics of cortical excitability revealed by transcranial
magnetic stimulation in spinocerebellar ataxias type 1, type 2 and
idiopathic sporadic cerebellar ataxia
S. Radovanovic, N. Dragasevic, J. Maric, M. Svetel, V.S. Kostic
(Belgrade, Serbia)
Objective: The aim was to compare cortical motor evoked potential
(MEP), central motor conduction time (CMCT) and cortical silent
period (CSP) duration in SCA patients in genetically homogenous
groups of ataxia patients with type 1, type 2 and IDCA (idiopathic
sporadic cerebellar ataxia) in Serbia.
Background: Spinocerebellar ataxias (SCA types 1-23) are characterized by their underlying genetic defect. The clinical classication of
the SCA has been difcult due to variations and overlapping of the
clinical signs.
Methods: We examined 27 patients, 14 with the diagnosis of SCA 1,
6 SCA 2 and 7 IDCA patients. Ten healthy control subjects were
gender and age matched. Transcranial magnetic stimulation (TMS) was
used to investigate parameters of cortical excitability such as: motor
threshold (MT) and MEP, CSP and CMCT. MT was established at rest,
MEP was calculated as the area in the rectied EMG recording. CSP
was evoked by 30% supra-threshold stimulation while subjects activated FDI muscle with contraction of 30% of their MVC. CMCT was
calculated as a difference between the shortest MEP latency after
cortical and after cervical stimulation.
Results: Results show that MT was increased in all ataxia patient
groups, compared to control subjects. CMCT has signicant increase in
SCA 1 patients. CSP in IDCA patients is signicantly longer then in
SCA 1, SCA 2 and control subjects, while no difference was found
between SCA 1, SCA 2 and control. Furthermore, ve of our patients
in SCA 1 group had extremely shortened and 3 extremely prolonged
CSP. MEP duration was signicantly increased in all ataxia groups
compared to control subjects.
Conclusions: Our study demonstrated signicant differences in parameters of motor cortex activations by TMS in patients with genetically different type of degenerative ataxia. These differences were more
related to genotype than to clinical symptoms. That point toward
different pathophysiological processes in genetic subtypes of ataxia.
Due to the cerebellar inuence on the cortico-spinal system through
control of inhibitory cortical interneurons, could be concluded that
different categories of ataxia patients have various degrees of disturbed
cerebellar inhibitory input.
47
Human recombinant erythropoietin increases frataxin in Friedreich ataxia
S. Boesch, B. Sturm, S. Hering, H. Goldenberg,
B. Scheiber-Mojdehkar, W. Poewe (Innsbruck, Austria)
Objective: To determine the role of rhuEPO in FRDA in vivo we
performed an open-label pilot study. Primary outcome measure was the
change of frataxin levels at week 8 versus baseline.
Background: Friedreichs ataxia (FRDA) is caused by a GAAtrinucleotide expansion in the frataxin gene, resulting in a reduced
expression of the mitochondrial protein frataxin. Recently we showed
that recombinant human erythropoietin (rhuEPO) signicantly increases frataxin-expression in lymphocytes from FRDA-patients in
vitro.
Methods: Twelve genetically proven, adult FRDA-patients were
included in the study and received 70 units rhuEPO/kg 3 times weekly
subcutaneously. Frataxin-levels from whole blood samples and urinary
8-Hydroxydeoxyguanosine concentrations were analyzed by ELISA.
Serum-peroxides were measured colorimetrically. Other blood param-

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

eters were monitored using routine laboratory methods. Neurological

state was assessed using the Scale for the Assessment and Rating of
Ataxia (SARA).
Results: Treatment with rhuEPO showed a persistent and signicant
increase in frataxin-levels in 7 out of 10 FRDA patients completing the
trial after 2 and 8 weeks (P0.01). In two patients frataxin-levels did
not increase, while one patients rise did not reach statistical signicance. All patients showed a consistent reduction of indicators for
oxidative stress. The rise in hematocrit was within the normal range in
the majority of patients. Serum ferritin and transferrin saturation decreased signicantly.
Conclusions: RhuEPO treatment signicantly increased frataxinlevels in FRDA-patients in vivo during an eight weeks clinical pilot
trial. Parameters that indicate for oxidative stress, in turn, signicantly
decreased during the observation phase.
48
Novel compound heterozygous mutations in SACS gene in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS)
I. Toyoshima, S. Kamada, S. Okawa, M. Sugawara, C. Wada (Akita,
Japan)
Objective: We present a clinical and genetic analysis of a Japanese
family with ARSACS with novel compound heterozygous mutations,
one in exon 6 and the other in exon 9 of the SACS gene.
Background: Autosomal recessive spastic ataxia of CharlevoixSaguenay (ARSACS) is a neurodegenerative disorder that was rst
described among French Canadians in the Charlevoix-Saguenay-LacSaint-Jean region of Quebec, Canada (OMIM 270550). Initially, the
SACS gene was thought to consist of a single gigantic exon, but eight
new exons (exon 1-8) located upstream of the gigantic exon were found
recently.
Methods: Patient 1, aged 51, was referred to our hospital for evaluation of ataxia. He was born to nonconsanguineous parents. He had
slurred and scanning speech, and saccadic ocular pursuit and horizontal
gaze-evoked nystagmus. Muscle weakness and atrophy were present in
the distal limbs. Pes cavus was observed. In coordination cerebellar
ataxia was observed. Deep tendon reexes were absent. Patient 2 is
three years younger than patient 1 and showed the same neurological
singns. Brain MRI revealed severe atrophy of the superior cerebellar
vermis. Genomic DNA of the two patients was extracted from leucocytes. Each coding sequence of the 9 exons and the intron-exon
boundaries of the SACS gene was amplied and directly sequenced.
Results: We identied two mutations in the SACS gene. One is a
single-base deletion, g.482delA (N161fsX175) in exon 6. The other one
is a TC transition at position g.2405 (L802P) in exon 9. These mutations were not found in 110 control chromosomes (55 healthy Japanese
volunteers). Both patient 1 and 2 had compound heterozygous mutations in exon 6 and exon 9. Their unaffected father had a heterozygous
mutation of L802P, and their unaffected elder brother had a heterozygous mutation of N161fsX175.
Conclusions: We identied two novel mutations in the SACS gene in
siblings with the phenotype of ARSACS. The frameshift-type mutation
(N161fsX175) leads to short truncation of the predicted sacsin protein,
and the missense mutation (L802P) is suggested to result in conformational change of the sacsin protein because of the substitution from
leucine to proline, which is demonstrated by Robson prediction for
protein secondary structure. These indicate that both mutations are
pathogenic in ARSACS.
49
Potassium channel blocker 4-aminopyridine is effective in late
onset episodic ataxia type 2 (EA2) a video case report
M. Lohle, W. Schrempf, M. Wolz, H. Reichmann, A. Storch
(Dresden, Saxony, Germany)
Objective: To examine efcacy of the potassium channel blocker
4-aminopyridine (4-AP) in a patient suffering from late-onset episodic
ataxia type 2.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Background: Episodic ataxia type 2 (EA2) is an autosomal-dominant

hereditary disorder caused by mutations of the calcium channel gene
CACNA1A encoding the CaV2.1 subunit of the P/Q-type calcium
channel which is mainly expressed in Purkinje cells. EA2 is characterized by recurrent attacks of ataxia and can often be successfully
treated with acetazolamide. However, treatment options for patients
that do not respond to this treatment are limited.
Methods: We examined a 63-year-old female patient suffering from
episodic ataxia who did not respond to acetazolamide. After we initiated treatment with 5 mg 4-AP three times daily she reported about a
marked relief of her symptoms. In order to objectify the efcacy of
4-AP in this case of late onset EA2 the patient was videotaped and rated
after withdrawal of 4-AP and 1 hour after the intake of a single oral
dose of 5 mg 4-AP using the recently validated scale for the assessment
and rating of ataxia (SARA).
Results: Our patient suffered from recurrent attacks with vertigo and
ataxia accompanied by dysarthria since the age of 57 years. During the
non-attack intervals the patient had difculties with visual xation of
objects due to a downbeat nystagmus, progressive gait ataxia requiring
the use of a walker and almost daily headaches. Without 4-AP, the
patient predominantly had problems with gait, stance, speech disturbance and coordination of her limbs. 1 hour after the intake of 4-AP,
there was a notable improvement in these symptoms as measured by
the SARA score.
Conclusions: Our case report suggests that 4-AP offers a treatment
option for patients with late-onset EA2 in whom treatment with acetazolamide failed to relieve symptoms. This effect is most likely due to
an increase in the inhibitory inuence of the Purkinje cells caused by
4-AP.
50
Correlation between clinical tests and accelerometry in the assessment of cerebellar tremor in multiple sclerosis
S. Seidel, D. Samal, J. Zezula, K. Vass, E. Auff (Vienna, Austria)
Objective: To evaluate (a) the effect of a 5-HT3 receptor antagonist
on the severity of cerebellar tremor in a patient suffering from multiple
sclerosis and (b) the correlation between clinical tests and accelerometry in the assessment of tremor severity.
Background: Cerebellar tremor is associated with several neurological diseases including stroke and multiple sclerosis. It has been previously shown that cerebellar syndromes are associated with a relative
cerebellar decit of 5-hydroxytryptamine (5-HT, serotonin). Previous
reports demonstrated that symptoms can to some extent be ameliorated
by the administration of 5-HT3-receptor antagonists, although a double-blind placebo-controlled study could not demonstrate a signicant
improvement.
Methods: The severity of tremor was assessed using a clinical test
(9-hole-PEG) and accelerometry before and after treatment with the
5-HT3 receptor antagonist ondansetron (Zofran).
Results: We report on a 34-year-old female patient, whom has been
suffering from secondary progressive multiple sclerosis for nine years
and whose symptoms included severe ataxia. Upon administration of
16 mg ondansetron per day the patient reported subjective improvement of quality of life. Most strikingly, there was a strong correlation
between the 9-hole-PEG test completion time and tremor power at rest;
both methods conrmed the signicant improvement of symptoms. The
medication was well tolerated by the patient.
Conclusions: We recommend combined use of clinical tests and
electrophysiological techniques to assess to efcacy of 5-HT3-receptor
antagonists in the therapy of tremor syndromes in multiple sclerosis.
51
Spinocerebellar ataxia type 7 in Venezuela
M. Gallardo, A. Soto, G. Orozco, M. Camacaro (Caracas, Miranda,
Venezuela)
Objective: To describe the clinical characteristics of a patient with
spinocerebellar ataxia type 7 in Venezuela.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Background: SCA 7 is a progressive autosomal dominant neurodegenerative disorder characterized by cerebellar ataxia with progressive
macular atrophy. SCA 7 is caused by the expansion of an unstable
trinucleotide CAG repeat encoding a polyglutamine tract in the corresponding protein, ataxia-7.
Methods: We reported the case of a 33-year-old man with spinocerebellar ataxia type 7 With a positive family history. He presented with
progressive decreased of visual acuity since three years ago followed
by slowly progressive ataxia and dysarthia. Increased reexes, and
lower limb spasticity, sphincter disturbances and oculomotor abnormalities (Slow saccades and absence optokinetic nystagmus) were
detected on physical examination. His visual acuity was right eye:
20/100 and left eye : 20/70. Extrapyramidal signs and cognitive impairments were not detected in this patient. Fundoscopy showed bilateral macular degeneration. A genetic test was performed that revealed
an expanded allele of 49 CAG repeats at the spinocerebellar ataxia type
7 locus. Brain MRI imaging does not showed atrophy in the cerebellum.
Conclusions: We present a case with SCA 7 with positive genetic
test conrmation. To the best of our knowledge this is the rst case of
spinocerebellar ataxia type 7 described in Venezuela.

52
Usefulness of the scale for assessment and rating of ataxia (SARA)
I. Yabe, M. Matsushima, H. Soma, H. Sasaki (Sapporo, Japan)
Objective: The objective of this study was to examine the usefulness
and validity of SARA as an assessment scale for cerebellar ataxia.
Background: The International Cooperative Ataxia Rating Scale
(ICARS) is widely used as a scale for assessing the severity of cerebellar ataxia and therapeutic effects. However, the application of this
scale in daily examinations of ataxic patients is limited due to the large
number of items to be evaluated. Recently, Schmits-Hubsch et al.
proposed a new scale, the Scale for the Assessment and Rating of
Ataxia (SARA) (Neurology. 2006;66:1717-20). Since the number of
items evaluated on SARA is smaller than that for ICARS, SARA may
be easier to use.
Methods: Subjects were 22 patients with spinocerebellar degeneration. After the chief physician assessed a Barthel index for each patient,
two neurologists examined the same patient. Neurologist one rated the
subjects on ICARS and SARA, and Neurologist two measured the body
sway of the subjects in the upright position by stabilometry {Gravicorder-GS10 (Anima Inc.)} after rating the same patient on SARA.
Results: Few differences were seen between the two neurologists for
the ratings on SARA (P0.843; Wilcoxon signed rank-order test,
correlation coefcient0.927, P0.0001; Pearson correlation analysis). The scores on SARA signicantly correlated with the Barthel
index (correlation coefcient0.620, P0.0016; Pearson correlation
analysis) and scores on ICARS (correlation coefcient0.921,
P0.0001; Pearson correlation analysis). Scores on ICARS (total),
ICARS (posture), SARA (total), and SARA (posture) did not correlate
with the total length traveled (TLT) and the root mean square area
(RMS) of the body sways. The P-value for scores on ICARS (total
P0.103, posture P0.175) was similar to that for scores on SARA
(total P0.169, posture P0.148). The time required to examine each
patient for SARA was approximately four minutes, almost one-third the
time required for ICARS.
Conclusions: Our results indicate that SARA is useful for the evaluation of cerebellar ataxic patients in daily examinations, and that body
sway analysis by stabilometry is inuenced by factors other than
cerebellar ataxia, such as muscle weakness, which should be taken into
account when body sway analysis is used to evaluate the severity of
cerebellar ataxia.

S17

BASIC SCIENCE
53
Vacuous chewing movements are related to striosome-dominant
activity in ventrolateral striatum
B. Bastan, G. Sahin, M. Hayran, E. Saka, B. Elibol (Lund,
Sweden)
Objective: To explore molecular mechanisms underlying tardive
dyskinesia (TD) by correlating behavioral changes and activity-mapping in striatal compartments in an animal model.
Background: TD develops in response to chronic exposure to
neuroleptics; and manifests by persistent and stereotypical involuntary movements. Rats treated chronically with neuroleptics develop
a syndrome of vacuous chewing movements (VCMs) that share
many characteristics of TD. As stereotypies induced by chronic
exposure to dopamine agonists are related to differential effects of
these agents to striatal compartments, a similar relation may exist
between anti-dopaminergic agents induced differential striatal compartmental activity and VCMs.
Methods: Rats were treated either with haloperidol (3mg/kg; i.p
n31) or saline (n 22) for 24 days. VCMs were video-typed for
5 minutes 24 hours after the last injection and counted by blindanalysis. Rats were then sacriced 2 hours after the injection of a
challenge dose of haloperidol (3 mg/kg; i.p.). To map neuronal
activity in different compartments (striosomes and matrix), brain
sections (20 m) were double-stained for FosB and mu-opioid
receptor immunoreactivity. Blinded-raters analyzed the striosomedominance of FosB activity in four striatal regions: dorsolateral,
dorsomedial, ventrolateral and ventromedial. We used a semi-quantitative scale (striosomal pattern score SPS) in which striosomal
dominance was rated from 1 to 5. The correlation of VCMs and SPS
in different striatal regions were carried out with Spearmans test.
Results: VCMs were higher in the haloperidol-treated rats (median:20, IQR: 11-30) compared to the saline-treated ones (median:10,
IQR: 1-18) (p0.003). SPSs were also higher in the haloperidoltreated rats (2.62 0.52 vs 1.99 0.40; p 0.001). The correlation
analysis between VCMs and SPS revealed a statistically signicant
but a moderate correlation in the ventrolateral striatum (p 0.03,
r 0.32).
Conclusions: These results suggest that anti-dopaminergic treatmentinduced repetitive behaviors may generate differential plasticity in
striatal compartments of ventrolateral striatum, as dopaminergic agents
do in dorsolateral striatum.
54
LRRK2 binds to membrane
T. Hatano, S.-I. Kubo, Y. Mizuno, N. Hattori (Bunkyo, Tokyo, Japan)
Objective: To study membrane association of Leucine-Rich Repeat
Kinase 2 (LRRK2).
Background: Parkinson s disease (PD) is the second most common neurodegenerative disease, arising from the selective loss of
the nigral neurons. LRRK2 was identied as a causative gene of
PARK8, one of autosomal dominant forms of PD. The gene encodes
a 2587-amino-acid protein consisting of Ras/GTPase and MAPKKK
domains. Although mutations in the LRRK2 gene give rise to PD,
the role of LRRK2 protein in the disease as well as its normal
function in neurons remains elucidated. Interestingly, most of responsible protein for familial PD have been shown to associate with
membranes despite their lacking of transmembrane domains. Then
we hypothesize that LRRK2 protein associates with membrane.
Methods: Anti-LRRK2 polyclonal rabbit antiserum was raised
against the 18-sequence at the C terminus of LRRK2. Cultured cells
were stained for LRRK2 and several markers of organelles by double
immunouorescence. Furthermore, COS cells were transfected with
GFP-tagged LRRK2 at the N-terminus. For biochemical analysis, we
fractionated cells into membrane and cytosolic fractions. To test
whether LRRK2 binds to lipid rafts, we solubilized cells in 1% Triton

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

X-100 at 4C and separated the remaining membranes on a sucrose

density gradient.
Results: LRRK2 localizes at Golgi apparatus in addition to punctuate
structutres suggestive of cellular vesicles. Furthermore, LRRK2 colocalizes with rafts markers such as CD55 and GM1. Fractionation study
showed that LRRK2 was detected in both cytosolic and membrane
fractions. The membrane association of LRRK2 resists solubilization
by ice-cold 1% triton-X 100, indicating its association through lipid
rafts. Unexpectedly, the mutants are collected in both membrane and
soluble fractions in a manner similar to wild-type. I2020T mutant
LRRK2 associates with lipid rafts, similar to the wild-type.
Conclusions: LRRK2 localizes to membranous orgenelles including
Golgi apparatus. Moreover, LRRK2 associates with lipid rafts. Intriguingly, LRRK2 mutants found in PD patients behave in a manner similar
to the wild-type, with regard to association with membrane including
lipid rafts. The results suggest that LRRK2 mutants cause PD by
inhibiting the normal function of wild-type or gain of function effects
on lipid rafts.
55
The GTPase and kinase activity of the LRRK2 protein are both
required for inclusion formation and cell toxicity in cell culture
models
D. Schweiger, M. van Doeselaar, B. Oostra, V. Bonifati (Rotterdam,
Netherlands)
Background: Mutations in the Leucine-Rich Repeat Kinase 2
(LRRK2) gene are associated with dominantly inherited and sporadic
Parkinsons disease (PD). The LRRK2 gene encodes a large and complex protein, which contains multiple enzymatic and protein-interaction
domains, each of which is targeted by pathogenic PD-associated mutations. The normal cellular function of the LRRK2 protein and the
mechanisms by which the PD-associated mutants are disease causing
remain mostly elusive.
Methods: We showed previously that in cell lines of various origins,
overexpressed LRRK2 forms cytoplasmatic juxtanuclear inclusions,
which are strongly stained by LRRK2 antibodies, and share morphological and biochemical characteristics with aggresomes.
Results: Using HEK293 cells, we have now overexpressed EGFPtagged, wild type LRRK2, and several PD-associated mutants as well
as GTPase-dead and kinase-dead LRRK2 mutants. We observed that
PD-causing mutants located in the different functional domains of
LRRK2 (including R1441C, Y1699C, and G2019S) consistently and
signicantly increase the formation of inclusions compared to wild type
LRRK2, and this phenomenon is associated with increased cellular
toxicity triggered by the stress-inducing reagent hydrogen peroxide.
Furthermore, ablating the LRRK2 GTPase or the kinase activity markedly and signicantly decreases the formation of inclusion bodies and
apoptosis induced by LRRK2 overexpression in HEK293 cells.
Conclusions: These ndings suggest that both the catalytic LRRK2
domains (GTPase and kinase) are involved in mediating the cellular
effects of overexpressed LRRK2.
56
Potency of CNBTX-A substantially exceeds labeled units in standard potency test
T. Hunt, K. Clarke (Irvine, California, USA)
Objective: To assess the potency of CNBTX-A, a botulinum toxin
type A product labeled as produced by Nanfeng Medical Science and
Technology Development Company of Shijiazhuang, China.
Background: Units of botulinum neurotoxin products from different
manufacturers are not equal. An understanding of the potency and
dosing of each individual product is critical to ensure safe and effective
clinical use. The CNBTX-A label indicates 55 U per vial.
Methods: A single lot of unexpired, commercially-available CNBTX-A was tested for potency in the assay used for Botox potency
release testing and for activity in a botulinum neurotoxin light-chain
assay. For potency testing, mice were injected intraperitoneally with
various dilutions of CNBTX-A alongside a Botox potency reference

Movement Disorders, Vol. 22, Suppl. 16, 2007

standard, and outcomes were recorded. Potency estimates were obtained using the probit analysis method for quantal responses. For the
light-chain assay, a SNAP-25 substrate was exposed to various dilutions of CNBTX-A or 100 U Botox as a control, and the amount of
cleavage product generated was measured.
Results: Initial potency tests of CNBTX-A, using the standard reconstitution volume, indicated that the potency was higher than the
upper detection limit of the assay. Subsequent re-testing using additional dilutions post reconstitution showed that the potency of CNBTX-A was calculated to be 243 U per vial. The light-chain assay also
indicated a substantially higher activity of CNBTX-A than the Botox
control. The light-chain assay also served as an identication test,
conrming that the CNBTX-A contained botulinum toxin-like activity;
i.e., specic SNAP substrate cleavage.
Conclusions: The potency of CNBTX-A measured 4.4 times higher
than the labeled 55 U per vial. This result was conrmed by a lightchain activity assay. These ndings indicate a potentially dangerous
deviation of CNBTX-A from expected potency, underscoring that units
of botulinum toxin products from different manufacturers are unique
and not interchangeable. Neither package insert nor dosing recommendations came with the CNBTX-A shipment.

57
MPTP-lesioned mouse model of the beginning-of-dose inhibitory
effect in Parkinsons disease
S.A. Gunzler, S. Shakil, N.E. Carlson, J.G. Nutt, C.K. Meshul
(Portland, Oregon, USA)
Objective: Create an MPTP-lesioned mouse model of the beginningof-dose inhibitory effect (BIE), by administering subthreshold doses of
apomorphine (APO), a D1/D2 dopamine agonist.
Background: Parkinsons disease (PD) patients often note a deterioration of motor function to below their usual baseline. The beginningof-dose inhibitory effect (BIE) is a decline of motor function to below
baseline, during the rising phase of the levodopa concentration curve.
This occurs after taking antiparkinsonian medications and before there
is drug-induced motor improvement. Previous studies did not consistently demonstrate BIE in MPTP-lesioned mice. We sought to determine if this pattern of response to dopaminergic agents can be seen in
Parkinsonian mice.
Methods: This was a randomized placebo-controlled study of BIE in
C57 black mice. One week after acute MPTP lesioning, 24 mice were
randomized to receive an intraperitoneal (IP) injection of placebo, APO
0.05mg/kg, APO 0.1mg/kg, or APO 0.2 mg/kg, then were placed in
locomotion chambers. We recorded locomotion (week 1) for 20 minute
time intervals, over 2 hours. Mice then received daily suprathreshold
(6mg/kg) levodopa for 1 week. After the week of levodopa, they again
received the same study drug (placebo, APO 0.05, 0.1, or 0.2mg/kg),
and were placed back into the locomotion chambers (week 2). ANOVA
was used to assess whether average activity in the rst 20 minutes
depended on apomporphine dose. A mixed effects model was used to
assess if the dose effect at 20 minutes differed after 1 week of levodopa,
to determine the effect of levodopa on the severity of BIE.
Results: One mouse was excluded from the 0.2mg/kg week 2 analysis, due to inactivity. Locomotion in the 0.1 and 0.2mg/kg groups was
signicantly decreased relative to placebo at the 20-minute timepoint.
The 0.1mg/kg groups locomotion rose above placebo at the 100minute timepoint on week 1, and above both placebo and the
0.05mg/kg group at the 100 and 120-minute timepoints on week 2.
There was no signicant difference in locomotion from week 1 to week
2.
Conclusions: Low doses of APO can initially depress locomotion
and subsequently stimulate locomotion, in a manner similar to what is
seen in some PD patients with BIE. This animal model may be useful
for exploring mechanisms behind BIE and methods to ameliorate it.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

S19

mg/kg, i.p.), or veh, was administered to mice for 5 days. From the second
day of MPTP, mice were treated with either PYM50028 (10 mg/kg, p.o.)
or veh once daily. On day 60, animals were killed and THve nigral cells
quantied by immunocytochemistry.
Results: MPP resulted in signicant loss of THve cells per eld
(55.33.2%) cf cultures not exposed to MPP (1003.2%). Pre-treatment
for 24h with PYM50028 (30nM) signicantly prevented loss of THve
cells (PYM50028, 97.93.6%). Exposure to MPP for 24h resulted in
signicant loss of THve cells per eld (75.84.0%, cf cultures not
exposed to MPP 1005.8%), that was signicantly reversed by treatment with PYM50028 (30nM) for 48h (PYM50028: 113.44.6%). On
day 60, a signicant decrease in nigral THve cells was apparent in
MPTP-lesioned animals treated with veh (22.22.2 THve cells, P0.01
cf non-MPTP-lesioned animals 38.63.3 THve cells). This decrease was
not apparent following PYM50028 (34.43.5 THve cells, P 0.05 cf
MPTP-lesioned animals and NS cf non-MPTP-lesioned animals).
Conclusions: These data demonstrate that PYM50028 prevents and
reverses dopaminergic neuronal loss in vitro and in vivo, suggesting
that PYM50028 is a promising future treatment for PD.
59
Evaluation of gastrointestinal function in a mouse model of Parkinsons disease
G. Anderson, G. Taylor, D. Bernhard, M. Anitha, S. Srinivasan,
J.G. Greene (Atlanta, Georgia, USA)

FIG. 1 (57).

58
PYM50028, a novel, orally active neurotrophic factor inducer,
protects and reverses the neuronal damage induced by MPP in
mesencephalic neuronal cultures and by MPTP in a mouse model
of Parkinsons disease
N.P. Visanji, T.H. Johnston, N. Callizot, A. Orsi, D. Rees,
J.M. Brotchie (Toronto, Ontario, Canada)
Objective: The present study investigated the ability of PYM50028,
a novel, orally active, non-peptide neurotrophic factor inducer, in
preventing and reversing dopaminergic neuronal loss in mesencephalic
cultures and MPTP-lesioned mice.
Background: We have previously shown that PYM50028 can protect
against striatal dopamine transporter loss in the MPTP lesioned mouse.
Methods: To assess the ability of PYM50028 to prevent MPP-induced
neuronal loss in vitro, rat primary mesencephalic neuronal cultures were
incubated for 24h with vehicle (veh) or PYM50028, followed by the
addition of veh or MPP (2M) for 48h. The number of DA neurons per
eld was quantied by tyrosine hydroxylase (TH) immunocytochemistry.
To assess the ability of PYM50028 to reverse MPP-induced neuronal
loss, cultures were rst exposed to veh or MPP for 24h, followed by
incubation with veh or PYM50028 for 48h. To assess the ability of
PYM50028 to prevent MPTP-induced neuronal loss in vivo MPTP (25

Objective: To evaluate gastrointestinal motility in the MPTP mouse

model of Parkinsons disease.
Background: Gastrointestinal dysfunction is a prominent nonmotor
feature of PD, causing symptoms such as nausea, bloating, and constipation. In addition, gut dysmotility complicates the clinical management of PD by causing erratic medication absorption, which can lead to
uctuations in motor symptoms. The underlying neuropathology of
gastrointestinal dysfunction in PD may be in either the intrinsic enteric
nervous system (ENS) of the gut or in central regions associated with
alimentary motility, such as the dorsal motor nucleus of the vagus.
Elucidation of the pathophysiology of gut dysmotility in PD and the
development of effective treatments has been hampered by lack of
investigation into animal models of GI symptoms.
Methods: Mice were injected intraperitoneally with 60 mg/kg of MPTP
(1-methyl-4-phenyl 1,2,3,6-tetrahydropyridine). Ten days later, we measured gastric emptying, small intestinal transit, stool frequency, and colonic muscle contractility. In addition, sections of distal ileum were immunostained to examine tyrosine hydroxylase (TH), choline acetyl
transferase (ChAT), and NADPH-diaphorase-positive enteric neurons.
Results: MPTP-treated animals showed transiently increased onehour stool frequency, increased contractility of colonic smooth muscle,
and a reduced number of TH-positive neurons.
Conclusions: The results indicate that loss of inhibitory dopaminergic neurons in the myenteric plexus induced by MPTP causes reduced
inhibition of gut smooth muscle contractility and accelerated colon
transit. Thus, as in the brain, MPTP is a dopaminergic neurotoxin in the
enteric nervous system, but GI symptoms in MPTP-treated mice do not
correlate with symptoms typically observed in PD.
60
Stability of Xeomin, a preparation of botulinum neurotoxin type
A, free of complexing proteins
S. Grein, G.J. Mander, S. Grafe (Frankfurt, Germany)
Objective: Xeomin is a botulinum neurotoxin type A formulation,
which, in contrast to other commercially available BTX-A preparations, only contains the pure 150 kD neurotoxin without complexing
proteins. Even though the product may be stored for up to three years
without refrigeration, and comprehensive long-term stability studies
have shown no detrimental effects on the quality of Xeomin at
elevated temperatures, no stability data are yet available for short-term
temperature stress. The study presented here is therefore focusing on
the inuence of temperatures above 40 C on the quality of Xeomin.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: Native botulinumtoxin type A (BTX-A) is a high

molecular weight complex of approximately 900 kD, which is composed of the biologically active 150 kD neurotoxin and several hemagglutinins and other nontoxic proteins. Although these accessory
proteins do not have any neurotoxic effect, the absorption/uptake of
botulinum neurotoxin type A from the gut appears to be strongly
facilitated in the presence of these complexing proteins. It has therefore
been speculated that one of the functions of these complexing proteins
is to protect the 150 kD neurotoxin from harsh environmental conditions, and that the puried neurotoxin might thus be highly susceptible
to degradation and/or inactivation.
61
Modulation of Akt signaling pathway by the interaction of DJ-1
with PTEN
C.Y. Kim, H. Kitaura, S.M.M. Iguchi-ArigaSanae, H. Ariga
(Sapporo, Hokkaido, Japan)
Objective: To evaluate the function of DJ-1 in the AKT signaling
pathway, interaction of DJ-1 with PTEN and phosphorylation status of
AKT were examined under the oxidative condition.
Background: DJ-1 is a multi-functional protein that plays roles in
transcriptional regulation and anti-oxidative stress, and loss of its
function is thought to result in onset of Parkinsons disease. DJ-1 has
been known to be a negative regulator of PTEN, a tumor suppressor
that negatively regulates the AKT signaling. The AKT signal promotes
growth of cells, including nerve cells.
Methods: To examine the interaction of DJ-1 with PTEN, proteins in
stable cell lines expressing Flag-tagged wild-type (wt) DJ-1 and
C106S DJ-1 were immunoprecipitated with an anti-Flag antibody and
the precipitates were analyzed with an anti-PTEN antibody. Phosphatase activity of PTEN and the phosphorylation level of AKT were also
examined in these cells into which H2O2 had been exposed.
Results: We found that both wt and C106S DJ-1 were associated
with PTEN in cells but that afnity of C106S DJ-1 to PTEN was
stronger than that of wt-DJ-1. The direct bindings of wt- and C106SDJ-1 to PTEN were conrmed by pull-down assays using recombinant
proteins. Stable cell lines were then treated with 1mM H2O2 for 5 and
15 minutes and phosphorylated and oxidative status of proteins were
examined. While the phosphorylation level of AKT was increased and
then decreased with time in wt DJ-1-containing cells concomitant with
reduced phosphatase activity of PTEN and with pI shifts of DJ-1 which
is a factor determining PTEN activity, was not changed in both cells.
Using puried recombinant DJ-1 and PTEN, it was found that C106S
DJ-1 suppressed PTEN phosphatase activity much more than did Wt
DJ-1 and that the pI of wt DJ-1 but not C106S-DJ-1 changed to acidic
points after treatment of proteins with H2O2. Co-localization of DJ-1
with PTEN was also changed with time in cells treated with H2O2.
Conclusions: These ndings indicate that suppression of PTEN
activity by DJ-1 activated the PKB/Akt signaling pathway and that the
oxidized level of DJ-1 at C106 affects activation of the pathway.
62
Decreased expression of alpha-synuclein in Parkinsons disease:
Multiple-level evidence
S. Papapetropoulos, N. Adi, L. Shehadeh, J. Ffrench-Mullen,
N. Bishopric, D.C. Mash (Miami, Florida, USA)
Objective: To investigate the expression of alpha-synuclein mRNA
and protein in human tissue (brain and blood) and in vitro models of
Parkinsons disease (PD).
Background: SNCA, the gene encoding the presynaptic protein alpha-synuclein, was the rst gene in which mutations were found to
cause PD. An increased copy number and elevated expression of
wild-type alpha-synuclein has been shown to cause early-onset familial
PD. However, it is not clear whether increased alpha-synuclein expression also plays a role in the pathogenesis of sporadic disease. Recent
studies have reported a decrease in alpha-synuclein mRNA and protein
levels in human postmortem brain, plasma and cerebrospinal uid from
PD sporadic patients.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Methods: For SNCA gene mRNA expression we utilized microarray

data from our previously published gene expression proling study [1]
and from the NCBI Gene Expression Omnibus (GEO) at http://www.
ncbi.nlm.nih.gov/geo/. We analyzed raw microarray data (cel les)
from available datasets in human postmortem brain (21 chips), peripheral blood (105 chips) and an in-vitro model of rotenone toxicity, (21
chips) in GeneSpring 7.2 (Agilent Technologies). Independent validation at the mRNA and protein levels was performed in the SN of PD
and control subjects by RT-PCR, ELISA and Western blotting.
Results: Available microarray data (substantia nigra, blood, 4wk
rotenone-treated cells) revealed decreased expression of at least one
alpha-synuclein transcript in PD patients (Figure 1). Independent validation studies in postmortem SN from 18 PD patients at different
disease stages (M:F 11:7; mean age at onset 63.011.25 years) and 8
controls (M:F 3:5; age at death 80.08.6) revealed a signicantly
decreased 70% expression of alpha-synuclein mRNA (Fold Change
0.320.15) and protein compared to controls.
Conclusions: Although it is established that alpha-synuclein overexpression may lead to PD neurodegenerative changes, it is possible that
signicant under-expression may have similar effects. Alternatively
one might suggest that in some cases alpha-synuclein aggregation is a
secondary rather than a primary effect. We suggest that conicting
results in alpha-synuclein expression may reect the intricate and
complex role of alpha-synuclein in the pathogenesis of PD.
1. Papapetropoulos S, et al. Gene Expr 2006;13(3):205-15.

FIG. 1 (62).

63
Mutant forms of parkin cause protein aggregation, alterations of
the ubiquitin-proteasome system and neuronal death in human
neuroblastoma cells
E. Kyratzi, M. Pavlaki, D. Kontostavlaki, H.J. Rideout, L. Stefanis
(Athens, Attiki, Greece)
Objective: To assess the effects of wild type (WT) and mutant Parkin on
the ubiquitin-proteasome system (UPS) and on neuronal cell survival.
Background: Parkin is an E3 ubiquitin ligase implicated in the UPS.
Mutations in Parkin are associated with autosomal recessive juvenile
parkinsonism and may manifest as loss-of-function mutations. However,
heterozygote patients have also been identied. Overexpression of Parkin
has been touted as a neuroprotective strategy. However, the consequences
of Parkin overexpression on the UPS have not been well characterized.
Methods: A human neuroblastoma (SH-SYSY) cell line stably expressing GFPu, an articial substrate of the UPS, was used as a reporter for UPS
function. Transient transfections were performed with myc-tagged WT
and mutant Parkin or with a control protein and expression was tested by
Western immunoblotting. GFPu levels were evaluated by green uorescence and Western immunoblotting. Apoptotic nuclei were determined by
labelling with Hoechst. Immunouorescence was performed using antibodies directed against myc, ubiquitin, parkin and proteasomal subunits.
Proteasomal activity was measured in SH-SY5Y cells, co-transfected with
EGFP and WT or mutant parkin, and sorted for green uorescence.
Results: All disease-associated mutants tested, but not the WT protein,
formed Myc-positive aggregates. Both WT and mutant Parkins induced
focal GFPu accumulation, but GFPu and ubiquitin colocalized with aggregated myc only in the case of the mutants. Mutant forms also induced
apoptotic death, which correlated well with their propensity to aggregate.
Moreover, specic mutants caused a decrease in chymotrypsin-like proteasomal activity, in contrast to the WT protein, which showed increased
activity. The reduction of proteasomal activity induced by the mutants
could at least in part be attributed to caspase activation.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Conclusions: Mutant forms of Parkin can exert toxic effects on neuronal
cells, possibly through their propensity to aggregate. Both WT and mutant
forms can induce localized UPS dysfunction, likely through different
mechanisms. This raises a note of caution regarding forced overexpression
of Parkin as a neuroprotective strategy in PD and suggests a possible toxic
gain of function for certain mutant forms of Parkin.
64
Reex control of jaw movement
K.S. Turker (Adelaide, SA, Australia)
Objective: The principal aim of this study was to discover the reex
control of jaw movements in human volunteers. Our specic aim was
to investigate the contribution of the skin/mucosal, periodontal and
muscle spindle receptors to the jaw muscles during simulated mastication.
Background: Reex control of jaw movement has been studied in
detail in experimental animals where direct recording from nerves and
motoneurons is possible. However, human work on this topic has only
used static conditions where the jaw did not move due to the difculty
in standardizing the stimuli. Therefore, changes in the contribution of
various orofacial receptors to the motoneurons that innervate jaw
muscles are not known.
Methods: Fifteen young healthy volunteers masticated on a simulator (Turker et al, 2004; Journal of Neuroscience Methods; 136:141149) and mechanical stimuli were delivered to the upper incisor tooth
or electrical stimuli were delivered to the lower lip each time the jaw
passed through a dened degree of jaw separation. Application of the
stimuli was repeated after locally anaesthetizing the involved teeth to
dissect the contribution by the periodontal receptors.
Results: We found that the inhibitory effects of the periodontal and
mucosal/skin receptors decrease during jaw opening and stay the same or
increase during jaw closing. On the other hand, the excitatory effects of
muscle spindles decrease during opening and increase during jaw closing.
Conclusions: Modulation of jaw reexes has been described. The
inhibitory reexes that are normally activated by rapidly rising forces
on the teeth and/or mucosa/skin, preserve their strengths during jaw
closure and hence help protect the teeth and supporting structures from
becoming damaged. The excitatory reexes also preserve their
strengths during jaw closure and hence help develop stronger forces
between the teeth to break the food stuff into pieces. Therefore,
depending on the receptor stimulated either the jaw stops or continues
to crush the bolus.
65
No age-related loss or morphological changes in nigral neurons of
substantia nigra pars compacta of normal Indian human brains: A
stereological study
P.A. Alladi, A. Mahadevan, T.C. Yasha, T.R. Raju, S.K. Shankar,
U. Muthane (Bangalore, Karnataka, India)
Objective: Stereological quantication and morphometric analysis of
nigral neurons in substantia nigra pars compacta of Indian human brains.
Background: Differential prevalence of Parkinsons disease between
races has been linked to variation in absolute number of melanized
neurons of substantia nigra. Several studies have reported age-related
loss of melanized neurons in Caucasians indicating a gradual loss of
nigrostriatal function. However, this loss was not observed in Indian
brains. Studies in Caucasians have been conducted either on a singlesection at emergence of the oculomotor nerve or using stereology while
in Non-Caucasians (Indians, Nigerians) they were using a single section. Stereological quantication is unbiased and therefore accurate.
Methods: In the present study we quantied volume, neuronal density, number of melanized and borderline/non-melanized and TH positive neurons as also neuronal nuclear and soma area in substantia nigra
pars compacta (SNpc) of Indian brains (n26, 28GW-69 years) using
stereology and image analysis.
Results: There was no decrease in number of melanized neurons with
increasing age. We conrmed the absence of apoptotic neuronal loss
using TUNEL technique. Moreover, neither there was any age-related
reduction in volume or neuronal density (r value 0.064-0.165) nor we

S21

observed any change in neuronal nuclear area or soma size. Comparison with a study from Chicago, USA (Chu et al, 2002; n19) revealed
comparable SNpc volume yet higher neuronal density in Indians.
However a further comparison of neuronal counts appears inappropriate due to possible difference in the sampled area.
Conclusions: Thus using stereology, we conrm that unlike Caucasians there is probably no age-related gradual loss of nigrostriatal
function in Indians. This could be due to genetic, environmental or
dietary differences between racial groups. Further studies on molecular
mechanisms involved in cell survival are required to explain these
quantitative differences in the SNpc of different ethnic populations.
66
Expression of MT1 & MT2 receptors in human postmortem amygdala and substantia nigra of Parkinsons disease and controls
subjects
N. Adi, L. Shehadeh, D.C. Mash, C. Singer, S. Papapetropoulos
(Miami, Florida, USA)
Objective: The aim of the present study was to determine the MT1 and
MT2 receptors expression in whole tissue postmortem amygdala and
substantia nigra (SN) of PD patients and non-neurologic control subjects.
Background: Melatonin (N-acetyl-5-methoxytryptamine) is primarily synthesized by the pineal gland and secreted into the blood circulation and cerebrospinal uid in a circadian fashion. Melatonin has
antioxidative, neuroprotective, vasoactive, immunological and oncostatic properties. The melatonin receptor 1 (MT1) and melatonin receptor 2 (MT2) are expressed throughout the human CNS in distinct
cell populations. The putative role of melatonin in the dopaminergic
system has been suggested based on studies demonstrating the effect of
melatonin on dopamine content, turnover and release as well as on
dopamine receptors. Several reports from animal models of Parkinsons
disease (PD) have demonstrated improvement of disease-related phenotypes and neuronal rescue after administration of melatonin.
Methods: Expression of MT1 & MT2 mRNA in amygdala & substantia
was characterized by real time PCR using primers and probes Assay IDs
(MT1: Hs_00195567_m1; MT2: Hs_00173794_m1) and the results were
normalized with 18s rRNA as an endogenous control (18s rRNA:
Hs_99999901_s1) from Applied Biosystems (Foster City, CA).
Results: We studied a total of 15 PD patients (Males:Females; mean
age at onset 63.0 years and mean age at death: 78.387.0; mean
disease duration 15.07.45 years) and 8 controls (Males:Females; 3:5;
age at death 80.08.6). Both MT1 and MT2 receptor expression was
signicantly down-regulated in PD patients relative to controls (Fold
Change: Amygdala 0.350.26; SN 0.160.16) in both regions.
Conclusions: Our study showed signicant down-regulation of both
MT1 & MT2 receptor gene expression in both amygdala and SN
regions of PD patients. Alterations in melatonin receptor expression
may affect melatonins benecial effects in PD. The antagonism of
melatonin, using methods such as constant light and pinealectomy, may
alleviate the symptoms during the more advanced stages of disease in
animals and humans. Future studies are needed to elucidate the role of
melatonin and its receptors in the treatment/cause of PD.
67
Human uncoupling-protein-4 protects neuronal cell death from
MPP induced toxicity by regulating mitochondrial membrane
potential, reducing generating ROS and maintaining ATP levels
C.Y. Chu, W.L. Ho, H.H. Kwok, Y.J. Wang, D.B. Ramsden, S.L. Ho
(Hong Kong, China)
Objective: To investigate the effects of UCP4 overexpression on
mitochondrial functions and oxidative stress in SH-SY5Ycells.
Background: Mitochondrial dysfunction, ATP deciency, and oxidative stress are associated with neuronal cell death in Parkinsons
disease (PD). UCP4, a novel member of uncoupling proteins, is exclusively expressed in the brain, but its function is unclear. We hypothesize that UCP4 is involved to regulate neuronal energy homeostasis by
modulating mitochondrial membrane potential and ATP production.
Methods: A full-length human UCP4 was stable expressed in
SHSY5Y cells. Subcellular localization of UCP4 was examined by

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

immunocytochemistry and Western analysis after cytosolic and mitochondrial fractionation. Stable lines and vector were incubated for 24hr
with either MPP (0.5mM, 1mM). Cytotoxicity was measured by 3H
thymidine incorporation assay. ATP levels were determined by luciferase-luciferin bioassay. MMP and oxidative stress were measured by
FACS after staining of JC-1 and DHE, respectively.
Results: Recombinant UCP4 is exclusively expressed in the mitochondria in SH-SY5Y cells. No difference in cell viability was observed among
the vector or UCP4-expressing constructs. After exposure of 0.5mM and
1mM MPP for 24 hours, UCP4 expressing cells showed 15% higher of
survival rate compare with the vector control. Vector control was incubated MPP for 24hr induced signicant oxidative stress, whereas UCP4
overexpressing cells did not. The relative mitochondrial membrane potential of UCP4 overexpressing group with 1.0 mM MPP was signicantly
restored towards levels seen in vector control with MPP group by 195%
. In addition, the intracellular ATP level in UCP4 expressing cells after
treated with MPP is signicantly higher than that in the vector control
treated with MPP in 38%.
Conclusions: We report that UCP4 can protect cell death from MPP
induced toxicity by restoring the depolarization of MMP, reducing production of ROS, and maintaining intracellular ATP levels. These ndings
might contribute to the understanding of physiological functions of neuronal UCP4 and provide insights on therapeutic possibilities against neurodegenerative diseases involving mitochondrial dysfunction.
68
Role of Neu4L sialidase and its substrate ganglioside GD3 in
neuronal apoptosis induced by catechol metabolites
T. Hasegawa, N. Sugeno, A. Takeda, M. Matsuzaki-Kobayashi,
A. Kikuchi, K. Furukawa, T. Miyagi, Y. Itoyama (Sendai, Miyagi,
Japan)
Objective: To explore possible roles of Neu4L sialidase in programmed cell death induced by catechol metabolites.
Background: Mammalian sialidases are key enzymes in the degradation of glycoconjugates. Neu4L sialidase is localized to mitochondria
and specically expressed in brain.
Methods: To elucidate the pathophysiological roles of Neu4L in the
nervous system, we investigated the possible involvement of Neu4L in
the apoptotic neurodegeneration under the existence of catechol metabolites generated by tyrosinase.
Results: We demonstrated that (i) the expression level of Neu4L was
dramatically decreased prior to apoptosis, (ii) the apoptotic phenotype was
characterized by cytochrome c release into cytosol concomitant with the
trafcking of ganglioside GD3 to mitochondria, and (iii) the inhibitor of
glucosylceramide synthase partially recovered cell viability.
Conclusions: Neu4L and its substrate GD3 may act as key molecules
in the mitochondrial apoptotic pathway in neuronal cells.
References:
1) Hasegawa T., Matsuzaki-Kobayashi M., Takeda A., Sugeno N.,
Kikuchi A., Furukawa K., Perry G., Smith MA., Itoyama Y.,
Synergistic interaction between -synuclein and oxidized catechol metabolites produced by tyrosinase: Implications for selective neurodegeneration in Parkinsons disease. FEBS Lett. 580:
2147-2152, 2006.
2) Furukawa K., M-Kobayashi M., Hasegawa T., Kikuchi A.,
Itoyama Y., Wang Y., Yao PJ., Bushlin I., Takeda A., High ion
permeability of plasma membrane caused by the -synuclein
mutations contributes to cellular degeneration. J. Neurochem.
97:1071-1077, 2006.
3) Takeda A., Hasegawa T., Matsuzaki-Kobayashi M., Sugeno N., Kikuchi A., Itoyama Y., Furukawa K., Mechanisms of neuronal death in
synucleinopathy. J. Biomed. Biotechnol. 2006(3):19365, 2006.
4) Hasegawa T., Sugeno N., Takeda A., Matsuzaki-Kobayashi M.,
Kikuchi A., Furukawa K., Miyagi T., Itoyama Y., Role of Neu4L
sialidase and its substrate ganglioside GD3 in neuronal apoptosis
induced by catechol metabolites. FEBS Lett. 581:406-412, 2007.

Movement Disorders, Vol. 22, Suppl. 16, 2007

69
Vesicular dysfunction may trigger dopaminergic cell death
M. Kobayashi, T. Hasegawa, A. Takeda, N. Sugeno, Y. Itoyama
(Sendai, Miyagi, Japan)
Objective: To elucidate cellular mechanism of dopaminergic degeneration in Parkinsons disease.
Background: Parkinsons disease is characterized by selective cell
death of nigral dopaminergic neurons. Dopamine and its metabolites
have been implied to be potentially toxic in dopamine neurons. However, the detailed pathomechanisms are still unrevealed.
Methods: We established neuronal cell line expressing tyrosine
hydroxylase under the control of Tet-On/Off system. In this system,
dopamine generation can be controlled by the Tet-regulated tyrosine
hydroxylase expression. Under the dopamine overproduction, cell viability was evaluated by MTT assay with the exposure of oxidative
stresses (FeCl2 and rotenone). Furthermore, the cells were treated with
reserpine, vesicular monoamine transporter 2 (VMAT2) inhibitor, and
changes in vulnerability were also evaluated by MTT assay.
Results: Although dopamine overproduction itself did not reduce cell
viability, treatment with the VMAT2 inhibitor induced increased cellular vulnerability against oxidative stresses.
Conclusions: These data suggested that dysfunction of dopamine
vesicles led to dopaminergic cell death possibly by the increased
cytosolic catecholamines.

70
Glucocerebrosidase mutations promote -synuclein aggregation
O. Goker-Alpan, D. Urban, B.K. Stubbleeld, M.R. Cookson,
E. Sidransky (Bethesda, Maryland, USA)
Objective: To explore the cellular basis of parkinsonian phenotype
associated with glucocerebrosidase mutations.
Background: Synucleinopathies are a group of disorders characterized by aberrant -synuclein brillization, leading to neuronal cell
death. Recent evidence suggests an association between mutations in a
lysosomal protein, glucocerebrosidase (GBA), and adult-onset progressive nervous system degeneration that encompasses the pathologic
spectrum of synucleinopathies.
Methods: cDNA constructs of GBA missense mutations, N370S and
L444P were generated using site directed mutagenesis. Wild-type and
mutant GBA were transiently expressed in Cos-7 cells transfected with
hA53T -synuclein with a CMV vector promoter. Immunouorescence
studies and confocal microscopy were performed to detect inclusion
formation. Synuclein solubility was assessed in cell lysates with detergent extraction using 1% Triton X-100, and standard Western blotting
technique. Immunoprecipitations were carried out with agarose immobilized anti- -synuclein antibodies following S-35 Cys/Met pulse
chase experiments.
Results: Co-expression of hA53T -synuclein and GBA mutants
induced spontaneous aggregation of synuclein with formation of both
small punctuate and large juxta-nuclear LB-like inclusions, immunoreactive to glucocerebrosidase. Synuclein aggregates were reactive to
aggresome markers and altered vimentin distribution. Furthermore, the
microtubule depolarazing drug nocadozole inhibited aggregate formation, indicating that the inclusions represented true aggresomes. In cell
lines overexpressing mutant GBA, -synuclein levels in soluble fraction was low and the half life was prolonged.
Conclusions: Mutant GBA promotes synuclein aggregation and may
also interfere with effective -synuclein clearance in the lysosomes,
which could account for an increased risk of synucleinopathy associated with glucocerebrosidase mutations.

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71
Leucine-rich repeat kinase 2 binds to lipid rafts in synaptic terminals
S.-I. Kubo, T. Hatano, Y. Mizuno, N. Hattori (Bunkyo, Tokyo, Japan)
Objective: To elucidate localization of Leucine-Rich Repeat Kinase
2 (LRRK2) in mouse brain and cultured cells and to investigate the
effect of mutations on the biochemical properties of LRRK2.
Background: LRRK2 is a causative gene for the autosomal dominant
form of Parkinsons disease (PD). The gene encodes the 280 kDa
LRRK2 protein composed of domains such as leucine-rich repeats, Roc
(Ras in complex proteins) followed by COR (C-terminal of Roc),
mitogen-activated protein kinase kinase kinase (MAPKKK), and
WD40. However, the normal function of the protein as well as its
contribution to the pathogenesis of PD remains largely unknown. Since
LRRK2 is implicated in the pathogenesis of PD with pleomorphic such
as synucleinopathy and tauopathy, characterization of LRRK2 should
enhance our understanding of the pathomechanism of not only LRRK2linked PD but also neurodegenerative diseases other than PD.
Methods: Anti-LRRK2 polyclonal rabbit antiserum was raised against
the 18-sequence at the C terminus of LRRK2. We performed doublelabeled for both LRRK2 and several markers of organelles in mouse
primary neurons. For biochemical analysis, mouse brain extracts were
fractionated by differential centrifugation. To investigate whether lipid
rafts contribute to the membrane association of LRRK2 in synaptic terminals, we solubilized mouse synaptosomes in 1% Triton X-100 at 4C and
separated the remaining membranes on a sucrose density gradient.
Results: Endogenous LRRK2 localized to Golgi apparatus, plasma
membrane, and synaptic vesicles in mouse primary neurons. The membrane association of LRRK2 resists solubilization by ice-cold 1%
Triton X-100, indicating its association through lipid rafts in mouse
brain. Unexpectedly, I2020T mutant LRRK2 colocalizes with synaptic
vesicles and lipid rafts, similar to the wild-type.
Conclusions: LRRK2 localizes to Golgi apparatus and synaptic vesicles
in mouse primary neurons. Moreover, LRRK2 associates with lipid rafts
which are known to play an important role in cellular functions such as
signal transduction, membrane trafcking, and cytoskeletal organization.
These ndings suggest that LRRK2 should play important roles in membrane trafcking system in synaptic terminals.
72
Pleiotrophin over-expression after intrastriatal and intranigral administration of a recombinant adenoviral vector containing human
pleiotrophin cDNA
I.R. Taravini, M. Chertoff, E. Cafferata, G.M. Murer, F. Pitossi,
O.S. Gershanik (Capital Federal, Buenos Aires, Argentina)
Objective: 1) To generate a recombinant adenoviral vector expressing human pleiotrophin (PTN), 2) to induce PTN over-expression in the
striatum and substantia nigra pars compacta (SNpc) of normal rats.
Background: Clinical signs of Parkinsons disease (PD) are not
evident until 50% of SNpc neurons have died and up to 90% dopaminergic neurons die despite treatment. Therefore, there is strong interest
in research on PD cause and on the development of novel therapeutic
strategies aimed at preventing the degenerative process or restoring the
structure and function of the damaged nigroestriatal system. PTN is a
neurite outgrowth-promoting factor expressed during embryonic and
early postnatal development. Furthermore, it is believed that PTN
promotes survival and differentiation of dopamine neurons from embryonic stem cells. Recently, we found an over-expression of PTN in
the striatum of adult rats with a unilateral 6-OHDA lesion of the
nigrostriatal tract chronically treated with levodopa.
Methods: We generated a recombinant adenovirus that expresses
PTN (AdPTN). The human PTN cDNA was cloned into a shuttle vector
with a human cytomegalovirus promoter and cotransfected into
HEK293 cells with a plasmid containing E1 to E3 deleted type 5
adenoviral genome using a calcium phosphate coprecipitation method.
Stocks of AdPTN were obtained from large-scale preparations in 293
cells and then puried by CsCl gradients. Final titer: 2.15109 pfu/l.
AdPTN was injected in the striatum or SNpc of adult rats (1107

S23

pfu/l/rat). Seven days after that, PTN was detected by immunohistochemistry on brain tissue sections.
Results: In control animals injected with an adenovirus expressing
-galactosidase, PTN expression was restricted to striatal interneurons
and a few neurons in the SNpc. AdPTN was effective in inducing PTN
over-expression. Preliminary analysis suggests that both glia and neurons were over-expressing PTN.
Conclusions: We have constructed a PTN-containing adenovirus
which successfully over-expressed PTN in vivo. This adenovirus will
be useful to increase our understanding of the functions of PTN in the
adult nigrostriatal innervation, and in particular, help us to determine
the potential of PTN as a preventive or restorative therapy for PD.
73
Selective suppression of REM sleep in MPTP non-human primates: A long term continuous electroencephalographic study by
telemetry
V. Lambrecq, C. Forni, F. Tison, E. Balzamo, B. Bioulac,
I. Ghorayeb (Bordeaux, France)
Objective: To investigate the sleep-wake cycles organization in
unrestrained non-human primate by using a totally implantable telemetric system for continuous electroencephalographic (EEG) recording.
To test the effects of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine
(MPTP) intoxication on the sleep-wake cycles organization in this
animal model of Parkinsons disease (PD).
Background: Previous sleep studies in experimental animals have
been fraught by the necessary use of restraint systems. The miniaturization of wireless telemetry technology for EEG monitoring in animals
offers reliable device to model the sleep-wake disorders in freely
moving monkeys particularly in the MPTP animal model of PD.
Although of interest, this animal model has not been used to investigate
the pathophysiology of sleep disorders in PD.
Methods: Experimentations were undertaken in accordance with the
guidelines of the French Agriculture and Forestry Ministry for the care and
use of laboratory animals.Three adult Macaca mulatta monkeys were
implanted with a three channels telemetry transmitter (1 EEG, 1 electrooculogram, 1 electromyographic channel). This setting allowed adequate recognition of sleep stages. These were scored according to the
Rechtschaffen and Kales criteria. Sleep-wake cycles were continuously
monitored over a four week baseline period. Two monkeys were subsequently injected with MPTP until the emergence of parkinsonian motor
signs.
Results: In baseline conditions, rapid eye movement (REM) and
non-REM sleep episodes were easily recognisable and the sleep architecture for each monkey showed remarkable night-to-night reproducibility although some interindividual differences were noticed. MPTP
intoxication resulted in REM sleep persistent suppression and daytime
sleepiness in both intoxicated monkeys. When severe parkinsonism
occured, complete sleep-wake disorganization was observed.
Conclusions: The MPTP non-human primate is a reliable model to
study the role of the dopaminergic denervation in the occurrence of the
sleep disturbances and excessive daytime sleepiness in PD. It will also
improve our understanding of the role of dopaminergic treatment in either
the aggravation or the improvement of these disorders. This study was
supported by: la Fondation pour la Recherche Medicale.
74
Neuronal a-synuclein overexpression affects lymphocytic gene networks in a transgenic mouse model of Parkinsons disease
B.A. Chase, G. Lu, K. Markopoulou (Omaha, Nebraska, USA)
Objective: To identify gene networks that are affected by alphasynuclein overexpression.
Background: Altered patterns of lymphocytic and brain mRNA
expression have been associated with neurological disease. We used a
transgenic mouse model of Parkinsons disease (PD) to investigate
whether alpha-synuclein overexpression results in patterns of lymphocytic mRNA expression that may serve as a molecular signature for PD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: A transgene for human alpha-synuclein was expressed

under the PDGF- promoter in a C57BL6 background. We assessed
blood RNA expression in pathologically characterized transgenic mice
and littermate non-transgenic controls at ages 2 and 11 months. Gene
expression proles from each of ve biological replicates per group
were obtained using Affymetrix 430 2.0 Arrays. Differential gene
expression was analyzed using GCOS, Affyminer, and Genelinker
software, and gene networks were identied using Ingenuity software.
Results: We identied replicable, robust changes in gene expression
patterns among 2- and 11-month old transgenic animals and their
non-transgenic littermate controls. Unsupervised learning methods
(e.g., clustering) were used to group samples. Supervised learning
methods were used to identify a potential classier. Sublinear association mining (SLAM) was used to identify associations between
genes in a class. These associations and a subset of the gene expression
data were used to train articial neural networks to classify the remaining data. The observed changes in gene expression patterns reected
co-regulation of genes lying within gene networks. Two examples
include a gene network involved in cell-cycle control, apoptosis, and
cellular proliferation and a gene network involved in chromatin structure and transcriptional regulation during cell proliferation.
Conclusions: We nd that relatively few genes are differentially
expressed in blood in this PD model. These genes are members of
co-regulated gene networks. Identifying gene networks that are coordinately regulated as a result of alpha-synuclein overexpression can
provide insights into disease pathogenesis and has the potential to offer
a reliable marker for PD status in humans.

75
Endogenous dopamine causes neurodegeneration in mice
L. Chen, Y. Ding, B. Cagniard, W. Chi, A.D. Van Laar, A. Mortimer,
T.G. Hastings, U.J. Kang, X. Zhuang (Chicago, Illinois, USA)
Objective: The potential toxicity of dopamine in Parkinsons disease
is controversial and evidence is lacking to show that dopamine causes
neurodegeneration in living animals.
Background: The mechanism for selective vulnerability of dopamine
neurons in Parkinsons disease patients is still unknown. One fundamental distinction between dopamine neurons and other neuronal types
is that the former expresses dopamine. If not sequestered from cytosol
into low pH vesicles by VMAT2, dopamine quickly undergoes degradation and produces potentially toxic metabolites. Therefore, dopamine
has been hypothesized to be a major vulnerability factor for neurodegeneration in PD.
Methods: We generated a transgenic mouse line with ectopic dopamine transporter (DAT) expressed in forebrain neurons. DAT takes up
pre-synaptically released dopamine into postsynaptic striatal neurons
that does not express VMAT2.
Results: We found that uptake of dopamine into postsynaptic neurons lead to striatal and cortical atrophy, neuron loss, astrocytosis and
hypolocomotion. Importantly, L-DOPA treatment worsened the behavioral phenotype, and unilateral lesion of medial forebrain bundle improved the behavioral phenotype, indicating that the behavioral phenotype is dependent on the dopamine supply. Moreover, these
transgenic mice showed signicantly increased oxidative modication
of proteins on cysteine residues by dopamine autoxidation products,
dopamine-quinone and DOPAC-quinone.
Conclusions: We show the rst in vivo model in which cytosolic
dopamine toxicity and resultant neurodegeneration can be demonstrated. Such a model will be a valuable tool for screening drugs that
may prevent or alleviate cytosolic dopamine toxicity and its contribution to neuronal degeneration in Parkinsons disease.

Movement Disorders, Vol. 22, Suppl. 16, 2007

76
Role of phosphorylation at serine 129 in cellular toxicity of
-synuclein
N. Sugeno, A. Takeda, T. Hasegawa, M. Kobayashi, A. Kikuchi,
Y. Itoyama (Sendai-City, Miyagi, Japan)
Objective: To verify the role of phosphorylation at serine 129 of
-synuclein.
Background: It is well known that -synuclein is a key molecule in
the pathogenesis of Parkinsons disease. It constitutes a major component and is phosphorylated at serine 129 in Lewy bodies.
Methods: Human wild-type (WT) -synuclein cDNA was cloned from
lymphocyte cDNA. The S129A mutant -synuclein, in which serine 129
was replaced by alanine to block the phosphorylation at this site, was
obtained by a site directed mutagenesis. The cDNA fragments were
subcloned into pCEP4 episomal expression vector at PvuII site and transfected into SH-SY5Y cells by using lipofection. Incidence of -synuclein
aggregation and activated-caspase 3 positive cells was immunohistochemically observed in wild type or S129A mutant expressing cells under the
exposure of rotenone. Furthermore, ER stress markers were investigated
by real-time PCR and Western Blotting methods.
Results: The S129A mutant expressing cells showed no -synuclein
aggregations and lesser incidence of activated-caspase 3 immunostaining compared to those of wild type overexpressing cells. These data
suggest that the phosphorylation at serine 129 is essential for the
pathophysiological metabolisms of -synuclein. In WT -synuclein
expressing cells, several ER stress markers were induced by the exposure of rotenone, but such incidence was were much less prominent in
the S129A mutant cells.
Conclusions: Our data showed that the S129A mutation alleviated
potential cytotoxicity of -synuclein, possibly by the inhibition of
phosphorylation at serine 129.
References:
1. Hasegawa, T., Sugeno, N., Takeda, A., Matsuzaki-Kobayashi,
M., Kikuchi, A., Furukawa, K., Miyagi, T., and Itoyama, Y.
(2007) FEBS Lett 581(3), 406-412
2. Furukawa, K., Matsuzaki-Kobayashi, M., Hasegawa, T., Kikuchi,
A., Sugeno, N., Itoyama, Y., Wang, Y., Yao, P. J., Bushlin, I., and
Takeda, A. (2006) J Neurochem 97(4), 1071-1077
3. Hasegawa, T., Matsuzaki-Kobayashi, M., Takeda, A., Sugeno,
N., Kikuchi, A., Furukawa, K., Perry, G., Smith, M. A., and
Itoyama, Y. (2006) FEBS Lett 580(8), 2147-2152.
77
Neuroprotective effect of human mesenchymal stem cell on dopaminergic neurons by anti-inammatory action
P.H. Lee, Y.-J. Kim, H.-J. Park, S.W. Yong (Suwon, Gyeonggi,
Republic of Korea)
Objective: To evaluate effect of human mesenchymal stem cell
(hMSCs) on modulation of neuroinammation.
Background: Parkinsons disease (PD) is a common progressive
neurodegenerative disorder caused by the loss of dopaminergic neurons
in the substantia nigra (SN). Numerous studies have provided evidence
suggesting that neuroinammation plays a critical role in the pathogenesis of PD.
Methods: We used lipopolysaccharide (LPS)-induced in vitro and in
vivo inammation models to investigate whether hMSCs have a protective
effect on the dopaminergic system through anti-inammatory mechanism.
Results: hMSCs treatment signicantly decreased LPS-induced microglial activation and production of proinammatory cytokines compared to
LPS-only treatment group. In co-culture cells of microglia and mesencephalic dopaminergic neurons, hMSCs treatment resulted in a signicant
reduction of dopaminergic neuronal loss induced by LPS stimulation. The
hMSCs treatment in rats showed that dopaminergic neuronal loss induced
by LPS stimulation in the SN was dramatically decreased, which was
clearly accompanied by a decrease in activation of microglia.
Conclusions: These data suggest that hMSCs have a neuroprotective
effects on dopaminergic neurons through anti-inammatory actions.

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78
Prolonged microglial activation in the substantia nigra of zitter rat
T. Kadowaki, A. Nakamura, K. Hashimoto, K. Nakadate,
S.-I. Sakakibara, K. Hirata, S. Ueda (Mibu, Tochigi, Japan)
Objective: In the present study, to characterize the microglial response in zitter mutant rat, a quantitative approach was undertaken in
the sections from the substantia nigra (SN) of postnatal developmental
stages of zitter rat using ionized calcium binding adapter molecule 1
(Iba1) antibody as a specic marker of microglia.
Background: A large body of evidence suggests that oxdative stressmechanism may be, at least, partially involved for the progressive and
selective degeneration of dopamine (DA) neurons in the substantia
nigra pars compacta (SNpc). In addition, microglial reaction is found in
Parkinsons disease (PD) patients as well as in experimental models of
PD. Especially, the increased levels of proinammatory cytokines and
reactive oxygen species (ROS) in post-mortal samples of PD suggest
the microglial activation might play a signicant role in the degenerative process found in PD and PD models. The homozygous zitter rat
(zi/zi) is an autosomal recessive mutant derived from the SpragueDawley (SD) rat, and is characterized by curled body hair, bent whiskers, ne tremor and accid paresis. The mutant rat has abnormal
metabolism of oxygen species. In this mutant rat, there is also a loss of
dopaminergic neurons with age and there is a corresponding loss of DA
bers and presence of abnormal DA bers similar to PD.
Methods: Zitter rats and age-matched SD rats were used. Freeoating frozen sections were stained for Iba1 and tyrosine hydroxylase.
Results: In the zitter rat brain, activated microglia cell-clusters were
increased from 2 weeks (W) to 2 months (M) of age. Then, these
number were decreased. Activated microglia cell-clusters restricted
within the SN after 2 M and continued until 12 M.
Conclusions: This study indicates that the microglial activation was
started before the degeneration of DA cells in the zitter SN.
79
Ubiquitylation of synphilin-1A modulates its aggregation and neurotoxicity in Parkinsons disease
R. Szargel, A. Eyal, J. Haskin, E. Avraham, E. Liani, R. Rott,
S. Engelender (Haifa, Israel)
Objective: We plan to investigate the role of the new synphilin-1
isoform, synphilin-1A, in the formation of Lewy body-like inclusions
and death of dopaminergic neurons.
Background: Synphilin-1 interacts with -synuclein in vivo and their
co-expression into mammalian cells lead to the formation of eosinophilic inclusions that resemble Lewy bodies, suggesting that synphilin-1 may modulate -synuclein aggregation. Synphilin-1 is present in
the core of most Lewy bodies of PD patients. It interacts with and is
polyubiquitylated by the E3 ubiquitin-ligases SIAH-1 and SIAH-2.
Inability of the proteasome to degrade ubiquitylated synphilin-1 leads
to a robust formation of cytosolic synphilin-1/SIAH inclusions. We
also found that synphilin-1 ubiquitylation is necessary for its inclusion
formation since a catalytically inactive mutant of SIAH is unable to
promote the formation of synphilin-1 inclusions. We recently isolated
a novel synphilin-1 isoform, which we named synphilin-1A. Synphilin-1A differs from synphilin-1 by their exon organization and initial
reading frames used for translation.
Methods: We carried out cell biology and biochemical experiments
to investigate how ubiquitylation affects synphilin-1A aggregationprone and neurotoxic nature.
Results: We characterized synphilin-1A as an aggregation-prone and
neurotoxic protein. Synphilin-1A binds to -synuclein and induces the
formation of intracellular aggregates in HEK293 cells, primary neuronal cultures and human dopaminergic cells. Overexpression of synphilin-1A in neurons results in striking cellular toxicity that is attenuated
by the formation of synphilin-1A-inclusions. Synphilin-1A is present in
Lewy bodies of PD and DLBD patients and is observed in detergentinsoluble fractions of brain protein samples obtained from DLBD
patients. We found that synphilin-1A can also be ubiquitylated in vivo

S25

and that this ubiquitylation modulates synphilin-1A aggregation and

neurotoxicity.
Conclusions: We propose that synphilin-1A ubiquitylation, together
with synphilin-1 and -synuclein, is important for Lewy body formation. Understanding the mechanisms that regulate Lewy body formation might shed light in the pathogenesis of PD and may open new
therapeutic avenues to treat the disease.
80
Relationship of -synuclein to chaperone mediated autophagy
T. Vogiatzi, K. Vekrellis, L. Stefanis (Athens, Attiki, Greece)
Objective: To examine whether wild type (WT) -synuclein
(ASYN) is degraded by Chaperone Mediated Autophagy (CMA) in
neuronal cells and study whether lysosomal dysfunction and neuronal
toxicity induced by a mutant form (A53T) of ASYN is CMA-related.
Background: ASYN is a neuronal protein of uncertain function that
is implicated in Parkinsons disease (PD) pathogenesis. Duplications/
triplications of the gene encoding for ASYN have been identied in
familial PD cases. This indicates that increased WT ASYN levels can
lead to PD. Thus, studying production and clearance of WT ASYN is
important for PD pathogenesis. Furthermore, point mutations in the
gene encoding for ASYN also lead to PD in rare familial cases.
However, the pathogenic effects of mutant ASYN are unknown. In
previous studies we found that cellular expression of A53T, but not
WT, ASYN can lead to lysosomal dysfunction, and that WT, but not
A53T can be degraded in isolated liver lysosomes via CMA, after
recognition and uptake by the lysosomal receptor Lamp2a. In the same
study, mutant ASYNS were shown to block CMA in vitro by binding
to the receptor without being internalised (Cuervo et al., 2004). Because the data suggesting a relationship between ASYN and CMA are
largely based on studies in isolated liver lysosomes, further studies are
needed in a neuronal cell context.
Methods: Inducible SHSY5Y and PC12 cells were generated expressing WT, A53T, a mutant form of ASYN (DQ) with mutations in
the KEFQR motif necessary for recognition by Lamp2a and double
mutant DQ/A53T ASYN. Half-life of ASYN and long-lived protein
degradation were measured using standard methodologies following
radiolabeling and pulse chase.
Results: The half-life of DQ ASYN was prolonged when compared
to WT ASYN in PC12 cells. These results suggest that the clearance of
WT ASYN may occur via CMA. Furthermore lysosomal degradation
was impaired in SHSY5Y and PC12 cells expressing A53T ASYN,
whereas in DQ/A53T ASYN-expressing SHSY5Y and PC12 cells,
this impairment was nearly completely restored. This suggests that
interference with normal CMA is responsible for the lysosomal dysfunction induced by mutant ASYN.
Conclusions: Our results highlight the importance of ASYN degradation via CMA, the impact of aberrant ASYN on CMA-dependent
degradation, and the potential importance of CMA-dependent degradation in a neuronal cell context.
81
Comparative analysis of progenitor cell populations in the adult
midbrain of wild-type and Parkinsonian mice models
A. Hermann, C. Suess, F. Pan-Montojo Puga, M. Jungnitsch,
S. Gehre, J. Schwarz, A. Storch (Dresden, Germany)
Objective: Is there neurogenesis in the region of interest in Parkinsons disease?
Background: Neurogenesis in the adult brain occurs within the two
principle neurogenic regions, the hippocampus and the SVZ of the
lateral ventricles. The occurrence of adult neurogenesis in non-neurogenic regions including the midbrain remains controversial, but isolation of neural stem cells (NSCs) from several parts of the adult brain
including the substantia nigra has been reported. Nevertheless it is
unclear whether adult NSCs can be found in non-neurogenic regions of
the adult brain and whether there are regional differences in their
phenotype.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: Coronal sections of the whole brain dissected from adult

mice were immunostained with antibodies against NSC type-specic
markers known to be involved in adult neurogenesis in the lateral
ventricle.
Results: Here we show evidence for adult NSC populations in the
subventricular regions of the whole ventricular system in the adult CNS
with bright nestin staining within the SVZ of the whole ventricular
system including the midbrain, with few cells being Nestin/GFAP
or Nestin/PSA-NCAM. We did not observe relevant expression of
Olig2 and no Nestin/Olig2-positive cells in the adult SVZ in all
regions investigated. We labelled the rapidly proliferating type-C cells
using standard short-time pulsing BrdU protocols. In contrast to results
in the SVZ of the lateral ventricle, no cells were positive for short-time
BrdU pulsing in the midbrain region. Consistently, type-C cells seemed
to appear exclusively in the SVZ of the lateral ventricle. A few cells
were co-labelled for Nestin and EGF receptor as well as for Musashi1.
Cells positive for CD24 could be demonstrated in the whole ventricular
system with the highest density in the SVZ of the lateral ventricle while
NG2 positive cells could be shown all along the subventricular regions
with no co-labelling to Nestin cells. Consistently with the age of the
mice examined, there was no positive staining for the radial glia marker
RC2. Quantitative immunhistochemical results of the A53T over-expressing mice will be reported at the meeting.
Conclusions: Together, these results imply that the Nestin cells are
resident precursors in the adult SVZ of the midbrain region.
82
Apoptotic mechanisms in mutant LRRK2-mediated cell death
C. Vitale, C. Iaccarino, C. Crosio, G. Sanna, M.T. Carri, P. Barone
(Naples, Italy)
Objective: The aim of the present study was to investigate the role of
apoptosis in mutant LRRK2-mediated cellular death and to explore the
role of the different functional domains of the protein in this process.
Background: Mutations in the gene coding for leucine-rich repeat
kinase 2 (LRRK2) cause autosomal-dominant Parkinsons disease. The
pathological mutations have been associated with an increase of
LRRK2 kinase activity although its physiological role and substrates
have not been identied yet.
Methods: LRRK2 cloning: Total RNA was prepared from SH-SY5Y
cells and subjected to RT-PCR amplication. PCR products were cloned in
pCS2-MTK plasmid, an eukaryotic expression vector carrying a N-terminal 5xMYC tag. Point mutations and deletions were generated by PCRmediated site-directed mutagenesis by using the QuikChange kit (Stratagene). Two types of mutants were generated: four familial-linked
mutations R1441C, Y1669C, G2019S, I2020T; deletions of different protein domains: LRR, RAS, kinase and WD40. Analysis of LRRK2 expression: Transient expression in human and murine cell lines of each plasmid
(1,5 g DNA/5-7105 cells) was obtained with LipofectAMINE Plus
reagent (Invitrogen) according to the manufacturers instructions. After 3
hours incubation with transfection reagents, the cells were cultured in
normal growth medium for 48h. The 5X-myc-LRRK2 protein expression
was detected by western blot experiments on protein extracts from transiently transfected cells. All LRRK2 constructs were analyzed by immunouorescence experiments.
Results: Our data indicate that LRRK2 has a cytoplasmic localization.
All different point mutations do not alter the LRRK2 cellular localization.
The over-expression of the mutated LRRK2 in all neuronal cell lines
induces apoptotic cell death as indicated by nuclei condensation and
activation of caspase3. Finally the absence of both LRR and WD40
domains unabled caspase 3 activation and nuclei condensation.
Conclusions: The data indicate that disease-associated mutant
LRRK2 cell toxicity is due to mitochondria-dependent apoptotic pathway. Moreover, we explored the function of two protein domains in
LRRK2 (LRR and WD40) and demonstrate that the lack of these
protein domains has a protective effect on mitochondria dysfunctions
induced by mutant LRRK2.

Movement Disorders, Vol. 22, Suppl. 16, 2007

83
The nociceptin/orphanin FQ receptor antagonist J-113397 enhances the effects of L-DOPA in the MPTP-lesioned non-human
primate model of Parkinsons disease
N.P. Visanji, S.H. Fox, R.M.A. Debie, A.C. McCreary, J.M. Brotchie
(Toronto, Ontario, Canada)
Objective: The present study examined the effect of co-administration with a nociceptin / orphanin FQ (N/OFQ) antagonist (J-113397) on
the anti-parkinsonian effects of L-DOPA in MPTP-lesioned marmosets.
Background: J-113397 has been shown to enhance the anti-akinetic
effects of L-DOPA in rodent models of Parkinsons disease (PD)
(Marti et al., 2005; 2007).
Methods: Long-term L-DOPA-treated MPTP-lesioned marmosets,
with established motor complications, (n5) were administered vehicle or L-DOPA/benseraside (12.5/3.125 mg/kg s.c. respectively) in
combination with either vehicle (0.5% methylcellulose), or J-113397
(30 mg/kg) s.c. Animals were placed in observation cages for 4 hours
during which time motor activity was measured using activity monitors
and behavior recorded for subsequent analysis by a neurologist blinded
to treatment.
Results: Analysis of motor activity at peak dose (40-110 min post
treatment) revealed that administration of L-DOPA resulted in an 81%
increase in motor activity as compared to vehicle-treated animals
(P0.05). However, co-administration of J-113397 (30 mg/kg) with
L-DOPA signicantly increased motor activity both compared to vehicle-treated animals (708%, P0.001), and compared to L-DOPAtreated animals (347% P0.001). Behavioral analysis at peak dose
revealed that treatment with L-DOPA alone afforded no signicant
anti-parkinsonian actions as compared to vehicle-treated animals. However, co-administration with J-113397 resulted in a signicant antiparkinsonian effect (P0.05 cf vehicle-treated animals) which was
accompanied by a signicant exacerbation of dyskinesia (P0.01 cf
vehicle-treated animals).
Conclusions: These data demonstrate that co-administration of an
N/OFQ antagonist enhances the anti-parkinsonian effects of L-DOPA
in MPTP-lesioned marmosets at the expense of enhanced dyskinesia.
Full investigation of the relationship of the effects of co-administration
of J-113397 and L-DOPA in animals with and without motor complications may reveal a situation where the anti-parkinsonian actions of
L-DOPA can be enhanced without the exacerbation of dyskinesia.
These data suggest that adjunct therapy of an N/OFQ antagonist may
allow for a reduction in the dose of L-DOPA required to achieve an
anti-parkinsonian effect in PD.

CHOREA
84
Botulinum toxin treatment in perioral dyskinesia
M.-W. Seo (Chonju, Jeonbuk, Korea)
Objective: The aim of this study was to determine the effectiveness
of botulinum toxin A (BTX-A) in treating perioral dyskinesia (POD).
Background: To date a few case reports have evaluated the efcacy
of BTX-A in the treatment of POD caused by neuroleptics. In our
experience, primary POD and POD with varied non-neuroleptic origin
also showed effectiveness on BTX-A as well.
Methods: Twenty-four patients with POD were enrolled in this
study. Each subject was treated with various doses of botulinum toxin
(Botox, 2050 U). The botulinum toxin was injected into the tongue
muscles, jaw opener, jaw closer, jaw deviating muscles, and the perioral muscles according to the EMG activities in each patient. A
familymember who cared for the patient carried out an assessment of
the effectiveness of the treatment. The subjects were assessed before
receiving the botulinum toxin treatment and at 6 weeks after the
injections. The degree of improvement in each patient was classied
into four categories: no response, mild improvement, moderate improvement, and marked improvement.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Results: Twenty out of the 24 patients showed a positive response to
the botulinum toxin treatment. Four patients showed mild improvement, 9 patients showed moderate improvement, and 7 patients showed
marked improvement following the botulinum toxin treatment. Four
patients, however, did not show any improvement. Some patients
reported transient pain (8), mild dysphagia (6), and mild dysarthria (2).
These complications disappeared within 14 days after receiving the
botulinum toxin injection.
Conclusions: These results demonstrated that botulinum toxin has
the potential to be one of the most effective treatments for primary
POD and POD with varied non-neuroleptic origins.
85
Huntingtons disease-like 2: The rst case report in Latin America
in a patient without African ethnic origin
H.A.G. Teive, N. Becker, R.P. Munhoz, S. Raskin, L.C. Werneck,
C. Cazeneuve, A. Durr, O. Russaouen, A. Brice (Curitiba, PR, Brazil)
Objective: To report a rst case of Huntingtons disease-like-2
(HDL2) in Latin America.
Background: In large genetic studies, about 1% of clinically typical
Huntingtons disease (HD) cases do not carry the HD mutation. To date
several hereditary HD phenocopies have now been dened. HDL-2 is
caused by a CAG/CTG repeat expansion in the juncthophilin-3 gene on
chromosome 16q24.3.
Methods: A 58-year-old white female patient had a history of evolution from ten years with neuropsychiatry abnormalities and facial
dyskinesia evolving to generalized chorea and then parkinsonism and
dementia. The familiar history was sugestive for an autosomal dominant neurodegenerative disease, including two descendants one with
chorea and another with parkinsonism.
Results: MRI demonstrated brain atrophy particularly of the caudate
nucleus. The mutations for HD, SCA 1, 2, 3, 6, 7, and 17 and
dentatorubral-pallidoluysian atrophy were negative. The repeat length
in juncthophilin-3 gene was 48.
Conclusions: This is the rst case of HDL2 in a patient without
African ethnic origin and the rst case of disease reported in Latin
America.
86
Disordered post-movement excitation in surround muscles in paroxysmal kinesigenic dyskinesia
Y.H. Sohn, J.-S. Kim, H.-W. Shin, S.Y. Kang (Seoul, Korea)
Objective: To investigate changes in surround inhibition within
human motor system in patients with paroxysmal kinesigenic dyskinesia (PKD).
Background: Surround inhibition, suppression of excitability in an
area surrounding an activated neural network, is a physiological mechanism to focus neuronal activity and to select appropriate neuronal
responses. It is proposed to be an essential mechanism in the motor
system where it could aid the selective execution of desired movements. Disturbances in surround inhibition have been demonstrated in
patients with dystonia and parkinsonism, but not in other movement
disorders, including PKD.
Methods: Subjects were 14 de novo patients with PKD and 10
young, healthy volunteers. Surface EMG was recorded from the abductor digiti minimi (ADM) and exor digitorum supercialis (FDS) of
the dominant hand. TMS was set to be triggered by self-paced, voluntary exion of the index nger, i.e., EMG activity of FDS, with a
duration around 100 ms. Seven sessions (18 trials) of self-triggered
TMS were applied in a random order at variable intervals (3, 15, 40,
80,200, 500, 1000 ms) between EMG onset and TMS trigger, with a
stimulation intensity of 140% resting motor threshold. Two control
sessions were given before and after self-triggered sessions. Peak-topeak MEP amplitudes obtained at self-triggered TMS were compared
to the average MEPs of control TMS.
Results: In controls, MEP amplitudes of ADM were unchanged
during movement (92112%), signicantly enhanced immediately after
movement (167%), and reduced to the baseline at 1000ms after EMG

S27

onset, compared to the resting state. In patients, however, post-movement excitation at 200 ms after EMG onset of FDS was not observed
in ADM (101%), although surround inhibition until 80 ms was comparable to that of controls.
Conclusions: These data demonstrate that surround inhibition exerted on ADM during voluntary movement of the index nger is
preserved, but post-movement excitation in ADM after FDS movement
is impaired in patients with PKD. This disturbance may be associated
with disturbances in stopping on-going movements. Considering previous ndings in dystonia and parkinsonism, our ndings suggest that
the pattern of disturbed surround inhibition is different among various
movement disorders.
87
Hemichorea secondary to striatal hemorrhage in hyperglycemic
hyperosmolar coma
S. Ozkan, G. Tekgol, S. Dagli, D. Ozbabalik (Eskisehir, Turkey)
Objective: Although transient magnetic resonance imaging (MRI)
lesions of striatum in acute chorea secondary to hyperglycemic hyperosmolar non-ketotic coma has been frequently reported before, the
nature of these lesions is still controversial.
Background: We aimed to report a similar patient who was followed
up with serial neuroimaging evaluations suggesting an hemorrhagic
pathology.
Methods: Case: Twenty-four year old male with diabetes mellitus
type I for 4 years has been admitted to our movement disorders out
patient clinic for his hemichorea on the right hand. This complaint had
suddenly begun one month ago after an acute confusional state. In
labarotary investigatements in a different center during this period,
blood glucose had been 543 mg/dl with negative urine ketone bodies.
Confusion had been improved after the regulation of blood glucose, but
hemichorea had been sustained. There was striatal hyperintensity in
acute phase CT and, striatal hyperintensity in T1-weighted and hypointensity in T2-weighted MRI images which was performed on the 10th
day. Striatal lesion was hyperintense in both T1 and T2-weighted scans.
Patent was given haloperidol treatment and the symptoms was improved in the second month. At the end of one year, lesion was
isointence in T1 and hypointens in T2-weighted scans suggesting an
hemorrhagic pathology.
Conclusions: Serial neuroimaging ndings in our case suggests that
striatal lesions in hemichorea after hyperglycemic hyperosmolar nonketotic coma can be a result of hemorrhage.
88
Levels of the light subunit of neurolament triplet protein in
cerebrospinal uid in Huntingtons disease
R. Constantinescu, M. Romer, L. Rosengren, D. Oakes, K. Kieburtz
(Goteborg, Sweden)
Objective: To evaluate (1) the levels of the light subunit of neurolament triplet protein (NFL) in the cerebrospinal uid (CSF) from
subjects with Huntingtons disease (HD) and (2) whether there is any
correlation between these levels and clinical parameters of interest in
HD.
Background: The neurolament is a major structural element of
neuronal cells. It is composed of a triplet proteins of which NFL has
been shown to be increased in several neurological diseases (vascular,
infectious, neurodegenerative) (2).
Methods: Secondary analysis of NFL levels in all available CSF
samples from the INTRO study which was a randomized, placebo
controlled clinical trial, examining the tolerability of OPC-14117 in 64
adult HD subjects (for results see ref. 1). Subjects were assessed at
baseline using the Unied Huntingtons Disease Rating Scale (UHDRS), the Clinical Global Impression Scale, and supplemental neuropsychological scores. Lumbar punctures were done at baseline and at
week 12. CSF was stored at -70 degree Celsius at Cornell University,
New York, NY. The Elisa for NFL in the CSF was performed at the
University of Gothenburg, Sweden. The controls were 35 age matched
healthy volunteers without history of neurological disease. A paired

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

t-test was used to assess whether CSF-NFL levels are elevated in the
HD population compared to controls. Scatter plots of CSF-NFL vs.
clinical parameters of interest in HD were created along with the
Pearson correlation and partial correlations to adjust for age at collection.
Results: CSF samples were available from 35 HD subjects and 35
controls. The age of the subjects was 47.2 (13.68) years [mean (SD)],
the CAG length 46.53 (6.11) repeats, and the disease duration 6.85
(4.14) years. The CSF-NFL levels in HD subjects were 944.6 (432.4)
ng/L [mean, (SD)] and in controls 154.5 (51.6) ng/L. The Pearson
partial correlation between clinical assessments scores and CSF-NFL
levels in HD subjects, adjusted for age at CSF draw, were: for Total
Functional Capacity -0.334 (p-value 0.05); for UHDRS Independence
-0.318 (p-value NS); and for STROOP word -0.317 (p-value NS).
Conclusions: The CSF-NFL levels were signicantly higher in HD
subjects compared with age-matched controls and were correlated with
the Total Functional Capacity scale.
89
Atypical onset movement disorders in Brazilian Huntingtons disease patients
H.A.G. Teive, N. Becker, R.P. Munhoz, S. Raskin, L.C. Werneck
(Curitiba, PR, Brazil)
Objective: Our primary aim was to describe the clinical features of
HD Brazilian patients who initially presented with movement disorder
other than chorea and investigate atypical motor presentations in relation to triplet CAG expansion.
Background: Huntingtons disease (HD) is an autosomal dominant
disorder characterized by irrepressible motor dysfunction, cognitive
decline and psychiatric disturbances, which lead to progressive dementia and death approximately 15-20 years after disease onset. Chorea, or
choreoathetosis, is the movement abnormality most frequently associated with HD.Although HD usually starts with choreic movements,
some individuals with the HD gene mutation have clinical motor
symptoms other than chorea.
Methods: Neurologists (Becker and/or Teive) have examined 44
individuals for information of de sex, age of onset and motor symptom
at onset of HD.Ten micro liters of whole blood were collected from 44
individuals with clinical and/or familial history of HD, after informed
consent. PCR was performed as described previously.
Results: Among the 44 patients, 54,5% was male. Ages of onset
range from 22 to 57 years with an approximately normal distribution
and a mean age of onset of 36,3 ( 8,3) years. Expanded CAG repeats
range from 41 to 55 (mean 46,5) and was responsible for 64 per cent
in variation of age of motor symptoms onset. Among the 07 (07/44 or
15,9%) patients with atypical motor onset symptoms, we distinguished
three types of movement disorders: parkinsonism (04), dystonia (02)
and tics (01). The mean age of onset and CAG repeats was respectively
for typical and atypical cases: 36.9 and 33.1 years and 45.8 and
50.1 CAG repeats (p 0.0001).
Conclusions: 1- HD patients may have different motor manifestations at the onset of the illness. 2-Patients with atypical movement
disorders have larger CAG expansion and an earlier age of onset than
HD patients with typical onset chorea.
90
Aripiprazole in Huntingtons disease: A rst case report
A. Ciammola, J. Sassone, F. Squitieri, B. Poletti, N. Mencacci,
A. Ciarmiello, V. Silani (Milano, Italy)
Objective: To evaluate the effects of aripiprazole on motor impairment and behavioural abnormalities in a patient with Huntingtons
disease.
Background: Huntingtons disease (HD) is an autosomal dominant neurodegenerative disorder caused by an unstable expansion of
a CAG trinucleotide repeat in the IT15 gene on the short arm of
chromosome 4. The classical HD presentation includes midlife
onset of dementia, personality disorders and chorea, whereas dystonia and parkinsonism features usually appear later in the disease

Movement Disorders, Vol. 22, Suppl. 16, 2007

course. Antidopaminergic agents represent the mainstay therapy for

controlling choreic disorder in HD. However the use of classic and
atypical antipsychotics may result in the development of extrapyramidal symptoms and in cognitive slowing that lead to increased
functional disability. Moreover several reports have raised concerns
about a possible association between atypical antipsychotic and
dangerous adverse metabolic changes.
Methods: Changes of motor impairment and behavioural abnormalities were evaluated according to UHDRS and BDI. Haematological
parameters were evaluated after 2 months of aripiprazole treatment.
Results: We report the case of an 56 years old man HD patient (47
CAG) who developed a metabolic syndrome (fasting glucose 119
mg/dL; triglycerides 408 mg/dL; HDL Cholesterol32 mg/dL) during olanzapine treatment. Therefore we switched to aripiprazole, a new
atypical antipsychotic which acts as a partial agonist at the D2 and
5HT1a receptors and antagonist at 5HT2a receptors. The patient reported a signicant improvement of chorea and dysphagia within the
rst week of treatment (dose 2,5 mg a day), so aripiprazole was raised
up to 5 mg a day, leading to a further improvement of involuntary
movements (UHDRS part I at baseline 51, UHDRS part I after 2
months of aripiprazole treatment 30). Furthermore there was a
lessening of the depressive symptoms and the metabolic dysfunction
also improved (fasting glucose 89 mg/dL, triglycerides 201 mg/dL,
HDL Cholesterol32 mg/dL).
Conclusions: At 2 months follow up the HD patient has ongoing
therapeutic responsiveness, demonstrating that aripiprazole treatment
has led to a considerable control of psychiatric and motor symptoms
and good improvement of the metabolic disorder.
91
Chorea following acute sensory deprivation
D. Franca, A.V. Giannetti, F. Cardoso (Belo Horizonte, MG, Brazil)
Objective: To describe one patient who developed chorea following
acute sensory loss resulting from surgical treatment of syringobulbia
and syringomyelia.
Background: Chorea is a Movement Disorder characterized by a
continuous and random ow of muscle contractions. Although a myriad
of genetic and non-genetic causes of chorea have been described, it has
not been widely acknowledged that sensory deprivation may be responsible for this Movement Disorder (Lancet Neurol 2006;5:589). Up to
22% of patients with syringomyelia may present with Movement
Disorders, mainly dystonia and tremor, thought to be caused by increased excitability of motor neurons (Neurology 1999;52:823). Chorea in association with syringomyelia has been rarely described and is
usually relieved by decompression of the syrinx (Mov Disord 1999;
14:684).
Methods: Case-report of a patient evaluated at the Movement Disorders Clinic of the Federal University of Minas Gerais.
Results: We describe a 55 year old woman who was evaluated at the
Neurology Service for bilateral, right greater than left, neck and arm
pain. Despite normal neurological examination, work up showed syringobulbia and syringomyelia reaching C4 level. Surgical treatment
consisted of decompression of the syrinx through a C1-C3 laminectomy. The procedure went uneventful. On the rst post-op day she was
on mechanical ventilation which was discontinued on the following
day. Upon the removal of the nasotracheal tube, she developed involuntary severe chorea and dystonia of the right upper extremity. Pain
sensation was normal but there was severely decreased proprioception
and tactile sensation in the limb affected by the Movement Disorder.
Along the following ten months there was complete remission of the
chorea, persistence of mild dystonia, improvement of the hypoesthesia
but development of severe neuropathic pain, treated with tricyclic and
methadone.
Conclusions: Our patient supports the notion that acute sensory loss
due to spinal cord lesion may lead to chorea. The Movement Disorder
improves with recovery of the sensory function. Acute sensory deprivation must be included among the causes of chorea.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

92
Clinical ndings in Titf-1 and SGCE-mutation carriers: Towards a
clinical differentiation of benign hereditary chorea and myoclonusdystonia
F. Asmus, A. Zimprich, M. Munz, T. Gasser, P.F. Chinnery
(Tuebingen, Germany)
Objective: To describe and compare the phenotype of patients with
genetically proven benign hereditary chorea (BHC) with myoclonusdystonia (M-D).
Background: The existence of benign hereditary chorea as a discrete
disease entity had been questioned until the identication of mutations
of thyroid transcription factor 1 (Titf-1, Nkx2.1) in several families.
Clinical similarities between BHC and Myoclonus-Dystonia (M-D,
DYT11) raise the possibility that the two conditions might be confused,
leading to the wrong clinical diagnosis with implications for patient
management, but no systematic comparisons of genetically proven
cases have been performed to date.
Methods: By direct sequencing and gene dosage assays genotypes of
Titf-1 and epsilon sarcoglycan (SGCE) were analyzed in three index
patients and in one index patients daughter. The movement disorder of
Titf-1 and SGCE mutation carriers were compared by a systematic
video review.
Results: A new point mutation at the splice acceptor site of intron
2 (376-2AC) of the Titf-1 gene (376-2 AC) was detected in a
mother and her daughter from the U.K. A SGCE nonsense mutation
in exon 3 (R97X) and a novel missense mutation (G227V) were
detected in two additional patients. Both Titf-1 mutation carriers
presented with infancy-onset non-progressive chorea with response
to alcohol intake. Neither patient had pulmonary infections in childhood nor an abnormal thyroid. Additional dystonia of the neck,
trunk and limbs could be observed in Titf1-mutation carriers. The
Titf1-mutation carriers had eeting jerky distal limb movements,
supercially similar but distinct from the typical triggering of lightning-like myoclonic jerks seen in both patients with SGCE mutations.
Conclusions: Titf1- and SGCE- mutation carriers can show obvious
similarities in respect of dystonia, jerks of neck and limbs and alcoholresponsiveness. However, triggering of myoclonus by action in SGCE
mutation carriers and continuous chorea of all limbs in Titf-1 mutation
carriers phenotypically discriminates both genetic disorders. Titf-1
mutations should be considered in all patients with infancy onset
choreatic movement disorders even in the presence of dystonia and
myoclonic jerks.
93
Chorea isolated on the both lower limbs associated with hyperglycemia
Y.-H. Sung, H.-T. Kim, D.-J. Shin (Incheon, Korea)
Objective: To describe the rst case of chorea isolated on the both
lower limbs associated with hyperglycemia.
Background: Metabolic disorders such as thyrotoxicosis, systemic
lupus erythematosus, and dysfunction of the glucose metabolism also
cause this hyperkinesias. Non-ketotic hyperglycemia (NKH) has occasionally abnormalities including Movement Disorders. Clinically
hemichrorea-hemiballism(HB-HC) caused by NKH often persents in
elderly patients with a history of non-insulin dependent diabetes mellitus(NIDDM) and their neuroimage studies often reveal contralateral
putamen hyperintensity. In some reports, HB-HC in hyperglycemic
patient is more often presented compared with bilateral chorea.
Methods: An 87-year-old woman with twenty-year history of
NIDDM suddenly developed a continuous, arrhythmic, and purposeless
choreiform movment, which was isolated on the both lower limbs. She
had poorly controlled diabetes mellitus.
Results: On admission, initial laboratory study reports showed signicant evidence of poorly controlled diabetes mellitus, ie fast blood
glucose level of 570mg/dl, serum osmolality of 313mosmol/kg and
glycosilated hemoglobin A1c concentation of 15.9%. Glucose was also
detectable in the urine (4) while ketones were negative. Brain mag-

S29

netic resonance imaging (MRI) showed that the bilateral dorsolateral

putamen were hyperintense on T1-weighted images and hypointense on
T2-weighted images. The patients blood sugar was controlled with
insulin, but chorea continued. The choreic movements disappeared
after 10days with the oral administration of haloperidol 10mg per day.
Conclusions: Experimental and clinical evidence supports the notion
that the putamen are somatotopically organized with dorsoventral arrangement of leg, arm and face. In this case, lesions in the bilateral
dorsolateral putamen are somatotopically correlated with chorea isolated on the both lower limbs bilateral lower limbs.
94
Late-onset Huntingtons disease in our movement disorder unit
A. De la Cerda, E. Munoz, E. Tolosa (Barcelona, Spain)
Objective: To ascertain the prevalence and to describe the main
clinical and genetic ndings of late-onset Huntingtons disease
(LOHD) patients seen in our outpatient clinics of movement disorders.
Background: LOHD is characterized by onset of symptoms after the
age of 50 and is usually associated with a milder course, with a low
incidence of dementia. However, there are scarce studies describing
LOHD or comparing LOHD with typical onset HD.
Methods: We reviewed our HD database (1997-2007) and the medical history of LOHD cases (onset 50 years). Age at onset, family
history, symptoms at onset, disease duration at review, the presence of
dementia (DSM-IV criteria), and the number of CAG repeats were the
main features analyzed.
Results: Nineteen (15M/4F) out of 130 HD patients were LOHD
(14.6%). Mean age at onset was 60.47.5 (range:50-75). There was no
family history for HD in 6 cases. Parents were known to be affected in
only 9 cases (50%). In 6 of them, the inheritance was paternal. Chorea
was the symptom at onset in 12. After a mean disease duration of
10,55 years, 3 patients had died and in other 3 the follow-up was lost.
Seven out of 14 well-documented patients became demented. The mean
CAG repeats number was 41,71.9 (range:39-46).
Conclusions: LOHD is not infrequent in our series. The negative
family history in a number of cases and the low range of CAG repeats
obtained in our study agree with previous results. However, both the
preponderance of paternal inheritance and the relatively high incidence
of dementia were unexpected. We believe that prospective, comparative studies with typical onset HD are necessary to better characterise
LOHD.
95
Bradykinesia in patients with history of sydenhams chorea
L.B. Barreto, F. Cardoso, D.P. Maia, A.L. Teixeira, Jr, R.G. Beato
(Belo Horizonte, Minas Gerais, Brazil)
Objective: To evaluate the presence of parkinsonian signs in a cohort
of patients with sydenhams chorea (SC) in remission.
Background: SC, a major manifestation of rheumatic fever, is characterized by the combination of motor, mainly chorea, and non-motor
features, such as obessive-compulsive behavior. These ndings are
thought to result from dysfunction of the frontal-striatal circuits. We
have previously described that, in comparasion with patients with
Tourette syndrome, subjects with SC have a high-risk of developing
neuroleptic-induced parkinsonism (J Neurosurg Psychiatry 2003;74:
1350). We have speculated that SC may be characterized not only by
a hyperkinetic Movement Disorder but that at least a proportion of
patients may also present with subclinical Parkinsonian ndings.
Methods: This study includes all patients of ages 16 years or greater
with SC in remission followed up in the UFMG Movement Disorders
Clinic and seen from March 2006 through February 2007. SC was
diagnosed according to the modied Jones criteria (Mov Disord 1997;
12:701) and remission was dened by disappearance of chorea and
discontinuation of antichoreic agents. Patients underwent assessment
with UPDRS and USCRS (Mov Disord 2005;20:585).
Results: 20 patients (16 females, mean age 21,4 years, range of
16-42) met the inclusion criteria during the study period. No chorea
was notice on the evalution. Bradykinesia was identied in a total of 11

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

(55%) patients (8 females). Mean UPDRS part III and total USCRS
scores were,0,9 and 1,4 respectively. In 10 patients, bradykinesia was
detected in the nger-tapping test (on the right side in 7 patients, on the
left side in 3 subjects and bilateral in 1 patient) and in four on the leg
agility test (on the right side in 2 patients and on the left side in the
other 2 subjects).
Conclusions: We have identied mild but unequivocal bradykinesia
in 55% of patients with SC in remission. The absence of chorea and
lack of exposure to antichoreic drugs in the patients herein studied
support the notion that a substantial proportion of SC patients present
with persistent Parkinsonian ndings.
96
Chorea and compulsive behavior an unusual presentation of
myasthenia gravis
M. Niethammer, M. Daras, S. Frucht (New York, New York, USA)
Objective: We present a case of myasthenia gravis with chorea and
behavioral changes as initial symptoms.
Background: Myasthenia gravis is an antibody-mediated immune
disorder limited to the neuromuscular junction that typically presents
with pathologic fatigability. To our knowledge, behavioral changes and
chorea have not been described in this disorder.
Results: The patient is a 58 year-old right-handed woman who
presented with changes in her mood, balance, and abnormal involuntary movements. Her initial symptoms followed a u-like illness. Over
the next year she developed involuntary movements of her hand,
depressed and anxious mood, and a tendency to unconsciously make
humming noises. She also noted severe fatigue, but no weakness. Her
exam was signicant for bilateral choreiform movements of her hands,
mild parkinsonism (mostly imbalance), and decits in memory and
attention on mental status testing. The patient was referred for genetic
counseling and testing for Huntingtons disease (HD), which was
negative, as well as a CT scan of her whole, which was unremarkable.
On follow-up 3 months later (15 months after onset of symptoms) she
complained of leg weakness when climbing stairs, as well as diplopia
and ptosis in the evening. On exam, she had signicant fatigability of
her proximal musculature, and dyspnea on mild exertion. An edrophonium test was performed, leading to transient but denite improvement.
Treatment with pyridostigmine was initiated. Two weeks later her
chorea and unconscious humming had disappeared along with resolution of the fatigability. CT scan of the chest showed no thymoma. She
is doing well after treatment with intravenous immunoglobulin (IVIG)
and pyridostigmine.
Conclusions: Chorea and behavioral changes have not previously
been described with myasthenia gravis. The resolution of these symptoms with pyridostigmine raises interesting questions regarding the
mechanisms of chorea and compulsive behavior. While the patient
currently has no signs of another disease, it will be important to follow
her for the development of a potential underlying disorder.
97
A case of CHAP syndrome
M.-W. Seo, S.-Y. Jeong (Chunju, Jeonbuk, Korea)
Objective: We report a case of CHAP syndrome and discuss the
pathomechanisms of CHAP syndrome.
Background: The syndrome consists of choreoathetosis and oralfacial dyskinesias, hypotonia, affective changes, and pseudobulbar
signs (CHAP). CHAP syndrome was initially described as having an
onset of 1 to 7 days after induced profound hypothermia and complete
circulatory arrest for congenital heart lesion repair. Until now, only a
few cases of CHAP syndrome had been reported since its initial
description. Therefore, up to now, hypothermia has been regarded as
one of the most plausable pathomechanisms of CHAP syndrome.
Methods: We report a patient in a severe state of cachexia who
demonstrated all of the signs and symptoms of CHAP syndrome with
normal body temperature.
Results: Brain SPECT (TC-99mECD) images showed decreased
perfusion in the left fronto-parieto-temporal lobe.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Conclusions: The pathomechanisms of CHAP syndrome are obscure. However, our case led us to suggest that hypoxia may be a
greater risk factor for CHAP syndrome than hypothermia. Furthermore,
CHAP syndrome also seems to be related to circulatory insufciencies
in the extrapyramidal nervous systems.
98
Choreoathetosis precipitated by subclinical hypothyroidism in an
Asian patient
W.S.S. Hameed, T.E. King (Singapore, Singapore)
Objective: We report a case of an elderly woman who presents with
choreoathetoid movements Her thyroid functions were abnormal. She
was diagnosed having choreoathetosis secondary to subclinical hypothyroidism and responded well to thyroxine replacement.
Background: An 80-year-old Chinese lady with background history
of hypertension presented to us with history of involuntary movements
of the left upper and lower extremities since three days prior to
admission to our hospital. She denies recent febrile illness and strokes
in the past. She is taking Hydrochlorothiazide 25mg OM, and Atenolol
100mg for her hypertension. On examination she was having choreoathetoid movements of left upper and lower limbs. Blood investigations, including routine hematology, biochemistry, caeruloplasmin,
copper and vitamin E levels were within normal limits. The patients
thyroid function tests revealed she has peripheral subclinical hypothyroidism with central correction: serum T3,T4 were normal and TSH
was elevated at 5.88 mu/l(0.36-3.24mu/l). AntiThyroglobulin antibodies and AntiThyroid peroxidase antibodies titres were markedly raised.
Imaging of brain MRI showed multiple old lacunar infarcts in both
corona radiata,thalamus and putamen. Diagnosis of choreoathetosis
precipitated by subclinical hypothyroidism was proposed and treatment
was initiated with thyroxine which fully resolved her involuntary
movements.
Methods: The aetiology of choreoathetosis is extensive, both acquired and inherited. Hormonal disorders including hyperthyroidism,
hypoparathyroidism are associated with choreoathetosis. Our case illustrates hypothyroidism also can manifest as choreoathetosis. The
pathogenesis of choreoathetoid movements in subclinical hypothyroidism remains unclear. Subclinical hypothyroidism is dened as a normal
serum free thyroxine with slightly high TSHlevels. Subclinical hypothyroidism prevalence is about 15% in women over the age of 60 years.
One third of those with subclinical hypothyroidism develop overt
hypothyroidism.
Conclusions: Our case denotes that the diagnosis of subclinical
hypothyroidism should be considered in patients who develop choreoatheotosis, because thyroxine substitution alleviates the choreiform
movements and as well prevent them from becoming overtly hypothyroid.
99
Encephalitis with hyperkinesias
A.E. Collins, S. Honarmand, C.A. Glaser (New York, New York, USA)
Objective: To describe encephalitis with hyperkinesias.
Background: The California Encephalitis Project (CEP) recently
identied a movement disorder clinical prole among encephalitis
patients. Except for a few reports, most described cases of encephalitis
with abnormal movements demonstrate Parkinsonism. We identied a
group of patients with hyperkinetic movements without bradykinesia,
rest tremor or rigidity within the CEP movement disorder cohort and
describe their clinical presentation, course, evaluation and treatment.
Methods: Patients were referred by treating physicians to the CEP. A
standardized case history form was used to collect demographic and
clinical data. Serum, cerebrospinal uid, and respiratory specimens
were obtained prospectively and were tested for the presence of viral,
chlamydial or mycoplasmal infection. Discharge summaries and charts
were reviewed when available.
Results: From 1998 through 2005, 26 of 47 patients in the Movement
Disorder prole had diffuse chorea with a mean age at presentation of
9.3 years. The prodrome included fever (54%), cough and coryza

POSTER SESSION I, TUESDAY, JUNE 5, 2007

(31%), and headache (23%), followed by irritability (54%), personality
change (50%), lethargy (89%) and possible seizures (88%). Progressive
encephalopathy, episodic agitation and hyperkinesias then developed in
all patients often with autonomic instability (82%). Initial CSF demonstrated a median of 23 WBC with a median lymphocytic predominance of 77%. Elevated CSF oligoclonal bands (OCB) were found in 5
of 6 tested. Initial brain MRIs were normal in 23 but later showed
global atrophy in 8. Despite extensive evaluations, possible VZV and
HHV6 infections were found in only 2 of 26 patients. Hyperkinesias
were uniformly very difcult to treat. Fourteen received immunosuppressive therapy without appreciable improvement. Seven required
tracheostomy and 13 gastrostomy tube placement. At hospital discharge, 15 were vegetative, 6 had a few words or purposeful movements, 4 had persistent language and behavioral problems, 1 was not
reported, and none were normal.
Conclusions: The etiology hyperkinetic encephalitis was elusive in
most patients referred to the CEP. While 5 patients had elevated CSF
OCB, the lack of response to immunomodulatory therapies does not
support an auto-immune cause in our cases. Further study is needed to
dene the underlying pathology, etiologies, potentially effective therapies and prognostic factors.

100
Oxidative stress parameters in plasma of Huntingtons disease
patients, asymptomatic Huntingtons disease gene carriers and
healthy subjects: A cross-sectional study
N. Klepac, M. Relja (Zagreb, Croatia)
Objective: To assess the presence of oxidative stress in Huntingtons
disease (HD) patients and its occurrence relative to the clinical symptoms.
Background: Animal data and postmortem studies suggest a role of
oxidative stress in HD, but in vivo human studies are missing.
Methods: Oxidative stress markers were determined in plasma of HD
patients (n19), asymptomatic HD gene carriers (with 38 CAG
repeats; n11) and their respective sex and age-matched healthy controls (n69) in a cross-sectional study. HD symptoms were evaluated
using the Unied Huntingtons Disease Rating Scale (UHDRS).
Results: With adjustment for age and sex, HD patients have signicantly
higher plasma lipid peroxidation (LP) levels (ratio 1.20, 95% CI 1.09 to
1.32, p0.001) and lower reduced glutathione (GSH) levels (ratio 0.72, CI
0.55 to 0.94, p0.011) than their age and sex-matched controls. Although
considerably younger, HD gene carriers did not differ from HD patients
regarding LP and GSH levels. They have higher plasma LP (ratio 1.16, CI
1.02 to 1.32, p0.016) and lower GSH than their matched controls (ratio
0.73, CI 0.5 to 1.05). They also have higher LP (ratio 1.18, CI 1.02 to 1.34,
p0.019) and lower GSH (ratio 0.75, CI 0.51 to 1.11) than the healthy
subjects matched to HD patients. Correlation analysis of oxidative stress
parameters and clinical features in HD patients indicated the following
associations: higher plasma lipid peroxidation and worse outcomes in the
verbal uency test (Spearman rho -0.620, p0.012), Stroop test (Spearman rho -0.496, p0.05) and a worse total chorea score (Spearman rho
0.497, p0.036).
Conclusions: Oxidative stress is more pronounced in HD patients and
asymptomatic HD gene carriers than in healthy subjects. Associations
between higher lipid peroxidation levels and worse total chorea score,
worse verbal uency score and worse results in the Stroop test in HD
patients observed in the present study may be viewed as circumstantial
evidence to support a role of lipid peroxidation in the onset/progression of
the clinical symptoms of HD. Differences in plasma LP and GSH are in
line with ndings in brain of HD animal models and might be indicator for
the brain pathology. One addition our data suggest is that oxidative stress
occurs before the onset of the HD symptoms.

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101
Early onset Huntingtons disease presenting with choreiform
movements in the abdominal muscles
A.A. Ege, B. Kocer, S. Bilen, N.S. Oztekin, F. Ak (Ankara, Turkey)
Objective: Here we present a case of atypical and early onset
Huntingtons disease (HD) presenting with choreiform movements in
the abdominal muscles. Being an unusual and atypical initial symptomatology not reported in the literature for HD before, we present this
case to emphasize the variability of syptoms in early onset cases.
Background: Early onset HD is an uncommon condition and it is
believed that these patients have a somewhat different clinical presentation
when compared to the typical adult onset form. Therefore, the presentation
of juvenile and childhood HD may lead to difculties in diagnosis. With
the development of molecular testing, diagnostic conrmation can now be
achieved with certainty if clinical suspicion is raised. The molecular basis
of the disease is the expansion of the trinucleotide CAG in the rst exon
of a gene on chromosome four (4p 16.3).
Methods: 25 year old patient presented with psychomotor deterioration, with unarticulated speech, progressive gait difculties, and
incoordination starting four years ago. He had progressive deterioration
of his ability for swallow, chew, and speech and developed urinary
incontinence. On physical examination, speech was signicantly dysarthric and hypophonic, almost incomprehensible, and he was unable to
extend his tongue. Muscle tone was diffusely increased. Deep tendon
reexes were increased.The patient had choreiform movemenets in
both rectus abdominus muscles.The family history was also positive for
HD we decided to perform genetic analysis.
Results: The 1-IT-15 gene CAG trinucleotide repeat revealed one
allel to be normal, while CAG repeat in the other allel was over 50 and
the result was consistent with HD.
Conclusions: Accurate genetic testing can now be offered in clinically
questionable cases and to presymptomatic subjects at risk for Huntingtons
disease and there is a correlation between the size of the expanded CAG
repeat and the age of onset in affected individuals. The predictive value of
this correlation, is limited due to the range of onset ages found at a given
repeat length in large series of patients. Expanded triplet repeats exhibit a
marked instability especially in male meiosis with a tendency to further
increase during transmission over the generations.
102
Therapeutics for Huntingtons disease: A systematic review
T. Mestre, J. Ferreira, M. Coelho, M.M. Rosa, C. Sampaio (Lisbon,
Portugal)
Objective: To evaluate the efcacy and safety of available pharmacological interventions to treat patients with HD.
Background: Huntingtons disease (HD) is an autossomal dominant
inherited movement disorder due to amplication of nucleic acids
triplets (CAG repeats). It is characterized by chorea, progressive dementia and psychiatric manifestations such as depression and psychosis. There is no specic therapy approved for HD. However, several
drugs are being used in clinical practice for its symptomatic control.
Methods: Conduct a systematic review about therapeutic interventions
for symptomatic control and modifying disease progression in HD. Search
strategy: electronic searches of The Cochrane Controlled Trials Register
(The Cochrane Library Issue 4, 2006), MEDLINE (1966-2006), EMBASE
(1974-2006), were conducted. Grey literature was hand searched and the
reference lists of identied studies and reviews examined. Selection criteria randomised controlled trials with more than 10 patients recruited
comparing any pharmacological treatment with placebo in the treatment of
patients with a clinical diagnosis of HD.
Results: 27 trials including a total of 1266 patients met the inclusion
criteria; 6 were on disease progression and 21 on symptomatic treatments.
15 were parallel studies and 12 were crossover studies. The majority of
trials had methodological and/or reporting shortcomings. Only 3 clinical
studies concluded on an effect on valid clinical measures (UHDRS motor
scale): tetrabenazine, amantadine and riluzole. No intervention was found
to be effective as a disease modifying therapy.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Conclusions: Based on the best available information, no condent

statement can be made about the effectiveness of pharmacological
interventions to treat patients with HD.
103
Serum brain-derived neurotrophic factor (BDNF) changes in Huntingtons disease subjects
J. Sassone, A. Ciammola, M. Cannella, B. Poletti, L. Frati,
F. Squitieri, V. Silani (Cusano Milanino, Milano, Italy)
Objective: To investigate possible changes in BDNF concentrations
in serum from patients with Huntingtons disease (HD).
Background: HD is a neurodegenerative disorder characterized by
motor, cognitive, and psychiatric symptoms and by a progressive
degeneration of neurons in basal ganglia and in brain cortex. Brainderived neurotrophic factor (BDNF) is a pro-survival factor for striatal
neurons. Some evidence implicates a brain BDNF deciency, related to
mutated huntington expression, in the selective vulnerability of striatal
neurons in HD.
Methods: We compared BDNF serum levels in 42 patients with HD
(range 28-72 years, mean age 51.9 11.5), and 42 age-matched
healthy subjects (range 25-68 years, mean age 48.2 12.5). We evaluated the potential relationship between BDNF serum levels, CAG
repeat number (range 40 to 54, mean 44.8 3.4) and duration of illness
(range 6-228 months, mean 103.6 62.1).
Results: The mean BDNF serum concentration was signicantly
lower in patients with HD than in healthy controls. Lower BDNF levels
were associated with a longer CAG repeat length and a longer duration
of illness. Severity of the illness, as assessed by the Unied Huntingtons Disease Rating Scale (UHDRS) motor and cognitive scores, was
negatively related to serum BDNF levels. As a preliminary investigation we assessed BDNF levels in 7 presymptomatic HD subjects,
nding values signicantly lower compared to age-matched controls.
Conclusions: These results in vivo conrm that the huntingtin mutation causes BDNF production to decline and show that the BDNF
deciency is detectable in HD patients sera. Although BDNF changes
could be present in other neurodegenerative disorders characterized by
cognitive and motor impairment, the signicant correlation of serum
BDNF with HD duration and mutation size strongly suggests that the
BDNF variability in peripheral HD tissues could be a useful in vivo
biomarker.
104
Sydenhams chorea may be associated with sustained monocyte
activation
K.C. Torres, W.O. Dutra, D.P. Maia, F. Cardoso, K.J. Gollob,
A.L. Teixeira (Belo Horizonte, Brazil)
Objective: To evaluate subsets of lymphocytes and monocytes in
Sydenhams chorea patients and matched control subjects.
Background: Sydenhams chorea (SC) is the neurological complication of group A beta-hemolytic streptococcal infection and constitutes
one of the major criteria of acute rheumatic fever (RF). It is considered
an autoimmune disorder determined by basal ganglia dysfunction mediated by autoantibodies. Previous studies assessed the cytokines involved in this process and they found a predominant Th2 prole which
is compatible with an antibody mediated disease. Nevertheless no study
evaluated directly the cellular immune response in SC.
Methods: Twenty subjects over 18 years-old were recruited for this
study: 9 non-acute SC patients (M/F, 2/7; mean age SD, 23.0
10.9) and 11 healthy individuals (M/F, 6/5; mean age SD, 23.2
5.4). Each patient was scored based on the UFMG Sydenhams chorea
Rating Scale (USCRS). Blood was collected aseptically and cells were
separated by a gradient of Fycoll and submitted to surface cellular
staining for ow cytometry analyses. The following surface markers
related to specic cell subsets were studied: CD5 (in CD19 B
cells), CD4 and CD8 (in helper and cytotoxic T cells, respectively)
and CD14 (in monocytes); functional and activation state for T cells
(CD25, CD68 and CD45RO) and monocytes (CD80, CD86 and

Movement Disorders, Vol. 22, Suppl. 16, 2007

HLA-DR). Statistical analysis was performed using the nonparametric

Mann-Whitney test.
Results: There was no difference in the percentage of B and T cells
expressing surface markers of activation in CS and control groups. The
percentage of CD14 cells, i.e. monocytes, expressing CD80 in SC
group (mean SD, 33.5 25.1) was much higher than in control
group (mean SD, 12.0 5.8; p0.001). The percentage of CD86
and HLA-DR expressing CD14 monocytes were not different from
control group.
Conclusions: No signicant alteration was found in B and T cells
percentages in peripheral blood of patients with non-acute SC. There is
some evidence of sustained monocyte activation in SC patients.
105
Chorea in adults after pulmonary thromboendarterectomy with
deep hypothermia and circulatory arrest
R.M.A. De Bie, H.M.M. Smeding, M.A.J. Tijssen (Amsterdam,
Netherlands)
Objective: To investigate features predictive for chorea after pulmonary thromboendarterectomy with deep hypothermia and circulatory
arrest.
Background: Pulmonary thromboendarterectomy (PTE) is indicated
for chronic pulmonary hypertension due to thromboembolic obstruction of the major pulmonary arteries. Four of the rst 21 consecutive
adult patients who underwent PTE in our hospital developed chorea as
an adverse effect. We did not nd other reports of chorea in adults
following deep hypothermia and circulatory arrest.
Methods: PTE involves median sternotomy, cardiopulmonary bypass, deep hypothermia, and periods of hypothermic circulatory arrest.
A neurologist evaluated the patients demonstrating neurological symptoms. The records of all patients were assessed for used medication,
cardiopulmonary bypass time, deep hypothermia time, number of periods and duration of hypothermic circulatory arrest, duration of reperfusion after hypothermic circulatory arrest, and minimum temperature.
Results: The generalized chorea started one to three days after
surgery and gradually faded. The mean age of the four patients with
chorea was 42.4 years (SD 11.5 years) and of the 17 patients without
chorea 53.8 years (SD 15.2; p0.12). Total time on pump and total
time of aorta occlusion were not statistically signicantly different
between the two patient groups. The total time of circulation arrest
lasted a mean of 61 minutes in the patients with chorea, range 51 to 73
minutes, and a mean of 44 minutes in the patients without chorea, range
11 to 75 minutes (p0.018).
Conclusions: Transient chorea following deep hypothermia with
cardiac arrest can also occur in adults and is related to lengthy circulation arrest.
106
Motor neuron disease and chorea
J. Klempir, O. Klempirova, Z. Lebedova, J. Roth (Prague, Czech
Republic)
Objective: We present a case of a woman, by whom mild dysartria,
dysphagia, weakness of proximal parts of extremities - predominatly in
arms - and perioral dyskinesias slowly and simultaneously started to
develop at the age of 58. Family and personal history were negative. At
the age of 65 years, the speech problems were quite stable with no
progression, but the swallowing difculties worsened and the dysphagia was already located in the pharyngeal phase of swallowing. An
inability to keep the tongue steadily protruded, fasciculations and signs
of atrophy were present. She had frequent blinking, perioral dyskinesias and vocal tics resembling clicking. We observed amyotrophy
mainly in proximal parts of arms, areexia, pyramidal signs and mild
choreatic dyskinesias of hands. There was also weakness of proximal
parts of lower extremities, but she had hyperreexia, clonus without
pyramidal spastic signs and spasticity and also choreatic and myoclonic
dyskinesias. She was not able to stand without strong support and she
could move only on a wheelchair. Repeated neuropsychological examinations indicated mild dysexecutive syndrome. According to electro-

POSTER SESSION I, TUESDAY, JUNE 5, 2007

myographic investigation patient fulled the El Escorial criteria for the
diagnosis of amyotrophic lateral sclerosis. Magnetic resonance imaging
revealed moderate diffuse atrophy of cerebral cortex, in contrast to
brain stem and spinal cord, which did not show any abnormalities.
Huntington disease, neuroacantocytosis and drug induced dyskinesias
were not conrmed. Paraprotein IgG was repeatedly identied in the
cerebrospinal uid and blood serum, but paraneoplastic syndrome was
absent. Percutaneous gastrostomy was performed. Treatment with riluzol did not show any effect. The parallel progression of both symptoms (chorea and motor neuron involvement) indicates multiple progressive lesions in the central and peripheral nervous systems, which
ethiopatogenic basis remains unknown. Furthermore, the progression
of motor neuron lesion is slower than usually expected in motor neuron
disease. We found only three similar cases described in the literature.
The video presentation of our patient will be available thorough the
poster section.
107
Inefcient deep brain stimulation in a young patient suffering from
choreoathetosis
K. Schumm, K. Kiening, M.C. Kraus, M. Krause, M. Kloss
(Heidelberg, Germany)
Objective: Although the term athetosis has been abandoned in favour
of dystonia, athetosis is a unique movement disorder caused by perinatal brain damage. Pallidal deep brain stimulation improves symptoms
of dystonia in most cases dramatically. Yet the effect of deep brain
stimulation on athetosis is unclear.
Background: We report of a 15 year old male patient with a pharmacological intractable severe athetosis, who was referred to our clinic
for deep brain stimulation (DBS). After a mild birth trauma he remained motor retarded with minimal cognitive impairment. From the
rst year on he had tonic posturing and athetotic hand movements.
With the age of three he developed faster more proximal choreoatic
hyperkinesias. With the age of 13 he had to be tied to the wheel chair
to prevent him from falling off. He was completely dependent in all
activities of daily living. Speaking and swallowing was impaired so that
the patient weighed only 19,1kg body weight. The initial MRI revealed
no abnormal ndings. Genetic testing for DYT 1 mutation was unremarkable. The clinical nding was that of a severe choreoathetosis with
slow, vermicular nger movements and faster proximal hyperkinesias
(see video).
Methods: DBS electrodes were placed bilaterally into the medial part
of the globus pallidus internus (GPi). Stimulation parameters were set
to 210s impulse width and 130 Hz and 2.5V in the second most distal
contact, which did not cause phosphenes.
Results: Although stimulation parameters have been changed (impulse width 450 s, high and low frequency, different contact, bi- and
monopolar) DBS did not improve symptoms within 2 years (BurkeFahn-Marsden Motor Score 112 before and after DBS).
Conclusions: So far, the pathophysiological alterations in choreoathetosis have not been fully elucidated. Two multicenter studies (Vidailhet et al. 2005, Kupsch et al. 2006) demonstrated a signicant benet
of DBS for primary dystonia. All reported choreoathetosis so far has
not been successful treated with GPi-DBS therefore from a therapeutic
point of view choreoathetosis has to be distinguished from other forms
of dystonia. Further randomised trials are necessary to validate these
results.

CLINICAL ELECTROPHYSIOLOGY
108
Peripheral neuropathy and plasma homocysteine level in Parkinsons disease patients: A pilot study
M. Nevrly, H. Vranova, Z. Chovancova, I. Nestrasil, P. Otruba,
J. Dufek, P. Kanovsky (Olomouc, Czech Republic)
Objective: The aim of this study was to correlate levels of homocysteine (Hcy) and nerve conduction parameters in both motor and
sensory nerves of lower limbs in Parkinsons disease (PD) patients

S33

Background: Hcy is a risk factor for vascular diseases, dementia, and

cognitive impairment. However, elevated Hcy serum level is also
considered a neurotoxic factor, inducing a predominantly axonal degeneration in peripheral nerves. Elevated plasma Hcy levels have been
found in PD patients treated with L-dopa. This has been veried by
several retrospective studies.
Methods: The assessment of sensory nerve conduction velocity
(SNCV) and sensory nerve action potentials (SNAP) of the right sural
nerve and the left peroneal supercial nerve, assessment of motor nerve
conduction velocity (MNCV) and motor nerve action potentials
(MNAP) of the right tibial nerve and left peroneal nerve and assessment of H-reexes latency of the bilateral tibial nerves were performed
in 18 PD patients (aged 29-73, mean 61.8 10.3 years). Hcy plasma
levels were also examined in all of these patients.
Results: Plasma Hcy levels in the patient group were: 13.3 4.4 (6.2
22.6) mean SD (min-max) (umol/l). Six patients had elevated
plasma Hcy level more than 15 umol/l. Abnormal SNCV (45m.s-1)
was found in 9 patients at right sural nerve and in 10 patients at left
supercial peroneal nerve. Abnormal MNCV (40m.s-1) was found in
4 patients at right tibial nerve and in 3 patients at left peroneal nerve.
However, no statistically signicant relationship between plasma Hcy
level and any electromyographical parameter of neuropathy was found.
Conclusions: Increase of plasma Hcy level was found in PD patients
treated with L-dopa in several studies. Axonal-accentuated degeneration in peripheral nerves was more frequently found in PD patients than
in other population. Although hyperhomocysteinemia is considered a
neurotoxic factor, results of this study do not conrm this hypothesis.
Other studies with a larger number of patients are necessary to address
this issue.
109
Differential modulation of cranial and limb muscle function by
levodopa in Parkinsons disease
P.B. Tawadros, J.A. Burne (Sydney, NSW, Australia)
Objective: To compare the effects of oral levodopa in limb and
cranial muscle function.
Background: Oral levodopa has well established efcacy in ambulatory and upper limb function in Parkinsons disease (IPD). Its response in cranial muscle function is infrequently studied and more
objective studies are needed. As a result opinion regarding its efcacy
in deglutition is divided. Current studies in deglutition in IPD have
chiey utilised videouroscopic methods and/or rating scales. Physiological methods such as accelerometry and EMG can provide accurate
information on timing, magnitude and duration of muscle activity
during deglutition and speech, providing a basis for comparison with
limb function.
Methods: A goniometer xed across the second metacarpal joint, an
accelerometer on the tip of the second digit and surface EMG from the
rst dorsal interosseus (FDI) muscle measured joint angle, acceleration
and muscle activity respectively during rapid voluntary alternating
nger movements. Deglutition was assessed (Ertekin et al.,1996) using
6 incremented water boluses 3-25 mls. Surface EMG from the submental and infrahyoid muscle and accelerometry (xed to the skin over
the coniotomy region) were recorded. Patients were assessed before
and 1.5 hours after levodopa.
Results: Preliminary analysis found consistent improvement after
medication in mean acceleration and velocity values in self-paced and
externally timed nger tasks to above 80%. Swallowing results were
variable between patients. A positive response to medication was
indicated by marked reduction in the number of attempted swallows per
bolus. In this case, burst durations of both sub-mental and infra-hyoid
EMG were reduced and peak-to-peak amplitude of the coniotomy
accelerometer signal increased by 29%. A negative response to medication was indicated by no reduction in the number of attempted
swallows per bolus. In this case, burst durations of both sub-mental and
infra-hyoid EMG were not reduced and peak-to-peak amplitude of the
coniotomy accelerometer signal not increased.
Conclusions: Preliminary results indicate that simple biomedical
measures can provide objective measures of swallowing and this func-

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

tion responds to medication inconsistently even in patients with signicant improvement in limb function.
110
Elecrically and auditory evoked brain stem reexes in cervical
dystonia
M.E. Kiziltan, A. Gunduz, O. Uyanik, R. Sahin (Istanbul, Turkey)
Objective: In this study, we aim to investigate reex responses from
orbicularis oculi (O.oc) and neck muscles elicited by different types of
stimuli (somatic trigeminal and extratrigeminal as well as auditory) in
cases of cervical dystonia (CD).
Background: Brain stem excitability is known to be abnormally
enhanced in CD. Previous studies indicated abnormal alterations in
blink reex (BR) recovery curves. On the other hand, auditory startle
reaction (SR) probabilities and magnitude were reported to be reduced.
Methods: Twenty-eight consecutive patients diagnosed as primary
CD (nine men, 19 women; age range between 27-62) were included and
results were compared with 12 healthy volunteers (two men, 10 women; age range: 23-60). Electrophysiological investigations were performed before the botulinum toxin treatment in all patients. Nonrectied surface electromyographic recordings were obtained
simultaneously from O.oc, sternocleidomastoid (SCM), splenius capitis
ve trapezius muscles. Reex responses were elicited by electrical
supraorbital and median as well as auditory stimuli.
Results: BR was obtained in all patients in both groups by both
supraorbital and auditory stimuli. R2, auditory BR and median BR
latencies in CD group 35.45.7, 38.98.6 and 53.512.2, respectively. Latencies of SCM response by these stimuli were 62.410.4,
83.838 and 8024.2, respectively. Probability of SCM responses
obtained by auditory stimuli was higher patients with CD (89.7% vs
50.0%, p0.006), whereas responses by supraorbital stimuli didnot
differ between two groups (89.7% vs 83.8%, p0.602). By median
stimulation, there were no responses from SCM in control group
compared to 14 (50%) in CD group, in all patients responses were
bilateral. Responses by all types of stimuli habituated easily in normal
group, however, in 10 patients we obtained stable and prolonged tonic
activity which could not be supressed by any stimulus modalities.
Conclusions: We observed increased response probability from neck
muscles by each modality and stable tonic activity in 10 patients which
couldnot be supressed by stimuli. These ndings suggest abnormal
brain stem reex excitability in CD. Contrary to some previous reports
we established that increased probability of SR indicates increased
brain stem excitability.
111
Reex inhibition of muscle cramp by electrical stimulation of
muscle tendons
S.I. Khan, B.A. John (Sydney, NSW, Australia)
Objective: To test the possibility that muscle cramp can be inhibited
by electrical stimulation of reex afferents in the muscle tendon.
Background: Muscle cramp is an intense, painful, and involuntary
muscle contraction. The debate over the origin of these discharges
within peripheral nerve, muscle or the central nervous system is ongoing. If we can modify cramp activity through the muscles reex
connection, it implies synaptic drive of the motorneorons by the central
nervous system.
Methods: Surface EMG was recorded from the medial and lateral
heads of gastrocnemius (GA). The EMG signals were rectied, low
pass ltered and averaged over 25 electrical stimuli. The area under the
baseline EMG was calculated to determine the strength of inhibition in
the interval 55-125ms after the stimulus. The inhibitory response to
tendon stimulation was assessed during normal voluntary contractions
of 20%, 40%, 60% and 80% of maximum contraction in 10 subjects. A
cramp was then induced in one head of GA in eight of thirteen subjects
by maximum contraction in full plantarexion. Cramp in GA was
painful, involuntary and localized. Induction of cramp was indicated
electrically by the presence of EMG activity in one head of GA while
the other head remained silent.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Results: In all subjects, reex inhibition of cramp EMG activity was

observed following tendon stimulation and the subjects reported relief
of cramp after 30 or more shocks had been delivered to the tendon.
When the inhibition of cramp-generated EMG and voluntary EMG was
compared at similar background EMG levels, the area and timing of the
inhibition did not differ signicantly. Thus the cramp-generated EMG
is also likely to be driven by spinal synaptic input to the motorneurons.
Conclusions: Our observations clearly show that muscle cramp can be
inhibited by stimulation of tendon afferents in the cramped muscle. This
strongly suggests that the same reex pathway was the source of the
inhibition in both cases. The muscle conditions that appear necessary to
facilitate cramp, a near maximal contraction of the shortened muscle are
also the conditions that render the inhibition generated by tendon afferents
ineffective. It is thus possible that reduced inhibitory feedback from the
tendon may have an important role in generating cramp.
112
Hyperexcitable motor responses to ash stimulation in Parkinsons
disease: A TMS study
S. Tamburin, A. Fiaschi, P. Manganotti, F. Milanese, A. Polo,
G. Zanette (Peschiera del Garda, VR, Italy)
Objective: To evaluate how photic stimulation modulate motor excitability in PD patients.
Background: Hyperexcitable motor responses to somatosensory
stimuli have been reported in Parkinsons disease (PD).
Methods: The effect of white-light ash stimulation on motor evoked
potentials (MEPs) to transcranial magnetic stimulation (TMS) was evaluated in thenar muscles of 8 PD patients and 8 normal controls. Flash
stimuli preceded TMS at interstimulus intervals (ISIs) of 20-100 msec.
Results: Flash stimulation caused MEP amplitude reduction in controls (up to 51% of test MEP) and on the less-affected side of patients
(up to 62% of test MEP) at ISIs of 40-70 msec. This inhibitory effect
was absent on the more-affected sides of patients (ANOVA: p 0.05).
No changes were found in MEP latency in controls and patients.
Conclusions: The physiological motor inhibition to ash stimulation
is absent in the more-affected sides of PD patients. This abnormality
may be one of the features of motor hyperexcitability in PD. Altered
photic responses may play a role in the pathogenesis of parkinsonian
symptoms.
113
Evidence of negative myoclonus in clozapine induced folding legs
phenomena and drop attacks
D. Murgia, L. Fabiano, N.J. Toms, C. Cordivari (London, United
Kingdom)
Objective: To describe the neurophysiology of tremor and folding
legs in a patient on treatment with clozapine.
Background: Clozapine is reported to induce myoclonus, leg folding
and drop attacks. We report a patient treated with clozapine for refractory schizophrenia who experienced intentional tremor, with dysgraphia, postural instability and unsteady gait. The intensity and the
duration of her lower limbs myoclonus was sufcient to cause her to
have falls.
Methods: Simultaneous video EEG-EMG recording of the bilateral frontocentral regions and right limbs was performed. Backaveraging, EEG-EEG and EEG-EMG coherence analyses were performed. The patient was recorded at rest, when sitting and holding
the upper or lower limbs outstretched against gravity and when
standing.
Results: The patient was alert and cooperative and EEG showed
occasional slow waves in the anterior regions. No jerks were recorded at rest. When holding the hand against gravity or lifting her
legs, sudden lapses of muscular activity and brief jerks were observed. EMG demonstrated that the jerks were associated with
bursts of muscle activity lasting for 20-50 ms and lapses with
sudden periods of electrical silence lasting 50-100 ms. There was a
clear rostro-caudal pattern of muscle activation for both positive and

POSTER SESSION I, TUESDAY, JUNE 5, 2007

negative phenomena, with progression and latency consistent with
the pyramidal pathway. However, back-averaging failed to show a
clear cortical correlate, probably because of the high frequency of
the events. EEG-EMG and EMG-EMG coherence analyses showed
coherence between proximal and distal muscles at high frequencies
(70Hz).
Conclusions: The study demonstrates that clozapine-induced folding
legs and drop attacks may be caused by negative myoclonus. Duration
of jerks, electrical silence, progression of muscle activation and interruption and exaggerated coherence at high frequency are suggestive of
positive and negative myoclonus of cortical origin.
114
Executive functions processed in the frontal and lateral temporal
cortices. An intracerebral event-related de/synchronization study
with writing of single letters
M. Bockova, J. Chladek, P. Jurak, J. Halamek, I. Rektor (Brno,
Czech Republic)
Objective: The study was designed to investigate the neurocognitive
network in the frontal and lateral temporal cortices that is activated by
complex cognitive visuomotor tasks with letter writing. We were
interested in the executive functions.
Methods: Eight epilepsy surgery candidates with implanted intracerebral depth electrodes (654 recording sites) performed two tasks
involving the writing of single letters. The rst task consisted of
copying letters that had been randomly projected on a monitor. In
the second task, the patients were requested to write any letter other
than this that appeared on the monitor, but not a letter that would
immediately precede or follow that letter alphabetically. The cognitive load of the second task was increased mainly by larger
involvement of the executive functions. The task-related eventrelated de/synchronization (ERD/ERS) of the alpha, beta and
gamma rhythms was studied.
Results: The cerebral network connected with processing the
writing of single letters comprised the primary sensorimotor cortex,
anterior cingulate, premotor cortex, SMA, the temporal pole, and
the parietal cortex. The increased cognitive load activated preferentially the dorsolateral and ventrolateral prefrontal cortex, orbitofrontal cortex and surprisingly also the temporal neocortex.
Conclusions: The executive fuctions are traditionally connected with
the frontal cortex, especially with the dorsolateral prefrontal cortex. In
our study other areas were specically activated by the increased
cognitive load, particularly the temporal neocortex. The lateral temporal cortex together with frontal areas forms a cognitive network processing executive functions in complex visuomotor task.
115
ParkinSense comparison to the unied Parkinsons disease rating
scale: Preliminary tremor and bradykinesia results
J. Giuffrida, L.C. Trout, E. Mather, B. Maddux, D. Riley (Cleveland,
Ohio, USA)
Objective: To design, build, and clinically assess a wireless movement disorder monitor, ParkinSense, to monitor and quantify motor
manifestations of Parkinsons disease.
Background: Two major Parkinsons disease (PD) manifestations
that affect quality of life are tremor and akinesia. The current
evaluation standard is the Unied Parkinsons Disease Rating Scale
(UPDRS), a qualitative ranking system completed during an ofce
visit. Continuous objective quantication of PD manifestations
would allow clinicians to capture complex uctuation patterns in
response to treatment interventions, and aid in evaluating efcacy of
treatment protocols.
Methods: ParkinSense is a small, lightweight, wireless user-worn
system consisting of orthogonal accelerometers and gyroscopes. ParkinSense was used to collect data from the hand of 40 PD subjects as
they completed a subset of the UPDRS upper extremity motor exam for
tremor and akinesia. ParkinSense clinical data collection consisted of
three parts: data collection, video editing, and clinical scoring. All

S35

clinical testing was completed at the Movement Disorders Center at

University Hospitals of Cleveland. Collected data were processed and
used to train and test an algorithm to generate scores similar to clinician
scores for each task.
Results: The ParkinSense output score for tremor produced values
similar to the average clinician scores. The RMS error was 0.056 for
the tremor training set and 0.119 for the generalization set. When the
ParkinSense output was regressed against the clinician scores the
R-squared values were 91.4% and 82.4%. For akinesia, the ParkinSense output score produced values similar to the average clinician
scores with an RMS error of 0.052 for the akinesia training set and
0.140 for the generalization set. When the ParkinSense output was
regressed against the clinician scores the R-squared values were 93.1%
and 85.4%.
Conclusions: A portable system that can automatically guide
subjects through a subset of the UPDRS exam and calculate scores
should provide great utility in many applications. This would provide a more continuous and objective measure for monitoring the
effects of dose times and levels for subjects at home and documenting the efcacy of novel pharmaceutical drug trials to control PD
manifestations.
116
Extracellular microrecordings during stereotactic neurosurgery
for Parkinsons disease: Spike descriptors in the human subthalamus and substantia nigra
S. Mrakic-Sposta, S. Marceglia, F. Cogiamanian, M. Egidi,
P. Rampini, M. Locatelli, G. Carrabba, M. Vergari, A. Priori
(Milan, Italy)
Objective: To obtain quantitative information on neuronal spike
descriptors, we systematically analyzed extracellular microrecordings
in the subthalamic nucleus (STN) and in the substantia nigra pars
reticulata (SNr) in awake patients undergoing stereotactic neurosurgery
for deep brain stimulation (DBS) electrode implantation.
Background: Intraoperative neuronal microelectrode recordings are
used to help localizing the target structure in DBS surgery. Intraoperative monitoring rests on the ability to distinguish the various anatomical structures by interpreting the available recorded signal. A way to
evaluate neuronal activity is to study action potential characteristics
through extracellular recordings. However, the spike descriptors in the
STN have been poorly characterized.
Methods: We evaluated spike amplitude, area, duration, rise time and
mean total ring rate in 11 patients undergoing surgical procedures for
DBS. Signals were captured through bipolar microelectrodes (Inomed
mod. 230760, tip impedance 1.5-2.5 M at 1KHz), bandpassed 300Hz5KHz and differentially amplied.
Results: Microelectrode recordings showed a larger spike area and
amplitude in the SNr than in STN ([meanSD] amplitude: 46.731.1 vs
36.329.6 mV; area: 25.624.2 vs 36.721.4 Vmsec), a higher total
ring rate at rest in the SNr than in STN (78.653.5 vs 61.940.8 Hz), a
longer duration and rise time in the SNr than in STN (duration: 2.01 vs
1.30.6 ms; rise time: 2.01.0 vs 1.30.6 ms). Our analysis also disclosed gender-related differences: males had larger spike area, larger
amplitude, and lower mean total ring rate than females (amplitude:
41.9732.57 vs 26.219.7 V, p0.001; area: 31.826.4 vs 13.010.6
Vmsec, p0.001; ring rate: 66.753.4 vs 82.850.8 Hz p0.05).
Conclusions: In conclusion, our results have implications for the
clinical practice and ground the development of algorithms for the
neurophysiological identication of the STN during stereotactic neurosurgery for PD.
117
Long-term effect of locally administered botulinum toxin a on
neuromuscular transmission: Longitudinal single-ber EMG study
S. Vohanka, B. Micankova, J. Bednarik (Brno, Czech Republic)
Objective: Longitudinal single- ber EMG (SFEMG) study in patients with involuntary movements treated with long- term administration of botulinum toxin A (BTXA).

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: There are no relevant data concerning on long- term

effect of BTXA on neuromuscular transmission in distant (non- injected) muscles.
Methods: Seven patients treated with BTXA for therapy of focal
dystonias (cervical dystonia, blepharospasm, and hemifacial spasm)
were examined using SFEMG (neuromuscular jitter) of the extensor
digitorum. communis muscle. Intramuscular axonal stimulation method
by Stalberg and Trontelj1 was used before the rst administration,
twice after rst injection (2 weeks and 4-9 weeks), and after 7-8 years
of the regular application of BTXA. In each patient at least 20 pairs
were examined and the mean individual jitter (mean MCD) was estimated.
Results: The average value of mean MCD was before rst injection
of BTXA 20.0 s (15-25 s, SD 3.1 s), 2 weeks after injection 21.7
s (17-32 s, SD 4.6 s), 4-9 weeks after application 18.6 s (16- 23
s, SD 2.4 s), and after 7-8 years 20.5 (17- 25 s, SD 3.2 s).
Differences are not signicant (paired t- test, p0.05). The abnormal
mean MCD (32 s; normal value 26 s) was found only in one
patient 2 weeks after injection. This mild abnormality (all individual
end-plate pairs were in normal limit 40 s) was without any clinical
relevance. We were not able to document any association between
SFEMG ndings and the cumulative dose of injected BTXA.
Conclusions: The study has shown that the small increase of neuromuscular jitter just after the BTXA injection is a transient effect and
long-term administration has no cumulative effect on the neuromuscular transmission in remote muscles.
118
Delayed blink reex in Lewy bodies dementia
L. Bonanni, F. Anzellotti, S. Varanese, A. Thomas, L. Manzoli,
M. Onofrj (Pescara, Italy)
Objective: We hypothesize that BR might be altered in patients with
LBD or other parkinsonisms presenting with RBD.
Background: The electric Blink Reex (BR) is a neurophysiological
technique exploring pontine structures through a reex arc connecting
nuclei of the 5th to the nuclei of the 7th cranial nerve.It consists of three
separate responses, R1, R2, R3, the rst one generated in the trigeminofacial reex arc, the second and third one generated in polysynaptic
pathways involving the brainstem reticular formation. Clinically, the
BR is used to evaluate brainstem lesions and it has been applied in
clinical-neurophysiological studies of brainstem lesions and neurodegenerative disorders.A recent pathophysiological hypothesis suggested
that in Lewy body dementia (LBD) and Parkinsons disease (PD) the
brainstem is the site of initial lesions, consisting of -synuclein deposits.
Methods: Blink reexes by electric stimuli of the supraorbital nerve
were recorded in 26 patients with LBD, 26 patients with MSA, 26
patients with PD, with or without RBD, and in 20 patients with AD and
20 with PSP without RBD, and compared with recordings in 30 healthy
controls.Surface electrodes were placed on the inferior part of the
orbicularis oculi muscles on each side, recording ipsilateral R1, and
ipsilateral and contralateral R2 and R3. Ground electrode was placed
under the chin.Stimuli of 0,1ms of duration with intensity of 5-10 mA
elicited stable R1 in repeated trials. Signals were amplied and ltered
(bandwidth 20-2000Hz). In case of recording repetition due to the
absence of R1, we used interstimuli intervals of at least 7 sec to avoid
habituation.
Results: BR were signicantly delayed (p0.001) only in LBD
patients in comparison with controls and with the other groups of
patients. 14 patients had BR latency above 2 SD of the control mean,
ranging from 31.2 to 46.3 ms. BR latency was not related with the
presence of RBD, while a Spearman correlation rho of 0.68 was found
with scores assessing the presence of cognitive uctuations. R2 delay
was prominently (71.5%) bilateral.
Conclusions: BR recordings might reveal brainstem dysfunction in
LBD, but not in other parkinsonisms where different yet denite
brainstem abnormalities are also described.

Movement Disorders, Vol. 22, Suppl. 16, 2007

119
The role of ipsilateral motor cortex in complex nger movements:
A rTMS study
L. Avanzino, A. Tacchino, C. Ogliastro, M. Bove, C. Trompetto,
G. Abbruzzese (Genova, Italy)
Objective: To analyze the effect of inhibitory repetitive transcranial
magnetic stimulation (rTMS) over ipsilateral motor cortex (ipsiM1) on
the execution of sequential movements of different complexity.
Background: Functional imaging studies in humans have shown that the
activation of ipsiM1 during unimanual nger movements depends on
movement complexity. Inhibitory rTMS over ipsiM1 has been shown to
improve sequential simple nger movements in normal controls.
Methods: Ten healthy subjects performed repetitive nger opposition movements of different complexity (at a 2 Hz metronome frequency) wearing a sensor-engineered glove with their right hand. The
following parameters were analyzed: inter tapping interval (ITI), touch
duration (TD), timing error (TE), and movement rate (MR). The tasks
were performed before, immediately after, and 15-30 minutes after 1
Hz rTMS (two conditioning trains of 750 stimuli at 90% of active
motor threshold) applied over ipsiM1. All the subjects also received
sham stimulation. Six subjects participated in a control experiment in
which the task was performed only before and 30 minutes after rTMS.
Results: In both sequences ITI signicantly decreased after rTMS
and remained stable at 30 minutes (ANOVA: p0.01); ITI did not
change after sham stimulation. Since the subjects were instructed to
follow the metronome frequency (2 Hz), 60% of the subjects increased
TD (ANOVA: p0.01), while 40% of the subjects tended to increase
the effective movement rate (ANOVA n.s.). In the control experiment
ITI did not change 30 minutes after the rTMS session.
Conclusions: Inhibitory rTMS over the ipsilateral motor cortex improves the execution of sequential nger movements of different complexity, making the movement faster. Repeated performance of the task
(every 15 minutes) seems to prolong the effect of rTMS session with a
sort of use dependant plasticity. These results may suggest new
therapeutic strategies for movement disorders.
120
Use of a geste antagoniste device in a case of cervical dystonia
N.J. Toms, C. Cordivari (London, United Kingdom)
Objective: To mimic a dystonic patients sensory trick using a simple
mechanical device.
Background: A 52-year old male presented with painful dystonia of the
neck. This was characterized by a marked head tilt to the right, accompanied by minimal rotation to the left. Injections of botulinum toxin had
helped to reduce muscle tension and pain, but did not improve posture.
Treatment with physiotherapy, acupuncture and osteopathy had only limited benet. His head posture could be dramatically improved by light
stimulation to the back of the head. This could be achieved by brushing,
either by hand, or by light contact with walls or furnishings. Wearing a hat
or similar item with no movement on the scalp produced no benet. We
built a simple mechanical device to provide this stimulation.
Methods: The device was designed to attach to the arms of the patients
spectacles. Three copper wires were soldered together into a T conguration. The cross-pieces were threaded into a standard spectacles strap,
allowing the down-piece to rest on the patients neck and two short upright
arms to rest higher up the patients head. EMG measurements were made
with surface electrodes on the Trapezius and Sternocleidomastoid and with
monopolar needle electrodes in the Splenius Capitis and Levator Scapulae
muscles bilaterally. Measurements were taken while the patient was relaxed, both with and without the device in place.
Results: The position and mobility of the head improved markedly
when the device was in place. Some abnormality of posture remained,
but the subjective effort required to normalize this was much reduced
compared to when the device was not used. EMG Data was analyzed
using in-house software and Matlab. When the device was not used,
there was signicant coherence between ipsilateral agonist muscle pairs
(Sternocleidomastoid and Trapezius versus Splenius Capitis and Levator Scapulae), with a peak at 5Hz, except between the Trapezius,

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Splenius Capitis and Levator Scapulae on the right hand side. These
coherences disappeared when the device was in place, except between
the left Levator Scapulae and the Sternocleidomastoid and Trapezius.
Conclusions: In this preliminary study we have demonstrated that a
simple mechanical device can be an effective treatment for neck
dystonia in a patient with a marked sensory trick. The device may work
by modifying the common drive to muscles, as evidenced by EMGEMG coherence.
121
Validation of spiral analysis for quantication of motor improvement in Parkinsons patients after deep brain stimulation
M.M. Kurtis, Q. Yu, A.G. Floyd, R.R. Goodman, G.M. McKhann,
L. Wineld, B. Ford, L. Cote, S.L. Pullman (New York, New York,
USA)
Objective: To evaluate baseline characteristics and improvement in
Parkinson disease (PD) patients after sub-thalamic nucleus (STN) deep
brain stimulation (DBS) using computerized analysis of freely drawn
spirals.
Background: Spiral analysis assesses upper limb kinematics via
calculations derived from handwritten spirals captured on a digitizing
tablet. Deviations from normal motor control are quantied based on
precise measurements of tremor, shape and speed irregularities, including spiral equivalents of micrographia, bradykinesia and deceleration.
Degree of severity (DOS) provides an overall evaluation proven equivalent to expert clinician ratings. Second order smoothness (2-OS) and
rst order zero crossing (1-OC) are important measures of spiral spatial
consistency. Other indices also quantify tremors, loop tightness (micrographia), speed and acceleration patterns.
Methods: 25 PD patients were evaluated pre (off meds) and sixmonths post-STN DBS (off meds/stimulator on) using spiral analysis
(10 spirals per hand) and the motor UPDRS. Spiral and clinical asso-

S37

ciations were determined by Spearman Rank Order correlations, and

pre-post results were analyzed using the Wilcoxon Signed Rank Test.
Results: 14 men and 11 women (age 61.17.6 years, disease duration 46.67.3 years, motor UPDRS 37.512, 20 right handed) underwent 20 bilateral and 5 unilateral DBS-STN procedures. Overall, spiral
DOS correlated with UPDRS rest tremor score (0.306, p0.05),
spiral tremor power correlated with UPDRS action tremor (0.508,
p0.01), and 1-OC correlated with total UPDRS (0.586, p0.01).
Post DBS, the dominant hand improved signicantly in spatial motor
control as reected by decreases of 30% in DOS, 66% in 2-OS, and
22% in 1-OC (p0.03). The number of trials with tremor decreased by
57% and tremor power decreased by 94% (p0.02). Speed, acceleration and micrographia notably showed improvement trends that were
not statistically signicant.
Conclusions: Computerized spiral analysis provides precise and sensitive measurements of ne motor control in PD that correlate with
motor UPDRS scores and objectively quantify improvement following
STN-DBS. Notably, we found greater benet in spatial consistency and
tremor than in speed regulation.
122
Putative central effects of botulinum toxin, possibly mediated by
changes in Renshaw cell activity, following intramuscular injection
in humans
R. Mazzocchio, R. Spidalieri, F. Dominici, T. Popa, M. Hallett,
A. Rossi (Siena, Italy)
Objective: To seek a direct central effect after botulinum toxin
(BoTN) injection in the soleus muscle of spastic subjects.
Background: Evidence for direct central effects of BoNT is lacking
in humans. However, there are indications that BoNT may indirectly
alter central sensorimotor integration through a peripheral mechanism.
Methods: Eight patients (6 with spastic hemiparesis, and 2 with
spastic paraparesis) received BoNT A in the soleus muscle (unilaterally
and in one spot only) for the rst time. A slightly smaller dosage than
standard was applied to avoid a drastic reduction of maximum M-wave.
Patients were evaluated before treatment (t0), and at 4 days (t1), 2
weeks (t2) and 1 month (t3) after the injection. Changes in spasticity
were assessed using the Ashworth scale. Stimulus-response curves to
tibial nerve stimulation were obtained in the soleus muscle and H-reex
and motor (M) response recruitment curves built. Recurrent Renshaw
inhibition of alpha-motoneurons was assessed using the paired H-reex
technique.
Results: Clinical improvement was evident at t2. The Hmax, Mmax
and the H/M ratio were not signicantly changed throughout the
evaluation period, while signicant modications of H-wave slope
were observed at t1 and t2, in the absence of changes in M-wave slope.
At t3, H- and M-slopes were comparable to t0, while a signicant
reduction in motoneurone recurrent inhibition, as indicated by the
increase in the size of the H reex, was evident.
Conclusions: These ndings may be explained by impaired cholinergic transmission at recurrent motor axon terminals, causing decreased
recurrent inhibition. This might be due to retrograde axonal transport of
BoNT. Following neuromuscular block; however, a change in motoneurone discharge properties might also be responsible.
123
The tonic stretch reex studied in Parkinsonism over a wide range
of stretch frequencies and contraction levels
V. Stanislaus, J.A. Burne (Sydney, NSW, Australia)

FIG. 1 (121).

Objective: To study the tonic stretch reex (TSR) in Parkinsons

disease (PD) over a wide range of stretch frequencies and contraction
levels.
Background: Clinical testing of the stretch reex uses brief stretch
perturbations such as the tendon reex. Such testing has failed to
disclose a signicant reex abnormality in PD. We aimed to test the
TSR response to a wide band of dened stretch frequencies. This
should provide a more complete analysis of the reex response and
permit it to be related to the Movement Disorder in PD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: Nine patients and 17 controls participated. The index

nger was perturbed sinusoidally at frequencies 2.5-40 Hz, while
subjects maintained 0, 10, 20, 30, 40, 50% of their maximal contraction
level. Joint angle data and reex EMG from the rst dorsal interreoseus muscle (FDI) were collected. Using cross correlation analysis,
TSR coherence (measure of correlation between stretch and reex),
TSR gain (reex amplitude/stretch amplitude) and TSR phase difference (timing delay between stretch and reex) were calculated.
Results: In normal subjects, coherence was high across the full band
of frequencies, provided that a small background contraction was
present. In PD, coherence was high at low frequencies but decreased
dramatically at all frequencies above 10 Hz. Reex gain showed a
bandpass character in normals and PD, with peaks at 10Hz and 40Hz
and a pronounced minima at 25Hz. This pattern was present at all levels
of contraction. In PD, reex gain was reduced by almost 50% across
the full band. Reex delay, calculated from the TSR phase data, was
not inuenced by the contraction level or frequency and found to be
638 ms for normals and 857 ms for PD.
Conclusions: In contrast to many previous reports, a pronounced
reex abnormality was found in PD. Since the reex delay is not altered
signicantly, we suggest that the reex loop remains intact in patients
but its response over a wide band of frequencies and contraction levels
is disturbed by failure to modulate spindle activity and/or central gain
appropriately to the task. This failure is likely to contribute to the
movement disorder in PD and to the differences seen in rest and action
conditions. Further analyses aims to determine the behavior of the
reex, and also joint stiffness, during more complex tracking tasks.
124
Abnormal excitability of inhibitory mechanisms at central nervous
system level in idiopathic primary vaginismus and vulvar vestibulitis syndrome
E. Frasson, A. Graziottin, G. Didone`, E. Garbin, S. Vicentini,
E. DallOra, L. Bertolasi (Cittadella, Padua, Italy)
Objective: To nd out whether the nervous system excitability is
altered in vaginismic women, we studied pelvic oor electromiographic
activity, the bulbocavernosus reex, and the recovery functions of the
pudendal somatosensory evoked potentials (SEPs).
Background: The diagnosis of vaginismus requires the presence of
involuntary pelvic oor muscle contraction, however few neurophysiological studies have been conducted in this disorder.
Methods: 10 patients with primary idiopathic vaginismus, 10 with
vulvar vestibulitis syndrome (VVS) and vaginismus, and 10 normal
subjects. EMG of the levator ani (LA) was recorded at rest, during
voluntary contractions and straining.Two responses of the bulbocavernosus reex were analyzed, the early response (R1) and late response
(R2). The recovery functions of lumbar spinal, cortical N37-P40 and
P40-N50 components of the pudendal SEPs were assessed by means of
the paired stimulus technique (conditioning stimulus: S1; test stimulus:
S2) at the intersimulus intervals (ISIs) of 5, 20 and 40 ms. SEPs of the
S2 were extracted by the subtraction of SEPs of the S1 only response
to paired stimuli (S1S2) and their amplitudes were compared with
those of the control response (S1) at each ISI. Throughout the somatosensory system this ratio (S2/S1)*100 analyses the functionality of the
inhibitory circuits.
Results: EMG showed basal hyperactivity of the LA and reduced
inhibition at straining in the patients. No signicant differences were
found in R1 and in R2 latency between patients and subjects, but
polysynaptic R2 was signicantly higher in amplitude (p0.01) and
duration (p0.01) in the patients. SEP amplitudes for single stimulus
were similar in patients and normal subjects. The (S2/S1)*100 ratio
was signicantly higher in patients, both with primary vaginismus and
VVS, at the ISI of 20 (p 0.05) and 40 (p0.05) ms for spinal lumbar
potential and at the ISIs 20 (p0.05) and 40 (p0.01) ms for cortical
P40 and N50. No difference was found at the ISI 5 ms.
Conclusions: Our ndings suggest that in primary idiopathic vaginismus and VVS there is an altered excitability of the spinal and cortical
inhibitory circuits that might give rise to an abnormal functioning of the
pelvic oor muscles.

Movement Disorders, Vol. 22, Suppl. 16, 2007

125
The syndrome of dystonia and cerebellar ataxia: Cortical excitability and pathophysiological implications
P. Talelli, B.P.C. van de Warrenburg, S.A. Schneider, P. Giunti,
N.P. Quinn, N.W. Wood, J.C. Rothwell, K.P. Bhatia (London, United
Kingdom)
Objective: To investigate the cortical excitability properties in patients with the clinical syndrome of dystonia and cerebellar ataxia using
Transcranial Magnetic Stimulation (TMS).
Background: Loss of inhibition in the motor cortex is well documented in primary dystonia. The meaning of this nding is, however,
uncertain. Cerebellar disease does not change cortical inhibition. We
have studied the cortical excitability in a small number of patients with
an unusual combination of focal/segmental dystonia with cerebellar
ataxia. Lack of a clearly dystonic electrophysiological prole would
suggest that other mechanisms are responsible for the dystonia in these
patients. This might have important implications for our understanding
of dystonia in general.
Methods: TMS was given over the hand motor area in 10 healthy
controls and 5 patients with a slowly progressive syndrome of cerebellar ataxia and dystonia (mainly cervical). TMS measures included:
motor threshold (MT); intracortical inhibition (SICI) and facilitation
(ICF); SICI recruitment curve, plotting the amount of SICI against the
conditioning intensity (70-100%AMT); cortical silent period (CSP).
Results: Motor thresholds, CSP, SICI and ICF were not different
from controls. Moreover, compared to controls, in whom SICI increases at intensities 80%, patients showed already strong inhibition
at 70% AMT.
Conclusions: In this group of patients with the rare syndrome of
dystonia and cerebellar ataxia, cortical inhibition was normal and the
overall pattern of excitability clearly differed from the dystonic one.
This could suggest that the loss of inhibition, proposed as part of the
pathophysiology in primary dystonia, might not explain all cases of
dystonia. Further research is needed to study the role of other structures, like the cerebellothalamic projections, midbrain structures, and
the spinal cord.
126
Voluntary and reex blinking in Parkinsons disease
R. Agostino, B. Gregori, L. Dinapoli, M. Bologna, D. Belvisi,
G. Fabbrini, A. Berardelli (Roma, Italy)
Objective: To study in Parkinsons (PD) patients ON and OFF
therapy eyelid kinematics during voluntary and reex blinking.
Background: It is still unclear whether in PD patients bradykinesia is also present during the execution of fast voluntary eyelid
movements.
Methods: We studied 14 PD patients and 8 age-matched control
subjects. Patients were clinically scored by means of UPDRS (section 3). Eyelid movements were recorded by means of the motion
analyzer system SMART that use three infrared cameras able to
follow the movement of a passive reective marker taped on the
right upper eyelid. Subjects were comfortably seated on a chair with
the head xed on a head holder. Before voluntary and reex blinking the subjects were asked to look straight ahead. For the voluntary
blinking the subjects were then asked to blink as fast as possible.
Reex blinking was obtained by suprathreshold electrical stimulation of the ipsilateral supraorbital nerve. Kinematics of the closing
and opening phases of blinking (duration, amplitude, peak velocity
of both phases and pause between closing and opening) was determined off-line with a dedicated software. For each subject (patients
and controls) and conditions (OFF and ON treatment) ten voluntary
and reex blinking movements were collected. ANOVA was used
for statistical analysis.
Results: Voluntary blinking: In patients and controls the closing
phase of eyelid movements was shorter than that of the opening
phase and the peak velocity of the closing phase was higher than
that of the opening phase. The duration, amplitude and peak velocity
of the closing and opening phase was similar in patients and

POSTER SESSION I, TUESDAY, JUNE 5, 2007

controls. The pause between closing and opening phase was significant longer in the patients than in controls. Although not signicantly the dopaminergic treatment tended to reduce the pause duration. Reex blinking: In both groups all the kinematic variables
were similar.
Conclusions: Slowness of blinking is due only to a lengthening of
the pause between the closing and the opening phases, suggesting
that the switching process between submovements performed in
sequence is abnormal. This abnormality is more evident in the
more affected patients. Dopaminergic treatment seems to improve
these abnormalities.

DYSTONIA
127
Blepharospasm associated with Sjogrens syndrome
J.-S. Liu, M.-Y. Lan, C.-S. Su, S.-L. Lai, H.-S. Wu, Y.-Y. Chang
(Kaohsiung, Taiwan)
Objective: To describe the patients of blepharospasm (BPS) associated with Sjogrens syndrome (SS), a correlation rarely noted previously.
Background: SS is a chronic progressive autoimmune disease characterized by dry mouth and dry eye. Many patients with BPS also have dry
eye symptoms and have reduced Schirmer test (ST) values. Whether dry
eye causes BPS for the increment of blinking rate to compensate for tear
lm deciency or distortion of sensory modalities following SS or related
focal sensory neuropathy needs further evaluation.
Methods: 81 patients with clinical diagnosis of BPS were enrolled
for this study. Eight patients (9.9%; one man and seven women) were
associated with SS according to ACR criteria for SS. All SS patients
had dry eye symptoms and reduced ST results. The patients reported
symptoms of pain, paresthesia or discomfort sensation in face or ocular
area, which preceded the development of BPS by months or years.
Sensory neuropathy and trigeminal neuropathy were documented in
three patients with SS. Anti-SSA or SSB antibodies were detected in
seven patients, and hepatitis B or hepatitis C related cryoglobulins were
found in three patients. Brain MRI showed multi-focal subcortical
white matter lesions in three patients. All patients were treated with
local botulinum toxin (BOTOX; Allergan, USA) injection and received
serial ST and questionnaire before, 1 month, and 3 months after
BOTOX injections.
Results: Before BOTOX therapy, all patients presented with
abnormal ST results without local anaesthesia (5 mm). BOTOX
treatment relieved blepharospasm in all patients with mean duration
about three months. According to the questionnaire, six patients
reported an increase of dry eye symptoms. Otherwise, one patient
reported a decrease of dry eye symptoms. Moreover, ST results were
signicantly reduced (p0.05) 1 month, and 3 months after BOTOX
treatment.
Conclusions: SS, either primary or secondary, may be accompanied
by central nervous system complications including BPS. The local
sensory irritation in SS status may induce plasticity changes in the
sensory cortex leading to the subsequent motor symptoms. Deterioration or dry eye symptoms is the most common side effect of botulinum
toxin therapy for BPS associated with SS. Furthermore, other treatment
modalities such as immuomodulatory therapy might be also mandatory
in this condition.
128
Impaired disinhibition of the motor cortex during development of
LTP-like plasticity in dystonia
S. Meunier, H. Russmann, M. Hallett (Paris, France)
Objective: To know if enhanced plasticity in dystonia is related to an
excess of disinhibition of the motor cortex.
Background: Paired associative stimulation (PAS) may represent
associative long term potentiation/depression (LTP/LTD)-like plasticity at a cell population level. PAS consists of pairing repetitively a
peripheral and a cortical TMS stimulation. Effects of PAS intervention

S39

are enhanced in dystonic patients suggesting an enhanced abnormal

plasticity. In dystonia long interval intracortical inhibition (LICI)
thought to reect activity of GABAB interneurons and afferent inhibition (AI) thought to reect activity of inhibitory circuits fed by peripheral sensory inputs are decreased. We hypothesize that, when a disinhibition is needed (during induction of LTP), the decreased levels of
LICI and AI may lead to an excess of disinhibition and to an enhanced
plasticity.
Methods: We recorded MEPs from the exor pollicis brevis muscles
in 18 healthy volunteers (HV) and 13 patients: seven writers cramp
and six musicians cramp. LICI and LAI were measured before, immediately and 45 minutes after a PAS intervention. LICI was tested by
pairing two suprathreshold TMS pulses (ISI 90 ms). Long-afferent
inhibition (LAI150) was evoked by stimulating the median nerve at the
wrist 150 ms before the TMS. PAS consisted to repeat at 0.2 Hz, 240
paired stimulations: a median nerve stimulation (2.5 x PT) followed 25
ms after by a TMS shock (targeting the FPB).
Results: Baseline level of LICI was not different in HV and patients
(HV: 44 % 5, P: 52 % -4). After PAS LICI decreased in HV (T45:
LICI: 76 % 10) but not in patients (T45: LICI: 57 % 9). Baseline
level of LAI150 was signicantly less in patients than in HV (HV: 54 %
5, P: 92 % 10). After PAS LAI150 was decreased in HV (T45 LAI:
76 % 6) contrasting with restoration of an inhibition in patients (at
T45 LAI: 41 % 8).
Conclusions: In healthy subjects, a PAS intervention aiming to
increase excitability of the M1 cortex induces a disinhibition of the M1
cortex by decreasing LICI and LAI. In patients our hypothesis was not
veried as PAS induced an excess of inhibition of the M1 cortex.
Perhaps enhanced plasticity in dystonia is not restricted to excitatory
synapses but also develops at the level of inhibitory synapses.
129
Diffusion tensor imaging in patients with primary adult onset focal
dystonias
G. Fabbrini, P. Totaro, V. Calistri, C. Colosimo, P. Pantano,
A. Berardelli (Rome, Italy)
Objective: We studied patients with blepharospasm (BFS), cervical
dystonia (CD) and writers cramp (WC) with diffusion tensor imaging
(DTI).
Background: Conventional neuroimaging studies of the brain are
usually normal in BFS, CD and WC. A previous study with DTI
showed abnormalities in patients with CD (Colosimo et al. 2005).
Methods: We studied 18 patients with CD (mean age 55.5 13.4
years), 16 with BFS (mean age 66.5 9.5 years), 9 with WC (mean
age 55.5 14.9 years) and 22 healthy controls (mean age 58.3 17.5
years). All patients were studied at the time of maximum therapeutic
response to botulinum toxin (2 to 4 weeks after last injection). MRI was
carried out using a 1,5 T scanner (Gyroscan NT 15, Philips Medical
Systems, Best, Netherlands). DTI parameters were TR 3694 ms,
TE 124 ms, matrix 128 128, FOV 250 250 mm, and two
b-values (0 and 1000 mm s-2) done in six directions. Data was processed to obtain maps of fractional anisotropy (FA) and mean diffusivity (MD). ROIs included corpus callosum, posterior limb of the
internal capsule, caudate head, putamen, globus pallidum, thalamus,
substantia nigra, supplementary motor area (SMA), prefrontal, lateral
premotor and primary motor cortices.
Results: Statistical analysis (ANOVA) of the results showed that FA
values differed between groups in body of corpus callosum (F 3.90;
p 0.01), left internal capsule (F 2.72; p 0.05), right thalamus
(F 2.907; p 0.04), left putamen (F 6.259; p 0.001) and right
putamen (F 4.001; p 0.01). DWI values differed between groups
in the following areas: right caudate (F 3.328; p 0.02), right (F
3.255; p 0.06) and left (F 3.278; p 0.02) prefrontal cortex.
Post-hoc multiple comparison Dunnets test showed that patients affected by CD differed from the healthy control subjects in body of the
corpus callosum (p 0.008), left putamen (p 0.001), right putamen
(p 0.005), right caudate (p 0.04) and in left prefrontal cortical area
(p 0.01). No signicant regional differences were found between
BFS and WC and healthy controls.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Conclusions: This study demonstrates that CD is associated with

structural abnormalities in the white matter of several brain areas,
including the prefrontal cortical, sensorimotor and basal ganglia. No
abnormalities were found in BFS and WC patients.
130
Clinical outcome predictors of pallidal stimulation in patients with
primary dystonia
I.U. Isaias, R.L. Alterman, J. Miravite, D. Weisz, J.L. Shils,
M. Tagliati (Monza, Italy)
Objective: To evaluate clinical outcome predictors of deep brain
stimulation (DBS) of the globus pallidus internus (GPi) in 31 consecutive patients with primary dystonia.
Background: GPi DBS is currently the most effective treatment for
medically refractory dystonia. However, the selection criteria are not
well dened. The cost and potential risks of this procedure warrant a
better determination of those patients that will likely have best postoperative outcomes.
Methods: We reviewed the clinical records of 31 patients with
medically refractory primary dystonia (20 DYT1 positive) who underwent stereotactic GPi DBS implants. Twenty-nine were implantedbilaterally (17 males) and two unilaterally (1 male). Three patients had
multiple thalamotomies before DBS. The Burke Marsden Fahn Dystonia Rating Scale (BMFDRS) scores at baseline and 12 months after
DBS were used to evaluate clinical outcome. Several possible outcome
predictors, including demographic (age, gender) and clinical factors
(duration and severity of disease, anatomical site of involvement,
previous ablative surgery) were correlated with BFMDRS score
changes using Pearson correlation coefcients.
Results: GPi DBS was successful in every patient, with an average
improvement of 69.4% at 12 months. Previous thalamotomy and involvement of speech and swallow were the only factors showing a
signicant negative correlation with clinical outcome at 1 year (Pearson
correlation; p0.01 and p0.05 respectively). Disease duration also
showed a negative correlation trend (p0.06). Even excluding the 3
patients with multiple thalamotomies, involvement of speech and swallow was still a negative predictive factor (p0.05) and disease duration
showed a negative predictive factor trend (p0.06). None of the other
analyzed factors showed any correlation with clinical outcome.
Conclusions: A history of multiple thalamotomies is a negative
outcome predictor for GPi DBS in patients with dystonia. Similarly,
patients with involvement of speech and swallowing muscles and
longer duration of disease may have worse outcomes. Age at onset and
at surgery, gender, DYT1 status, dystonia severity and involvement of
axial and limb muscles do not predict DBS outcome.
131
Muscle hypertrophy in cervical dystonia: A magnetic resonance
imaging (MRI) based analysis
R. Cakmur, S. Men, E. Karakas, E. Yaka, F. Uzunel (Izmir, Turkey)
Objective: This study was conducted to investigate MRI-measured
muscle sizes in patients with cervical dystonia (CD) and compare these
with normal values. We hypothesized that signicant side-to-side differences in muscle measurements would be found in CD. We also
hypothesized that the muscle sizes in CD would be larger than the
normal subjects.
Background: Hypertrophy of dystonic muscles is accepted as one of
the classical features of CD. In clinical practice, muscle hypertrophy is
used as a clinical indicator for identication of dystonic muscles to
inject botulinum toxin. Surprisingly, no information is available on
objective measurements of muscle sizes in CD. MRI provides the
ability to perform noninvasive studies of muscle morphology in live
subjects.
Methods: Axial gradient echo T2-weighted MRI images were obtained through sella and thoracic 4th vertebra in 16 patients with CD
and in 21 age and sex matched controls. Subsequently, cross-sectional
area (CSA) of muscles was measured at predetermined level for each of
the following six muscle pairs: sternocleidomastoid, splenius capitis,

Movement Disorders, Vol. 22, Suppl. 16, 2007

semispinalis capitis, postero-medial scalene, anterior scalene and levator scapulae.

Results: When compared to normals, signicant differences in sideto-side muscle measurements were observed in all cervical muscles of
CD. Cervical muscle volumes were also larger in patients with cervical
dystonia than normal controls. In 14 (88%) patients with CD, there was
signicant side-to-side difference of muscle size in at least one of the
cervical muscles. In 5 (31%) patients with CD, there was signicant
absolute hypertrophy in at least one of the cervical muscles.
Conclusions: Cervical muscle hypertrophy is common nding in
cervical dystonia and this may suggest a possible cause-effect relationship between muscle activation and hypertrophy. More studies are
needed to determine whether muscle hypertrophy can be used as a
clinical indicator for identication of dystonic muscles to inject botulinum toxin.
132
Exercise as an environmental trigger for focal dystonia
E.L. Peckham, P.T. Lin, E.A. Shamim, M. Hallett (Bethesda,
Maryland, USA)
Objective: To present three cases where exercise contributed to the
development of dystonia.
Background: Although the etiology of focal dystonia remains unknown, specic environmental inuences are thought to play a role in
their development. Dystonia has been described in patients with various
occupational triggers: professional musicians developing hand or embouchure dystonia, excessive mantra recitation resulting in lower lip
dystonia1, and in runners as a lower limb dystonia2.
Methods: Three patients were evaluated by history and physical
exam. Videotapes were performed prior to treatment in two patients
and after treatment in the third patient.
Results: Patient #1: 49-year-old right-handed female who developed posturing of her right foot (inversion and plantar exion) while
using the elliptical machine and walking. Her exercise routine
consisted of 6.5 miles per day on the elliptical machine or power
walking for the past 6 years. For 16 years prior to this, she exercised
daily for at least one hour per day and trained for a marathon. She
had no sensory trick and symptoms improved with the use of
botulinum toxin. Patient #2: 48-year-old right-handed male with the
onset of abdominal muscular pulling and tightness manifest on exam
as camptocormia that developed over a one month period with
walking or running. Since college, he ran 25 miles and performed
1200 sit-ups per week. At times, he would do 500 sit-ups in one day.
He had a sensory trick and symptoms improved with the use of
lorazepam, weekly massage and an inversion table. Botulinum toxin
was ineffective. Patient #3: 57-year-old right-handed male with the
onset of an adduction of his left thigh while running approximately
fty yards. He was a life-long runner and usually ran about 7 miles
per day. The symptoms progressed to involve walking as well. He
had no sensory trick and his symptoms have not improved with one
dose of botulinum toxin.
Conclusions: Extreme exercise apparently can contribute to the
development of focal dystonia.
References:
1. Bonanni L, Thomas A, Scorrano V, Onofrj M. Task specic
lower lip dystonia due to mantra recitation. Mov Disord 2006 Dec
5 (Epub ahead of print)
2. Wu LJ, Jankovic J. Runners dystonia. J Neurol Sci 2006 Dec 21;
251 (1-2): 73-6.
133
Is uoxetine innocent as thought
S. Bilen, F. Ak (Ankara, Turkey)
Objective: The aim of the presentation is to emphasize that dystonia
due to uoxetine use can be permenant and can need botulinum toxin
type A treatment.
Background: Selective serotonin reuptake inhibitors (SSRI) are prescribed frequently because of having less adverse effects than tricyclic

POSTER SESSION I, TUESDAY, JUNE 5, 2007

antidepressants and MAO inhibitors. Since they are becoming popular
their adverse effects call more attention. Thus, movement disorders
such as dystonia, dyskinesia and parkinsonism related to SSRI group
drug use have been reported. Fluoxetine is a commonly used antidepressant drug of SSRI group. Parkinsonism, impairment in Parkinsons
disease, acute dystonia, tardive dyskinesia, tremor, myoclonus and
complex movement disorders related to uoxetine use also have been
reported.
Methods: Here we report a case of permanent dystonia thought to be
associated with short term uoxetine use and needed to be treated with
botulinum toxin type A.
Conclusions: Fluoxetine can cause secondary permanent dystonia.
Botulinum toxin type A can be a treatment choice.
134
Sensorimotor organisation of the hand area is differently modulated by proprioceptive training in musicians dystonia and writers cramp
K. Rosenkranz, K. Butler, A. Williamon, C. Cordivari, A.J. Lees,
J.C. Rothwell (London, United Kingdom)
Objective: To test whether behavioural proprioceptive training reverses abnormalities of sensorimotor integration (SMO) in focal hand
dystonia.
Background: Abnormalities of SMO in focal hand dystonia are
thought to provoke the development of dystonic symptoms. Previously
we devised a method to measure SMO using short-term vibration
applied to the abductor pollicis brevis (APB) or rst dorsal interosseus
(FDI) muscles. Transcranial magnetic stimulation of the motorcortical
hand area during vibration enhances the excitability of projections to
the vibrated muscle, while reducing it in projections to the non-vibrated
ones. We showed that SMO was modulated by long-term musical
practice in musicians and that whereas musicians dystonia patients had
a more abnormal pattern than healthy musicians, writers cramp patients failed to show any modulation of motor excitability by vibratory
input.
Methods: In the present study on 6 non-musicians, 6 musicians, 6
musicians dystonia and 6 writers cramp patients, we tested the
effect of 15-min period of behavioural proprioceptive training on
SMO.
Results: Behavioural training where healthy non-musicians attended
to the proprioceptive input from APB (AttVIB) led to an expansion of
the effect of subsequent short-term APB vibration whereas attention to
cutaneous inputs applied to the index nger (AttIndex) attenuated this
effect. Interestingly, the effect of training differed in each group, such
that AttVIB instead of enlarging, now focussed the SMO in musicians
and in musicians dystonia. In writers cramp, the same training restored a small inuence of proprioceptive input on motor output, but
did not normalise it. AttIndex training attenuated the effect of APB
vibration in healthy non-musicians and musicians but failed to do so in
musicians dystonia and writers cramp. Finally, during the behavioural
training there were differences in the sensory discrimination between
writers cramp and musicians dystonia.
Conclusions: It may be possible to retrain the SMO of patients
with focal hand dystonia with attended proprioceptive input. The
different sensory discrimination and neurophysiological effects of
behavioural proprioceptive training in writers cramp and musicians dystonia suggest a different pathophysiology.
135
Efciency of botulinum toxin in treatment of writers cramp:
Long-term follow-up results
Z. Matur, H. Hanagasi, Y. Parman (Istanbul, Turkey)
Objective: To evaluate the effectiveness of botulinum toxin (BT) in
the our patients with writers cramp (WC) who were followed longterm.
Background: Writers cramp is the most common form of taskspecic dystonia. Botulinum toxin has been used to treat limb dystonias

S41

for over fteen years. This study describes fteen patients with writers
cramp treated with BT injections.
Methods: Fifteen patients (13 men, 2 women) with WC who were
resistant to oral medication are treated with BT injections and were
folllowed during a long period. All patients were evaluated in
efciency and adverse effect at four and/or twelve weeks after every
injection. The efciency graded range of 0 to 4 point.
Results: The patients aged between 28 to 69 years (average
4410 years). The mean follow-up time was 2.51.5 years (range
18 year). The median age at onset was 419 (range 25-59) years.
The mean time between onset of symptom to BT treatment was 56
(range1-24) years. Ten patients had abnormal contractions in the
forearm and hand exor muscles, two in the extensors and three in
both exors and extensors. Botulinum toxin was administered under
EMG guidance. The doses ranged between 25 to 150 units of
BOTOX or 110 to 400 units of DYSPORT at each visit. Total
number of injections was 83. Eight patients reported signicant
improvement with BT. Six had moderate or slight amelioration, and
one did not have any improvement. No prominent side effects were
observed except mild to moderate weakness. The benecial effect of
a single injection lasted from 2 to 9 months.
Conclusions: We conclude that BT injections may provide safe and
effective treatment in patients with writers cramp also during a long
period.
136
Pediatric writers cramp in myoclonus-dystonia. Maternal imprinting hides positive family history
M.C.F. Gerrits, E.M.J. Foncke, J.H.T.M. Koelman, M.A.J. Tijssen
(Amsterdam, Netherlands)
Objective: Pediatric writers cramp in myoclonus-dystonia.
Background: Myoclonus-dystonia (M-D) is an autosomal dominantly inherited Movement Disorder with myoclonic jerks and dystonic contractions, which are frequently alcohol-responsive. Onset
of myoclonus is usually in the rst or second decade of life. Mild
dystonic features are usually observed. In approximately half of
affected individuals (54%) focal or segmental dystonia is found,
presenting as cervical dystonia and/or writers cramp. M-D is most
frequently caused by mutations in the e-sarcoglycan (SGCE) gene
on chromosome 7q21-q31 (DYT11). Penetrance of SGCE mutations
is reduced in mutation carriers who inherit the mutation from their
mother due to maternal imprinting.
Methods: We present two unrelated children with M-D who presented with writers cramp.
Results: Both children were recognized as M-D patients only after
extensive study of their family which was initiated after identication of an index patient with M-D in the third line of inheritance.
Conclusions: In children with writers cramp screening of the
SGCE gene should be considered, even with a negative family
history.
137
Long-term motor learning in focal hand dystonia (FHD)
E.A. Shamim, S.Y. Kang, M. Hallett (Bethesda, Maryland, USA)
Objective: To demonstrate abnormality in long-term motor learning
over 3 months in FHD patients compared to age-matched healthy
volunteers by showing 1) that FHD patients have slower motor skill
acquisition, 2) that FHD patients have difculty in performing newlyacquired motor skills automatically, and 3) that there are differences in
brain dynamics (cortico-cortical connectivity and motor cortex excitability).
Background: Sequential motor learning has not been well studied in
FHD patients. Repetitive practicing of ne motor skills, a known
environmental trigger for FHD, may lead to aberrant motor learning
resulting in dystonia. FHD rarely occurs during the acquisition phase of
learning a ne motor skill; rather it occurs while these learned movements are being ne-tuned. In healthy volunteers, the acquisition of ne
motor skills occurs rapidly and is associated with cortical modications

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

as noted by transcranial magnetic stimulation (TMS) and electroencephalography (EEG). A dual task paradigm can be used to show
learning by measuring automaticity of sequential nger movements
while performing another unrelated task.
Methods: Right-handed FHD patients and age-matched healthy volunteers were followed for 12 weeks as they learned a sequence of eight
key presses using 4 ngers of their left hand. The task was practiced
daily for 30 minutes for the rst week and then daily for 15 minutes. All
subjects were followed daily for the rst 10 days. Sequence error rates
(SER), training logs, and clinical evaluations were performed at about
2 week intervals. TMS and EEG studies were performed at the beginning, middle and end of the study.
Results: We present preliminary data from 2 FHD patients and 2
healthy volunteers. The patients are both 58-year-old men, and the
healthy volunteers are women aged 50 and 54 years. Both groups
quickly mastered the task. FHD patients never achieved the same
prociency as the healthy volunteers as measured by the SER. The dual
task paradigm provoked more errors in the FHD patients. An apparent
physiological difference was a greater decrease in the resting motor
threshold over time in FHD patients.
Conclusions: These preliminary results indicate an abnormality in
long-term motor learning in FHD patients. There may be some physiological correlates of this abnormality.

138
A patient with Meige-like psychogenic Movement Disorder
S. Turan, D. Uluduz, S. Ozekmekci (Istanbul, Turkey)
Objective: To present an unusual case resembling to Meiges syndrome, whose diagnosis was compatible with psychogenic movement
disorder (PMD).
Background: Among PMD, tremor and dystonia were the most
frequent abnormal movements. Up to date, Meiges syndrome in psychogenic origin has not been reported. Meiges syndrome is a form of
cranial dystonia characterized by spasms of orbicularis oculi and oromandibular muscles.
Methods: A 48-year-old man suddenly developed blepharospasm
and oromandibular dyskinesias nine months ago following a stressful
event.
Results: These involuntary movements continued through the whole
day, but they disappeared during sleep. He was on topiramate for two
years and valproate for one month with the diagnosis of epilepsy. His
family history was negative. He was uneducated and jobless for four
years. Biochemical investigations, MRI of the brain, EEG and polysomnography were normal. Screening for Wilsons disease and acanthocytosis were negative. EMG did not show any dystonic pattern in
affected muscles. Frequency and directions of the involuntary movements were changed or completely suppressed with distraction and
suggestion. He exhibited uneconomic postures and give-way weakness while nger-tapping. One of his seizure-like attacks we observed
was typically compatible with a conversion disorder. Concordantly, his
psychological tests revealed that he had conversion disorder. He did not
have any seizure even after stopping valproate. He had secondary gain.
Conclusions: This was the rst case presented with Meige-like PMD.
He had an abrupt onset PMD and displayed distractibility, uneconomic
postures and ndings of conversion disorder. Based on clinical features, labotarory investigations and the established diagnostic criteria
for PMD, we have thought his movement disorder was psychogenic in
origin. Since PMD may be misdiagnosed as neurogenic movement
disorders phenomenologically, patients with Movement Disorders
should also be screened for psychologically, if necessary.

Movement Disorders, Vol. 22, Suppl. 16, 2007

139
Inuence of coffee drinking and cigarette smoking on the risk of
primary late-onset blepharospasm: Evidence from a multicentre
case-control study
A. Berardelli, G. Abbruzzese, P. Girlanda, D. Martino, M. Tinazzi,
G. Defazio (Rome, Italy)
Objective: To investigate prior coffee and smoking habits in patients
presenting with primary late-onset blepharospasm (BSP).
Background: An exploratory case-control study assessing a large
number of variables found a protective association between late-onset
dystonia and cigarette smoking. Potentially confounding variables including coffee drinking are as yet unexamined. Another open question
is whether coffee independently modies the risk of primary late-onset
dystonia.
Methods: Our study included 166 patients primary BSP, 228 hospital
control patients with primary hemifacial spasm, and 187 population
control subjects from ve Italian centres. Information on age of disease
onset, smoking and coffee drinking status at the reference age, average
number of packs or cups of coffee drunk/cigarettes smoked per day
reached high and similar test-retest reproducibility in case and control
patients.
Results: Unadjusted logistic regression analysis yielded a signicant
inverse association of prior coffee drinking and cigarette smoking with
case status considering either control group. After adjustment for age,
sex, referral centre, disease duration, years of schooling and coffee
drinking/cigarette smoking as appropriate, smoking estimate lacked
signicance, whereas the association of coffee intake and BSP did not
(case patients vs. hospital control patients: Odds Ratio0.37, 95%
condence interval 0.20 to 0.67; case patients vs. population control
subjects: Odds Ratio0.44, 95% condence interval 0.23 to 0.85). The
strength of the inverse association between BSP and coffee intake
tended to increase with the average number of cups drunk per day.
There was a signicant correlation between age of BSP onset and
number of cups per day (adjusted regression coefcient 1.73; p
0.001), whereas no correlation was found with number of packs of
cigarettes per day.
Conclusions: Coffee drinking may be inversely associated with the
development of primary BSP and this association may partly depend
upon the amount consumed.

140
Focal limb dystonia with ipsilateral cerebellar hemiatrophy
J.S. Baik, J.H. Park, J.Y. Kim, S.W. Han, J.H. Kim (Seoul, Korea)
Objective: Case
Background: Hemiatrophy of a cerebellar hemisphere subsequently
is developed by contralateral cerebral vascular accidents, hemorrhages,
cerebral tumor or arteriovenous malformation. But cerebellar hemiatrophy without cerebral hemispheric abnormalities is extremely rare.
Dystonia is usually thought to be a basal ganglia disorder, but recent
data suggest that the cerebellum may also play a role.
Methods: Case
Results: A 33-year-old right handed woman experienced a four-year
history of slowness and a two-year history of writing difculty of right
upper extremity. There was no history of birth injury, infection and
head trauma. When she was asked to keep her arms outstretched,
dystonic posture of the right arm was developed. She showed mild
dysmetric movement and external rotation of the right hand on ngerto-nose test. But there was normal on cerebellar function test. Brain
MRI revealed marked isolated atrophy in right cerebellum.
Conclusions: We present an unusual patient associating focal limb
dystonia and ipsilateral cerebellar hemiatrophy.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

141
Sepiapterin reductase deciency masquerading as hypotonic cerebral palsy
G.M. Wali, B. Thony, N. Blau (Belgaum, Karnataka State, India)
Objective: 1.To report two siblings of Indian origin with Sepiapterin
reductase deciency who had global hypotonia with diurnal variation
as the dominant feature and responded dramatically to L-dopa/carbidopa therapy. 2.To justify a trial of L-dopa/ carbidopa therapy in
patients who are suspected to have hypotonic form of cerebral palsy(CP).
Background: SR deciency is a potentially treatable autosomal recessive disorder of BH4 metabolism without hyperphenylalaninemia.
Dominant clinical features include mental retardation,dystonia showing
diurnal variation, truncal hypotonia and limb spasticity. The motor
symptoms show dramatic response to L-dopa/carbidopa therapy. It may
remain underdiagnosed due to lack of neonatal screening tests.
Methods: The clinical, biochemical and genetic proles of these two
patients are summarized.
Results: These siblings were born to consanguineous parents. Patient
1. was a boy aged 10 years that had psychomotor delay, global
hypotonia and hypersomnia as the dominant features. Additional motor
features included dgety movements of the hands and oculogyric
crisis.His limb or trunk dystonia was absent and motor symptoms
showed sleep benet. He was previously diagnosed as a case of
hypotonic CP. Patient 2. was the youngest sister of patient 1, aged 11
months and had mild psychomotor delay along with global hypotonia
and hypersomnia. She had episodes of oculogyric crisis but no dystonia
of the limbs or trunk. Both siblings showed dramatic improvement to
L-dopa/carbidopa therapy(1 mg/Kg/day). CSF analysis revealed very
low neurotransmitter metabolites and slightly elevated biopterin indicative of SR deciency in both the patients(Table). Genomic DNA
analysis revealed the mutation c.413TC or p.V138D which was
found in the homozygous state in the two patients and in the heterozygous state in the parents. It was absent in the healthy sibling. The SR
mutant alleleV138D has not been described previously, and may be
responible for the reduced enzyme activity.
Conclusions: Hypotonic CP is usually an outcome of severe untreatable brain damage.SR deciency may present with global hypotonia as
the dominant feature masquerading as hypotonic CP.Considering the
treatability, it could be prudent to give a trial of L-dopa/carbidopa
therapy to patients who are labelled as hypotonic CP especially when
diurnal variation,hypersomnia and oculogyric crisis co-exist.

Biogenic amines and Pterins in CSF

Patient 1 (10 years)

Patient 2 ( 1year)

Value (nM)

Ref range

Test

Value (nM)

Ref range

6
103
17
36

88-178
144-801
1.5-3.5
10-30

5HIAA
HVA
HVA/5HIAA
Bio

12
189
16
45

114-336
295-932
1.5-3.5
15-40

5HIAA 5-Hydroxyindoleacetic acid, HVA Homovanillic acid,

Bio Biopterin

S43

Methods: We used the associative stimulation protocol (PAS) to

evaluate synaptic plasticity in the sensori-motor cortex in 10 patients
with clinically denite psychogenic dystonia, 10 patients with organic
dystonia and 10 age-matched healthy control subjects. MEP amplitude
at rest was assessed before and after PAS from right abductor pollicis
brevis (APB) muscle and right rst dorsal interosseus (FDI) muscle.
Results: In patients affected by organic dystonia PAS induced a
stronger and less focal increase in MEP amplitude compared to patients
with psychogenic dystonia and healthy controls. PAS after affects were
similar in psychogenic dystonia and healthy controls.
Conclusions: Abnormal plasticity within sensori-motor cortex along
with loss of topographical specicity is a peculiar alteration of organic
dystonia, possibly representing an endophenotypic trait.
143
Autosomal dominant myoclonus dystonia: Unusual phenotype with
prominent hypotonia/motor impersistence and positive celiac serology
V.S.C. Fung, N. Mahant, C.M. Sue, A. Grunewald, C. Klein (Sydney,
NSW, Australia)
Objective: The aim of this study is to report an unusual clinical
phenotype in a patient with myoclonus-dystonia secondary to an epsilon-sarcoglycan gene mutation.
Results: The proband was born 6 weeks premature. He had delayed
motor milestones, but completed 10 years of normal schooling. Involuntary movements were rst noted at age six months and worsened in
his mid-teens. His speech became slurred and he lurched from side to
side when walking. He developed severe generalised spasms of the
trunk lasting seconds-minutes leading to weekly falls and self-injury.
There was a history of involuntary movements and celiac disease
affecting his mother and maternal grandfather. When examined at age
16, he had generalised and multifocal myoclonus, worse on the left that
the right, affecting the upper limbs, trunk and face. There was no
stimulus sensitivity. He uctuated between anterocollis and retrocollis.
He had difculty in maintaining an upright posture, with a tendency for
his trunk to ex due to hypo- rather than hypertonia. There was motor
impersistence of the tongue and eye closure, as well as when attempting
to maintain the arms outstretched. Surface EMG from the upper limbs
showed frequent intermittent bursting of 100-250ms duration with
occasional sustained co-contracting bursts lasting between 5002500ms. Median SEPs were normal with no C reex present. The
patient had positive anti-gliadin antibodies with a negative small bowel
biopsy. MRI brain was normal. A known pathogenic c.304CT mutation of the epsilon-sarcoglycan gene was detected. After treatment
with clonazepam 1.5mg bd, there was marked improvement in his
dystonic spasms with abolition of his falls. He recently has reported
improvement after 1.5 units of alcohol.
Conclusions: Hypotonia and motor impersistence should be considered part of the clinical phenotype of myoclonus-dystonia. Further
studies are planned to determine whether gluten hypersensitivity cosegregrates with the epsilon-sarcoglycan gene mutation and therefore is
potentially contributing to the phenotype in this family, and whether the
patient represents an unusual example of clinical expression of a
maternally inherited gene mutation.
144

142
Associative plasticity in psychogenic dystonia
A. Quartarone, V. Rizzo, C. Terranova, S.A. Schneider, F. Morgante,
P. Girlanda, K.P. Bhatia, J.C. Rothwell (Messina, Italy)
Objective: The aim of the present study was to investigate whether
the abnormal plasticity within sensori-motor cortex of patients affected
by organic dystonia is also present in patients with psychogenic dystonia.
Background: It has been recently demonstrated that psychogenic and
organic dystonia share similar neurophysiological abnormalities possibly related to the maintenance of abnormal postures (Espay et al.,
2006).

Brainstem reexes in essential blepharospasm

G. Benbir, M.E. Kiziltan (Istanbul, Turkey)
Objective: We aimed to investigate the characteristics of brain stem
reexes in primary blepharospasm, including trigeminal, extra-trigeminal (somatosensorial) and auditory blink reex (BR), in addition to
trigeminal and auditory posterior auricular muscle responses (PAMR).
Background: Essential blepharospasm (EB) is a focal dystonia characterized by excessive involuntary closure of the eyelids. Loss of
inhibition of various brainstem reexes has previously been reported in
patients with EB.
Methods: Fifteen consecutive patients with EB and 16 sexmatched controls were recruited in the study. None had secondary

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

causes of dystonia, basal ganglia lesions, or previous drug exposure.

R1/R2 latency, R2 amplitude and duration were calculated. Both
trigeminal and extratrigeminal stimulation from median nerve were
performed in BR. Auditory BR and PAMR were elicited by sudden
onset acoustic stimuli at 105 decibell tone delivered bilaterally
through earphones.
Results: The mean latencies and amplitudes of trigeminal BR were
not different in EB patients and controls.The mean R2 durations of BR
were bilaterally longer in EB patients (p0.01). The mean latencies
and amplitudes of auditory BR were similar. The mean duration of BR
was longer in EB patients on right (p0.05) and left side (p0.007).
Auditory PAMR was not different between groups. The comparison of
gender in EB patient group revealed shorter mean distal R1 (p0.035)
and R2 latencies (p0.003) of trigeminal BR in females EB patients.
The mean duration of somatosensorial BR was not statistically different
between groups.
Conclusions: Our results support the presence of a hyperexcitability in blink reex circuits. Although basal ganglia dysfunction
and dopamin depletion may underlie this condition in primary
blepharospasm patients, the presence of hyperexcitable reex blinks
in the absence of hyperexcitable PAMR reexes may also suggest
some alterations in the trigeminal sensory-motor system. Not only
trigeminal but also auditory blink reex exhibited hyperexcitability.
In other words, reexes with an effector organ of orbicularis oculi
muscle showed increased excitability, while other muscles with
facial nerve innervation did not. Somatosensorial reex, on the other
hand, was not increased in EB patient, which may suggest another
mechanism in this reex circuit.
145
A case of paroxysmal dyskinesia: Atypical or psychogenic?
J.S. Baik, J.H. Park, J.Y. Kim, S.W. Han, J.H. Kim (Seoul, Korea)
Objective: Case
Background: Paroxysmal movement disorders are a group of heterogeneous entities that have been categorized based on their typical
clinical features. The major difculty in the diagnosis of sporadic
paroxysmal non kinesigenic dyskinesia (PNKD) is to differentiate it
from a psychogenic Movement Disorder, particularly in sporadic cases.
Methods: Case
Results: A 43 year-old, right-handed woman experienced a sixmonths history of paroxysmal dystonia on left arm and legs. Dystonic
movement was occurred up to ten times per day and symptom duration
was not over maximum ve minutes. Most events occurred during the
day, but were also occasionally present at night. There were no precipitating factors affecting such movements. There was no family
history of a Movement Disorder. An MRI scan of the brain and EEG
were normal. We tried placebo to differentiate with psychogenic Movement Disorder, but sometimes her symptoms were disappeared, sometimes not. She has been maintained on clonazepam with modest improvement.
Conclusions: This case displayed clear, paroxysmal dyskinesia involving the extremities and face. Her symptom is begun too late,
considering of average onset age of paroxysmal dyskinesias. Her symptom attacks both while awake and during sleep, this is not usual. We
present an unusual patient with paroxysmal dyskinesia who has late
onset and attack during sleep.
146
Long-term therapy of blepharospasm and facial hemispasm with
botulinum toxin type A
H. Streitova, M. Bares, I. Rektor (Brno, Czech Republic)
Objective: The goal of study was to evaluate effect of long-lasting
therapy of blepharospasm and facial hemispasm with BTX-A and a
comparison of therapeutic outcome in these diseases.
Background: The facial spasm and blepharospasm belong to most
frequent facial dyskinesias treated by botulinum toxin.
Methods: Selection criteria: The regular treatment with BTX-A at
least 10 years and continuous good clinical response for the BTX-A

Movement Disorders, Vol. 22, Suppl. 16, 2007

injections. Studied group consisted of 27 patients (9 patients with

blepharospasm - mean age of disease onset 53 years and 18 patients
with facial hemispasm mean age of disease onset 48 years. The
efciacy of treatment was evaluated by clinical neurological exam,
patients self-assessment and Jankovics rating scale.
Results: Mean follow-up of the patiens with blepharospasm was 11.4
years (range 10-13 years), with facial hemispasm 11.1 years (range
10-13 years). Mean number of treatment sessions in patiens with
blepharospasm was 41 (range 34-46 sessions), in patients with facial
hemispasm 38 (range 26-45 sessions). Mean period between two consecutive treatment sessions in patiens with blepharospasm was 11
weeks (range 9-13 weeks), with facial hemispasm 12 weeks (range
10-20 weeks). Side effects were not ascertained in four patients with
blepharospasm in the course of treatment. In ve patients side effects
appeared from one to seven times and manifested by transient double
vision or eyelid ptosis. In nine patients with facial hemispasm side
effects were not present during the treatment. In nine patients side
effects appeared infrequently, and were manifested mostly by transient
lower facial weakness.
Conclusions: Administration of BTX-A injections is effective and
safe treatment in long-term therapy of facial dyskinesias. Despite of
different pathophysiology of blepharospasm and facial hemispasm,
effectivness of therapy is comparable.

147
Bilateral cortical grey matter changes support the sensory endophenotype hypothesis in familial adult onset primary torsion dystonia: A VBM study
R. Walsh, R. Wheelan, J.P. ODwyer, S. ORiordan, S. Hutchinson,
R. Reilly, R. OLaoide, K. Malone, M. Hutchinson (Dublin, Ireland)
Objective: Our hypothesis was that unaffected relatives of patients with adult onset primary torsion dystonia (AOPTD) who have
abnormal spatial discrimination thresholds (SDTs) would also have
morphometric differences in the striato-thalamo-cortical circuit
compared to those with normal SDTs and unrelated healthy control
subjects.
Background: Regional volumetric grey matter changes and loss of
normal neural somatotopy in the primary sensory cortex and putamen have been described in AOPTD. Abnormal SDTs are found in
patients with AOPTD and as an endophenotypic trait in unaffected
relatives.
Methods: Voxel based morphometry (VBM) was used to analyse
high-resolution T1-weighted MRI images. Thirty-one unaffected subjects from 5 multiplex AOPTD families were recruited. Images of 14
unaffected relatives with abnormal SDTs were compared with those of
14 unaffected relatives with normal SDTs and 14 unrelated control
subjects. Subjects were matched for age, sex and handedness. Paired
t-tests were performed with correction for multiple comparisons using
a false discovery rate (FDR) of 0.05.
Results: No difference in grey matter volume (GMV) was observed
comparing family groups with normal and abnormal SDTs. A post-hoc
comparison of all 31 family members with 26 healthy control subjects
revealed a bilateral increase in GMV in the primary sensory cortex that
remained signicant following FDR correction. An increase in putaminal GMV was also identied bilaterally which did not survive correction.
Conclusions: VBM did not identify an abnormality specic to relatives with abnormal SDTs. SDT testing may not have sufcient specicity and sensitivity in reecting altered cortical morphology as an
endophenotype in AOPTD. Statistical power may also be an issue.
However, comparing all of the unaffected relatives of patients with
AOPTD with unrelated controls, we found evidence of a structural
disease endophenotype involving the primary sensory cortex and possibly the putamen.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

148
Tactile training with or without 1Hz rTMS to primary motor
cortex: A case study in two patients with focal hand dystonia
A.J. Nelson, W. Chau, B. Ross, G. Carolyn, R. Chen (Toronto,
Ontario, Canada)
Objective: Focal hand dystonia (FHD) is a movement disorder
characterized by excessive, involuntary contractions of the muscles of
the hand. Though clearly a disorder of movement, there are welldocumented abnormalities in touch perception and in the organization
of the digit tips within the primary somatosensory cortex (SI); spatial
acuity is diminished and digit maps are overlapping or even disordered.
Background: We hypothesize that improvements in tactile perception will reduce the occurrence of dystonia symptoms, and that these
changes will be accompanied by changes in somatotopic digit organization.
Methods: Tactile training was directed at the most affected digits in
an operant conditioning paradigm whereby the detection of targets
was rewarded. Two FHD patients experienced 15 days of either 1) 1Hz
rTMS to primary motor cortex followed immediately by tactile training
or 2) tactile training only. Measures acquired before and after the
15-day training include 1) somatotopic digit representation maps acquired with fMRI (blocked-design, affected hand only), and MEG
(steady-state response) 2) psychophysics via temporal acuity (minimum ISI between two stimuli), and spatial acuity (JVP domes), and 3)
motor performance via pinch grip force, and clinical rating scales.
Results: Tactile spatial perception improved in both patients and
changes in tactile cortical areas were also revealed. Dystonia symptoms
were mildly improved in the patient receiving tactile training only.
Conclusions: Tactile training alone can improve tactile perception
and lead to subtle improvements in writing quality. Tactile training
coupled with 1Hz rTMS led to improved tactile perception without
changes in writing quality.
149
Nuclear envelope phenotype in Dyt1 mutant mice
Y. Li, F. Yokoi, M. Dang (Birmingham, Alabama, USA)
Objective: Dystonia is dened clinically as involuntary, often sustained co-contraction of agonist and antagonist muscles causing abnormal and often painful postures or movements. It is generally believed
to be mediated by the central nervous system and represents the third
most common movement disorders after Parkinsons disease and
tremor. Oppenheims dystonia (DYT1 dystonia) is an inherited, generalized dystonia that usually begins in childhood, and becomes progressively worse. It is transmitted as an autosomal dominant disorder
with 30-40% penetrance. Genetic studies have linked a 3-bp deletion
(GAG) that deletes a glutamic acid residue (E) in the C-terminal coding
region of DYT1 (DYT1GAG) to early-onset dystonia in patients. DYT1
codes for torsinA that is a novel member of the AAA (ATPase
associated with a variety of cellular activities) superfamily which
includes ATPases involved in protein chaperone functions, vesicle
trafcking, and membrane fusion. The DYT1 gene is expressed widely
both inside and outside the brain. The function of torsinA protein and
the mechanisms by which the mutant tosinA protein (torsinAE) works
to lead to dystonia are largely unknown. Recently, torsinA has been
linked to nuclear envelope. Overexpression of mutant torsinA appears
to move the mutant torsinAE protein to nuclear envelope (Bragg et al.,
2004; Gonzalez-Alegre and Paulson, 2004; Goodchild and Dauer,
2004; Hewett et al., 2003; Naismith et al., 2004). More recently,
abnormalities of nuclear envelope, or blebbing, have been demonstrated in young neurons isolated from late term embryos or new born
pups that lack torsinA protein or express only torsinAE (Goodchild et
al., 2005). However, these developmental stages are known to associate
with massive neuronal death that could display similar abnormalities of
nuclear structures. To resolve this issue, we have used cre-loxP technology and generated mutant mice that either lack torsinA or express
only mutant torsinAE protein in restricted brain regions. These conditional torsinA knockout mice could survive at least one year postnatal
thus permitting the analysis of the nuclear envelope in mature neurons

S45

that have well passed early development. The analysis of nuclear

phenotype in these mice will provide new insight into the roles torsinA
or mutant torsinAE protein play in the functional integrity of nuclear
envelope and eventually, in the pathogenesis of DYT1 dystonia. Supported by Dystonia Medical Research Foundation, BachmannStrauss
Dystonia & Parkinson Foundation, Inc, and NINDS NS47692.
150
Neuroanatomy of dystonia: A motor network concept
V.K. Neychev, E.J. Hess, V.I. Mitev, H.A. Jinnah (Baltimore,
Maryland, USA)
Objective: To investigate the motor network model by evaluating the
inuence of basal ganglia lesions on the expression of dystonia in two
well characterized mouse models where disorder is known to be caused
by dysfunction of the cerebellum.
Background: The neuroanatomical substrates for dystonia are incompletely understood. Some studies implicate the basal ganglia while
others implicate the cerebellum or other regions. These observations
suggest dystonia may reect the nal outcome of lesions in different
motor systems, or abnormal communication between different nodes in
a broader motor network.
Methods: The two mouse models of cerebellar dystonia included a
pharmacological model elicited in normal mice by intracerebellar injection of excitotoxin kainic acid and a genetic model of paroxysmal
dystonia in tottering mice carrying mutant P/Q type Ca2 channels.
We performed bilateral intrastriatal microinjection of 6-hydroxydopamine or quinolinic acid in each model to induce subclinical nigrostriatal
or striatal damage. After full recovery from the lesions, dystonia was
compared among respective lesioned and sham-lesioned animals by
two different dystonia rating scales.
Results: Lesioned and unlesioned mice exhibited normal baseline
motor behavior, conrming a successful subclinical lesion. However,
dystonia was worsened by the lesions in both models. For both models,
there was a longer duration of dystonia. There was also an increase in
the frequency of spontaneous dystonic attacks in tottering mice.
Conclusions: These data support a motor network model for dystonia
and suggest a more unifying hypothesis that accommodates evidence
previously considered conictory.
151
New onset or worsening psychosis in patients with Wilsons disease
on treatment
A. Aggarwal, A. Nagral, M. Bhatt (Mumbai, Maharashtra, India)
Objective: To study the phenomenology & therapy of psychosis in
Wilsons disease (WD) patients on treatment.
Background: WD is an inherited disorder resulting in copper deposits in liver & brain.
Methods: We prospectively studied 25 WD patients for new onset or
worsening psychosis. All underwent a structured interview, detailed
systemic, neurologic, cognitive & psychiatric examinations. We dened psychosis in WD & evolved a grading (from 0-4) for it as a part
of our Global Assessment Scale for WD (GAS for WD) 1. We correlated psychosis with other clinical features of WD & studied its
treatment implications.
Results: 7 patients (mean age:17.4 years; range 12-25) developed
psychosis while on treatment for WD for an average of 6.3 years
(range1-12). 5 patients had a new onset & 2 had worsening of preexisting psychosis. 4 of the former were initially bedbound & mute
from dystonia. Psychosis became manifest in them with motor improvement. Most prominent & frequent complaints were of irritability,
aimless wandering, running away from home, hypersexuality, disinhibition and threat of suicide. Other symptoms included variable combination of aggressiveness, paranoia, delusions & hallucinations. Psychosis led to social isolation, dropping grades, expulsion from school,
necessitated contact supervision & in 2 cases hospitalization. All patients were non-compliant with WD drugs resulting in deterioration of
motor symptoms in 3. Pearsons correlations showed that psychosis
was an independent of other neurologic & systemic manifestation of

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

WD. 5 patients required antipsychotics. All necessitated review and

modication of WD drugs. Emergence of or worsening of psychosis
during treatment of WD was intriguing & had several different explanations - 1) Aggressive de-coppering therapy resulting in sudden mobilization of excessive copper from liver to brain, 2) Inadequate decoppering therapy of copper or noncompliance, 3) Unmasking of
pre-existing psychosis on improvement in mutism & motor disability,
4) And possibly a susceptibility among adolescents to manifest psychosis.
Conclusions: Psychosis can emerge as a serious problem even after
long term treatment for WD. It can be disabling, require hospitalization
& antipsychotics. A review and modication of WD treatment is
essential.
1 Bhatt et al. A novel Progressive 3 Tier (P3T) scale for Wilsons
Disease. Mov Disord 2006. 21;S15:432.
152
Defective inhibition and functional connectivity in pianists with
musicians dystonia (MD): An EEG study
M. Herrojo Ruiz, P. Senghaas, M. Grossbach, H.-C. Jabusch,
M. Bangert, F. Hummel, C. Gerloff, E. Altenmuller (Hanover,
Germany)
Objective: (1) To investigate the neural correlates associated with the
activation (ACT) and inhibition (INH) of motor memory traces in
pianists with MD, a form of focal task-specic dystonia (FTSD), during
a relevant motor task under constraint timing conditions with multichannel EEG. (2) To study the relation of the EEG oscillatory properties with the degree of motor control impairment.
Background: Recent neurophysiological studies have provided evidence for the hypothesis that FTSD is associated with impaired inhibitory function. It has been reported that alpha oscillatory EEG activity
increased over the sensorimotor areas of control subjects during INH,
whereas this increase was absent in patients with FTSD.
Methods: We investigated the functional connectivity and oscillatory
activity of cortical motor areas in 9 pianists with MD and 9 healthy
pianists during the execution and inhibition of externally paced longterm overlearned motor programs. Subjects played C-Major scales on
a digital piano in a go/no-go paradigm. EEG was recorded from 28
surface electrodes. Performance was recorded by MIDI technology.
The mean standard deviation of inter-onset intervals (IOI), previously
described for quantication of motor impairment in pianists with MD,
was analyzed.
Results: During INH, in pianists with MD the physiological power
increase of alpha (8 Hz) oscillations over the sensorimotor cortex was
reduced as compared to healthy pianists, indicating defective inhibitory
control in these patients. Additionally, functional connectivity in the
theta band (6-8 Hz) between SMA and contralateral premotor and
primary sensorimotor cortex was reduced in pianists with MD. During
ACT, the degree of inhomogeneity (standard deviation) of the decrease
in beta oscillations (SDbetaERD) correlated signicantly (r0.6 and
p0.02) with the IOI parameter in pianists with MD.
Conclusions: Our ndings support the hypothesis of a decient
information ow between the neuronal assemblies required to inhibit
motor memory traces in patients with MD. Further, the electrophysiological parameter SDbetaERD was found to reect the motor impairment in pianists with MD.

laterocollis and/or shift of the head). Little is known about RC since

cases are usually excluded from clinical trials. Treatment of RC includes bilateral botulinum toxin injections in splenious capitis, trapezius, semispinalis capitis and levator scapulae muscles. Although RC
has been reported in up to 29% of patients with CD there is a very
limited number of studies focused on RC. A Medline title search
revealed only 8 manuscripts since 1955, mostly single case reports.
Methods: We performed a review of consecutive CD patients seen in
our Division over a 15-year period. The predominant type of CD
(torticollis, laterocollis, RC and anterocollis) was determined primarily
by the degree of restriction of head movements and the dystonic head
position. The Fahn-Marsden Movement scale was used for rating of
dystonia.
Results: Out of 399 CD patients 59 (14.8%) had clinical features of
RC (mean age 61.613.6, mean duration 10.78.5). Pain in the
cervical region was very frequently reported among patients (80%).
RC was associated with neuroleptic exposure (20.3%) and a history of
head or neck trauma (23.7%). Of the patients injected with botulinum
toxin type A 24.5% reported excellent, 32.1% moderate, 16.9% mild
and 24.5% no response to injections (mean duration of effect 3.81.6
months). The splenius capitis, trapezii, scalenes, levator scapulae, semispinalis and obliques were injected for the treatment of RC. Oral
antidystonic medications had limited contribution to symptom relief.
Conclusions: In contrast with previous reports RC does not seem to
differ signicantly from other forms of CD. Risk factors include
neuroleptic exposure, trauma, spinal pathology and weight-lifting.
Treatment with BTX injections was successful and was infrequently
associated with side effects. Our ndings suggest that patient with any
form of RC should not be excluded from feature clinical trials on CD.
154
The Thorburn posture: See it again for the second time
P.J. Sweeney (Cleveland, Ohio, USA)
Objective: To demonstrate, via a video presentation, a very unusual
Movement Disorder assumed by a patient with an intramedullary
cervical cord lesion.
Background: In 1885, William Thorburn, a surgical registrar practicing at the Manchester Inrmary, described a series of nine patients
all with severe traumatic cervical spine and cord damage ultimately
resulting in the death of six of the patients. Two of the fatal cases, who
survived for several days before expiring, developed this unusual
posturing. From a photograph of one, Thorburn made a hand drawing
of the patient which was included in his original article. Post mortem
hand drawings again by Thorburn illustrated, in both cases, extensive
crush injury to the cervical spine and cord immediately below the
level of the fth cervical nerves.
Methods: 1. MR Imaging of patients cervical cord 2. Video of patient
movement 3. Video of recorded international event.
Results: Correlation between neurophenomenology of the 19th Century and the present.
Conclusions: Despite being a rare movement phenomenon, this
posturing has nevertheless been observed by millions of individuals
via the format of a tourist movie which, serendipitously, recorded
one of the great tragedies of the 20th Century in which the victim
assumed the Thorburn posture. A video of the event will be presented.

153

155

Retrocollis: Classication, clinical phenotype, treatment outcomes

and risk factors
S. Papapetropoulos, S. Baez, J. Zitser, C. Sengun, C. Singer (Miami,
Florida, USA)

Secondary nonkinesigenic paroxysmal dystonia after thalamic infarcts

L.C. Shih, M. Sobeih, D. Tarsy (Boston, Massachusetts, USA)

Objective: To describe the demographics, phenotypic characteristics,

treatment outcomes and risk factors for retrocollis (RC).
Background: RC is a form of cervical dystonia (CD) that produces
patterned, repetitive muscle contractions that result in neck extension.
RC may be primary or secondary (i.e. tardive). Focal RC may be (i)
simple/pure or (ii) complex (combination of RC and torticollis and/or

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To describe a patient with secondary paroxymal nonkinesigenic dyskinesia (PNKD) following ischemic lesions of the thalamus and responsive to anticonvulsant treatment.
Background: In a minority of cases, paroxysmal dyskinesias are
secondary to structural brain lesions. In some of these, vascular
etiologies of secondary PNKD have been described. They have been
associated with infarcts in several sites including thalamus, puta-

POSTER SESSION I, TUESDAY, JUNE 5, 2007

men, globus pallidus, and other subcortical locations. Most have
been poorly responsive to anticonvulsant medications. The relationship of these to cortically based epilepsy is ambiguous given the
equivocal EEG ndings which are often present and the response of
kinesigenic forms of paroxysmal dyskinesia to anticonvulsants.
Methods: Case report and PubMed search.
Results: A 7 year old girl with a history of vein of Galen malformation treated with coil embolization at age 6 months developed
paroxysmal stiffening of the right arm beginning at age 5. Initial
episodes consisted of exion posturing of the right elbow. Over several
months these evolved to include right facial droop, pursing of the
mouth, rightward tongue deviation, anarthria, and stiffening and inversion of the right foot. Initially, the episodes were triggered by stressful
situations such as being disciplined. Episodes lasted 5-90 minutes and
at their peak occurred 6-10 times daily. Some were observed during
sleep. Neurologic exam between episodes was normal. There was no
family history of neurologic disease. EEG showed frequent left central
and midtemporal spikes but inpatient telemetry EEG during these
episodes showed no electrographic-ictal correlates. Oxcarbazepine signicantly decreased the frequency of episodes and, after phenytoin was
added, the episodes ceased completely. Brain MRI immediately after
embolization at age 6 months showed large infarctions in anterior and
posterior portion of left thalamus. MRI at age 6 revealed T2 and FLAIR
hyperintense signal in the same locations.
Conclusions: Ischemic lesions of the thalamus are a very rare cause
of paroxysmal dyskinesia. Two cases of paroxysmal kinesigenic dyskinesia and one case of PNKD accompanied by xed neurologic
decits have been reported. Our patient was unique in having PNKD
secondary to thalamic infarction without neurologic decit who exhibited an excellent response to anticonvulsant treatment.

156
Treatment of post-traumatic segmental axial dystonia with zolpidem
M.-W. Seo, S.-Y. Jeong (Chunju, Jeonbuk, Korea)
Objective: We report this case and discuss the pathomechanisms of
post-traumatic segmental axial dystonia (SAD) as well as the pharmacotherapeutic mechanisms of zolpidem on SAD.
Background: It has been well-established that trauma to the central
nervous system can cause dystonia. The types of dystonia secondary to
craniocerebral trauma include hemidystonia, cervical dystonia, and
spasmodic dysphonia. Isolated dystonia of the trunk and neck muscles
without involvement elsewhere has been termed segmental axial dystonia (SAD). To the best of our knowledge, only one case of posttraumatic SAD has been reported thus far. Zolpidem, a selective agonist for
benzodiazepine 1 receptors, exhibited hypnotic effects in most cases,
but it has also been shown to improve catatonia, mutism, aphasia,
ataxia, parkinsonism, and jumpy stumps. We recently experienced a
case of SAD that responded well to zolpidem.
Methods: Case report A 35-year-old man diagnosed with SAD
suffered from severe truncal and neck dystonia when walking or
standing. He lost consciousness for 2 weeks after experiencing a
traumatic trafc accident. Two months after experiencing the trauma,
he noticed truncal and neck dystonia when walking and standing. He
visited our clinic 2 years after the trauma without any improvement
with various medications, including levodopa and clonazepam.
Results: He was then prescribed zolpidem. His dystonia dramatically
improved after he began taking the zolpidem (10 mg/day). He has
experienced marked improvement in his SAD with zolpidem (20-10-10
mg TID/day) treatment. He reported mild visual dimness as a side
effect of zolpidem.
Conclusions: His response to zolpidem suggests that zolpidem can
be used as an effective therapeutic treatment for SAD. The action of

S47

zolpidem as a selective inhibitor for the GABAergic neurons in the

ventral GP and GN might be one of the most plausible pharmacotherapeutic mechanisms of zolpidem.
157
Quantitative characteristics of limb tremor in cervical dystonia
A.G. Shaikh, H.A. Jinnah, R.M. Tripp, S. Ramat, D.S. Zee
(Baltimore, Maryland, USA)
Objective: We quantied limb tremor associated with cervical dystonia.
Background: Patients with cervical dystonia (CD) often show limb
tremor, but its characteristics are not well described.
Methods: We quantied limb tremor in 19 patients with cervical
dystonia and compared it with that of 35 patients with essential tremor
(ET). Postural, resting, and intention tremor was recorded using threeaxis accelerometer. The amplitude, amplitude-weighted mean frequency, and regularity of the tremor were quantied.
Results: The amplitude-weighted mean frequency of the limb tremor
in both cervical dystonia and essential tremor ranged between 4-10 Hz.
There was no signicant difference between the amplitude of limb
tremor in CD and ET. As in ET there was cycle-to-cycle variability in
the amplitude and frequency of tremor in CD. The most remarkable
difference between ET and CD was in the regularity of the tremor, i.e.,
the cycle-to-cycle variability of the frequency. The difference in frequency from cycle to cycle during a given recording session was
signicantly greater (by about 50%) in CD (frequency spread: 1.44
0.45) than in ET (frequency spread: 1.0 0.33 Hz) (ANOVA, Tukey
Kramer Honest Statistical Difference, p0.05) (Figure 1).
Conclusions: It is likely that limb tremor in CD arises from pathological central oscillations in more than one neural network (like ET).
The variability in the cycle-to-cycle frequency in tremor could be
explained by the differences in the natural frequency of the underlying
central oscillators, which in turn could be determined by the prole of
ion channels expressed on the cell membrane of the constituent neurons. Therefore, it is possible that the expression proles of the active
ion channels that determine the membrane kinetics of the underlying
central oscillators in ET are more similar (resulting in less cycle-tocycle frequency variability) than in those that produce the limb tremor
in CD.

FIG. 1 (157).

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

158
Disturbed topographic specic plasticity in cervical dystonia and
blepharospasm
A. Schramm, D. Weise, M. Beck, K. Reiners, J. Classen (Wuerzburg,
Germany)
Objective: To assess the gain and the spatial organization of neuronal
plasticity of the hand-associated motor cortex in cervical dystonia (CD)
and blepharospasm (BS).
Background: Maladpative plasticity may be involved in the pathophysiology of focal dystonia. We have previously shown (1) that in
writers cramp, a focal dystonia of the hand, long-term potentiation-like
neuronal plasticity, as assessed by an external paired stimulation protocol targeting the hand-associated motor cortex, is enhanced and the
strongly topographic organization of induced plasticity is lost. It is,
however, unknown how these abnormalities relate to the dystonic
phenotype.
Methods: Paired associative stimulation (PAS) was performed by
combining repetitively (0.1Hz, 180 pulses) electric stimulation of the
right median nerve with subsequent transcranial magnetic stimulation
(TMS) of the dominant motor cortex at 21.5ms. The magnetic coil was
placed over the optimal cranial position for eliciting a motor evoked
potential (MEP) in the contralateral abductor pollicis brevis muscle
(APB). 7 patients with CD (age 5110y; means.d.) and 6 patients
with BS (age 653y) were examined. Excitability changes (MEPamplitudes in APB and abductor digiti minimi muscle (ADM)) were
monitored by TMS for up to 55 min after intervention.
Results: After PAS, MEP amplitudes of the APB, whose cortical
representation received input from spatially homotopic sources, increased moderately in CD (10%) and BS (14%), substantially less
than the excessive increase of excitability (53%) noted previously in
writers cramp patients. MEP amplitudes of heterotopically stimulated
ADM increased no less (BS 14%) or even more (CD 62%) than in
APB. Excitability of ADM-representation was increased during the
whole time interval.
Conclusions: In BS and CD, the topographic organization, but not
the magnitude of PAS-induced plasticity, is deranged in cortical areas
not involved in the control of the dystonic body part. Somatotopical
disorganization of plasticity may represent a permissive pathophysiological disturbance in dystonia. In contrast, enhanced gain of plasticity
may be related to the development of the dystonic phenotype.
(1) Weise et al., Brain 2006;129:2709-21.
159
Natural course of idiopathic torsion dystonia: Is focal dystonia
actually focal?
M.V. Svetel, T. Pekemzovic, N. Ivanovic, J. Jovic, N. Dragasevic,
V.S. Kostic (Belgrade, Serbia)
Objective: The aim of our study was to follow clinical course and
mode of symptoms spreading, and to nd out risk factors which dene
patients at risk for spreading of dystonia.
Background: The clinical course of dystonia shows that certain
number of focal dystonias can spread involving of nearby structures
with subsequent generalisation of symptoms, while in some percentage
of patients spontaneous remission appears.
Methods: We examined 132 patients with different forms of dystonia
(96 with focal dystonia, 26 with segmental dystonias, and 10 with
generalised dystonias).
Results: The youngest were patients with lower limb dystonia
(14.52.2), while patients with blefarospasm were the oldest
(54.610.0). Difference was statistically signicant (p0,0001). Risk
for symptoms spreading was the greatest in patients with blefarospasm
in comparison with other dystonias (RR2.0, p0.190). In patients
with arm dystonia the risk was RR1.3, p0.541. The risk was lowest
in patients with spasmodic dysphonia. In torticolis patients the relative
risk was RR0.9; p0.621. Symptoms were spreading by involving

Movement Disorders, Vol. 22, Suppl. 16, 2007

left and right arm (38%), lower part of face and torso (each 23%), in
graphospasm involving opposite arm (49%), oromandibul muscles, lids
and neck (each 17%), while in blepharospasm patients-lower parts of
the face (40%), neck(30%) and upper extremities (30%) were further
regions disturbed by dystonia. In patients with torticollis as a presenting
feature the disease stays focal in 80.4%, while 13.7% patients develop
segmental, and in 5.8% generalised dystonia. Appearing of rst symptoms before the age of 20 always mean (90%) progression of symptoms
to generalised (66.7%) or segmental form of the disease (33.3%), while
only 19.2% adult patients develop segmental dystonia. In patients
younger than 20, risk of symptoms spreading is twice higher than in
older (RR2.0, p0.007). In majority of patients (40%) disease was
progressing during the rst 12 month, in 14.5% in the course of the
second year, and in 12.7% in the third year after disease onset.
Conclusions: Cox hazard proportional model extract as main predictors for spreading of dystonia age at onset, presence of tonic dystonic
movement and tremor at the distant body part, as well as disease
severity.
160
Transcranial magnetic stimulation in myoclonus-dystonia
S.M.A. van der Salm, A.-F. van Rootselaar, E.M.J. Foncke,
J.T.H.M. Koelman, L.J. Bour, K.P. Bhatia, J.C. Rothwell,
M.A.J. Thijssen (Amsterdam, Netherlands)
Objective: The aim of this study is to investigate cortical excitability
in patients with genetically proven myoclonus-dystonia (M-D), using
transcranial magnetic stimulation (TMS).
Background: The identication of genes in the hereditary forms of
dystonia gives the opportunity to study its pathophysiology in a well
dened homogeneous group. M-D is an autosomal dominantly inherited disorder, clinically characterized by myoclonic jerks and dystonic
postures or movements of the upper body, which are responsive to
alcohol, combined with psychiatric features such as anxiety and obsessive compulsive disorder. The major gene for M-D, the epsilon-sarcoglycan gene (SGCE, DYT11), is located on chromosome 7q21.
Methods: We assessed silent period (SP), recruitment curve (RC),
intracortical inhibition (ICI), intracortical facilitation (ICF) and short
interval intracortical facilitation (SICF), with very short interstimulus
intervals ranging from 1.2 to 3.2 ms, in 15 DYT11-positive M-D
patients and 15 healthy controls.
Results: All these parameters were normal compared to healthy
controls. Interestingly, M-D patients showed polyphasic and more
variable MEPs compared to controls in the paired pulsed SICF. This
was not seen in MEPs elicited by single TMS pulses. Cross-covariance
analysis of MEP area revealed a signicant correlation difference at ISI
2.2, 2.4 and 2.8 ms with a signicant time course effect on MEP
variability over all ISI (p0.0001).
Conclusions: The DYT11 mutation in M-D appears to lead to neuron
membrane instability, as reected by asynchronicity of MEPs. Our
ndings may indicate abnormal motor processing of cortical excitatory
and inhibitory mechanisms underlying I-wave generation, perhaps contributing to impaired control of movement resulting in M-D. Furthermore, this study shows that the SICF protocol has potential to provide
new insights into the pathophysiology of Movement Disorders.
161
A new locus for adult-onset Focal Idiopathic Torsion Dystonia
M.Y. Frederic, C.-M. Dhaenens, C. Davin, R. Mazzoleni, A. Kreisler,
I. Vuillaume, M. Marinez, M. Claustres, S. Tuffery-Giraud,
G. Collod-Beroud (Montpellier, France)
Objective: To map the gene implicated in a large French family
presenting with varied symptoms of adult-onset Focal Idiopathic Torsion Dystonia (FITD).
Background: Primary FITD are the most common forms of dystonia.
Currently, 3 loci associated with primary FITD have been mapped:

POSTER SESSION I, TUESDAY, JUNE 5, 2007

DYT6, DYT7 and DYT13 but no gene has been yet identied. It seems
likely that these loci account for only a small proportion of FITD as
linkage analyses excluded these loci in several families with similar
phenotypes.
Methods: We studied a 3-generation family composed of 30 subjects,
with 6 denitely affected living individuals and one asymptomatic
obligate carrier. The average of onset is 43 20 years. For each of the
known localizations and for genome wide study, Genethon microsatellites markers were studied. Linkage analyses have been performed
with a parametric model. To take into account the incomplete penetrance observed in this disorder, incomplete age-dependant penetrance
has been dened as different liability classes.
Results: Haplotypes excluded the DYT6, DYT7 and DYT13 regions
for this family. Genome-wide linkage analysis identied a new highly
probable locus, DYTL, with several lod scores 2 for contiguous
markers and a maximum of 2.37 (maximum lod score estimated in the
family: 2.62) dening a 40 cM candidate region.
Conclusions: Our study highlighted a new probable region associated with adult-onset dystonia. This result has now to be validated by
obtaining a statistically signicant value of more than 3 by studying
new families in order to identify possible new recombinants. Moreover,
the DYTL analysis in other families shows possible segregation of this
locus with the disease in ten families and exclusion in 4 families. This
last result illustrates the great genetic heterogeneity of FITD and
indicates the existence of more than one unassigned genes for this
pathology.

S49

side effect; migration of one electrocatheter was the sole major adverse
event.
Conclusions: GPi-DBS is an effective and safe treatment for primary
segmental dystonia refractory to medical therapy.

163
Abstract Withdrawn

164
A case of cerebello pontine angle tumor presenting as cervical
dystonia
S. Chandran, Y.R. Godge, P.J. Oak, S.H. Ravat (Mumbai,
Maharashtra, India)
Objective: To report a case of cervical dystonia secondary to cerebello pontine angle tumor.
Background: Cervical dystonia as the presenting manifestation of a
cerebello pontine angle tumor is a rarity. We report a 52 year old man
who presented to us with cervical dystonia and on evaluation was found
to have a right cerebello pontine angle tumor.

162
Pallidal deep brain stimulation for primary segmental dystonia
S. Biguzzi, M. Sensi, M.A. Cavallo, C. Lettieri, R. Quatrale, E. Sette,
V. Tugnoli, E. Fainardi, M.R. Tola, E. Granieri, R. Eleopra
(Ferrara, Italy)
Objective: To report the long-term outcome of bilateral GPi-DBS in a
group of 9 clinically similar patients with primary segmental dystonia.
Background: GPi-DBS is now the preferred surgical treatment for
generalized dystonia refractory to medical treatment; however, the
indications for DBS in the treatment of segmental dystonia are still
debated.
Methods: We evaluated 9 consecutive subjects (7F, 2M) with idiopathic segmental dystonia (cranial-cervical-brachial pattern and DYT1
negative), who underwent bilateral GPi-DBS between 2000 and 2005 at
St. Anna Hospital, Ferrara, Italy. Electrodes were positioned with
intra-operative monitoring, and placement conrmed with post-op
MRI. The best caudal lead contact was used in monopolar mode; the
settings for mean amplitude, pulse width, and frequency were 3.5 V,
120 sec, 130 Hz, respectively. All patients were evaluated pre and
post surgery with the Burke-Fahn-Marsden Dystonia Rating Scale, the
quality of life scale (SF36) and a neuropsychological assessment.
Results: At 3 months, motor scores improved in all patients, with
30% to 90% reductions in the BFMDRS. Further improvements were
observed throughout the next 12 months, and these persisted in longterm follow up at 36 months. Sub-scale analysis revealed an early and
striking benet for all manifestations of dystonia, including cranial
dystonia, as well as speech and swallowing difculties, and these
results correlated positively with improvements on the disability and
quality of life scales. The most rapid and signicant responses were
seen in patients with phasic-hyperkinetic dystonias. Neck pain decreased dramatically in subjects who were free of arthritis, but xed
posturing associated with muscular-skeletal abnormalities responded
poorly. Younger age, earlier onset, and shorter duration of disease were
positive prognostic factors. None of the patients worsened cognitively,
and most reported improved mood. Dysarthria was the most common

FIG. 1 (164).

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: Case description.

Results: A 52 year old gentleman presented with 6 month history
of progressive abnormal posturing of neck. There was no preceding
head or neck trauma, encephalitic illness or stroke. Drug history was
unremarkable. He did not give any history of headache, loss of
hearing, tinnitus or vertigo. On examination he had a right laterocollis with mild torticollis. KF ring was absent bilaterally. He had
sensorineural deafness in right ear. MRI brain showed a right
cerebello pontine angle tumor. Laboratory investigations including
serum ceruloplasmin and thyroid function tests were unremarkable.
Patient underwent excision of the tumor. Histopathology was suggestive of schwannoma. Post operative his cervical dystonia improved.
Conclusions: The combination of cervical dystonia with right sensorineural hearing loss and improvement of the dystonia with surgery
suggests a causal relationship between right cerebello pontine angle
tumor and the dystonia. Although cervical dystonia secondary to tumors is rarely seen, it is to be kept in mind in the differential diagnosis.
Early recognition of such cases prevents progressive neurological deterioration and increase in tumor size. A complete resolution of the
dystonia may be possible if intervened early.
References
1. Krauss JK, Seeger W, Jankovic J. Cervical dystonia associated
with tumors of the posterior fossa. Mov Disord. 1997 May; 12(3):
443-7.

165
Botulinum toxin for treatment of task-specic orofacial dystonia
C.-H. Pek, R.C.S. Seet, J.-H. Yik, E.C.H. Lim (, Singapore)
Objective: To describe a patient with task-specic orofacial dystonia,
and its treatment with targeted injections of botulinum toxin (BTX).
Background: A 57 year-old Chinese woman was admitted with a
3-day history of twitching of the lower half of the left hemiface. She
had no fever, pain, headache, numbness, limb weakness, speech or
swallowing difculties. Past history was signicant for hyperlipidemia and Stage I cancer of the left breast, for which she underwent
lumpectomy and adjuvant radiotherapy. Clinical examination was
unremarkable, except for intermittent upward displacement of the
left side of the mouth. Contrast CT of the brain, electroencephalogram and cerebrospinal uid studies were normal, as were her
electrolytes. A diagnosis of left hemifacial spasm was made, the
twitching improved with clonazepam 0.5mg 3 times a day, and she
was discharged to our movement disorders service, where she was
seen one month later. Over the next month, the twitching had
extended to the right lower hemiface. On examination, it was noted
that she did not twitch, except when she closed her mouth or pursed
her lips together, at which time she demonstrated rabbit-like
orofacial movements due to overactivity of her obicularis oris,
mentalis, zygomaticus major and levator anguli oris bilaterally
(Video). These movements abated when she opened her mouth or
spoke. She did not demonstrate any sensory tricks. Brain MRI,
thyroid and liver function tests were normal. Initially, she refused
injections of type A botulinum toxin (BTX-A) to treat her taskspecic orofacial dystonia, but agreed to injections after she developed drowsiness with increased doses of Clonazepam 1mg 3 times
a day.
Methods: She was injected with 25 units of BTX-A to the orbicularis
oris, zygomaticus major, levator anguli oris and mentalis muscles
bilaterally.
Results: The movements resolved completely within 3 weeks, with
continued benet 9 months later.
Conclusions: Task-specic orofacial dystonia, a rare condition, can
be effectively treated with BTX.

Movement Disorders, Vol. 22, Suppl. 16, 2007

166
Survey of families presenting with late-onset focal idiopathic torsion dystonia in France
M.Y. Frederic, C.-M. Dhaenens, B. Sablonniere, R. Mazzoleni,
A. Kreisler, I. Vuillaume, M. Claustres, S. Tuffery-Giraud,
G. Collod-Beroud, the INSERM National Dystonia Network and GIS
Maladies Rares (Montpellier, France)
Objective: To recruit families presenting with late-onset Focal Idiopathic Torsion Dystonia (FITD) and evaluate disease transmission type.
Background: Primary adult-onset dystonia are the most common form
of dystonia. Clinical expression is variable, usually with focal onset (blepharospasm, oromandibular dystonia, cervical dystonia, laryngeal dystonia or
arm or leg dystonia) and with a limited tendency to spread to adjacent body
regions. Focal dystonia prevalence is estimated to 11.7:100,000. The fact
that different forms of focal dystonia may result in spreading of the disease
suggests that the different clinical forms share aetiological factors, among
them genetic factors are likely to be major.
Methods: In the aim of identifying the largest number of families
presenting with FITD, a mailing has been realized in September 2006
to contact more than 370 practitioners implicated in the follow up of
these patients in France (Neurologists, Ophtalmologists, Otorhynolaryngologists. . .) and the 103 regional representatives of the associations
of patients.
Results: We got in touch with more than 70 unrelated affected
individuals. After the search of positive familial history and the construction of the genealogic trees, we identied 43 isolated cases and 26
cases in a potential familial context. Collaborative relatives were referred to neurologists in order to assign an unambiguous diagnosis. In
addition, 17 families have been identied thanks to the collaboration
with B. Sablonnieres and A. Destees teams in Lille. The observed
inheritance of the disease in these families is in accordance with an
autosomal dominant transmission with incomplete penetrance.
Conclusions: Overall, this project has led to the identication of 213
affected patients of which 43 are isolated cases. A total of 43 families
(2617) including 170 individuals have been identied for which
intrafamilial clinical variability was observed. This number accounts
for 14 of the expected number of patients. This is the rst French cohort
of FITD patients that will contribute to the identication of genes
involved in these diseases.
167
Spasmodic dysphonia and writers cramp in the Korean patient
with novel missense mutations in the PANK2 gene
J.Y. Kim, W.Y. Lee, C.S. Ki, H.-Y. Shin, W.T. Yoon, E.J. Chung
(Seoul, Republic of Korea)
Objective: To report a 37 year-old Korean PKAN patient with two
novel missense mutations in the PANK2 gene, who presented late-onset
atypical clinical features such as spasmodic dysphonia, writers cramp
with tremor and parkinsonism later.
Background: Pantothenate kinase-associated neurodegeneration
(PKAN; OMIM 234200), which is previously called Hallervorden-Spatz
syndrome, has been reported to be caused by mutations in the pantothenate
kinase 2 (PANK2) gene. So far, a large spectrum of clinical features of
PKAN and various gene mutations of PANK2 are reported.
Methods: We performed brain MRI and sequencing analysis for
seven exons of PANK2 on chromosome 20p13 with the primers designed to amplify each exons.
Results: His brain MRI showed the appearance of the eye-of-thetiger sign in axial T2-weighted MRI (Figure 1). Sequence analysis
revealed two novel missense mutations in the patient: c.765AG
(Asp255gly) and c.950GC (Arg317Pro) based on the reference
mRNA sequence of NM_153638 (Figure 2).
Conclusions: We observed unreported atypical features such as
spasmodic dysphonia and writers cramp in the Korean patient with
novel missense mutations in the PANK2 gene.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

S51

various ethnic groups are caused by a unique and recurrent autosomal

dominantly inherited deletion (c.907delGAG). Based on prevalence in
Europe which is estimated between 0.3 and 0.5:100,000, the calculated
number of French symptomatic carriers range from 184 to 307. No
founder effect, as was reported in the Ashkenazi Jews (AJ), has been
described in France.
Methods: Data on positive TOR1A carriers have been collected from
the 4 laboratories. Whenever possible, the presence of the deletion in
each parent, the number of relatives carrying the mutation, and their
phenotypic status (symptomatic or asymptomatic) has been investigated. TOR1A anking microsatelites were studied.
Results: Fifty-two cases of EOTD carry the c.907delGAG mutation
among which 26 are familial cases. In apparently isolated cases, whenever the two parents could be analyzed, an asymptomatic carrier was
always found. Among 167 individuals (52 probands and their families)
who agreed to be informed of their carrier status, 108 carry the
mutation, among them 20 were asymptomatic. Haplotypes with TOR1A
anking microsatelites could be constructed in 31 families. Only 7 are
recurrent and correspond to the AJ haplotypes in two cases, to the other
AJ haplotype previously reported (Lebre 1999) in 3 families, and to 1
another haplotype in two families.
Conclusions: A lower number of individuals and families carrying
the mutation than expected has been identied in France (88 symptomatic patients versus 184). These results can be explained by the nonexhaustive testing of all mutation carriers and/or by a lower prevalence
of TOR1A-linked dystonia in France. Furthermore, haplotype analyses
conrm the quasi absence of founder effect for the TOR1A mutation in
France.

SURGICAL THERAPY
169
Apraxia of eyelid opening after subthalamic deep brain stimulation
may be caused by reduction of levodopa
A. Umemura, T. Toyoda, M. Mizuguchi, F. Ishii, K. Yamada
(Nagoya, Japan)

FIG. 1 (167).

168
Assessment of TOR1A mutation carriers identied through the network of TOR1A diagnostic laboratories in France
M.Y. Frederic, F. Clot, A. Durr, A. Brice, G. Lesca, I. Vuillaume,
A. Calender, B. Sablonniere, T. Besnard, D. Thorel, C. Saquet,
L. Ozelius, L. Hjermind, A. Roubertie, L. Cif, M. Claustres,
S. Tuffery-Giraud, G. Collod-Beroud (Montpellier, France)
Objective: To evaluate the number of families and individuals carrying the TOR1A mutation from the 4 national reference laboratories
and characterize the associated TOR1A haplotypes.
Background: The only currently identied primary early-onset torsion dystonia (EOTD) gene is TOR1A. The majority of cases from

Objective: We report on two patients who suffered from disabling

ALO after bilateral STN DBS. Their ALO improved by resuming
levodopa medication that was discontinued after the surgery.
Background: Apraxia of eyelid opening (ALO) is an infrequent side
effect of deep brain stimulation (DBS) of the subthalamic nucleus
(STN) for Parkinsons disease (PD). However, the mechanism of ALO
is not well understood.
Results: Patient 1 is a 56-year-old male with a 16-year history of PD.
He underwent bilateral STN DBS for severe motor uctuations and
dyskinesia. Although his symptoms were signicantly improved and
dopaminergic medication was markedly reduced after the surgery, he
suffered from disabling ALO beginning a month later. His ALO was
treated by adjusting the stimulation parameters or transiently turning
off the stimulators, then by injection of botulinum toxin. However, his
ALO was not relieved by these treatments. Six months later, his ALO
gradually improved by resuming levodopa, which had been completely
discontinued after the surgery. Patient 2 is a 63-year-old female with a
19-year history of PD. She underwent bilateral STN DBS for severe
motor uctuations. Although her symptoms improved and levodopa
was completely discontinued after the surgery, she suffered from disabling ALO beginning a month later. At rst, her ALO was successfully treated with pretarsal injection of botulinum toxin. However, the
effect of botulinum toxin wore off four months later and ALO appeared
again. At that point, levodopa was resumed based on the experience
with the previous patient. Her ALO dramatically improved afterward.
Conclusions: Although pathogenesis of ALO after STN DBS is not
well understood, postoperative modication of dopaminergic medication may be a cause of AOL after STN DBS.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

170
Determining factors for the reduction of dose of dopaminergic
drugs after bilateral subthalamic nucleus stimulation
S.J. Chung, S.R. Kim, T.Y. Lee, M.J. Kim, S.K. Lee, C.S. Lee,
M.C. Lee (Seoul, Republic of Korea)
Objective: We aimed to investigate the factors determining the
reduction of dose of dopaminergic drugs in patients who received
bilateral STN deep brain stimulation (DBS).
Background: Bilateral stimulation of the subthalamic nucleus (STN)
in advanced Parkinsons disease (PD) is associated with improvement
of motor complications and reduction of levodopa dose.
Methods: We studied 29 consecutive patients with PD who underwent bilateral STN DBS between March 2002 and January 2006.
Patients were assessed at baseline, 6 months, and 1 year after surgery.
Results: There were 11 men and 18 women with an age range of
39 to 68 years (57.0 7.2). Mean age at onset was 44.5 years
(range, 30 - 55 years) and mean disease duration was 12.0 years
(range, 4 - 26 years). At 6months, the scores of UPDRS III and total
UPDRS were improved by 41% (p 0.001) and 42% (p 0.001),
respectively. The dose of dopaminergic drugs was signicantly
decreased (16.9%) (p 0.05). At 1 year, the scores of UPDRS III
and total UPDRS were improved by 41% (p 0.05) and 40% (p
0.001), respectively. The dose of dopaminergic drugs was decreased
by 9.0% (p 0.067). Reduction rate of dose of dopaminergic drugs
after bilateral STN DBS was positively correlated with dose of
dopaminergic drugs at baseline (p0.05) and was negatively correlated with the scores of off-period UPDRS II (p0.05), III
(p0.001), and total UPDRS (p0.001) at baseline. The difference
of UPDRS scores between on and off periods on levodopa challenge
test was negatively correlated with the reduction rate of dose of
dopaminergic drugs after bilateral STN DBS (p0.001). However,
DBS settings, age, age of onset, and duration of disease were not
correlated with reduction of dose of dopaminergic drugs. On stepwise regression analysis, difference of the UPDRS III score between
on and off periods on levodopa challenge test and dose of dopaminergic drugs at baseline were important.
Conclusions: The main determining factors for the reduction of
dose of dopaminergic drugs after bilateral STN DBS in advanced
PD were difference of the UPDRS III score between on and off
periods on levodopa challenge test and dose of dopaminergic drugs
at baseline.
171
Cognitive and motor effects of globus pallidus externus stimulation
in patients with Huntingtons disease
P. Krystkowiak, D. Devos, K. Dujardin, C. Delmaire, E. Bardinet,
A. Delval, M. Delliaux, O. Cottencin, Huntington French Speaking
Group (Lille, France)
Objective: To examine cognitive and motor effects of globus pallidus stimulation in patients with Huntingtons disease (HD).
Background: The degenerative process in HD leads to rapid loss of
inhibitory neurons in the striatum which project to the globus pallidus
externus (GPe). On the basis of GPe hyperactivity, it has been demonstrated that lesion of this structure in animal models of HD improved
motor and cognitive performance. Bilateral, chronic, high-frequency
stimulation of the GPe in patients with HD could thus induce the same
benecial effects as in animal models.
Methods: This is an open label, non-controlled pilot study to be rst
performed with ve early HD patients who all received bilateral stimulation of the GPe. Before and six months after surgery, motor and
cognitive outcomes were evaluated (blinded procedure using video for
motor assessment) using the cognitive and motor part of the UHDRS
scale, UHDRS motor subscores (chorea and dystonia) and timed motor
tests. Cognitive UHDRS and motor tests were performed under two
randomized conditions (off and on stimulation), with a double-blinded
procedure. Independence Scale and Total Functional Capacity were
evaluated. Overall behavioural and cognitive tolerance (Mattis Demen-

Movement Disorders, Vol. 22, Suppl. 16, 2007

tia Rating Scale, Marins apathy scale, MADRS depression scale,

behavioural part of the UHDRS), as well as overall tolerance, were also
assessed.
Results: In one patient, stimulation improved in most motor and
cognitive tests. In three other patients, the improvement was more
heterogeneous. The last patient was impaired by stimulation. All the
active electrodes contacts were located within the GPe. Overall tolerance was excellent, especially the behavioural one.
Conclusions: In Huntingtons disease, GPe stimulation can improve
not only motor symptoms but also cognitive ones, with a good tolerance.
172
Social cognition and emotional recognition in early and late stages
of Parkinsons disease
J. Peron, I. Biseul, S. Fournier, S. Drapier, D. Drapier,
V. Thomas-Ollivier, R. Cohen, M. Verin (Rennes, France)
Objective: To replicate previous results showing social cognition
impairment in Parkinsons disease (PD) and to explore the involvement
of the dopaminergic pathways in social cognition.
Background: The few studies exploring social cognition, in particular theory of mind (ToM), in PD (Mimura, Oeda, & Kawamura, 2006;
Saltzman, Strauss, Hunter, & Archibald, 2000) seemed to provide
preliminary evidences of impairement. Nervetheless, the patients included in these studies were not accurately described, making it difcult to determine if social cognition decits were due to either a non
specic global cognitive deterioration in advanced disease or a more
specic dopaminergic decit.
Methods: Social cognition was investigated in twelve newly diagnosed PD patients (Early PD group), twelve PD patients with advanced
disease (Advanced PD group) and fourteen matched controls using an
extensive battery of tasks exploring several facets of social intelligence,
ToM, and recognition of facial emotions. In addition, the Early PD
group was studied in two conditions: with and without dopamine
replacement therapy.
Results: Social cognition and facial emotion recognition remained
intact in PD patients in comparison with the control group, regardless
the stage of the disease (early or advanced). In addition, there was no
difference between Early PD patients in the medicated versus unmedicated condition.
Conclusions: In contradiction with previous studies, our results demonstrate no impairment in social cognition in PD. Two main reasons are
proposed: (1) the homogeneity of our patient groups was far better than
those of previous studies (for example, in terms of age, duration and
severity of the disease) and (2) in opposition with previous studies, our
PD patients didnt show dysexecutive syndrome nor depression.
173
Gait disturbances induced by stimulation of the subthalamic region
in Parkinsons patients
G. Tommasi, M. Lanotte, E. Fincati, M. Zibetti, G. Moretto,
L. Lopiano (Verona, Italy)
Objective: To describe gait disturbances related to unilateral stimulation of the peri-subthalamic nucleus (STN) region in two cases of
Parkinsons disease (PD) patients treated by bilateral deep brain stimulation (DBS) of the STN.
Background: Several studies in PD patients have demonstrated that
freezing of gait (FOG) and hypokinesia of gait (HOG) can be signicantly ameliorated by STN-DBS, in a manner similar to that observed
with levodopa treatment. FOG and HOG in the medication-on condition are, however, often fairly resistant to both dopaminergic and
electrical stimulation.
Methods: Case reports: two males, one 66 years old (case #1) and the
other 57 years old (case #2) with a 13-year and 9-year history of PD,
respectively, were selected for STN-DBS.
Results: Despite a signicant global improvement in motor score
(46% for #1 and 47% for #2, as assessed by UPDRS III) obtained by
bilateral stimulation compared to the medication-off/stimulation-off

POSTER SESSION I, TUESDAY, JUNE 5, 2007

S53

condition, patients developed a disabling FOG (especially start and turn

hesitation) and HOG, which were evident before surgery only in the
medication-off condition. In case #1, FOG and HOG were caused by
stimulation through a left side misplaced electrode, which was more
antero-medial than the planned trajectory. Four months later, because
of persistent gait disturbances with bilateral stimulation, and poor relief
of the parkinsonian signs by right side stimulation alone, the patient
underwent repositioning of the left electrode, obtaining complete relief
of gait disturbances. In case #2, FOG and HOG were caused by
stimulation through too medially a placed right side electrode. On
turning off this electrode the patient recovered his normal gait. Moreover, no repositioning of the right side electrode was required, since left
side stimulation alone allowed fairly good control of the parkinsonian
signs.
Conclusions: FOG and HOG may be side effects of DBS of the
subthalamic region due to current spreading antero-medially to the
STN. These side effects are not subject to habituation and restrict any
increase in stimulation parameters. On the basis of the anatomical
location of the misplaced electrodes and physiological considerations
we hypothesized involvement of pallidal projections to the pedunculopontine nucleus in our patients gait disturbances.

174
Response of subthalamic nucleus neurons to vocalization
M.D. Richardson, S.G. Ojemann, O.S. Klepitskaya (Denver,
Colorado, USA)
Objective: To characterize the neurophysiological response of neurons in the subthalamic nucleus (STN) to vocalization in patients with
Parkinsons Disease (PD).
Background: Voice and speech impairment is a common and disabling feature of PD. It affects up to 90% of all patients, eventually
leading to a greatly diminished ability to communicate, social isolation,
and decline in quality of life. While the effectiveness of STN deep brain
stimulation (DBS) on motor function is well-established, its effect on
speech is variable and literature on this subject is controversial. Understanding the neurophysiology of basal ganglia and their role in
speech production is essential for optimizing and predicting outcomes.
Single neuron microelectrode recording (MER) is used to ensure precise placement of electrodes in the sensorimotor region of the STN,
affording the opportunity to study the neuronal response to speech in an
awake patient. Presented are the pilot data of recordings from 2 patients
with advanced PD.
Methods: B-STN DBS surgery was performed with the use of
frameless stereotactic technique and functional mapping by MER.
Upon accounting the neuron responsive to orofacial movements, patients were asked to pronounce single syllable words that appeared on
a computer screen. Multi-channel data acquisition hardware and spike
sorting software were used to digitize and analyze the neuronal signal.
Surface EMG from anterior neck muscles, voice, and digital video were
recorded simultaneously. Multivariate ANOVA statistical analysis was
performed for evaluation of ring characteristics.
Results: Recordings from patient number one demonstrated an increase in ring prior to vocalization with a further peak at the time of
vocalization (Fig.1). Recordings from patient number two showed
suppression of the signal during vocalization (Fig.2).
Conclusions: Neuronal response to vocalization from the sensorimotor region of the STN is variable. Preparation for vocalization may be
characterized by increased ring rates. Vocalization itself can have
either a positive or negative relationship with ring patterns of STN
neurons. STN neurons showing a positive correlation with speech may
be suppressing unintended movements via the indirect pathway. Units
showing a negative correlation may be allowing intended motor movements to proceed via the direct pathway. These data show a signicant
role for the STN in human vocalization.

FIG. 1 (174).

175
Complication avoidance with multitrack microelectrode recording
in STN-DBS for Parkinsons disease
H. Toda, H. Saiki, S. Matsumoto (Osaka, Japan)
Objective: To avoid stimulation-induced adverse effects, proper
placement of electrode is important in the subthalamic nucleus deep
brain stimulation (STN-DBS). The objective of the present study was
to investigate the effectiveness of the routine use of multitrack microelectrode recording (MultiMER) system with intraoperative stimulation
to avoid stimulation-induced complications.
Background: The best STN-DBS electrode has maximam therapeutic
effects and minimum adverse effects. MultiMER helps to compare the
electroanatomical proles of different trajectories without repetition.
We evaluate its benet and risk in this study.
Methods: MultiMER system Leadpoint (Medtronic) was systematically used with intraoperative stimulation during STN-DBS surgery in
20 procedures of 16 patients with Parkinsons disease. Targets were
determined based on preoperative MR images. The subthalamic nucleus was localized directly or indirectly. The trajectories was planned
on Framelink (Medtronic) to avoid cerebral veins, sulci, and lateral
ventricles. With Leksell stereotactic system (Elekta), multiMER identied the location of the STN and intraoperative stimulation eliminated

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

the improper trajectories. After comparison of test stimulation effects,

DBS electrode model 3387/3389 (Medtronic) were placed under uoroscope guidance.
Results: The trajectories entried from the middle frontal gyrus and
avoided vital structures. Two microelectrodes were used in 2 procedures, 3 in 4 , 4 in 15, and 5 in 1. Among ve trajectories, center was
used in 20 procedures, anterior in 19, posterior in 15, lateral in 18, and
medial in 1. The STN was identied with more than 4 mm in length in
all patients. Identication of adverse effects with intraoperative stimulation inuenced the selection of the nal trajectory with preserving
benet of STN-DBS. Test stimulation induced diplopia in 3 trajectories, dysesthesia in 4 trajectories, pyramidal tract sign in 3 trajectories.
Finally selected trajectories were central in 7 procedures, anterior in 5,
posterior in 3, and lateral in 5. There were no cases with cerebral
hemorrhage, infection, and hardware-related complications.
Conclusions: Our study supports the routine use of multiMER system with its intraoperative stimulation as a safe and reliable tool for
proper placing macroelectrodes without signicant complications.

176
Inuence of subthalamic deep brain stimulation and levodopa on
motor perseveration in Parkinsons disease
J. Herzog, B. Moller, K. Witt, G. Deuschl, J. Volkmann (Kiel,
Germany)
Objective: To evaluate the acute effect of subthalamic deep brain
stimulation (STN-DBS) and levodopa on motor perseveration in patients with Parkinsons disease (PD).
Background: PD patients show impairment in generating random
motor sequences. While STN-DBS has been found to signicantly
improve motor symptoms of PD, its impact on motor perseveration
behavior has never been elucidated.
Methods: In 35 PD patients, we evaluated the effect of STN-DBS
and levodopa on motor perseveration using the Vienna perseveration
task. The task was performed at six months follow-up in the following
three conditions: STIM OFF/ MED OFF (baseline), STIM ON/ MED
OFF and STIM OFF/MED ON. Patients were instructed to randomly
press nine circles avoiding the use of systematic or repetitive strategies.
Perseverative behaviour was assessed by relative redundancy of second
order (R2).
Results: In STIM OFF/ MED OFF, mean baseline R2 value in PD
patients (35.711.5) was signicantly higher than in the control group
(18.91.8, P0.001) indicating more severe perseveration in PD patients. In STIM ON/ MED OFF, ANCOVA analysis revealed that
change of R2 by STN-DBS signicantly depended on severity of
baseline R2 (F4.69, P0.035) whereas age, percentage improvement
of motor UPDRS by STN-DBS and Mattis dementia rating scale
showed no impact. Classifying PD patients in two groups according to
baseline R2 value revealed that STN-DBS in patients with moderate
baseline perseveration (R2 35) was not associated with change of R2
(0.92.4). In contrast, STN-DBS in patients with severe baseline
perseveration (R2 35) signicantly reduced R2 (9.72.6, P0.001).
In STIM OFF/ MED ON, levodopa led to signicantly less reduction of
R2 in patients with severe baseline perseveration (3.71.6) compared
to STN-DBS and even deteriorated performance in patients with moderate baseline perseveration (-4.12.2).
Conclusions: We show that STN-DBS favorably inuences repetitive behaviour in PD patients with severe motor perseveration whereas
there was no effect in moderately affected patients. These results
indicate that severe motor perseveration may depend from dysfunctional network activity which can be partially restored by dopamine but
more effectively by high-frequency stimulation of the subthalamic
nucleus.

Movement Disorders, Vol. 22, Suppl. 16, 2007

177
Experiences from microrecording during DBS neurosurgery of the
basal ganglia
L.J. Bour, E.M.J. Foncke, M.-F. Contarino, H.D. Speelman,
R. Schuurman (Amsterdam, Netherlands)
Objective: During DBS surgery of the basal ganglia, microrecording
has been performed in addition to the approach with macrostimulation
to rene target localization.
Background: Microrecording in DBS surgery is a matter of debate.
Several drawbacks including increased risk of hemorrhage and increased duration of surgical procedure have been mentioned. Due to the
limited accuracy (about 2 mm) of pre-operative 3D MRI imaging,
microrecording may help to increase the accuracy to the 1 mm range,
and may be useful in DBS surgery with poor macrostimulation reliability.
Methods: The procedure for DBS is a one stage bilateral stereotactic
approach using microrecording and macrostimulation. Over a four year
period, 63 patients for subthalamus nucleus (STN) and 28 patients for
internal Globus Pallidus (GPi) were included. Extracellular single/
multi-unit recordings were performed using a microelectrode with 10
m exposure mounted on a sliding canula. Starting from a remote
position of the MRI-calculated target, electrodes were advanced in
steps of 500 m using Bens gun. Signals were digitized, stored and
analyzed with the Leadpoint.
Results: Three to ve channels were used. Channels with the best
activity were selected for macrostimulation. The dorsal border of the
STN was recognized by a sudden increase in discharge rate characterized by rhythmic bursts of activity with 15 to 25 Hz. Activity of the
substantia nigra, necessary to recognize the ventral STN border, consisted of various patterns. The borders of the GP were clearly observable. To separate the external and internal part of the GP, clues like
discharge rates or the presence of laminae were not always indicative.
The central channel was chosen for the permanent electrode in 46% of
the STN and in 67% of the GP cases. In 85% of the STN cases the
channel with the best multi-unit activity was chosen for the permanent
electrode. The depth of the deepest contact point deviated from the
MRI-calculated target by /- 2 mm.
Conclusions: Microrecording during DBS surgery is useful to improve target localization. Multichannel recording also helps to select
the optimal channel for macrostimulation.
178
Where is the optimal target for STN-DBS? A retrospective analysis
of our 56 cases
T. Agari, T. Matsui, S. Kuramoto, A. Kondou, I. Date (Okayama,
Japan)
Objective: To investigate whether there is a difference about an
appropriate target for the STN-DBS due to each symptom or not.
Background: Deep brain stimulation of the subthalamic nucleus
(STN-DBS) has proven to be a highly effective treatment for major
symptoms (tremor, rigidity, and/or akinesia) in Parkinsons disease.
Regardless of its effectiveness, the optimal site for STN-DBS for each
symptom remains to be accurately dened.
Methods: We retrospectively analyzed location of 99 electrodes in
56 patients with advanced Parkinsons disease, who received STNDBS from April 2003 to November 2006. The patients were classied
into 2 groups: tremor and non-tremor group (rigidity and/or akinesiadominant). Three-dimensional coordinates of active contacts were measured on surgical planning images which are consisted of preoperative
MR images and postoperative computed tomography images. Location
of active contacts were plotted on the basis of AC-PC coordinates.
Results: The active contacts of tremor group were clustered in the
posterodorsal to the subthalamic nucleus while those of non-tremor
group were scattered within the area of the subthalamic nucleus.
Conclusions: Our study revealed that stimulation of dorsal subthalamic nucleus area is effective for tremor control, while stimulation of
STN itself is effective for control of motor uctuation. Therefore
tailor-made planning should be done for STN-DBS.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

179
Deep brain stimulation of subthalamic nucleus for Parkinsons
disease: Anatomical localization of effective contacts
P.-P. Derost, M. Ulla, B. Stephanie, B. Debilly, L. Ouchchane,
J.-J. Lemaire, F. Durif (Clermont-Ferrand, France, Metropolitan)
Objective: We carried out a study in order to explore systematically
effectiveness and anatomical localization of the four contacts of both
electrodes in 30 parkinsonian patients treated by bilateral DBS-STN.
Background: Deep brain stimulation of subthalamic nucleus (DBSSTN) is now a well documented strategy for management of motor
complications in parkinsonian population. However, the precise nature
of the implied anatomical structures remains discussed.
Methods: A direct targeting of STN was used for electrodes implantation. At the three-month follow-up, we systematically explored the
acute motor effects of the four contacts of the electrode on each side to
select the most effective one. Clinical evaluation used subscores of the
UPDRS III (rest tremor, nger taps, tap heel on ground, arm and leg
rigidity). Contact localization was determined by superimposing postoperative magnetic resonance images (MRI) and preoperative MRI
acquired in stereotactic conditions.
Results: Contacts were numbered from 0 for the more ventral one to
3 for the more dorsal one.The contact 2 offered the best clinical
improvement [2 (64%);1 (61%); 3 (55%);0 (45%); p0.0001]. 30.4 %
of contact 2 were localized in dorso-lateral part of STN and 34.4% in
Zona Incerta (ZI). Contacts 1 were placed in 23.5% of case in dorsolateral part of STN and in 9.1% in the ZI whereas only 5% of contacts
3 were situated in dorso-lateral part of STN and 43.4 % in the ZI.
Concerning the deeper contact (contact 0), none of them were localized
in the ZI and 17.4% were in the dorso-lateral part of STN.
Conclusions: Most effective contacts are localized at the interface of
STN and ZI probably due to a cumulative therapeutic effect of those
both structures.
180
PET cerebral glucose metabolism in PD patients after STN-DBS
M.A. Volonte, V. Garibotto, S. Lalli, D. Ceppi, F. Spagnolo,
P. Picozzi, A. Franzin, A. Panzacchi, F. Fazio, G. Comi, D. Perani
(Milan, Italy)
Objective: To evaluate cerebral metabolism after Deep Brain Stimulation of Subthalamic Nucleus (STN-DBS) in patients with Parkinsons disease (PD).
Background: STN-DBS is used to treat advanced PD patients with
severe complications due to long term therapy with levodopa.Despite
the clinical benets,the precise mechanism through which DBS acts is
still not completely understood.Recent PET studies indicate that suppression of abnormal activity is a feature of STN stimulation.
Methods: We studied the cerebral glucose metabolism with 18F-FDG
and PET in 17 subjects (mean age 59 8,4; mean disease duration 13,1
4.6) after STN-DBS (6 months mean, 1 SD) either in switched ON and
OFF stimulation (switched off lasting 12 hours),both conditions in off drug
therapy.All the patients showed a marked benet on motor symptoms after
the procedure (UPDRS III improvement in OFF drug: 36%) with a marked
reduction in levodopa daily intake.
Results: Statistical parametric mapping (SPM) analysis of cerebral metabolism in PD group vs. controls (threshold p0.001)
showed,in ON stimulation,a bilateral increase in the whole sensorimotor cortex,dorsal putamen,ventral striatum and the SMA.In OFF
stimulation,the same pattern of increased cerebral metabolism was
found,plus an increase in the cerebellar vermis and nuclei.Hypometabolism in ON and OFF conditions is seen in the thalamus,caudate
nucleus and globus pallidum and may reect degenerative aspects of
the disease.Hypometabolism is also seen at a cortical level: in the
premotor cortex, inferior parietal lobule,frontal medial and anterior
cingulated cortex and may be the result of deafferentation aspects.In
OFF stim the condition of hypometabolism extends also to dorsolateral frontal cortex.
Conclusions: The hypermetabolism in the sensorimotor cortex,in the
putamen and the hypoactivation of the frontal medial cortex and the

S55

anterior cingulated cortex,are typical features in advanced PD and

indicate that STN-DBS does not correct completely the parkinsonian
state.Despite of it,the direct differences between the two states (ONOFFstim),in the dorsolateral frontal cortex and cerebellar structures,
may indicate that STN-DBS provides modulation of the compensatory
mechanics developed in the advanced phase of PD.
181
The atypical subthalamic nucleus
J. Herzog, M.O. Pinsker, F. Wodarg, A. Morsnowski, G. Deuschl,
J. Volkmann (Kiel, Germany)
Objective: To describe the prevalence and morphology of subthalamic
nuclei (STN) with atypical location in a cohort of patients with Parkinsons
disease (PD) treated by subthalamic deep brain stimulation (STN-DBS).
Background: The preoperative targeting procedure in STN-DBS is
usually based on anatomical mapping of the STN within AC-PC-based
stereotactic coordinate system. In most cases, atlas-based standard
coordinates provide a reasonably good approximation of the patients
individual STN position and result in sufcient reduction of PD symptoms. However, in few patients, due to an atypical STN position,
targeting based on standard coordinates fails to reach the STN and may
cause insufcient stimulation efcacy.
Methods: In three patients (5 STN) out of 228 patients, intraoperative
evaluation by microelectrodes at standard coordinates failed to show
typical neuronal STN discharge and to reduce symptoms by stimulation.
Stereotactic T2W-MRI revealed an atypical anteromedial STN position.
After adjustment of the target according to delineation of the STN within
the MRI, we found characteristic recording of subthalamic activity and
marked symptom reduction. In patients with atypically STN, we retrospectively dened the nucleus border within the T2W-MRI and position of
implanted macroelectrodes. We compared these values to 49 patients (98
STN) with sufcient intraoperative stimulation effect at standard coordinates. Long-term efcacy in both groups was evaluated by percentage
change in UPDRS III at six months follow-up.
Results: The atypical STN showed a more anteromedial position with a
signicant shift of 3.30.8 mm in the anterior and 3.00.9 mm in the
medial direction compared to the standard STN location (P0.05). Electrodes in patients with atypical STN were implanted 2.80.7 mm more
anteriorly and 2.50.6 mm more medially than in patients with standard
STN. Evaluation of percentage change in UPDRS III by STN-DBS revealed no signicant difference between patients with atypical
(60.620.5%) and standard STN (45.426.8%) (P0.05).
Conclusions: In our PD patients operated for STN-DBS, gross
anatomical deviations of the individual STN location from the
standard target were found in 1%. Based on preoperative T2W MRI
and intraoperative microelectrode recording, adjustment of the target point to the atypical STN position leads to a benecial postoperative outcome.
182
Effect of contact site and voltage amplitude of STNDBS on speech
and movement in Parkinsons disease
E. Tripoliti, L. Zinzro, E. Borrell, E. Frost, S. Tisch, I. Martinez,
M. Hariz, P. Limousin (London, United Kingdom)
Objective: To study the role of contact site and amplitude of stimulation on speech and movement, following subthalamic nucleus deep
brain stimulation (STNDBS).
Background: Dysarthria occurs in the majority of Parkinsons disease
(PD) patients at some stage of the disease process. STNDBS is particularly
effective in improving limb movements. However, dysarthria shows a
variable response. Contact site and amplitude of stimulation have been
implicated clinically as factors inuencing speech response. Dissociation
between speech and motor improvement has also been observed.
Methods: This is an acute, double blind within-subjects design
study. Ten patients (mean age 56 years) were assessed 6-12 months
post bilateral STNDBS, unmedicated and stimulated under four
conditions in a random order: inside the STN at low voltage (2V);
inside the STN at high voltage (4V); above the STN at 2V; above

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

the STN at 4V. The site of stimulation was dened based on the
post-operative MRI stereotactic data. The assessment comprised a
recording of the Sentence Intelligibility Test (SIT) and a oneminute monologue using the Computerised Speech Lab. Movement
was assessed using the UPDRS-III. Perceptual and acoustical data
from the SIT and the UPDRS-III were compared using a two-factor
analysis of variance with contact site as factor A with two levels
(inside and outside the nucleus) and voltage amplitude as factor B
with two levels (high-4V and low-2V).
Results: STN stimulation at 4V signicantly reduced speech intelligibility (p.001). There was no signicant effect of site of stimulation on
speech. Examination of individual data (gure1) showed two distinct
groups of patients, one negatively affected by high voltage (6 out of 10
patients) and one not as severely affected. Contrarily STN stimulation at
4V signicantly improved movement as measured by UPDRS-III
(p.002). There was a signicant effect of site of stimulation on UPDRSIII data (p.02) with STN stimulation inside the nucleus being signicantly more effective than outside the nucleus.
Conclusions: The signicant improvement in movement coupled
with signicant deterioration in speech intelligibility when patients are
stimulated inside the nucleus at high voltage warrants case by case
examination of contact site and type of speech involvement.

FIG. 1 (182).

183
Weight gain after DBS STN in Parkinsons disease is related to
electrode placement rather than to the stimulation
L. Novakova, R. Jech, J. Roth, D. Urgosik, F. Ruzicka, E. Ruzicka
(Prague, Czech Republic)
Objective: To evaluate weight changes in patients with advanced
Parkinsons disease (PD) treated with deep brain stimulation of the
subthalamic nucleus (DBS STN) and to verify if they are related to the
placement of electrodes or rather to the stimulation itself.
Background: Weight gain has been reported as one of the most
common side effects of DBS STN in PD. The reasons for weight
changes remain unclear.
Methods: 16 subjects with advanced PD (mean age 56.6, range
42-67yrs) were prospectively evaluated following implantation of electrodes for DBS STN. Control group consisted of 7 subjects with DBS
VIM implanted for essential tremor (mean age 60.3, range 46-74yrs).
Body weight and body mass index (BMI) were measured under standardized conditions on the day of the implantation, and one, two and 12
months later. The stimulation was initiated one month after the surgery.
Results: In contrast to the DBS VIM group, PD patients signicantly
gained weight after DBS STN. The mean weight gain in 12 months
after the implantation was 3.12 kg (-3.6 to 7.5 kg), median 2.35
kg (p0.03). The BMI increased at average by 1.04 kg/m2, median
0.65 kg/m2 (p 0.03). During the rst month after electrodes implantation and prior to the initiation of stimulation we found an average
increase of weight by 1.13 kg (-1.6 to 4.6 kg), median 0.85
(p0.007). In the VIM group, no weight gain was observed.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Conclusions: A signicant weight gain was observed in patients

treated with DBS STN for PD but not in the control group of patients
treated with DBS VIM for essential tremor. Our data suggest that
weight changes observed following DBS may be connected to lesions
caused by placement of electrodes rather then to the stimulation.
184
Bilateral pallidotomy failed to improve generalized dystonia in
Wilsons disease
R. Bhidayasiri, T. Srikijvilaikul, N. Kanjanasut, L. Tuchinda,
S. Lerdlum, S. Kaoroptham (Bangkok, Thailand)
Objective: To evaluate the efcacy and safety of bilateral posteroventral pallidotomy in the patient with Wilsons disease (WD) presenting with intractable dystonia.
Background: WD is an autosomal recessive disorder characterized by an
accumulation of copper in basal ganglia resulting in varied extrapyramidal
symptomatology, including wing-beating tremor, dystonia and parkinsonism. We recently encountered WD patient who presented with generalized
dystonia refractory to conventional therapies.
Methods: A 20-year-old woman presented with a 1-year history of
dystonia of right upper limb. The diagnosis of WD was established on
the basis of K-F ring, ceruplasmin level of 6 mg/dl (N: 20-60), and
24-hr urine copper was 2413 g/L (N: 0-80). The dystonia later became
generalized, most severe in the craniocervical and bilateral upper
extremities region. MRI brain disclosed T2 hypersignal intensities in
bilateral lentiform nuclei. Despite aggressive therapies as well as chelating agents, her dystonia progressed, markedly affecting her speech,
swallowing, the use of both hands, and axial muscles. Therefore, the
decision to perform pallidotomy was made on compassionate ground of
severe generalized dystonia refractory to all conventional treatment.
Dystonia severity was assessed using BFMDRS.
Results: Simultaneous bilateral posteroventral pallidotomies were
performed with macrostimulation for a physiologic conrmation of the
GPi. The patient was evaluated with BFMDRS by an unblinded rater at
1, 30, 60, 90, 120, and 180 days postoperatively. Unfortunately, her
dystonia persisted with no functional improvement. The mean baseline
and follow-up BFMDRS motor scores were 38, 36, 32, 35, 29, and 34
respectively. In addition, the mean baseline, and follow-up BFMDRS
disability scores were 7, 6, 5.5, 6, 6, and 6 respectively, reecting no
signicant improvement.
Conclusions: Bilateral posteroventral pallidotomies failed to improve generalized dystonia in the patient with WD, up to a 6-month
follow-up period. These results are in contrast to previous published
studies of patients with primary dystonia who experience excellent
outcome. Abnormal MRI scans, as in our patient, may be one of poor
prognostic indicators since favorable outcome in dystonia patients
undergoing pallidal procedures depends on normal brain integrity and
anatomic substrates.
185
Parkinsons disease in developing countries transfer from exclusively conservative treatment to deep brain stimulation. Successes,
difculties, preliminary experience
M. Ivanov, C. Ivanov, L. Cucos (Iasi, Romania)
Objective: PD is recognized as one of the most common neurological
disorders, affecting approximately 1% of individuals older than 60
years. Cardinal features include resting tremor, rigidity, bradykinesia,
and postural instability. Bilateral deep brain stimulation in advanced
cases with Parkinson disease with predominant bradikinesia, with diskinesia induced by dopaminergic therapy is the rst choice therapeutic
alternative. Surgical approaches to the treatment of Parkinsons disease
(PD) have developed primarily in response to the failure of medical
therapies to provide long-term relief from the disabling motor symptoms of the disease. The introduction of levodopa (L-DOPA) in the
mid-1960s, an event that revolutionized the medical management of
PD, sets the focal point around which the history of movement disorder
surgery may be examined. In developing countries there are still serious
difculties to implement in current practice the latest achievements in

POSTER SESSION I, TUESDAY, JUNE 5, 2007

treatment of Parkinson disease. Most developed countries tended to
have incidence rates between 12 and 20 cases per 100,000 per year. In
Romania, with population of 23 million people an incidence of 27603600 new cases a year is expected.
Methods: The retrospective study included a number of 118 patients
with Parkinsons disease admitted to the University Hospital
Sf.Treime from Iasi, Romania in a period 01.01.2004-31.12.2006
with age between 39 and 84 years. The patients were assessed using
United Parkinsonian Disease Rating Scale, Schwalb and England Scale
and Hohen and Yahr Score.
Results: Twelve of admitted patients developed drug-resistance or
didnt tolerate medical therapy and represent good candidates for
neurosurgical treatment (DBS) but due to nancial limitations only two
of them received DBS in a neurosurgical department from other European country (Germany, France).
Conclusions: DBS led to an improvement of clinical benet in
patients who received it. Until now the DBS in Romania is a new
technique that requires a trained team, complex equipment and governmental nancial support. The authors will share the preliminary
experience of this high technique implementation in one of the major
hospitals from Romania Questions: How to nd a regional adapted
solution for patients with drug-resistance, candidates for DBS?

186
Long term superiority of bilateral STN stimulation over unilateral
pallidotomy, four years follow-up of a RCT
R.A. Esselink, R.M. Bie, R.J. Haan, R.P. Schuurman, A.D. Bosch,
J.D. Speelman (Nijmegen, Netherlands)
Objective: 1) To compare the efcacy of bilateral subthalamic nucleus
(STN) stimulation and unilateral pallidotomy in Parkinsons disease (PD)
after four years follow-up of a randomized observer-blind multi center trial
2) To describe the clinical course in the treatment arms.
Background: Patients with advanced PD often suffer from response
uctuations and dyskinesias. Unilateral pallidotomy and bilateral STN
stimulation can both improve PD symptoms in the off phase and dyskinesias in the on phase. We did the rst randomized trial that compared
unilateral pallidotomy and bilateral STN stimulation. At six months follow-up STN stimulation is more effective than pallidotomy, and the
number of adverse effects was approximately the same for both operations.
Methods: Thirty-four patients with advanced PD were randomly
assigned to have unilateral pallidotomy or bilateral STN stimulation.
Outcome measures were the change from baseline to four years in the
motor part of the Unied Parkinsons Disease Rating Scale (UPDRS 3)
in the off phase and on phase, dyskinesias (Clinical Dyskinesia Rating
Scale), functional status (UPDRS 2), and adverse effects. For patients
allocated to unilateral pallidotomy the protocol allowed STN stimulation at least six months after pallidotomy.
Results: After four-years follow-up off phase UPDRS 3 score improved from 46.5 to 34 in the pallidotomy group and from 51.5 to 28
in the STN group (p0.04). The on phase UPDRS 3 improved significantly in favor of the STN patients. One patient in each group had a
major adverse effect. Moderate and mild adverse effects were approximately the same for both operations. Three pallidotomy patients had
bilateral STN stimulation during follow-up. The clinical course in the
treatment arms demonstrates that after initial improvement at one year,
off phase motor symptoms worsen in the STN group at four years after
surgery, though there is still benet. The pallidotomy group shows the
same pattern, but scores are in the preoperative range four years after
surgery. The scores of the three patients with STN stimulation after
pallidotomy remained stable between one and four years follow-up.
Conclusions: We found that bilateral STN stimulation is superior to
unilateral pallidotomy in the treatment of advanced PD at long term
follow-up.

S57

187
Objective monitoring of tremor and bradykinesia during deep
brain stimulation of STN for Parkinsons disease: A pilot study
S. Papapetropoulos, J. Jagid, C. Sengun, C. Singer, B.V. Gallo
(Miami, Florida, USA)
Objective: We conducted a pilot study to evaluate intra-operative
outcomes of unilateral macro-stimulation of the STN-DBS in Parkinsons (PD) patients using an electronic symptom registration system
(CATSYS 2000 System).
Background: The use of non-invasive, simple, objective registration
techniques during the different treatment stages combined with the
traditional subjective rating scales may aid in optimization and standardization of DBS procedures in PD. Such techniques may be applicable in everyday practice and in clinical trial settings and may help
reduce the bias introduced by subjective measures.
Methods: We studied 12 consecutive PD patients that received
unilateral STN-DBS implants and 10 male control subjects free of
neurological decits using a simple portable MS Windows-based
system with 2 sensors: a Tremor Pen and a touch recording plate.
PD patients were evaluated in the pre-operative suite of our institution 12 hours after their last dose of antiparkinsonian medications
during off state and intra-operatively for rest, postural tremor
intensity and frequency of nger tapping. Comparisons between
pre- and post-macrostimulation were made using ANOVA for repeated measures.
Results: Results revealed excellent test-retest reliability for postural tremor in control subjects. Electronic rest tremor registration
revealed a mean improvement of x12.5 in tremor intensity measurements in the stimulated/contralateral side (p0.002). An overall
x3.8 improvement was registered on the non-stimulated/ipsilateral
side. Signicant improvements following STN-DBS were also recorded for postural tremor and frequency of nger tapping. A
typical pre- (A) and intra-operative (B) rest tremor registration
reading (subject PD3) is presented in Figure 1. The tremor intensity
pre-operatively was 0.57 m/s2. Postoperatively this improved to
0.13 m/s2. The pre-operative center frequency was 4.9 Hz and the
post-operative center frequency was 4.3 Hz. The overall tremor
intensity is presented in a relative scale.
Conclusions: Using a non-invasive, simple and sensitive electronic
recording method of intra-operative motor symptom registration we
were able to supplement clinical observation by objectively quantifying
the characteristics of tremor and nger tapping in response to STNDBS macrostimulation.

FIG. 1 (187).

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

188
Correction of esthetic complications of facial nerve pathology with
the use of Botulinum toxin Type A (DYSPORT)
O.R. Orlova, A.I. Nerobeev, S.V. Surovykh, M.O. Sokolova (Moscow,
Russian Federation)
Objective: Facial nerve neuropathy (FNN) (Bells palsy) is a polyetiologic disease, with infection-allergic, compression-ischemic and traumatic
factors being the principal causes of facial nerve lesion. Currently, the
number of patients with facial nerve lesions has increased due to an
increase in the number of house and gunshot traumas, oncological diseases, pathology of parotid salivary gland, and complications after plastic
and reconstructive surgery and cosmetic manipulations. FNN is characterized by loss of both voluntary and involuntary motions of the mimetic
muscles, loss of reexes, muscular atony and degenerative atrophy of
muscles. Outwardly, this manifests itself in facial asymmetry, which can
arise both in facial expression motions and in rest. Patients suffer from
serious physical and functional disturbances, which reduce the quality of
life. Aesthetic perception of face is changed, which results in psychological
trauma and social disadaptation. FNN should be treated both conservatively and surgically, depending on its ethiology, severity and duration.
However, even in case of successful treatment, it is difcult to achieve the
complete functional recovery of mimetic muscles, and facial asymmetry
can persist. According to clinical study and electroneuromyographic
(ENMG) data, the healthy side of the face is also affected. Contractures
and synkineses are formed in paretic muscles; hypertone of mimetic
muscles is formed on the healthy side, which aggravates the asymmetry.
Hence, a correction of lesions should be performed both on the affected
and healthy sides. Botulinum toxin is injected in hypertonic muscles of
healthy side and in the contracture and synkinesis zones on the affected
side. Denervation and lack of motion lead to partial atrophy of muscles and
to retraction of soft tissues. In hypertonic zones, mimetic wrinkles become
accentuated, nasolabial sulcus becomes more pronounced. These
changes accentuate facial asymmetry, thus, aggravating the esthetic
defect. Hence, after carrying out the treatment, llers can be used for
lling wrinkles and tattooing can be used for alignment of lip contours,
as means of esthetic correction. Thus, in patients with pathology of
facial nerve, combination of conservative and surgical treatment with
the use of botulinum toxin preparations on the affected and healthy
side, llers, and tattooing lead to improvement of the functional and
estetic outcomes of the treatment.

of the SF-36 quality of life scale were substantially improved. She demonstrated increased latency to symptom onset after standing, slower build up of
tremor severity, and improved tolerance for prolonged standing ( 3 minutes).
Her EMG evaluation showed 50% decreased amplitude of EMG activity in
the forearm muscles with lesser change in the leg muscles. The residual
oscillatory activity continued to exhibit strong coherence at 15 Hz.
Conclusions: Vim DBS is a novel and promising treatment strategy in
drug-refractory, disabling OT. Further studies using chronic stimulation to
disrupt the abnormal oscillatory activity in this disorder are warranted.
190
Bilateral benet from unilateral deep brain stimulation of the
subthalamic nucleus in PD at 1 year: A prospective analysis of 34
consecutive cases
H.C. Walker, S. Guthrie, D. Wang, B.L. Guthrie, R.L. Watts
(Birmingham, Alabama, USA)
Objective: To quantify the benet of unilateral STN DBS on contralateral, ipsilateral, and axial symptoms of advanced Parkinsons
disease.
Background: DBS of the STN has is an effective treatment for
advanced PD, but controversy exists about the relative merits of

189
Orthostatic tremor improvement after ventrointermedial deep
brain stimulation implantation: Clinical and electrophysiologic observations at 3 months
A.J. Espay, A.P. Duker, E.T. Barrett, J. Devoto, M. Gartner, N. Burton,
H.A. Miranda, G.T. Mandybur, F.J. Revilla (Cincinnati, Ohio, USA)
Objective: To report the rst patient with medication-refractory OT successfully treated with ventrointermedial deep brain stimulation (Vim DBS).
Background: Orthostatic tremor (OT) is a disabling movement disorder
presumed to arise from the activity of a single oscillatory generator in the
posterior fossa. Medical therapy often yields disappointing outcomes.
Methods: Review of clinical and electrophysiological data.
Results: This 73-year-old woman had a 30-year slowly-progressive history
of unsteadiness upon standing, initially relieved by walking and sitting. She
experienced gradual shortening, from minutes to seconds, of the latency to
unsteadiness after rising. Tremor of the legs and arms became prominent when
upright but persisted, to a lesser extent, while sitting. Clonazepam (10 mg/day),
valproate (500 mg/day), and primidone (225 mg/day) were tried to no avail.
She eventually was unable to arise from a chair or walk without assistance, and
could not tolerate the stationary upright position longer than 30 seconds.
Surface electromyography (EMG) revealed a narrow 15-Hz oscillatory peak in
all active muscles of the upper and lower limbs, with strong coherence
between them. She underwent bilateral simultaneous Vim DBS implantation
without complications. At 3 months after initial programming (left: 3.3 V;
pulse width, 90 ms; rate, 185 Hz; right: 3.0 V; pulse width, 60 ms; rate, 185
Hz), she was able to stand and walk unassisted for at least one block, and no
longer needed help for bathing and dressing. Both physical and mental scores

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (190).

POSTER SESSION I, TUESDAY, JUNE 5, 2007

bilateral versus unilateral stimulation. Most centers implant bilateral
STN electrodes initially in one surgery, however, patients undergoing unilateral stimulation may achieve sustained motor benet and
be spared potential complications of bilateral implantation.
Methods: Thirty-four patients received unilateral DBS of the STN.
UPDRS scores and timed tasks of motor function were measured in the
practically dened off state (off medications for 12 hours) at the preoperative baseline and at 3, 6, and 12 months postoperatively. Changes over
time in total motor function and in contralateral, ipsilateral, and midline
motor subscores were analyzed statistically.
Results: UPDRS motor scores improved signicantly at 3, 6, and 12
months relative to baseline (p 0.001). There was improvement in the
contralateral UPDRS subscore (p 0.001), and although contralateral
benet was quantitatively larger on all outcome measures, ipsilateral
benet was present at 3 and 6 months on the UPDRS subscore (p 0.001
and p 0.01, respectively). A trend towards ipsilateral benet was present
at 12 months, however, the effect was not statistically signicant. Two of
the timed tasks did show statistically signicant ipsilateral benet at 12
months (p 0.001 and p 0.01, respectively).
Conclusions: Considering the sustained effects of unilateral STN
neurostimulation on contralateral, ipsilateral, and axial symptoms, unilateral DBS followed by a contralateral procedure later (if necessary) is
a reasonable option for patients with advanced PD.
191
Treatment of sporadic dystonia-parkinsonism by bifocal pallidal
and subthalamic deep brain stimulation
J.C. Woehrle, C. Blahak, H.-H. Capelle, W. Fogel, H. Baezner,
J.K. Krauss (Hannover, Germany)
Objective: We report the results of chronic deep brain stimulation in a
patient with sporadic dystonia-parkinsonism, using a multifocal approach
and eventually stimulating the subthalamic nucleus (STN) and posteroventral lateral globus pallidus internus (GPi), bilaterally.
Background: Rapid onset dystonia parkinsonism (RDP) is characterized
by development of axial and appendicular dystonia within hours to few
weeks accompanied by rigidity, postural disturbance and bradykinesia.
RDP occurs as autosomal dominant or sporadic disease.
Methods: We report a 39-year-old man with sporadic RDP. Therapy
with L-dopa lead to a partial improvement of the parkinsonian features; but
over time, motor uctuations developed and dystonia was never satisfactorily controlled. He underwent bifocal bilateral implantation of quadripolar DBS electrodes in the STN and GPi in one operative session. Postoperatively, the electrodes were initially externalized to control the effect of
stimulation of the targets.
Results: Initially, stimulation of STN improved both parkinsonism and
dystonia. Gpi stimulation alone reduced the dystonia, but additional dopaminergic medication would have been required for control of tremor,
bradykinesia and rigidity, which was not appreciated by the patient. Therefore, the STN electrodes were connected to a dual-channel pacemaker for
chronic DBS. Parkinsonian symptoms improved favorably. Within 2
months after the operation severe dystonia recurred. Therefore, the Gpi
electrodes were connected to a second dual-channel pacemaker. With this
bifocal approach of bilateral STN and Gpi DBS, tremor, rigidity, bradykinesia and dystonia could be well controlled in our patient at a follow-up
of 12 months (at 12 months follow-up: BFM 67% improvement (motor
score); UPDRS III 39% improvement, on medication/on stimulation).
Conclusions: GPi DBS might be necessary in addition to STN DBS
in RDP to achieve adequate control of dystonia. In contrast to PD, in
RPD with the presence of marked dystonia early on, this approach may
even be the rst choice.
192
Bilateral pallidal chronic deep brain stimulation improves quality
of life in segmental dystonia
H.-H. Capelle, C. Blahak, H. Baezner, K. Kekelia, R. Weigel,
J.C. Woehrle, J.K. Krauss (Hannover, Germany)
Objective: To evaluate the effect of pallidal deep brain stimulation
(DBS) on quality of life in patients with segmental dystonia.

S59

Background: DBS of the globus pallidus internus (GPi) has been reported as a reversible and modiable surgical treatment to improve both
motor symptoms and qualtiy of life in primary generalized dystonia. In
patients with segmental dystonia reports on the effectiveness of pallidal
DBS on quality of life are sparse. We prospectively evaluated the effect of
pallidal DBS on quality of life using the SF-36 questionnaire.
Methods: Ten patients (mean age 57.4 years) suffering from idiopathic segmental dystonia were treated with CT-guided stereotactic
bilateral GPi DBS. In all patients indication for DBS was insufcient
control of dystonic symptoms by oral medication including anticholinergics and BTX injections. Assessment included MMSE, Hamilton
depression scale, BFM and the SF-36 preoperatively and at follow-ups
at a mean of 7.5 months (range 4-10) and 17 months (range 12-20).
Results: All 10 patients experienced sustained benet from pallidal
GPi as indicated by a mean 60% decrease of BFM scores. The total SF
36 score, the physical and the mental health subscores improved
signicantly postoperatively by 40% at the rst follow-up and by 51%
at the second one. Out of the eight dimensions of the SF-36, role
limitations due to physical problems, social functioning and general
perception of health subscores showed the most improvement, whereas
role limitations due to emotional problems and bodily pain subscores
were not signicantly changed.
Conclusions: Regarding the limited effectiveness of medical treatment, the improvement of quality of life by pallidal stimulation underlines the role of chronic DBS as a valuable treatment option in patients
with primary segmental dystonia.
193
GPi-DBS in Huntingtons disease: Results on motor function and
cognition in a 72-year-old case
A. Fasano, P. Mazzone, C. Piano, G. Loria, D. Quaranta,
A.R. Bentivoglio (Rome, Italy)
Objective: Huntingtons disease (HD) produces debilitating motor
abnormalities that are poorly responsive to medical therapy.
Background: Deep brain stimulation (DBS) of the posteroventral
globus pallidus internus (GPi) may offer a treatment option for patients
with diskinetic phenotype and minimal cognitive impairment, but its
role in the management of HD remains unclear since to date only two
cases have been reported.
Methods: We report on a 72-year-old man with genetically conrmed HD who developed motor symptoms at age 55. DBS leads were
bilaterally implanted, under stereotactic guidance, into the posteroventral GPi (gure); the procedure was well tolerated.
Results: Disease progression and symptom control were assessed at
regular postoperative intervals. In the rst published HD patient undergone to GPi-DBS, Moro and colleagues stated that both stimulations
at 40 and 130Hz improve chorea although stimulation at 130Hz slightly
worsens bradykinesia overall (1). We conrm this specic effect of
frequency on motor function also when maintaining constant the total
electrical energy delivered (TEED) (2). This observation conrms that
the choice of frequency is crucial for the effect of pallidal stimulation
in HD patient: 130 Hz stimulation caused a rapid amelioration of
chorea producing freezing of gait whereas 40 Hz stimulation was
associated with a less evident benet while the ability to walk was
preserved so was possible the withdrawal of tetrabenazine. Moreover,
we documented cervical extensors and paraspinal muscles hypotonia
when stimulating ventral contacts. The cognitive prole showed a
progressive deterioration, with an extension of decit from the mainly
dysexecutive alterations at baseline to a more diffused cognitive deterioration with relatively preserved working memory, ideomotor and
oro-facial praxis, and naming abilities.
Conclusions: In a patient with HD GPi-DBS leads to the marked
improvement of chorea but it might be associated with side effects: the
worsening of parkinsonism (also when using 40 Hz stimulation), the
occurrence of head drop (ameliorable stimulating dorsal contacts); the
worsening of cognitive function is difcult to say to what extent might
be ascribed to DBS or to progression of the disease.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

195
Single unit and local eld potential recordings from human STN
during reach-to-grasp movements
M. Poetter, F. Steigerwald, J. Herzog, R. Wenzelburger, M. Pinsker,
G. Deuschl, J. Volkmann (Kiel, Germany)

FIG. 1 (193).

194
Neuropsychiatric therapy in the follow-up of PD patients after
STN-DBS
M. Zibetti, M. Pesare, A. Cinquepalmi, M. Rosso, L. Castelli,
B. Bergamasco, M. Lanotte, L. Lopiano (Torino, Italy)
Objective: The aim of the present study is to characterize the timecourse of pharmacological therapy modications for neuropsychiatric
disturbances in PD patients treated with STN-DBS.
Background: A wide range of neuropsychiatric (NP) disturbances commonly occurs in patients with Parkinsons disease (PD). NP disturbances
contribute considerably to reduced quality of life and distress for the care
giver. Deep-brain stimulation of the subthalamic nucleus (STN-DBS) is an
effective treatment for advanced PD. Although the effectiveness of the
procedure in the treatment of motor symptoms is accepted, its effects on
non-motor symptoms is less clear. Several studies of neuro-behavioural
outcome of STN-DBS for PD exist in the literature. A complementary
approach is to look at the pharmacological therapy for NP conditions of
PD patients treated with STN-DBS. Such therapy can be considered an
indirect measure of an underlying NP condition.
Methods: Forty-eight consecutive parkinsonian patients treated with
STN-DBS were assessed. The evaluation was performed preoperatively
and included postoperative follow-up evaluations at four, twelve and
thirty-six months. The dose and type of NP drugs (benzodiazepines,
antidepressants, antipsychotics) used by each patient at every visit was
recorded. Effects on parkinsonian symptoms were evaluated using the
Unied Parkinsons Disease Rating Scale (UPDRS). A neuropsychologist
also evaluated the patients at every visit assessing depression and anxiety
with appropriate rating scales (Beck depression inventory, STAIX1STAIX2). Levodopa equivalence daily dose (LEDD) was calculated.
Results: The use of benzodiazepines dropped sharply after surgery,
then gradually returned to preoperative values at one and three years
follow-ups. The use of antidepressants increased gradually over time at
each follow-up visit. The use of atypical neuroleptic drugs also gradually increased with time, though less markedly than antidepressants.
No correlations were found between neuropsyciatric drugs consumption, motor outcome (UPDRS), neuropsychological rating scales and
the degree of change of dopaminergic medication.
Conclusions: Our ndings supply information about the post-operative time-course of therapy used to treat NP symptoms in advanced PD
patients after STN stimulation.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To examine movementrelated single unit and ensemble

activity of the subthalamic nucleus (STN) during reach-to-grasp movements in Parkinson disease (PD).
Background: The function of the basal ganglia in the control of human
hand movements has mostly been studied by indirect methods, such as
functional neuroimaging. Implantation of STN electrodes in PD patients
represents a unique opportunity to record neuronal activity in relation to
the different phases of externally cued reach-to-grasp movements.
Methods: 7 PD patients (4 men, 3 women, mean age 58.6SD, 7.9
years) underwent microelectrode recordings (Ben-Gun) during therapeutic insertion of electrodes into the dorsolateral STN. Signals were
amplied (gain 10.000), bandpass ltered to separate single unit activity (SUA, 300Hz-10kHz) from local eld potentials (LFP, 3Hz-100Hz)
and digitized. The motor task started with a warning cue to prepare for
the movement followed by a directional cue for motor planning and the
nal go cue at variable interstimulus intervals (2-3sec). The patients
were instructed to initiate a right-handed reaching movement to the
target and to press a button between thumb and index nger (10 trials).
The movement was monitored by an ultrasound motion recording
system with markers xed at the shoulder, elbow, wrist and the tip of
thumb and index nger (40Hz) along with EMG of the rst dorsal
interosseus and the deltoid muscle (gain 100, Filter 30Hz-1kHz). SUA
(identied by using standard spike sorting algorithms) and LFP were
analyzed by calculating peristimulus time histograms or averaging on
the cues and different movement phases.
Results: Movement related SUA anf LFP changes were prominent in
the dorsolateral (motor) region of the STN during motor preparation
and execution. Most neurons were active during the reaching phase of
the reach-to-grasp-movement, but individual neurons exhibited activity
only during the grasp phase.
Conclusions: Neuronal activity during naturally occurring movements can be recorded from human subthalamic nucleus during stereotactic surgery demonstrating the feasibility to study the basic neurophysiology of human motor control.
196
Holmes tremor caused by midbrain cavernomas
J. Zhong, S.-T. Li (Shanghai, China)
Objective: In this paper, a patient with Holmes tremor who harbored
a cavernoma in the anterior midbrain region was presented.
Background: Holmes tremor is a combination of rest, postural and
kinetic tremors, which follows different lesions of the brainstem/cerebellum and thalamus.
Methods: This 42-year-old woman had suffered tremor for 10 years.
The tremor involved her left shoulder and the proximal and distal limb,
which was exacerbated by any attempted movement and became
grossly uncontrollable. She had been treated with medication of Ldopa. However, the symptom deteriorated continuously and nally she
could not use her left arm due to the disabling tremor. Despite the risk
of neurological impairment related to the location of such lesions
within the brainstem, a total resection of cavernoma without damage to
the surrounding vital structures is possible, as there is always a hemosiderinstained rim between the nidus and the brain parenchyma. To
access the target, a frontotemporal transsylvian route via an orbitozygomatic craniotomy was selected. Through a longitudinal incision
between the frontopontine bers and the pyramidal tracts in the peduncle, the cavernoma was removed en bloc.
Results: The tremor symptom completely disappeared after this
ventral mesencephalic cavernoma was surgically removed.
Conclusions: The authors data revealed that surgery is the rst
choice to treat the Holmes tremor arose from midbrain cavernoma, as
long as a fairly safe entry zone on the mesencephalon is accessible.

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for at least 12 hours (Off state). Differences between groups were

statistically evaluated using the Wilcoxon Signed Rank test.
Results: All patients improved their UPDRS scores as compared to
baseline. Patients without BVH before surgery showed a total UPDRS
average improvement of 48%; subscore analysis revealed a mild worsening of part I scores (-10%) and solid improvement of part II (48%) and part
III subscores (52%). In patients with history of BVH, total UPDRS scores
improved on average 35%; they showed an improvement of UPDRS-I
(14%), part II (31%) and part III subscores (39%). Statistical analysis
revealed that the only signicant difference among the two groups was in
the UPDRS part II subscores (P0.015, Wilcoxon).
Conclusions: History of BVH is associated with a less dramatic
motor response to bilateral STN DBS. In addition, subjective perception of improvement, as evaluated by the UPDRS part II, is signicantly less positive in BVH patients. Interestingly, mentation, mood
and behavior subscores (UPDRS part I) showed a mild improvement,
suggesting that cognitive deterioration may not be responsible for the
difference in ADL subscores. Further studies are needed to conrm and
clarify these issues.
198
Movement Disorder surgery: Experience of single surgeon
S. Peker, Y. Akgun, D. Kaya, U. Isik (Istanbul, Turkey)

FIG. 1 (196).
197
Bilateral STN DBS in PD patients with history of benign visual
hallucinations
C.E. Martin, C. Chau, R. Alterman, M. Tagliati (New York, New
York, USA)
Objective: To evaluate the outcome predicting value of benign visual
hallucinations (BVH) in patients with advanced Parkinsons disease
(PD) undergoing bilateral Deep Brain Stimulation (DBS) in the subthalamic nucleus (STN).
Background: Bilateral STN DBS is commonly used to treat advanced
PD, with dramatic improvement of disabling motor symptoms. Dementia
is considered a poor outcome predictor for DBS therapy. Although BVH
have been associated with dementia in PD, they are not considered an
absolute contraindication for DBS. The outcome predicting value of BVH
on STN DBS results in PD patients is currently unknown.
Methods: We retrospectively analyzed STN DBS results obtained
from 20 patients, 10 with history of BVH prior to DBS surgery and 10
age and severity-matched PD patients who never experienced BVH.
They were all bilaterally implanted by a single neurosurgeon (RA). The
Unied Parkinson Disease Rating Scale (UPDRS) was used before and
at yearly intervals after surgery to evaluate DBS results. Motor UPDRS
(part III) was performed after withholding dopaminergic medications

Objective: The aim of this poster is to report our experience on

Movement Disorder surgery.
Background: Surgery for Movement Disorders has been performed
in our institution for 7 years.
Methods: Since January 2000, 34 patients with different Movement
Disorders have been operated by single surgeon. Age of the patients were
between 6 and 72 (20 female, 14 male). There were 24 patients with
Parkinsons disease, 7 patients with secondary distonia, 2 patients with
primary dystonia and one patient with rubral tremor. Surgical procedures
for these patients were; 15 RF thalamotomies, 5 RF pallidotomies, 5 STN
DBS implantation, 4 pallidal DBS implantation and one VIM DBS implantation. All patients were preoperatively and postoperatively evaluated
by a neurosurgeon and neurologist. Available scales were used in all
patients for evaluation of the results of surgery.
Results: The rate of tremor disappearence in the patients which had
thalamotomy is 95%. In all patients with STN DBS implantation for
Parkinsons disease demonstrated improvement in on and off scales.
Rubral tremor is almost totally disappeared after DBS implantation.
Primary dystonias responded well to pallidal DBS implantation. The
success rate in pallidal DBS for secondary dystonia is 50%. There were
no mortality in this series. There were no neurological complication
after surgery. One patient with pallidal DBS implantation demonstrated
an infection of extension cables over the mastoid. This patient was
treated with antibiotics and the infection was resolved without need of
system removal.
Conclusions: This small series demonstrated that, different type of
Movement Disorders can be treated by surgery with acceptable morbidity levels.
199
Effect on the quality of life of patients with Parkinsons disease by
subthalamic stimulation, using subjective evaluation of Japanese
PDQ-39
T. Matsui, S. Kojima, A. Kondo, S. Kuramoto, T. Agari, I. Date,
T. Hayashi, K. Abe (Takasago, Hyogo, Japan)
Objective: To examine effects of subthalamic deep brain stimulation
(STN-DBS) for patients with Parkinsons disease (PD), using Japanese
PDQ-39.
Background: STN-DBS has proven to be highly effective treatment for motor uctuation and dyskinesias in advanced PD. Effects
of STN-DBS have been reported objectively with clinical rating
scales (UPDRS) by clinicians or medical staffs, but not subjectively.
Methods: Fourteen patients (9 men and 5 women) were enrolled
in this study. The patients evaluated their lives by themselves,
applying PDQ-39 scale under best medication before surgery and

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

under best medication and stimulation 3 or 12 months after surgery.

Each item of PDQ-39 was scored from 0 to 4, i.e. full score was 156.
Neurological status was also evaluated pre- and postoperatively
with UPDRS by a neurologist who didnt participate in the patient
care. Patients were evaluated in the best and the worst status during
a day.
Results: UPDRS part III (motor symptoms) preoperative and postoperative best scores were 21.1 and 16.5, respectively. Preoperative
and postoperative worst scores were 44.0 and 21.9, respectively. UPDRS part II (ADL) preoperative and postoperative scores were 8.0/21.8
and 5.9/11.5 (best/worst). In all patients but two, PDQ-39 total scores
were decreased. Preoperative and postoperative scores were 73.1 to
34.9, respectively. In all 8 subcategories, scores decreased 31.9 to
77.8% of preoperative scores.
Conclusions: Both subjectively and objectively, the STN-DBS improved motor symptoms and quality of life of patients with PD.
200
Predictors of neuropsychological and psychosocial outcome one
year after STN DBS in Parkinsons disease
H. Smeding, H.M. Huizenga, J.D. Speelman, P.R. Schuurman,
B. Schmand (Amsterdam, Netherlands)
Objective: To nd predictors of cognitive decline and psychosocial
outcome one year after bilateral subthalamic nucleus stimulation (STN
DBS) in Parkinsons disease (PD).
Methods: A total of 101 STN patients were evaluated before and 12
months after surgery. A control group 40 PD patients were also
evaluated to control for effects of repeated testing and disease progression. At baseline and at follow-up we administered a comprehensive
neuropsychological assessment. We determined individual cognitive
decline, and individual changes in mood and quality of life using a new
statistical method that controls for multiple comparisons. We analyzed
possible predictors of the cognitive and psychosocial outcome such as
age, levodopa response, preoperative mental status, executive dysfunctions and prior stereotactic surgery.
Results: Twelve months after surgery, 37 out of the 101 STN
patients showed a prole of cognitive decline not seen in the control
group. 15 STN patients showed an improvement in mood and 15
showed worsening of mood. 30 patients of the STN group showed
an increase in quality of life. Objective cognitive decline correlated
strongly with subjective cognitive complaints of both the patients
and the relatives, but not with decline or improvement in mood,
quality of life, nor with decrease in medication. A good response to
levodopa medication at baseline predicted an increase in quality of
life at 12 months, a low levodopa response and low scores on
speeded tests at baseline predicted cognitive decline.
Conclusions: STN DBS leads to an improvement of quality of life in
30% of the patients, but to cognitive decline in about 40% of patients.
Levodopa responsiveness and mental speed at baseline are predictors of
postoperative outcome.
201

yearly intervals in four clinical states: 1) Off medications - stimulators Off (Off/Off); 2) Off medications - stimulators On; 3) On
medications - stimulators Off; and 4) On medications - stimulators
On. UPDRS-III scores obtained at baseline Off statewere compared
with those obtained Off/Off after surgery.
Results: The average percent change of UPDRS-III Off/Offscores
was virtually unmodied up to ve years follow-up. At one year
(n19) the average percentage change from baseline was 0.037.
The average changes recorded for the following years were 0.023
percent at two years (n18); 0.930 percent at three years (n13);
1.027 percent at four years (n13); and 3.2 19 at ve years (n5).
Conclusions: Our data show that untreated PD motor scores do
not worsen over time in patients undergoing STN DBS, suggesting
that there is no progression of disease severity. These results could
be explained either by a natural stabilization of PD progression
after many years or neuroprotective properties of STN DBS. However, possible study design artifacts, including insufcient stimulation washout times, could have affected these data.
202
Role of subthalamic deep brain stimulation for levodopa-induced
dyskinesia in Parkinsons disease
A. Umemura, T. Toyoda, K. Yamamoto, M. Mizuguchi, F. Ishii,
T. Yamanaka, K. Yamada (Nagoya, Japan)
Objective: Subthalamic deep brain stimulation (STN DBS) for
Parkinsons disease (PD) is especially effective in patients with
motor complications from levodopa such as uctuations and dyskinesia. We studied the role of STN DBS for levodopa-induced
dyskinesia in PD.
Methods: We retrospectively reviewed the clinical course of nine
patients who suffered from signicant levodopa-induced dyskinesia
and underwent bilateral STN DBS. Patients whose preoperative
dyskinesia score (the sum of UPDRS IV item 32 and 33) was more
than four points, were enrolled in this study. All patients were
female and age at surgery ranged from 48 to 82 years.
Results: In all patients, bilateral STN DBS yielded marked improvement in the motor scores in the medication-off state and in the
dyskinesia/uctuation score. In addition, dopaminergic medication
could be signicantly reduced after surgery. Levodopa could be
completely discontinued in seven patients. In the follow-up period,
a small dosage of dopaminergic medication was resumed in six of
these patients, however the dyskinesia score was further improved
in most patients.
Conclusions: STN DBS is especially effective in PD with signicant
levodopa-induced dyskinesia. STN DBS seems to improve levodopainduced dyskinesia by reducing the need for dopaminergic medication
in the early stage after surgery, and by the direct effect of stimulation
in the chronic stage.
203

Long-term lack of motor symptom progression in Parkinsons

disease patients with bilateral STN deep brain stimulation
C.E. Martin, R. Alterman, M. Tagliati (New York, New York, USA)

Low rate of complications with unilateral STN-DBS in advanced

Parkinsons disease
S. Papapetropoulos, J. Jagid, N. Ahmad, J. Zitser, C. Sengun,
C. Singer, B.V. Gallo (Miami, Florida, USA)

Objective: To evaluate the long-term progression of motor symptoms in Parkinsons disease (PD) patients treated with subthalamic
nucleus (STN) deep brain stimulation (DBS).
Background: STN DBS improves motor function in PD, but the
duration of benets is still poorly dened. In particular, the effects of
STN DBS over the natural progression of PD and its possible neuroprotective function are debated.
Methods: We retrospectively analyzed data from 48 PD patients
implanted with bilateral STN DBS. Clinical records at baseline and
at several yearly intervals after the implant were reviewed. Preoperative UPDRS scales were performed after withholding medications for at least 12 hours (Off state) and after taking the usual dose
of levodopa (On state). Postoperative evaluations were completed at

Objective: To assess the rate of intra- and post-operative deep brain

stimulation (DBS) related-complications in Parkinsons disease patients with unilateral subthalamic (STN) nucleus DBS at a single
center.
Background: STN DBS therapy was developed as an alternative
method to ablative surgery, providing reversible therapy with a
lower rate of complications. Although hardware and software advances and experience have reduced the rate of adverse events, DBS
surgery still carries a signicant risk of transient and permanent
complications. Intracranial hemorrhage occurring during microelectrode-guided implantation, infection, malfunction and lead migration or fracture may increase patient morbidity and mortality should
be considered when evaluating the risk/benet ratio of this therapy.

Movement Disorders, Vol. 22, Suppl. 16, 2007

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Methods: Ninety consecutive patients (mean age 628.6, mean
disease duration 11.76.1, mean on UPDRSIII 20.29.2, mean
off UPDRS III 41.211.5) operated for DBS in our department
from 2/2003 to 9/2006 were retrospectively analyzed to document
adverse events (104 procedures/electrodes). All patients were implanted by the same DBS team. Only PD patients fullling the
CAPSIT criteria for STN DBS were implanted after microelectrode
recordings were obtained. All patients received a unilateral implant
and only when needed underwent surgery on the contralateral side
in a staged fashion.
Results: There were only 3 (2.9%) intra-operative complications.
One was related to intracerabral hemorrhage (ICH-patient sustained
some persistent neurological sequelae) and 2 to body positioning of the
patients that interfered with normal breathing. Three (2.9%) postoperative, hardware-related complications were reported (2 infected implantable pulse generator-IPGs and one early battery replacement). One
patient chose to be explanted due to loss of DBS function. No fatalities
were recorded.
Conclusions: In our experience DBS surgery has proven safe for the
treatment of advanced, medically-refractory PD. The low rate of complications (individual and cumulative as compared to recent metaanalysis data from Kleiner-Fisman et al.) may be attributed to the
unilateral approach and concise microelectrode recordings obtained
intra-operatively.
204
Recognition of negative emotions is impaired by subthalamic nucleus deep brain stimulation in Parkinsons disease
J. Peron, I. Biseul, S. Fournier, S. Drapier, D. Drapier, P. Sauleau,
C. Haegelen, M. Verin (Rennes, France)
Objective: To conrm our previous results showing the impact of
bilateral subthalamic nucleus (STN) deep brain stimulation (DBS)
in Parkinsons disease (PD) on recognition of facial expressions
(RFE).
Background: It is now well documented that STN DBS in PD
could produce adverse events like emotional disorders. In line with
these studies, we recently reported, using an inter-group design, a
fear recognition impairment induced by STN DBS in PD (Biseul et
al., 2005). These changes have been related to a disturbance of the
STN limbic territory. These results have to be conrmed not only by
using an intra-group design but also by evaluating the effect of the
natural evolution of the disease.
Methods: RFE was investigated in a cohort of 24 PD patients
three months before and three months after STN DBS and a cohort
of 10 PD patients three months before and three months after the
introduction of subcutaneous continuous infusion of apomorphine
(APO). The two patients groups in each of the two conditions
(before and after) were compared with a group of 29 matched
control subjects. One series of 55 pictures of emotional facial
expressions was presented to each subject (Ekman & Friesen, 1976).
Subjects had to classify the pictures according to seven basic
emotions (happiness, sadness, fear, surprise, disgust, anger and no
emotion). The intact ability to percept faces was rst assured using
the Benton Test.
Results: Before surgery, the patients showed no impairment in
RFE in comparison with the control group. Three months after
surgery, they had signicant impairment in recognition not only of
fear but also of sadness expressions. This was not related to prosopagnosia. By contrast, the APO group didnt show any modication of RFE capacities before and after the onset of treatment.
Conclusions: Our results conrm the selective reduction of RFE
of fear and demonstrate a decit in RFE of sadness after STN DBS.
In addition, they prove for the rst time that this decit is not due
to the natural evolution of the disease but to a specic effect of the
DBS.

S63

205
Apathetic patients after deep brain stimulation of the subthalamic
nucleus in Parkinsons disease have an associated fear recognition
impairment
D. Drapier, J. Peron, P. Sauleau, S. Drapier, D. Travers,
A. Bourguignon, B. Millet, M. Verin (Rennes, France)
Objective: To demonstrate that motivation and emotional recognition share the same functional circuit including the subthalamic nucleus
(STN).
Background: In previous reports we have shown that STN deep brain
stimulation (DBS) could induce both apathy (Drapier et al, 2006) and
fear recognition impairment (Biseul et al, 2005).
Methods: A consecutive series of 18 parkinsonian patients was
assessed three months before and three months (M3) after STN-DBS.
Mean ( SD) age at surgery was 59.7 7.6. Mean disease duration at
surgery was 12.2 2.8 years. Apathy was evaluated using the Apathy
Evaluation Scale (AES). Patients were assessed as well using a computerized paradigm of recognition of emotional facial expressions
(Ekman & Friesen, 1976). The intact ability to percept faces was rst
assured using the Benton Recognition test.
Results: Apathy signicantly worsened at M3 (43.3 9.3 p
0.011) after STN-DBS in comparison with the preoperative evaluation
(38.1 6.5). Emotion recognition was globally impaired after surgery
(83.95 % 8.1 vs 78.42 % 8.6, p 0.041) mainly because of a
selective reduction of percentages of recognition of facial expressions
of fear (54.4 % 18.8 vs 41.96 % 22.8, p 0.049) and sadness
(73.78 % 25.75 vs 55.85 % 19.9 p 0.007). Furthermore, there
was a signicant correlation between apathy score and fear recognition
impairment (r 0.54, p 0.019) at M3.
Conclusions: As we hypothesized, our results show a correlation
between apathy and fear recognition impairment after STN-DBS. This
strongly suggests that apathy and fear recognition networks share same
territories in the limbic circuit, particularly in the limbic part of the
STN.
206
Quality of life and behavioral changes after STN DBS in Parkinsons disease
F. Tamma, E. Caputo, G. Rodol, S. Molteni, F. Cogiamanian,
M. Egidi, M. Locatelli, S. Marceglia, A. Priori, S. Sposta-Mrakic,
P. Rampini (Milano, Italy)
Objective: To analyze the behavioral changes observed after Subthalamic (STN) chronic stimulation, in order to consider the relationship between psychic and motor features with the aim to improve
patients selection and outcome.
Background: STN Deep Brain Stimulation (DBS) is an accepted and
effective treatment for advanced stage Parkinsons disease (PD), allowing long-term control of Parkinsonian symptoms. On the other hand
several reports of behavioral changes attracted medical attention. Moreover some pts experience a worsening of their Quality of Life (QoL)
after STN-DBS, despite good motor result.
Methods: 53 PD pts underwent STN-DBS and were studied before
surgery (T0) and at 12 month follow-up (T12) with several rating
scales, to investigate both motor and cognitive / behavioral aspects. For
the purpose of present study, we consider UPDRS, PDQ 39, Beck
Depression Inventory (BDI), Big 5 Questionnaire, Neuropsychiatric
Inventory.
Results: Motor part of UPDRS improved in all 53 pts, comparing
T12 medoffstimon vs T0 medoff conditions (mean improvement
7116.7%, minimum 35%). We divided the whole group according to
the change in PDQ39 scale at T12: 10 out of 53 showed a stable or
worsened PDQ39 score (group W), while the remaining 43 pts showed
an improvement of the PDQ39 score (group B). There was a signicative difference in age (p0.049), pts in group W being older (64 yrs)
than pts in group B (58.5 yrs). The mobility subscale was signicatively worsen in group W (p0.001) and improved in group B
(p0.0001). In a subgroup of 26 pts (6 from group W and 20 from
group B) the Big 5 Questionnaire showed a signicative difference

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

between the two groups only in the scale of amicality (p0.004) and
cooperativity (p0.006). The BDI score turned pathological at follow-up in 16 out of 52 pts. There was a signicant correlation between
the group B and their improvement in BDI (p0.001). Neuropsychological assessment conrmed the functional neutrality of STN-DBS on
cognitive performance.
Conclusions: Despite global motor improvement, some pts reported
a worsening of their QoL at T12. Behavioral issues (poor amicality and
cooperation), older age, persistance of walking difculties after surgery
appear to be linked to a worsening of PDQ39 at T12.
207
Different cerebral cortical areas inuence the effect of subthalamic
nucleus stimulation on parkinsonian motor decits and off gait
freezing
C.H. Lyoo, S. Aalto, J.O. Rinne, K.O. Lee, S.H. Oh, J.W. Chang,
M.S. Lee (Seoul, Republic of Korea)
Objective: To dene brain areas associated with the expression of
subthalamic nucleus deep brain stimulation (STN DBS) effect on
parkinsonian motor decits and off gait freezing (OGF).
Background: Nearly all parkinsonian motor decits respond to STN
DBS. However, STN DBS effect on OGF is inconsistent, suggestive of
involvement of different pathophysiology of OGF.
Methods: This study included 10 Parkinsons disease patients with
typical OGF. One month before STN DBS, quantitative [18F]-deoxyglucose PET scan studies were performed after 12 hours of overnight
withdrawal of levodopa. We measured the UPDRS motor and modied
freezing of gait (mFOG) scores during levodopa off (mean UPDRS
motor score 38.5 10.5, mean mFOG score 12.9 3.7) and on
period (mean UPDRS motor score 22.1 7.9, mean mFOG score
1.1 2.0). After adjusting stimulation parameters for two months,
UPDRS motor and mFOG scores were measured in four different
conditions (levodopa off/DBS off, levodopa off/DBS on, levodopa
on/DBS off and levodopa on/DBS on). The percentage improvement of
mFOG and UPDRS motor scores by STN DBS during levodopa off
period was calculated. Using statistical parametric mapping analysis,
we searched for brain areas in which glucose metabolism before STN
DBS correlated with the improvement of mFOG and UPDRS motor
scores by STN DBS.
Results: During the levodopa off period, STN DBS improved the mean
UPDRS motor scores by 32.3% (from 37.3 8.7 to 25.3 7.8, P 0.01)
and the mean of mFOG scores by 56.6% (from 12.9 4.7 to 6.4 6.3,
P 0.01). There was no correlation between the improvement of UPDRS
motor and mFOG scores (Kendalls correlation, P 0.47). The improvement of UPDRS motor score correlated with the metabolic activities of
rostral supplementary motor area (Brodmanns area 8; BA8), anterior
cingulate cortex (BA32) and prefrontal cortex (BA9) (cluster-level corrected P 0.05). On the other hand, there was a positive correlation
between the improvement of mFOG and the glucose metabolic activity of
the parietal, occipital and temporal sensory association cortices (clusterlevel corrected P 0.05).
Conclusions: Dysfunction of two different cerebral cortical areas limit
the benecial effects of DBS on parkinsonian motor decits and OGF.
208
Effect of subthalamic nucleus deep brain stimulation on emotional
experience in Parkinsons disease patients
S. Fournier, I. Biseul, J. Peron, P. Philippot, S. Drapier, D. Drapier,
P. Sauleau, C. Haegelen, M. Verin (Rennes, France)
Objective: To investigate the impact of subthalamic nucleus (STN)
deep brain stimulation (DBS) on emotional experience (i.e. subjective
feeling of emotion) in Parkinsons disease (PD) patients in order to
specify the more general role of the STN in emotion processing.
Background: The benecial effect of STN DBS on PD motor symptoms
is now well documented. However, a number of studies reported behavioral and emotional disorders induced by STN DBS. In line with these
studies, we recently reported a specic fear recognition impairment induced by STN DBS in PD (Biseul et al., 2005). These changes have been

Movement Disorders, Vol. 22, Suppl. 16, 2007

related to a disturbance of the STN limbic territory hypothesized to be

implicated in emotion processing. The present study investigated whether
STN DBS also affects emotional experience in PD.
Methods: A validated battery of lm excerpts (Schaefer et al., in press)
was used to elicit different emotions (anger, happiness, fear, disgust,
sadness and neutral) in 12 pre-operative PD, 12 post-operative PD and 18
matched controls. Participants reported the intensity of their emotions in
the Differential Emotion Scale consisting of 10 emotion categories to be
rated on a ve-point scale (1 not at all, 5 very intense).
Results: A global analysis of participants mean responses in each
lm excerpt revealed no signicant difference between the three
groups. However, a ne-grained analysis of the response type (proportion of low intensity versus high intensity responses) indicated a
tendency for an enhanced feeling of anger and a reduced feeling of fear
in the post-operative group as compared with the pre-operative group.
Conclusions: These preliminary ndings indicate that STN DBS
affects the feeling of emotion in PD and strongly suggest that the STN
limbic area is not only involved in emotional recognition but also in
emotional experience.
209
Thalamic deep brain stimulation in patients with tremor due to
multiple sclerosis: A series of 11 patients
H.-H. Capelle, C. Schrader, R. Dengler, J.K. Krauss (Hannover,
Germany)
Objective: To evaluate the long-term effect of thalamic deep brain
stimulation (DBS) in a series of patients with severe tremor due to
multiple sclerosis.
Background: DBS of the thalamic VIM has been shown to improve tremor in patients suffering from various tremor disorders.
The issue of DBS in patients with tremor due to multiple sclerosis
has been a matter of debate. Kinetic and postural tremors in multiple
sclerosis may result in considerable functional impairment. Since
tremor is only one of many symptoms in multiple sclerosis patients
the impact of thalamic DBS on overall disability remains unclear in
the long-term.
Methods: We evaluated retrospectively the results of thalamic
DBS on long-term outcome in a series of 11 patients. Mean age was
57 years (range, 31-75, 6 F/5 M). All patients had a chronic
progressive course of multiple sclerosis. Tremor was disabling in all
instances. CT-stereotactical implantation of quadripolar electrodes
(Medtronic, 3387) in the VIM was performed without surgical
complications in all patients. In 10 patients the electrode was
implanted on the left side, in 1 patient on the right.
Results: All patients were available for long-term follow-up. Initial
symptomatic improvement was present in all instances. This, however,
did not correlate with functional improvement. Both, symptomatic and
functional improvement on long-term was found only in single instances. Stimulation power was higher on average than in other tremor
disorders. In particular, patients with cognitive impairment did not
benet from thalamic DBS.
Conclusions: Thalamic DBS is a safe and modiable treatment
option for patients with severe disabling tremor due to multiple sclerosis. Nevertheless, patient selection remains a challenging task in this
type of tremor.
210
Radiological and clinical predictive factors of long-term outcome of
bilateral subthalamic stimulation in advanced Parkinsons disease
T. Rouaud, S. Drapier, J. Peron, E. Leray, P. Sauleau, Y. Rolland,
M. Verin (Rennes, France)
Objective: To dene radiological and clinical predictive factors of
motor, cognitive and quality of life (QoL) outcomes in patients with
advanced Parkinsons disease (PD) one year after bilateral deep brain
subthalamic stimulation (STN DBS).
Background: Preoperative clinical predictive factors of STN DBS
outcome are now well established. Little is known however about
radiological predictive factors.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Methods: A consecutive series of twenty-seven patients (10
women, 17 men; age : 59,6 7,8 years; disease duration : 12,7
3,6 years) was assessed three months before and twelve months after
STN DBS. All of them underwent a standardised brain MRI three
months before surgery. The main postoperative parameters of interest were the Unied Parkinsons Disease Rating Scale (UPDRS) II
and III total and axial scores under stimulation in both off and
on-medication conditions, the neuropsychological performances and
the QoL evaluation.
Results: As compared with baseline, the patients motor scores at one
year while off-medication improved by 41,1% (p 0,0001). Onmedication axial motor symptoms worsened for 40% of patients and
tended to be less responsive to dopaminergic treatment after surgery.
Neuropsychological tests showed a signicant decline of verbal initiation, verbal uency and conceptual reasoning. The most robust clinical predictive factors of motor and cognitive outcomes were age at the
time of surgery, preoperative residual total motor score, Hoehn and
Yahr stage in the ONdopa condition and the preoperative performances
on the trail-making-test. The strongest radiological predictive factors
were signal abnormalities on both putaminal and pedunculopontine
areas for the global and axial motor outcome, and on the pedunculopontine area for the cognitive outcome.
Conclusions: Radiological abnormalities observed in PD might help
to select the best candidates for surgery and contribute to a greater
understanding of the mechanisms of action of STN DBS, especially on
the pedunculopontine nucleus, identied as a marker of non-dopaminergic brain lesions associated with axial motor disturbance and cognitive decline after surgery.
211
Prospective evaluation of deep brain stimulation in a series of 15
patients with segmental dystonia
J.K. Krauss, C. Blahak, H.-H. Capelle, K. Kekelia, H. Baezner,
R. Weigel, J.C. Woehrle (Hannover, Germany)
Objective: To investigate prospectively the long-term effects of DBS
in a larger series of patients with segmental dystonia.
Background: Deep brain stimulation (DBS) has been shown to be a
valuable treatment option in patients with otherwise refractory generalized and cervical dystonia. Little is known, however, on its effect on
segmental dystonia affecting various body regions.
Methods: Fifteen patients with refractory segmental dystonia
were selected to undergo DBS (7 women, 8 men, age range 30 - 74).
Thirteen patients suffered from primary dystonia, and two patients
had secondary dystonia. The family history was negative in all
instances. The preoperative duration of symptoms ranged between 1
and 35 years. Patients were scheduled either for pallidal DBS or for
bifocal pallidal and thalamic DBS (patients with secondary dystonia
or marked tremor). The prospective study protocol included assessment of the dystonia by the BFM, UDRS and the GDRS dystonia
rating scales.
Results: The stereotactic surgeries were unremarkable and without
side effects in 14 instances, while the operation had to be aborted in one
patient due to cardiopulmonary problems. All operated patients were
available for short-term follow-up (F I, at 4-9 months p.o.) and longterm follow-up (F II, at 12-36 months p.o.). Eight patients underwent
pallidal DBS , and six patients had bifocal DBS. On the long-term
follow-up 12 patients had pallidal DBS, and 2 had thalamic DBS. There
was stable improvement of motor scores both on short- and long-term
follow-up (BFM pre 53.8, F II 15.5; UDRS pre 35.4, F II 16.9; GDRS
pre 27.5, F II 11.3) which was paralled by improved disability scores
(BFM disability pre 6.0, F II 3.9). In three patients stimulation-induced
dysarthria occurred with higher voltages which limited the therapeutical benet in these patients.
Conclusions: Bilateral DBS is an effective treatment modality in
patients with segmental dystonia. Overall, the results are comparable to
those obtained in patients with generalized dystonia.

S65

212
Deep brain stimulation perioperative course in older (> 70 years)
vs. younger Parkinsons patients
B. Navi, K. Strybing, M.J. Nirenberg, L. Steinberg, T.F. Burgut,
B. Nikolov, M.G. Kaplitt, C. Henchcliffe (New York, New York, USA)
Objective: To determine whether Parkinsons disease (PD) patients
70 years undergoing deep brain stimulator (DBS) surgery have
higher rates of perioperative complications than younger patients.
Background: Surgical trials of DBS in PD have largely excluded older
elderly patients, who might be at increased risk of perioperative complications. However, there is little data to support or refute these concerns.
Methods: We conducted a retrospective chart review of consecutive
PD patients admitted to New York Presbyterian Hospital-Cornell for
DBS insertion from 2002 to 2006. Age, hospital morbidity and mortality, length of stay (LOS), and disposition were recorded. Patients
were divided into (1) older patients: age70 years (n14, mean
74.6 3.7, range 70-81 years), and (2) younger patients: age70 years
old (n26, mean 61.1 6.6, range 41-69 years) at the time of surgery.
Results: 40 PD patients underwent DBS insertion of the subthalamic
nucleus (38 bilateral). Disease duration was comparable in the older and
younger age groups (0.34.0 vs 9.14.2 years). There were no fatalities
in either group. Perioperative morbidity in the older group included:
postoperative delirium (n2, 14%), pneumonia (n2, 14%), intracerebral
hemorrhage/seizure (n1, 7%), intraparenchymal/ventricular hemorrhage
(n1, 7%), seizure (n1, 7%), and gait apraxia (n1, 7%). Both patients
with intracerebral hemorrhage signicantly recovered neurological function. In the younger group, morbidity included: postoperative delirium
(n3, 12%), seizure (n3, 12%), urinary tract infection (n2, 8%),
dysarthria (n1, 4%), pneumonia (n1, 4%), and no intracerebral hemorrhage. Mean LOS was signicantly longer in the older (5.65.8 days)
vs. younger (3.34.3 days) group (p0.017). Older patients were more
likely to be discharged to rehabilitation or skilled nursing facilities (50%)
than younger patients (19%, p0.011).
Conclusions: In this study, there was no perioperative mortality.
Morbidity included delirium, infection, and seizure in each age group,
but hemorrhage only in the older group. Association of advanced age
with longer LOS and greater requirements for supportive care after
discharge indicates a need for larger prospective studies to evaluate
possible age-related perioperative risks.
213
Long-term follow-up of pallidal DBS for primary dystonia
M. Tagliati, I.U. Isaias, D. Weisz, J. Shils, R. Alterman (New York,
New York, USA)
Objective: To evaluate long-term safety and efcacy of pallidal deep
brain stimulation (DBS) therapy in patients with primary dystonia.
Background: DBS is becoming the standard of care for medically
intractable, disabling primary dystonia. Initial reports describing DBS
results in generalized dystonia have been encouraging. However, little
data is available on long-term follow-up.
Methods: We reviewed clinical outcomes, evaluated using the
Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS), in 11 consecutive patients with medically refractory primary dystonia treated
with chronic pallidal DBS. All had stereotactic implantation of DBS
electrodes by one surgeon (RLA), employing magnetic resonance imaging and intraoperative microelectrode recording for targeting. Mean
age at disease onset was 16.6 years (range 6-50) and mean age at
surgery was 31.3 years (range 13-63). Six patients tested positive for
the DYT1 gene mutation, none had previous ablative surgery. Average
baseline BFMDRS motor score was 52.6 (range 17-81). Stimulation
settings were systematically adjusted according to clinical effect. All
patients were followed-up for at least three years, six patients for four
years and ve patients for ve years.
Results: All patients showed progressive improvement of their dystonic
symptoms after DBS, with an average BFMDRS motor score decline of
73% (range 50-97) at one year. Such progress was sustained at successive
yearly follow-ups, with average improvements of 78% (range 48-98) at 2
years; 77% (range 45-99) at three years; 80% (6 patients, range 50-97) at

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

four years, and 85% (5 patients, range 69-99) at ve years. Complications

were limited to one scalp infection (requiring lead revision) and one
extension wire break (requiring replacement). Seven patients needed their
IPGs changed because of battery end-of-life.
Conclusions: Pallidal DBS is a safe and effective therapy for medication resistant primary dystonia. In our cohort of well-selected patients, improvement ranged from 50 to 99%, with a low complication
rate. DBS benets were sustained up to ve years.
214
Microlesion effects and tremor outcomes in ventrointermediate
deep brain stimulation (VIM-DBS)
O. Sitburana, W.G. Ondo (Houston, Texas, USA)
Objective: To investigate the relationship between the peri-operative
microlesion effect (MLE) and the subsequent tremor response and
parameter settings of VIM DBS after DBS placement in essential
tremor (ET).
Background: A microlesion effect is dened by the abolition or
reduction of tremor after insertion of DBS electrodes but before activating the stimulator. The exact mechanism of this is not known. MLE
might predict patient outcome, although formal data is lacking.
Methods: 16 thalami of 10 ET patients, who underwent unilateral
(n4) and bilateral VIM DBS (n6) were assessed pre-operatively, at
24-hour post-operatively, and at initial DBS activation, and 6 month
post-operatively while the DBS was On and Off. The severity of
tremors was rated with the Tremor Rating Scale (0-4) including hand
tremor at rest, outstretched, wing beating, nger-nose-nger, dot approximation, and spiral drawings (total score 0-24). The difference of
tremor scores pre-operatively and 24-hours post-operatively (MLE)
were segregated into 3 groups based on MLE: mild (0 - 2), moderate
(2-4), and marked (4).
Results: The mean percentage change of tremor scores between preand 24-hour post-operative DBS in groups 1, 2, 3 were -8.9% (SD
0.06), -22.6% (SD 0.04), -41.82 % (SD 0.09). At initial activation
(24.33 days), MLE resolved in group 1 and 2. In group 3, ve of six
sides still had more than a 10 % improvement in tremors. At month 6
there was no meaningful MLE. The mean percentage reduction of On
scores at initial activation compared to baseline were -31.4%, - 47.7%
and -70.7% in groups 1, 2 and 3. However at 6 months MLE did not
predict On scores. The mean amplitude at 6 months were 3.4V (SD
0.6), 3.7V (SD 0.5) and 2.9V (SD 0.9) in group 1, 2 and 3.
Conclusions: MLE predicts tremor outcome at initial activation, but
does not effect either efcacy or device parameters at 6 months.

Results: Eleven patients have been studied so far (on-going project). The
mean time of DBS treatment was 28.17 months (14-38) with an improvement of 50% from the off-stim state using UPDRS-III. The acute effect of
the adjustments produced a further improvement of 20.2 % in the UPDRSIII that was not maintained a mean time of 41 days later (chronic effect)
except on timed tests (g 1). An increase in voltage produced the most
sustained effect. When adjustments involved changes in the AC the acute
benet was not maintained. Fourteen AC (of 22 sides) were located in the
STN. The position of the AC did not predict the adjustment made (g 2).
Data of i-LFPs were available in 10 sides. The AC was the same as the
contact showing the maximum power in the a in 8 sides early after
surgery and in 7 sides during chronic stimulation.
Conclusions: These results highlight that acute and chronic effects of
adjustments of SP differ and that combinations of SP providing a more
stable improvement can be determined. The larger population to be studied
will determine if MRI and i-LFP can predict the most effective contact.

215
Optimisation of the stimulation parameters in STN-DBS for Parkinsons disease
I. Martinez, L. Zrinzo, C.C. Chen, S. Tisch, E. Tripoliti, E. Borrell,
P. Brown, M. Hariz, P. Limousin (London, United Kingdom)
Objective: To determine the combinations of stimulation parameters
(SP) that provide longer lasting improvement and to evaluate the role
of MRI and intraoperative recordings of STN local eld potentials
(i-LFPs) in the selection of the most effective contact.
Background: Bilateral STN-DBS is an effective treatment for selected PD patients (Limousin, 1998). Postoperative management involves frequent adjustments of SP. Selection of SP is based on the acute
effect and it is uncertain if all adjustments produce long lasting benets.
The location of the contacts within the STN can be determined by a
physiological index of the STN (the maximum power in the LFP
activity (Chen, 2006)), and by postoperative MRI; however their values
in selecting the most effective contact have not been evaluated.
Methods: Blinded clinical evaluations with respect to adjustments of SP
(off-med/on-stim) included UPDRS-III and timed tests before and after
adjustments (acute effect, clinical impact immediately after the adjustment) and one month later (chronic effect). The position of the active
contact (AC) was evaluated using stereotactic MRI. The contact corresponding to the level of maximum LFP activity was compared with the
AC

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (215).

216
Does subthalamic nucleus deep brain stimulation (STNDBS) inuence personality in Parkinsons disease?
A. Gronchi-Perrin, S. Aybek, A. Jaques, C. Pollo, P.R. Burkhard,
F. Vingerhoets (Lausanne, Vaud, Switzerland)
Objective: To investigate perceived personality traits changes in
Parkinsons disease (PD) patients after STNDBS.
Background: Studies and clinical experiences have suggested that STNDBS may have adverse effects on behaviour and mood. A previous study

POSTER SESSION I, TUESDAY, JUNE 5, 2007

showed that perception of changes in quality of life may depend upon
studys design.
Methods: 22 PD patients and twenty caregivers (CG) were independently administered twice (within three months preceding STNDBS
and six months thereafter) the Iowa rating scales, that explore 30
specic characteristics of personality change, according to the degree
of disturbance and changes from the pre-STNDBS status. Statistical
analyses were based on Wilcoxon tests for correlated samples (P0.05).
Results: Retrospectively, CG reported signicantly more impulsivity
after STNDBS while PD noticed less stamina capacity of planning, more
social withdrawal and vulnerability to pressure. In contrast, prospectively,
CG observed less anxiety, PD less obsessiveness, excitability, social inappropriateness and more insight. Differences between preoperative status
and its postoperative estimate showed that CG and PD do signicantly
underestimate postoperatively the preoperative behavioural disturbances:
CG underestimate the lack of stamina, social inappropriateness and vulnerability to pressure; PD the behavioural rigidity, the lack of stamina and
planning, poor frustration tolerance, social withdrawal, excitability, indecisiveness, apathy and vulnerability to pressure.
Conclusions: Prospective evaluation, that demonstrates objective
effects of STNDBS, shows a decrease of anxiety (CG), obsessiveness,
excitability, social inappropriateness and more insight (PD). Retrospective evaluation, that may reect subjective variations in perceived
outcomes, shows rather an increase of impulsivity (CG) and vulnerability to pressure and a decrease of stamina, planning capacities and
social withdrawal (PD). These discrepancies are secondary to a postoperative change of the patients and caregivers estimate of the preoperative status with tendency to underestimated preoperative negative
personality traits. Reported effects of STNDBS on traits of personality
depend upon the design of evaluation.
217
PD patients can perform activities of their own choice after DBS
in STN
A.L. Tornqvist, G. Ahlstrom, H. Widner, S. Rehncrona (Lund, Sweden)
Objective: The objective was to compare the groups of patients treated
with Subthalamic (STN) Deep Brain Stimulation (DBS) who reported total
overall fulllment with those who reported partial or no fulllment of their
own formulated outcome goals, with respect to demographic data, lead
placement, mobility, perception of health and satisfaction with the performance of individual goals.
Background: Subthalamic (STN) Deep Brain Stimulation (DBS) has
been shown to reduce motor symptoms in Parkinsons disease (PD).
Improvements have earlier also been shown in patient reported outcome questionnaires, there are however few papers presenting the
patients own outcome goals.
Methods: Twenty-six PD patients with a mean age 62 7 years
and 14 6 years of PD duration scheduled for bilateral DBS in STN
were consecutively included. Preoperatively, the patients formulated their own personal outcome goals, and evaluated the fulllment of the goals 12 months postoperatively. The fulllment of
goals was rated as total, partial or no. Comparisons between the
grades of fulllment were made based on: demographic data, DBS
settings, UPDRS III, SF36, mobility and amount of separate goals
performed with a satisfactory quality.
Results: At the 12 months follow-up the motor and social goals were
reported to be totally overall fullled by 46% and 44% of the patients. The
remaining patients evaluated their goals to be partially fullled and no one
reported no fullment. Preoperatively, there were no signicant differences between the two groups. Postoperatively the patients with total
overall fullment of goals did perceive better health and mobility than the
ones who evaluated the fullment to be partial. The patients with total
overall fullment were also more satised with the performance of the
separate goals than those with partial fullment of their goals.
Conclusions: The data suggests that the patients who reported total
overall fullment of their goals, perceived better health and mobility
and were more satised with the performance of their separate goals,
than the patients who reported partial fullment.

S67

218
Hemichorea/hemiballism after craniopharyngeoma resection:
Treatment with bifocal brain stimulation
J.K. Krauss, T. Kinfe, H.-H. Capelle (Hannover, Germany)
Objective: To report the rst patient with hemichorea hemiballism
who underwent bifocal implantation of DBS electrodes.
Background: Hemichorea - hemiballism is a very rare movement disorder. While it usually has a benign cause when it is related to ischemia,
hemichorea hemiballism due to other causes may be persistent.
Methods: After resection of a craniopharyngeoma at age 49 in
another hospital, hemichorea hemiballism developed with a latency
of several weeks. The movement disorder was refractory to medical
treatment. Since the patient was greatly impaired by the movement
disorder, two years later, she was scheduled for bifocal deep brain
stimulation contralateral to the side of the movement disorder. At age
52, she underwent implantation of DBS electrodes in the thalamic VIM
and the posteroventral lateral GPi.
Results: There were no postoperative complications. Microelectrode
recording was helpful to further rene the targets. Frequencies in the GPe
and GPi were lower as compared to Parkinsons disease patients. When
comparing the results of intraoperative test stimulation, thalamic stimulation had a more immediate and thorough effect on the contralateral
movement disorder than pallidal stimulation. One year follow-up with
chronic thalamic stimulation (0,3 V, 130 Hz, 210 sek, bipolar modus) has
resulted in a complete subsidence of the movement disorder.
Conclusions: Deep brain stimulation is a very efcient treatment modality for refractory hemichorea hemiballism. Thus far, this option
appears to be underused. In contrast to the experience with radiofrequency
leasoning, thalamic targets may be more effective than pallidal targets.
219
Bilateral GPi stimulation with 4 leads in primary generalized
dystonia
B. Biolsi, L. Cif, S. Gil Robles, X. Vasques, S. Gavarini, S. Plagnol,
P. Coubes (Montpellier, France)
Objective: Internal Globus Pallidus stimulation is a validated procedure in the treatment of primary generalized dystono-dyskinetic syndromes (SDD). Very long term follow-up conrms a very good improvement (70%).
Background: In 14 out of 59 primary generalized patients we observed an initial improvement inferior to 70% or a late worsening under
stimulation. Such a result was estimated sub-optimal and we decided to
extend the volume of stimulation pallidal by implanting 2 supplementary leads.
Methods: Burke Fahn Mardsen Dystonia rating scale was used preand postopereratively. Mean score of improvement is 76. 2% /-6%
(SEM) at 5 years follow-up in our primary generalized dystonia population. Is considered sub-optimal a result inferior to 70 %. 14 patients
(9 DYT1, 5 DYT1 -) were reoperated. These patients could be
divided into 2 groups: patients with a score 70% one year after initial
surgery and patients with an secondary worsening of dystonia, after a
good initial improvement. The mean follow-up after the second surgery
was 27.5 months.
Results: No surgical complication was observed. 12 out of 14 patients took advantage from reoperation.
Conclusions: DBS for non-parkinsonian movement disorders is particularly challenging when treating primary DDS. In fact, when observing either an incomplete result or facing a secondary worsening,
many items can be systematically questioned. The somatotopic organization of the GPi has already been studied and supports the methodology we propose here. In selected patient, the reinforcement of the
therapeutic effect could be obtained by reimplantation of additional
electrode in the same target. In primary generalized dystonia, adjunction of a supplementary pair of elead in the GPi could be proposed in
patients with a good initial response and secondary worsening of the
disease.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

220
Effective treatment of myoclonus-dystonia syndrome (MDS) by
bilateral internal pallidal stimulation
L. Cif, B. Biolsi, A. Saux, S. Gavarini, S. Gil Robles, X. Vasques,
P. Coubes (Montpellier, France)
Objective: To evaluate in four patients with Myoclonus-dystonia syndrome (MDS) the efcacy of internal globus pallidus stimulation (GPi).
Background: Myoclonus-dystonia syndrome (MDS, DYT11, MIM:
159900) is a genetically heterogeneous disorder due to mutations in the
gene encoding epsilon-sarcoglycan (SGCE). MDS is characterized by
myoclonic jerks associated with dystonia and more rarely with psychiatric comorbidities. Deep brain stimulation (DBS) has been proposed in
primary generalized, segmental and/or focal dystonia. Furthermore,
little information has been published about the efcacy of DBS in MDS
and, surgical protocols (including target) were different from one study
to another.
Methods: We proposed high frequency internal globus pallidus (GPi)
stimulation in 4 patients (mean age at onset 4.94.3 years, mean age
at surgery 21.4321.50 years) with genetically conrmed MDS. Clinical characteristics and genetic prole are shown in table 1. Stereotactic
neurosurgery for bilateral GPi was based on direct visualisation of the
target and performed under general anaesthesia. Dystonia was assessed
by the movement and disability sections of the Burke-Fahn-Marsdens
Dystonia Rating Scale (BFMDRS) and myoclonus by the Unied
Myoclonus Rating Scale (UMRS).
Results: Detailed scores for the BFMDRS and UMRS and electrical
settings are presented in table 2. The mean pre-and post-operative
UMRS absolute scores were 111.75 (34.71) and 29.5 (16.4) repectively. In 3 patients, improvement of the UMRS was superior to 80 %
and of 46% in patient 3. The mean pre- and postoperative absolute
scores for the movement section of the BFMDRS was of 14.3 and 11
repectively. For the disability section, the mean pre- and postoperative
scores were 9.25 and 5.25 respectively. Mean improvement scores were
hindered by the spread of symptoms under DBS in patient 3, as shown
by the switching off of the stimulators performed during one week. In
patient 1, with 5 years follow-up, the effect of DBS is markedly
maintained in both, myoclonus and dystonia.
Conclusions: Bilateral GPi stimulation can be considered as an
effective treatment in severe pharmacologically resistant forms of
MDS. Homogeneous results under DBS have been obtained in 3 out of
4 patients treated using the same surgical targeting technique.

Mutations of the SGCE gene and clinical characteristics for

the 4 patients
Patient Gender
1

Age
of onset
(years)

Mutation
Splice site mutation
233-1G3A
Splice site mutation
233-1G3A
Nonsense mutation
c.300GA
Nonsense mutation
c.304CT

Sign at Myoclonus
onset distribution

0.8

Neck

Neck

LL

11

Dystonia Age at
distribution surgery

Neck,Trunk,
RUL
6.5
UL
Trunk, neck, Neck, trunk 53.2
UL
LL, UL, trunk Trunk, LL 10.5

Trunk Trunk, neck, Neck, trunk 15.5

UL, LL

M, male; F, female; LL, lower limbs; UL, upper limbs; R, right; L, left.

Pre- and postoperative scores of myoclonic and dystonic

symptoms and electrical settings at one year
Preoperative
UMRS
(/377)

Postoperative
UMRS
(/377)

Pre-operative
BFMDRS
-section I
(/120)

Post-operative
BFMDRSSection I (/
120)

Pre-operative
BFMDRSSection II
(/30)

Post-operative
BFMDRSSection II
(/30)

69

13

1.5

13

113

21

25.5

111

60

33.5

11

154

24

18

Patient

Movement Disorders, Vol. 22, Suppl. 16, 2007

Electrical
settings
L:E1-, 1.1V;
R:E1-, 1.3V
L:E1-, 1.7V;
R: E1-E2-,
1.6V
L:E0-E1-E2-,
0.9V; R:E0E1-E2-, 0.9V
L:E0-E1-E2-,
1.7V; R:E0E1-E2-,1.7V

UMRS: Unied Myoclonus Rating Scale (/377); BFMDRS: BurkeFahn-Marsdens Dystonia Rating Scale; Section I, movement section
(/120); Section II-disability section (/30); L, left; R, right; E-, negatively activated electrode (contact); all the post-operative scores are
given with one year follow-up. A frequency of 130Hz and 450
seconds pulse width was applied in all patients.
221
Bilateral subthalamic nucleus stimulation in advanced Parkinsons
disease: Five years follow-up
H. Gervais-Bernard, J. Xie-Brustolin, P. Mertens, G. Polo,
H. Klinger, I. Benatru, E. Broussolle (Lyon, France)
Objective: To assess the long-term efcacy and safety of chronic
bilateral stimulation of the subthalamic nucleus (STN) in patients with
advanced Parkinsons disease (PD).
Background: Twenty-one consecutive patients with idiopathic Parkinsons disease treated with bilateral stimulation of the STN were
studied. Three patients died and two were lost of follow-up because of
carcinoma, and 16 patients were assessed at ve years.
Methods: Parkinsonian status was assessed preoperatively and at
one, three and ve years postoperatively using the Unied Parkinsons
Disease Rating Scale (UPDRS) and neuropsychological evaluation in
on and off-medication / on and off stimulation conditions.
Results: At 5 years follow-up, STN stimulation reduced the UPDRS
motor score by 51.45 % compared to baseline in the off-medication
conditions. Tremor, rigidity, bradykinesia, postural stability, and gait
improved by 58.8 %, 62.8 %, 54.9 %, 35.9 % and 30.7 %, respectively.
UPDRS part II scores were reduced by 53.9 %. The overall dopaminergic drugs dose was reduced by 63.6 % at ve years, and only one
patient was no longer taking dopaminergic drug at ve years. However,
axial dopa-unresponsive signs worsened in some patients.
Conclusions: Our data demonstrate that: 1) Bilateral STN stimulation is relatively safe, improves the motor symptoms and drug-related
motor complications of PD, and reduces the daily dosage of medication; 2) This benet is sustained over time despite the occurrence of
axial doparesistant signs in some patients.
222
Do neuropsychological changes after deep brain stimulation of the
subthalamic nucleus relate to alterations of cerebral glucose metabolism in Parkinson patients?
M. Haarer, E. Kalbe, T. Weber, G.R. Fink, R. Hilker (Koln,
Germany)
Objective: To examine whether alterations of cognitive functions
after deep brain stimulation of the subthalamic nucleus (STN-DBS) in
patients with advanced Parkinsons disease (PD) relate to changes of
cerebral glucose metabolism.
Background: STN-DBS is an effective surgical treatment for motor
symptoms in patients with advanced PD. Effects of STN-DBS on
cerebral glucose metabolism and cognition have also been reported, but
the relationship of these effects remains unclear.
Methods: 8 patients with advanced PD (5 men, 3 women, mean age:
62 years, SD5.4, median Hoehn & Yahr 4, range 3 to 4) and bilateral
STN-DBS were enrolled in the study. Positron emission tomography
(PET) with 18-uorodeoxyglucose (FDG) as well as neuropsychological testing (letter uency and word list learning with immediate and
delayed recall) were administered preoperative (t1) and postoperative 6
months after surgery (t2). Several cortical and subcortical regions-ofinterest (ROI) were dened in individual co-registered MRI, and ROIspecic FDG uptake was quantied before and after STN surgery.
Cognitive test results at t1 versus t2 were analyzed with paired t-tests,
and difference scores of neuropsychological test performance (t2 minus
t1) were correlated with metabolic changes (t2 minus t1) in all ROIs
under investigation.
Results: No signicant STN-DBS group effects on cognitive test
scores were observed. However, signicant positive correlations were
found between changes in the word rate of the uency task and
metabolic changes in Brodmann area (BA) 10 (r0.72, p0.05) and

POSTER SESSION I, TUESDAY, JUNE 5, 2007

BA 46 (r0.77, p0.05), and the immediate recall condition of the
word list learning task and BA 24 (r0.75, p0.05) and BA 47 / 11
(r0.71, p0.05), respectively.
Conclusions: The results demonstrate systematic relationships between alterations in cognitive functions and alterations of cerebral
glucose metabolism in functionally related brain regions after STNDBS in PD. While the performance of executive functions depend on
the dynamics of glucose metabolism in the dorsolateral prefrontal
cortex, verbal memory encoding is specically related to metabolic
changes in orbital prefrontal and anterior cingulate areas.
223
Psychosis induced by subthalamic nucleus stimulation in a patient
with Parkinsons disease
M. Pilleri, A. Caria, G. Nordera (Arcugnano, Vicenza, Italy)
Objective: A psychotic episode induced by Sub Talamic Nucleus
Deep Brain Stimulation (STN-DBS) occurred in a previously psychiatrically healthy male patient, at 62 years old. He has been affected
with PD since age 46. In treatment with levodopa since age 51, he
experienced motor uctuation since age 54.
Background: Before surgery the patient was thoroughly examined:
Brains Magnetic Resonance Imaging (MRI) , Unied Parkinson Disease Rating Scale (UPDRS), and psychological assessment with Mini
Mental State Examination (MMSE), and Frontal Assessment Base
(FAB) effectively ruling out any counter indication to surgery.
Methods: The surgery procedure was remarkable: under stereotactic
conditions the patient underwent bilateral implant of stimulators in
STN. After the surgery the patient was calm and collaborative, and no
depression or hallucinations were detected.
Results: After two weeks from the surgery, the stimulators were
turned on with the following parameters: CA1(right STN) 3.0V/60microsec/130 Hz monopolar 1- CA2(left STN)3.0V/60microsec/130Hz
monopolar 6- with substantial improvement of motor performances. A
few hours following the switch-on of the device, the patient started a
progressive change in behaviour, becoming more and more aggressive,
confused and mildly hallucinated. A Computed Tomography (CT) Scan
was performed to rule out organic explanations such as encephalitis or
hemorrhage, for example. Anti-psychotic treatment with quetiapine
was promptly started and progressively titrated up to 200 mg TID with
sedation, but without substantial change in behaviour. Levodopa was
stopped with clear worsening of motor performances, but without
improvement in behaviour.
Conclusions: After 2 weeks, we turned the devices off and the patient
started a progressive return to the pre-operative psychiatric status.

PARKINSONS DISEASE
224
Rotigotine transdermal patch for the treatment of early morning
and night time motor symptoms in patients with idiopathic Parkinsons disease
N. Giladi, B. Boroojerdi (Tel Aviv, Israel)
Objective: Transdermal rotigotine patch (Neupro) is approved in
the EU for treatment of all stages of Parkinsons disease (PD). Given
the stable plasma levels of rotigotine achieved with the transdermal
delivery, this trial investigated the rotigotine effects on early morning
motor function in PD patients.
Background: Rotigotine (Neupro, SCHWARZ PHARMA) is a
new, non-ergot, selective D3/D2/D1 dopamine agonist that has been
formulated in a silicone-based transdermal delivery system.6,7 Pharmacokinetic studies in patients with PD have shown that the continuous
drug delivery from the rotigotine transdermal patch provides doseproportional and stable plasma levels of rotigotine over 24 hours.
Methods: In this open-label, multinational trial, subjects received
once-daily rotigotine doses up to 16mg/24h. Baseline and end of
maintenance UPDRS (motor score) were performed in the morning,
after being hospitalized and taken off medications for 12 hours. In
addition, nger tapping rates were measured in the evening and in the

S69

morning, and number of nocturias and night-time akinesia, dystonia

and cramps were measured.
Results: A total of 54 patients were treated in this trial. Treatment
with rotigotine improved early morning UPDRS III scores off medication by 11.5 6.8 points (P0.0001) comparing baseline score to
rotigotine treatment. The mean NADCS sum score decreased from 3.38
to 1.25 (P0.0001), indicating improvement in both motor performance and sleep-related disorders. The mean daily number of nocturias
decreased from 2.1 to 1.4 (P0.0001). Both morning and evening
average tapping rates increased (from 125 and 136 taps/min at baseline
to 152 and 154 taps/min (P0.0001). on rotigotine, respectively)
indicating improved control of Parkinsons disease. The most common
adverse events were application site reactions and nausea (20% and
19% of subjects, respectively).
Conclusions: In this open-label trial, transdermal rotigotine at doses
up to 16mg/24 h signicantly improved early morning motor state as
well as nocturnal symptoms.
225
Improved motivation and initiative with levodopa, DDCI and entacapone compared with traditional levodopa and DDCI: Pooled
analysis of UPDRS I in four Phase III double-blind studies in
Parkinsons disease patients with wearing-off
H. Nissinen, M. Kuoppamaki, M. Leinonen (Espoo, Finland)
Objective: To evaluate the effect of levodopa, DDCI and entacapone
vs levodopa and DDCI on mentation, behavior and mood in levodopatreated Parkinsons disease (PD) patients with wearing-off.
Methods: A retrospective pooled analysis of four double-blind, placebo-controlled Phase III studies conducted in PD patients with wearing-off symptoms. Patients on levodopa and DDCI were randomized to
either entacapone or placebo and followed for 24 weeks. A total of 808
PD patients (entacapone: 475 vs placebo: 333), were included in
theanalysis. UPDRS I scores at Week 24 were available for 673
patients (entacapone: 382 vs placebo: 291) for ITT-OC analysis and
773 patients (entacapone: 454 vs placebo: 319) for ITT-LOCF.
Results: At baseline, mean age was 62.8 vs 62.6 years, duration of
PD was 9.5 vs 10.5 years, duration of levodopa treatment was 8.1 vs 8.6
years and mean daily dose was 675 vs 696 mg. At baseline (ITT-OC),
the majority of patients in both treatment groups had no symptoms
(severity class 0) in UPDRS I questions (Q) regarding intellectual
impairment (Q1, 68.3 vs 63.6%), thought disorder (Q2, 73.6 vs 80.1%)
or depression (Q3, 63.9 vs 62.9%). Regarding motivation and initiative
(Q4), only half of patients (56.3 vs 54.3%) in both treatment groups
were asymptomatic at baseline, with the remainder comprised of severity class 1 (35.6 vs 38.1%) or severity class 2 (8.1 vs 7.5%). At
Week 24, improvement in severity of motivation and initiation from
baseline was more frequent (16.5 vs 11.3%) and worsening was less
frequent (12.8 vs 18.9%) in levodopa and DDCI-treated patients on
entacapone vs placebo (p0.01, ITT-OC, Cochran-Mantel-Haenszel
test). Similar ndings were seen in the ITT-LOCF analysis (p0.05).
The majority of patients in the total population had no change in
severity from baseline regarding intellectual impairment (Q1, 79.3%),
thought disorder (Q2, 79.9%) and depression (Q3, 75.8%), with no
signicant differences between treatment groups.
Conclusions: In PD patients with wearing-off, levodopa, DDCI and
entacapone provided superior benet in motivation and initiative compared with traditional levodopa and DDCI as measured by UPDRS I
(Q4, mentation, behavior and mood).
226
Validation of PDQ-8 as an independent instrument in English and
Chinese
P.N. Lau, N. Luo, W.L. Au, L.C.S. Tan (Singapore, Singapore)
Objective: Previous validation studies of the PDQ-8 were performed
with the PDQ-8 nested in PDQ-39, a disease-specic quality of life
instrument. We undertook this study to validate the PDQ-8 as an
independent instrument when used apart from the PDQ-39 in English
and Chinese in Singapore.

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: Consecutive patients with Parkinsons disease (PD)

who could read English and Chinese seen at the Parkinsons Disease
& Movement Disorders Centre of National Neuroscience Institute
over a period of 5 months were recruited. All participants met the
NINDS criteria for the diagnosis of PD. PD patients were asked to
self-administer the PDQ-8 in English or Chinese independently
during clinic visits. Patients with concurrent dementia with Chinese
mini mental state examination of equal or less than 20 were excluded. Factor analysis was used to determine the dimensionality of
the PDQ-8.
Results: A total of 183 PD patients completed the PDQ-8, 104 in
English and 79 in Chinese. The item-to-scale correlation was similar
and high for the 8 items of the PDQ-8 for both English and Chinese
version. The correlation coefcients ranged from 0.44 to 0.67 for the
English PDQ-8, and 0.57 to 0.68 for the Chinese version. The
PDQ-8 was also found to be internally consistent (Cronbachs alpha:
0.81 for English version and 0.87 for Chinese). For both versions of
the PDQ-8 a one-factor solution was derived from principal component factor analysis, suggesting the 8 items of the instrument can
be summed to generate a single index score. Factors analysis yielded
a component on which all 8 questions were substantially loaded
(loading range from 0.55 to 0.77). Seree plots also showed the
one-factor solution thereby validating the PDQ-8 summary index.
Construct validation against the Hoehn & Yahr stage and UPDRS
motor scores showed signicant correlations between higher PDQ-8
summary index and increased disease severity in both English and
Chinese version.
Conclusions: Our results show the PDQ-8 to be a valid and reliable
disease-specic HRQoL instrument for PD when used independently of
the PDQ-39.
227
Parkinsons disease (PD) patients retain the capacity for postural
recovery following imposed sensory incongruence
L.A. Brown, S.A. Cooper, J.B. Doan, C. Dickin, I.Q. Whishaw,
O. Suchowersky (Lethbridge, Alberta, Canada)
Objective: To investigate postural control among PD patients following a period of imposed sensory incongruence.
Background: Current theory implicates the basal ganglia for the
integration of sensory information for postural control. This theory
is based on the nding that postural control among PD patients is
compromised when sensory feedback sources convey incongruent
information (Bronte-Stewart et al., 2002). We explored the capacity
of PD patients to recover postural control following imposed sensory incongruence. Our hypothesis was that PD patients would show
continued postural disruption after sensory incongruence ended.
Methods: We assessed standing postural control among medicated PD patients (n 7; Mage 63.6 5.2 yrs) and age-matched
controls (CTRL; n 7; Mage 63.8 4.9 yrs) during a 45 sec
standing trial. Each trial progressed through a 15 sec baseline of
quiet standing (time 0 to 15 sec), a manipulation of sensory
congruency (sustained for 15 sec; time 15 to 30 sec), and a second
period of quiet standing following the termination of the imposed
sensory incongruence (15 sec; time 30 to 45 sec). The congruency
of sensory information was manipulated using a SMART Balance
Master (Neurocom) to provide three conditions of the Sensory
Organization Test: (1) Visual Incongruence (2) Somatosensory Incongruence (3) Visual and Somatosensory Incongruence. Incongruence was achieved by time-locking the movement of the visual
surround and/or the oor to postural sway, such that feedback from
the manipulated sensory system(s) implied constant verticality. The
ability to recover from sensory incongruence was assessed during
the second quiet stance phase (time 30 to 45 sec).
Results: Contrary to our expectation, PD patients showed patterns of
postural recovery similar to CTRL following each sensory incongruence condition.
Conclusions: Despite known disruption to postural control during
periods of sensory incongruence, PD patients appear to retain the
capacity to reintegrate accurate sensory information to control posture.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Our ndings support the suggestion that a fundamental decit for PD

patients may be the capacity to dissociate erroneous sensory feedback
from congruent sources (Smiley-Oyen et al., 2002).
228
Blinding strategies in a clinical trial involving cell transplantation
the STEPS trial
J.E. Jimenez, T. McClain, B. McMurray, M.-L. Musante, D. Turpin,
L. Wilson, H. Steiner (Houston, Texas, USA)
Objective: To evaluate safety, tolerability and efcacy of Spheramine implanted bilaterally into the postcommissural putamen of patients with advanced Parkinsons disease.
Background: Randomized, double-blind, sham-surgery controlled
clinical trials involving brain surgery provide efcacy and safety
data that can not be obtained in open studies. In a phase II clinical
trial studying bilateral implants of human retinal pigment epithelial
(hRPE) cells on microcarriers into the putamen of Parkinsons
disease patients, sham surgery was used as control group treatment.
Although no placebo substance is administered, sham surgery is
invasive and can only be justied if the study results will allow an
unambiguous risk/benet assessment of the investigated therapy.
Study design must provide appropriate biometrical design (discussed elsewhere) and measures for keeping patients, caregivers and
investigators unaware of treatment assignment.
Methods: In the STEPS Trial, 68 patients were randomized for
bilateral implantation of appr 325 000 hRPE cells on microcarriers,
or sham surgery. Surgical procedures: Those were the same for all
patients with the exception of Spheramine injection. Only persons
who had undergone blinding training were allowed in the OR.
Unblinding documentation is stored in a sequestered cabinet. Separation of neurosurgical and neurological teams: Patients underwent 3 months pre-treatment and 24 months follow-up (ongoing) in
a Movement Disorder center close to their residence. For surgery,
patients traveled to the neurosurgical site located in a different
town. Imaging: Pre-surgical imaging was part of the inclusion
criteria and was examined by the neurologist and the neurosurgeon.
An internet-based viewing system with safe access rules admitted
only neurosurgeons to postsurgical images. Blinding of the rater:
Ratings of the primary and secondary endpoints are performed by a
person without access to the patients post treatment assessments
documentation. Emergency unblinding: The protocol allows unblinding of the treating neurologist and access to imaging in case of
medical emergencies.
Results: The trial is still ongoing.
Conclusions: Lavish working procedures for maintenance of the
blind are required to ensure proper conduct of a double-blind, shamsurgery-controlled trial involving brain surgery.
229
Behind the masked face: Emotion self-perception and apathy in PD
A.E. Mikos, B. Skoblar, U. Springer, I.L. Kellison, A. Nisenzon,
H. Fernandez, M.S. Okun, D. Bowers (Gainesville, Florida, USA)
Objective: To learn (1) whether Parkinsons disease (PD) patients
view themselves as experiencing and expressing their emotions as
intensely as others and (2) whether PD self-ratings of expressivity
correlate with traditional measures of mood or disease characteristics.
Background: A masked expressionless face is one of the hallmark
features of PD. Additionally, PD patients may exhibit blunted psychophysiologic reactivity to emotional stimuli (Bowers et al., 2006). The
potential contributions of masked facies and blunted emotional reactivity to self-reports of emotional experience and expression are unknown.
Methods: Twenty-ve PD patients and 20 controls completed two
self-report measures: the Emotional Expressivity Questionnaire
(EEQ) and the Affect Intensity Measure (AIM-s). The EEQ broadly
indexes expressivity, whereas the AIM-s measures intensity of emotional experience. Mood measures included the Beck Depression

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Inventory (BDI-2) and a modied Marin apathy scale (MAI). Clinical disease characteristics included UPDRS total motor score and
levodopa equivalent dosage (LED).
Results: Aim 1: On the EEQ, PD patients rated themselves as
signicantly less expressive than did controls, with no group differences on the AIM-s. Aim 2: For PD patients, higher levels of apathy
were associated with lower affect intensity (AIM-s). Neither expressivity (EEQ) nor intensity (AIM) correlated with BDI scores, LED, or
UPDRS total motor score. In an exploratory analysis, two items from
the EEQ were examined further: item #15 (I experience emotions very
strongly), and item #16 (What I am feeling is written all over my
face). Control ratings of these items were correlated, whereas those of
PD patients were not.
Conclusions: PD patients endorsed similar intensities of experienced emotion as controls, yet viewed themselves as less expressive. This may be due in part to a disconnect between the
experience of emotion and the facial expression of these emotions.
Self-ratings of expressivity and affect intensity were not related to
depression, motor severity, or medication level. Importantly, lower
levels of self-reported affect intensity were associated with greater
apathy. These ndings highlight the importance of apathy and
masked facies in PD patients experience and expression of emotion.
230
Parkinsons disease mimicking multiple system atrophy, with involvement of Onufs nucleus
S.S. OSullivan, J.L. Holton, L.A. Massey, L. Silveira-Moriyama,
D.R. Williams, T. Revesz, A.J. Lees (London, United Kingdom)
Objective: To describe a case of Parkinsons disease mimicking
multiple system atrophy, with pathologically-conrmed involvement of
Onufs nucleus.
Background: Onufs nucleus is a group of neurons located in the
sacral spinal cord, which innervate the external urethral and external
anal sphincter muscles via the pudendal nerve. Degeneration of cells in
this nucleus is one of the classical pathological ndings in MSA.
Involvement in Onufs nucleus has been described in PSP and ALS. To
our knowledge, involvement of Onufs nucleus has not been documented in PD.
Methods: A male patient with the onset of urological dysfunction
at the age of 52, followed by the development of parkinsonism aged
55, is described. Investigations and clinical details were suggestive
of a diagnosis of MSA. He died at the age of 69, and underwent
post-mortem neuropathological examination.
Results: Histological examination revealed a diagnosis of PD, with
neuronal loss in the substantia nigra and the locus coeruleus with
-synuclein immunoreactive Lewy bodies and Lewy neurites in both of
these nuclei. Cortical Lewy bodies were present corresponding with
transitional Lewy body pathology. In the spinal cord -synuclein
immunohistochemical staining revealed small numbers of Lewy bodies
in the intermediolateral column and also scattered Lewy neurites in
Onufs nucleus. No neuronal or glial -synuclein immunoreactive
inclusions of the type associated with multiple system atrophy were
identied.
Conclusions: There is a high prevalence of urinary symptoms in
patients diagnosed with PD, although neurogenic causes of urinary
symptoms in PD have been thought to be due to cerebral rather than
sacral cord pathology. We suggest that pathological involvement of
Onufs nucleus may also be implicated in bladder dysfunction in
PD.
231
Pulmunary dysfunction in uctuating Parkinsons disease
M. Peraino, A. Zarzana, L. Ferri, G. Marsili, P. Rigon, F. Stocchi
(Roma, Italy)
Objective: To dene, characterize, and study possible consequences
of pulmonary dysfunction in patients suffering from Parkinsons disease (PD).

S71

Background: Contradictory results have been reported in pulmonary

function studies in PD patients.
Methods: In this study on idiopathic uctuating PD patients,
nocturnal hemoglobin oxygen saturation, the breathing pattern and
the perception of dyspnea at rest, were measured. Thirty consecutive
non smoker uctuating PD patients (F 18; M 12; mean age 66,33
10,53 yrs) were recruited in the study. Severity of PD according to
the UPDRS motor score was 54.814.9 in OFF and 21.58.7 in
ON. The pulmonary function tests (PFT) included spirometry with
ow-loops, lung volumes and airway resistance by body plethysmography and maximal inspiratory pressure were performed. In all
patients sensation of dyspnea was measured using a modied Borg
scale (0-11).
Results: 26.6% of pts were unable to perform PTF because of low
compliance in ON condition and 33.3% in OFF. Pulmonary function
tests in ON condition was normal in 22.7%, PTF showed obstructive
syndrome in 9.1%, a peripheric obstructions in 18,2%, a restrictive
syndrome in 36,4% and a mixed syndrome in 13.6%. Expiratory
ow peaks (PEF) and maximal inspiratory pressure (MIP) were
80% of predicted value in 68.2%. In OFF motor phases PTF showed
normal value in 10% of pts, obstructive syndrome in 10%, a peripheric obstructions in 15%, a restrictive syndrome in 45% and a
mixed syndrome in 20%. Expiratory ow peaks was 80% predicted values in 70% and MIP in 95%. 54.5% of pts reported
dyspnea at rest in ON phase and 80% in OFF phase. Nocturnal pulse
oximetry showed hemoglobin oxygen saturations 90% for a
recording time 30% of total sleep time in 16.6%.
Conclusions: Our data show that PD patients present pulmonary
abnormalities in about 78% of cases in ON and in about 90% of
cases studied during OFF phase. Restrictive syndrome is the most
frequent pulmonary alteration recorded in ON and in OFF condition
(36,4% and 45% respectively) and MIP results severely reduced in
a large number of patients in both motor phases. The alteration of
dyspnea perception, most evident in OFF condition (80%) could be
due to the bradykinesia and rigidity of thoracic chest muscle.
232
Quantication of subtle movement changes in healthy subjects with
increased echogenicity of the substantia nigra
W. Ilg, I. Liepelt, C. Urban, M.A. Giese, D. Berg (Tuebingen,
Germany)
Objective: Goal of the study was the identication of subtle movement abnormalities of clinically healthy subjects with
hyperechogenicity of the SN to show that specic abnormalities
may represent a functional correlate of hyperechogenicity of the SN,
and potentially an early sign of developing Parkinsons disease
(PD).
Background: Increased echogenicity of the substantia nigra (SN)
in transcranial sonography is found in more than 90% of patients
with PD and 8-10% of healthy adults. This hyperechogenicity has
been suggested as biomarker for PD as well as for increased vulnerability of the nigrostriatal system.
Methods: Out of a cohort of more than 800 subjects older than 50
years without the clinical diagnosis of PD we analyzed different
movements (gait, nger tapping, alternating movements) in 10
randomly selected subjects with normal echogenicity (SN-), 10 with
hyperechogenicity showing no neurological signs (SN) and 10
with hyperechogenicity and mild movement abnormalities (SN).
Movements were recorded with a VICON motion capture system,
and correlations between Parkinson-related features (frequency, amplitude, etc.) and SN echogenicity were determined. Using macine
learning algorithms, subjects were categorized based on the combination of multiple movement features.
Results: In gait, we found a signicant correlation (p0.03)
between arm swing and the echogenicity of the contralateral SN and
a signicant group difference between SN- and SN (p0.03) for
arm swing. However, individual movement features were not sufcient to categorize the subjects into subgroups SN-, SN. Combining classication and feature selection algorithms, we extracted

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

a small set of movement features that categorize the groups SN- and
SN with 93% correct performance. The SN group exhibits thus
subtle movement abnormalities distributed over multiple movement
features.
Conclusions: Correlations between movement features and
SN echogenicity indicate a functional relevance for movement generation. Learning methods allow the identication of subtle movement changes in subjects without neurological signs but with a
vulnerability of the nigrostriatal system and possibly pre-clinical
stages of PD.
233
Tripchlorolide protects against 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced neurotoxicity in C57BL/6 mice
S. Chen, Z. Hong (Shanghai, China)
Objective: To investigate TW397s effect in vivo in the PD model of
1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) lesioned C57BL/6
mice.
Background: Many current theories of Parkinsons disease (PD)
suggest that inammation is involved in the neurodegenerative process.
Tripchlorolide (TW397), a traditional Chinese herbal compound with
anti-inammatory and immunosuppressive properties, has been shown
to protect and restore dopamine neurons against the neurotoxicity
induced by MPP in vitro.
Methods: Tyrosine hydroxylase (TH)-immunoreactive (IR) neurons in the substantia nigra or TH-IR bers in the striatum was
detected by immunohistochemistry. Dopamine (DA), 5-hydroxytryptamine (5-HT), noradrenaline (NE) and their metabolites homovanillic acid (HVA), 3,4-dihydroxyphenylacetic acid (DOPAC)
and 5-hydroxyindoleacetic acid (5-HIAA) were assayed in the
left and right striatum and substantia nigra were measured by
using high-performance liquid chromatography (HPLC) with electrochemical detection.Locomotor skill was evaluated by Rota-Rod
test.
Results: The neurotoxin MPTP reduced the survival ratio of TH-ir
neurons in the substantia nigra pars compacta (SNpc) and TH-ir
bers in striatum to 25.1% and 9.8% of the normal control, respectively. Intriguingly, treatment with TW397 of 1 g/kg for 16 days
once per day dramatically improved the survival rate of TH-ir
neurons in the SNpc and TH-ir bers in striatum to 53.9% and
27.4% of the control, meanwhile, signicantly improved DA
level in SN and striatum to 157% and 191% of the vehicle-treated
group, respectively. In addition, the Rota-Rod performance of the
animals treated with 0.5 g/kg or 1 g/kg TW397 were signicantly improved for about 2 or 3 fold relative to vehicle-treated
animals.
Conclusions: These data demonstrate a neuroprotective action of
TW397 in vivo against MPTP toxicity that has important implications
for the treatment of PD.
234
Correlation of patient quality of life and a clinical rating scale in
advanced Parkinsons disease (PD) results from the PD SURG
trial
S. Mistry, C.E. Rick, N.J. Ives, A. Williams, C. Jenkinson, S. Gill,
T. Varma, K. Wheatley (Birmingham, United Kingdom)
Objective: To compare patient quality of life (QOL) and clinician-assessed physical symptoms of PD in patients in the PD SURG
trial.
Background: Traditionally, composite clinician-assessed rating
scales including measures of motor function and activities of daily
living (ADL) have been used in trials in PD, most commonly the
Unied Parkinsons Disease Rating Scale (UPDRS). There is increasing interest in using patient rated QOL measures to make
results more meaningful to the patients experience. The PD SURG
trial contains a patient population whose PD is not well controlled
by medical therapy. We investigated the relationship between patient QOL and UPDRS in advanced PD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Methods: In PD SURG, patient QOL is assessed using the Parkinsons Disease Questionnaire (PDQ-39) and clinical assessment
by UPDRS. Individual sections of the UPDRS including mentation/behaviour/mood, motor, ADL and complications of therapy
were compared with PDQ-39 scores using standard correlation
methods.
Results: 366 patients were enrolled in the PD SURG trial. 354
(97%) patients completed PDQ-39 at baseline and UPDRS was
performed on 353 (96%), with both available for 347 (95%). The
table shows the strongest (all p0.0001 except * p0.001) correlations (Pearson coefcients given) between PDQ-39 and UPDRS.
In general, the stigma domain of PDQ-39 was not correlated with
UPDRS. The communication and bodily discomfort domains were
correlated with UPDRS ADL, complications and total (off state)
scores (all p0.0001), but less well with other aspects of UPDRS.
The mental domain on UPDRS is correlated with emotional wellbeing, social support, cognition, communication and overall score
on PDQ-39 (all p0.0001).
Conclusions: These results demonstrate that PDQ-39 and UPDRS correlate
strongly with the comparable domains within each instrument in patients with
advanced PD. This implies that it may not be necessary to use both in future
studies, with PDQ-39 perhaps being preferable since it evaluates the patients
own perceptions rather than relying on external judgements.

Pearson correlations between PDQ-39 and UPDRS

UPDRS
PDQ-39

ADL
on

ADL
off

Motor
on

Motor
off

Complications

Total
on

Total
off

Mobility
ADL
Overall Score

0.39
0.48
0.41

0.51
0.46
0.50

0.23
0.30
0.19*

0.27
0.30
0.23

0.38
0.26
0.39

0.36
0.44
0.38

0.42
0.40
0.42

235
How to deal with controversies about iron concentration in parkinsonian and control SN
J. Galazka-Friedman, A. Friedman, K. Szlachta (Warsaw, Poland)
Objective: To review results of studies determining total iron concentration in parkinsonian and control substantia nigra (SN).
Background: Iron may play an important role in neurodegeneration of
SN, the milestone of Parkinsons disease (PD). There were many attempts
to determine the iron concentration in human substantia nigra. While many
authors presented data showing an important increase of the total iron
concentration in parkinsonian SN, several others could not conrm this.
Methods: All experimental results published in the literature in which
the total iron concentration in whole SN was determined were analyzed.
Results: The results for wet tissue measurements are shown in the
table. Wherever the range of the results was given by the authors, or
could be taken from gures, it is given in parentheses. The results are
given in g/g if not otherwise stated.

Iron concentration in control and parkinsonian SN

Year

Method

No of
control

Iron concentration
(range)

1958
1988
1993
1993

col
SPH
TXRF
AA

52
8
5
6

18565
488
410220
14013

1994
1995
1996
2001
2004
2005
2006

ICP
col
MS
NAA
NAA
AA
MS

22
8
8
8
18
40
20

1159379 ng/mg protein

5600400 ng/mg protein
16312 (124-204)
160* (109-199)
175* (100-250)
160** (60-280)
17117 (70-290)

No of
PD

Iron concentration
(range)

8511

6
18
14
6

28122
1813846 (900-4000)
ng/mg protein
4600300 ng/mg protein
15913 (125-200)

3
9

190** (140-240)
17421 (125-231)

* values taken from gures, ** in this study median not mean was given
Col colorimetry, XRF X-ray uorescence, SPH spectropho-

POSTER SESSION I, TUESDAY, JUNE 5, 2007

tometry, TXRF total reection X-ray uorescence, AA atomic
absorption, ICP inductively coupled plasma spectroscopy, MS
Mossbauer spectroscopy, NAA neutron activation analysis.
Conclusions: There is a huge range of the results both in PD and
control. The total iron concentration in control SN ranges from 40 to
410 g/g a factor of 10. Some of these differences could be explained
by differences in methods used, however the main problem is related to
a very large variability of iron concentration both in control and PD.
Computerized simulation dening the number of samples in control
and PD are needed to unequivocal answer whether there is an increase
of the total iron concentration in PD SN.
236
Electroconvulsive therapy for olfactory hallucination in a patient
with Parkinsons disease
E. Hoshiyama, T. Kadowaki, K. Suzuki, M. Tatsumoto, T. Miyamoto,
K. Hirata (Mibu, Tochigi, Japan)
Objective: To report Parkinsons disease (PD) with olfactory hallucinations and its treatment.
Background: Olfactory hallucinations have been rarely described in
patients with PD. In addition, there is few report how to manage
olfactory hallucinations in PD. We report a patient with PD who
showed olfactory and auditory hallucinations.
Methods: A 61-year-old woman presented with tremor of left arm
and bradykinesia in 1998 at the age of 54. She was diagnosed with PD
and started on cabergoline with signicant benet in 2002. Then, the
addition of amantadine hydrochloride and zonisamide ameliorated
tremor and bradykinesia. Since February 2005, she had sudden onset of
olfactory and auditory hallucinations. Subsequently she was admitted
to our hospital.
Results: Although brain MRI was normal ndings, SPECT of brain
showed hypoperfusion in the basal ganglia. Marked reduced uptake was
seen in cardiac 123I-MIBG scintigraphy. Olfactogram was performed and
dissociation level between detection and recognition threshold indicated.
quetiapine temporarily improved olfactory and auditory hallucinations,
however the effect lasted only for three months. Electroconvulsive therapy
(ECT) was performed with signicant benet.
Conclusions: This case report is considered to be important because
olfactory hallucination in PD is very rare. In addition, it was suggested
that ECT may be useful as the treatment of olfactory hallucinations in
PD. For the pathophysiological mechanism of olfactory hallucination,
disproportion between dopaminergic system and serotoninergic system
in brain was suggested.
237
Intracellular presentation of LRRK2- examined by our antiLRRK2-antibodies
K. Hasegawa, H. Ichinose, I. Toyoshima, S. Yaghisita (Sagamihara,
Kanagawa, Japan)
Objective: Attempt to generate anti-LRRK2-antibodies and to clarify
their characterization of LRRK2 expression pattern.
Background: PARK8 parkinsonism that caused by point mutation of
LRRK2 is regard as the most common familial parkinsonism. However, intracellular expression and function of LRRK2 is still obscure.
So, the generation of reliable antibodies against LRRK2 will be an
essential tool to characterize of this protein. We attempt to make
anti-LRRK2 antibodies, and to check its intracellular expression.
Methods: We used LRRK2 peptide (1-198aa in N terminal, and
kinase domain) to generate monoclonal and polyclonal antibodies.
Antibodies were screened against LRRK2 transfected COS1 cells using
standard immunoblot and immunochemistry techniques.
Results: We made two highly specied antibodies against human
LRRK2. Immunohistochemical results were follows: No1 anti-LRRK2
antibodies: 1) Human brain; Most nuclear membrane, nuclei among the
Purkinje cell and neurons in the thalamic nuclei were stained by this
No1 antibodies. However, some neuronal nuclei were not stained by it.
2) Mouse brain; Nuclei and soma in cortical neurons both cerebrum and
cerebellum were stained by No1 antibodies. On the other hands, neu-

S73

rons in deep nuclei were stained only in perikaria. No2 andi-LRRK2

antibodies: 1) Human brain; Neuronal soma, dendrite and axon were
stained this antibodies. Their are co-localized MAP2 and neurolaments. 2)Mouse brain; Stained same as human brain.
Conclusions: 1. We made two specic polyclonal antibodies against
human LRRK2. 2. From the immunohistochemical results, we think
that LRRK2 is concerned protein kinase processing. So, we are now
checking the relation to other neuronal proteins such as cytoskelton and
other proteins which concerned to hereditary parkinsonism.
238
Use of rehabilitation services in Parkinsons disease (PD): A preliminary survey
V. Dahan, S. Chouinard, M. Panisset (Montreal, Quebec, Canada)
Objective: To determine, in a group of patients with PD, 1) the
prevalence of and 2) the reasons for referral to physiotherapy (PT),
occupational therapy (OT), speech therapy (ST), psychology and
social services, 3) the delay between referral and treatments, 4) the
level of satisfaction for the treatments/advices received, and 5) the
unmet needs.
Background: A survey by the Parkinson Disease Society in England
revealed that despite the positive effects of rehabilitation interventions,
only a small proportion of individuals had been referred to rehabilitation services since their diagnosis.
Methods: We devised a questionnaire of 36 questions. Eighty-ve
consecutive patients with PD were asked to ll out this questionnaire.
Results: The prevalence of referral to physiotherapy was 33/85
(38.8%), occupational therapy 24/85 (28.2%), speech and language
therapy 16/85 (18.8%), psychology 16/85 (18.8%), and to social
services 25/85 (29.4%). The most frequent reasons for referral to the
aforementioned services were prevention, technical aids, difculty
with communication, anxiety and link with local health center
services respectively. Mean delay from referral to onset of treatment
was 10.2 weeks. Virtually all patients were said to have found the
therapy/advices to be very or moderately useful. 12/26 of people
who had not been referred to PT would have liked to, 11/25 for OT,
13/27 for ST, 13/40 for psychology and 4/21 for social services.
36/61(59 %) expressed interest in attending information sessions
relevant to PD.
Conclusions: This survey revealed that only approximately a third
of patients had been referred to rehabilitation services since their
diagnosis. The majority waited between 2-3 months to begin therapy. The great majority found it to be useful. Subjective unmet
needs pertaining to referral to the different rehabilitation services
were identied.
239
Knowledge and awareness of Parkinsons disease: A comparison
among various religions and ethnicities in the state of Florida
M.P. Silverstein, V. Gosein, C.E. Jacobson IV, M.S. Okun,
R.L. Rodriguez, H.H. Fernandez (Gainesville, Florida, USA)
Objective: To conduct a pilot study on knowledge and awareness of
Parkinsons disease (PD).
Background: It has been suggested that there may be a tremendous
lack of awareness of Parkinsons disease across the world. In North
America, 1.5 million North Americans are estimated to be suffering
from Parkinsons disease, with approximately 60,000 new cases diagnosed each year. PD affects all ethnic, gender, social, and economic
backgrounds.
Methods: A questionnaire assessing the knowledge and awareness of
PD was developed and distributed to members of local religious organizations and ethnic organizations in the State of Florida during the
groups gatherings. All surveys were given in-person. Survey participants were 18 years or older and were not given prior notice of the
survey topic.
Results: 199 Florida residents (92 males and 107 females) from
various religions and ethnicities participated in the study (see Table 1).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S74

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

A detailed analysis of every question will be presented during the

presentation.
Conclusions: The general knowledge and awareness of PD across
ethnic and religious groups are not uniform. Greater outreach education
is needed to correct this inconsistency.

The percentage of survey participants responding yes to

selected items in the questionnaire.
White
Asian
Black Protestant Catholic Jewish
Hindi
(123/199; (35/199; (21/199; (117/199; (18/199; (16//199; (6/199;
61.8%)
17.6%) 10.6%)
58.8%)
9.0%)
8.0%)
3.0%)
PD is a disease
of the brain.
PD is a disease
of the
muscles.
PD is
commonly
hereditary
linked.
PD initially
presents with
weakness.
Memory loss is
initially
present in
PD.
Shaking is a
feature seen
in PD.
Speech
problems are
seen in PD.
Stiffness is a
feature seen
in PD.
PD is seen only
in old
people.
PD patients live
for 10 years.
There is no cure
for PD.

72.4%

71.4%

28.6%

70.9%

5.6%

68.8%

50.0%

33.3%

25.7%

42.9%

33.3%

38.9%

25.0%

33.3%

47.2%

77.1%

57.1%

46.2%

50.0%

62.5%

50.0%

49.6%

71.4%

33.3%

47.9%

55.6%

25.0%

50.0%

9.8%

14.3%

28.6%

0.9%

5.6%

12.5%

83.3%

96.8%

60.0%

38.1%

83.8%

50.0%

100.0%

83.3%

60.2%

25.7%

38.1%

56.4%

38.9%

43.8%

66.7%

47.2%

60.0%

23.8%

36.7%

55.6%

75.0%

66.7%

8.3%

11.4%

19.1%

5.1%

11.1%

0%

33.3%

25.2%

11.4%

19.1%

23.1%

11.1%

25.0%

16.7%

85.4%

37.1%

28.6%

65.8%

38.9%

93.8%

50.0%

240
Swallowing efciency in Parkinsons disease
L. Leow, M.-L. Huckabee, T. Anderson (Christchurch, New Zealand)
Objective: To evaluate the effects of Parkinsons disease (PD) and
disease severity on swallowing efciency.
Background: Dysphagia is well described in PD but tends not to
be an early predominant symptom. However, as with other complex
coordinated muscle activities in PD, swallowing may be affected by
rigidity and bradykinesia, both of which are early symptoms of PD.
Longer mealtimes and the need for smaller mouthfuls are frequent
complaints in this patient cohort. Hughes and Wiles (1996) describe
a timed test of swallowing efciency that measures number of
swallows, duration, average volume per swallow (ml), average time
per swallow (s) and swallowing capacity (ml/s). This test has been
standardised on healthy adults and validated as a screening test for
impaired oral pharyngeal swallowing biomechanics.
Methods: We administered a validated timed test of swallowing
efciency described by Hughes and Wiles (1996) on 16 healthy
elders, age and gender-matched to 16 patients. We also compared
performance of 16 patients in the early stages (Hoehn-Yahr 1 and II)
to 16 patients in the late stages of PD (Hoehn-Yahr III and IV).
Results: Independent samples t test showed a signicant difference in swallowing capacity between healthy elders (M15.5,
SD5.3) and those with PD [M10.7, SD5.3; t(30)2.14,
p0.016]. Those with PD had signicantly more swallows
(p0.035) and demonstrated signicantly less volume per swallow
(p0.03) compared healthy elders. There were no signicant differences in any of the swallowing efciency measures between
those with mild PD and those with severe PD.
Conclusions: Our data suggests that the presence of disease but
not disease severity affected swallowing efciency. This is contrary
to expectations since symptoms of dysphagia are known to be more

Movement Disorders, Vol. 22, Suppl. 16, 2007

apparent in the later stages PD. Compensatory mechanisms may

play a part but a larger patient sample would be warranted to
conrm this.
241
Neural networks underlying pathological gambling in Parkinsons disease measured by resting-state perfusion SPECT
R. Cilia, C. Siri, G. Marotta, D. De Gaspari, M. Canesi,
I.U. Isaias, G. Pezzoli, A. Antonini (Milan, Italy)
Objective: To determine whether Parkinsons disease (PD) patients
with pathological gambling (PG) exhibit differential brain activity at
rest compared to healthy subjects and non-PG PD controls.
Background: PG is an impulse control disorder that has been reported in patients with Parkinsons disease (PD) on dopamine replacement therapy.
Methods: We studied 11 right-handed PD patients exhibiting PG.
Data were compared with 29 healthy subjects and 40 matched PD
controls (Table1). Patients and their caregivers were interviewed
about gambling and other compulsive behaviors by two experienced
neuropsychologists. None had dementia or previous history of psychiatric illness. Diagnosis of PG was established according to
DSM-IV criteria. All subjects underwent resting state brain perfusion SPECT on-medication using 99mTc-ethylcysteine dimer. Statistical Parametric Mapping (SPM2) was used for data analysis
(p0.005, FDR corrected).
Results: PD gamblers vs. healthy subjects. We found increased
perfusion in a right-hemisphere network that included the orbitofrontal cortex (OFC), anterior cingulate cortex, dorsolateral prefrontal cortex, frontal-opercular cortex, insula, hyppocampus, parahippocampal gyrus and the ventral striatum. We found no areas of
perfusion decrement. PD gamblers vs. PD controls. PD gamblers
showed areas of relative increased perfusion in the right OFC,
insula, hyppocampus, parahippocampal gyrus, globus pallidus and
bilateral precuneus.
Conclusions: We found several areas of signicantly increased
brain activity at rest between PD gamblers and controls. In particular, our analysis outlined that PG in PD is associated with a
right-sided network that involves the OFC and its connections with
cortical (limbic and associative) and subcortical (ventral striatum) regions. The OFC is linked to the mesolimbic dopamine
system that is critical for reward and the OFC-striatum-pallidusthalamus-OFC loop is known to play a key role in emotional
and behavioural responses to stimuli, which are essential components of motivational-directed behavior. We conclude that PD
patients with pathological gambling harbour resting-state abnormal activity within a neural network associated with reward responses.

Table 1.
Disease
Duration

LEDD
(LdopaDA)

DA (% of
LEDD)

UPDRS III

57 5 8.5 3.1
55 7 8.4 5.1
56 6
-

833.3 220.6
911.5 328.0
-

32.5%
37.3%
-

18.0 11.0
19.1 8.5
-

Age
PD gamblers (n11)
PD controls (n40)
Healthy subjects (n29)

Clinical features of PD gamblers, PD controls and healthy subjects.

All values are given as meanSD. LEDD: levodopa equivalent daily
dosage. DA: dopamine agonists. UPDRS III: Unied Parkinsons Disease Rating Scale motor score (on-medication).
242
What aspects of quality of life show the greatest improvement after
DBS among Parkinson patients?
I.U. Haq, M.S. Okun, R. Rodriguez, K. Foote, C. Jacobson,
C. Garvan, H. Fernandez (Gainesville, Florida, USA)
Objective: In this study we propose to look at quality of life (QOL)
measures to assess which aspects of QOL respond to DBS in Parkinsons disease (PD) patients.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Background: Most successes of deep brain stimulation (DBS)
therapy have been reported only on the basis of motor improvement
through the rater-administered UPDRS or through the use of an
on-off diary.
Methods: We compared the pre- and 6 months post-lead implantation PD QOL Questionnaire (PDQ-39) scores of parkinson patients who underwent DBS surgery at the University of Florida. In
our study each of the PDQ s eight subscales (mobility, activities of
daily living, emotional satisfaction, stigma, social support, cognition, communication, and discomfort) were separately summed. We
then looked for correlations between the 6-month-postoperative
PDQ-39 improvement and the subscale scores of each patient. We
then formed difference scores for pre and post DBS procedure for
each (e.g., mobility) and formed comparison scores by subtracting
subscale difference scores (e.g., mobility difference score minus
emotional difference). We analyzed the difference and comparison
scores using the Wilcoxon signed rank test and used a 0.05 level of
signicance for all tests.
Results: 44 PD patients met our inclusion criteria. We found a
signicant overall QOL improvement in mobility (p0.0003), ADL
(p0.0002), emotional satisfaction (p0.0001), stigma (p
0.0001, cognition (p0.0022), and discomfort (p 0.0001). No
signicant improvement was seen in social support or communication. When comparing subscores, mobility was less responsive than
ADL, emotional satisfaction, or stigma. Cognition was less responsive than emotional satisfaction. Other comparisons did not reach
statistical signicance.
Conclusions: Not all aspects of QOL improve uniformly following DBS. While there was no single QOL subscale that improved more than the other subscales, it was interesting to nd
that non-motor QOL subscales responded similarly to motor subscales.
243
The role of telephone counseling in patients with Parkinsons disease
M.S. Kim, S.J. Chung, S.R. Kim, T.Y. Lee, C.S. Lee, M.C. Lee
(Seoul, Republic of Korea)
Objective: We aimed to investigate the role and effects of telephone
counseling for PD patients.
Background: Nonpharmacologic managements are fundamental
elements of the overall management of patients with Parkinsons
disease (PD). Telephone counseling may play a key role in managing PD patients who need optimal adjustment of multiple medications or those having motor complications.
Methods: Between November 2006 and January 2007, we studied
243 PD patients who had regularly followed-up at the Asan Medical
Center out patient clinic. All patients fullled UK Brain Bank criteria
for the diagnosis of PD. Detailed telephone interview and counseling
were made with structured questionnaire.
Results: There were 73 men and 170 women with an age range of
17 to 85 years (mean age, 64.9 9.92 years). Mean age at onset was
59.5 years (range, 14 - 82 years) and mean disease duration was 5.6
years (range, 0.3 - 25 years). The contents of telephone counseling
included adverse effect of anti-parkinsonian medications (24.4%),
aggravation of motor symptoms of PD (18.7%), problems due to
comobidities (17.8%), how to take medicine (13.6%), activities of
daily living (diet, bowel, sleeping and safety) (12.6%), complementary or alternative medicines (3.9%) and knowledge about PD
(3.0%). Persons who used the telephone counseling included the
patient himself (37.9%), offspring (36.2%), spouse (17.7%) and
other relatives (7.4%). Persons who needed the telephone counseling was determined by level of education, sex, cohabitation with
patients and Hoehn-Yahr stage of PD. Contents of telephone counseling were signicantly associated with Hoehn-Yahr stage of PD
and persons who used the telephone.
Conclusions: Our results suggest that a support system with telephone counseling may provide benecial therapeutic intervention in
patients with PD, especially for those with advanced PD. The most
cost-effective method for telephone support needs to be studied.

S75

244
Speech rate in Parkinsons disease
S.K. Skodda, W. Visser, U. Schlegel (Bochum, Germany)
Objective: To analyze articulatory rate and pause time in a standardized reading task subjected to disease duration and motor impairment.
Background: Parkinsonian speech or hypokinetic dysarthria is a
multidimensional impairment of phonation, articulation and prosody.
The dysprosody is characterized by alterations in pitch variation,
speech intensity, speech rate and pause time.
Methods: N108 patients with Parkinsons disease and n71
healthy controls were tested. Disease duration in the parkinsonian
group ranged from 1 to 20 years (mean 6,7 years); UPDRS motor
scale ranged from 4 to 61 points (mean 22,5 pts.). The acoustical
analysis was performed on the rst and last sentence of a standardised 170-syllabic passage using a standard head-worn microphone
for voice recordings and a commercial audio software (WaveLab).
Articulatory rate and speech to pause ratios were obtained by
measurement of the length of each syllable and each pause both at
the end of words and within polysyllabic words. The maximum
vowel keeping time was assessed as a simple parameter of speech
respiration.
Results: No signicant difference in articulatory rate was found
between parkinsonian patients and controls but both groups showed
a higher speech rate in the last sentence compared to the rst. In the
rst sentence, the patient group exhibited a signicantly reduced
percental pause duration relating to total speech time. Percental
pause time within polysyllabic words was reduced in parkinsonian
patients compared to controls. With regard to the number of pauses,
Parkinsonian patients made signicantly less pauses at the end of
words and within polysyllabic words. Parkinsonian patients made
fewer but longer pauses at the end of words. There was no correlation between articulatory rate and number or duration of pauses
and disease specic parameters as motor impairment (UPDRS III)
or subtype of disease (akinetic rigid vs. equivalent type of PD). No
correlation was seen between speech rate and vowel keeping time.
Conclusions: The characteristics of parkinsonian speech uency
in a standardized reading task involves a signicant reduction of
total numbers of pauses, numbers of pauses at the end of words and
numer of pauses within polysyllabic words, indicating an impaired
speech rhythm and timing organisation independent from severity of
motor impairment or subtype of the disease.
245
Restoration of gait for the Parkinsons disease and vascular parkinsonism patients using the tempo-rhythmic correction method
D.V. Pokhabov, V.G. Abramov (Krasnoyarsk, Siberia, Russian
Federation)
Objective: To investigate the efciency of tempo-rhythmic gait
correction method, that was developed in our department.
Background: Parkinsons disease (PD) and vascular parkinsonism
(VP) are accompanied by gait disorders, including the changes in a
rhythmic pattern and increase of step variability (for example, step
variability factor (SVF), which is calculated by formula;
SVF(ASL-min_step_lengthASL-max_step_lenth)/ASL, usually equals - 0,1950,006 for the healthy aged people, 0,7100,011
for PD and 1,1230,009 for VP patients. It is known that under the
effect of rhythmical patterns and music, the improvement can appear.
Methods: We have completed a course of tempo-rhythmic gait
correction for two groups of patients. One group (experimental PD)
contained 40 PD patients, aged 661,8, stage 2-3 of PD, the second
group (experimental VP) contained 30 VP patients, aged
712,5years. For these groups, daily 30-40 minute exercises were
held for 20 days. During these exercises, the patients synchronized
their steps with an optimal tempo of exogenous rhythmic stimulation. The tempo was selected individually. We have used two
control groups (without correction course). Control VP contained 18
patients, aged 732,2years; control PD 21 patient, aged 641,5,

Movement Disorders, Vol. 22, Suppl. 16, 2007

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

S76

stages 2-3. For all the groups medical treatment was preliminary
selected and remained unchanged during the research. Investigation
of gait using the special computer hardware-software complex
Dorozhka in a free tempo mode was held before and after the
rehabilitation course for experimental group (or after 20 days for
control group) (measured average step length (ASL) and SVF).
Results:

Results before and after the rehabilitation course

Group
Experimental
PD
Control PD
Experimental
VP
Control VP

SVF, Before the

course

SVF, After the

course

ASL, Before the

course

ASL, After the

course

0,7300,011
0,7210,016

0,2880,010*
0,4520,013*

42,011,4 cm
44,110,7 cm

48,76,9 cm*
46,311,4 cm

1,1460,021
1,1220,026

0,3120,009*
0,9320,024*

40,99,1 cm
41,58,4 cm

54,57,4 cm*
46,49,2 cm*

*p0,01
ASL increase after the course of rehabilitation, but distinction is not
so expressed. This increase is more in experimental groups vs. control
groups (p0,01). Change SVF is more important and shows efciency
of course of tempo-rhythmic correction of gait, in comparison with data
before - decreases SVF in two times at PD (p0,01) and almost three
times at VP (p0,01). Change SVF in control group is insignicant vs.
experimental groups (p0,01).
Conclusions: The method of tempo-rhythmic correction of gait can
be used at PD and VP, thus is observed restoration of a normal
stereotype of walking, the ASL increases and variability of a step(SVF)
decreases. It is especially interesting, considering an inefciency of
medicines at VP.
246
Hallucinations in Parkinsons disease treated by subthalamic deep
brain stimulation (STNDBS)
S. Aybek, A. Gronchi-Perrin, P. Burkhard, C. Pollo,
F.J.G. Vingerhoets (Lausanne, Vaud, Switzerland)
Objective: To evaluate the evolution of hallucinations in a cohort of
Parkinsons disease patients (PD) treated by STNDBS.
Background: STNDBS, allowing dramatic reduction in medication,
brings important information regarding the debated issue, whether
hallucinations are part of the classical picture of PD or related to
dopaminergic treatment. Hallucinations have been associated to a
higher risk of cognitive decline in medically treated PD but its impact
in surgically treated PD is still uncertain.
Methods: We studied a cohort of 87 non-demented PD patients
treated by bilateral STNDBS who completed a 12-month follow-up
period (mean age 63.18 y, 1410 y PD duration). Hallucinations were
studied using the UPDRS I thought disorder. The levodopa equivalent
dose (LED) was recorded. Dementia diagnosis was based on DSM-IV
criteria.
Results: At baseline 8/87 (9%), patients had hallucinations (UPDRS
I: 2-3) and 18/87 (20%) had vivid dreams (UPDRS I:1). At 12 months,
all hallucinations (LED reduction 100%) and 17 (94%) vivid dreams
had ceased (73% LED reduction). In contrast, post-operatively, one
patient developed hallucinations and seven vivid dreams. These eight
patients had a lower LED reduction (50.3%) then the rest of the cohort
(74%)(p0.05). Patients with baseline hallucinations were older
(69.75.9 y) then those with vivid dreams or without thought disorder
(62.57.8 y)(p0.05) with similar disease durations (12.93.8y and
14.5 5.2y; p0.3). Five patients developed dementia over 12 months;
three of the eight with baseline hallucinations (38%), one with vivid
dreams (5.5%) and one without pre-operative thought disorder (1.6%).
Conclusions: After STNDBS and medication reduction, thought
disorders dramatically decrease in PD. This improvement occurs in
patients with greater LED reduction when new thought disorders appear in patients with lesser reduction, conrming that dopaminergic
medication reveals thought disorders. Even if the STNDBS procedure

Movement Disorders, Vol. 22, Suppl. 16, 2007

decreases hallucinations, the increased risk for the development of

dementia in affected patients remains.

247
Association of catechol O-methyltransferase gene Val158Met
polymorphism with Parkinsons disease
A. Sazci, G. Akpinar, E. Ergul, H.A. Idrisoglu, I. Kara, K. Bayulkem
(Kocaeli, Turkey)
Objective: To determine whether catechol-O-methyltransferase gene
Val 158Met polymorphism is associated with Parkinsons disease.
Background: Parkinsons disease is a neurodegenerative disease
associated with a number of genes. There is no single gene to account
for early parkinsons disease or late one. Hence the search for genes
responsible for Parkinsons disease continues. We were interested in
determining whether catechol-O- methyltransferase gene Val 158Met
polymorphism is associated with Parkinsons disease, since catecholO-methyltransferase (COMT) plays a critical role in the modulation of
dopaminergic systems.
Methods: The study cohort included 225 patients with Parkinsons
disease, and 306 unrelated healthy controls. In the analysis of genotypes, a PCR-RFLP method was used. An amplied 217 bp fragment
was digested with the Hsp 92II restriction endonuclease. The electrophoresis of the digested fragment was carried out at 20 W for 35 min
on 10% polyacrylamide gel followed by silver staining. Statistical
analysis was carried out using SPSS (v12).
Results: We provide evidence for allele and genotype association
between the COMT-L allele, COMT-LL genotype and Parkinsons
disease. The COMT-L allele was signicantly associated with Parkinsons disease (Chi27.667; df2;P0.022). Likewise the COMT-LL
genotype was signicantly associated with Parkinsons disease
(OR1.818; 95%CI1.131-2.920; P0.013). The COMT-LL genotype was overrepresented in patients with Parkinsons disease (20.0%
in Parkinsons disease patients versus 12.1% in controls). The frequency of COMT-L allele was 48.22% in cases and 40.69% in controls.
Conclusions: The COMT-L allele, and COMT-LL genotype were
associated with Parkinsons disease.

248
Perception of Parkinsons disease: How do various religions and
ethnicities compare?
M.P. Silverstein, V. Gosein, C.E. Jacobson IV, M.S. Okun,
R.L. Rodriguez, H.H. Fernandez (Gainesville, Florida, USA)
Objective: To determine differences in perception of Parkinsons
disease (PD) between religions and ethnic groups.
Background: Approximately 1.5 million North Americans are estimated to be suffering from PD with approximately 60,000 new cases
diagnosed each year. PD affects all backgrounds and ethnicities; however, there remains a paucity of knowledge on how groups differ.
Methods: A survey was administered to various and diverse religious
and ethnic groups in the state of Florida to determine differences in
perception of PD. A two page questionnaire was distributed to all members, age 18 or older, of religious and ethnic organizations during typical
group gatherings. The survey was administered without prior introduction
to PD.
Results: 199 Florida residents (92 males and 107 females) from various
religions and ethnicities participated in the study (see Table 1). A detailed
analysis of all the questions will be presented during the presentation.
Conclusions: The perception of PD varies among different ethnicities and religions. More research is needed to determine how the
difference in perception may inuence willingness to seek treatment
and to participate in trials of new therapies.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

S77

250

The percentage of survey participants responding yes to

selected items in the questionnaire.
White
Asian
Black Protestant Catholic Jewish Hindi
(123/199; (35/199; (21/199; (117/199; (18/199; (16//199; (6/199;
61.8%) 17.6%) 10.6%) 58.8%)
9.0%)
8.0%) 3.0%)
PD is a curable
disease.
PD will
spontaneously get
better.
Patients with PD can
live for 15 years.
There is a lot of
research
underway.
If diagnosed with PD,
you would keep it
to yourself.
Being the President is
a hazardous
profession to
perform with PD.
Being a Dentist is a
hazardous
profession to
perform with PD.
It is hazardous to be a
licensed motor
vehicle driver with
PD.

2.4%

14.3%

4.7%

3.4%

11.1%

0%

16.7%

2.4%

5.7%

4.8%

65.8%

1.7%

5.6%

33.3%

21.6%

14.3%

9.5%

19.7%

22.2%

6.3%

16.7%

47.2%

37.1%

33.3%

45.3%

27.8%

50.0%

33.3%

0.8%

11.4%

14.3%

0.9%

16.7%

0%

0%

25.2%

37.1%

38.1%

36.8%

33.3%

31.3%

33.3%

82.1%

48.6%

57.1%

71.8%

72.2%

62.5%

50.0%

74.0%

62.9%

52.4%

67.5%

61.1%

62.5%

66.7%

249
Weight loss in Parkinsons disease (PD)
E.L. Wooff, B. Wood (Wigan, Lancashire, United Kingdom)
Objective: This study aimed to observe uctuations and patterns in the
weight of PD patients, along with levels of investigation and management.
Background: Weight loss is a common sign in PD seen in around
50% of patients (Pfeiffer RF. Gastrointestinal dysfunction in Parkinsons disease. Lancet Neurology. 2(2):107-16, 2003). It has been observed at all stages of the disease. There are a number of possible
explanations including dysphagia, dyskinesia increasing elevated resting energy expenditure (Levi SL, Cox M, Lugon M, Hodkinson M,
Tomkins A. Increased energy expenditure in Parkinsons disease. BMJ
1990;301:1256-1257.), changed dietary habits and depression.
Methods: This study looked at the recording and assessment of
weight loss in 176 patients with idiopathic PD from the initial diagnosis. Weight was recorded annually from 2002 until the present day.
Percentage weight change was calculated yearly. A weight loss of
greater than 5% of body mass was seen as signicant. Other comorbidities known to cause weight loss, further investigations or management for weight loss and whether or not the patient had a dyskinesia
were also recorded.
Results: Whilst most patients showed a small weight loss, a significant weight loss was seen in 50 (22.7%) patients, only ve (10%) of
whom had a co-morbidity known to cause weight loss. An average
weight loss occurred during the rst ve years following diagnosis
though this weight loss was less than 5% of total body mass. At four
and ve years following diagnosis, there was a greater percentage of
patients with a signicant weight loss than those with a small weight
loss. A signicant weight loss was seen in greater proportion of patients
with dyskinesia.
Conclusions: The results suggest that weight loss is a prominent
feature of PD which worsens with time and is possibly explained by the
increasing complexity of the disease. However, the standard deviations
for the sample used were relatively large suggesting the need for a
larger sample size to provide better statistical signicance. Whilst there
is much work to be completed in this area, the fact remains that weight
loss is an important sign seen in all stages of PD. Care needs to be taken
in order to recognise and manage it appropriately.

Sensorimotor integration is abnormal in asymptomatic parkin mutation carriers a TMS study

T. Baumer, P.P. Pramstaller, S. Schippling, H.R. Siebner, C. Gerloff,
C. Klein, A. Munchau (Hamburg, Germany)
Objective: To study asymptomatic carriers of a single mutant Parkin
allele for changes of sensorimotor and motor cortex excitability.
Background: In patients with Parkinsons disease, transcranial magnetic stimulation (TMS) studies have consistently demonstrated a reduced inhibitory tone in the sensorimotor cortex. So far, it remains
unclear whether this is related to motor symptoms or represents adaptive compensatory changes to degeneration of dopaminergic neurons.
Here, we used short-interval afferent inhibition (SAI) and intracortical
paired-pulse inhibition and facilitation to probe intracortical sensorimotor excitability in clinically asymptomatic carriers of a single mutant
Parkin allele who have a latent nigrostriatal dopaminergic dysfunction.
Methods: Nine heterozygous mutation carriers and nine healthy
controls were investigated. For SAI testing, electrical pulses were
applied to the right index nger followed by TMS pulses over the left
motor cortex at interstimulus intervals (ISI) of 25, 30 and 40 ms.
Intracortical paired-pulse excitability was tested at ISIs of 2-15 ms.
Results: SAI was signicantly reduced at an ISI of 25 ms in carriers
of a single mutant Parkin allele, whereas paired-pulse TMS was normal.
Conclusions: We conclude that reduced paired pulse TMS in Parkinsons disease is related to motor symptoms. In contrast, the relative
decrease in sensorimotor inhibition might reect adaptive mechanisms
at the cortical level in response to subcortical neurodegeneration.
251
Sharing medical communication with Parkinsons disease patients
S. OHanlon, L. Robinson, B. Wood, A. Hand, R. Walker (North
Shields, United Kingdom)
Objective: To ascertain whether patients nd it benecial to receive
copies of their clinic letters.
Background: From April 2004 the DOH implemented a policy that
letters about an individuals care will be copied to the patient. PD
Northumbria adopted this policy. 18 months later we undertook a
satisfaction survey. When initiating this policy there were many different things to consider, including letter style, condentiality and
workload.
Methods: A 20 item satisfaction questionnaire was developed by the
team, with input from service users, to reect the sort of information we
felt was important to gather. The questionnaire was sent out to every
patient presently attending the PD clinics in Northumberland and North
Tyneside in October 2005. Pre-paid envelopes were provided. At the
same time a four item questionnaire was sent to medical and nursing
staff asking about practicability, advantages and problems with the new
policy. Secretarial staff were asked about the impact on their time.
Results: 96.1 % of patients thought the letters were a good idea
90.3% of the patients could understand the terminology in the letters
19.7% of patients had needed to clarify information 82.7% of patients
kept their letters Under 1% of patients were offended by their letter.
Conclusions: Almost all of the patients thought getting a copy of
their letter is a good idea and the majority of patients could not think
of circumstances when they would not want a copy. It was suggested
that information about poor prognosis, recurrence or other bad news
was not a reason for withholding a letter, providing that this has been
discussed during the consultation. A good clinic letter should not
contain new information, which has not previously been discussed with
the patient. The letter should be a reection of the consultation which
has taken place and patients should be given time for explanation of
new clinical ndings or management plans. Copy letters should help
patients to understand their condition better, improving empowerment.
Most patients would keep their copy as a reference. Dictating the letter
in front of the patient may increase accuracy and it has been suggested
that it increases attendance rate at out patients.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S78

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

252
Reward processing associated with novelty seeking in Parkinsons
disease
J. Koerts, M. Keitz, M. Van Beilen, K.L. Leenders (Groningen,
Netherlands)
Objective: To investigate whether Novelty Seeking (NS) is related to
reward and whether it is associated with altered reward processing in
Parkinsons disease (PD).
Background: NS is a personality trait which is dened as a tendency
toward novel stimuli and cues for potential rewards which leads to
exploratory activity in pursuit of potential rewards. NS is thought to be
associated with dopaminergic reward processing. PD is characterized
by a dopaminergic decit in the striatum, which might result in low NS.
The association between NS and reward processing in PD was, however, never directly investigated.
Methods: NS was assessed in 23 moderately advanced PD patients
and 23 age and gender-matched healthy controls. A subgroup of 11 PD
patients and 12 healthy controls performed a reward task during fMRI.
Results: PD patients and healthy controls scored equal on NS. In
healthy controls, higher NS scores were associated with relatively
increased left putamen activity in response to monetary reward. In
contrast, in PD patients, higher NS scores were associated with relatively decreased left putamen activity and relatively increased mPFC
region activity in response to monetary reward.
Conclusions: NS may be a reward related personality trait, which is
associated with striatal dopaminergic functioning in healthy controls.
The nigrostriatal degeneration in PD does not seem to result in low NS.
Instead, reduced putamen activity is compensated by increased mPFC
region activity, which may partly mediate NS.
253
Fundamental frequency variation in parkinsonian speech
W. Visser, U. Schlegel, S.K. Skodda (Bochum, Germany)
Objective: Our aim was to analyze pitch variation declared as variation of fundamental frequency (F0) in conversional speech and in a
standardized reading task in patients with Parkinsons disease and
healthy controls in correlation to articulatory rate and disease specic
parameters.
Background: Parkinsonian speech or hypokinetic dysarthria is a
multidimensional impairment of phonation, articulation and prosody.
The dysprosody is characterized by alterations in speech rate and pause
time, speech intensity and pitch variation.
Methods: N100 patients with Parkinsons disease and 70 healthy
controls were tested. Disease duration in the Parkinsonian group ranged
from 1 to 20 years (mean 6,7 years); UPDRS motor scale ranged
from 4 to 61 points (mean 22,5 pts.).The participants had to accomplish a monologue (free speech with a minimum duration of 20 sec) and
a standardized 4 sentence reading task. The acoustical analysis was
performed using a standard head-worn microphone for voice recordings and a commercial audio software (WaveLab /Antares AutoTune).
Articulatory rate was obtained by calculating speech and pause rations
in relation to total speech time. For the determination of fundamental
frequency variation we used a computer analysis program for graphical
description of pitch variation and counted the interval from minimum
to maximum semitone.
Results: F0 variation in conversional speech was signicantly decreased
in parkinsonian patients. In the reading task the patients group showed a
tendency to higher pitch variation but still a signicant reduction of F0
variation in comparison to the control group. No signicant correlation
between F0 variation and participants age and general articulatory rate was
seen. In the parkinsonian group no coherence was found between disease
duration and pitch variation but a negative correlation between motor
impairment (UPDRS motor score) and F0 variation in the reading task. F0
variation in both speech tasks was similar in patients with Parkinsons
diease of akinetic rigid type and equivalent type.
Conclusions: Fundamental frequency variation in Parkinsons disease is signicantly restricted compared to healthy controls. The tendency to a higher pitch variation in the reading task than in conver-

Movement Disorders, Vol. 22, Suppl. 16, 2007

sional speech indicates an inuence of motivation and alertness on

prosody in parkinsonian speech.
254
A new measure for quantifying the bilateral coordination of human
gait: Effects of aging and Parkinsons disease
M. Plotnik, N. Giladi, J.M. Hausdorff (Tel Aviv, Israel)
Objective: To characterize the effect of age and Parkinsons disease
(PD) stages on the level of bilateral coordination of gait.
Background: Mechanisms underlying bilateral coordination of gait
in human subjects are not fully understood. Recently we described that
patients with PD who suffer from freezing of gait (FOG), have reduced
ability to coordinate left/right stepping
Methods: We examined the gait of young adults (n15), elderly
subjects (n11), PD patients in mild stage of the disease (n20), PD
patients in advanced stage of the disease that do not suffer from FOG
(PD-FOG, n13) and PD patients in advanced stage of the disease who
suffer from FOG (PDFOG; n21), during straight line normal walking (i.e. freezing episodes were excluded if occurred). PD patients in
sever stage of the disease were tested both Off and On L-dopa
medication. Subject wore force sensitive insoles timing the events
within gait cycle. Specically, we quantied the stride duration of one
foot as a gait cycle or 360, determined the relative timing of contralateral heel-strikes, and dened this as the phase, (ideally, 180
for every step). The sum of the coefcient of variation of and the
mean absolute difference between and 180 was dened as the Phase
Coordination Index (PCI), representing variability and inaccuracy, respectively, in phase generation.
Results: PCI values varied between the groups. Young adults had the
lowest mean values, and PDFOG patients in Off state had the
highest mean value. See table for detailed results.
Conclusions: In healthy subjects, left/right coordination of stepping
becomes more variable and less consistent with age. Distinctive impairments in the bilateral coordination of gait exist among PD patients
and aggravate with the progression of the disease. FOG in PD is related
to extreme aggravation in bilateral coordination of gait.
Group
PDFOG Off
PDFOG On
PD-FOG Off
PD-FOG On
Mild PD On
Elderly
Young Adults

PCI (%) Mean (SE)

15.1
9.9
7.3
7.0
5.6
3.3
2.5

(3.4)*
(1.8)
(0.7)
(0.9)
(0.6)
(0.2)
(0.3)

*P0.05; PDFOG Off Vs. PD-FOG Off; Mild PD On Vs. Sever

PD On; Mild PD On Vs. Elderely; Young Vs. Elderely
255
Ambulatory monitoring of freezing of gait in Parkinsons disease
S.T. Moore, H.G. MacDougall, W.G. Ondo (New York, New York,
USA)
Objective: The aim of this study was to develop techniques for
detection of freezing of gait (FOG) using an ambulatory gait monitor
(Moore et al. Gait Posture 2006).
Background: FOG occurs sporadically and is often resistant to dopaminergic treatment. Recent laboratory studies have demonstrated
high-frequency components (2-6 Hz) in insole pressure during FOG
(see Bloem et al. 2004). To date there is no objective measure of FOG
and subsequent falls outside the laboratory.
Methods: Patients (10) had a clinical history of FOG, and were
lmed walking (with gait monitor attached to the left shank) pre and
post levodopa administration at 15-min intervals over 90 min. FOG and
standing events were identied post-hoc from video review. Six subjects experienced a total of 44 FOG events (7.3 [SD 5.1]); 4 subjects
did not freeze. FOG was divided into 3 categories; gait initiation (14),
turning (18) and obstacles (doorways, furniture - 12). During FOG,
high-frequency components (2-8 Hz) were present in the vertical ac-

POSTER SESSION I, TUESDAY, JUNE 5, 2007

celeration of the shank that were not apparent during quiet stance (Fig.
1). A simple freeze index (FI) at time t was dened as the area under
the power spectra of a 5 s window of data (centered at time t) in the
freeze band (3-8 Hz), divided by the area under the spectra in the
locomotor band (0.5-3 Hz). This minimized the inuence of high
frequency harmonics ( 2 Hz) during walking on FOG detection. FI
was a dimensionless function (Fig. 2; lower panel) and scaled such that
the largest value encountered was set to 100.
Results: During FOG, peak FI ranged from 0.05 to 100 (7.2 [SD
20.2]), and was larger (p0.03) than peak FI during periods of quiet
standing (0.12 [SD 0.3]). A global threshold was chosen at 0.1 such that
an FI of equal or greater value was dened as a freeze event. Of the 44
FOG events, 34 (77.3%) were detected (Fig. 2). Of the 46 periods of
standing (i.e., intentional, not FOG) from the 10 subjects, 9 (19.6%)
were erroneously marked as FOG. However, establishing an individual
FI threshold for each subject signicantly improved accuracy and
sensitivity of the freeze monitor. The individual threshold ranged from
0.1 to 0.8 (0.22 [SD 0.23]), and increased FOG detection to 39 of the
44 freeze events (88.6%) and decreased false positives to 5 of 46 stand
events (10.9%).
Conclusions: These results demonstrate the feasibility of ambulatory
freeze monitoring in Parkinsons disease.

S79

Background: Pathological substrate of Parkinsons disease (PD) with

dementia (PDD) is not clearly established, but Alzheimers type lesions
are frequently encountered along with Lewy bodies. Although cerebrospinal uid (CSF) phosphotau levels are increased in Alzheimers
disease (AD), few studies have specically addressed their use as
markers of dementia in PD. We present preliminary ndings from an
ongoing study of a large panel of possible CSF markers of dementia in
PD.
Methods: CSF samples obtained from all subjects (ten controls, nine
PD, and nine PDD, matched for age and sex) were centrifuged, aliquoted, and stored at -80C. CSF studies were performed with commercially available phosphotau ELISA kit (Innogenetics, Belgium).
Results: Comparison of CSF phosphotau levels between all three
groups showed no statistically signicant differences (controls:
23.24.5pg/mL; PD: 22.44.7pg/mL; PDD: 23.16pg/mL) (Kruskal
Wallis test; p: 0.96). The highest CSF phosphotau levels detected were
observed in two PDD patients (34.47 and 30.89 pg/mL, respectively).
Conclusions: Although preliminary, these results do not support CSF
phosphotau measurement as a biomarker of dementia in PD. A possible
explanation for such ndings is that even though AD-type lesions are
believed to play a role in PDD, their burden is smaller in this condition
than in AD. Collection of a larger sample and the study of other
putative CSF markers of progression of the disease are warranted and
in progress.
257
Pure Parkinsonian tremor: Clinical follow up study of 23 cases
A. Leventoglu, A.I. Baysal (Ankara, Turkey)

FIG. 1 (255).

256
Cerebrospinal phosphotau levels in Parkinsons disease with and
without dementia
Y. Compta, M.J. Marti, M. Ezquerra, E. Tolosa (Barcelona,
Catalonia, Spain)
Objective: To determine if CSF phosphotau levels are increased in
patients with PDD.

Objective: To analyze the long term outcomes of patients presenting

with pure parkinsonian tremor, and to determine whether or not such
patients develop the other features of Parkinsons disease.
Background: Resting tremor is one of the characteristic features of
Parkinsons disease. The etiology of an isolated resting tremor is still
obscure. The phenomenon has been examined in few studies and is
poorly understood.
Methods: Two hundred fty-one patients with Parkinsons disease
followed at our referral center, were examined regularly. Patients were
divided in two groups as tremor dominant and rigid-akinetic according to their rst symptoms. The tremor dominant group was further
divided into 3 subgroups as patients with tremor as the sole manifestation who dont develop any other signs (T), patients with tremor
who develop rigid-akinetic features (T-AR) and patients with initial
tremor dominant but minimal rigid-akinetic features (T-ar). In this
study we evaluated the long term follow up in thepatients with tremor
as the sole manifestation who dont develop any other signs (T)subgroups.
Results: Forty-four of 251 patients with Parkinsons disease had
patients with tremor as the sole manifestation who dont develop any
other signs (T), followed up for 2 years. In this group, we found 23
patients who typically had pure parkinsonian tremor for over 2 years in
the absence of other features of Parkinsons disease. This study included 7 female and 16 male patients with a mean age of 66.610.8
years (median:65 range: 50-89). The mean tremor duration was
4.82.3 years (median:4 yr, range:2-10) . Four groups of patients were
identied. The rst group consisted of 15 patients followed for an
average of 5.5 years, presenting with rest tremor most prominent in one
lower limb and/or contralateral upper limb. The second group comprised 3 patients followed for an average of 5 years, with isolated jaw
tremor. In the third group there were 3 patients, followed for an average
of 3 years, who had parkinsonian tremor greatest severity in one lower
limb and ipsilateral upper limb. In the fourth group there were only 2
patients, followed for 2.5 years, who had parkinsonian tremor in only
one lower limb.
Conclusions: We suggest that some patients with pure parkinsonian
tremor may remain without any signs of bradykinesia or rigidity for
many years, which we considered to be a benign form of Parkinsons
disease.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S80

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

258
The CISI-PD: Data from a multi-centre study
P. Martinez-Martin, C. Rodriguez-Blazquez, S. Arroyo, M.J. Forjaz,
The ELEP Group (Madrid, Spain)
Objective: To test the metric attributes of the Clinical Impression of
Severity Index for Parkinsons disease (CISI-PD) in a multi-centre
study.
Background: Clinical Global Impression is frequently used in outcomes research (e.g., clinical trials). CISI-PD has recently been proposed as a specic measure of clinical global impression for PD
(Martinez-Martin P et al. Mov Dis 2006; 21: 208-14).
Methods: Participants were PD patients from the Spanish study
ELEP, an observational, multi-centre, longitudinal study (ELEP Group.
Rev Neurol 2006; 42: 360-5). Neurologist-based assessments were the
CISI-PD, Hoehn and Yahr staging (HY), SCOPA-Motor and -Cognition (SCOPA: Scales for Outcomes in PD), and the Cumulative Illness
Rating Scale for Geriatrics (CIRS-G). Patient-based evaluations were
the Hospital Anxiety and Depression Scale (HADS), SCOPA-Autonomic, SCOPA-Sleep, and visual analogue scales for pain and fatigue.
CISI-PD acceptability, scaling assumptions, internal consistency, construct validity, and precision were explored on cross-sectional data
from the baseline assessment.
Results: 290 PD patients were included. Mean CISI-PD total score
was 7.324.10. No patients reached the upper end of the CISI-PD total
score range (0-24). Floor or ceiling effects were absent. Item-total
correlations were above the threshold value in all subscales (r0.440.77). Cronbachs alpha (0.79), and item homogeneity (0.51) were
satisfactory. As a whole, internal validity was adequate, except for
Motor complications-Cognitive status (r0.27). CISI-PD total score
and HY were closely related (r0.78). Most correlations of CISI-PD
with the other scales were low to moderate and slightly higher than
those with HY (table 1). CISI-PD items and total scores signicantly
increased with increasing disease severity (Kruskal-Wallis test,
p0.0001).
Conclusions: CISI-PD shows satisfactory psychometric attributes
and is an eligible measure for clinical global impression of severity in
PD.

Table 1
SCOPA-Motor
SCOPA-Cognition
CIRS-G Index
HADS-Anxiety
HADS-Depression
SCOPA-Autonomic
SCOPA-Sleep (nocturnal sleep)
SCOPA-Sleep (daytime sleepiness)
Pain
Fatigue

CISI-PD

HY

0.83
-0.37
0.24
0.33
0.45
0.42
0.26
0.20
0.18
0.27

0.76
-0.28
0.25
0.22
0.35
0.36
0.19
0.18
0.15
0.19

259
Effects of B-vitamins on plasma homocysteine concentrations in
L-dopa treated Parkinsons disease patients
S. Zoccolella, R. Mastronardi, G. Iliceto, C. dell Aquila,
A. Fraddosio, P. Livrea, P. Lamberti (Bari, Italy)
Objective: Dietary supplementation with folic acid and vitamin B12
lowers blood Hcy concentrations. We examined current evidences
concerning the effectiveness of B-vitamins administration on plasma
hcy levels in L-dopa treated PD patients.
Background: Elevated plasma homocysteine (Hcy) concentrations
are associated with an increased risk for systemic vascular diseases,
Alzheimers disease and vascular dementia. Several cross-sectional
reports and two prospective clinical studies have recently reported
elevated plasma Hcy levels in L-dopa treated Parkinsons disease (PD)
patients. Data from different single observations have raised the question whether Hcy may play a role in the development of long-term
L-dopa motor complications (such as wearing-off, on-off phenomena

Movement Disorders, Vol. 22, Suppl. 16, 2007

and dyskinesias) and in the rate of progression of the disease. Therefore

it is matter of debate whether Parkinsons disease patients should
receive preventative therapy.
Methods: A search for all studies (both randomized, open-label and
cross-sectional) involving B-vitamins in the treatment of L-dopa related Hyperhomocysteinemia was carried out. The date of last search
was 15/2/2007.
Results: At censoring date, 2 published studies have been found. One
was a 4 week prospective open-label study conducted in a tertiary
center from Italy, while the other was a 6-week, multicenter, randomized, double-blind, placebo-controlled trial conducted in Canada. They
included a total number of 55 patients (35 in the randomized trial and
20 in the cross-sectional study). B12 dosage was similar (500 microg/
day) in both studies, while folate dosage was higher in the study from
Italy (5mg/day versus 1mg). In both studies B-vitamin therapy resulted
in a decrease in Hcy compared to placebo (2.64micromol/L in the
randomized trial and 7.4 in the cross-sectional study).
Conclusions: Available data on this argument are very small to date
and concern only a short period of observation. Preliminary data seem
to indicate an effect of B-vitamins in the management of L-dopa related
Hyperhomocysteinemia.

260
The effects of Tai Chi training on general wellbeing and motor
performance in patients with Parkinsons disease (PD): A pilot
study
M.A. Purchas, D.G. MacMahon (Truro, Cornwall, United Kingdom)
Objective: To evaluate (1) safety and tolerability and (2)effects on
general well-being and motor performance of 3 months Tai Chi training
in patients with Parkinsons disease, using the timed up and go test
(TUG), the Parkinsons disease Questionnaire (PDQ 39), and the
Unied Parkinsons Disease Rating Scale (UPDRS).
Background: Tai Chi is a Chinese martial art involving a slow,
carefully coordinated series of movements owing together in one
continuous motion. Tai Chi has been shown to reduce the risk of falls
in elderly patients (Province MA et al, JAMA 1995). Patients with PD
are known to be at risk of falling and could potentially benet from this
intervention.
Methods: Two groups of 10 patients in the maintenance phase of PD
were offered 1 hour of Tai Chi training for 12 weeks. Patients were
randomised to immediate or delayed intervention, each group acting as
crossover controls. UPDRS, PDQ 39, TUG were assessed at baseline,
3 and 6 months. Patients were requested to hold falls diaries and
complete questionnaires about the classes.
Results: Tai Chi training was well tolerated and seemed safe. Subjectively 100% of patients enjoyed and 83% would denitely recommend Tai Chi. 72% of patients felt that their balance had improved.
Falls diaries were not kept. 5 patients noted improvement in their
walking, and others reported improved sleep patterns and fewer headaches. There was no signicant change in mean UPDRS or TUG. Initial
mean PDQ score at 3 months were encouraging: a drop of 3 points in
the intervention group compared with an increase of 2 points in the
control group. However after crossover these were not signicantly
different. The different domains of the PDQ assessment were of interest: there appears to be an improvement in activity of daily living
(ADL), emotional and social support scores but a worsening in stigma
and pain scores compared with controls (non-signicant).
Conclusions: Tai Chi is a well tolerated intervention in patients with
PD with subjective positive effects on balance and walking, but has no
measurable effect on motor performance as measured by UPDRS or
TUG. Tai Chi may improve ADLs, emotional and social support, at the
expense of stigma and pain; but overall well-being appears unchanged,
as measured by PDQ scores. At present there is insufcient evidence of
benet to recommend Tai Chi.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

261
Features on development of dopamine dysregulation syndrome in
patients with Parkinsons disease
G. Kenangil, M. Sohtaoglu, S. Ozekmekci, E. Erginoz (Istanbul,
Turkey)
Objective: To identify the frequency and phenomenology of dopamine dysregulation syndrome (DDS) in patients with Parkinsons disease (PD) and its probable relation to dopaminergic drug use.
Background: A small group of PD patients may develop compulsive
behavioral disorders called DDS. An association between occurrence of
DDS and dopaminergic medications in PD patients was reported.
Methods: Among patients diagnosed as idiopathic PD according to
Brain Bank criteria, followed in a period of three years, we investigated
the frequency of DDS by a given questionnaire. Disease duration,
gender and age-matched PD patients without DDS were selected as
controls. The patients who use only dopamine agonist drugs with or
without levodopa were included. We have noted the age-at-onset and
duration of the disease, PD severity, dose and the types of the dopaminergic treatment as well as presence of motor complications and
REM behavioral disorder (RBD) at the time of the DDS diagnosis.
Results: Of 650 PD patients (421 men, 229 women), 33 patients
(5%) developed DDS, of whom 27 were men (81%). The mean ageat-onset of PD was 48 years. The most frequent behavioral problem
was punding (54%). Compulsive dopaminergic drug use was 21%. The
severity of PD, presence of levodopa-induced motor complications,
levodopa equivalent doses of the dopaminergic agents administered at
the time of DDS diagnosis did not show any statistical difference
between the two groups. Although RBD was more common in patients
with DDS (45% vs 24%), the difference was not statistically signicant.
Conclusions: Our results were compatible with the previous reports
that DDS occurs in a small group of PD patients, frequently in young
onset male patients. Although it was not statistically signicant, RBD
seemed to be more common in patients with DDS. Severity of PD,
disease duration, daily doses and types of dopaminergic agents were
not associated with the emergence of DDS. Since DDS causes social
disruption for patients and a burden for their families, probable predisposing factors on development of DDS should be assessed in larger
series of PD patients.
262
Behavior and hippocampal pathology in parkin null mice overexpressing human mutated tau
J.A. Rodriguez-Navarro, R.M. Solano, M.J. Casarejos, I. Rodal,
A. Gomez, J. Garca de Yebenes, M.A. Mena (Madrid, Spain)
Objective: To explore if parkin deletion increased tau pathology in
mice over expressing human mutated tau (TauVLW).
Background: Patients with mutations of the tau gene in chromosome
17 develop fronto-temporal dementia, parkinsonism and amyotrophy
(FTDPA-17), and tau deposition in brain. But the clinical phenotype of
these patients is variable, suggesting that the impact of the tau mutations is modulated by others factors, perhaps genetic. We have demonstrated that suppression of parkin (PK-/-) in mice over expressing
human mutated tau (TauVLW) increases nigrostriatal dopamine cell
loss, and causes the shortening of the stride, two hallmarks of parkinsonism (Menendez et al. Hum. Mol. Gen. 15: 2045-58, 2006).
Methods: 12-month-old mice were anesthetized and perfused with
4% paraformaldehyde (PFA). The whole brain was immersed in PFA
for 24 h and then included in parafn, sectioned at a thickness of 4
microns, and stained for haematoxylin/ eosine (H&E), Gallyas, glial
brilar acidic protein (GFAP) and phosphorilated tau (AT-8) immunoreactivity were made.
Results: Here we show that the mice with the above described
combined mutation of these two genes have slightly decreased motor
activity, but a notable increment of anxiety as measured in an open eld
test. There was also an increased number of tau immunoreactive
plaques, more astrogliosis and greater number of neurobrillary tangles
in the PK-/-/TauVLW mice.

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Conclusions: PK-/-/TauVLW mice may be a valuable model for test

neuroprotective drugs in tauopathies with parkinsonism.
263
Hipersialorrhea: Treatment with botulinum toxin
F. Vivancos-Matellano, F.J. Rodriguez-Ribera, F.J. Arpa,
E. Diez-Tejedor (Madrid, Spain)
Objective: To analyze the response to treatment with botulnum
toxin, in patients with Parkinsons disease (PD) and different parkinsonism with HS, and the incidence of adverse effects.
Background: Hipersialorrhea (HS) is common in neurodegenerative
diseases. Many treatment options do not exist. Anticholinergic drugs
obtain generally few benets but adverse effect, like hypotension,
dizziness, confusion and orthostatism limit their use. Botulinum toxin
is considered a sound therapeutic option.
Methods: In the last year, 20 patients with criteria diagnosis of PD
(10) and parkinsonism (10) which included: (6) patients with Multiple
System Atrophy (MSA), (2) with Cortico-Basal Degeneration and (2)
with Progressive Supranuclear Paralysis, presenting severe HS (bib
necessity). All of them were treated with botulinum toxin dose (Botox)
(25 ui each parotid gland). The follow-up was made to 6 months, with
evaluation of maximal effectiveness at 2 months, using evaluation
scales of objective and subjective improvement of the patient and
adverse effect.
Results: We found signicant differences on treatment response
between both groups. Most of the patients who suffered parkinsonism
showed better improvement than those who suffered PD (p 0.012).
The adverse effects found were in form of moderate masseterian
atrophy and less frequently dysphagia.
Conclusions: Botulnum toxin is a treatment that can be used in
severe HS that accompany PD and parkinsonism. In our series, the
better response obtained in the latter is remarkable. Probably this effect,
could be explained by a greater compromise of the cholinergic activity
of the Parasympatic Nervous System. Therefore we consider botulinum
toxin as a therapeutic tool for this type of patients.
264
Dopaminergic cells do not show particular susceptibility to proteasomal inhibition
H.-Y. Zhou, Y.-Y. Tan, Z.-Q. Wang, G. Wang, G.-Q. Lu, S.-D. Chen
(Shanghai, China)
Objective: To explore if dopaminergic cells are particularly susceptible to proteasomal inhibition and what detailed mechanisms are
involved in its toxicity.
Background: Ubiquitin proteasome system dysfunction is believed to
play an important role in the development of Parkinsons disease, while
whether it is a primary mechanism still remains to elucidate.
Methods: We treated four representative cell lines, including dopaminergic PC12 cells and SK-N-SH cells, glioma C6 cells and cholinergic SN56 cells, with various concentrations of lactacystin. Then MTT
assay was used to valuate the cellular viability and the AnnexinV/PI
method was used to detect apoptosis. Both cellular soluble and insoluble polyubiquitinated proteins were detected by western blot. Furthermore, the mitochondrial membrane potential was analyzed using JC-1
and the intracellular production of ROS was determined using the
uorescent probe CM-H2DCFDA.
Results: Both dopaminergic cells were less sensitive to lactacystin
toxicity than cholinergic cells. Lactacystin surely inhibited the proteasomes in all cell lines, leading to polyubiquitinated proteins accumulation; however, such accumulation was not linearly correlated with
toxicity. In cholinergic SN56 cells, the amounts of polyubiquitinated
proteins dramatically increased with high concentrations, while the loss
of cells did not increase accordingly. Mitochondrial membrane potential and the reactive oxygen species production were detected, which
also indicated that the damage to both dopaminergic cells was the
mildest and only the highest concentration decreased the potential,
although with no effect on oxidative stress. In contrast, in cholinergic

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cells even low concentrations were enough to decrease the membrane

potentials and concomitantly produce oxidative stress.
Conclusions: Our results suggest that dopaminergic cells are not
particularly susceptible to ubiquitin proteasome system dysfunction,
which exerts its toxic effect by causing mitochondrial dysfunction and
subsequent oxidative stress, not through polyubiquitinated proteins
accumulation.
265
Patient quality of life impacts on carer quality of life in advanced
Parkinsons disease results from the PD SURG trial
C.E. Rick, S. Mistry, N.J. Ives, A. Williams, C. Jenkinson, S. Gill,
T. Varma, K. Wheatley (Birmingham, United Kingdom)
Objective: To examine the relationship between patient and carer
quality of life (QOL) in the PD SURG trial.
Background: Most trials in Parkinsons disease (PD) have used
clinician assessed rating scales (eg. UPDRS) to evaluate disease severity, while a few have assessed the QOL of the patient. Little work has
been done on the QOL of people caring for PD patients. Patients
enrolled in the PD SURG trial have reached the stage where their PD
is no longer well controlled by medical therapy. By this point in the
disease, carers are likely to have to provide an increased level of
support. We evaluated the impact of patients QOL (both physical and
mental) on their carers QOL.
Methods: Patient QOL was assessed by the Parkinsons Disease
Questionnaire (PDQ-39) questionnaire (8 domains, plus overall score),
and carer QOL was assessed by Short Form (SF-36) questionnaire (8
domains, plus a transition score). Standard Pearson correlation methods
were used to compare scores within these two measures.
Results: 366 patients were enrolled into PD SURG; 284 (78%) of
their carers also agreed to take part in the trial. Both baseline PDQ-39
and SF-36 questionnaire were available for 272 (74%) patient/carer
pairs. Patient emotional well-being, social support, communication and
overall PDQ-39 score were all highly correlated with carer physical
problems, emotional problems, social functioning, mental health, energy/vitality, pain, general health perception and overall change in
health of their carer (all p0.01, most p0.0001). Patient mobility,
activities of daily living (ADL), stigma, cognition and bodily discomfort were less well correlated with carer QOL, while carer physical
function was less well correlated with patient QOL. All correlations
observed were positive i.e. poorer patient QOL was associated with
poorer carer QOL.
Conclusions: These results show that poorer patient QOL has a
substantial adverse effect on carer QOL. In particular, non-motor
aspects of patient QOL (emotional well-being, social support, communication) have more impact on carers than physical aspects (mobility,
ADL). This suggests that studies to identify interventions that improve
patient non-motor functioning might have important implications for
carers as well as patients.
266
Detecting uctuations in Parkinsons disease with the Wearing Off
Questionnaire (WOFF)
M. Panisset, H. Turcotte, S. Chouinard (Montreal, Quebec, Canada)
Objective: The goal of this study was to determine how many
patients would be detected with wearing off phenomena with the use of
the WOFF.
Background: The WOFF was designed to be patient self-administered to help detect wearing off symptoms for the busy clinicians. The
patient scored questionnaire was tested against clinician scored questionnaires and showed a high inter rater reliability. Patients were also
tested with a standardized video showing cases of patients with ON,
OFF and dyskinetic states. This showed that patients were able to
recognize accurately the different parkinsonian states.
Methods: We had consecutive patients coming to our clinic ll the
WOFF that was handed to them by a receptionist. No special instructions were provided. This study ran between the 15th of November and
the 22nd of December 2006.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Results: 57 patients were given the WOFF. The mean age was
66,0 13,6 years and the disease duration was 11,5 3,9 years. 52
were taking a levodopa preparation at the time. 100% lled the questionnaire out. Forty answered that they had uctuations and 17 did not.
There was no signicant difference in age (65.9 13.0 vs 66.1 15.3
years) and in duration of disease (9.9 4.8 vs 7.9 5.7 years). Of the
40 uctuating patients, 3 were newly discovered (7.5 %). These three
patients had a disease duration of 3, 6 and 6 years and were 37, 80 and
84 years of age. Two further patients reported subjective OFFs that
could not be clearly dened by the clinician. If these two patients are
taken into account, it brings the rates to 12.5% of uctuators and 8.8%
of all consecutive patients.
Conclusions: This study shows that the WOFF can detect wearing
off symptoms that were not known to exist. We believe that the WOFF
is an efcient way to detect uctuations and save busy clinicians
precious time. The WOFF can be provided in the waiting room with no
specic information.
267
Cardiac failure secondary to rasagiline treatment in two patients
with Parkinsons disease
M. Blazquez-Estrada (Oviedo, Asturias, Spain)
Objective: We report two patients with Parkinsons disease (PD)
who developed cardiac failure secondary to rasagiline treatment.
Background: Although heart failure is predominantly caused by
cardiovascular conditions such as hypertension, coronary heart disease
and valvular heart disease, it can also be an adverse reaction induced by
drug therapy. In addition, some drugs have the propensity to adversely
affect haemodynamic mechanisms in patients with an already existing
heart condition.
Methods: CASE 1: A 68 year-old woman with PD since 2005, being
treated with ropinirole 15 mg/day from the beginning with excellent
response. After one year of monotherapy we added rasagiline 1 mg/day
and two weeks later edema of lower extremities, dyspnea, fatigue and
weight gain appeared in a progressive way. One week later she was
admitted with minimal efforts dyspnea and widespread severe edema.
Investigations revealed moderate tricuspid insufciency and pulmonary
hypertension. CASE 2: A 78 year-old woman with PD since 1992,
without heart or pulmonary known disease, was receiving treatment
with high doses of L-dopa, pramipexole and tolcapone. She developed
disabling off periods and rasagiline 1 mg/day was introduced. Ten days
later progressive edema appeared in lower extremities and spread out
adding dyspnea and arterial hypertension. She was admitted at Emergency Unit with atrial brillation and cardiac failure. The echocardiography revealed slight-moderate tricuspid insufciency.
Results: In case 1 rasagiline was immediately withdrawn and specic
drugs were initiated with an optimum outcome. Four days later she
discharged from hospital. In case 2 she continued taking rasagiline as
well as specic drug therapy during one more month. When she came
to checkup still remained moderate generalized edema and dyspnea
which disappeared after denitive leaving off rasagiline.
Conclusions: In spite of rasagiline is a selective and irreversible
inhibitor of MAO-B that has demonstrated in several trials efcacy and
safety for the treatment of PD, in patients with an already existing heart
condition may precipitate or worsen heart failure.
268
Infections, chronic diseases and trauma as risk factors for Parkinsons disease: A case-control study
J.M. Maksimovic, H.D. Vlajinac, S.B. Sipetic, J.M. Marinkovic,
E.D. Dzoljic, V.S. Kostic (Belgrade, Serbia)
Objective: The objectives of our study were to determine risk factors
for Parkinson s disease (PD).
Background: PD is an important cause of morbidity and mortality in
neurological patients.
Methods: In order to investigate risk factors for PD case-control
study was conducted in Belgrade during the period 2001-2005. The
study comprised 110 newly diagnosed PD cases, and 220 controls

POSTER SESSION I, TUESDAY, JUNE 5, 2007

chosen among patients with degenerative joint disease and some diseases of digestive tract. In the present paper data about association of
PD and some events from past medical history (infectious and noninfectious diseases, surgical operations, head trauma, x-ray diagnostic
and therapeutic radiation) were analyzed.
Results: According to the results of conditional multivariate logistic
regression analysis, PD was signicantly related to rheumatic diseases
(Odds ratio OR 4.52, 95% Condence interval 95% CI
1.19-17.15), allergic diseases (OR 11.96, 95% CI 1.20-119.05),
mumps (OR 7.12, 95% CI 3.05-16.61), inuenza (OR 5.21, 95%
CI 2.84-9.56), herpes simplex infections (OR 10.09, 95% CI
1.65-61.73), and trauma of head (OR 5.49, 95% CI 1.66-18.21).
Association of neurological and psychiatric diseases with PD was at the
borderline of statistical signicance (OR 5.81, 95% CI 1.00-33.78
and OR 4.79, 95% CI 0.99-23.04).
Conclusions: The results obtained suggest that some infectious diseases, immunological disorders and head trauma may play a role in the
development of PD.
269
Angiotensin I-converting enzyme gene I/D polymorphism and Parkinsons disease
G. Akpinar, E. Ergul, A. Sazci, I. Kara, H.A. Idrisoglu, K. Bayulkem
(Kocaeli, Turkey)
Objective: To examine whether angiotensin I-converting enzyme
gene I/D polymorphism is associated with patients with Parkinsons
disease.
Background: Parkinsons disease is one of the most common neurodegenerative disorder. Clinically early onset and late onset Parkinsons disease have been shown to exist. Almost a dozen of genes have
been identied to be involved in Parkinsons disease. The products of
these genes have also been determined to interact with one other.
Therefore, Parkinsons disease is a complex disorder requiring multiple
genes interactions. Angiotensin I-converting enzyme (ACE) is one of
the key enzymes modulating vascular tension and blood pressure. In
humans, serum ACE levels are genetically determined.
Methods: We studied 220 patients with Parkinsons disease, and 321
healthy controls. A polymerase chain reaction was carried out on the
genomic DNA samples isolated from blood. The PCR product was a
190 bp fragment when it was D allele. When the fragment was a 490
bp, it was I allele. The electrophoresis was performed on a 10%
polyacrylamide followed by silver staining.
Results: Neither alleles nor genotypes of the ACE I/D gene were
associated with Parkinsons disease. The distribution of the ACE DD,
ID, and II genetype was 37.3%, 45.0%, and 17.7% in cases and 44.2%,
38.6%, and 17.1% in controls respectively. The ACE I allele was
40.23% in cases and 36.45% in controls. The ACE D allele was 59.77%
in cases and 63.55% in controls.
Conclusions: ACE I/D polymorphism is not associated with Parkinsons disease.
270
Prevalence of LRRK2 mutations in Australians with Parkinsons
disease
Y. Huang, G.M. Halliday, H. Vandebona, G.D. Mellick,
F.L. Mastaglia, J. Stevens, J. Kwok, M. Garlepp, P.A. Silburn,
M.K. Horne, K.E. Kotschet, A. Venn, D.B. Rowe, J.P. Rubio,
C.M. Sue (St. Leonards, NSW, Australia)
Objective: To determine the prevalence of the two most common
LRRK2 gene mutations (G2019S and R1441G/C/H) in a cohort of
Australian patients with PD.
Background: Mutations in the leucine-rich repeat kinase 2 (LRRK2)
gene can cause familial and sporadic Parkinsons disease (PD). Australian population frequencies for LRRK2 mutations are currently unknown.
Methods: We performed PCR/RFLP based genetic analysis for mutations in exons 31 and 41 in the LRRK2 gene in 830 Australian patients

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with PD. We conrmed the presence of mutations using direct sequence analysis. Haplotype analysis was also performed.
Results: Eight were heterozygous for the G2019S mutation and two
were heterozygous for the R1441H (4322 GA) mutation. In addition,
one familial patient had a novel A1442P (4324 GC) mutation. Haplotype analysis showed that all LRRK2 G2019S-carriers had the common founder haplotype and a different founder haplotype for the
R1441H mutation carriers. Patients with LRRK2 mutations were more
likely to have a family history of PD (2.4% of patients with familial PD
and 0.3% of patients with sporadic PD).
Conclusions: Mutations in the LRRK2 gene are less common in
Australian patients with both familial and sporadic PD when compared
to other Caucasian based populations. Australian G2019S mutation
carriers share the same cofounder as European carriers. The G2019S
LRRK2 mutation is more common than mutations in exon 31 (R1441H
and A1442P) in Australian PD patients.
271
The treatment of sialorrhea with botulinum toxin (BTXA) in Parkinsons disease
M. Panisset, L. Spevack, M. Wiseman (Montreal, Quebec, Canada)
Objective: We report here our initial experience with this treatment.
Background: Siallorrhea is a problem in up to 40% of patients with
Parkinsons disease (PD). As it affects patients in their more advanced
stages of the disease, treating sialorrhea with anticholinergics poses a
threat for hallucinations and confusion. Injections of botulinum toxin in
salivary glands have been proposed as a potential treatment.
Methods: We injected 12 patients with PD with disturbing sialorrhea
in either their parotid or their submandibular glands. We measured
unstimulated salivary production, pH and buffering capacity and submitted patients to a questionnaire pre and 1 month post injections.
Results: Patients were 71.3 12.3 years of age, had had PD for
8,8 6,0 years and were at a Hoehn and Yahr stage of 3,5 1,2. At
baseline, salivary production was 0,29 0,25 g/min (normal 1.1
0.7), consistency was 2,4 0,8 (normal 3), pH was 6.0 0.5
(normal 6.8 to 7.8) and buffering capacity of saliva was 3.8 2.5
(normal 10 to 12). Patients received a mean of 86,7 22,3 MU of
BTXA. There was no signicant difference in salivary production,
consistency, pH and buffering capacity of saliva. There was a signicant decrease in the severity of sialorrhea (z -2,692, p0.007) and in
social handicap (z -2,203, p 0.028) as measured on a 1 to 5 point
scale, but no difference on a quality of life scale (PDQ39).
Conclusions: This pilot study shows that BTXA is efcacious in
decreasing sialorrhea and its social impact. Salivary production does
not correlate to the subjective evaluation. Patients with PD have too
little of a poor quality saliva that could affect their teeth. A doubleblind placebo controlled study is important to clarify these issues.
272
Apathy is severe in right-side dominant Parkinsons disease patients
K. Kannari, A. Arai, M. Tomiyama, M. Baba, M. Shoji (Fujisakimachi, Japan)
Objective: (1) To determine the prevalence of apathy in Parkinsons
disease (PD), and (2) to determine the extent to which apathy is
associated with various clinical features, with special interest in the
laterality of symptoms.
Background: Recent clinical studies have demonstrated the presence
of apathy in PD patients. However, there is still controversy on the
relationship between apathy and depression. Little is known which
clinical factors are associated with apathy in PD patients.
Methods: We examined 66 outpatients with PD. Depression was
assessed using Zungs self-rating depression scale (SDS), and apathy
was assessed using Apathy Score (modied from Starksteins apathy
scale). Second, we examined the relation between apathy and various
clinical variables including age, sex, disease severity (Yahrs stage and
the total UPDRS score), and the laterality of symptoms.

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Results: Among PD patients, 58% showed depression (SDS; cut-off

score 40), and 59% showed apathy (Apathy Score; cut-off score
16). With respect to Apathy Score, there are signicant relationships
between Yahr stage (p0.05), total UPDRS score (p0.01), and SDS
(p0.001). However, no relationship was found between depression
and any clinical variables. Apathy Score was signicantly low in
patients with left-side dominant symptoms (p0.05, compared to those
with right-side dominant symptoms; p0.01, compared to those with
equal severity).
Conclusions: At least some part of depression seems to be associated
with apathy. Apathy is much more related to the pathological process
of PD than depression. Higher Apathy Score in right-side dominant PD
patients suggests that apathy in PD may be related to dysfunction of the
left cerebral hemisphere.
273
Emotional experience in early and late stages of Parkinsons disease
S. Fournier, J. Peron, I. Biseul, P. Philippot, S. Drapier, D. Drapier,
M. Verin (Rennes, France)
Objective: To examine emotional experience (i.e. subjective feeling
of emotion) at different stages of Parkinsons disease (PD) in order to
specify the role of the dopaminergic system and the impact of disease
duration on emotion processing.
Background: A large number of studies examined the inuence of
PD and the contribution of the dopaminergic system on the recognition
of emotion. However, to our knowledge, the impact of PD on emotional
experience has not been explored yet.
Methods: A validated battery of lm excerpts (Schaefer et al., in
press) was used to elicit different emotions (anger, happiness, fear,
disgust, sadness and neutral) in 12 newly diagnosed PD patients (early
PD group), 12 patients with advanced PD (advanced PD group) and 14
matched controls. Participants reported the intensity of their emotions
in the Differential Emotion Scale consisting of 10 emotion categories to
be rated on a ve-point scale. Mood state was also assessed through
anxiety and depression scales. Finally, the Early PD patients were
examined in both medicated and unmedicated conditions.
Results: The pattern of emotional experience in the early PD group
was comparable to the one reported in the control group, whereas the
advanced PD group reported a less intense feeling for two negative
emotions, namely anger and disgust, in comparison with the two other
groups. In addition, results showed that emotional experience was
correlated with mood state in the early PD group, but not in the
advanced PD group. Finally, the pattern of emotional experience in the
early PD group was strictly comparable in the medicated versus non
medicated conditions.
Conclusions: The present ndings suggest that changes in emotional
experience in PD patients are not related to the dopaminergic decit but
rather to the course of the disease itself. The gradual diffusion of the
lesions to limbic structures (Braak et al., 2003) may be responsible for
the observed changes.
274
Clinical experience with continuous levodopa infusion therapy in
Parkinsons disease
V. Puente, O. De Fabregues, C. Oliveras, G. Ribera, C. Pont,
G. Cucurella, E. Cuadrado, T. Delgado, J. Espinosa, R. Campo,
A. Seoane (Barcelona, Spain)
Objective: We report our experience using duodenal levodopa infusion in advanced Parkinson disease patients with severe motor uctuations.
Background: With the aim of maintaining a constant dopamine
concentration at the striatal receptors, a continuous duodenal levodopa
infusion (CDLI) therapy has been developed over the past decade, and
has become a new treatment strategy for advanced Parkinson disease
(APD) with motor uctuations.
Methods: We present 7 APD patients (4 men and 3 women) who
were taking an individualized oral and subcutaneous conventional

Movement Disorders, Vol. 22, Suppl. 16, 2007

treatment. All of them suffered motor uctuations with moderate to

severe dyskinesias during On-time that lead to a poor quality of life. All
patients underwent previous treatment tests with levodopa infusion
using a nasoduodenal tube. Later a permanent Percutaneous Endoscopic Gastrostomy (PEG) was performed. Unied Parkinson Disease
Rating Scale (UPDRS) and home diary patients were used for evaluation.
Results: The 7 patients followed this treatment for an average period
of 11 months (range 7-12). All patients increased On-time and decreased Off-time during the infusion period. Average On-time increased from 40 % to 77, 7%, leading to an effective control of motor
uctuations. UPDRS score reduced 23 points average, with a greater
benet in sections II and IV. Time with dyskinesias did not change, but
intensity was considerably reduced. Outcomes remained unchanged
during the follow-up time. Technical problems with the infusion system
were the most common adverse events.
Conclusions: During this period, the increase in daily On-time was
maintained and motor uctuations were successfully reduced by CDLI
as compared to conventional therapy. Amelioration in quality of life
was remarkable. From our experience, CDLI is a successful treatment
for patients with APD when conventional therapy has failed. CDLI
should be taken as an alternative for patients who are poor candidates
or do not tolerate apomorphine infusion or brain stimulation.
275
Hypersexuality in Parkinsons disease
D.A. Gallagher, S.S. OSullivan, A. Schrag, A.J. Lees (London,
United Kingdom)
Objective: Analysis of published case series on hypersexuality (HS)
in Parkinsons disease (PD).
Background: HS in PD has serious social consequences and potential
forensic implications.
Methods: PubMed literature search (N78).
Results: HS occurs predominantly in males 70/74(94.6%), has mean
age of presentation of 57.0 years(11.4), mean age of onset of PD of
46.9 years(11.5), and mean L-DOPA equivalent dose(LED) of
1272mg(1117). Analysis includes two patients with pathologically
conrmed MSA and one of pathologically conrmed PSP. Dopamine
agonists (DA) were used in 62/78 (79.5%) of cases (pergolide 22,
pramipexole 18, bromocriptine 11, ropinirole 10, unknown 1), as
monotherapy in 6 patients. The mean DA LED was 271 mg (4.0mg
pramipexole salt). L-DOPA was used in 70/78 (89.7%) of cases, as
monotherapy in ve. Selegiline was used in 11/78 (14.1%), as monotherapy in two. amantadine was used in 10/78 (12.8%). One case of HS
was linked to moclobemide. Additional impulse behaviour was noted;
excessive shopping (N4), excessive eating (N6) and pathological
gambling (N10). Co-existing psychopathology was noted; depression/cyclothymia (N22), psychosis (visual hallucinations or delusional thought disorder) (N18), hypomania/mania (N9), dopamine
dysregulation(DDS)(N11). Where details of treatment were given
(N51), 7/51 (13.7%) required no pharmacolocgical alterations (4
resolved spontaneously, 3 coping strategies/sexual counselling). In
2/51 (3.9%) cases, HS resolved on switching DA. HS resolved or
improved in 20/51 (39.2%) following reduction of dopaminergic medication: reducing/stopping DA (N12), reducing L-DOPA
alone(N5), stopping selegiline(N2), reducing amantadine (N1) or
stopping moclobemide (N1). A neuroleptic was required in 12/51
(23.5%) of cases (clozapine 6, quetiapine 3, olanzepine 2, thioridazine
1). These often improved but did not resolve HS. In three patients HS
improved on a SSRI, indicated for depression/OCD traits. Valproate
was used successfully (N2) (in one in addition to quetiapine) and
lithium was used in one in addition to olanzepine. One patient, with
frontal lobe dysfunction, responded to donepezil. Two patients responded to STN DBS. There was minimal improvement in two patients.
Conclusions: HS is often associated with other impulse control
disorders and psychiatric sequelae, including mania, psychosis and
DDS.

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276
Effect of UCH-L1 protein on the dopaminergic neurotoxicity of
accumulated -synuclein in vivo
T. Yasuda, K. Wada, H. Mochizuki, Y. Mizuno (Tokyo, Japan)
Objective: The elucidation of molecular pathogenesis and new clinical approaches for Parkinsons disease (PD).
Background: The familial PDs PARK1 and PARK4 are caused by
missense mutations in and multiplication of -synuclein (Syn) gene,
respectively. Syn protein is also known as a major component of
Lewy bodies, a pathological hallmark found in sporadic form of PD.
We have previously reported that the recombinant adeno-associated
viral (rAAV) vector-mediated overexpression of Syn caused a significant dopaminergic (DA) cell loss in rats. The PARK5 is caused by a
missense mutation in ubiquitin carboxy-terminal hydrolase-L1 (UCHL1) generesulting in Ile93Met substitution in its gene product. These
familial PDs are known to be inherited in an autosomal-dominant
mode. However, the PARK5 mutation has been found only in one
German family, and the Ile93Met-substituted UCH-L1 protein was
initially shown to have decreased enzymatic activity. These evidences
raised a controversy whether the Ile93Met mutation gives rise to a
gain-of-toxic-function or loss-of-function of UCH-L1 protein.
Methods: The serotype-1 rAAV vector to produce Syn protein was
introduced into the substantia nigra of the transgenic (Tg) mice that
overexpress wild-type or Ile93Met-substituted UCH-L1 of human origin, and of gracile axonal dystrophy (gad) mice that lack an expression
of mouse UCH-L1 protein systemically.
Results: At 4-weeks post-injection period, a signicant loss of DA
cell bodies was observed in Tg mice that express Ile93Met-substituted
UCH-L1, while there was not an apparent cell loss in Tg mice that
express wild-type UCH-L1 or in non-Tg mice. On the other hand, we
found a signicant DA cell loss in gad mice at 8- and 13-weeks
post-injection periods; however, the decrease of DA cell number was
not signicantly different compared with wild-type littermates both at
8- and 13-weeks post-injection periods.
Conclusions: Our present study strongly supports the hypothesis for
the dominant gain-of-toxic-function mutation of UCH-L1 as the cause
of PARK5.
277
Interleukin-10 gene transfection of C17.2 cells improves behavior
in rat model of Parkinsons disease through inhibition of microglia
activation
X.-J. Wang, W.-G. Liu, Y.-H. Zhang, G.-Q. Lu, S.-D. Chen
(Shanghai, China)
Objective: To investigate whether IL-10 can inhibit immune reaction
after grafting and improve the therapeutic effect of neurotransplantation in Parkinsons disease (PD).
Background: At present, to seek effective measures to inhibit immune reaction after grafting and improve the therapeutic effect of
neurotransplantation has become one of the hot topics in PD research.
Methods: In the present study, c17.2 neural stem cells transfected
with Interleukin-10 gene (IL-10-c17.2 NSCs) were transplanted into
the brains of 6-OHDA-lesioned PD model rats. IL-10 expression and
immune responses after grafting were examined by immunohistochemistry staining. Proinammatory factors (TNF-alpha, NO and superoxide) were assayed in microglia-enriched cultures. Behavioral testing
was performed every month after transplantation.
Results: From 10 to 60 days after grafting, IL-10 expression was
detected in IL-10-c17.2 NSCs in vivo. Behavioral testing indicated that
IL-10 gene transfection improved the rotational behavior of the rats.
Immunohistochemistry analyses revealed that IL-10 gene transfection
of C17.2 cells inhibited intracerebral cellular (ED1 and CD8) and
humoral (C3 and IgM) immune responses in the rats. Correlation
analyses indicated that the effect of IL-10 gene transfection of c17.2
cells on rat behavior was signicantly correlated with its effect on ED1
staining in rat brain, but not with its effect on CD8, C3 and IgM
staining. Furthermore, IL-10 was shown to inhibit the production of
TNF-alpha, NO and superoxide in microglia-enriched cultures.

S85

Conclusions: Our results demonstrate the potential application value

of IL-10 in the transplantation treatment of PD and its inhibition of
microglia activation may be a mechanism for IL-10 neuroprotection.
278
Assessment of different Best Medical Treatment strategies as
potential alternatives to early surgical intervention in Parkinsons
disease
E. Pourcher (Quebec, Quebec, Canada)
Objective: To explore the benets of mood and motor driven treatment strategies (M&M) vs a motor only driven treatment strategy
(M.O.) on the incidence and severity of treatment related motor complications.
Background: Schupbach and co-workers recent paper (Neurology,
January 2007) highlights the notion of Best Medical Treatment in the
comparison of the evolution of treatment induced motor complications
in DBS-treated vs Best Medically Treated patients over an 18
month period. The paper concludes that Deep brain stimulation (DBS)
may represent a better management of mild to moderate stages of
Parkinson disease (PD) in professionally active young onset patients.
The Best Medical Treatment is described as a combination of DA
agonists and Levo-Dopa with secondary addition of entacapone for
wearing-off, and amantadine for emerging dyskinesia. This is theorically and practically recognized as such by most PD specialists, considering a motor status driven pharmacological strategy. This study
evaluates different medical treatment strategies as potential comparators to early surgery.
Methods: Parallel group comparison of UPDRS part IV items, retrospectively collected from a clinical database in 2 groups differing by
the focus of pharmacological treatment during the rst ve years of
management.
Results: 8 patients with disease onset 50 were compared in each
group. These patients did not differ for age, age at onset, disease
duration, Levo-Dopa exposure duration, presence of DA agonists and
average DA agonist dose in pramipexole equivalents. Both groups
differed for Levo-Dopa daily dosage and the presence of entacapone
and amantadine. Non parametric comparison of UPDRS part IV items
dyskinesia (32-35) and item 39 (off duration) signicantly favoured the
M&M group at p 0.05. Unpredictable off (37) tended to be more
prevalent in M.O. patients.
Conclusions: Although the small number of patients precludes a
generalization of these results, the possibility of a better outcome in a
more complex medical approach, currently available, could lead to a
reexamination of the best medical treatment when DBS surgery is
considered a possible alternative. General principles of mood and
motor driven strategies will be further developed.
279
Health-related quality of life in Parkinsons disease patients undergoing deep brain stimulation
C. Kenney, A. Diamond, A. Davidson, L. Shinawi, J. Jankovic
(Houston, Texas, USA)
Objective: To determine the effect of subthalamic nucleus (STN)
deep brain stimulation (DBS) on health-related quality of life (HRQoL)
in Parkinsons disease (PD) patients.
Background: Several studies have concluded that DBS improves
motor function in medically-refractory PD patients; less emphasis has
been placed on HRQoL measures. We sought to capture clinically
relevant endpoints with an emphasis on quality of life (QoL) using
disease-specic instruments.
Methods: PD patients who underwent STN-DBS were assessed
prospectively using several clinical scales at baseline and 6 months
after implantation: Unied Parkinsons Disease Rating Scale (UPDRS), Lang-Fahn Activities of Daily Living Dyskinesia Scale (LFADL), Questions on Life Satisfaction Module (QLSm), EQ-5D, MiniMental Status Examination (MMSE), and Geriatric Depression Scale
(GDS).

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Results: At total of 9 patients (5 male), age 57.2 8.4 years,

consented to be enrolled in this study. The UPDRS motor score (part
III) improved by 36.3% (p0.02) from baseline to 6 months in the
medication off state (Table 1). The UPDRS total score (Part I-III)
decreased 29.3% (p0.03) at 6 months compared to baseline while off
medications. During this same time, UPDRS dyskinesia severity decreased from 1.5 1.4 to 0.4 1.1 (p0.05) while dyskinesia
duration decreased from 1.7 1.2 to 0.4 0.5 (p0.08). The LF-ADL
scale improved signicantly in total score from 13.9 4.8 to 3.7 3.9
(p0.01) and in each individual subscore: handwriting (p0.04), cutting (p0.06), dressing (p0.01), hygiene (p0.01), and walking
(p0.02). Several portions of the QLSm improved signicantly including QoL in relationship to general health (p0.01), hobbies/leisure
activities (p0.05), energy/enjoyment of life (p0.01), inconspicuousness of illness (p0.03), and hand dexterity (p0.02). The EQ5D self
care score improved during the course of this study from 1.8 0.4 to
1.3 0.5 (p0.04). On average, patients were moderately to very
satised with several variables related to the neurostimulator: reliability, inconspicuousness, manipulation, and absence of false bodily sensations. Neither the GDS nor MMSE scores changed appreciably.
Conclusions: Improvements in motor function for PD patients undergoing STN-DBS translate into improved QoL using disease-specic
clinical scales.

Summary of Clinical Endpoints at Baseline and 6 Months

after STN-DBS
UPDRS III off medications
UPDRS III on medications
UPDRS Total (I-III) off meds
UPDRS Total (I-III) on meds
UPDRS Dyskinesia Duration
UPDRS Dyskinesia Severity
LF-ADL
EQ-5D Self Care
Geriatric Depression Scale
MMSE

Baseline

6-month follow-up

Statistical signicance

49.5 4.8
27.4 6.6
70.4 18.3
37.2 16.3
1.7 1.2
1.5 1.4
13.9 4.8
1.8 0.4
4.9 1.0
28.7 0.9

31.5 4.8
17.0 2.9
49.8 20.4
25.0 7.2
0.4 0.5
0.4 1.1
3.7 3.9
1.3 0.5
4.9 1.0
29.7 0.3

p0.02
p0.23
p0.03
p0.23
p0.08
p0.05
p0.01
p0.04
p0.50
p0.48

280
Fluctuations in Parkinsons disease despite deep brain stimulation:
Resurrection of the beast
M.H. Strothjohann, N. Kuehnl, G.A. Fuchs, D. Dschunja (Wolfach,
Germany)
Objective: Deep brain stimulation (DBS) of the subthalamic nucleus
has proven successful in reducing motor symptoms and treating paroxysmal uctuations in Parkinsons disease patients. However, the
effect of mitigating paroxysmal uctuations does not last, at least in
some patients.
Background: In pre- intra- and post-operational periods DBS requires intensive material and personel resources over a considerable
course of time. The most common serious adverse event, intracranial
hemorrhage, is reported in nearly 4%, dysarthria and psychiatric sequalae are common. Non dopaminergic problems (fatal falls due to
axial features and problems with stance and gait) increases nearly
regularly. Benet lasting over time of disease progression and side
effects have to be deliberated to make a decision towards the pros and
cons of the invasive method of DBS.
Methods: We report on three patients with Deep brain stimulation,
who could be treated sucessfully over about 4 years. After that time
paroxysmal fuctuations, hyperkinetic dyskinesias and end of dose dystonia reappeared.
Results: Deep brain stimulation of the subthalamic nucleus (SN) can
alleviate paroxysmal uctuations and hyperkinetic dyskinesias and end
of dose dystonia, but at least in some patients the most severe features
of advanced Parkinsons disease reappeared.
Conclusions: The most wanted effects of DBS can not be taken for
granted over time. At least in some patients, the effect vanished in
about four years. This fact should be taken into consideration especially

Movement Disorders, Vol. 22, Suppl. 16, 2007

in younger patients: the lasting benet is not granted, but the side
effects are common and severe.
281
Presenting symptoms in patients with Parkinsons disease: A prospective, cross-sectional, observational study
P. Stathis, V. Tsagaraki, The Early Symptoms Study Group (Athens,
Greece)
Objective: The aim of the study was to identify early symptoms of
Parkinsons disease (PD) in order to assess the possibility to unmask
high risk subjects for this disease in every day clinical practice.
Background: Neuroprotective and early treatment of PD is promising. Nevertheless, screening the whole population for preclinical PD is
not realistic. Olfactory disturbances, cognitive dysfunction, mood and
sleep disorders, have lately been suggested to precede or accompany
early clinical motor hallmarks of PD.
Methods: A predened questionnaire is administered to patients with
idiopathic PD from neurologists across the country. The questions
referred to: extensive medical history, Hoehn and Yahr (H&Y) disease
severity classication and items concerning the identication of early
symptoms in patients with PD including the following: A) motor
symptoms :Tremor at rest, Rigidity, Gait difculties, Bradykinesia and
other. Laterality was assessed as well. B: non motor symptoms Rapid
Eye Movement (REM) sleep behavior disorder, other sleep disorder,
depression, olfactory dysfunction, Restless legs syndrome (RLS), musculoskeletal pain and other.
Results: Data collected from 1331 patients (56,9% male).A preliminary analysis revealed that the age (mean SD) was 68.8 (3.3) years
and the severity of the disease according the H&Y scale was 2,1(1).The
percentages of patients who reported specic motor or non motor early
PD symptoms and the age when they occurred are shown in table 1.
Early symptoms were predominant at the left side (55%).The probability of depression as initial symptom was 1.45 (p0.001) times
higher and olfactory dysfunction was 0.432 times lower (p0.004) in
women compared to men. Depression was more evident OR2.66 95%
CI[ 1.285-5.516] in lower compared to high education levels while the
opposite occurred for olfactory dysfunction (OR4.362 95% CI[2.09.497].The geographic distribution has an impact in the occurrence of
early PD symptoms.
Conclusions: Patients have difculties in describing exactly the early
and initial PD symptoms. It seems like tremor at rest and depression are
the rst symptoms to report. The sex and the educational level could
interfere with the evolution of depression and the possible olfactory
dysfunction. The geographic area may have an impact on the occurrence of specic motor symptoms.

Frequency of the initial symptoms of PD in clinical practice

Initial symptom
Motor symptoms
Tremor at rest
Walking difculties
Rigidity
Bradykinesia
Non motor symptoms
REM disturbances
Olfactory dysfunction
Depression
RLS
Musculosceletal pain
Sleeping problems

% percentage

Age (mean/-SD)

73.6
22.1
27
17

65.3(8.8)
66.6(8.5)
65.5(8.7)
65.2(10)

9
5
33.7
10.9
28.4
12.2

65.9(9.6)
66.4(9.5)
64.5(9.3)
64.3(9.3)
64.9(9.1)
66.3(8.7)

282
Abnormalities of tau processing in aged parkin null mice
J.A. Rodrguez-Navarro, M.J. Casarejos, R.M. Solano, A. Gomez,
I. Rodal, J. Garca de Yebenes, M.A. Mena (Madrid, Spain)
Objective: We have investigated the putative abnormalities of tau in
aged PK-/- mice.
Background: Abnormal protein processing produces neuronal dysfunction in neurodegenerative disorders which are often related to

POSTER SESSION I, TUESDAY, JUNE 5, 2007

abnormal interactions of these proteins. Tau and parkin have interactions of great interest (J. Menendez et al. Human Mol. Gen. 2006; 15,
2945-2958).
Methods: Parkin-null (PK-/-) and wild-type (WT) mice of 3, 8 and 18
months of age were used for the measurement of their gait. Twomonths and two-year-old mice of each genotype were used for sarcosyl-soluble and insoluble tau protein evaluation. In addition, aged (24
months old) mice were used for histological tau immunoreactivity in
hippocampus.
Results: The Kaplan-Meier curves of survival revealed a reduced life
expectancy in PK-/- mice. The analysis of gait revealed a shortening of
the hind limbs strides and the magnitude of the differences between
both groups increased with age. The levels of both soluble and sarkosyl
insoluble total tau, were increased, by around 200% in the brain of aged
PK-/- mice. Similar differences but of smaller amplitude, were observed
in soluble and sarkosyl insoluble phosphorylated tau (AT-8 antibody).
The number of tau immunoreactive plaques (tau 5 antibody) was
increased in the hippocampus of 22 months-old PK-/- mice.
Conclusions: Our results conrm that the interaction of tau and
parkin is relevant in aged parkin null mice and raises the possibility that
tauopathies may be present in patients with abnormal parkin function.
283
Frequent doses of levodopa/carbidopa/entacapone (Stalevo) are
associated with an improved levodopa plasma prole compared
with traditional levodopa/carbidopa in healthy volunteers
J. Hanninen, K. Korpela, M. Kailajarvi, P. Ruokoniemi,
M. Kuoppamaki, J. Ellmen (Turku, Finland)
Objective: To demonstrate that higher levodopa minimum concentration (Cmin) values are maintained following repeated doses, four
times daily or ve times daily, of levodopa/carbidopa/entacapone (L/
C/E) (Stalevo) compared with levodopa/carbidopa (L/C) (Sinemet)
in healthy volunteers.
Methods: This was an open-label, randomized, 2-period crossover
study with 4 parallel groups. Levodopa dosing schedules were 100 or
150 mg, 4-times daily with 3.5 hourly intervals, or 100 or 150 mg,
5-times daily with 3 hourly intervals in Groups I, II, III and IV,
respectively. In each group, subjects were randomized to receive either
uptitrated L/C/E or L/C during Period 1, subsequently crossing over to
Period 2 without drug-free washout. Blood samples were taken before
the rst dose (0 hr) and thereafter for up to 16 hr (17 hr in Groups III
and IV) for pharmacokinetic (PK) analysis. The following PK values
for levodopa were determined: Cmin (primary variable), area under the
curve (AUC), maximum concentration (Cmax), uctuation and the
elimination half-life (t1/2) after the last dose. Meals were standardized
during the study.
Results: A total of 41 healthy subjects (33 female, 8 male) were
included. Mean plasma levodopa Cmin was signicantly higher following L/C/E treatment in all groups compared with L/C treatment. L/C/E
treatment also demonstrated signicantly higher mean Cmax (p0.05),
and mean levodopa AUC in all groups (p0.0001) compared with L/C.
No difference was seen in uctuation. All treatment regimens were well
tolerated. The most frequent adverse events were nausea and vomiting.
Conclusions: Levodopa Cmin was signicantly higher following
L/C/E treatment in all dosing regimens compared with traditional L/C
treatment, suggesting that L/C/E provides more continuous delivery of
levodopa.
Mean
Cmin (ng/
mL)
Patient
group
I
II
III
IV

Levodopa dosing

L/C/E

4 100 mg (3.5
hourly)
4 150 mg (3.5
hourly)
5 100 mg (3
hourly)
5 150 mg (3
hourly)

566
952

Difference
in Cmin
(ng/mL)

P-value

313

249

0.0001

200

298

506

446

0.0005

265

627

L/C

90% CI
lower

S87

284
Restoration of normal motor control in Parkinsons disease during
REM sleep
V. Cochen De Cock, M. Vidailhet, S. Leu, A. Texeira,
A. Emmanuelle, A. Elbaz, E. Roze, J.C. Willer, J.P. Derenne,
Y. Agid, I. Arnulf (Toulouse, France)
Objective: To characterize the quality of movement during REM
sleep behavior disorder (RBD) in patients with Parkinsons disease.
Background: Although normal subjects do not move during REM
sleep, patients with Parkinsons disease may experience RBD. The
characteristics of the abnormal REM sleep movements in RBD have
however not been studied.
Methods: We interviewed one hundred consecutive non-demented
patients with Parkinsons disease and their bed partners using a structured questionnaire assessing the presence of RBD. They rated the
quality of movements, voice and facial expression during RBD as being
better, equal or worse than in awake ON levodopa condition. Nighttime sleep and movements were video-monitored during polysomnography in 51 patients to evaluate the presence of bradykinesia, tremor,
and hypophonia during REM sleep.
Results: Fifty-nine patients had clinical RBD with 53/59 bed
partners able to evaluate them. All 53 (100%) reported an improvement of at least one component of motor control during RBD. By
history, movements were improved in 87% patients, speech was
better in 77% patients, and facial expression was normalized in 47%
patients. Thirty-eight percent bed partners reported that movements
were much better, even in the most disabled patients. The videomonitored purposeful movements in REM sleep were also surprisingly fast, ample, coordinated, and symmetrical, without obvious
sign of parkinsonism.
Conclusions: REM sleep improves movement in Parkinsons disease. The restored motor control during REM sleep suggests a
transient levodopa-like reestablishment of the basal ganglia loop.
Alternatively, parkinsonism may disappear by REM sleep-related
disjunction between pyramidal and extra-pyramidal systems. We
suggest the following model: the movements during the RBD would
be generated by the motor cortex and would follow the pyramidal
tract bypassing the extrapyramidal system. These movements would
eventually be transmitted to the lower motor neuron because of
brainstem lesions interrupting the pontomedullary pathways which
mediate the REM sleep atonia.

90% CI
upper

901

445

452

0.0005

284

621

1575

798

769

0.0001

563

976

FIG. 1 (284).

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

285

286

Qualitative changes in ultrasonic vocalization in rats after unilateral dopamine depletion or haloperidol
M.R. Ciucci, T.-S. Ma, C.M. Fox, J.R. Kane, L.O. Ramig,
T. Schallert (Austin, Texas, USA)

Early impairment of verbal learning and recall memory in Parkinsons disease with dorsolateral prefrontal and mesio-frontal
dysfunction
S. Bohlhalter, D. Weniger, B. Weder (Tschugg, Switzerland)

Objective: To gain insight into the postulated role of dopamine in

human speech/voice, we analyzed changes in rat ultrasonic vocalization (USV) associated with unilateral infusion of the dopamine
neurotoxin 6-OHDA and low doses of the dopamine antagonist
haloperidol.
Background: The sensorimotor speech/voice decits associated with
Parkinsons disease have been well-documented in humans. They are
largely resistant to pharmacological and surgical treatment, but respond
to intensive speech treatment. The mechanisms underlying this phenomenon are not well understood and are difcult to systematically test
in humans. Thus we turn to the rat as a model.
Methods: Sexually experienced male rats were paired with female
rats in estrous and their USVs were recorded in a sound-treated chamber that isolated male rat calls. USVs were analyzed in terms of
acoustic parameters for the most common type of call (trill) for the
control, 6-OHDA, and haloperidol conditions.
Results: Bandwidth of the USV sonograms were signicantly reduced in both dopamine-altered conditions. The data do not appear to
reect a decreased interest in the female, as all rats mounted the female
and the time to mount was not signicantly different between control
and dopamine-altered conditions.
Conclusions: This is the rst study to document a degradation of
the acoustic signal of frequency-modulated 50-kHz calls as a result
of interfering with dopamine synaptic transmission in rats. The data
suggest that mild transient dopamine depletion with haloperidol or
even unilateral degeneration of dopamine neurons is associated with
changes in the USV acoustic signal. This study provides a foundation to examine the role of dopamine in sensorimotor processes
underlying USV production and potentially to explore treatments
for dopamine deciency-related impaired vocal outcome.

Objective: The aim of the present study was to evaluate the neuropsychological prole in patients with non-demented Parkinsons disease who demonstrated dorsolateral prefrontal and mesio-frontal dysfunction during a discriminatory haptic task.
Background: We previously hypothesized that impaired tactile object recognition in non-demented Parkinsons disease may be related to
executive dysfunction, notably impaired working memory, based on
reduced right dorsolateral and bilateral mesial frontal activation detected by O-15-labeled water PET (Weder et al. 2000).
Methods: Twelve right-handed patients with Parkinsons disease (4
female, age range 41-66) and 12 healthy subjects (6 female, age range
32-64) participated in the study. The patients scored 28.5 1.4
(mean SD) in mini mental status scale and 36.7 19.3 in total
UPDRS. Mean disease duration was 7.7 4.1 years. The neuropsychological test battery included Rey complex gure, Rey auditory
verbal and gural learning test, gural and verbal uency, as well as
Kramer and Stroop test. To assess differences between patients and the
control group unpaired t-tests were performed for all neuropsychological tests. Signicantly different measures (p 0.05) were further
analyzed by univariable logistic regression and the results were expressed as odds ratio (OR) with 95% condence intervals (CI).
Results: The ndings demonstrate signicantly decreased mean total
correct scores for verbal learning (OR 5.48, 95% CI 1.22-24.61, p
0.026) and verbal retrieval (OR 2.05, 95% CI 1.08-3.88, p 0.028) in
patients with Parkinsons disease compared to age-matched normal
controls. No signicant differences were found for the rest of the test
battery, notably verbal and gural working memory as assessed by the
rst trial of the Rey auditory verbal and gural learning test were intact.
Conclusions: The study points to an early impairment of verbal learning
and recall memory in Parkinsons disease, consistent with dorsolateral
prefrontal and mesio-frontal dysfunction, while other executive functions
including verbal and gural working memory are still relatively preserved.
Hence, the ndings raise the question whether working memory related to
object information maybe dissociable and selectively affected in Parkinsons disease, requiring further exploration.
287
PARK2 screening reveals high frequency of unique hotspots and
non smokers among Indian PD population signicantly altering the
parkin expression in blood
S. Prabhakar, M. Bhatia, M. Khullar, A. Anand (Chandigarh, Utah,
India)

FIG. 1 (285).

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To evaluate 1) the contribution of PARK 2 mutations in

the pathogenesis of Parkinsons disease in North India, 2) investigate
the relationship between the PARK 2 and the expression of Parkin in
PBMCs and 3) study the age, demographic and religion based prevalence of mutations.
Background: Parkinsons disease (PD) is a multifactorial, complex age
related disorder characterized by bradykinesia, tremors and rigidity. Several studies have described PARK 2 mutations where several exons have
been reported. It is also believed that smoking, free radicals and lifestyle
also inuences the progression of this disorder, however, the prevalence of
PARK 2 mutations among non-smokers has not been documented earlier.
Also, the evidence of alteration in Parkin expression in the blood as a
marker of PD progression has not been previously established.
Methods: We conducted PCR SSCP, sequencing and FACS analysis
inorder to investigate 70 untreated PD patients and genetically unrelated healthy demgraphic controls. Immunostaining was also performed to demonstrate immunolocalization of Parkin in the leukocytes
of the healthy and PD patients before performing FACS. The study was
cleared from Institute ethical committee and stadard inclusion criteria
was used without invoking UKPDBBCDC regimen.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Results: We report PARK2 mutations constituting 40 % of 70
sporadic Parkinsons disease (PD) patients of North India as analyzed
by PCR-SSCP and sequencing. This also correlated with reduced
parkin expression in the lymphocytes of a proportion of these patients
as analyzed by FACS. There was a signicant predisposition of Sikhs
that were from rural background to PARK 2 mutations. The age of
those patients that displayed mutations as compared to those that did
not was signicantly lower.
Conclusions: This report constitutes the rst evidence that aberration
in parkin protein expression in blood correlates with pathogenesis of
PD. These investigations not only suggest that PARK2 mutations signicantly modify parkin protein expression in such patients, but also
indicate a non-redundant relationship with smoking since all the PD
patients were found to be non-smokers. As demographic controls did
not display any PARK2 mutations, PARK 2 screening can not be ruled
out as tool for diagnosing PD in this subcontinent.
288
Patient factors associated with caregiver strain in Parkinsons disease
H. Munger-Clary, D. Breslow, L. Vainio, T. Simuni (Chicago, Illinois, USA)
Objective: Among Parkinsons disease (PD) patients, to assess the
cross-sectional association between caregiver strain and patient depression, sleep disturbance, social support, subcategories of health-related
quality of life, and PD disease duration.
Background: Caregiver strain is a major predictor of institutionalization of chronic disease patients. Although many PD patients have
informal caregivers, very few studies have assessed the association
between patient factors and caregiver strain.
Methods: For a longitudinal study of PD related disability, 72
participants were recruited from a random sample of university-based
PD center patients. This is a cross-sectional study of the subpopulation
(N44) who identied a primary caregiver. Patients completed a
demographics questionnaire, the PD Sleep Scale (PDSS), the Epworth
Sleepiness Scale (ESS), the PDQ-39, the Geriatric Depression Scale-15
(GDS), and the Lubben Social Network Scale-6 (LSNS). Caregivers
completed the Caregiver Strain Index (CSI), a validated instrument
with scale range of 0 to 13 (7 indicates signicant caregiver strain).
Results: Nine of the 44 (20.5%) caregivers reported signicant strain
(mean CSI score 8.50 vs. 1.78 for those without strain). Caregiver
strain was associated with the following: greater mean duration of PD
(11.4yr vs. 5.37yr; p.002), depression (GDS mean 6.22 vs. 3.25;
p.011), and excessive daytime sleepiness (ESS mean 14.4 vs. 9.65,
p .016). Patients with strained caregivers reported signicantly worse
mean health related quality of life on the following PDQ-39 subscales:
mobility (68.6 vs. 37.9; p.001), ADLs (58.8 vs. 39.7; p.010),
cognition (54.2 vs. 35.4; p.006), and body pain (57.4 vs. 36.3;
p.006). Sample size was insufcient for multivariate analysis. No
signicant association was found between caregiver strain and demographic variables, disease stage (Hoehn & Yahr), social support
(LSNS), quality of nocturnal sleep (PDSS), or the PDQ-39 emotional,
stigma, social, or communication subscales.
Conclusions: Among PD patients, duration of disease, depression,
excessive daytime sleepiness, mobility, ADLs, cognition, and body
pain are associated with caregiver strain. Most of these variables reect
a more advanced stage of the disease, however, some of them like
depression and pain can be effectively treated which should lead to
reduction of caregiver strain.

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the hypothesis that the anterior olfactory structures may be involved

early in PD. Lewy-bodies and severe dopamine cell loss were observed
in the olfactory bulb at neuropathology.
Methods: To investigate the relationship between history of prolonged neurotoxin exposure, such as hydrocarbons and solvents, and
hyposmia in PD patients, using a structured questionnaire. All patients
fullled UK brain bank diagnostic criteria for PD and had been followed at our Parkinson institution for many years.
Results: We evaluated 45 PD patients with severe olfactory dysfunction. Eighteen patients (mean age: 67 ys 10 ys; 13 male, 5 female)
reported no-past exposure (IPD); 27 patients (mean age: 68 ys 9 ys;
22 male, 5 female) presented a prolonged ( 10 ys) history of environmental exposure (expPD). In all patients, exposure was by inhalation and/or skin contact. No differences were observed in motor signs:
UPDRS part III (motor scoreDS) was 177 in IPD and 18-9 in
expPD; Hoehn&Yahr stage was 2 in both groups. Mean duration
(ysSD) of PD was 96. Mean age (ysSD) at onset of PD was
5811 in IPD and 5612 in expPD; mean age (ysSD) at onset of
hyposmia was 5213 and 5514 in IPD and expPD respectively. The
time interval between onset of hyposmia and PD symptoms was signicantly shorter in expPD vs. IPD (-5.6 yrs P 0.05).
Conclusions: Our results suggest that hyposmia precedes PD symptoms and that environmental neurotoxin exposure may inuence disease progression.
290
Food vs levodopa: What worsens hypotension in Parkinsons disease?
M. Ragothaman, N. Sarangmath, S. Koshy, C.J. Mathias,
U. Muthane (Bangalore, India)
Objective: To determine the nature and magnitude of BP fall after
levodopa and food.
Background: Orthostatic hypotension (OH) occurs in 30 to 58% of
Parkinson disease (PD) and is further worsened by food and dopaminergic drugs.
Methods: Eighteen PD patients satisfying the UKPDS criteria were
tested while fasting and in a practical off state for three consecutive
days. OH was diagnosed if there was 20-mmHg systolic BP fall or a
diastolic BP fall of 10 mmHg. Day 1 Liquid meal test: Patients were
given a standard liquid meal. BP and HR were recorded every 3
minutes for 45 mins while supine and after tilting for 15 mins. Day 2
Levodopa challenge test: BP and HR were recorded while supine,
sitting and standing at 15 min intervals for 2 hours after a taking two
pills of levodopa (50/200). Day 3 Liquid meal and levodopa challenge
test: After a standard liquid meal and two pills carbidopa/levodopa
(50/200), BP and HR were recorded every 15 minutes for 3 hours while
supine, sitting and standing.
Results: After tilting 7(38%) patients developed OH. Liquid meal often
causes supine hypotension (n10, 55.5%) than OH (n3, 17%). Levodopa causes OH more often (n6, 33.3%) than supine hypotension (n1,
5.55%). Liquid meal and levodopa when given together frequently cause
OH (n11, 61%) than supine hypotension (n9, 50%). Two had symptoms of OH during liquid meal. During levodopa challenge test 11(61%)
had symptoms at 45 minutes and in 10 it persisted for 60 minutes.
Conclusions: Levodopa and food together cause orthostatic hypotension while food causes supine hypotension. Levodopa induced OH
is symptomatic between 45 and 60 minutes. Food worsens supine
hypotension can cause excessive postprandial drowsiness in PD.

289

291

Olfactory dysfunction and environmental exposure in Parkinsons

disease patients
M. Canesi, I.U. Isaias, G. Pezzoli (Milano, Italy)

Do Israeli patients with Parkinsons disease who carry the LRRK2

G2019S mutation have different professional preferences and occupational background than non-carrier?
C. Shifrin, A. Orr-Urtreger, I. Alroy, A. Hillel, N. Giladi (Tel Aviv,
Israel)

Objective: To investigate the relationship between history of prolonged neurotoxin exposure, such as hydrocarbons and solvents, and
hyposmia in Parkinsons disease (PD) patients, using a structured
questionnaire.
Background: Olfactory dysfunction is amongst the rst and most
prevalent clinical manifestations of (PD). Pathological studies support

Objective: To assess the professional preferences and occupational

background in a large group of Parkinsons disease (PD) patients
carrying the leucine-rich repeat kinase 2 (LRRK2) G2019S mutation
and in a cohort of patients without this specic mutation.

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: PD patients have a tendency to choose specic occupations possibly due to pre-morbid personality trait. Further more, the
occupational exposure might play a role in the risk to develop PD.
Carrying the LRRK2 G2019S mutation might inuence the pre-morbid
personality and as a result carriers of this mutation might choose
different professions and might have a different occupational exposure
to environmental toxins.
Methods: DNA samples from 590 patients diagnosed with PD were
collected and tested for the LRRK2 G2019S mutation from September
2005 to February 2007. All subjects were interviewed regarding their
occupational history, a description of their working environment, the
use of special protection measures, shifts work and the use of toxic
substances, and a detailed database was established. The reported
occupations were sub classied into 15 categories including: agriculture, ofce workers, education, medicine and health professions etc.
Occupational characteristics of LRRK2 G2019S carriers compared to
non-carriers were analyzed using chi-square tests for categorical data.
Results: The occupational variables were determined in 67 PD
patients carrying the LRRK2 G2019S mutation and in 523 PD patients
without this mutation. There were no signicant differences between
non-carriers and carriers in all 15 categories compared (p0.67). We
could not discriminate a specic occupation to be signicantly more
prevalent among the LRRK2 G2019S carries or non-carriers. No signicant differences were noted between carriers and non-carriers with
regard to the self-reported hazardous work environment (p0.76),
self-reported use of hazardous materials (p0.83), or working in shifts
(p0.86).
Conclusions: Our data suggest that occupational preferences and
exposure to environmental toxins in PD are independent variables not
associated with LRRK2 G2019S mutation carrier status.
292
The Mini Mental Parkinson brief cognitive test: Comparison with
the Mattis dementia rating scale in 289 patients with Parkinsons
disease
G. Di Virgilio, A. Leroy, P. Cunin, F. Mahieux, A.-C. Bachoud-Levi,
G. Fenelon (Creteil, France)
Objective: To compare the results of Mini Mental Parkinson (MMP)
and Mattis Dementia Rating Scale (MDRS) in patients with Parkinsons disease (PD).
Background: The MMP (maximum score: 32) is a short cognitive
test which was previously constructed and validated, using specic
cognitive tests, for the identication of cognitive disorders in PD
(Mahieux et al, Behav Neurol 1995;8:15-22). It takes about 10 minutes
to administer. The MDRS (maximum score: 144), a longer scale
assessing general cognitive status, is widely used in the evaluation of
PD.
Methods: 289 consecutive PD patients (mean age: 62.4 8.6 years;
mean duration of PD: 12.2 5.3 years; men: 62%), referred for
neuropsychological examination (preoperative screening in 84% of the
cases), were prospectively evaluated. The association between MMP
and MDRS scores were described using Spearmans rank correlation
and cut-off values were studied using ROC curves. Educational level
(EL) was divided into 3 groups: EL 1 ( 9 years of education), EL 2
(9-12 years) and EL 3 ( 12 years).
Results: Mean scores were for MMP: 28 3 [range: 14-32], and for
MDRS: 134 8 [98-144]. The MMP scores and the MDRS scores
were signicantly correlated (r 0.71, p0.01). The score of the
MMP was correlated with each MDRS subtests (r comprised between
0.24 and 0.54; p0.01). Both MMP and MDRS scores were positively
correlated with higher EL and with younger age. For cut-off values of
130 at MDRS, the most performing cut-off values at MMP were
respectively 28 for EL1 and EL 2 (sensitivity 80% and 77%
respectively, specicity 87% and 78%) and 29 for EL 3 (sensitivity74%, specicity80%).
Conclusions: The high prevalence of dementia or mild cognitive
impairment in PD stresses the need for a brief tool exploring general
cognitive status and adapted to the prole of cognitive decits in PD.
This study demonstrates that MMP scores correlate well with MDRS

Movement Disorders, Vol. 22, Suppl. 16, 2007

scores, further justifying the use of MMP for the screening and follow-up of cognitive disorders in PD.
293
Does learning curve of subthalamic nucleus deep brain stimulation
for advanced Parkinsons disease exist in a developing center? 5
years experience
S.-T. Tsai, S.-H. Lin, S.-Z. Lin, S.-Y. Chen (Hualien, Taiwan)
Objective: We analyzed the surgical results of 5 years experience of
subthalamic nucleus deep brain stimulation (STN-DBS) for advanced
Parkinsons disease (PD) in a single hospital.
Background: STN-DBS has been proved its benet on reduction of
levodopa-related motor complications in advanced PD in large multicenter studies.
Methods: We retrospectively reviewed our consecutive 57 Parkinsonian patients who received STN-DBS during 2002-2006. We compared the results according to the operation done in different time
period. Patients were assessed by the change in the off medication
score of the Unied Parkinsons Disease Rating Scale motor part
(UPDRS-III) and PD cardinal symptoms preoperatively and at least 12
months postoperatively.
Results: The rst 22 patients done STN-DBS in rst 2 years (2002-3)
showed less improvement in change of UPDRS-III comparing to other
35 patients (2004-5) (31% vs 48%, P0.05) 12 months postoperatively. Concerning to each cardinal symptoms (tremor, rigidity, bradykinesia, gait and axial symptoms) and levodopa related drug morbidity
(UPDRS-IV), this signicant different surgical results only exist in
gait, axial symptoms and UPDRS-IV which might result from uneven
benet of symptoms from stimulation. Although there was no signicant difference in UPDRS-III of levodopa drug test between patients
who received STN-DBS in rst 2 years (N22) with operations done
thereafter (N35), it is a trend that rst 22 parkinsonian patients
demonstrated less response to levodopa. Most adverse events were mild
to moderate except one patient with permanent neurological decit due
to intracerebral hemorrhage (ICH). There was 3% incidence of complications which could only be relieved through revision surgery (lead
misplacement, ICH, hardware problems). Another 15% of adverse
events including speech difculty, gait disorders and neuropsychological effects could be relieved through adjustment of stimulation parameters and medication and did not show relation to surgical experience
in different time periods (2002-3 vs 2004-5).
Conclusions: Learning curve did exist. STN-DBS done in single
specialized hospital demonstrated consistent benet and acceptable
adverse event comparing to high-volume centers under strict control of
selection criteria and surgical procedures.
294
Regulation of alpha-synuclein phosphorylation in mammalian cells
D.W. Miller, N.R. Patel, J. Clarimon, M. van der Brug,
M.R. Cookson (Bethesda, Maryland, USA)
Objective: The goal of the current study is to determine the serine/
threonine kinase responsible for phosphorylating serine 129 (Ser129)
of alpha-synuclein in mammalian cells.
Background: Phosphorylation of alpha-synuclein at Ser129 is
thought to enhance the toxicity of this protein. We have previously
shown that this form of alpha-synuclein (Pser129-asyn) is disproportionately elevated in a Lewy body-containing brain region from SNCA
triplication cases. This result supports the theory that hyperphosphorylation of alpha-synuclein plays a critical role in the pathogenesis of
Parkinsons disease. Furthermore, we have also revealed that overexpression of alpha-synuclein drives the phosphorylation of this protein
in cultured dopaminergic M17 neuroblastoma cells. Various kinases,
including casein kinase 2 (CK2), have been implicated in phosphorylating alpha-synuclein although it is unknown which of these phosphorylates alpha-synuclein in mammalian cells.
Methods: To determine if CK2 is the culpable kinase in mammalian
cells, we treated an alpha-synuclein-overexpressing cell line with a
wide concentration range of three different casein kinase inhibitors

POSTER SESSION I, TUESDAY, JUNE 5, 2007

(DRB, TBB, DMAT) that have increasing selectivity for CK2 over
CK1. Cells were exposed to these compounds for 48 hours and
Pser129-asyn levels were determined via western blot analysis. Given
the promiscuous actions of most kinase inhibitors, we also employed
RNA interference to selectively target either CK1 or CK2.
Results: Relatively selective pharmacologic inhibition of CK2 resulted
in a concentration-dependent decrease in Pser129-asyn. CK2 inhibition
was veried by parallel decreases in phosphorylated p53 (Ser392), a
conrmed CK2 substrate. shRNA-mediated kinase knockdown revealed
that a 50% decrease in CK2 results in a 30% decrease in Pser129-asyn.
Knockdown of CK1 had no effect on phosphosynuclein.
Conclusions: Our data indicate that the activity of CK2, but not CK1,
can mediate alpha-synuclein phosphorylation in mammalian cells and may
serve as a therapeutic target for Parkinsons disease. Since CK2 blockade
or knockdown only has a partial attenuating effect on Pser129-asyn levels,
further studies will examine the potential role of other previously implicated kinases (GRK2, GRK5) in the phosphorylation of alpha-synuclein.
295
Ropinirole 24-hour prolonged release as adjunct to L-dopa in
patients with advanced Parkinsons disease efcacy according to
baseline depression score
F. Stocchi, N.P. Stover, L. Giorgi (Roma, Italy)
Objective: Evaluate the efcacy of ropinirole 24-hour prolonged
release in patients with advanced Parkinsons disease (PD), according
to baseline depression score.
Background: Ropinirole is effective in treating PD and a prolongedrelease formulation offers a convenient, once-daily treatment option.
Methods: In the EASE-PD Adjunct study (101468/169), patients not
optimally controlled with L-dopa were randomized to adjunctive ropinirole 24-hour (n202) or placebo (n191), once-daily for 24 weeks.
Initial dose was 2.0 mg/day, titrated to a maximum of 24 mg/day. At
8.0 mg/day and at each subsequent increase, L-dopa dose reduction was
required. Analyses (post hoc) assessed mean changes from baseline in
daily off time and Beck Depression Inventory II (BDI-II) total score
at Week 24 last observation carried forward (LOCF), according to
BDI-II score at baseline: 19 (minimally/mildly depressed; n271) or
19 (moderately/severely depressed; n117). Note that patients with
signicant and/or uncontrolled psychiatric disorders were excluded
from the study.
Results: In the overall population, there was a signicantly greater
reduction in off time (adjusted mean treatment difference [AMTD]:
1.7 hours; p0.0001) and BDI-II total score (AMTD: 1.6;
p0.0130) with ropinirole 24-hour versus placebo at Week 24 LOCF.
There was also a signicantly greater reduction in off time with
ropinirole 24-hour in both BDI-II subgroups (19 group: AMTD: 1.6
hours, p0.0001; 19 group: AMTD 2.0 hours, p0.0001). A
greater reduction in BDI-II score with ropinirole 24-hour versus placebo was observed in both subgroups, with a trend towards greater
reduction in more depressed patients (19 group: AMTD 1.3,
p0.074; 19 group: AMTD 2.6; p0.0598). Mean doses (SD) of
ropinirole 24-hour were 18.7 (6.41) and 19.2 (5.66) mg/day in the 19
and 19 subgroups, respectively.
Conclusions: Ropinirole 24-hour prolonged release reduces off
time, compared with placebo, regardless of depression score (moderately/severely depressed versus minimally/mildly depressed) in patients with advanced PD not optimally controlled with L-dopa.
296
A case report of a deliberate overdose of ropinirole
I. Ahmed (London, United Kingdom)
Objective: To report a case of a single overdose of ropinirole of 110
mg.
Background: Ropinirole is a potent dopamine agonist indicated for
the treatment of Parkinsons disease (PD) as a monotherapy as well as
an adjunct to levodopa therapy. Ropinirole is usually initiated at a dose
of 0.25mg tid and titrated up weekly. After the initial titration, weekly
increments of 0.5 to 1mg tid (1.5 to 3mg/day) may be given. A

S91

maximum dose of 24 mg has been recommended. The largest overdose

in clinical trials was 435mg taken over a 7-day period (62.1 mg/day).
This is the rst case report of a single overdose of 110 mg.
Methods: A 65 year old gentleman presented to an Accident and
Emergency in the Midlands,UK. The patient had taken 22 of 5 milligrams tablets (110 mg in total) at a single go ve hours before
presenting to the AE department. He is a patient of PD for the last seven
years on monotherapy with ropinirole. He was on 20 mg once a day.
Hoehn and Yahr stage 3.UPDRS score was 80. The main complaint
was abdominal pain. He was nauseous. He had dyskinesias in left leg.
There was epigastric tenderness present. Bowel sounds were normal.
Central nervous system examination was normal except brisk deep
tendon jerks bilaterally. There was no peripheral oedema Pulse rate was
74 per minute with a normal blood pressure and there was no signicant postural drop.The symptoms subsided within 8 hours of ingestion
and as per Toxbase advice he was sent home. Examination of his
FBC,UE, LFT and Clotting revealed no abnormality except a neutrophilia of 8.74%, Lymphopenia of 0.64% with a White cell count of 9.6.
Results: The patients symptoms of abdominal pain, nausea and
involuntary movements have been reported earlier in the literature but
marked absence of other recognised features like drowsiness, leg oedema, syncope, hallucinations and postural hypotension are interesting.
Dyskinesias are reported mainly as side effects in adjunct therary with
Laevodopa but in this case it was noted in ropinirole monotherapy.
Conclusions: 1) Ropinirole was quite well tolerated in spite of a
hefty overdose. 2) Increase in the neutrophil/lymphocyte ratio may
have been due to this drug and not previously reported before. 3)It was
interesting to note dyskinetic movements in this patient as he was only
on ropinirole monotherapy for PD.
297
Ability to arise from the oor in persons with Parkinsons disease
J.A. Kraakevik, R.G. Blehm, S. OConnor, C. Tepper, J.G. Nutt
(Portland, Oregon, USA)
Objective: 1) To determine the prevalence of persons with Parkinsons disease who are unable to arise from the oor without assistance,
and 2) To nd factors which may predict who is not able to arise from
the oor safely.
Background: Falls are a major cause of injury and disability in
Parkinsons disease. Previous studies of community-dwelling elderly
population have shown that nearly half have difculty arising from the
oor after a fall. Clinic-based studies of the general elderly population
found that persons who had difculty arising from the oor were much
more likely to sustain an injurious fall. No study to date has looked at
ability to arise from the oor in Parkinsons disease.
Methods: 27 subjects with idiopathic Parkinsons disease without
concurrent orthopedic or other neurologic diseases were asked to arise
from a prone position on the oor while in on state. They were given
a score based ability to arise independently (1safe, 2safe but
required assistance of a chair, 3unsafe). Subjects also answered a
brief questionairre including self-reported history of recent falls, and
were assessed with UPDRS, Hoehn and Yahr staging, Get up and go
test, and ABC balance scale. Using Spearmans correlation coefecient, we assessed relationships between difculty arising from the oor
and UPDRS, Hoehn and Yahr, ABC scale, and Get up and go testing.
Results: Mean age of the subjects was 69.6 years old (49-82) with
mean UPDRS of 23.1 (6-50). Twenty one subjects (78%) were able to
arise from the oor safely and without assistance, ve (18%) could
arise only with assistance of a chair, and one (3.7%) was deemed
unsafe to arise indepedently. Using Spearmans correlation testing, we
found that the oor rise score was signicantly correlated with history
of falls in the last month (R2 .606, p0.001), motor UPDRS (R2 583,
p0.002), Hoehn and Yahr stage (R2 .589, p0.013) (gure 1), and
ABC balance scale (R2 .443, p0.031). There was no signicant
correlation between oor rise score and get up and go testing.
Conclusions: Among Parkinsons disease subjects, the ability to
arise from the oor is correlated with markers of disease severity and
self-reported history of falling. More work needs to be done to deter-

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

mine if including oor rise assessment to standard physical therapy

protocols will help predict persons likely to fall.

References:
1. Katzenschlager, R.; Lees, A. J. Curr. Opin. Neurol. 17,
417-423 (2004).
2. Braak, H. et al. Aging 24, 197-211 (2003).
3. McKeith, I. G. et al. Neurology 65, 1863-1872 (2005).
299
Cortical atrophy patterns in PSP and MSA patients detected via
3D cortical morphometry on MRI
D. Tosun, S. Duchesne, A.W. Toga, C. Barillot, Y. Rolland, M. Verin
(Los Angeles, California, USA)

FIG. 1 (297).

298
The primary olfactory cortex in Parkinsons disease (PD) and
incidental Lewy body disease (ILBD)
L. Silveira-Moriyama, J.L. Holton, A. Kingsbury, H. Ayling,
W. Sterlacci, W. Poewe, H. Maier, A.J. Lees, T. Revesz (London,
United Kingdom)
Objective: To evaluate the involvement of the piriform cortex and
olfactory tubercle (Tu) by -synuclein (-syn) pathology in PD and
ILBD.
Background: Loss of smell occurs in 70-100% of PD patients1.
Braak and colleagues2 proposed that the olfactory bulb and tract are
among the sites affected by -syn pathology early in the disease. They
also described that cortical areas related to olfaction are affected in
subsequent stages of pathology corresponding to ILBD. Nevertheless
-syn pathology in the primary olfactory cortex has not been systematically examined.
Methods: The forebrain and mesial temporal cortex were sampled in
7 ILBD, 10 PD and 4 control cases. Ten m thick parafn sections
were stained with luxol fast blue/cresyl violet method and used for
immunohistochemistry with a monoclonal antibody to -syn (Novocastra). The severity of Lewy body and Lewy neurite pathology was
graded in the frontal piriform cortex (PiF), temporal piriform cortex
(PiT) and olfactory tubercle (Tu), using the approach proposed by
McKeith et al3 for the assessment of DLB. Data were analyzed using a
two-factor mixed factorial ANOVA.
Results: None of the control cases was affected by -syn pathology,
but this was present in all the PD and ILBD cases. There was a
signicant difference in the severity of -syn load between the three
areas of interest (p0.012). A Bonferroni pair-wise comparison
showed a signicant difference in -syn burden between Tu and PiT
(p0.022), but only a trend was seen between PiF and PiT (p0.054).
Conclusions: Our data support the notion that the primary olfactory
areas are affected in ILBD and PD. The data also show that there are
signicant regional differences in the severity of -syn pathology, the
piriform cortex being more severely affected than the olfactory tubercle. Therefore, the components of the primary olfactory cortex should
be assessed independently for -syn pathology in PD and ILBD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To study cortical atrophy in progressive supranuclear

palsy (PSP),multiple systems atrophy (MSA) and idiopathic Parkinsons disease (IPD) as seen on MRI using 3D morphometry techniques.
We aimed to test the hypothesis that there exists group differences in
cortical atrophy patterns among PSP,MSA and IPD.
Background: Differential diagnosis of patients with Parkinsonian
syndromes is a challenging but clinically important task, necessary to
guide prognosis and treatment strategies. MRI can play an important
role by identifying specic differences in atrophy patterns among
diseases. We focus on studying group differences in local cortical
features.
Methods: We used a surface-based cortical analysis framework to
study T1-weighted MRI acquired for PSP,MSA and IDP patients(8 in
each group). A cortical reconstruction method was used to extract the
surface at the geometric center of 3D gray matter(GM) tissue. For each
individual surface,two local curvature features(shape index and curvedness) were computed at every point,yielding a dense estimate of the
cortical folding pattern.GM thickness was also estimated in 3D. To
perform group analysis,individual cortical surfaces were warped to a
reference surface(average from 53 healthy subjects), allowing correspondence between anatomically homologous points of individual surfaces. Population averages as well as point-based statistical comparison

FIG. 1 (299).

POSTER SESSION I, TUESDAY, JUNE 5, 2007

were computed within the common cortical coordinate system of the
average brain.
Results: All groups were age-matched (Tukey-Kramer HSD,alpha0.05). Population average maps revealed different curvedness and
cortical thickness proles across groups.Subtle yet signicant differences in shape index and curvedness maps were found, more pronounced in frontal lobe when comparing PSP to MSA.Thickness maps
displayed tissue loss in frontal lobe and motor cortex,reaching significance in motor cortex region when comparing PSP to MSA.
Conclusions: We interpret these nding as indicative of increased
sulcal atrophy in frontal lobe in PSP when compared to MSA and
IPD.These preliminary results conrm our hypothesis of distinct patterns of sulcal atrophy and GM tissue loss,demonstrating potential use
of routine MRI and cortical morphometry in performing differential
diagnosis in PSP,MSA and IPD, and encourage further investigation in
larger dataset.
300
Effect of adjunct rasagiline on dopaminergic and non-dopaminergic motor features of Parkinsons disease
J.M. Rabey, C.J. Fitzer-Attas (Zerin, Israel)
Objective: To investigate the effect of rasagiline on dopaminergic
and non-dopaminergic motor features in Parkinsons disease (PD), as
adjunct to levodopa in patients with motor uctuations.
Background: Rasagiline, a novel MAO-B inhibitor, has shown efcacy as adjunct to levodopa in PD patients with motor uctuations. In
the LARGO study, rasagiline signicantly improved UPDRS-Motor
score vs placebo during both ON time (p0.001) and practically
dened OFF time (p0.05). Entacapone, as the active comparator arm,
was signicantly effective vs placebo in ON time only. Recently, Levy
et al (Arch Neurol 2005; 62 (3): 467) clustered the motor features of PD
measured with UDPRS by their dopaminergic or non-dopaminergic
status. Here, we assessed LARGO data using this classication system.
Methods: The randomised, double-blind LARGO study (n687)
investigated the efcacy of rasagiline 1.0 mg/day vs placebo, in levodopa-treated PD patients. Entacapone was given as 200 mg/levodopa
dose. Post-hoc analyses were made in patients during ON time
(n668), and in the practically dened OFF state (a substudy; n105).
UPDRS-Motor scores were analyzed after grouping into 2 categories
and 6 subdomains: dopaminergic (subdomains tremor, rigidity, bradykinesia, facial expression), and non-dopaminergic (subdomains
speech, axial impairment). Bonferroni correction for multiple comparisons was performed (p0.0031).
Results: During ON time, both rasagiline and entacapone produced
signicant benets in UPDRS-Motor score vs placebo in the dopaminergic symptom category (p0.001). In the dopaminergic subdomains,
rasagiline signicantly improved tremor (p0.01) and bradykinesia
(p0.001), with benecial effects in rigidity and facial expression that
did not reach signicance after Bonferroni correction. In contrast to
entacapone, the benet of rasagiline on bradykinesia during ON time
was also signicant during OFF time (p0.001), with a trend for
improvement in facial expression, and speech (non-dopaminergic subdomain).
Conclusions: In levodopa-treated patients, once-daily rasagiline produced signicant benets in dopaminergic motor features of PD in both
ON and OFF time, with a trend towards benet in speech (nondopaminergic feature) during OFF time. Such effects could be clinically meaningful to patients with motor uctuations maintaining
movement and communication.
301
Ropinirole 24-hour prolonged release in advanced Parkinsons
disease: Relationship between treatment response and disease severity
K.D. Sethi, F. Stocchi, L. Giorgi (Augusta, Georgia, USA)
Objective: Evaluate the relationship between the efcacy of ropinirole 24-hour prolonged release and disease severity at baseline in
patients with advanced Parkinsons disease (PD).

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Background: Ropinirole, as monotherapy or as an adjunct to L-dopa,

is effective in treating early and advanced PD. A 24-hour formulation
allows for a convenient dose-titration regimen and the potential for
enhanced compliance and tolerability.
Methods: In the EASE-PD Adjunct study (101468/169), patients
with PD not optimally controlled with L-dopa were randomized to
receive adjunctive ropinirole 24-hour (n202) or placebo (n191),
once-daily for 24 weeks. Initial dose was 2.0 mg/day, titrated to a
maximum of 24 mg/day. At 8.0 mg/day and at each subsequent increase, L-dopa dose reduction was required. Primary endpoint was the
mean change in daily off time at Week 24 last observation carried
forward (LOCF). A post hoc analysis of covariance (ANCOVA) was
conducted to determine any relationship between the magnitude of
treatment effect, in terms of change in daily off time, and baseline
Unied Parkinsons Disease Rating Scale (UPDRS) motor score (part
III; assessed at least 2 hours after previous L-dopa dose, regardless of
off or on state) having adjusted for country, baseline off time,
and baseline UPDRS total motor score.
Results: There was a statistically signicant treatment benet in
favor of ropinirole 24-hour, vs. placebo, for change in off time at
Week 24 LOCF (adjusted mean treatment difference: 1.7 hours;
p0.0001). At baseline, mean UPDRS motor scores were similar
between treatment groups (ropinirole 29.8 [SD 12.91]; placebo 30.7
[SD 14.41]). There was a statistically signicant interaction between
treatment effect and baseline UPDRS motor score in terms of daily
off time (p0.0444). The magnitude of the treatment difference
between ropinirole 24-hour and placebo increased with increasing
baseline UPDRS motor score. Mean dose (SD) of ropinirole 24-hour at
last visit was 18.8 (6.26) mg/day.
Conclusions: The magnitude of response to ropinirole 24-hour prolonged release increases with increasing disease severity.
302
Does pregnancy affect the progression of Parkinsons disease?
B. Robottom, J. Mullins, L.M. Shulman (Baltimore, Maryland, USA)
Objective: To assess the effect of pregnancy on the progression of
early PD in an untreated female patient, and to compare the rate of
progression during pregnancy to a cohort of untreated patients.
Background: Pregnancy in patients with Parkinsons disease is rare.
Previous reports have focused on the clinical course of pregnant patients receiving antiparkinsonian therapy, but little is known about the
effect of pregnancy on progression of PD in the untreated patient.
Methods: A woman with untreated PD (age 35 years, PD duration 2
years, Hoehn & Yahr I) was assessed for disease severity with the
UPDRS before, during and after pregnancy. The rate of disease progression (change UDPRS/year), as measured by total and motor UPDRS during the pregnancy and postpartum was compared to a cohort of
untreated PD patients (N12, mean age 62 years, mean PD duration 3
years, mean Hoehn & Yahr stage 1.8)
Results: Over the 2 years before pregnancy, the total UPDRS increased by 1 point each year. During pregnancy and postpartum, the
total UPDRS increased by 37 points over the course of one year. The
UPDRS III motor subscale increased by 27 points during this time
period. In comparison, in the cohort of early untreated PD patients, the
total UPDRS increased by 2.8 points per year, with the motor subscale
increasing by 2 points during this time period.
Conclusions: Pregnancy and the postpartum period were associated
with a marked increased rate of progression in this patient with early
PD. The absence of antiparkinsonian therapy permits a better assessment of the inuence of pregnancy alone. The rapid rate of progression
of PD during pregnancy and postpartum may be related to hormonal
uctuations including estrogen. Comparison to the rate of progression
in a similar cohort of untreated PD patients suggests that pregnancy has
a detrimental effect on PD progression. Longer follow up is necessary
to determine if this decline is reversible.

Movement Disorders, Vol. 22, Suppl. 16, 2007

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303
Mild cognitive impairment in Parkinsons disease
E. Stefanova, M. Petrovic, M. Svetel, N. Dragasevic, V. Kostic
(Belgrade, Serbia)
Objective: To estimate the prevalence of amnesic and non-amnesic
mild cognitive impairment (MCI) in patients with Parkinsons disease
(PD), and several demographic, clinical neurological and psychiatric
factors were tested as predictors.
Background: PD is often associated with mild cognitive impairment
and dementia. The frequency of dementia is about 28% and PD patients
have an almost 6-fold increased risk of developing dementia compared
to healthy controls. The aetiology and risk factors are not known, but
old age and severity of the disease are predictive factors for cognitive
impairment.
Methods: The assessment included comprehensive neurological and
psychiatric examinations and a battery of neuropsychological test, and
subtypes of MCI were classied as according to revised Petersens
criteria. The smell test was also included. Three hundred and sixteen
consecutive patients with PD were included in the study.
Results: Thirty patients (9.4%) were diagnosed with dementia. A
total of 172 were cognitively intact, whereas 114 (36%) were diagnosed
with MCI (amnesic 61; single non-memory domain 15; and multiple domains, n 18). Age, severity of the disease, presence of
depression and apathy were shown to be signicant predictive factors,
as well as axial parkinsonian signs. The smell dysfunction early in the
course of the disease is strongly associated with MCI.
Conclusions: These data suggested that PD patients with more prominent axial signs smell decits and older age are in higher risk for
developing cognitive impairment. Therefore, early detection of mild
cognitive impairment is very important diagnostic step.
304
Tolcapone as an alternative to entacapone for adjunctive therapy
in Parkinsons disease: An evidence-based efcacy comparison
A.J. Lees, H. Achenbach (London, United Kingdom)
Objective: To evaluate the relative efcacy of tolcapone and entacapone in the treatment of patients with Parkinsons disease (PD) and
motor uctuations.
Background: Catechol-O-methyltransferase (COMT) inhibitors are an
important class of medications for the management of PD. There are only
two COMT inhibitors, tolcapone and entacapone, used in clinical practice.
An almost exclusive focus on safety has obscured the crucial question of
whether one of these agents is more efcacious than the other.
Methods: We reviewed the literature on tolcapone and entacapone
(Medline English language search, MDS abstracts) and identied 14
randomized, double-blind, placebo-controlled trials of these agents;
one randomized, double-blind, 3-week entacapone-to-tolcapone switch
study; one large, uncontrolled entacapone-to-tolcapone switch study;
and one uncontrolled tolcapone-to-entacapone switch study. We used
these sources to compare the efcacy of tolcapone to that of entacapone. Since the Cochrane Collaborative had performed a thorough
meta-analysis of the placebo-controlled studies, we used their efcacy
analyses in our evaluation.
Results: The Cochrane meta-analysis showed a placebo-corrected
weighted mean decrease in OFF time of 1.60 hours (95% condence
interval [CI], 1.10 2.10) for tolcapone vs 0.68 hour (95% CI, 0.22
1.13) for entacapone. Similarly, the increase in placebo-corrected
weighted mean ON time for tolcapone was 1.86 hours (95% CI,
1.29 2.42) vs 1.01 hours (95% CI, 0.621.39) for entacapone. In the
controlled switch study intent-to-treat analysis, the mean increase in
ON time among patients switched to tolcapone was 1.34 hours, compared with 0.65 hour for those who remained on entacapone. In the
per-protocol analysis, 29% of tolcapone-treated patients had a 3 hour
increase in ON time compared with 12% of patients remaining on
entacapone. The uncontrolled studies reported similarly superior efcacy for tolcapone.
Conclusions: The multiple sources of evidence presented in this
review are highly consistent with one another and indicate favorable

Movement Disorders, Vol. 22, Suppl. 16, 2007

efcacy for tolcapone. These data suggest that patients suffering from
PD with motor uctuations who fail to benet from a trial of adjunctive
entacapone, or who are no longer obtaining much benet from its use,
may be responsive to tolcapone.
305
Health-related quality of life in Parkinsons disease: Development
and predictors during long-term disease progression
E.B. Forsaa, K. Herlofson, T. Wentzel-Larsen, J.P. Larsen, G. Alves
(Stavanger, Norway)
Objective: To investigate (1) how Health-Related Quality of Life
(HRQL) changes over time in a population-based cohort of patients
with Parkinson disease (PD) and (2) which factors predict lower levels
of HRQL in these patients.
Background: Prospective longitudinal studies of HRQL are rather
few, are strongly limited in their duration of follow-up, and only one of
these is community-based.
Methods: In a community-based study, 227 patients with PD were
assessed for their HRQL as measured by the Nottingham Health Prole
(NHP), followed prospectively and re-evaluated after four (111 patients) and eight years (82 patients). Population-averaged regression
models were used to estimate the rate of changes in HRQL over time,
and to identify baseline features predicting lower levels of HRQL
during follow-up. Baseline variables included in the regression models
were follow-up time, age at baseline, age at onset, sex, UPDRS parts
I-III, motor complications (UPDRS part IV, items 32-39), Hoehn and
Yahr staging, Schwab and England score, MADRS score, MMSE
score, dementia (yes/no), insomnia (yes/no), and hallucinations (yes/
no).
Results: All NHP subdimensions expect for sleep, as well as the
NHP total score (p0.001), increased signicantly during followup. Steepest slope was found for the domain physical mobility (2.98,
95 % CI 2.20-3.76), followed by the domains social isolation (2.18,
95% CI 1.48-2.88) and emotional reactions (1.35, 95 % CI 0.741.96). In addition to follow-up time, higher Hoehn and Yahr staging,
higher MADRS scores, and presence of insomnia at baseline predicted lower levels of overall HRQL as measured by the NHP total
score.
Conclusions: PD has an increasing impact on HRQL along with
the progression of the disease. Our results indicate that during
long-term disease progression deterioration in physical mobility has
to be considered the most important single factor contributing to
decline in HRQL in PD. Distress of non-motor character, most
prominent in the dimensions social isolation and emotional reactions, also considerably contributed to the progression of HRQL.
More advanced disease, higher severity of depressive symptoms and
presence of insomnia at baseline independently predicted poor
HRQL in our cohort.
306
Neurocircuitries associated with tremor and dyskinesia: Insights
from an fMRI case study
S. Sen, M. Lewis, X. Huang (Chapel Hill, North Carolina, USA)
Objective: To understand the neurocircuitries underlying tremor
and levodopa-induced dyskinesia (LID) in Parkinsons disease
(PD).
Background: PD is characterized by resting tremor, bradykinesia,
akinesia and postural instability. Levodopa, the gold-standard for
treating PD, is associated with dyskinesia after long-term use.
Although it has been hypothesized that the pathways associated with
tremor and dyskinesia are largely segregated, the exact mechanisms
underlying the pathogenesis of tremor and LID are not known. In
order to gain further insight into these phenomena, we performed a
functional magnetic resonance imaging (fMRI) study on a PD
subject who displayed tremor on one side of the body and dyskinesia
on the other.
Methods: The study subject is a 79 year old PD patient (stage III;
Hoehn and Yahr) identied through a tertiary Movement Disorders

POSTER SESSION I, TUESDAY, JUNE 5, 2007

clinic. The patient has a history of PD for 7 years that started with
tremor on her left side. At the time of the study she had further
developed right side tremor (2 years) and left side LID (3 years). Her
principal PD medication was Sinemet (25/100 mg) 3 tablets per day in
divided doses. We videotaped her UPDRS examination and performed
fMRI studies while she was both on and off levodopa. The fMRI
data was collected during a sequential nger opposition task using
either the right or left hand.
Results: There was a differential activation pattern for the subject
depending on the hand used to complete the task. Specically, we
observed that prior to levodopa cortical, subcortical, and cerebellar
recruitment patterns were different when using the dyskinetic versus
the tremor-predominant hand. In addition, levodopa differentially
modulated these patterns of activation depending on whether the
task was performed with the dyskinetic hand or tremor-predominant
hand.
Conclusions: To our knowledge, this is the rst fMRI study of a
patient who exhibits tremor on one side and dyskinesia on the other
side of the body. This case supports the hypothesis of the segregation
of neuronal pathways underlying PD tremor and LID. The implications
of the differential activation patterns observed and their roles in tremor
and LID shall be further explored.

307
Ropinirole 24-hour prolonged release delays the onset of dyskinesia
compared with carbidopa/levodopa in patients with Parkinsons
disease treated with levodopa
R.L. Watts, K.D. Sethi, R. Pahwa, B.E. Adams, N.L. Earl
(Birmingham, Alabama, USA)
Objective: Evaluate time to onset of dyskinesia in patients with
Parkinsons disease (PD) not optimally controlled with levodopa (Ldopa) and treated with adjunctive ropinirole 24-hour prolonged release
or adjunctive carbidopa/levodopa (C/L).
Background: Ropinirole is efcacious in treating both early and
advanced PD. Ropinirole 24-hour, a once-daily formulation, offers a
convenient dose titration regimen and the potential for enhanced compliance and tolerability.
Methods: This randomized, multicenter, double-blind, parallelgroup, exible-dose study (101468/228) included patients with PD
(HY stage IIII) who were on 600mg L-dopa therapy for 3
years, and demonstrated lack of symptom control (e.g. mild wearing
off, simple on/off uctuations). Patients were randomized to adjunctive treatment with ropinirole 24-hour once-daily (n105; 224mg/
day) or C/L three-times-daily (n104; 50 1000mg/day) for 2 years.
Initial doses were titrated to optimal therapeutic effect. No reduction
in baseline L-dopa dose was allowed. Primary endpoint was time to
onset of dyskinesia.
Results: The study was terminated early for reasons unrelated to
patient safety, including low patient recruitment. Overall, 21 patients developed dyskinesia (ropinirole 24-hour n3 [3%]; C/L
n18 [17%]). There was a statistically signicant delay in time to
onset of dyskinesia for patients treated with ropinirole 24-hour,
compared with C/L (p0.001). Post hoc analysis with censoring of
observations after termination of the study also showed a signicant
delay in onset of dyskinesia in the ropinirole 24-hour group
(p0.002). At Week 104 last observation carried forward, mean
doses (SD) were 10.0 (6.15) mg/day for ropinirole 24-hour and 284
(222.33) mg/day for C/L. Both treatments were generally well
tolerated. The most common adverse event was nausea (ropinirole
24-hour 25%; C/L 15%).
Conclusions: Adjunctive therapy with ropinirole 24-hour, compared
with C/L, prolongs the time to onset of dyskinesia in patients with
mild-to-moderate PD already treated with L-dopa.

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308
Growth hormone stimulation test (CGHST) detects autonomic failure earlier than clinical autonomic testing in Parkinsons disease
M. Ragothaman, S. Koshy, D.K. Subbakrishna, C.J. Mathias,
U. Muthane (Bangalore, India)
Objective: To determine if Clonidine Growth Hormone Stimulation
test (CGHST) detects sympathetic (adrenergic) dysfunction in PD
before clinical autonomic testing.
Background: Clonidine is an alpha adrenoceptor agonist that reduces
cerebral sympathetic activity and lowers blood pressure. In presence of
normal sympathetic activity, Clonidine reduces plasma noradrenaline
levels and increases Growth Hormone (GH) levels. Clonidine Growth
Hormone Stimulation test (CGHST) detects central sympathetic failure
and helps distinguishing it from peripheral sympathetic failure (da
Costa et al 1984). Pathological studies in Parkinsons disease (PD)
show that central autonomic nuclei (locus ceruleus, subcoeruleus and
dorsal motor nuclei of vagus) are involved before appearance of motor
symptoms. Symptoms of sympathetic failure in PD might be masked
due to compensatory mechanisms.
Methods: Ninety controls (Mean SD: 4714.7yrs) from St Marys
hospital, London, UK and 98 PD patients (Mean SD: 54.111.6 yrs)
from NIMHANS, India underwent the CGHST test. PD was diagnosed
using the UKPDRS Brain Bank Criteria. Patients had Parkinsonism for
less than 3 years. Patients underwent Autonomic testing and the
Clonidine Growth Hormone Stimulation test (CGHST). Clonidine (2
mg/kg) was administered intravenously over 10 minutes in a practical
off state after overnight fasting. Sample 1 was collected after resting
for 10 minutes before Clonidine injection and, subsequently, samples
were collected at serial intervals of 15 minutes for one hour. All
patients underwent clinical autonomic testing.
Results: Twelve (12.2%) PD patients had OH on head up tilt.
However, even at baseline, PD patients had lower levels of Growth
hormone than controls and they remained below normal at all time
points. Levels of GH signicantly dropped in PD, 30 minutes after
Clonidine injection and this difference persisted until 60 minutes. GH
levels were low even after the age was adjusted.
Conclusions: Abnormal CGHST detects central sympathetic failure
in all PD patients. This dysfunction occurs when only few of patients
show abnormalities on clinical autonomic testing.

GH Levels Before and after Clonidine stimulation

GH Pre clonidine
GH 15 mins Post Clonidine
Gh 30 mins post clonidine
Gh 45 mins post Clonidine
Gh 60 mins Post clonidine

Controls (90)

PD(98)

p-Value

2.83.7
6.48.5
14.2114.7
17.318.9
17.318.9

2.32.7
4.515.16
6.312.21
6.914.8
7.318.5

NS
NS
000
001
000

Gh-Growth Hormone
309
Pathological hypersexuality in Parkinsons disease: A clinicianrated survey and a working denition
T. Thomsen, J.M. Miyasaki, M. Zurowski, M. De Sousa,
R.M.A. De Bie, P. Wadia, G. Adeli, A.E. Lang, V. Voon (Toronto,
Ontario, Canada)
Objective: To determine the prevalence of pathological hypersexuality (HS) in Parkinsons disease (PD) using a clinician-rated survey
and a working denition of HS.
Background: A range of impulse control and repetitive behaviors related
to dopaminergic medications have been reported in PD that include HS,
pathological gambling, compulsive eating, shopping, compulsive medication use and punding. Recent studies using patient-rated questionnaires
suggest the prevalence rate of HS with medication use is 2-3%.
Methods: The patient-rated HS questionnaire used in our original
prevalence study was adapted to a clinician-rated questionnaire. The
questionnaire was administered to 156 consecutive PD patients attend-

Movement Disorders, Vol. 22, Suppl. 16, 2007

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11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

ing an ambulatory PD clinic. Movement disorder neurologists were

trained to administer the survey which took place in a private setting.
Patients identied above a dened threshold were interviewed by a
neuropsychiatrist using a semi-structured interview. The same working
denition of HS used in the patient-rated survey was applied.
Results: Of 156 patients surveyed, 13 (8%) met criteria for HS at any
time during their course of illness. Two patients had current symptoms
(1.2%). One patient declined psychiatric interview. 12 patients were
interviewed; 11 fullled criteria for pathological hypersexuality. Of
those who did not score above threshold, two were interviewed by
neuropsychiatry for alternate reasons both of whom were found to have
HS. The positive predictive value of the clinician rated survey was
92%. The results of the patient rated HS questionnaire in the same PD
clinic documented 7/297 (2.4%) wiith HS which was signicantly
lower than the results of this current clinician rated survey (Fishers
exact test: p0.006).
Conclusions: The clinician-rated HS survey for PD has good positive
predictive value. The prevalence of HS based on clinician inquiry was
signicantly higher than previously reported. This suggests that that
future clinical assessments and studies should utilize a clinician-rated
interview method. Further investigation is required to validate our
survey.
310
FP0011 extends the duration of the anti-parkinsonian actions of
L-DOPA and reduces L-DOPA-induced dyskinesia in the MPTPlesioned macaque model of Parkinsons disease
J.M. Brotchie, T.H. Johnston, S.H. Fox, P. Zerr, F. Tiberghien,
L. Bossi (Toronto, Ontario, Canada)
Objective: To assess the effect of FP0011 on L-DOPA-induced
dyskinesia and shortening of duration of anti-parkinsonian action in the
MPTP-treated macaque.
Background: Motor complications, specically dyskinesia and shortening of duration of anti-parkinsonian action, i.e. wearing-off, limit
the utility of dopaminergic therapies in Parkinsons disease. FP0011 is
an anti-glutamatergic agent with potential to reduce such problems.
Methods: Six cynomolgus monkeys were rendered parkinsonian
with MPTP. Motor complications were induced by oral L-DOPA
(25mg/kg, bid) for 4 months. For each animal, a dose of L-DOPA was
dened that provided a maximal anti-parkinsonian benet (30-50mg/
kg, sc) though this was compromised by disabling dyskinesia. The
effects of administration of L-DOPA (sc) with either vehicle or FP0011
(5, 15 and 45mg/kg, po) were assessed over six hours.
Results: In combination with L-DOPA, FP0011 (15mg/kg) extended
the duration of L-DOPA anti-parkinsonian actions (by 23%). These
extended anti-parkinsonian actions were equivalent to those afforded
by L-DOPA alone at peak effect though were not associated with
disabling dyskinesia. FP0011 also reduced the severity of dyskinesia
seen at peak anti-parkinsonian action of L-DOPA. In combination,
these actions provided a signicant increase (from 50 to 118 min,
136%) in the duration of on-time with non-disabling dyskinesia,
following treatment with L-DOPA and FP0011 (15mg/kg). Similarly,
although not statistically signicant, in another measure of good
on-time, there was a clear trend to increase the duration of on-time
without disabling dyskinesia from 117 to 188 min, or 61%, following
combination of FP0011 (15mg/kg) with L-DOPA. In contrast, the
duration of on-time during which dyskinesia was disabling was not
signicantly modied by FP0011.
Conclusions: In combination with L-DOPA, FP0011 therapy can
reduce the problems of dyskinesia and wearing-off by providing a
dramatic increase in the duration of good on-time. This action is
likely due to a reduction in the severity of dyskinesia at time of peak
anti-parkinsonian effect and an extension in duration of anti-parkinsonian action in a manner whereby the extended on-time is not associated
with disabling dyskinesia.

Movement Disorders, Vol. 22, Suppl. 16, 2007

311
Reexive eye and arm movement in Parkinsons disease and the
gap effect
Y. Shirakura, M.M. MacAskill, D. Myall, T.J. Anderson
(Christchurch, New Zealand)
Objective: To determine if (1) reexive arm movements mirror
reexive saccadic movement in PD and (2) the gap effect exists for
reexive arm movements as it does for reexive saccades in both PD
and controls.
Background: Reexive saccades are normal in PD other than mild
hypometria in advanced disease. In contradistinction, reexive arm
movements show increased reaction time (akinesia) and slowness (bradykinesia). In saccadic reexive tasks, introduction of a gap of 100 ms
to 400 ms between the offset of the xation target and the onset of the
peripheral target is associated with a quicker reaction time.
Methods: 19 PD and 19 controls participated. Eye movements were
recorded with videooculography. In the standard saccadic paradigms a
red target jumped randomly 60 times between the seven horizontal
positions at -15, -10, -5, 0, 5, 10 and 15 from the centre of the screen
coincident with a tone. The gap saccadic and arm paradigms were
identical to the standard ones other than a gap of 200 ms between the
extinction of the current (xation) target and the appearance of the next
target. Reexive arm movements were measured using a customised
virtual environment electromagnetic movement tracking system. Reexive movements were made to a target that appeared randomly in
one of the ten equally spaced locations on a 50 to 130 arc with a
radius of 0.15 m from the home (xation) target.
Results: (1) PD arm movement was slower than that of the controls,
with decreased peak velocity and lengthened acceleration and deceleration times; (2) PD subjects exhibited a longer latency than the controls
for reaching but not for saccades; (3) Primary saccades of the PD
subjects were slightly more hypometric than that of the controls,
whereas the PD subjects were as accurate as the controls in arm
movement; (4) The gap reduced latency in both groups for saccades
and arm movements.
Conclusions: Reexive eye and arm movements differ in established
PD. Saccades are hypometric (i.e. inaccurate) but normal in latency. In
contradistinction, arm movements are delayed and slow but accurate.
Thus the pathophysiology of PD affects these two motor systems in a
completely different manner.
312
Non-motor symptoms in patients with incident and untreated Parkinsons disease the Norwegian ParkWest study
B. Mueller, G. Alves, K. Herlofson, I. Hoegen-Esch, W. Telstad,
O.B. Tysnes, J.P. Larsen (Stavanger, Norway)
Objective: To study the frequency and severity of various non-motor
symptoms in a representative cohort of patients with incident and
untreated Parkinsons disease (PD).
Background: PD is increasingly accepted to be a multisystem brain
disease leading to both motor and a variety of non-motor problems.
However, knowledge on the prevalence and severity of non-motor
symptoms in patients with early, untreated disease is limited.
Methods: A prospective longitudinal study of patients with incident PD, the Norwegian ParkWest study, have been enrolled in four
counties of South-Western Norway, comprising a total population of
1,061,000 inhabitants by January 1st, 2006. Inclusion of all patients
with incident PD within the study area between November 1st, 2004
and August 31st, 2006 was intended. The study is approved to last
over 10 years, with follow-up visits at least every 6 months and
comprehensive examinations after 1 year, 3 years, 5 years, 7 years
and 10 years. The following instruments were used to clinically
assess the frequency and severity of different non-motor symptoms
at baseline: Montgomery and Aasberg Depression Rating Scale
(MADRS), Mini-Mental State Examination (MMSE), Starkstein
Apathy Scale (SAS), Neuropsychiatric Inventory (NPI), Stavanger
Sleepiness Questionnaire (SSQ), Epworth Sleepiness Scale (ESS),
Parkinsons Disease Sleep Scale (PDSS), Fatigue Severity Scale

POSTER SESSION I, TUESDAY, JUNE 5, 2007

(FSS), and the Unied Parkinsons Disease Rating Scale (UPDRS)
subscale I. In addition, information was obtained on urinary function, constipation, pain and symptomatic orthostasis using clearly
dened 5-point scales.
Results: Baseline data on the frequency and severity of various
non-motor problems in a representative cohort of patients with incident
and untreated PD will be presented.
313
A pilot data and analysis validity study on a NPF center of excellence database
C.W. Garvan, C.E. Jacobson, R. Stephen, K.D. White,
R.L. Rodriguez, K.D. Foote, H.H. Fernandez, M.S. Okun
(Gainesville, Florida, USA)
Objective: To evaluate utilization of best practices at the University
of Floridas Parkinson s Disease and Movement Disorder Center
(UFPDMDC), we conducted a validity study of 11 retrospective analyses of prospectively acquired data.
Background: The National Parkinsons Foundation (NPF) INFORM
database supports clinical care and research. INFORM is a comprehensive database that contains motor, functional, QOL, behavioral and
cognitive scales used in the initial and follow-up assessment of patients
seen at the UFPDMDC.
Methods: We gathered available material (e.g., data sets, abstracts,
manuscripts) related to 11 presented projects (4 were also published
manuscripts) and used a standard protocol for systematic review and
reanalysis. Each was evaluated in the following areas: data integrity,
analytic framework, statistical methods, transparency (i.e., the needed
materials and documentation to reproduce the research result existed),
and accuracy of reporting.
Results: We discovered examples of problems with data integrity:
missing data and data with out of range values (e.g., a Parkinson
disease patient having a Hoehn and Yahr stage of 0). Improvements were indicated for the analytic frameworks of 64% (7/11) of
the projects with evidence of lack of statistical planning (e.g.,
multiple regressions run without adjusting statistical signicance
levels). In one analysis only a portion of the available data were
utilized resulting in a biased estimate. We found that statistical
methods were used when their assumptions were violated (e.g., a
t-test was used for to analyze non-normal data in a small sample),
and more powerful or more appropriate methods were available
(e.g., a logistic regression was used to analyze count data in lieu of
Poisson regression). Lack of transparency affected some projects.
Inclusion/exclusion criteria generally were not coded and data dictionaries were not used.
Conclusions: While the errors identied in our study did not substantially change published results or conclusions, potentially correctable
threats to research validity were identied. An interdisciplinary academic
research model is needed that integrates best practices in data collection,
management and analysis throughout the entire research process.
314
Can we modify the factors inuencing selection of drug therapy in
Parkinsons disease (PD)?
A. Nasar, P. Dyer, C. Short, J. Cowling, W. Lynda, K. Turner,
L. Wheelhouse, E. Howard (Bridlington, East Yorkshire, United
Kingdom)
Objective: The aim of this audit was to identify factors which may be
inuencing in the selection of drug therapy for treating Parkinsons
disease (PD).
Background: Disability in PD is not always related to the stage or
duration of the illness.Many associated comorbidities such as arthritis,stroke,hypertension with angina, heart failure,diabetes with
neuropathy,hypotension may inuence in the selection of drug therapy and quality of life.Non-ergoline dopamine agonists whether
conventional or patch form have revolutionized the PD management.COMT inhibitors in combination with levodopa and new generation monoamine oxidase B inhibitors added new dimension.To

S97

maximize therapy selection of drugs should be tailored to individual

need.
Methods: Sixty three patients (male 43 female 20) aged between
62 to 95 years attending our PD clinic are randomly selected for this
audit.Assessment included Hoehn & Yahr staging,disease duration,comorbidities,medication
details,UPDRS
score,PDQ39,MMSE.Unit manager coordinated documentation.Patients/ care-givers are supplied with a diary to record and report for
change of symptoms and of any change of medications or of any
adverse events.No PD patients are discharged from our clinic.They
have open access to see consultant any day of the week without prior
appointment.
Results: Among the 63 patients,13(20%)in stage 1,28(44%) in stage
II,16(25%) in stage III and six in stages IV and V. Duration varied
between 3 to 23 years.Mobility affected in 37(58%) due to osteoarthritis,hypertension with angina in 23(36.5%),with stroke in
11(17%).Non-ergoline dopamine agonists found to be safe in many
except in patients with hypertension and angina,in peripheral vascualr
disease (PVD).Caution required in patients on SSRI or lithium for
depression.Patients with frequent uctuations did well with COMT and
levodopa combination.Ergoline dopamine agonists are avoided to prevent lung brosis and vulvulopathy.Two patients required amantadine
for dyskinesia. In this series 28 patients(44.4%) remained mobile and
independent in the community.
Conclusions: From these results it became apparent that comorbidities such as osteoarthitis (58%) affected mobility signicantly.Modifying drug therapy in relation to comorbidities improved compliance
and quality of life.
315
Parkinsons disease and apomorphine an Indian experience
N. Surya (Mumbai, Maharashtra, India)
Objective: To study the effectiveness and usefulness of subcutaneous
apomorphine in Parkinsons disease in Indian population.
Background: All patients with severe motor uctuation and dyskinesia were selected.
Methods: Five patients (4 male and 1 female) between 55-70 years
with Parkinsons disease of 5-15 years having motor uctuation, with
H Y scale of 3-4 was treated. All patients were hospitalised and
Domperidon was started 48 hours before treatment. The starting dose
was 1mg which gradually increased up to 5mg in few patients, 2-4
times a day.The total duration of treatment was 2-15days.
Results: Improvement in H Y stage noted in 3 patinets with with
drawl of syndopa in 1 patient and reduction of dose of syndopa in 2
patients. One patient died due to MI (Myocardial Infarction), unrelated
to apomorphine.
Conclusions: Subcutaneous apomorphine given intermittently is a
useful therapy in patients with severe motor uctuation and dyskinesia
and it improves the QOL (Quality of Life) in selected patients.
316
Postural impairment in Parkinsons disease: Diagnostic utility of
the rst trial effect
J.E. Visser, L. Janssen, C.M. Bastiaanse, G.F. Borm, J.E.J. Duysens,
B.R. Bloem (Nijmegen, Netherlands)
Objective: To investigate the diagnostic utility of the rst trial
effect to evaluate postural impairment in Parkinsons disease patients.
Background: Dynamic posturography as it is currently used has
limited clinical utility in diagnosing balance disorders and the evaluation of the risk of falling in individual patients. Here, we investigated
the diagnostic utility of a new approach, where we analyzed the balance
responses to the very rst and fully unpracticed trial, rather than a
pooled mean response to a series of predictable perturbations.
Methods: We included 14 patients with Parkinsons disease (PD) and
17 age-matched controls. Subjects received a series of multidirectional
postural perturbations, induced by support surface rotations. The kinematics of trunk movement and electromyographic responses to the very
rst trial which was always a pure backward perturbation were

Movement Disorders, Vol. 22, Suppl. 16, 2007

S98

11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

compared with those induced by a habituated backward perturbation.

Electromyographic responses were recorded from the masseter, erector
spinae, tibialis anterior and soleus muscles.
Results: The large forward exion of the trunk, induced by the rst
and unhabituated perturbation, was decreased in patients. The rst trial
also elicited smaller electromyographic responses in the tibialis anterior
muscle of patients. Of note, both groups showed prominent masseter
activity with an early onset, suggesting a startle-like response, which
was most pronounced in patients. Masseter activity was not attenuated
in the habituated trial. Trunk exion and soleus muscle activity did
show signicant habituation in both groups. The rate of this habituation
was comparable between patients and controls.
Conclusions: We observed a clear rst trial effect in both PD
patients and controls, which was associated with early and prominent
masseter activity, suggesting the presence of a startle-like component to
the overall balance response. Further studies are warranted to disentangle this startle component and to evaluate its role in the pathophysiology of balance disorders. Postural responses were abnormal in PD
patients, but equally so for the rst trial and the habituated trial. In
terms of diagnostic utility, evaluation of the rst trial response is no
better than the pooled responses to a series of perturbations.
317
Evaluation of cognitive impairment in Parkinsons disease by computerized neuropsychological tests
H. Shabtay, A.D. Korczyn (Tel Aviv, Israel)
Objective: To use a novel computerized neuropsychological assessment battery, NexAde, in order to evaluate cognitive function in
Parkinsons disease (PD) patients, and to compare results with those of
a memory impaired (MI) group and with a normal population.
Background: Cognitive impairment is common in patients with
Parkinsons disease (PD), but is not always symptomatic. Tests to
measure cognition of these patients are difcult to standardize and
perform when signicant motor dysfunction occurs.
Methods: Non demented PD patients (n22, 16 males, mean age
67.5 years) were recruited. The MI subjects (n55, 20 males, mean age
68 years) were non-demented and had MMSE scores of 24 and higher.
A control group consisted of 102 cognitively intact volunteers (n49
males, mean age 69). T-tests were performed for comparison of cognitive evaluation between all subject groups. correlation coefcients
were calculated between the cognitive measures.
Results: All subjects were able to undergo the computerized cognitive tasks, including those with no computer experience. Twenty out of
22 PD patients were able to complete the examination and reported a
pleasant user experience. Performance was not inuenced by computer
skill. NexAdes scores indicated that two out of 20 patients who
completed the battery had cognitive decline, similar to the MI subjects.
Performance on executive tests of NexAde was lower among PD
patients as compared to controls and to the MI group.
Conclusions: This study conrms that NexAde is easily used by PD
patients and can detect subtle cognitive impairments. The affected
cognitive measures in PD were similar to those of memory impaired
(MI) subjects. Study results are limited by the small number of participants.
318
Weight gain post deep brain stimulation of the subthalamic nucleus
in Parkinsons disease: Exploring possible causes
H.U. Jorgensen, L. Simonsen, L.M. Werdelin, S. Rusborg,
A. Lokkegaard (Copenhagen, Denmark)
Objective: To establish factors of importance for the weight gain
commonly seen in patients with Parkinsons disease after initiation of
treatment with deep brain stimulation of the subthalamic nucleus
(DBS-STN).
Background: Patients with Parkinsons disease frequently gain
weight after initiation of DBS-STN treatment. Reduction in basal
energy expenditure or total energy expenditure due to a decrease in

Movement Disorders, Vol. 22, Suppl. 16, 2007

Parkinson symptoms could be an explanation but also the postoperative

reduction in medication could be a contributing factor.
Methods: Patients with Parkinsons disease referred for DBS-STN
treatment were assessed before neurosurgery and subsequently 3 and
12 months post operation with regard to body composition, maximum
oxygen uptake, basal energy expenditure and daily energy intake and
physical activity. Body composition, was assessed using Dual Energy
X-ray Absortiometry. Maximum oxygen uptake tests were performed
on an ergometric bicycle, by using a facemask and breath-by-breath
technique.Basal energy expenditure was measured in both the treated
and untreated state after 12 hours of fast by a ventilated hood system.
The basal energy expenditure was measured both with and without
medication and postoperative also with and without stimulation. The
degree of Parkinson symptoms was assessed by Unied Parkinsons
Disease Rating Scale (UPDRS) immediately after the measurement of
basal energy expenditure.To determine the daily energy intake, patients
recorded the caloric value of food and beverages consumed over a 3
day period.To establish the level of activity the Physical Activity Scale
of the Elderly questionnaire was used.
Results: Due to ongoing analyses of data no data is ready at present.
At summer 2007 we expect data to be available from 8 patients. Data
will be forwarded as soon as available.
Conclusions: Conclusions will be drawn following the data analysis.
319
Clinical ndings with Austrian LRRK2 mutation-subtype PD patient
G. Daniel, T. Bruecke, Z. Alexander (Vienna, Austria)
Objective: To show the previously undescribed clinical symptoms
and demographics of a recently detected LRRK2-type mutation.
Background: Recently a novel LRRK2-mutation located in the
PARK8 region has been detected (MDS,Vol21 ,Suppl.15,2006). We
would like to present this patient and his clinical disease development.
Methods: We descibe the patients clinical development according to
our written documentation from 2004 to 2007.
Results: Our patient is male and lives in the Austrian countryside.
His family-history is positive for Parkinsons disease (PD) with one
affected relative. He was diagnosed IPS in 1990 at the age of 55. His
presenting symptom was resting tremor in the left upper limb as well as
distinct rigor there. He spent his honeymoon-phase with good response to L-Dopa treatment until 2004, when dyskinesia became evident. In early 2005 he presented with equivalent type of PD, frequently
suffered from freezing, sudden-offs as well as painful dyskinesia of
predominantly choreatic type. Under COMT-Inhibition he was clinically stable until 9/06, when we last saw him. His recent(9/2006)
UPDRS-Subscores are: mental status - 5 points; ADL 27; motor scale
41. His recent medication consists of 4 doses 100mg L-Dopa plus
Entacapon, one dose of 200mg L-Dopa as retard formulation, a total of
1.05mg Pramipexol in three doses and nally 10mg Budipin twice
daily. We suggested DBS to our local neurosurgeon and will discuss
this decision together with our patient along the following visit next
month.
Conclusions: Concluding we describe a patient with PD of equivalent type, who developed L-Dopa longterm complications after 14
years of disease. These clinical features do not vary from regular
PD-patients except for the slightly reduced age of onset. Our ndings
are consistent with other clinically analysis of patients with LRRK2mutation.
320
An integrated speech and physical therapy approach for Parkinsons disease: Training big and loud
C.M. Fox, B.G. Farley, L.O. Ramig, D.F. McFarland (Denver,
Colorado, USA)
Objective: To evaluate the impact of a novel treatment program that
simultaneously targets speech and limb motor disorders in people with
PD.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Background: Over the past 15 years an efcacious speech treatment
for PD has been developed. This treatment, called LSVT/LOUD, is
organized around a simple but powerful therapeutic principal: increase
movement amplitude of speech output (vocal loudness) while retraining sensorimotor processes of disordered speech production. Recently, a derivative program (Training BIG) utilized LSVT/LOUD
concepts to train increased movement amplitude in whole-body movements and documented improved gait, balance, and quality of life in
people with PD. It is possible that training movement amplitude, as a
single, simultaneous control parameter, may facilitate organization of
speech and limb motor systems thereby increasing transfer of effects
while promoting activity-dependent neural plasticity.
Methods: Eight subjects (4 men, 4 women) ranging from 51 to 72
years of age; 2 mos to 9 years in disease duration; and Hohen and Yahr
Stages I-II were included. Data were collected one-week before and
after intervention. A range of standardized speech, voice, gait and
balance measurements, dual and transfer tasks, quality of life (Parkinsons Disease Questionnaire) and motor impairments (UPDRS) were
assessed.
Results: Data revealed all subjects increased vocal SPL (loudness)
during reading (average 10 db SPL) and increased stride length/velocity during gait (average of 9 cm). Gains in vocal SPL and gait stride
length were comparable to published data from independently training
LSVT/LOUD (range 8-13 dB SPL) or Training BIG (range 9-30 cm).
These changes had a positive impact of 28% on disease severity
(UPDRS-motor section) and 27% on quality of life (PDQ-summary
score). In addition, 3 of 7 subjects improved in an untrained handwriting task. These data document that individuals with early PD are able
to improve beyond baseline levels.
Conclusions: There is a great need to simplify rehabilitation approaches for people with PD due to the progressive nature of the
disorder, cognitive challenges, and logistical/nancial burdens that
intensive speech and physical therapies present. An amplitude-based
treatment program, that simultaneously delivers speech and physical
therapy, may be one possible solution.
321
Clinical factors associated with freezing of gait in Parkinsons
disease: A multidisciplinary approach
A.A. Zylstra, A.F. Grifth, M.L. Glisky (Kirkland, Washington, USA)
Objective: To identify clinical factors associated with freezing of
gait (FOG) in patients with Parkinsons disease (PD).
Background: Freezing of gait is a disabling and poorly understood
clinical problem in PD. It interferes with daily functioning and quality
of life, often resulting in falls and other injuries. This study seeks to
identify cognitive, emotional, motor, and treatment related factors
associated with freezing of gait. An increased understanding of the
factors associated with freezing may be helpful in developing appropriate treatment interventions.
Methods: Data from 83 patients with PD, mean age 65.5 years,
Hoehn & Yahr stages II-IV, who had undergone neuropsychological
testing, was compiled. Those who reported freezing (n 46) were
compared to those who did not (n 37) across a number of variables
including reports of falls, frequency of PD medications, cognitive and
emotional functioning and perceived quality of life.
Results: There were no signicant differences between the groups
(freezers versus non-freezers) on basic demographic variables (age,
gender, and education), or cognitive functioning. There was a trend
towards higher disease severity (H&Y score) in freezers (p .11).
Those individuals with freezing had a higher report of falls (p .01),
took PD medications more frequently during the day (p .01), and had
higher scores on all subtests of a measure of quality of life (PDQ-39),
indicating more impairments. Signicant differences between the
groups were found on the PDQ Activities of Daily Living, Social
Stigma, Cognition, and Bodily Discomfort subscales (p .05). Those
with freezing were signicantly more likely to report symptoms of
anxiety (78% of the freezers versus 51% of non-freezers, p .05).

S99

Conclusions: Freezing of gait is associated with more frequent

reports of falls, more reported anxiety, increased dosing frequency of
PD medications and a worse quality of life. Age, gender, or cognition
did not appear to be signicantly associated with freezing of gait. A
multidisciplinary approach may be helpful in treating freezing of gait,
with emphasis on psychological and quality of life factors.
322
Toll-free helpline reveals diverse needs of Parkinsons disease community
R.A. Elliott, J. Rosner, L. Pituch, P. Wiener, C.M. Evers (New York,
New York, USA)
Objective: To better understand the informational and practical
needs of people living with Parkinsons disease (PD).
Background: Recognizing that people with PD have personal, specic questions that need answers, the Parkinsons Disease Foundation
(PDF) launched the Parkinsons Information Service (PINS) toll-free
helpline in 2005. This condential service offers guidance and resources to people living with PD.
Methods: 1079 individuals (67% female and 23% male) contacted
the PINS helpline between the six-month period of July 1 and December 31, 2006. 49% of the callers were people with PD, 41% were
spouses, children or friends of a person with PD and 10% were dened
as healthcare professionals or other. 48% were newly diagnosed with
PD. Callers were representative of each region of the United States but
were largely from the Northeast (31%) and the South (27%). Over 70%
of the callers were referred to the helpline by a PDF publication or the
PDF website (www.pdf.org).
Results: The results revealed that people living with PD are seeking
information on a wide variety of topics. Calls to the helpline lasted on
average 13.4 minutes. The topics that were most frequently addressed
included understanding treatment options (34%), psychological/emotional needs (25%), referrals to other resources (17%) and explaining
the symptoms of PD (17%). Within these categories, the subject matter
ranged from understanding the side effects of a particular drug to the
role of diet and exercise (understanding treatment options); concerns
about depression to end-of-life issues (psychological/ emotional
needs); where to nd a neurologist, support group and sources of
nancial assistance (referrals to other resources); and understanding
possible risk factors to questions about diagnosis (explaining the symptoms of PD).
Conclusions: This study provides new evidence of the wide-ranging
needs of people with PD as they struggle to meet the challenges of
living with a chronic disease. The data suggest the need for more
comprehensive programs and services to address the diverse needs of
the PD community. One possible limitation of this study is that the
study population is disproportionately composed of English-speaking
people with PD living in the United States. Therefore, these results
might not be representative of the larger PD community.
323
Overlap of cognitive decits in Parkinsons (PD) and Alzheimers
(AD) diseases: Potential use of sanamide
T. Sharma, R. Anand, R. Hartman, S. Rossetti (Newark, Delaware,
USA)
Objective: We performed a comparison of the cognitive domains
affected in AD and in early PD patients and also determined the
potential benets of sanamide in treating the cognitive decits and the
activities of daily living(ADL) of AD based on recent results from a
trial in early PD patients.
Background: Cognition is impaired in early AD (perceptual speed,
executive functioning, episodic and working memory) and early PD
(reaction time, working memory and executive function). Comparisons
using the same computerized cognitive battery have not been performed.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S100 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: The Cogtest database on 225 early AD patients from 3
prospective studies was compared to data from a Phase III double-blind
placebo-controlled randomized 24-week trial evaluating sanamide, a
selective reversible MAO-B and glutamate inhibitor, as add-on therapy
to a single DA-agonist in 151 early PD patients. Auditory Number
Sequencing(ANS), Spatial Working Memory(SWM), Strategic Target
Detection(STDT), Tapping Speed, Simple Reaction and Choice Reaction Time were chosen. Data from AD and PD patients were converted
to z-scores, based on healthy control data from the Cogtest database
and expressed in SD units.
Results: Decits in AD patients compared to controls showed signicant decrements (all p values 0.05) of z scores of processing speed
(4.5SD), episodic(5SD) and working memory (4SD) and executive
function(1SD). At baseline, all PD patients showed cognitive decits in
at least one domain and 50% had decits in 2 domains. The domains
affected were verbal working memory (3.5SD), nger tapping (1.5SD),
spatial working memory (6.5SD) and executive function (1SD). Sanamide produced statistically signicant benets in executive function
(STDT; p0.037) and working memory (ANS; p0.035), as well as in
ADL(p0.024).
Conclusions: Using Cogtest, cognitive decits noted in early PD and
AD overlapped in working memory and executive function. AD patients were impaired, in addition, in episodic memory. Sanamides
cognitive improvement in early PD patients on DA-agonists suggests
that the mechanism of benet may be non-dopaminergic. Based on the
similarities in PD and AD, data suggest that sanamide may be of use
in treating ADL and cognitive decits of AD.

324
Rasagiline is effective in treating patients with early Parkinsons
disease, regardless of disease duration at treatment initiation (<1
year; >1 year)
J.M. Bertoni, R. Pahwa (Omaha, Nebraska, USA)
Objective: To assess whether the effect of rasagiline in patients with
early Parkinsons disease (PD) was inuenced by disease duration at
baseline.
Background: Rasagiline is a novel, selective, irreversible, secondgeneration MAO-B inhibitor that has demonstrated safety and efcacy
as monotherapy for patients with early PD (TEMPO study).
Methods: The 26-week, multicentre, randomised, double-blind
TEMPO study included patients (n404) receiving once-daily rasagiline 1 mg, 2 mg, or placebo. The primary efcacy endpoint was change
from baseline in Unied PD Rating Scale (UPDRS)-Total score. In this
post-hoc analysis, the patient population was stratied according to
baseline disease duration (1 year, n263; 1 year, n141), and
outcome measures assessed were change from baseline in UPDRSTotal, -Motor, and -ADL scores, and responder analysis (dened as
20% improvement from baseline in UPDRS-Total score).
Results: Within each stratum (1 year; 1 year) the rasagiline and
placebo groups had similar baseline mean disease duration and UPDRS-Total scores. Rasagiline 1 mg/day signicantly improved UPDRS-Total score compared with placebo in both the 1-year stratum
(-0.52 vs 3.33; p0.001) and the 1-year stratum (0.81 vs 4.76;
p0.01). Effect sizes were comparable between strata for UPDRSTotal score (-3.85 vs -3.95; p0.9617) as well as for UPDRS-Motor
and -ADL subscores. The rasagiline group had more responders versus
the placebo group in both the 1-year stratum (25.6% vs 11.5%) and
the 1-year stratum (16.7% vs 4.8%), with no signicant interaction
between treatment effect and disease duration.
Conclusions: Once-daily rasagiline monotherapy provides a consistent benecial effect on symptoms of early PD, regardless of disease
duration (1 year; 1 year).

Movement Disorders, Vol. 22, Suppl. 16, 2007

325
Evaluation of c-Abl tyrosine kinase mediated regulation of parkin
as therapeutic target for Parkinsons disease
S.Z. Imam, S. Sriram, H.S. Ko, D.W. Pearson, A.J. Valente,
J.M. Savitt, E. Andres-Mateos, D.B. Trinkaus, O. Pletnikova,
J.C. Troncoso, A. Yamamoto, P.J. Kahle, S.F. Ali, V.L. Dawson,
S. Li, J.L. Roberts, T.M. Dawson, R.A. Clark (San Antonio, Texas,
USA)
Objective: Mutations in parkin, an E3 ubiquitin ligase, are the most
common cause of autosomal recessive Parkinsons disease. Whether
parkin plays a role in sporadic forms of Parkinsons disease remains
unclear.
Background: Previously, we have shown that parkin interacts with
and is phosphorylated on tyrosine 143 by the stress signaling nonreceptor tyrosine kinase, c-Abl and under oxidative and dopaminergic
stress in vitro, c-Abl is activated and tyrosine-phosphorylates parkin,
causing loss of parkin activity and solubility and the accumulation of
parkin substrates, AIMP2 (p38/JTV-1) and FBP-1 leading to loss of
parkins cytoprotective function as parkin is unable to rescue cells from
AIMP2-induced toxicity.
Results: Here, we show that c-Abl mediated tyrosine phosphorylation of parkin is observed in the striatum of MPTP-treated mice and
inpatients with Parkinsons disease. This activation of c-Abl and tyrosine phosphorylation of parkin in MPTP mice model leads to loss of
striatal dopamine and nigral TH neurons. STI-571, a specic c-Abl
inhibitor, prevents tyrosine phosphorylation of parkin and restores its
E3 ligase activity and cytoprotective function both in vitro and in vivo.
Conclusions: Our results show that tyrosine phosphorylation of
parkin by c-Abl is a major post-translational modication that leads to
loss of parkin function. Moreover we describe a novel and compelling
role for the stress signaling kinase c-Abl, in the pathophysiology of
Parkinsons disease and inhibition of c-Abl may offer new therapeutic
opportunities for the treatment of Parkinsons disease.
326
Correlation between UPDRS-III scores and [11C]dihydrotetrabenazine (DTBZ) PET measures in early Parkinsons disease
M. Wieler, J. Stoessl, W. Martin (Edmonton, Alberta, Canada)
Objective: To estimate the degree of dopaminergic neuronal loss in
early Parkinsons disease (PD) vs. age-matched controls using a
positron emission tomography (PET) indicator of disease severity and
to evaluate the relationship between clinical features and PET changes.
Background: PET is an imaging technique that may be used to
quantify the functional integrity of presynaptic dopaminergic nerve
endings. Tracers, such as [11C]dihydrotetrabenazine (DTBZ), that bind
to the presynaptic vesicular monoamine transporter (VMAT) site can
provide an estimate of the number of intact VMAT sites and hence an
index of the functional integrity of presynaptic nerve endings. This
should be a method to quantify neuronal loss in PD.
Methods: Twenty-seven subjects (ages 60.4 8.4 years) with early,
untreated PD were evaluated as part of a larger prospective, longitudinal study. They underwent neurological examination, functional,
cognitive and PET assessment. Binding potential (BP) was calculated
for both the caudate and putamen. The PD PET data were compared to
data from 16 healthy controls (ages 55.9 11.5 years) and to the motor
subscale of the UPDRS.
Results: All subjects in the PD group had BP values at least 2
standard deviations below the mean of the control group. Specically,
control caudate values were 0.96 0.07, compared to PD caudate
values of 0.59 0.09; control putamen values were 0.96 0.09,
compared to PD putamen values of 0.42 0.10. The UPDRS-III scores
(mean 15.2 7.1) were correlated to the BP of both the caudate and
putamen and were shown to be highly signicant (r -0.52, p0.005
and r -0.60, p0.001 respectively).
Conclusions: These data suggest that the binding potential of DTBZ
is impaired in early, untreated PD subjects and that there is a signicant
relationship between disease severity (as measured by the UPDRS-III)

POSTER SESSION I, TUESDAY, JUNE 5, 2007

and this measure of dopaminergic neuronal loss in both the caudate and
the putamen.
327
The pharmacokinetic prole of levodopa administered with and
without tolcapone in patients with advanced PD
D. De Lucrezia, F. Guadagni, N. Santucci, L. Vacca, F. Stocchi
(Rome, Italy)
Objective: To evaluate the effects of different doses of levodopa,
with and without tolcapone, on levodopa plasma pharmacokinetics, and
to identify a regimen of levodopa, with or without tolcapone, whose
pharmacokinetic (PK) prole most closely resembles that of continuously infused levodopa.
Background: Although levodopa remains the gold standard for the
treatment of patients with Parkinsons disease (PD), long-term dopaminergic therapy is associated with the development of motor complications that impair quality of life and can cause signicant disability.
Continuous dopaminergic stimulation (CDS)-based therapy reduces the
risk of inducing motor complications in patients with early PD and
reverses motor complications in patients with advanced PD. Optimization of CDS therapy may lead to more durable responses. Tolcapone,
a catechol-O-methyltransferase inhibitor, extends the biologic action of
levodopa by prolonging its half-life.
Methods: This single-center, open-label, parallel-group study was
designed to assess the plasma PK prole of different levodopa/carbidopa regimens and the effect of tolcapone on levodopa pharmacokinetics. Ten patients with PD have been enrolled. The rst three patients
received levodopa/carbidopa (100/25 mg) four times at 4-hour intervals
on day 1. The next three patients received the same dose of levodopa/
carbidopa ve times at 3-hour intervals. On day 2, all six patients
received levodopa/carbidopa with tolcapone. The remaining four patients will be studied, based on the best plasma prole obtained with the
rst six patients. Plasma levodopa levels were measured prior to
treatment and every hour over a 12-hour period following the rst
levodopa dose. Selected pharmacokinetic parameters were assessed
(Table).
Results: Six patients (5 women, 1 man) have been studied. Median
age is 73 years (range, 68 to 78 years), and median duration of disease
is 12.8 months.
Conclusions: This study is ongoing and conclusions are pending nal
analysis of the data.

AUC
Cmax
Cmin
FI0-12

Unit

Day 1

Day 2

g/mL per hour

g/mL
g/mL

13.62 4.84
2.64 0.58
0.36 0.20
2.28 0.46

22.43 7.67
3.37 0.90
0.98 0.40
2.38 0.56

0.026
0.101
0.025
0.358

328
Development and evaluation of a community-based exercise programme for people with Parkinsons disease
K.J.E. Reinikka, A. MacLeod, M. Johnson, M. Bedard, M. Jog
(Thunder Bay, Ontario, Canada)
Objective: To develop and evaluate the impact of an 8-week community-based exercise programme delivered by a community tness
facilitator on performance and self-report measures in participants with
Parkinsons disease (PD).
Background: Innovative utilization of health care resources is necessary
to meet the needs of communities and clients in rural and remote regions.
The absence and need for an ongoing, physical activity programme for
people with PD was identied in Northwestern Ontario, Canada. A computer-based module was developed by physiotherapists using Competency
Based Education Training principles. This asynchronous learning tool
contains multimedia, text, and quizzing components and was used as the
basis for a community exercise programme for PD.
Methods: 11 participants (mean age, 70.45 1.98) with Idiopathic
PD, modied H&Y 1.0 to 2.5, and stable medication use were assessed
at baseline and following the 8-week exercise programme using the

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UPDRSm, Berg Balance Scale, Timed Up and Go (TUG), Parkinson

Disease Questionnaire-39, and Activities-specic Balance Condence
Scale. Attendance rates and occurrence of any adverse events were
recorded by the tness facilitator.
Results: Data analysis resulted in removal of one participant outlier.
Analysis of remaining participants (10) demonstrated a signicant
decrease in the UPDRSm (7.70, p 0.001), and a signicant increase
in TUG scores (2.03, p 0.005). Although statistical signicance was
not achieved in the other outcome measures, signicant effect size was
demonstrated. All eleven participants completed the study with an
adherence rate of 90.8% attendance in the exercise programme. No
adverse events were reported.
Conclusions: A community-based exercise programme, developed
by physiotherapists and delivered by a community tness facilitator,
can be safely provided to a group of people with PD. The exercise
programme appears to have demonstrated sufcient intensity to elicit
changes in motor function, with the potential for changes in all outcome
measures using a larger sample population. It is hoped that this unique
process of identifying and addressing the needs of a particular client
population in Northwestern Ontario will help to facilitate the ongoing
development of physiotherapy initiatives which address the changing
reality of healthcare.
329
The Norwegian ParkWest study study design and incidence
calculations
G. Alves, B. Mueller, K. Herlofson, I. Hoegen-Esch, W. Telstad,
O.B. Tysnes, J.P. Larsen (Stavanger, Norway)
Objective: To present the design of the Norwegian ParkWest study
and the calculated incidence gures within the study area.
Background: There are substantial geographical differences in reported prevalence and incidence gures of Parkinsons disease (PD).
As yet, no information does exist on the incidence of PD in Norway. In
additon, there is need for understanding the natural development of the
full spectre of the disease from the very early stages.
Methods: The Norwegian ParkWest study is a prospective longitudinal cohort study of patients with incident Parkinsons disease (PD)
from South-Western Norway, designed and co-ordinated by the Norwegian Centre for Movement Disorders, Stavanger University Hospital, and approved by the Regional Ethics committee located at the
University of Bergen. The study area comprises the four counties of
Sogn and Fjordane, Hordaland, Rogaland and Aust Agder, with a total
population of 1,061,000 inhabitants by January 1st, 2006. Inclusion of
all patients with incident PD within the study area between November
1st, 2004 and August 31st, 2006 was intended. The study is approved to
last over 10 years, with follow-up visits at least every 6 months and
comprehensive examinations after 1 year, 3 years, 5 years, 7 years and
10 years. All patients gave written informed consent to participate in
repetitive clinical, 3-D MRI, and blood sample examinations. In addition, the majority of patients gave their consent in cerebrospinal uid
examinations, derived by lumbar puncture at study start.
Results: Details of the study design, as well as calculated incidence
gures will be presented.
330
Specicity and sensitivity of transcranial ultrasound in the differential diagnosis of Parkinsons disease
A. Gaenslen, B. Unmuth, I. Liepelt, A. Di Santo, K. Schweitzer,
J. Godau, T. Gasser, M. Reimold, D. Berg (Tubingen, Germany)
Objective: To determine specicity and sensitivity of transcranial
sonography (TCS) in the differential diagnosis of Parkinsons disease
(iPD) versus Parkinsonian syndromes (aPS) in a prospective blinded
study.
Background: Increased echogenicity of the substantia nigra (SN)
determined by TCS has been shown to be a characteristic sign for iPD,
observable already in the very early stages of the disease. This feature
is seldom found in aPS, indicating that SN hyperechogenicity might be
valuable to discriminate between these diseases.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S102 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: 60 de novo patients with the clinical presentation of
Parkinsonism with predominant rigidity, akinesia as well as possible
features not typical for iPD have been examined by TCS. An investigator blinded to the TCS results investigated all patients concerning
L-Dopa-responsiveness, smelling and clinical follow-up examinations
over a period of 12 months. In patients with no clear clinical diagnosis
after one year, functional neuroimaging is being performed.
Results: In 44/60 results of the clinical examination and L-dopa test
point towards the diagnosis of iPD, 39 of them have increased SN
echogenicity. In 5 normal SN echogenicity was found. In 16 patients
the clinical diagnosis of aPS was established, 14 displayed normal and
2 increased echogenicity of the SN. The specicity of the SN echogenicity in diagnosis of iPD at baseline was 0.88, sensitivity 0.89, after
follow ups 0.92 and 0.91. Assessment of basal ganglia echogenicity
had a specicity of 0.73 and a sensitivity of 0.92, after follow ups
0.68 und 0.91 for the diagnosis of aPS Functional neuroimaging has
been performed in 15 patients, in 8 with SN hyperechogenicity the
initial clinical diagnosis of iPD was conrmed. In 7 patients the
baseline diagnosis was amended during follow up and after functional
neuroimaging.
Conclusions: First results of this still ongoing study provide evidence
that TCS is valuable in the early differential diagnosis of Parkinsonian
syndromes, which might be helpful in early, and disease specic
therapy.
331
Improved compliance with levodopa/carbidopa/entacapone (L/C/E;
Stalevo) vs levodopa/carbidopa and entacapone (L/C E) as separate tablets in Parkinsons disease (PD)
T.E. Delea, S.K. Thomas, M. Hagiwara, L. Mancione, M. Stacy
(Brookline, Massachusetts, USA)
Objective: To compare compliance with L/C/E (Stalevo; a singletablet formulation) vs L/C E as separate tablets in patients with PD.
Background: Observational studies have shown that single-tablet
formulations are associated with improved compliance to drug therapy
compared with the same components administered as separate tablets.
L/C/E incorporates L/C and E in one tablet and is indicated as a
replacement for immediate-release L/C without E in PD patients experiencing the symptoms of end-of-dose wearing off.
Methods: This was a retrospective observational cohort study using
health-insurance claims databases spanning Jan 00 Dec 05, representing 50 million beneciaries in the US. Subjects included persons with
a PD diagnosis (ICD-9-CM 332 excl. 332.1) who were receiving L/C
without E and then initiated on either L/C/E or E plus existing L/C as
separate tablets (L/CE). The date of rst prescription for L/C/E or E
was designated the index date. Patients with 365 days of enrollment
before and after index date were excluded. Compliance was assessed
based on medication possession ratio (MPR) for combination therapy
during follow-up (365 days post-index); MPR was calculated as the %
of days with supply of L/C/E or both L/C and E on hand. Supply was
estimated based on pharmacy rells. Other outcomes included MPR for
combination therapy 80% and time to therapy discontinuation (30
days w/out supply).
Results: Baseline characteristics were generally similar in the L/C/E
(n390) and L/CE (n825) groups, although L/C/E patients had
lower mean MPR for L/C in the 365 days pre-index (84 vs 87%;
p0.006). In multivariate analyses controlling for differences between
groups, receipt of L/C/E was associated with improvements of 17%
(95%CI: 10 24%; p0.001) in mean MPR vs L/CE (77 vs 66%) and
107% (95%CI: 59 169%; p0.001) in odds of MPR 80%, in addition to a 46% reduction (95%CI: 36 55%; p0.001) in risk of therapy
discontinuation.
Conclusions: L/C/E (Stalevo) is associated with improved compliance compared with L/CE administered as separate tablets. Additional studies are needed to conrm the results of this observational
study and assess the potential effects of differential compliance on
outcomes and costs.

Movement Disorders, Vol. 22, Suppl. 16, 2007

332
Effects of pramipexole on tremor and anxiety in de novo patients
with Parkinsons disease
H. Takahashi, F. Yoshii, K. Fujimoto, H. Chiba, R. Kumazawa,
S. Kobori, S. Takagi (Isehara, Kanagawa, Japan)
Objective: To evaluate the long-term effect of pramipexole on
tremor and anxiety, using UPDRS, Clinical Rating Scale for Tremor
(CRST) and State-Trait Anxiety Inventory (STAI).
Background: Tremor is the most frequently reported initial symptom
of Parkinsons disease (PD) and is known to be worsened by mood
disturbances such as anxiety.
Methods: Fifteen de novo patients with idiopathic PD (age: 62.1
7.7 years, duration of the disease: 2.2 1.5 years Hoehn and Yahr
stage: 1.9 0.7) were evaluated using the following scales every 3
months after the initiation of pramipexole treatment. None of them took
levodopa through out the follow up period. All patients underwent a
complete neurological examination, including the modied Hoehn and
Yahr Staging, UPDRS part 1 to 4 and CRST. They were also asked to
complete a self-report measure State-Trait Anxiety Inventory (STAI) to
assess the degree of anxiety.
Results: The mean dose of pramipexole after 3 and 6 months of
treatment were 1.7 0.8 and 1.9 0.9 mg/day, respectively. Scores of
UPDRS part 2 and 3 as well as total UPDRS improved signicantly
after 3 and 6 months of treatment. Among items in part 3, improvement
in tremor scores (sum of item 20 and 21) was higher than improvements in other items. The mean total CRST score improved signicantly from baseline (16.6 9.2) in 3 month, but not in 6 month
period. UPDRS tremor scores (sum of item 16.20.21) showed a highly
signicant correlation to CRST scores (Spearmans correlation coefcient: r 0.666, P 0.007), but not to STAI score. Visual hallucination in two, somnolence in two and constipation in one case were
seen as side effects, but they were of mild degree and no one dropped
out. There was no severe adverse event.
Conclusions: Pramipexole is effective in treating tremor in de novo
patients with idiopathic PD without affecting underlying anxiety.
333
Screening for LRRK2 mutations in UK familial Parkinsons disease
patients
A.J. Lewthwaite, T.D. Lambert, N.W. Wood, D.J. Nicholl,
K.E. Morrison (Birmingham, West Midlands, United Kingdom)
Objective: To determine frequency of known and potentially novel
LRRK2 mutations in familial Parkinsons disease (PD) patients in the
United Kingdom (UK).
Background: Genetic inuence on PD has been recognized as far
back as 19th century. Indeed up to 10% of PD patients may have a
positive family history. Study of familial PD has led to identication of
a number of PD genes, most recently LRRK2. This is a large gene with
51 exons and putative protein kinase function. Importantly a single
LRRK2 mutation G2019S appears to account for up to 5-6% of familial
and 1-2% of sporadic PD cases, although the frequency of this mutation
appears to be much higher in North African familial PD cases.
Methods: We recruited patients with PD from around the UK with a
history of at least 1 other affected family member and apparently
autosomal dominant inheritance. Patients either self-referred or were
referred by colleagues. Each affected participant had detailed clinical
assessment of parkinsonian features including use of UPDRS and
Hoehn & Yahr scales and Video recordings. We are currently completing screening these familial PD patients by genomic sequencing for
known and novel LRRK2 mutations in all 51 exons.
Results: We recruited 51 affected participants from 44 families
which have 2 or more members affected by PD. There are 28 males and
23 females, average age of affected participants is 69, range 39 to 89.
Average age of disease onset is 58 with an average disease duration of
11 years. Sequencing results from 44 probands show heterozygous
G2019S mutations in 3 probands (6.8%) and heterozygous R1441C
mutation in 1 proband (2.3%). These are similar to mutation frequencies identied in previous studies. The phenotype associated with these

POSTER SESSION I, TUESDAY, JUNE 5, 2007

LRRK2 mutations generally appears to be of typical PD. Where mutations have been identied in probands and samples are available we
have also screened other affected and unaffected family members.
Further sequencing results from remaining exons are awaited.
Conclusions: In this large cohort of UK PD families we are screening
for mutations in all 51 LRRK2 exons. From data available to date we
have identied pathogenic LRRK2 mutations in 4 probands (9.1%).
This supports results from previous studies and further emphasises the
importance of this gene in our understanding of the pathogenic mechanisms involved in PD.
334
Patient report of initial symptom in Parkinsons disease, ataxia,
and essential tremor
D.A. Hall, M.A. Leehey, K. Howard, P. Hagerman, G. Zerbe,
T. Byers (Denver, Colorado, USA)
Objective: To determine the initial symptom of disease reported by
patients with Parkinson disease, ataxia, and essential tremor.
Background: Previous studies using chart review methods have
found that tremor is the initial symptom reported by 72% of patients
with Parkinsons disease. Similar studies describing the initial symptoms of essential tremor and ataxia have not been conducted.
Methods: We assessed the reports of initial symptoms among a
group of 88 patients who were evaluated in a genetic screening study
being conducted in the movement disorder clinic. All subjects had
parkinsonism, ataxia, or tremor. Diagnostic criteria for Parkinsons
disease (Gelb criteria), cerebellar ataxia (ICARS score greater than
4/18), and essential tremor (Louis criteria) were assessed on each
patient as part of the neurological examination by a movement disorder
specialist. Each patient was asked their initial symptom by the genetic
counselor and a second time by the movement disorder specialist.
Results: Parkinsons disease was present in 54/88 patients (64%),
17/88 (19%) had ataxia, and 17/88 (19%) had tremor. Probable ET was
present in 5/17 patients, 5/17 had possible ET, two had head tremor,
and 5/17 had hand tremor too mild to fulll Louis critieria. Mean age
was 60 years, and 51% were male. Among the ataxia patients, 70%
reported their initial symptom as balance difculties. Among the patients with essential tremor, 82% reported tremor as their initial symptom. Among the patients with Parkinson disease, only 44% reported
tremor as their initial symptom. Other symptoms commonly reported
included dragging of a leg, change in handwriting, shoulder droop, leg
jerks, balance problems, stiffness, and decreased ne nger movements.
Conclusions: The most common initial symptom reported by patients
with ataxia is balance difculty. Tremor is the most frequently reported
initial symptom in essential tremor. However, patients with Parkinsons
disease often report initial symptoms other than tremor. These ndings
suggest that initial symptomatology in Parkinsons disease may be
subject to variation in the manner in which symptom recall is ascertained.
335
Neurophysiological correlates of Parkinsonian dyskinesias in subthalamic oscillatory activity
S. Marceglia, A. Leone, G. Foffani, F. Cogiamanian,
S. Mrakic-Sposta, F. Tamma, E. Caputo, S. Barbieri, A. Priori
(Milan, Italy)
Objective: To study local eld potentials (LFPs) rhythms and their
non-linear synchronizations to characterize the pathophysiology of
dyskinesias in patients with Parkinsons disease (PD).
Background: The neurophysiological correlates of dyskinesias are
still unclear. LFPs recorded through DBS electrode helped clarifying the functional state of the basal ganglia at the network level.
Methods: We analyzed LFPs in 5 patients (6 nuclei) with PD in the
non-dyskinetic on condition and in the dyskinetic on condition, and
compared with the off levodopa condition (%change). LFP rhythm
power was analyzed in the low-frequency band (LF, 2-7 Hz), the
low-beta band (LB, 13-20 Hz), and the 300 Hz band (HF, around 300

S103

Hz); LFP non-linear synchronizations within the LF and the LB bands

were characterized through bispectral analysis.
Results: When compared with the off levodopa condition, the LF
rhythm power more markedly increased in the dyskinetic than in the
non-dyskinetic on condition ([meanSE] 23.948.9 vs
548.2341.2 %), whereas the LF non-linear synchronizations decreased in the non-dyskinetic on condition and increased in the
dyskinetic on condition (-18.8 18.5 vs 1239.8749.8%). Both the
LB rhythm power and the LB non-linear synchronizations decreased
more in the dyskinetic than in the non-dyskinetic on condition
(-27.8 12.8 vs -60.15.3 and -0.0944.5 vs -36.237.3%). Finally, HF rhythm increased more in the dyskinetic than in the
non-dyskinetic on condition (20.4 8.5 vs 150.8122.2%).
Conclusions: Our results suggest that, in addition to the known LF
rhythm power increase, the dyskinetic condition is characterized also
by an abnormal increase in the LF non-linear synchronization pattern
and in the HF rhythm power and by a strong decrease in both the power
and non-linear synchronization of the LB rhythm. Hence, abnormal BG
LFP rhythm interactions contribute to the pathophysiology of parkinsonian dyskinesias.

336
Adherence to antiparkinson medication in a multi-centre European study
D. Grosset, A. Antonini, M. Canesi, G. Pezzoli, A.J. Lees, K. Shaw,
E. Cubo, P. Martinez-Martin, O.O. Rascol, L. Negres-Pages,
A. Senard, J. Schwarz, K. Strecker, H. Reichmann, A. Storch,
M. Lohle, K. Grosset (Glasgow, United Kingdom)
Objective: To characterise patient adherence to antiparkinson medication in a 7-centre European study in 5 countries.
Background: Medication adherence is often sub-optimal, even in
symptomatic and severe disorders. Sub-optimal antiparkinson medication adherence is reported in 2 single centre studies using electronic monitoring (Philadelphia, US; Glasgow, UK), which is surprising given the careful approach to medication observed in many
PD patients, and the reminder to take medication expected from
wearing-off symptoms.
Methods: Patients with idiopathic Parkinsons disease (PD) had
one month of antiparkinson medication monitoring by electronic
bottles (MEMS, Aardex, Switzerland) which record the date and
time of bottle opening. Data are mean (SD) except adherence
measures which are median (interquartile range, IQ). Adherence
measures used standard denitions: (a) Total adherence: the overall
dosage taken during the monitoring period, as a percentage of the
total prescribed (b) Days adherence: the number of days on which
the correct number of doses was taken (c) Timing adherence: the
proportion of doses taken at the correct time interval, based on
pharmacological principles.
Results: Mean age was 65 years (SD 10), 68% male, Unied Parkinsons disease motor rating score 23 (SD 10). In 69 patients taking a
total of 168 antiparkinson drugs, medication was taken 4.3 (SD 2) times
per day, with a total of 8 tablets (SD 4) per day. Total adherence to
antiparkinson medication was 98% (IQ 93-100), the correct number of
doses per day was taken on 86% of days (IQ 62 - 95), and overall 23%
of doses (IQ 7 - 48) were taken at correct time intervals. Once daily
drugs were taken more consistently (96% at correct time intervals) than
medication taken more frequently (range 7-25% at correct time intervals, p0.0001).
Conclusions: Total adherence to antiparkinson medication is
good, but timing is not, resulting in uctuating therapeutic coverage
and pulsatile dopaminergic stimulation. Methods of smoothing therapeutic coverage (eg. setting xed time intervals by pharmacological principles, developing agents with longer half-lives) merit further study.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S104 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

337
Safety of Zydis selegiline orally disintegrating tablet (ODT) with
concomitant antidepressant therapy in Parkinsons disease (PD)
M.F. Lew, K.D. Sethi, G. Kricorian (Augusta, Georgia, USA)
Objective: To determine the safety of selegiline ODT and concomitant antidepressant therapy in patients with Parkinsons disease (PD).
Background: Coadministration of monoamine oxidase inhibitors
(MAOIs), such as selegiline, with antidepressant medications can increase the risk of adverse events (AEs). A new orally dissolving tablet
(ODT) formulation of selegiline bypasses the hepatic circulation and
has been associated with lower rates of AEs than oral selegiline.
Methods: Of 254 patients with PD who received selegiline ODT 2.5
mg/day for up to 4 years in a long-term open-label extension study, 49
(19.3%) received concomitant antidepressant medication. Adverse
events were recorded for these patients.
Results: Of the 49 patients receiving selegiline ODT and an
antidepressant, 33 (67.3%) were male and 47 (95.9%) were white.
Thirty-two patients received a tricyclic antidepressant (TCA), 14
received a selective serotonin or serotonin-norepinephrine reuptake
inhibitor (SSRI/SNRI), and 18 received another class of antidepressant (eg, norepinephrine and specic serotonergic antidepressant,
tetracyclic antidepressant, norepinephrine-dopamine reuptake inhibitor). Eleven patients received more than 1 antidepressant, and 2
patients received more than 1 antidepressant concurrently. The most
common indications for antidepressant therapy were insomnia
(57.1%), and depression/anxiety (31.7%). In total, 69.4% of patients
treated for insomnia with an antidepressant received a TCA. Of
patients treated for depression/anxiety, 50.0% received an SSRI/
SNRI and 20.0% received a TCA. There were no signicant interactions, changes in vital signs, or occurrence of the serotonin
syndrome.
Conclusions: Selegiline ODT taken with antidepressants in this small
cohort was safe and well tolerated with no signicant interactions. A
prospective study with a larger cohort is needed to further evaluate the
safety in conjunction with antidepressant therapy.
338
Switch from an oral dopamine agonist to rotigotine transdermal
patch in Parkinsons disease
P.A. LeWitt, J.M. Patton, D.G. MacMahon, J. Jankovic (Southeld,
Michigan, USA)
Objective: This trial assessed the safety and tolerability as well as the
effect on Parkinsons disease (PD) symptoms in subjects with idiopathic Parkinsons disease when switched overnight from ropinirole,
pramipexole, or cabergoline to transdermal rotigotine.
Background: Rotigotine, a nonergolinic, D3/D2/D1 dopamine receptor agonist delivered via a transdermal system, is under investigation
for once daily treatment of idiopathic Parkinsons disease.
Methods: This Phase 3b, open-label trial included a pre-treatment
period (within 28 days before the overnight switch), a baseline visit
(Day 0), and a 28-day treatment period. Subjects received 2-8 mg/24h
rotigotine transdermal patch corresponding to their previous total daily
dose of oral dopamine agonist (maximum doses at entry were 9 mg
ropinirole, 2 mg pramipexole, and 3 mg cabergoline). Rotigotine dose
adjustments were permitted to achieve the most efcacious dose. Effects on PD symptoms were assessed by changes from baseline to the
end of treatment in the UPDRS and other scales. Subjectstreatment
preferences also were assessed.
Results: One hundred and nineteen subjects enrolled, 116 of whom
switched to rotigotine (47 from ropinirole, 47 from pramipexole, and
22 from cabergoline). Ninety percent of the subjects who switched
completed the Treatment period. Eighty percent of subjects did not
require dose adjustment after switching to rotigotine, while 11 of 116
subjects (9.5%) had one adjustment. Twelve subjects withdrew early, 5
due to AEs (dermatitis, tachycardia, insomnia, depression, nausea). For
all subjects completing the Treatment period, mean changes in UPDRS
parts II and III were -1.4 and -2.5, respectively. More than 75% of
subjects preferred using a patch to taking a pill or capsule for PD. Most

Movement Disorders, Vol. 22, Suppl. 16, 2007

AEs reported during rotigotine treatment were consistent with stimulation of dopamine receptors and use of transdermal delivery systems.
Application site reaction (8.6%) was most often reported followed by
nausea (6%), somnolence (6%), and headache (5.2%).
Conclusions: In this trial, an overnight switch from ropinirole,
pramipexole or cabergoline to rotigotine not only was well tolerated,
without exacerbating PD symptoms, but resulted in improvement in
mean UPDRS scores and patient preference for the rotigotine transdermal system over oral dopamine agonists.
339
Vascular events in Parkinsons disease with hyperhomocysteinemia
R. Ribacoba, M. Menendez, J.R. Virgili, G. Jimenez, C. Huerta,
V. De la Vega (Mieres, Asturias, Spain)
Objective: Describe vascular events in the history of Parkinsons
disease (PD) patients treated with L-Dopa and evaluate the presence of
hyperhomocysteinemia as risk factor for vascular disease.
Background: Hypermocystinemia has been associated with L-dopa
therapy.
Methods: We reviewed the records of 88 patients (30 men and 58
women) suffering from PD who underwent blood test to determine
plasma homocysteine level and the MTHFR gen polymorphism. PD
diagnosis was made according to the Brain Bank of London Criteria.
Normal lomocysteine level was 12 milimol/L, High homocysteine
level was 15 milimoles/L; between 12 and 15 it was considered as
undened. We checked the presence of vascular events after being put
on L-Dopa treatment. The mean time in treatment with L-Dopa was 84
months and the mean dose 650 mg.
Results: We detected 37 patients with hyperhomocysteinemia: eight
suffered stroke (polymorphisms: 5CC, 4 CT, 2TT); six suffered ischaemic cardiopathy (3 CT, 2 TT, 1 CC); three suffered independent
event of stroke and IC (1 CC, 1 CT, 1 TT) and 2 PA (both TT). Six
patients had high blood pressure and one associated diabetes mellitus
type II. Nineteen patiens showed homocysteina levels in indened
region; three suffered stroke and four ischaemic cardiopathy, six had
HBP. 32 patients had normal homocysteine level, two of them with
normal blood presion suffered a stroke.
Conclusions: The most frequent allele for the MTHFR polymorphism in our population is C. The presence of vascular events is higher
in L-Dopa treated PD patients with hyperhomocysteinemia than in
those with normal homocysteine level. Hyperhomocysteinemia might
be an independent risk factor for vascular events in PD patients treated
with L-Dopa, but we not observed new vascular after hyperhomocysteinemia was known.
340
Safety of concomitant therapy with rasagiline and antidepressants
in Parkinsons disease
M. Panisset, S. Schwid, W. Ondo, C. Fitzer-Attas, J.J. Chen
(Montreal, Quebec, Canada)
Objective: To assess the safety of combining antidepressant therapy
with rasagiline therapy in patients with Parkinsons disease (PD).
Background: Certain serotoninergic drugs, when used in combination, may trigger a potentially serious serotonin toxicity (ST) characterized by major adverse events (AEs) (confusion, fever/hyperthermia,
diaphoresis, myoclonus, hypertonia, tremor, shivering, and hyperreexia) along with other minor psychic, autonomic, and neuromuscular
AEs. Such ST has not been reported to date with rasagiline, a novel,
selective, and irreversible MAO-B inhibitor now in use for the treatment of Parkinsons disease.
Methods: All records of PD patients who had taken antidepressants
concomitantly with rasagiline in rasagiline clinical trials were assessed
for 15 AEs suggestive of ST. The incidence rates of the selected AEs
in these patients were compared with incidence rates in patients taking
rasagiline only, using a Fisher Exact 2-tailed test. Possible combinations of AEs suggestive of ST were analyzed.

POSTER SESSION I, TUESDAY, JUNE 5, 2007

Results: From a total of 1361 patients who ever took rasagiline in
controlled clinical trials, 323 were noted to have taken a concomitant
antidepressant, for a median time of 367 days (range 1-3067 days). The
most common exposures were to amitriptyline (n112), sertraline
(n75), paroxetine (n70), trazodone (n60), and citalopram (n34).
Rate ratios indicated that sleep disorder (10.1 vs 6.3; P.0005), dyspnea (2.9 vs 1.6; P.03), and confusion (2.9 vs 1.5; P.02) occurred
signicantly more frequently in patients taking rasagiline plus antidepressant than in patients taking rasagiline alone; these are all known
AEs of antidepressants and symptoms of depressive disorders. Termination rates were similar with and without antidepressants, and no
apparent cases of ST were identied.
Conclusions: Use of antidepressants in PD patients taking rasagiline
does not appear to cause any unexpected AEs or increase the rate of
termination. Antidepressants appear to be safe for use in conjunction
with rasagiline for PD.
341
Dening features of subsyndromal depression in Parkinsons disease
D.A. Nation, H.L. Katzen, R.A. Rodriguez, J.A. Ledon, A. Capano,
S. Papapetropoulos, B.V. Gallo, J.R. Jagid, B.E. Levin (Miami,
Florida, USA)
Objective: To characterize the dening features of subsyndromal
depression (SSD) in Parkinsons disease (PD).
Background: Individuals with SSD report depressive symptoms, but
do not meet criteria for major depression. The incidence of depression
in PD is estimated to be 40%. Although there is a high prevalence of
SSD in normative samples, less is known about the frequency and
characteristics of SSD in PD.
Methods: The sample consisted of 107 PD patients undergoing
pre-surgical evaluation for deep brain stimulation (DBS). Mean age
was 64.7 years (SD9.3), mean education was 13.9 years (SD4.3),
and mean Mini Mental Status Examination (MMSE) was 26.6
(SD2.7); 72.9% were male. Participants were administered the Beck
Depression Inventory Second Edition (BDI-2). SSD was dened as a
BDI score between 4 and 13, with none of the endorsed items rated
severe in frequency or intensity.
Results: Fifty participants (41.7%) were classied as having SSD, 49
(40.8%) were classied as depressed and 8 (6.7%) were nondepressed. The
groups did not differ signicantly in age, education, or mental status.
Within the subsyndromal group, the most frequently endorsed items were
in the somatic-affective dimension, with loss of energy and fatigue being
the most commonly endorsed symptoms (94%). Participants with SSD
were much more likely to report loss of pleasure (54%) rather than sadness
(0.08%) or loss of interest (22%). The most common cognitive symptom
among those with SSD was indecisiveness (38%).
Conclusions: These data suggest that among PD patients considering
DBS surgery, SSD is at least as common as clinical depression. These
data further suggest that the symptoms experienced by those with SSD
are predominantly somatic-affective in nature, and may be largely
driven by a loss of the ability to experience pleasure in the context of
a general condition of fatigue and low energy.
342
Quality of life inuenced by presence of patients with Parkinsons
disease relatives
S.M. Nica, I.E.-V. Davidescu, G. Mihailescu (Bucharest, Romania)
Objective: To identify which disease related factors are the most
relevant for quality of life (QoL) in patients with idiopathic Parkinsons
disease (PD) and the correlation of QoL with the presence of relatives
near the patients.
Methods: We studied 29 patients with early and advanced PD. 18
patients had some relatives around them almost all the time (spouse,
brothers or sisters) and 11 of them had no relatives (or their relatives
lived far away and came only sporadically to look after them). All the
patients were treated correctly for their PD and were not depending of
their caregivers for any on their daily living activities. All the patients

S105

were assessed using Unied Parkinsons Disease Rating Scale (UPDRS

part III), Hoehn and Yahr staging, and Mini Mental State Examination
(MMSE) and a disease specic QoL questionnaire for patients with PD,
the PDQ-39.
Results: The most closely associated factor with QoL was the presence of depression and anxiety, but the depression itself was closely
associated with the presence of some relatives around (more than
caregivers) the patient; of course, postural instability, motor disability,
and cognitive impairment decrease the QoL scores. In addition, patients
with freezing and akinetic PD had worse QoL scores than those with
tremor dominant disease. The presence of dyskinesia did not decrease
the QoL scores, even in patients with troublesome dyskinesia.
Conclusions: Mood disturbance (depression, anxiety, sleep disturbances), postural instability, presence of motor uctuation, cognitive
impairment and the presence of some relatives around the patient
almost the time are the most important factors inuencing QoL of our
patients. In some patients, the QoL scores were less inuenced by
specic symptoms of PD than the presence of one active relative.
343
The effects of loudness and noise on speech intelligibility in Parkinsons disease
A. Halpern, J. Spielman, L. Ramig, J. Cable, I. Panzer, A. Sharpley
(Denver, Colorado, USA)
Objective: The purpose of this study was to examine the effects of
increased loudness on speech intelligibility in individuals with Parkinson diseases (PD), by means of the Diagnostic Rhyme Test (DRT) in
noise and non-noise conditions.
Background: Previous studies utilizing the LSVT for individuals
with PD have generated the only published Level I data for speech
treatment in PD. While the focus of the LSVT is to increase vocal
loudness, past studies have documented other system wide effects
such as increased articulatory precision. Increases in speech intelligibility following LSVT have been reported clinically; however,
single word intelligibility measures have been difcult to evaluate,
due to a ceiling effect in quiet environments. Thus, we chose to use
a standard for measuring intelligibility (DRT) in both noise and
non-noise conditions.
Methods: Fourteen subjects (Ss) with PD were randomly assigned to
participate in 16 sessions (one month) of LSVT, or 16 sessions (one
month) of a control enunciation treatment (ENUNC). Ss were recorded
while reading a 12 DRT word list pre and post treatment in a sound
treated booth. DRT sound les were analyzed by trained listeners at
Dynastat, Inc. in three noise conditions: No noise, Mall and Babble.
Results: Both the ENUNC and the LSVT groups increased in mean
dB SPL pre to post treatment (LSVT 4.5 (p0.01); ENUNC 1.2 (not
signicant)). Mean DRT scores indicate a signicant interaction between treatment Method (LSVT, ENUNC) and Noise (F18.37,
p0.0001). More words were correctly identied for both treatment
methods post treatment under Babble and Mall noise conditions. The
differences in mean pre/post scores were greater for LSVT (Babble:
15.4; Mall: 14.8) than for ENUNC (Babble: 0.2; Mall: 5.1).
Conclusions: These ndings show that speech intelligibility improved more when treatment was focused on increased loudness than
on enunciation, thus highlighting the role of loudness on improving
speech intelligibility. Based on these results, utilizing the DRT in noise
appears to provide a means to quantify the increase in intelligibility that
LSVT patients have for many years subjectively reported. This research
was funded by an NIH/NIDCD R01 DC01150.
344
What looks like a duck and sounds like a duck, may not really be
a duck: Acute hemiparesis 3 weeks after DBS
Q.A. Shamim-Uzzaman, E.A. Shamim, C.G. Kalhorn, A.S. Mandir,
F.L. Pagan (Washington, District of Columbia, USA)
Objective: To present an unpublished complication of DBS.
Background: DBS is rapidly emerging as one of the most promising
modalities in the treatment of many Movement Disorders such as

Movement Disorders, Vol. 22, Suppl. 16, 2007

S106 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

idiopathic Parkinsons disease (PD) and essential tremor (ET). Although reports abound detailing hardware complications such as migration, fracture or infection, much is still to be learned about the
effects of the electrodes on the brain tissue itself.

Methods: Here we present the history and physical examination of a

patient with ET and PD with bilateral DBS placed in the subthalamic
nucleus (STN) and the ventroposterior (Vop) nucleus. Ours is a novel
complication.
Results: A 71-year-old right-handed gentleman with known ET
and PD presented to the ER with sudden right-sided weakness 2
days after a fall. Physical examination revealed mild weakness in
the right upper and lower extremities (strength 4/5). Three weeks
earlier, he had undergone implantation of DBS (Activa 3387) electrodes spanning the STN and Vop bilaterally to help both the PD and
ET. Post-op recovery was uneventful and initial programming one
week prior to admission showed an excellent response in both
tremor and rigidity. Unfortunately, as is common for this treatment,
the patient did not appreciate improved balance. In the ER, a
non-contrast brain CT showed an ill-dened ovoid hypodense focus
in the frontoparietal region will minimal mass effect and without
midline shift. There was no hemorrhage. These changes were not
present on the post-op CT 24 hours after implantation of the DBS
electrodes. An MRI of the brain showed hyperintensity surrounding
the electrode tract with a negative DWI. Subsequent MRI one month
later showed near complete resolution of the previously noted
edema surrounding the left DBS wire. The electrodes themselves
were unchanged from initial insertion (i.e., no migration or dislocation).
Conclusions: The patients symptoms likely resulted from focal
edema sustained after trauma. The symptoms resolved as the edema
improved. Interestingly, the edema was localized entirely around the
DBS wire and electrode. It was felt the edema occurred from the DBS
electrodes during the fall. To our knowledge, this is the rst reported
case of trauma leading to a transient symptomatic event localized to the
presence of DBS hardware.
345
Complications of spinal surgery in Parkinsons disease: Case reports of 3 patients
E. Wolf, K. Mair, A. Muigg, K. Twerdy, W. Poewe (Innsbruck, Tirol,
Austria)

FIG. 1 (344).

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: We want to report the outcome of instrumented spinal

surgery in 3 PD patient to highlight potential complications.
Background: Previous studies have reported an increased risk for
surgical failure and postoperative complicatations in Parkinsons disease (PD) patients undergoing spinal surgery.
Results: A 61 year old PD patient with motor uctuations suffered
from a claudicatio spinalis. His spinal CT scan revealed a rethrolisthesis L5/S1 with a spinal canal stenosis. The patient received a
dorsal fusion of L5/S1. Postoperatively he did well, but on the 8.
postoperative day he complained a sudden pain and paresis of his
right foot. The CT scan showed a blow out fracture of the right L5
pedicle. A decompression of the radix L5 improved pain but his
paresis persisted. A second 69 year old PD patient with dyskinesias
and uctuations suffered from a pain in L4 and L5 on the right side
due to a lumbal spinal stenosis. He underwent a laminectomy
L2-L5. 5 month later he developed again pain in L3 right as a
consequence of a kyphoscoliosis L3/L4. A second operation with a
dorsal fusion of L2/L5 was performed. Again 5 month later he
complained pain in L2 right. The CT scan showed a blow in fracture
of L2 with dislocation of the screw. The patient needed a further
operation with decompression of the right radixL2 and an explantation of the whole instrumentation. Patient 3 underwent a L4-L5
laminectomy and a dorsal fusion because of lumar pain due to a
rethrolisthesis L4/L5. Symptoms resolved for 1 month when severe
low-back pain reoccured. X-rays demonstrated a severe conjunctional scoliosis at the level of L3/L4. In a second operation the
instrumentation was extended L2/L5 which improved pain. 10
month later she returned with a left sided radiculopathy L5. Again
an extension of the dorsal fusion L2 to S1 and cage implantation
L5/S1 was performed. Only 3 month later she suffered from a right
sided radiculopathy L5. CT scan showed an instability of L5/S1

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

with an dislocated cage, requiring a forth operation to decompress
the L5 root on the right.
Conclusions: With our case reports we want to demonstrate the
increased risk of failure after spinal surgery in PD patients. Abnormal
movements, scoliosis and reduced bone density may contribute to this
risk. PD patients undergoing spinal surgery should be counselled carefully regarding this operational risk.

POSTER SESSION II
Wednesday, June 6, 2007
9:00 AM 4:00 PM
Rumeli Hall, Lower Level
Poster Numbers 346 662 and Poster
Number 788
Authors Present: 12:30 PM2:30 PM
DRUG-INDUCED MOVEMENT
DISORDERS

S107

Results: Of the 194 African-Caribbean inpatients that participated at

baseline, 166 (mean age 54.4 years, 74.1% males, 9.6% on lithium) had
at least one follow-up assessment giving a total of 758 follow-up
observations. The use of lithium was negatively correlated with both
persistence (adjusted -2.29, 95% CI: -4.29, -0.29) and onset of
abnormal movements (adjusted -3.21, 95% CI: -5.67, -0.76),
independent of possible confounding factors. In patients with TD this
reects a 2 to 4 point reduction on the AIMS (range 0-23).
Conclusions: Lithium may confer a benecial neurotrophic and
neuroprotective effect with regard to the treatment and prevention of
TD in patients receiving long-term antipsychotic treatment.
References:
Van Harten PN, Matroos GE, Hoek HW, Kahn RS (1996): The
prevalence of tardive dystonia, tardive dyskinesia, parkinsonism and
akathisia The Curacao Extrapyramidal Syndromes Study: I. Schizophr
Res 19:195-203.
Van Harten PN, Hoek HW, Matroos GE, Os J van. (2006) Incidence
of tardive dyskinesia and tardive dystonia in African-Caribbean patients on long term antipsychotic treatment. The Curacao Extrapyramidal Syndromes Study: V. J Clin Psychiatry. 67:1920-1927.

Table 1. Associations between lithium and change in AIMS

score* (N166 yielding n758 follow-up observations)
Crude

346
Veralipride: A case report of irreversible dystonia
M.T. Rivas, J. Pascual, A. Sesar (Santiago de Compostela, Spain)
Objective: We present a case of a female who developed a very
severe dystonic picture after treatment with veralipride for six months.
Background: Veralipride is a benzamide derivative effective in the
treatment of menopausal syndrome (particularly hot ushes) because of
its antigonadotropic action. It may rarely cause extrapyramidal adverse
effects such as Parkinsonism and dyskinesias. In May 2005, the Spanish Agency for Medical Products decided to suspend the commercialization of this drug, due to a negative benet risk balance.
Methods: 44 year old female with no relevant previous history
started with tongue tremor, left hemifacial spasm and left laterocollis.
Subsequently, she developed masseter muscle dystonic contracture that
prevented her from open her mouth, blurred speech and severe dysfagia. She had been on veralipride for six months.
Results: Blood tests (including copper and ceruloplasmin), EEG, CT
scan and MRI were normal. Benzodiazepines, antiepileptic drugs and
neuroleptics were of no use to ameliorate the symptoms. Type A
botulinum toxin provided slight improvement.
Conclusions: Since no other possible cause for the extrapyramidal
picture was found, veralipride was considered the responsible for the
dystonic condition suffered by the patient. Some cases, unlike this one
reversible, have been described. Lack of response to drug withdrawal
and to several medications has provoked a situation of severe deterioration in the patients quality of life.
347
Evidence that lithium protects against tardive dyskinesia: The
Curacao Extrapyramidal Syndromes Study VI
P.N. van Harten, H.W. Hoek, G.E. Matroos, J. van Os (Amersfoort,
Netherlands)
Objective: To determine whether the use of lithium has an effect on
the course and onset of tardive dyskinesia (TD).
Background: TD is a severe and potentially irreversible side effect of
antipsychotics. Lithium may have neuroprotective properties and therefore could affect the occurrence or severity of tardive dyskinesia.
Methods: The psychiatric inpatient population of the Caribbean
island Curacao was investigated at baseline and six follow-ups over a
period of nine years. The effect of lithium was calculated for (i) change
in Abnormal Involuntary Movement Scale (AIMS) for individuals with
any baseline value on the AIMS and (ii) onset of any AIMS abnormality for individuals who at baseline had a score of zero on the AIMS.

Predictor variable

95%CI

Adjusted**
P

95%CI

Prevalent baseline lithium (n166) -2.81 -4.83,-0.79 0.006 -2.29 -4.29, -0.29 0.025
Incident follow-up lithium (n150) -2.66 -4.95, -0.36 0.023 -2.93 -5.20, -0.66 0.011

*Analyses are adjusted for baseline score on the Abnormal Involuntary Movement Rating Scale (AIMS); ** Analyses are also adjusted for
the following baseline variables: age, sex, diagnosis, dosage of antipsychotics, anticholinergics, antidepressants, benzodiazepines and other
medication.

Table 2. Associations between lithium and onset of tardive

dyskinesia (N66 yielding 318 follow-up observations)
Crude
Predictor variable
Prevalent baseline
lithium (n66)
Incident follow-up
lithium (n55)

Adjusted*

95%CI

95%CI

-3.28

-5.66, -0.89

0.007

-3.21

-5.67, -0.76

0.010

-2.78

-7.22, 1.65

0.22

-4.56

-9.25, 0.14

0.057

* Analyses are adjusted for the following baseline variables: age,

sex, diagnosis, dosage of antipsychotics, anticholinergics, antidepressants, benzodiazepines and other medication.
348
Ephedrone-induced Parkinsonism: Clinic-neuroimaging study
Y. Sanotsky, M. Selikhova, L. Fedoryshyn, Y. Matviyenko,
I. Komnatska, M. Kyrylchuk, A. Friedman, L. Krolicki, A.J. Lees
(Moscow, Russian Federation)
Objective: To detail the clinical and radiological features of Ephedrone induced Parkinsonism and conrm its cause as chronic manganese poisoning.
Background: An epidemic of Parkinsonism has been recently reported in Russia, Ukraine, Estonia and other parts of Eastern Europe
due to intravenous Ephedrone abuse, a potent amphetamine-like agent
prepared by mixing the pseudoephedrine contained cold remedy (Coldact 10tablets) with about 0.7gm potassium permanganate. The chemical reaction leads to manganese accumulation in the brain and other
body tissues.
Methods: 13 male patients (mean age 29.9) have been carefully
studied and following tests carried out: UPDRS, MMSE, FAB, BDS,
L-Dopa challenge test, Snifn Sticks and brain MRI 5 have had DAT
SPECTscans.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S108 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Results: Symptoms began on average 8.53.2mths after daily 42
28ml IV Ephedrone use.The patients developed a highly distinctive
clinical picture with retropulsion, a severe dysarthria, bradykinesie,
rigidity and dystonia.Emotional lability, apraxia of eyelid opening,
micrographia and palilalia were also note in some patients but apart
from mild hypometric saccades eye movements were normal. All
patients had preserved olfaction and failed to respond to levodopa. FAB
revealed a mild executive dysfunction, Beck scale- mild depression
186, MMSE 28.71.2. In 2.5 1.6yr of disease duration UPDRS
score was 49.813.6 and had some rough correlation with the duration
of Ephedrone use (r0.66, t(N-2)2.61, p0.02) and daily dose
(r0.63, t2.81, p0.02); MRI showed symmetrical hyperintense T1weighted signals in the globus pallidus, putamen, and less intense
abnormalities in SN, nn. caudatus and dentatus. DATscan was normal.
Symptoms stabilised after drug elimination.
Conclusions: Ephedrone abuse results in an irreversible distinctive
neurological syndrome characterized by l-dopa unresponsive Parkinsonism, retropulsion, dysarthria and dystonia due to chronic manganism. Public health prevention and early manganese chelation are the
most important treatment approach.
349
Reversible parkinsonism induced by short-term treatment with
valproate in Alexanders disease
G. Sechi, K.S. Paulus, G.A. Cocco, G.M. Pes, G. Sau, V. Agnetti
(Sassari, Italy)
Objective: To report the rst case of reversible parkinsonism induced
by short term treatment with valproate in a patient with Alexanders
disease (AD), and to suggest a pathophysiological mechanism.
Background: The risk of parkinsonism on valproate is higher than
with other antiepileptic drugs, but the pathophysiology of this disorder
is currently unknown. AD is a sporadic disorder affecting central
nervous system white matter, that can rarely arise in adults, usually due
to mutations in the gene encoding glial brillary acidic protein (GFAP).
In vitro studies indicate that valproate has specic gliotoxic activity. In
particular, this drug affects, also at low doses, radial astrocytes, which
comprise a cell population during distinct developmental periods and
radial glial-line cells persisting in the adult brain.
Methods: Case report: a 40-year-old female noticed progressive
bladder dysfunction since age 35, the appearance of a restless legs
syndrome at age 39 treated with pramipexole for few months, and a
progressive blurred speech in the last ve months. Neurological examination showed a severely dysarthric speech, hyperactive tendon reexes and a rhythmic palatal myoclonus at 1.5-2 Hz. Brain MRI
showed thinning of the medulla with multiple, white matter scattered
lesions involving mainly the brainstem and the cerebellum. Molecular
investigations showed a G to A substitution at nucleotide 209 in exon
1 of the GFAP gene in the heterozygous form resulting in change of
arginine to glutamine at position 70 (R70Q), in combination with a G
to A substitution at nucleotide 469 in exon 2 resulting in change of
aspartate to asparagine at position 157 (D157N).
Results: The administration of valproate at increasing dosages, until
500 mg twice daily, for the palatal myoclonus, led within 3 months to
severe bradykinesia and moderate coghwheel rigidity in all limbs.
Valproate was ineffective on palatal myoclonus. After discontinuation
of valproate the parkinsonism cleared within one month.
Conclusions: The astroglial changes typical of AD may facilitate the
occurrence of valproate-induced parkinsonism. A dysfunction of radial
glial-line cells may play a role both in valproate-induced parkinsonism
and in the pathophysiology of AD.
350
Parkinsonism induced by meoquine
M.G. Senol, M. Saracoglu (Istanbul, Turkey)
Objective: Meoquine, a 4 quinoline-methanol (Lariam) derivative
drug, is widely employed for oral prophylaxis and treatment of chloroquine-resistant and multidrug-resistant P. falciparum malaria.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Background: Neuropsychiatric disturbances (seizures, encephalopathy, psychosis) occur in approximately 1 in 10,000 travellers receiving
meoquine prophylaxis.
Methods: Recently we attended a 35-year-old man who developed
Parkinson symptoms after meoquine chemoprophylaxis.
Results: This secondary effect has not been reported before and
should be considered when evaluating patients on meoquine treatment
who present with Parkinsons signs.
Conclusions: It would be interesting to explore if this metabolite
could explain some of the neurotoxic effects attributed to meoquine.
351
Tardive eating dystonia: A case report
Y. Kutukcu, S. Bek, F. Ozgen, Z. Odabasi (Ankara, Turkey)
Objective: Tardive dyskinesia is a movement disorder caused by
the long-term use of dopamine receptors blocking agents such as
neuroleptics and it is most commonly characterized by repetitive,
stereotypic and rhymical involuntary movements. Tardive dystonia
is one of the most disabling variant of tardive dyskinesia and
characterized by sustained muscle or muscle group contractions that
produce repetitive movements or abnormal postures. Tardive dystonia commonly affects the face or neck, but may also involve the
arms, legs or trunk. Some involuntary tardive movements may be
partially suppressed by voluntary movements of the affected muscles. We report a patient of a 26-year-old-girl with schizophrenia
who developed tardive eating dystonia after seven months of treatment with olanzapine. This is the rst case report who developed
tardive eating dystonia induced by atypical neuroleptic. She was
admitted for evaluation of involuntary movements mainly involving
of orolingual and cervical muscles during eating. The spasms of
masticatory and lingual muscles is like jaw opening dystonia. This
patients dystonia became manifest only during eating and swallowing such as task-specic dystonia. There were no spontaneous
involuntary movements at rest. Her neurological and physical examination were normal. Her olanzapine treatment was discontinued,
and quetiapine treatment was initiated. There were no changes in her
dystonia. Treatment with clonazepame, biperiden Hcl and clozapine
was unsuccessful. She denied botulinum toxin treatment. All the
treatment was discontinued for 3 months, then her dystonia had
decreased by 80%, and she felt very well. After a few months she
was rediagnosed as a bipolar disorder with a manic episode. Two
months ago, valproic acid (1500mg a day) and aripirazole (20 mg a
day) were initiated. But her dystonia was worsen slightly again. This
case suggests that tardive eating dystonia may develop after atypical
antipsychotic drugs and the most effective treatment is to withdraw
the offending medication.
352
Oromandibulary dyskinesia and dystonia with khat chewers
L. Harms, F. Sporkert, H. Alwarith, F. Pragst, L. Dognitz (Berlin,
Germany)
Objective: The chewing of fresh leaves of the khat shrub is prevalent
in some countries of East Africa and in Yemen. Depending on the
amount chewed khat has a stimulating effect. Diverse health risks have
been described, however, no disorders of movement have been known
so far.
Background: During consultations in Sanaa, Republic of Yemen, in
2000 and 2001 of a number of ca. 300 neurological out-patients there
were three patients with severe oromandibulary dyskinesia and ve
with oromandibulary dystonia. None of these patients had ever been
given neuroleptic or dopaminergic medications, while all of them
chewed khat for several hours daily.
Methods: In addition to the neurological assessment, an analysis of
the khat abuse was made. A specially developed method made it
possible to determine the concentration of the most important metabolits in the hair. The hair of ve patients was analyzed as well as that of
people chewing khat with a varying degree of intensity without displaying neurological symptoms.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Results: In all test persons chewing khat up to 42 hours per week the
metabolites cathinone, norephedrine and pseudonorephedrine were
found in the hair. If khat was used more than 42 hours per week there
was a rapid increase in the concentration. The concentrations found
with the patients did not show any signicant difference to those of the
control group.
Conclusions: The frequent occurence of oromandibulary Movement
Disorders suggest a connection with excessive khat use. It seems
interesting that it is the oromandibulary muscles, which are immensely
used for khat chewing, which are especially affected. It is assumed that
the metabolites can, in case of a corresponding disposition, trigger
these symptoms. An inuence of khat metabolites on the dopaminergic
system is described. Pehek et al. (1990) showed that Cathinone in the
same way as Amphetamine increases the level of Dopamine in the
anterior Caudatus and Putamen. Thiel et al. (1994) reported about two
patients with dyscinetic disorders after medication of the sympathicomimetic Norpseudoephedrine. For the rst time it could be shown that
there seems to be a relation between excessive chewing of khat and the
manifestation of oromandibulary dyskinesia and dystonia. The presented method of hair analysis makes further investigations in a eld
possible which is also important under a socio-medical aspect for the
respective developing countries.
353
The course of tardive dystonia: A population based study the
Curacao Extrapyramidal Study VIII
P.N. van Harten, G.E. Matroos, J. van Os (Amersfoort, Netherlands)
Objective: To measure the various manifestations and the course of
Tardive dystonia (TDt) in patients with prevalent TDt at baseline and
incident TDt during follow-up.
Background: TDt is a severe side effect of antipsychotic treatment.
Previous published case series suggest a persistent course but suffer
from referral bias.
Methods: In a population based nine-year follow up study (one
baseline, six follow-ups) of chronic psychiatric patients (N194) on a
Caribbean island with a predominantly African-Caribbean population,
the course of prevalent and incidence TDt was measured with use of the
Fahn-Marsden rating scale. TDt was diagnosed if one body area was
involved with at least a mild rating on a severity factor or if two or
more body areas got a slight rating.
Results: Of the 194 patients included in the study, 26 had TDt at
baseline. Table 1 shows the course of TDt over the nine year
follow-up in the 26 patients with TDt at baseline. One patient had no
follow-up assessments and of the other 25 patients with TDt at
baseline 64% recovered, 20% persisted, and 16% had an intermittent
course. The severity of the TDt at baseline was signicantly higher
in the ve patients with a persistent course versus the 16 patients
who recovered (t3.01, P0.008). Between baseline and the rst
follow-up assessment, seven patients with severe TDt started with
clozapine; three had a total remission, three persisted and one
patient had an intermittent course (see table 1). Over the 27 patients
that developed TDt during the follow-up period 25 had at least one
follow-up assessment and of them 80% had a remission, 12% an
intermittent course and 8% persisted. In the 53 patients with TDt (26
patients at baseline plus 27 incidence cases), 67 body areas with at
least a score of mild (for eyes a moderate) were affected. The most
affected body areas were dystonia of the left (30%) and or right arm
(28%), followed by eyes i.e. blepharospasm (25%), neck dystonia
(23%), mouth dystonia (23%), trunk dystonia (13%), left leg (4%),
right leg (4%) and spasmodic dysphonia (2%).
Conclusions: The natural course of TDt is better than previously
suggested but severe cases tend to persist. The most frequently affected
body areas were hands, eyes (blepharospasm), neck and mouth.
References:
van Harten et al (2006) The Curacao Extrapyramidal Syndromes
Study V. J Clin Psychiatry 67:1920-27.

S109

The severity of tardive dystonia based on the score of the

Fahn-Marsden rating scale during the nine years follow-up
(one baseline and six follow-up assessments)#
Case
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18*
19*
20*
21
22*
23
24*
25*
26*

Age

Sex

T0

T1, T2, T3

T4, T5,
T6

Outcome

81
34
54
74
79
54
67
59
91
61
62
38
39
42
60
67
37
36
31
25
64
55
71
42
24
41

M
M
M
F
F
M
M
M
F
M
M
M
M
M
F
M
M
M
M
M
F
M
F
M
M
F

1,5
2
4
4
4.5
6
6
6.5
8
8
10
10
12
12
12.5
14
14
16
19
20
21
26
28
37
53
61

0, ., .
0, 0, 0
8, 0, 0
9, 0, 0
0, 30, 1,5
4, 0, 0
8, 16, 8
8, 0, 0
0, ., .
12, 0, 12
., ., .
24, 10, 0
9, 8, 16
24.5, 14, 19
0, 0, 0
13, 0, 0
19, 37, 37
10, 4, 0
0, 0, 0
0, ., .
., ., .
10, 16, 14
26, ., .
28, 36, 31
2, 0, 4
53, 55, 49

., ., .
0, 0, 0
4, 0, 10
0, 0, 0
., ., .
., ., .
8, 6, 0
0, 4, 0
., ., .
0, 0, 0
., ., 0
0, ., 0
16, 0, 0
10, 4, 10
0, 0, 0
., 0, 0
28, 0, 10
8, 0, 0
0, 0, 0
., ., .
., ., .
11, 12, 14
., ., .
33, ., .
0, 3, 0
48, 40, 33

Remission
Remission
Intermittent
Remission
Intermittent
Remission
Remission
Remission
Remission
Remission
Remission
Remission
Remission
Persistent
Remission
Remission
Intermittent
Remission
Intermittent
Remission
No follow-up
Persistent
Persistent
Persistent
Remission
Persistent

. missing data; *Switched to clozapine between baseline and the

rst follow-up (see text); #Baseline was in 1992, post baseline follow-up assessments were in 1993, 1994, 1996, 1997, 1998 and 2001
354
Clinical features of motor disturbances at toxic encephalopathy
provoked by using of substitute psychoactive substances
N. Fedorova, N. Amosova, T. Ismailova (Moscow, Russian
Federation)
Objective: Parkinsonian, dystonic and pseudobulbar syndromes are
the main motor disturbances at manganic encephalopathy (ME).
Methods: 65 patients with ME were observed (mean age 24.43.9
years; mean duration of the disease 8.75.6 months). All patients
were assessed using Unied Parkinsons Disease Rating Scale (UPDRS), scale of dystonia (Fahn S. et al., 1985), and Tinnettis scale. The
parkinsonian syndrome (hypokinesia, postural instability, rigidity and
tremor) was revealed in all patients. Hypokinesia (UPDRS 12.75.5)
was early symptom, predominated in axial muscles. The hypokinesia
was more expressed than the rigidity (UPDRS 5.93.2) (p0.05).
Postural instability manifested as violation of postural reexes, walking
with frequent falls. It was noted that the falls in the beginning of the
disease occurred in 53% of cases, propulsion - in 30%.
Results: The dystonic syndrome was revealed in 96% of patients. It
occurred more lately than the parkinsonian syndrome (during the rst
year from the onset) and was clinically multifocal. The determined
groups of muscles were more affected: low extremities (91%), vocal
and orofacial (89%), upper extremities (51%). Dystonia prevailed in
clinic of ME and provoked signicant disability. The severity of
dystonia correlated with parkinsonian syndrome, gait and balance disturbances. Pseudobulbar syndrome manifested in 69% of patients in
form of severe spastic-hypokinetic aphasia, disartria, dysphagia, dysphonia, increase of reexes of oral automatism.
Conclusions: Thus the clinical features of motor disturbances in ME
were different combinations of parkinsonian, dystonic and pseudobulbar syndromes. These syndromes developed in the beginning of the
disease or during the rst year. Dystonic syndrome led to signicant
disability in patients with ME.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S110 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

355
Tardive antidepressant drug-induced dyskinesia: Report on 5 cases
and search for MRI predictors
P.J. Blanchet, N. Ouatik, Y. Kuznetsov, A. Khiat, Y. Boulanger
(Montreal, Quebec, Canada)
Objective: 1) To report on 5 patients with antidepressant drug
(ADD)-induced tardive dyskinesia (TD). 2) To correlate ADD-induced
TD with apparent diffusion coefcient (ADC) changes measured by
MRI in the basal ganglia.
Background: ADDs have been associated with the early development of various hyperkinetic disorders. TD constitutes a rare and less
familiar complication. No reliable method of predictive value is available to identify subjects at risk for TD.
Methods: Thirteen age-matched patients under ADDs (5 TD cases, 8
control TD-free subjects) participated to the study after providing
consent. MRI experiments were conducted on a 1.5-T Sigma whole
body imager. Diffusion-weighted images were recorded using echoplanar imaging with the following acquisition parameters: TR 10,000
msec; TE95 msec; 160256 matrix; eld of view, 3636 cm2; slice
thickness, 3 mm with 1 mm gap; diffusion gradient factors b0 and
1000 s/mm2; total acquisition time, 20 sec. The ADC was estimated in
the basal ganglia on an MRI slice 4 mm above the anterior commissure,
using the Stejskal-Tanner equation. A two-sample t-test was used, and
a multinomial logistic regression ascertained whether ADC differences
remained signicant after age adjustment.
Results: Persistent orofacial TD developed within 4 yrs of ADD therapy
(N4) or upon withdrawal (N1) in our 5 cases. Mean (SEM) age at
TD induction was 6310.4 yrs (range 50-79). A single SSRI (paroxetine)
was ever used in 2 cases, 2 cases used several consecutive ADDs with
paroxetine last in series, and one case had abruptly stopped doxepin. Three
TD cases were hypertensive. The mean ADC value (mm2/sec) was higher
in TD subjects (0.8880.06710-3) than controls (0.7840.01710-3)
(p0.0014), and remained predictive after age adjustment (Chi
Square5.9, df1, p0.02). Age alone was not a predictor of TD (Chi
Square1.5, df1, p0.2).
Conclusions: Although performed on a small sample size, this analysis showed for the rst time an increase in basal ganglia ADC values
in ADD-induced TD patients relative to control subjects. The nding is
nonspecic but suggests a microangiopathic etiology. This method may
provide a useful marker for TD risk assessment and warrants further
studies. Caution should be used in exposing elderly subjects with basal
ganglia damage to long-term ADD.
356
Effects of repetitive transcranial magnetic stimulation on levodopa
induced dyskinesias and motor performance in Parkinsons disease
S. Sayin, R. Cakmur, E. Yaka, G. Yener, F. Uzunel (Izmir, Turkey)
Objective: To evaluate the effects of low frequency repetitive transcranial magnetic stimulation (rTMS) of supplementary motor area
(SMA) on levodopa induced dyskinesias (LID) and motor performance
in Parkinsons disease (PD).
Background: Neuroimaging studies have shown the over activation
of SMA in dyskinetic PD patients. This altered cortical excitability can
be transiently inhibited with 1 Hz rTMS of the stimulated region.
Methods: Seventeen dyskinetic Parkinsons disease patients were
randomized into two groups and 1Hz rTMS or sham rTMS was applied
for ten consecutive days. Patients were assessed both at baseline and
one day after rTMS treatment with L-dopa challenge test and videorecordings were performed. Dyskinesias and motor performance were
rated ofine by two blinded raters with video-recordings.
Results: After ten days of treatment with rTMS, we observed that 1
Hz rTMS induced a signicant reduction of dyskinesias (P 0.024)
without any change in motor symptoms. In the placebo group, there
was no signicant difference in dyskinesia scores.
Conclusions: These results showed that low frequency rTMS had
decreased LID, lasting for 24 hours, without a change in motor performance. However, in order to suggest rTMS as an effective treatment
for L-dopa induced dyskinesias in Parkinsons disease patients, long-

Movement Disorders, Vol. 22, Suppl. 16, 2007

term observations and further investigations with a larger patient population are essential.
357
Sleep and periodic leg movements in schizophrenic patients with
neuroleptic-induced parkinsonism
T.C. Wetter, S. Fulda (Munich, Germany)
Objective: To explore the hypothesis that neuroleptic-induced parkinsonism (NIP) is associated with the occurence of periodic leg
movements during sleep (PLMS).
Background: The incidence of PLMS has been shown to be markedly lower in middle-aged schizophrenic patients than it would be
expected in this age group. This nding may be linked to high dopaminergic levels in subcortical areas that have been found in this
disorder. Neuroleptic medication decreases dopaminergic activity and
in the present study we predicted that patients with NIP will be more
likely to exhibit PLMS.
Methods: We compared sleep and PLMS in two groups of schizophrenic patients that were comparable in terms of pharmacological
treatment (duration and dosing of monotherapy with haloperidol) but
differed in the development of extrapyramidal symptoms.
Results: Twenty-two consecutive patients with the diagnosis of
schizophrenia (paranoid type, DSM-IV: 295.30) and a monotherapy
with haloperidol (6-10 mg/day) participated in the study. During treatment with haloperidol 12 patients developed a NIP, while the other 10
patients did not experience any extrapyramidal-motor side effects. NIP
patients had a signicantly shorter sleep duration, a higher number of
nocturnal awakenings and an increased percentage of wake compared
with the symptom free patients. NIP patients also exhibited a signicant
higher number of PLMS per hour (PLMS-index). Nine of 12 (75%)
NIP patients had a PLMS-index 5 and 7 (58%) also had a PLMSarousal-index 5. In the ten control subjects 3 had a PLMS-index
5 and only one a PLMS-arousal-index 5.
Conclusions: Lowering of dopamine levels by neuroleptic medications is associated with the emergence of periodic leg movements in
schizophrenic patients.
358
Movement disorder caused by injections of manganese containing
compounds
I. Khatiashvili, K. Akhvlediani, M. Megrelishvili, M. Janelidze,
N. Lobjanidze (Tbilisi, Georgia)
Objective: To investigate the clinical course of Movement Disorder
caused by manganese-containing compounds injection.
Background: The condition referred as manganese-induced Parkinsonism is being seen among the people whose job is related to regular
inhalation of manganese-containing dust. The similar syndrome with some
peculiarities can be seen among the injecting drug users (IDU) after
parenteral usage of hand-made mixture (HMM) containing KMnO4.
Methods: Ten patients - IDUs were investigated. All of them were men,
(age range 21-45 years old) who have been regularly injecting HMM with
KMnO4. The duration of HMM using period varied from 2 months to 1
year. The patients were evaluated clinically using UPDRS, Tinnetti scale,
Scale for Ocular Motor Disorders in Parkinsonism. They underwent also
neuropsychological testing. They have been seen by neurologist on rst
visit, after 3 months and after 1 year of the treatment. Brain MRI was
performed in 7 cases. ENMG- in 5 cases. In all cases blood ceruloplasmine
was normal and there were no Kaizer-Flasher rings. Five patients received
chelation therapy with CaNaEDTA (tetacin-calcium), 9 of them were
treated with amantadine, levodopa and SSRIs.
Results: In all 10 cases prominent symptoms were shufing gait with
start and turn hesitation with frequent falls, hypophonia, dysarthria. In
all cases depression along with involuntary laughing were present. Two
patients have ENMG conrmed brillations. Signs of anterior horn
injury were prominent in one case (this case was a diagnostically
thoughtful: in contrast to the rest of the patients the main feature after
1 year was amyotrophic lateral sclerosis syndrome). In 6 of the 7 MRI
images there were T1 signal hyperintensity in basal ganglia bilaterally.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

In one case (with prominent ALS syndrome) the MRI revealed pathological signal from pyramidal tract. Only in one case positive effect of
chelation therapy with CaNaEDTA was seen. Amantadine and levodopa were ineffective. After 1 year 4 patients have a new symptom
ambulatory spasm of the foot.
Conclusions: The intravenous injections of manganese containing
mixtures cause syndrome with prominent gait and postural disorders
with typical MRI. Despite possible normalization of MRI, symptoms
may persist or even get worse.

S111

359
Deep brain stimulation for tardive dyskinesia and akathisia
C. Kenney, R.L. Barbano, J.K. Shefeld, J. Jankovic (Houston,
Texas, USA)
Objective: To describe a patient treated with GPi deep brain stimulation (DBS) for severe tardive dyskinesia (TD) and akathisia (TA).
Background: TD and TA are hyperkinetic movement disorders causally related to dopamine receptor blocking drug (DRBD) exposure.
Besides tetrabenazine, treatment options are limited and there are no
trials of surgical interventions.
Methods: A 61-year-old right-handed retired female teacher presented for evaluation of restlessness and involuntary movements. During her teens, she displayed excessive eye blinking and other subtle
motor tics, but no phonic tics. These movements remitted spontaneously, but recurred in her late twenties with head/arm/leg jerking and
bouncing of her body. Simultaneously, she developed obsessive
compulsive behaviors (OCB) such as touching walls before leaving a
room. She was treated with haloperidol and other DRBDs over 4 years.
The aforementioned symptoms improved and she required no pharmacologic intervention for 20 years. At age 54, without any clear precipitating event, she noted gradual onset of an intense feeling of inner
restlessness debilitating enough to force her into an early retirement.
She also became aware of involuntary movements in the face and upper
body. She was diagnosed with tardive akathisia/dyskinesia and tried
over 100 medications, including tetrabenazine, DRBDs, beta-blockers,
benzodiazepines, antidepressants, anticholinergics, anticonvulsants,
dopaminergics, opiates, botulinum toxin, and more, without lasting
benet. On examination, she displayed oro-facial lingual stereotypy,
cranial cervical dystonia, and continuous pacing about the room associated with a feeling of restlessness and an urge to move.
Results: After obtaining informed consent, the patient underwent
bilateral GPi-DBS without any adverse events. We captured the patients degree of psychologic and physical impairment using several
scales (Table 1). After 6-month follow-up, involuntary movements
decreased 55%; akathisia decreased by 21% according to the Barnes
Akathisia Scale and 57% using the Prince Henry Hospital Akathisia
Rating Scale. Anxiety decreased by 35% on the Hamilton Anxiety
Rating Scale while depressive and OCB scores improved minimally.
Conclusions: Although a single case, this rst report suggests that
GPi DBS may be a safe and effective treatment of disabling akathisia;
TD also improved.

Clinical Endpoints

Abnormal Involuntary Movement Scale

(AIMS)
Prince Henry Hospital Akathisia Rating Scale
Barnes Akathisia Scale
Beck Depression Index (BDI)
Hamilton Anxiety Rating Scale (HAM-A)
Yale-Brown Obsessive Compulsive Scale
(Y-BOCS)

Baseline/Preoperation

3-month
Follow-up

6-month
Follow-up

38
30
14
15
26

23
22
13
6
8

17
13
11
12
17

25

20

24

360
Acute dystonia induced by adding midodrine to Perphenazine
A. Castrioto, L. Pierguidi, N. Tambasco, A. Rossi, P. Calabresi
(Perugia, Italy)

FIG. 1 (358).

Objective: We report a case of acute dystonia induced by adding

Midodrine to Perphenazine.
Background: Acute dystonic reaction is a well-known side effect of
neuroleptic drugs.
Methods: A 53 years old woman, treated for ve years with antipsychotic perphenazine 4mg/ daily, presented an acute cranio-cervical
dystonia, three days after midodrine introduction, because of orthostathic hypotension. Midodrine was immediately stopped. Laboratory
analysis, ECG and brain CT scan were normal.
Results: The dystonic movements spontaneously disappeared after
24 hours of Midodrine withdrawal.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S112 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Conclusions: Midodrine, a vasopressor commonly used for the management of orthostathic hypotension, doesnt cross the blood brain
barrier therefore it should be lacking in central nervous system adverse
effects. However, both Perphenazine and Midodrine are metabolized
by the cytochrome P450 isozyme CYP2D6 and then Midodrine, acting
as a CYP2D6-inhibitor, could increase Perphenazine bioavailability
and its central side effects. In conclusion, although Midodrine is a safe
drug, one should be careful of adding it to antipsychotic because of risk
of extrapyramidal side effects.
361
Capecitabine-induced oromandibular dystonia
P.K. Manharlal, C.S. Pin, L.Y. Long, T.Y. Albert, S.S. Ju,
P. Ratnagopal (Singapore, Singapore)
Objective: To report a case of capecitabine-induced oromandibular
dystonia.
Background: Capecitabine, an oral chemotherapy converted to 5-uorouracil, is used in advanced breast and colorectal malignancies.
Several case reports of capecitabine-induced encephalopathy has recently been reported. We report the rst case of capecitabine-induced
oromandibular dystonia.
Methods: A 54-year-old man diagnosed with carcinoma of rectum with
liver metastasis had anterior resection of the rectum. Subsequently, he was
started on adjuvant chemotherapy with oral capecitabine. Almost 1 week
after consuming capecitabine he developed sudden onset of disability to
talk and swallow. Clinical examination revealed he had dystonia of tongue
and pharyngeal muscles as well as jaw clenching. Blood laboratory results
were normal. MRI brain showed non-enhancing abnormalities with restricted diffusion involving bilateral corona radiata, centrum semiovale and
splenium of corpus callosum. The MRI ndings were similar to those
reported by Videnovic et al and classical for capecitabine-induced multifocal leukoencephalopathy. Capecitabine was stopped and the patient
improved completely within 2 days. A repeat MRI was done 3 weeks after
discontinuation of capecitabine and showed resolution of the earlier
changes.
Conclusions: To our knowledge, this is the rst case report of
capecitabine-induced oromandibular dystonia and had complete clinical and MRI resolution after discontinuation of the drug. Capecitabine
therapy has been used in many patients and will continue to be used.
Awareness among clinicians about this reversible chemotherapy adverse effect will avoid delay in diagnosis and management.

DYSTONIA
362
Extreme task specicity in writers cramps
E.A. Shamim, J.M. Savitt, H.A. Jinnah, M. Hallett (Bethesda,
Maryland, USA)
Objective: To present 2 cases of extremely specic task-related
dystonia in patients with writers cramp.
Background: The task specicity of focal hand dystonia is a common
aspect, but not much is understood. In general, it does seem to reect
repetitive movements done for prolonged periods of time. Writers
cramp is a common form of focal hand dystonia and usually affects an
individuals ability to write anything. It appears that, in unusual cases,
the task specicity maybe so specic to affect only a particular letter or
part of a letter or number.
Methods: Two patients were evaluated for writers cramp with
history and physical examination. Parts of the patients examinations
were video taped.
Results: Patient 1: This is a 55 year old right-handed white male
who had been healthy until 1 year ago, when he developed difculty
with signing his name. As a part of his job, he signs his name over
1000 times per day. He has no other complaints with his handwriting or other aspects of hand use. He initially had difculty with
writing the rst letter of his rst name which starts with the letter
J. When he writes a capital J, he is unable to make the initial top
half of the J, written in script. He has no difculty with printing

Movement Disorders, Vol. 22, Suppl. 16, 2007

the letter J.. He feels that he is trying to move against a wall while
his upper extremity tightens up. Mirroring movements are present in
his right hand while writing with the left hand. He uses a geste
antagoniste with the left hand to help him write. He now uses a
stamp to sign his name. Rest has been of some benet to him and he
now uses a stamp to sign his name. Patient 2: This is a 49 year old
left-handed white male who has enjoyed good health until 2003
when he developed progressive difculty with writing the number 7.
After making the horizontal part of the 7, he is unable to continue
with the vertical line. He has similar difculty with number 9.
Writing the number 1 is not a problem. The patient now has
difculty with writing and posturing during the activity. He also
complains of a cramping sensation affecting mainly the forearm. He
has mirroring movements in the left hand while writing with the
right hand, and sensory tricks have not beneted him.
Conclusions: Sometimes task specicity in writers cramp can be
extremely specic especially early in the disease.
363
Chronic low back pain related to idiopathic extensor truncal dystonia
G. Sau, V. Agnetti, E. Coco, B. Nieddu, I. Magnano, I. Aiello
(Sassari, SS, Italy)
Objective: To describe a very rare case of idiopathic extensor truncal
dystonia in a patient suffering from chronic low back pain.
Background: Among the primary dystonias, axial dystonia has a very
low frequency accounting for 4 to 6% of the patients with segmental
idiopathic dystonias (Duffey P et al. Adv Neurol 1997). Focal axial
dystonia is even less common and it is more frequently characterized
by a forward posture with exion spasms (Kailash P et al. JNNP1997)
so that extensor truncal dystonia is a very rare clinical entity.
Methods: We report a case of a 56 years old man affected by severe
low back pain for three years misdiagnosed as lumbar disc prolapse
disease. At neurological examination the patient presented with a
marked back muscular contracture in dorsal and lumbar segments with
a truncal hyperextension posture. The patient was markedly disabled by
continuous low back pain that was not responsive to pharmacologic
therapy and presented a severe motor disability during walking and
truncal ection attempts. Brain and spinal cord MRI, conduction velocity studies and multimodality evoked potentials were normal. Needle EMG analysis revealed the dystonic nature of the disturbance, so
the diagnosis of adult idiopathic truncal extension dystonia was made.
Results: Using electromyographic guidance, we injected botulinum
toxin A (Dysport) into the paravertebral muscles of the dorsal and
lumbar regions, in six sites, using 100 U per site. Clinical and EMG
evaluation were performed 20 days after the botulinum injections
showing an objective improvement in posture and movement range,
with a marked reduction in the spontaneous EMG activity of spinal
extensor muscles. Moreover spontaneous back pain disappeared and no
adverse effects occurred.
Conclusions: This case report revealed that chronic unresponsive
low back pain may be related to idiopathic truncal dystonia that may be
treated successfully with botulinum toxin injections.
364
Mental rotation of body parts in DYT1 carriers
M. Fiorio, M. Gambarin, C. Stanzani, E.M. Valente, G. Defazio,
G. Moretto, M. Loi, P. Soliveri, N. Nardocci, A. Albanese,
A. Fiaschi, M. Tinazzi (Verona, Italy)
Objective: To investigate whether the presence of the DYT1 gene
mutation impairs the inner simulation of actual movements, which is
a fundamental function for accurate motor planning and execution
and which rely upon cortical and subcortical systems, dysfunctional
in dystonia (e.g. motor and premotor areas and basal ganglia).
Background: Subclinical functional and structural consequences of
the DYT1 gene mutation, leading to the onset of primary torsion
dystonia in 30%-40% of cases, have been recently revealed in manifesting and non-manifesting mutation carriers. Namely, metabolic,

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

sensory and motor abnormalities exist at a subclinical level in DYT1
carriers. However, it is still unknown whether subclinical abnormalities
also exist prior to movement execution, that is in movement planning.
Methods: We applied a mental rotation task to DYT1 manifesting
patients (n 12), non-manifesting mutation carriers (n 12) and
healthy control subjects (n 12). The experimental stimuli consisted
of realistic photos of left or right hands or feet and the head of a young
men with a black patch on the left or the right eye. Stimuli were
presented at six different degrees of orientations. Subjects were asked
to verbally report whether the hands or feet were left or right, or
whether the patch was on the left or the right eye. Reaction times and
accuracy in performing the laterality tasks on the three stimuli were
collected.
Results: DYT1 carriers, both manifesting and non-manifesting, were
slower in mental rotation of body parts than control subjects.
Conclusions: This nding suggests that the cognitive representation
of body movements may be altered subclinically in dystonia and may
represent another endophenotypic trait of disease. Whether this alteration is due to impaired sensory processing, to defective motor circuit
or to the abnormal integration of both systems is still unknown and a
matter of future investigation.
365
A slow ow arterio-venous malformation as a cause of a neuropsychiatric syndrome comprising hemi-dystonia and behavioral
changes
M.A. Sierra-Beltran, U. Rodr`guez-Ortiz, M.S. Rodr`guez (Mexico
City, DF, Mexico)
Objective: To present a very rare case of dystonia and behavioral
changes due to a putaminal slow ow arterious venous malformation.
Background: A 16 year old male patient suffering from a neuropsychiatric syndrome, comprising hemi-dystonia and behavioral
changes case is presented. He carries a familial history of Parkinsons disease. His current illness began in 1997 (at age 7) with right
arm movement limitation. By year 2000, right forearm and hand
involuntary movements developed. As a matter of fact, he was right
handed; however, after 1997 he gradually became left handed. A
pediatric neurologist examinated him in 2001. Who then required
image studies (CT cranial scan) that showed a hyperdense image at
the left putamen, interpreted as a calcication. He was then medicated with Haloperidole, later shifted to Propranole. This later
yield a better clinical response, unfortunately without a signicant
functional improvement. Since 2004, stereotyped right upper limb
involuntary movements (coreo-athetosis) and intentional tremor,
appeared; these sort of movements were also evident on the right
lower limb, but spontaneously remitted. His family noticed an
attitude of social interaction avoidance. He was admitted to the
Neurology and Neurosurgery National Insitute (Mexico City) in
May 2006.
Methods: Another series of images studies were requested, which
included at rst and MRI cranial scan. Which revealed a left putaminal
vascular maze and a dilated Rosenthal vein. Thus, an angioprhapic
cranial vessels study was requested. A Neuro-psychiatric evaluation
found a verbal memory, as well as attention impairment with a marked
social isolation tendency.
Results: The angiographic ndings showed an slow ow left putaminal arterious- venous malfromation (AVM).
Conclusions: Because of the nding of a hyperdense putaminal
lesion, resembling a calcied lesion, it was considered that the case
may have corresponded to an infrequent presentation of Fahr Syndrome. His current condition was misdiagnosed. However, a detailed
analysis of the MRI scan images lead to the nding of a basal ganglia
AVM partially occluded, after a prior hematoma (later calcied) associated to a left thalamic angioma. Putamen is an AVM rare location.
Parkinsonian syndromes.
Gilman S - Clin Geriatr Med 01 NOV 2006; 22(4): 827-42.

S113

366
Improvement of treatment effect with a higher dilution of botulinum toxin type A: Results of a controlled blepharospasm
study
S. Grafe, G. Comes, P. Roggenkaemper (Frankfurt, Germany)
Objective: The data of a randomized double-blind controlled clinical
trial with NT 201 (trade name Xeomin), a neurotoxin free of complexing proteins vs. a botulinum toxin complex (trade name Botox) in
the treatment of blepharospasm, were used to investigate the impact of
different dilutions on the treatment effect.
Background: A total of 300 patients with a conrmed clinical diagnosis of blepharospasm were enrolled in this study. Patients received an
injection according to the previous two injection sessions with Botox.
The investigators selected the injection points, units (up to 50 units per
eye), and the dilution of the botulinum toxin type A preparations. A
control visit took place at Week 3 after injection and patients were
followed for up to 16 weeks (nal visit).
Methods: In this study, the Jankovic Rating Scale (JRS), assessed by
the investigators and the Blepharospasm Disability Index (BSDI) rated
by the patients were used to determine the treatment effect. We reanalyzed the data of this study whether various dilutions revealed a
different response to Xeomin or Botox.
Results: The mean total doses of study medication injected were
similar in the two groups (Xeomin: 40.7 units, SD: 14.6 units);
Botox: 41.7 units, SD: 15.9 units). Xeomin and Botox were diluted
on average in 3.0 ml 0.9% sodium chloride solution (SD1.2 ml,
range: 1.0 to 5.0 ml) per vial. The treatment effect increases in each
treatment group by using a high dilution (p0.01 for the change in the
JRS score three weeks after injections, p0.05 for the change in the
BSDI), see Table below: This dilution effect can further be improved
when a low dose of Xeomin or Botox is administered. Using a
dilution of more than 2.5 mL and a dose less than 20 units per eye, the
change in the JRS at control visit was 3.5 for Xeomin and -3.9 for
Botox. No differences were seen between the Xeomin and Botox
group.
Conclusions: The optimal treatment effect of botulinum toxin Type
A can be achieved administering a low dose (20 Units per eyes) and
a high dilution ( 2.5 mL). A dose reduction may be possible by
administration of a higher diluted botulinum toxin type A.
Change in the JRS 3
weeks after injection
Treatment/Dilution
Xeomin
2.5 mL
2.5 mL
Botox
2.5 mL
2.5 mL

Change in the BSDI 3

weeks after injection

Mean (SD)

Mean (SD)

71
73

-2.6 (1.7)
-3.1 (2.2)

71
73

-0.8 (0.7)
-1.0 (0.8)

80
69

-2.3 (1.5)
-3.1 (2.4)

79
68

-0.8 (0.6)
-0.9 (1.0)

367
Tonic versus phasic cervical dystonia: Persistence and inuence of
botulinum toxin treatment on dystonic type
D.D. Duane, K.B. Zebatto, J.M. Johnson, R.L. Owen, J.H. Flutie,
K.A. Shunk (Scottsdale, Arizona, USA)
Objective: To retrospectively analyze the character of cervical dystonia (CD) type of contracture, i.e., tonic versus phasic through serial
evaluations over time and the effects on CD type 3-6 months post
botulinum toxin (bot tox) if applied.
Background: Whereas direction of head or neck posture is often
referred to in the literature re: CD (lateral, rotational, retro, anterocollis)
only occasionally is the character of the motion, that is xed (tonic)
versus mobile (phasic) distinguished. This characteristic, among others,
has at the Arizona Dystonia Institute consistently been recorded on
sequential examinations, by the same examiner, often additionally
preserved on video. Since a variety of CNS sites may produce dystonic
symptoms (neck and otherwise), topology of CD may differentiate
whether a solitary or more than one site affects this characteristic of CD

Movement Disorders, Vol. 22, Suppl. 16, 2007

S114 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

(D. D. Duane in G. Bouvier F. de Soultrait and P. Molina-Negro, Eds.
Spasmodic Torticollis: Clinical Aspects and Treatment, 2006, Paris:
Expressions Sante, 39-46).
Methods: 294 CD patients (213 female, 81 male), mean age 55 years,
(range 28-72 years), were evaluated over the period from 1988 through
2006. 223 of these have been evaluated on two or more occasions at
least one year apart (mean 6 years, range 1-19 years). Of these 223
subjects, 179 have been treated at least one time with bot tox (type A
in 170, type B in 23, 14 with both). The number of toxin exposures per
patient averaged 10. Records and videos were reviewed to record which
of the two types occurred, phasic versus tonic.
Results: Of the 223 subjects meeting the above criteria, 92 were
classied as tonic, 131 as phasic. Over several sequential examinations
(mean 8, range 2-24), 18 converted to the opposite characteristic, 13
from tonic to phasic. In 16 of the 18, bot tox therapy was used but in
only 3 was treatment with toxin associated with change in CD type.
Conclusions: Tonic versus phasic CD characteristic appears to be a
relatively stable feature, whether untreated, treated with oral medication or with bot tox. This nding suggests that this characteristic may
reect distinguishing underlying pathophysiologic sites or mechanisms
between tonic and phasic CD.
368
Changes of perfusion pattern using ECD-SPECT in patients with
primary focal or generalized dystonia
N. Kawashima, E. Horiuchi, K. Hasegawa, Y. Ujihara, Y. Hasegawa
(Fujisawa, Japan)
Objective: To clarify the regional cerebral blood ow (rCBF)
changes at rest in patients with primary dystonia.
Background: In previous neuroimaging studies about primary dystonia, different tasks over patients and the restriction of concerned brain
areas might lead various results.
Methods: Single photon emission computed tomography studies
using 99mTc-ethyl cisteinate dimer (ECD) were performed in 10
patients with focal dystonia (3 men; mean age 60.9 years) and in 3 with
generalized dystonia (2 men; age 34.7). The former were divided into
4 groups; 4 with blepharospasm (BS), 3 with cervical dystonia (CD), 2
with writers cramp (WC) and the other. All patients were right-handed
with normal conventional brain MRI studies. Differences in rCBF
across groups were assessed by two-sample t-test on a voxel-based
basis using statistical parametric mapping, with statistical thresholds
set at p0.001.
Results: In focal dystonia, rCBF increased in the left (lt) substantia
nigra (SN); the right (rt) amygdala (Am); bilateral (bil) temporal lobes
(TLs), prefrontal areas (PFs) and cerebellum and decreased in the lt
caudate head (CH) and cuneus (Cu); bil precuneus (PCu). The BS
group revealed increased rCBF in the lt SN, thalamus (VPL); bil Am,
TLs, cingulate gyri and cerebellum, decreased in the lt CH; bil Cu and
PCu. In CD, rCBF increased in the lt SN, claustrum and hippocampus;
bil cerebellum and decreased in the lt Cu; bil PCu. In WC, rCBF
increased in bil TLs, and decreased in the rt postcentral gyrus. In
generalized dystonia, rCBF increased in the lt SN; the rt thalamus; bil
TLs and cerebellum, and decreased in the lt CH and sensorimotor
cortex (SMC); bil PFs.
Conclusions: This study showed the increase of rCBF in the lt SN,
bil TLs and cerebellum, and the decrease in the left CH in the patients
with primary dystonia. The rCBF decreased in bil occipital lobes of
focal dystonia and in the lt SMC and bil PFs of generalized dystonia.
The discrepancy suggests the pathophysiological difference between
two groups. As our study size is small, the further investigation of the
large sample size is warranted.
Acknowledgements: The authors wish to thank Fumio Yamashita
MMS (Medical Sciences for Control of Phathological Processes, Department of Clinical Neuroscience, University of Tsukuba, Japan) for
the analysis of rCBF.

Movement Disorders, Vol. 22, Suppl. 16, 2007

369
Neuropathology in idiopathic cervical dystonia
M.C. Zerrate, C.A. Pardo, H.A. Jinnah (Baltimore, Maryland, USA)
Objective: To re-evaluate the possibility of subtle microstructural
neuropathological defects in idiopathic cervical dystonia.
Background: The most common form of primary dystonia in adults
is cervical dystonia. Based on a handful of published post-morten
neuropathological studies and the lack of abnormalities on structural
imaging studies, it is generally believed that the brain shows no overt
pathology. However, multiple recent studies suggest the possibility of
subtle microstructural defects that would not be evident in traditional
pathological or imaging studies.
Methods: Post-mortem brain specimens from four patients with
idiopathic cervical dystonia were examined. Regions sampled included motor and somatosensory cortex, caudate, putamen, globus
pallidus, midbrain, cerebellar cortex, and deep cerebellar nuclei. In
addition to routine Nissl stains, immunostains for reactive astrocytosis and immune activation were conducted in view of studies
suggesting a link between cervical dystonia and autoimmune or
inammatory processes. Stains for ubiquitinated inclusion bodies
were conducted in light of other studies showing such microstructural defects in other dystonias.
Results: Subtle pathological changes were found in all four brains.
The majority occured in midbrain and cerebellum. In the midbrain, all
brains exhibited blood vessels with patchy areas of satellitosis, and two
exhibited astrogliosis with increased microglia. Ubiquitin immunostains in the midbrain revealed intranuclear inclusions resembling Marinesco bodies in melanized neurons for three brains. In the cerebellum,
varying abnormalities were seen in all brains. Two had proliferation of
Bergman glia, two showed patchy loss of Purkinje cells, two showed
torpedo bers, and one showed extracellular ubiquitinated inclusions
between the Purkinje and molecular layers. Pathological abnormalities
were rare or absent in the cerebral cortex, caudate, putamen, or globus
pallidus.
Conclusions: Subtle microstructural defects can be detected in the
brains of idiopathic cervical dystonia patients with special staining
methods. These studies call into question current belief that the brain is
structurally normal. Further studies of additional dystonia brains and
control specimens are needed to determine which abnormalities are
most consistent.
370
Neuropathology of primary dystonia unrelated to DYT1 mutations
J.L. Holton, S.A. Schneider, S. Gandhi, T. Ganesharajah, C. Strand,
P. Shashidharan, J. Barreto, N.W. Wood, A.J. Lees, K.P. Bhatia,
T. Revesz (London, United Kingdom)
Objective: To establish whether pathological changes recently reported for genetic dystonia (DYT1 and DYT3) are present in primary
adult-onset sporadic dystonia
Background: Idiopathic adult-onset primary dystonia usually affects the upper body and remains focal. The underlying mechanisms
are not fully understood. Neuropathological studies are limited in
the literature. Recently, ubiquitinated perinuclear inclusion bodies
in the brainstem of patients with DYT1-related dystonia have been
described. [1] In DYT3, causing X-linked recessive dystonia-parkinsonism, neuronal loss in the striosome compartment of the striatum has been reported.[2] However, it was unclear whether these
changes are characteristic of these particular disorders or an epiphenomenon of dystonic conditions in general.
Methods: We present pathological data of six cases with primary
adult-onset dystonia and four controls. We used immunohistochemistry
to determine the presence of inclusion bodies immunoreactive for
torsinA, ubiquitin and laminA/C in the brainstem. The degree of
neuronal loss was determined by the calcineurin expressing neurons in
the striosome compartment of the striatum.
Results: In contrast to early-onset DYT1 dystonia, neuronal inclusions immunoreactive for torsinA, ubiquitin and laminA/C were not

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

present in the brainstem nuclei. There was no apparent loss of the
striatal striosome compartment.
Conclusions: Our ndings suggest that the underlying mechanism in
the adult-onset primary torsion dystonia is different from that of earlyonset DYT1-related dystonia and also DYT3 X-linked recessive dystonia-parkinsonism. Alternative mechanisms may underlie these two
conditions.
References:
[1] McNaught KS, Kapustin A, Jackson T, Jengelley TA, Jnobaptiste R, Shashidharan P et al. Brainstem pathology in
DYT1 primary torsion dystonia. Ann Neurol 2004; 56(4):540547.
[2] Kaji R, Goto S, Tamiya G, Ando S, Makino S, Lee LV. Molecular dissection and anatomical basis of dystonia: X-linked recessive dystonia-parkinsonism (DYT3). J Med Invest 2005; 52
Suppl:280-283.
371
Repetitive TMS of the somatosensory cortex improves writers
cramp
R. Jech, P. Havrankova, N.D. Walker, J. Vymazal, E. Ruzicka
(Prague, Czech Republic)
Objective: Our study focused on the functional modulation of the
primary somatosensory cortex (SI) induced by repetitive transcranial
magnetic stimulation (rTMS) performed to improve symptoms of writers cramp.
Background: It has been suggested that the somatosensory system is affected in focal dystonia, alongside the involvement of the motor circuits.
Methods: Nine patients with writers cramp of the right hand were
treated with two ve-day blocks of 30-minute low-frequency (1-Hz)
rTMS, one using stereotactically guided real-rTMS to the contralateral
SI cortex, the other using sham-rTMS both employed in random
order. Prior to the start and after the end of real-rTMS, 1.5 T functional
magnetic resonance imaging was performed with the execution of a
simple active and passive movement of the ngers of the right hand.
Results: Soon after the block of real-rTMS, four patients (responders) experienced subjective and partially objective improvement in
writing, while in another patient, improvement was delayed until one
week after the end of rTMS. No improvement was noted in response to
sham-rTMS. Responders exhibited rTMS-induced increase of activation in the left SI cortex and right cerebellum. The SI activity increase
was observed during active (P10-6) as well as in passive movements
(P0.001). No similar changes were observed in non-responders.
Conclusions: As shown by our results, low-frequency rTMS of the SI
cortex can improve clinical manifestations of writers cramp. Handwriting improved in most responders and, in a similar way to the
subjective benet, lasted for two to three weeks. The failure of therapy
in non-responders was probably due to the different positioning of the
stimulating coil as the specic area of the postcentral sulcus was only
stimulated in responders. The benecial effects of rTMS also paralleled
the functional reorganization of the primary somatosensory cortex,
reecting the impact of this therapeutic approach on motion control and
supporting several previous ndings suggesting the involvement of the
sensory system in the pathophysiology of writers cramp.
Study support: IGA MZ CR 1A/8629-5, NR8937-4 and Research
Program MSM0021620849.
372
Longitudinal effects of pallidal stimulation on motor cortex function in dystonia
S. Tisch, D. Ruge, P. Limousin, M. Hariz, K.P. Bhatia, N.P. Quinn,
L. Zrinzo, M. Jahanshahi, J.C. Rothwell (London, United Kingdom)
Objective: To study the effects of globus pallidus internus (GPi)
deep brain stimulation (DBS) on motor cortex excitability and LTP-like
plasticity using transcranial magnetic stimulation (TMS) in dystonia.
Background: GPi DBS is effective treatment for primary generalised
dystonia. There is clinical and physiological evidence that neural reorgani-

S115

sation (plasticity) may play a mechanistic role, although the longitudinal

effects of continuous GPi DBS on motor cortex function are unknown.
Methods: We have so far studied 5 dystonia patients (3 primary generalised, 1 myoclonic dystonia, 1 secondary generalised) before, 1 and 3
months after bilateral GPi DBS. TMS measures included resting and active
motor thresholds, 1 mV resting motor evoked potential (MEP), inputoutput (IO) curves, intracortical inhibition/facilitation, contralateral premotor-motor cortex inhibition, and paired associative stimulation (PAS).
Results: In the three patients with primary generalised dystonia, the
resting and active motor thresholds decreased, the 1mV MEP amplitude
increased by 200% at 1 month and 143% 24 at 3 months, similarly
the slope of the IO curve increased by 202% 78 and 117% 39.
Intracortical inhibition, as a percentage of the unconditioned MEP size,
for ISI2 ms, increased from 115% 33 to 76% 5 at 1 month and
57% 30 at 3 months. Intracortical facilitation for ISI8-15 ms,
decreased from 164% 5 at baseline to 103% 2 at 3 months.
Contralateral premotor-motor cortex inhibition for ISI10 ms, pre-op
was 97% 6, at 1 month 100% 3 and 77% 5 at 3 months. Before
surgery PAS resulted in a 39% 30 increase in MEP amplitude and by
3 months 6% 9. Similarly before surgery PAS resulted in a 40%
increase in slope of the IO curve and by 3 months a 9% decrease.
Conclusions: GPi DBS increases motor cortex excitability, however
it also appears to restore intracortical and premotor interhemispheric
inhibition and reduce LTP-like plasticity. Since reduced inhibition and
excessive plasticity are important components of dystonia pathophysiology, our preliminary ndings suggest GPi DBS may favorably
modify these abnormalities in a manner consistent with neural reorganisation. Morever our results provide novel insights into the mechanisms of action of GPi DBS for dystonia.
373
Clinical characteristics of dystonia in a Movement Disorder Centre
in Venezuela
M. Gallardo, A. Soto, G. Orozco, M. Camacaro, G. Ramirez,
R. Weiser, L. Vink (Caracas, Miranda, Venezuela)
Objective: To report the clinical features of a group of patients with
dystonia in Venezuela.
Background: Dystonia is a disorder of involuntary sustained muscle
contractions classied by age of onset, distribution of involved body
regions, and aetiology.
Methods: Ninety-three (93) patients were evaluated by a qualied
neurologist using the diagnosis criteria proposed by Fahn et al.
Results: Ninety-three (93) patients were evaluated by a qualied
neurologist using the diagnosis criteria proposed by Fahn et al. Sixtytwo (66%) females, (mean age 59), and 31 (34%) males (mean age 48).
Mean onset of disease was 35 years in men and 50 years in women.
Focal Dystonia was found in 64 patients (69%), including 35 patients
with cervical dystonia (55%), 24 blepharospasm, four braquial dystonia
and one oromandibular dystonia. The most common cervical dystonia
observed was Torticollis in 56% of cases, retrocolis in 11 %, laterocolis
in 4% and a combination of these in 29%. Blepharospasm was 88% in
women and 2% in men. Segmental dystonia was present in 26 patients
(28%) including 13 segmental-craneocervical dystonia, seven segmental cranial dystonia, one segmental-cervicobraquial and 1 segmentalaxial. Generalized dystonia was observed in three cases (3%).
Hemidystonia and Multifocal Dystonia were not found. Primary dystonia was found in 76 patients (82%) and secondary dystonia in 18%.
Use of neuroleptic drugs and perinatal injury was the main cause of
secondary dystonia with the same percentage in each group.
Conclusions: Cervical Dystonia was the most common form of
dystonia observed in this study. We found an earlier onset of Dystonia
in men than women. We detected a difference in sex presentation in
subtypes of focal dystonia with women having a higher percentage of
blepharospasm. Primary dystonia was the most frequent cause observed. Perinatal injury and use of neuroleptic drugs was the main
cause of secondary dystonia.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S116 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

374

376

Homocystinuria and dystonia case presentation

O. Akan, S. Ozbakir, S. Ozturk, S. Ozturk, A. Findik (Ankara,
Turkey)

Quality of life in patients with different types of focal dystonias in

Serbia
T.D. Pekmezovic, M. Svetel, N. Ivanovic, N. Dragasevic, I. Petrovic,
V.S. Kostic (Belgrade, Serbia)

Objective: We aim to present a case with homocysteinuria with

presenting symptom dystonia.
Background: Homocysteine (Hcy) is a sulfhydryl amino acid derived
from the metabolic conversion of methionine, which is dependent on
vitamins (folic acid, B12 and B6) as cofactors or cosubstrates. Severe
hyperhomocysteinemia (homocystinuria), due to inherited metabolic
defects of Hcy metabolism, may be associated with movement disorders.
Methods: 15 year old girl was admitted to our clinic with dystonia.
She had cataract operation history. In neurological examination generalized dystonia was found. Rutin laboratory tests and serum copper,
urine copper and serum ceruloplasmin levels were normal. Serum
lactate, methionine levels were signicantly increased. Homocystinuria
was reported with cystine chromatography. Her cranial MRI was normal. Other potential causes of dystonia were excluded with clinical and
laboratory investments.
Results: With Betaine, pyridoxine and folic asid and L-Dopa therapy, her symptoms improved signicantly.
Conclusions: Homocystinuria should be remembered as a rare and
treatable cause in the patients with secondary dystonia.
375
A novel mutation (64-65DelGGInsAACC(G21fsX66)) in the GTP
cyclohydrolase 1 gene causing Segawas disease (DYT5 dystonia)
M. von Mering, H. Gabriel, G.F. Hoffmann, A. Storch (Dresden,
Germany)
Objective: DYT5 dystonia (Segawass disease) is an autosomal
dominant inherited progressive dystonia evoked by various known
mutations of the GTP cyclohydrolase 1 (GCH1) gene, which codes for
the enzyme catalyzing biopterin synthesis. Segawas disease is a rare
disorder with an estimated prevalence of 0.5 per million.
Background: We here report a novel mutation of the GCH1 gene in
a 25 year old caucasian female presenting in our outpatient clinics. She
described the development of a gait disturbance beginning at the age of
5 years. She was increasingly unable to walk on her soles, but was only
walking on the outer edges of her feet (pedes equinovarus). Several
stays at hospitals did not reveal the diagnosis, so that at last the gait
disturbance was classied as mentally induced. The patient was introduced to our movement disorder outpatient clinic just before an operation of the feet abnormalities. On examination the were severely
adducted and suppinated. A treatment with a low dose of levodopa was
capable of resolving the symptoms completely.
Results: Sequencing of exons 1 to 6 of the GCH1 gene revealed a
heterozygous deletion of two guanines at positions 64 and 65 and an
insertion of 4 bases (AACC) leading to a frameshift from amino acid 21
and subsequent termination of the protein after amino acid 66 within
exon 1 (64-65DelGGInsAACC(G21fsX66)). Multiplex ligation dependent probe amplication (MRC, Amsterdam, The Netherlands) of the
whole GCH1 did not detect any further deletions. This novel combined
deletion-insertion mutation leading to protein truncation within exon 1
has not been reported before despite of up to 85 reported abnormalities
of the GCH1 gene including exon (start point change, missense, nonsense, and frameshift mutations) and intron mutations and deletions.
The unaffected parents did not show any mutation in the GCH1 gene.
In cerebrospinal uid of the patient the neurotransmitters 5-hydroxyindolacetate, homovanillic acid and 3-ortho-Methyldopa as well as the
pterines showed extraordinary severe changes.
Conclusions: We describe here a hitherto unknown mutation in the
GCH1 gene leading to DYT5 in combination with extraordinary severe
changes in CSF-levels of 5-hydroxyindolacetate, homovanillic acid and
3-ortho-Methyldopa as well as the pterines.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: The aim of this study was to estimate quality of life of

patients with different types of focal dystonia.
Background: Focal dystonia is not physically disabling disorder, but
it inuence quality of life.
Methods: From January to July, 2005, 157 consecutive patients with
adult onset primary focal dystonia, from outpatient settings of the
Movement Disorders Department, Institute of Neurology, Belgrade,
were included in this cross-sectional study. There were 91 (58%)
patients with cervical dystonia (CD), 47 (30%) with blepharospasm
(BSP), and 19 (12%) with graphospasm (GSP). Patients who were
unwilling to participate were excluded, as well as those with secondary
dystonia, exposure to neuroleptic drugs and presence of cognitive
disturbances. All patients included had completed a Short form 36
(SF-36) questionnaire (Serbia translation), which was outcome measure
for health related quality of life (HRQoL). We also collected baseline
demographic and clinical data for each patient. Depression and anxiety
were measured by Hamilton depression rating scale (HDRS) and Hamilton anxiety scale (HAMA). The discriminant analysis was performed
as a method to optimize the discriminant power of scales, particularly
quality of life scales, for differentiation between patients with different
types of dystonia.
Results: Patients with GSP scored the best for physical functioning
(PF) (p0.020), and the worse bodily pain (BP) (p0.001). Patients
with CD and BSP scored signicantly better than patients with GSP for
general health (GH) (p0.001). Patients with BSP scored the worst for
social functioning (SF) (p0.002). For role physical (RP), vitality
(VT), role emotional (RE) and mental health (MH) there were no
signicant differences among patients with CD, BSP and GSP. BP and
SF showed the highest discriminant powers (0.722, 0.434).
According to the classication analysis, 67.5% originally grouped
cases were correctly classied. Signicant positive correlation is obtained between duration of the disease and BP, while actual age is
signicantly inversely correlated with PF. HDRS and HAMA signicantly correlated with all domains of SF-36, except with VT.
Conclusions: The results show that dystonia signicantly inuence
HRQoL in different domais.
377
Clinical genetics of musicians dystonia
A. Schmidt, H.-C. Jabusch, J. Hagenah, L. Enders, N. Bruggemann,
K. Lohmann, R. Saunders-Pullman, S.B. Bressman, P.L. Kramer,
A. Munchau, E. Altenmuller, C. Klein (Hannover, Germany)
Objective: To investigate a potential role of genetics in musicians
dystonia (MD).
Background: MD is generally considered a sporadic disorder but a
genetic contribution has recently been suggested.
Methods: The families of 28 index patients suffering from MD (14
with a reported positive family history (FH) of focal task-specic
dystonia (FTSD) and 14 with no known family history (FH?)) underwent a standardized telephone screening interview using the Beth Israel
Dystonia Screen (BIDS). Videotaped neurological examinations were
performed in all participants screened positive for dystonia. Videotapes
were independently reviewed by three neurologists and consensus
diagnoses were established. All patients were investigated for the
presence of the GAG deletion in DYT1 and suitable families were
tested for linkage to DYT7 locus.
Results: Besides the 28 index patients, 15 relatives directly reported a
form of FTSD. BIDS screening of another 78 family members identied
additional 11 possibly affected individuals. MD was established by personal examination and videotape review in all 28 index patients. With the
same procedure, a type of dystonia was conrmed in 24/26 (MD: n8,
other FTSD: n16) of the putatively affected relatives, 7 of whom were
members of FH? families. In 48 of the 52 affected individuals, at least one

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

S117

rater found evidence for an additional form of dystonia, and other movement disorders were seen in 19 patients. In total, 19 families were multiplex families with 2-4 affected members in one (n6), two (n11) or
three (n2) generations, compatible with autosomal dominant inheritance
in at least 13 families. The DYT1 mutation was not present in any of the
tested patients. Linkage to the DYT7 locus could not be excluded in any of
the 11 informative small families.
Conclusions: Dystonic signs, mostly FTSD, are present in a considerable number of relatives of index patients with MD. Our results
suggest a genetic contribution to FTSD with phenotypic variability
including MD and may lead to the identication of FTSD predisposing
genes.

gene dosage analysis in selected family members. Linkage to the

SGCE, DYT15, DYT1, DRD2 and SLITRK1 loci was also tested.
Results: We included three healthy and 11 affected family members
with M-D (n3), dystonia alone (n2), GTS (n1), tics (n1) or a
combination of these with OCD (M-DOCD: n2; dystoniaOCD:
n1; M-DGTSOCD: n1). There was no linkage to the SGCE,
DYT15, DYT1 or DRD2 loci. No changes were found in the SLITRK1
gene.
Conclusions: The presence of both M-D and GTS in one family, in
which all known M-D loci and a recently discovered GTS locus were
excluded, suggests a novel susceptibility gene for both M-D and GTS.

378

Deep brain stimulation of the globus pallidus internus (Gpi-DBS)

in a patient with generalized dystonia due to tyrosine hydroxylase
deciency
A. Kaelin-Lang, J. Abu-Isa, M. Schuepbach, A. Stibal (Bern,
Switzerland)

Low-frequency rTMS of the premotor cortex in pantothenate kinase-associated neurodegenerative disease

V. Mylius, A. Gerstner, A. Leonhardt, D. Hellwig, F. Rosenow,
W.H. Oertel (Marburg, Germany)
Objective: We investigated the effect of a 1-Hz repetitive transcranial magnetic stimulation (rTMS) - applied over the premotor cortex on motor symptoms of pantothenate kinase associated neurodegenerative disease (PKAN) in a 6-year-old male patient.
Background: PKAN is an autosomal recessive disorder and can
present with secondary generalized dystonia and axial spasms with
early-onset and rapid progression. In dystonia a reduced cortical inhibition leads to an enhanced cortico-spinal output resulting in the
observed symptoms. Therefore, inhibitory repetitive transcranial magnetic stimulation (rTMS) of the premotor cortex has been reported to
decrease cortical excitability in different forms of primary and secondary dystonia with temporary therapeutic benets (Murase, N et al.
Brain 2005;128:104-115; Lefaucheur, JP et al. Neurophysiol Clin
2004;34:141-145).
Methods: Effects of 1200 stimuli of 1-Hz rTMS at 95% of the resting
motor threshold (RMT) applied over the left dominant premotor cortex
for ve consecutive days were evaluated one day before, during treatment and two days following treatment. Frequency of axial spasms and
the need of additional benzodiazepine medication were counted. Dyskinesia was assessed by the AIMS and Goetz scale and dystonia was
scored by the Burke-Fahn-Marsdens Dystonia (BFMD) movement and
disability score once before and once after the stimulation.
Results: Frequency of axial spasms reduced gradually from 2-3 attacks
daily to one attack daily, whereas the the dyskinesia and the dystonia rating
scores remained unchanged. Additional benzodiazepine was given by an
average of 3 times daily during the month before and by an average of 1.2
times daily during the month following stimulation.
Conclusions: Increased cortico-spinal output might have been reduced
by inhibitory rTMS of the premotor cortex in PKAN. Although further
conrmation is warranted, we propose that rTMS might be useful for
temporary control of the frequency of severe axial spasms in PKAN.
379
Autosomal dominant myoclonus-dystonia and Tourette syndrome
in a family without linkage to the SGCE gene
M. Orth, A. Djarmati, T. Baumer, S. Winkler, A. Grunewald,
K. Lohmann-Hedrich, K. Kabakci, J. Hagenah, C. Klein,
A. Munchau (Hamburg, Germany)
Objective: To report clinical details and results of genetic testing for
mutations in the epsilon-sarcoglycan (SGCE) gene, the Slit and Trklike 1 (SLITRK1) gene and for linkage to the DYT15, DYT1 and
DRD2 gene loci in a family with autosomal dominant myoclonusdystonia (M-D) and Gilles de la Tourette syndrome (GTS).
Background: M-D is known to be associated with obsessive compulsive disorder (OCD) but co-morbidity of M-D and GTS has so far
not been reported.
Methods: Fourteen family members, from three generations, underwent a detailed clinical assessment and donated DNA samples. The
SGCE and the SLITRK1 gene were sequenced and investigated by

380

Objective: To demonstrate that Gpi-DBS can improve dystonia in

patients with tyrosine hydroxylase deciciency.
Background: A 28-year-old woman with progressive generalized
dystonia since childhood presented with painful dystonic spasms of
both legs, ataxia, weight loss, swallowing difculties and joint contractures. The etiology was unknown and previous treatment with
levodopa was not successful. CSF investigation revealed the typical
nding of tyrosine-hydroxylase deciency.
Methods: Several drug treatment including levodopa (repeated),
benzodiazepine and intrathecal baclofen injection did not show any
benecial effects on the disabling dystonic spasms and neurosurgery
for bilateral Gpi-DBS was performed.
Results: Under bilateral Gpi-DBS, dystonia markedly improved with
complete disappearance of the painful spasms. Swallowing also improved and the patient gained weight. Furthermore, the improvement of
dystonia allowed orthopaedic surgery to correct joint contractures. In
contrast, dysarthria and ne motor control of the ngers did not
improve.
Conclusions: Gpi-DBS is a therapeutic option to treat dystonic
symptoms in patients with tyrosine-hydroxylase deciency but without
response to dopaminergic treatment.
381
The entitity of jaw tremor and dystonia
S.A. Schneider, K.P. Bhatia (London, United Kingdom)
Objective: To describe the entity of jaw tremor and dystonia.
Background: Jaw tremor in the context of dystonia is not well
recognized.
Methods: We describe seven women (average onset age 70) with jaw
tremor and dystonia.
Results: All had features of primary dystonia, affecting the neck
(n5), the eyes (n2), the hand (n1) or the laryngx (n1). Previous
drug exposure (particularly neuroleptics), dental intervention and other
causes were excluded. Applying MDS criteria for tremor, three had
dystonic jaw tremor and four had jaw tremor associated with dystonia. Drugs were unhelpful. In two cases, jaw tremor was aggravated
by tetrabenazine treatment. Botulinum toxin injections proved effective
in one case.
Conclusions: We propose the entity of jaw tremor and dystonia. It
is distinct from jaw tremor due to other causes including Parkinsons
disease and essential tremor.
382
Early dystonia in probable Creutzfeldt-Jakob disease with diffusion weighted MR images
S.-H. Lee, S.-B. Koh, K.-W. Park, D.-H. Lee (Seoul, Korea)
Objective: We report a patient who showed generalized dystonia
followed by focal dystonia in early stage disease with concomitant
lesions on diffusion-weighted MR images (DWI).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S118 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: Creutzfeld-Jakob disease (CJD) is a progressive spongiform encephalopathy that is mainly characterized by rapidly progressive dementia, myoclonus, ataxia, epilepsia partialis continua, visual
disturbances and movement disorders. Several Movement Disorders
including myoclonus, dystonia, choreoathetosis, tremor, and Parkinsonism have been described in a signicant number of patients with
CJD. However, dystonia as an early symptom is rare.
Methods: A 64-year-old woman admitted to the hospital with a
four-week history of behavior change, emotional instability, gait disturbance and twisting of left upper extremity.
Results: She had synchronous contractions of the left nger, wrist
and elbow muscles, which caused a dystonic posturing of the left upper
extremity. The dystonic posture of the left upper extremity caused
levitation of the arm with exed ngers and an extended wrist. Stimulus sensitive generalized myoclonic jerks were also observed. The
initial DWI scans on admission showed high signal intensity at the right
caudate nucleus, putamen and frontal cortex. The dystonic posture and
myoclonic jerks worsened even with anticonvulsive therapy (valproic
acid 600 mg/day, clonazepam 0.5 mg/day).Ten days after admission to
the hospital, the dystonic posture spread over the right side to include
the lower extremity. The follow up DWI scans showed high signal
intensity bilaterally at the basal ganglia and cortex.
Conclusions: In our case, the initial DWI revealed a corresponding
striatal lesion with contralateral focal dystonia; the follow up DWI also
revealed bilateral striatal lesions, which correlated with generalized dystonia. These DWI ndings suggest that the functional changes in the basal
ganglia may be related to early dystonia in patients with CJD. However,
abnormalities on DWI may not closely correlate with neuropathological
ndings (especially early in the course of CJD), suggesting a more functional, rather than anatomical, basis to the changes identied with DWI.
Therefore, we thought that most CJD patients, with unilateral DWI abnormalities, do not present with focal dystonia in early stage CJD.
383
Long-term treatment of cervical dystonia with botulinum toxin A
retrospective assessment of the clinical and quality of life impact in
patients treated for 10 years
M. Bares, I. Rektorova, M. Balaz, H. Streitova, E. Minks,
P. Kanovsky, I. Rektor (Brno, Czech Republic)
Objective: The goal of the study was to assess the long-term treatment of idiopathic cervical dystonia in patients treated with botulinum
toxin A.
Background: The possible impact on the quality of life of patients
with cervical dystonia has been assessed too.
Methods: Selection criteria: (i) Regular treatment with botulinum toxin
for at least ten years and (ii) continuous good clinical response to the
botulinum toxin injections. Group consisted of 46 patients [26 females, 20
males, mean age 56 years (SD11.9), mean disease duration 18.3 years
(SD9.74), mean age at disease onset 37.5 years (SD12.75). The
efcacy of the treatment was assessed by a clinical neurological exam,
patients self-assessment, patients questionnaire, and Tsui scale.
Results: Mean follow-up of the patients was 10.6 years (range 10-13
years). Mean number of botulinum toxin injections per patient was 35.7
(range 22-50); mean dose per treatment session was 490 units of
Dysport (SD141) and 107 units of Botox (SD28). The mean
period between two consecutive treatment sessions was 110 days
(SD61.8). The mean Tsui score before the treatment was 11.7
(SD2.69), after ten years the Tsui score declined to 7.9
(SD2.81). We did not observe the need to change the botulinum
toxin dose. Adverse events were rare, occurring in less than 1% of
treatment sessions (dysphagia, local pain, weakness). Based on the
patients questionnaires, the most positive impact on their quality of life
was in social communication.
Conclusions: Long-term treatment of cervical dystonia with botulinum toxin A is safe and efcacious. Chemical denervation with botulinum toxin A improves the quality of life and social communication.

Movement Disorders, Vol. 22, Suppl. 16, 2007

384
Can blepharospasm herald multiple sclerosis?
G. Loria, F. Soleti, S. Servidei, A. Evoli, A.P. Batocchi,
A.R. Bentivoglio (Rome, Italy)
Objective: To evaluate the occurrence of movement disorders in
demyelinaling disease.
Background: Movement disorders are an uncommon symptom of
multiple sclerosis. Blepharospasm (BS), a spasmodic contracture of the
orbicolaris oculi, in particular, was described in two cases.
Methods: We describe a 49-year-old man, who presented with a tenmonth history of asymmetric BS (worst in the right side). He suffered optic
neuritis in the right eye 1 month before the onset of BS, improved with
steroids. He had no history of exposure to neuroleptic drugs, alcohol or
drug addiction, perinatal asphyxia, traumas, hypertension or diabetes. No
family member was reportedly affected by movement disorders. When
excluding BS, neurological examination was normal. Brain and spinal cord
MRI, at 11 months after clinical symptoms, revealed three non-enhancing
lesions in periventricular, paratrigonal and parietal areas in the brain and
one lesion at C5-C6 level. VEP showed an increased P100 latency in the
right eye. The analysis of CSF showed hyperproteinorraquia and no
infections. Oligoclonal bands were present. The patient was treated with
low doses of botulinum toxin 11 month after the presentation of BS, with
partial benet.
Results: The diagnosis of multiple sclerosis (MS) in this patient,
according to the currently accepted diagnostic criteria by McDonald et
al (two attacks, clinical evidence of one lesion, three MRI detected
lesions consistent with MS plus positive CSF) is discussed.
Conclusions: Can we consider BS as the second attack of a demyelinating disease? Should MS be included in the differential diagnosis
of BS? Are the 2 symptoms a simple coincidence?
385
Deep brain stimulation in dystono-dyskinetic syndromes secondary
to mitochondrial diseases: Predictive value of 18F-FDG PET
L. Cif, F. Comte, B. Biolsi, H. Elfertit, S. Gavarini, A. Saux,
X. Vasques, P. Coubes (Montpellier, France)
Objective: To study the in vivo glucose metabolism in mitochondrial diseases and to correlate it with the phenotype, the electrophysiological data and the results of GPi stimulation.
Background: Deep Brain Stimulation (DBS) represents an effective surgical treatment of primary dystono-dyskinetic syndromes
(DDS) but its efcacy is difcult to predict in degenerative DDS. In
mitochondrial encephalopathies, severe DDS can occur in association with other neurological impairments. The assessment of the
residual metabolism of the targeted structures but also of the connected networks is of high interest in this group. This information
should be added to the anatomical and electrophysiological data for
selecting patients for DBS.
Methods: Four patients with DDS secondary to mitochondrial diseases were included in the study. DDS was assessed by the two sections
of the Burke Fahn Marsdens Dystonia Rating Scale. To measure the
functionality of the pyramidal tract, motor evoked potentials were
performed and DaTScan to assess the presynaptic dopamine transporters. The indication for DBS was accepted only for patients with no or
mild pyramidal tract involvement. These patients were candidates for
DBS independently of the FDG-PET results, later on correlated with
the impact of the DBS on dystonia and dyskinesia. Patients data are
shown in table 1.
Results: Two phenotypes were dened: rst, with prominence of
the DDS supported by non altered motor evoked potentials (MEP),
normal or mild impaired DaTScan and mild abnormalities in MRI
and second, with prominence of the motor decit (impaired MEP,
altered MRI and DaTScan). In patient 1, DBS was proposed for
prominent DDS compared to the mild pyramidal tract involvement.
In patient 3, DBS was performed as DDS was severe and pyramidal
tract functional. At 6 months, patients 1 and 3 were improved by
18.14% and 35.6% respectively for the movement scores and by
10.35% and 17.9% for the disability scores. Because of the prom-

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

inence of pyramidal tract involvement, DBS was not proposed in
patients 2 and 4.
Conclusions: A preserved pyramidal tract and a normal metabolic
pattern of the target and the related networks are necessary to obtain the
therapeutic effect.

Genetical, clinical, electrophisiological, MRI, DATscan and

PETdata in the four patients
Brain
MRI

Clinical
presentation

DaTSCAN
index of
xation

Etiology

BFMDRS

Complex 1
decit (
MELAS
mutation 3243 )

Pre:102/120,
29/30; Post:
83.5/120,26/
30

Severe
bilateral
striatal
necrosis

Severe
dystonia

LC:0.48,
RC:0.37,
LP:0.32, RP:
0.29

Pyruvate
deshydrogenase
deciency

Pre:39/120,
16/30

Minor
dystonia
component

LC:1.44,
RC:1.25,
LP:1.07, RP:
1.10

Complexe 1
decit of the
respiratory

Pre:88.5/
120,28/30;
Post:57/120,
23/30

Bilateral
thalamic,
putaminal,
external
and
internal
pallidal
lesions
Moderate
bilateral
putaminal
and
external
pallidum
necrosis

Prominent
dystonia

LC:1.65,
RC: 1.33,
LP: 1.17,
RP: 1.07

NARP mutation
8993 of the
ATPase

Pre:4/120,
24/30

Bilateral
putaminal
and
caudate
necrosis

Upper
limb
Myoclonus
Motor
decit in
lower
limbs

LC:2.25,
RC: 1.98,
LP: 1.59,
RP: 1.51

FDG striatal
metabolism
A. Severe
hypometabolism;
B:unchanged
severe
hypometabolism
A. Moderate
bilateral striatal
hypometabolism;
normal cortical
metabolism

MEP
Pyramidal
tract
involvement
Mild
pyramidal
tract
involvement

Normal
A .Moderate
bilateral putaminal
hypometabolism,mild
cortical
hypometabolism,
normal thalamic
metabolism; B:not
yet performed
Pyramidal
A. Normal
tract
cortical
involvement
metabolism;
severe bilateral
striatal
hypometabolism
(more pronounced
for putamen) and
bilateral thalamic
hympometabolism

BFMDRS: Burke-Fahn-Marsdens Dystonia Rating Scale; Pre, preoperative assessment; post, postoperative assessment; /120, movement
score; /30, disability score; LC, left caudate; RC,right caudate; LP, left
putamen; RP, right putamen; A, preoperative PET scan; B, postoperative PET scan
386
Case reports: Blepharospasmus in Hashimoto disease
M. Arnaoutoglou, E. Koutsouraki, V. Costa, E. Avdelidou,
S.A. Kapsali, E. Kalliolia, C. Karamanidis, N. Arnaoutoglou,
G. Spanos, G. Xiromerisiou, S.I. Baloyannis (Thessaloniki, Greece)
Objective: The aim of our study was to present two case reports of
patients with chronic active Hashimoto disease with concomitant
blepharospasmus.
Background: Hashimoto disease in euthyroid patients has been associated with encephalopathy, featuring cognitive deterioration, strokelike episodes and focal or generalized seizures. Furthermore, Hashimoto encephalopathys laboratory ndings are non- specic (high titers
of antithyroid antibodies, high CSF protein levels without accompanying pleocytosis, focal or generalized slowing in EEG and cerebral
leukoencephalopathy on MRI). Focal or segmental dystonia as a neurological complication of autoimmune thyroiditis to our knowledge has
not been described.
Methods: We report two patients: Patient A, female, age 56, with
prior history of Hashimato thyroiditis and blepharospasmus (for 3
years) and patient B, female, age 60, with prior history of Hashimato
thyroiditis, blepharospasmus and oromandibular dystonia (for one
year).Cognition was normal and clinical evaluation revealed no other
focal neurological sign. Both patients were euthyroid (while being on
thyroxin replacement therapy), with high titers of autoantibodies
against thyroglobulin on successive screening. Clinicalaboratory evaluation for myasthenia (endophronium test, repetitive stimulation test,
anti- Ach and anti-MUSK autoantibodises) and myopathy (EMG, muscle biopsy) was negative and EEG was normal. Neuroimaging (Brain
MRI and DAT-scan) was normal in patient A whereas patients B MRI
showed non-specic white matter lesions
Results: Patient A was treated with baclofen, clonazepam and topiramate with no signicant amelioration, whereas patient B responded to
topiramate treatmement (100mg/day) with a signicant decrease on the
frequency and duration of dystonic episodes.

S119

Conclusions: Although our study is merely a presentation of case

reports, it suggests that these dystonic presentations could be associated
with chronic active Hashimato disease, giving ground to future research
of autoantibody involvement in dystonia.
387
The characteristics of adult onset Segawa disease
M. Segawa, Y. Nomura, K. Kimura, R. Hanajima (Tokyo, Japan)
Objective: Investigation of the pathophysiological characteristics of
adult onset patients with Segawa disease.
Background: Segawa disease is classied into two types, postural
dystonia and action dystonia depending on the family or the loci of
mutation1). In the families with action dystonia type there are cases
with focal or segmental dystonia. In both types, D2 indirect pathway is
not involved. In the postural type the ascending pathway of the basal
ganglia is not involved2). However, it is not claried whether other
pathways of the basal ganglia which mature later are involved in adult
onset patients.
Methods: Three adult onset patients were subjected to this study.
Patient 1, 41-year-old male started with writers cramp at 35 years of
age and generalized hypertonus developed by 39 years. Patient 2,
43-year-old female, started leg tremor at 28 years of age which became
generalized by 34 years of age. Patient 3, 64-year-old male, started with
left hand tremor at 58 years of age, which generalized with rigid
hypertonus in one year.
Results: The symptoms showed asymmetry, i.e. the side predominantly
involved was ipsilateral between sternocleidomastoideus and extremity
muscles with tremor, but contralateral with rigid hypertonus. Diurnal
uctuation, short stature or exaggeration of tendon reexes were not
observed. Psychomental activities were normal. Voluntary saccades, sensory evoked potential (SEP) and paired pulse transcranial magnetic stimulation (PPTMS) were performed in cases 1 and 3. In contrast to the
patients with postural dystonia type, in these cases memory guided saccades were more involved than the visually guided saccades. Gating was
not observed in SEP and the inhibition of the response was abnormal in
PPTMS. Patients 3 showed normal corporation in uoroDopa and Spiperon PET. In cases 1, 2 and 3, levodopa 150 mg, 200mg and 200mg
showed sustained favorable effects respectively.
Conclusions: These results showed that in adult onset patients of
Segawa disease, the dopamine neurons innervating to the subthalamic
nucleus with D1 receptor are predominantly involved, and cause hyperkinetic disorder through disfacilitation of the ascending pathway.
References:
1 Segawa M et al. Ann Neurol 54 (Suppl 6); S32-45, 2003.
2 Hanajima R et al. Intracortical inhibition of the motor cortex in
Segawas disease (DYT5). Neurology. (in press).
388
No difference in efcacy between Xeomin and Botox in the
treatment of cervical dystonia a detailed subgroup analysis
H. Hefter, R. Benecke, G. Comes, S. Grafe (Duesseldorf, Germany)
Objective: To support the 1:1 dose ratio of Xeomin and Botox, we
re-analyzed the data of this study whether various subgroups of patients
revealed a different response to Xeomin or Botox.
Background: Treatment of cervical dystonia (CD) with the highly
puried botulinum neurotoxin type A (BTX-A) free of complexing
proteins (Xeomin) is mainly based on the largest controlled study of
BTX-A ever performed (Benecke et al. 2005) demonstrating that Xeomin is non-inferior to Botox and that in average the efcacy of one
Xeomin unit is equivalent to one Botox unit.
Methods: The data of a multi-center, randomized, double-blind,
active-controlled clinical study in patients with CD pre-treated with
Botox (N466) were re-analyzed.
Results: The separate analysis of the TWSTRS - Severity score with
regard to subgroups by age (50 years vs. 50 years) and gender
revealed a higher improvement of the score in the younger patients
which was additionally slightly more pronounced in male than in
female patients. No difference between Xeomin or Botox could be

Movement Disorders, Vol. 22, Suppl. 16, 2007

S120 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

detected. The change in the TWSTRS Severity score was higher in the
low dose group (120 U) in comparison to the high dose group ( 120
U): Xeomin: -6.6,-6.4, Botox: -6.7, -6.0, respectively). The percentage of non-responders (20% improvement) or golden responders
( 70% improvement) did not differ signicantly in the individual
severity subgroups. The treatment effect increases with higher baseline
severity. No difference between Xeomin and Botox was found
regardless whether mildly, moderately, or severely affected patients
were treated.
Conclusions: In summary, the present detailed subgroups analysis
between Xeomin or Botox did not reveal any statistically signicant
differences. Therefore no relevant difference in medical practice can be
expected (as long as quality standards for Xeomin and Botox are
kept constant).

Change in TWSTRS-Severity Score after 4 Weeks by

TWSTRS Severity at Baseline
Change in TWSTRS-Severity Score after 4 weeks
Xeomin
TWSTRS Severity at Baseline
mild (15)
moderate (16-21)
severe (22)

N
58
140
32

Mean (SD)
-5.6 (3.3)
-6.6 (4.0)
-8.2 (5.0)

Botox
N
60
127
39

Mean (SD)
-5.1 (3.8)
-6.4 (3.7)
-8.1 (4.0)

389
A retrospective study of Botox versus Dysport in patients with
movement disorders
A.L.Z. Rosso, D.H. Nicaretta, J.P. Mattos, S.A.P. Novis (Rio de
Janeiro, RJ, Brazil)
Objective: The aim of our study was to compare the efcacy and
safety of Botox and Dysport in the treatment of various movement
disorders.
Background: Two different brands of botulinum toxin type A (Botox
and Dysport) are available commercially, but much controversy remains about their respective potencies.
Methods: An retrospective study involving 42 patients with movement disorders was performed. The patients received either Botox at
the usually effective dose or Dysport at a dose of 1:2 (Botox 1 unit
Dysport 2 units). The improvement was assessed through a subjective
scale of 0 to 4 (null to 100%). The incidence of adverse events and the
duration of the improvement were also assessed.
Results: Of the 42 patients, 31 were female and 11 were male; 18 has
hemifacial spasm, 5 cervical dystonia, 5 generalized dystonia, 5 blepharospasm, 5 Meige syndrome, 2 hemidystonia, 1 hand dystonia and 1
with sialhorrhea secondary to Parkinsons disease. The mean age at the
onset was 58.8 years and the mean time of disease duration was 62.5
months. The mean number of injections was 3.5 for Botox and 2.4 for
Dysport. The mean dose was 134.4 U for Botox and 245.5 U for
Dysport. There were 9 patients with mild adverse effects with Botox
and 4 with mild to moderate adverse effects with Dysport. Symptoms
improvement was not statistacally different from both toxins (3.7 Botox
and 3.8 to Dysport) as well as the duration of the improvement (3.3 to
Botox and 3.1 to Dysport).
Conclusions: The results suggest that the therapeutic effectiveness is
equal for both botulinum toxins. Although with Botox we had more
patients with adverse effects, these were found only with the earlier version
of the botulinum toxin and were also mild in comparison with Dysport.
390
Post-traumatic adult-onset focal dystonia: A retrospective study of
six consecutive cases
R. Riemer, Y.-L. Zheng, H. Luo, C. Lindsey, V. Wheelock, L. Zhang
(Sacramento, California, USA)
Objective: To analyse the association between cervical-focal limb
dystonia and prior trauma.
Background: While the relationship between prior trauma and adultonset dystonia remains controversial, a collection of scientic studies and

Movement Disorders, Vol. 22, Suppl. 16, 2007

clinical observations strengthen the probability of a causative link between

dystonia and trauma. The observation that thermal injury to the hind leg of
a rat resulted in a reduction in neurotransmitters responsible for pain
modulation in the basal ganglia implies a connection between the peripheral pain receptors to this deep brain structure. Clinical observations also
bolster the position of peripheral trauma as an inducer of movement
disorders. For instance, peripheral limb dystonia can be seen in conditions
such as complex regional pain syndrome. It may be deduced that abnormal
physiologic processing of sensory information, which in turn inuences
motor cortices, implicates a role of the somatosensory system dystonia.
Methods: Patients with dystonia who presented to the Movement
Disorders Clinic of UC Davis Medical Center were systematically
asked for history of trauma at their rst interview. Inclusion criteria
were as follows: (1) onset of dystonia within 2 months of the trauma;
(2) no history of cortical, cerebellar, sensory decits or stroke; (3) no
history of paroxysmal dystonia; (4) no abnormality on imaging studies;
and (5) no signicant laboratory abnormalities.
Results: Of 87 patients in our database, 6 met the inclusion criteria.
They ranged from 27 to 53 years of age and noted onset of dystonia
within 2 months after injury. Patients were treated with muscle relaxants, levodopa, botulinum toxin and/or surgery, and were followed up
to 7 years. Of the 6 patients, 4 demonstrated arm dystonia with one
having additional shoulder dystonia; one with cervical dystonia; and
one with leg focal dystonia. None of the patients developed into any
other type of dystonia. Three of the patients have responded to botulinum toxin. One patient was treated surgically for a concurrent thoracic outlet syndrome with minimal postoperative improvement.
Conclusions: These results support prior trauma as a possible environmental factor modifying the risk of developing adult-onset cervicalfocal dystonia. The pathogenesis and medical-legal implications of
such association await further investigation.
391
Secondary dystonia related to celiac disease
A.L. Diamond, P. Agarwal, V. Segro (Englewood, Colorado, USA)
Objective: To describe a relationship between celiac disease and
dystonia.
Background: Secondary causes of dystonia result from a variety of
primary neurological diseases, inherited metabolic defects, parainfectious,
autoimmune disorders, structural brain lesions, and medication reactions.
We describe an association of secondary dystonia and celiac disease.
Methods: We performed a retrospective chart review of 2 patients
with dystonia and celiac disease at a Movement Disorder Center.
Results: A 52-year old woman with no gastrointestinal symptoms was
referred for a 1 year history muscle spasms in her back, abdominal muscles
and buttocks associated with camptocormia and wide-based gait. During
prolonged ambulation her camptocormia became more prominent. Jogging
and walking backwards resulted in a marked improvement in symptoms.
EMG of her lower extremities and paraspinal muscles revealed spontaneous high frequency burst discharges consistent with dystonia. Neuroimaging, DYT-1, anti-GAD, acanthocytes, autoimmune, ceruloplasmin, and
CPK were normal. There was a positive HLA-B27, elevated anti-gliadin
(IgA) but normal tissue transglutaminase and endomysial antibodies. She
was referred for nutrition consultation. A 44-year old woman with history
of irritable bowel syndrome and peripheral neuropathy was referred for a
20 year history of cervical dystonia and 5 year history of truncal and lower
extremity dystonia. Her examination revealed marked right laterocollis
with left rotation, postural and kinetic tremor, truncal dystonia with intermittent inversion of both feet. Gait was characterized by erect posture,
exed hips and knees with inverted gait pattern. Neuroimaging, DYT-1,
ceruloplasmin, CPK, acanthocytes, and autoimmune studies were normal.
Anti-gliadin (IGA, IGG), tissue transglutaminase and endomysial antibodies were elevated. She was referred for nutrition consultation.
Conclusions: Celiac disease is an autoimmune disorder from an
immune response to gluten. Extra-intestinal manifestations include
psychiatric disturbances, epilepsy, ataxia, neuropathy and tremor. The
strength of the association of elevated serologic markers to the pathophysiology of dystonia is subject debate. A gluten-free diet is the
standard of treatment, although its effect on the extra-intestinal mani-

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

festations is variable. An epidemiologic study screening a large cohort
of dystonia patients and controls may be warranted.
392
Behavioral abnormalities in DRD
I. Trender-Gerhard, P. Mir, M. Edwards, M. Sweeney, L. Majahi,
A. Gerhard, N. Wood, K. Bhatia (London, United Kingdom)
Objective: To evaluate the prevalence of psychiatric abnormalities in
autosomal-dominant GCH1 deciency.
Background: DRD was hitherto recognized as a purely dopaminedecient movement disorder. Only recently psychiatric disorders have
been described in 4 families.
Methods: We assessed 43 symptomatic and non-motor-affected
GCH1 mutation carriers using a questionnaire including questions
for depression, mood changes as well as the Beck Depression
Inventory in 14 patients. Further clinical information was obtained
by analysis of medical records as well as neurological examination
and interview.
Results: Half of all mutation carriers, including asymptomatic
subjects without motor dysfunction, presented with neuropsychological abnormalities. They often antedated the initiation of L-dopa
or reoccured despite efcient and stable treatment, and even presented in asymptomatic subjects unaware of their genetic status. The
frequency of recurrent depressive disorders (47%), either non-reactive mood swings or major depression, was increased compared to
the population incidence. Additional features such as panic attacks,
obsessive-compulsive disorder, concentration problems and verbal
memory decits occurred in several patients. We found a clear
familial accumulation of depressive disorders in DRD independent
of the motor status: 3/4 of all carriers affected by depressive
disorders had at least one mutation-positive rst grade relative with
a depressive disorder.
Conclusions: Psychiatric features seem not to be induced by
L-dopa in many cases nor are they related to the severity of DRD.
It is conceivable that the co-existence of DRD and familial occurrance of depressive disorders in particular might be related to the
site of the GCH1 mutation which may not only cause a dopamine
deciency but a clinically relevant dysfunction in the serotonin and
noradrenaline biosynthetic pathway. Thus we hypothesize that the
serotonergic and noradrenergic, decit in DRD could enhance the
susceptibility to depression which, in turn, may manifest on additional genetic or environmental risk factors.

S121

394
Immobilization followed by motor training is an effective therapeutical approach in patients with writers cramp
K.E. Zeuner, M. Peller, A. Knutzen, I. Holler, M. Hallett,
G. Deuschl, H.R. Siebner (Kiel, Germany)
Objective: To combine immobilization with motor training to treat in
patients with writers cramp.
Background: Task-specic hand dystonia, such as writers cramp,
may result from aberrant plasticity triggered by excessive repetitive
use of the hand. Previous studies assessed the effect of immobilization of the affected limb or sensorimotor re-training alone and
reported some clinical improvement. Here we combined immobilization with motor training to potentiate the efcacy of both interventions.
Methods: Seven patients with simple and 19 with dystonic writers
cramp were randomly assigned to two groups. All patients wore a
plastic splint to immobilize the wrist and nger joints of the affected
right hand for four weeks. After immobilization, group A received a
specic training of individual nger movements, which involved drawing exercises using Stax nger splints with a pen attached to the splint.
Group B received occupational hand and nger training with Theraputty. All patients completed a xed training program for four weeks,
and continued the learned exercises for another four weeks. The Writers Cramp Rating Scale (WCRS) was used as the primary outcome
measure. The Arm Dystonia Disability Score (ADDS) and kinematic
handwriting analysis served as secondary outcome measures. All measurements were assessed at baseline (week 0), after immobilization
(week 4), and training (weeks 8 and 12).
Results: Regardless of the type of training, blinded assessment of the
video tapes that were obtained during a standardized handwriting task
revealed an improvement in 15/21 patients. The mean WCRS gradually
decreased from 12.38 6.09 at baseline to 8.14 6.29 at week 12
(p 0.005). Likewise, the mean ADDS score gradually improved from
week 0 (52.36 19.34 %) to week 12 (57.30 20.70 %; p0.015).
The kinematic handwriting analysis showed a decrease in pressure
during continuous writing (p0.050). Improvement in pressure correlated with the WCRS (p 0.014).
Conclusions: Immobilisation followed by motor training improves
writers cramp and might be an alternative treatment option to botulinum toxin injections. Occupational nger and hand training with
Theraputty is as effective as a specialized nger training with nger
splints after four weeks of immobilization.

393

395

Atypical phenotype in DYT1 dystonia with paroxysmal dystonia

B. Biolsi, L. Cif, S. Gil Robles, S. Gavarini, X. Vasques, S. Plagnol,
P. Coubes (Montpellier, France)

Abnormal low frequency drive in Myoclonus-Dystonia patients

correlates with presence of dystonia
E.M.J. Foncke, L.J. Bour, J. van der Meer, J.H.T.M. Koelman,
M.A.J. Tijssen (Amsterdam, Netherlands)

Objective: DYT1 dystonia is a dominantly inherited movement

disorder. Since the advent of widespread testing for the presence of the
DYT1 gene mutation, the range of phenotypes that have been associated with this genetic abnormality has expanded.
Background: Here we report a member of an Italian DYT1 family
who presents an atypical presentation.
Methods: The proband was a 45-year-old woman, her disease started
at 20 years old, after her last pregnancy. She had paroxysmal dystonia
with a kinesigenic component, movement of upper and lower limbs
precipitated spasms. The spasms were very painful. She reported also
a worsening of symptoms during her periods. Moreover she suffered
from migraines. Neurological examination is normal between spasms.
The diagnosis of psychogenic dystonia was initially suspected because
of abrupt onset, intermittent and episodic pattern of symptoms together
with normal neurological examination.
Results: In this family, ve members are affected with clinical
variability. Mutation in the TOR1A gene was positive. EMG recording
showed agonist antagonist co-contractions.
Conclusions: To our knowledge, no cases presenting with paroxysmal dystonia have been described in DYT1 dystonia.Paroxysmal dystonia may be a clinical feature of DYT1 dystonia.

Objective: To investigate whether the myoclonic and the dystonic

features are the result of an abnormal common drive to the muscles in
M-D.
Background: The pathophysiology of Myoclonus-Dystonia (M-D), a
autosomal dominantly inherited movement disorder characterized by
myoclonic jerks and dystonic contractions is largely unknown. In some
forms of inherited dystonia, abnormal intermuscular coherence is
present.
Methods: EMG-EMG and EEG-EMG coherence analysis were performed in 20 DYT11 mutation carriers (MC) and 13 healthy controls
during resting condition and during weak isometric contraction of the
arm and neck.
Results: The EMG-EMG coherence analysis showed signicantly
increased intermuscular coherence in the 3-10 Hz frequency band in
4 DYT11 MC with clinical pronounced (mobile and static) dystonia
during weak isometric contraction. This coherence was not present
in DYT11 MC with mild (static) dystonia and/or predominating
myoclonus. The EEG-EMG analysis showed signicant coherence
in the 15-30 Hz frequency band during weak isometric contraction
of the arm in ve healthy controls, but in none of the DYT11 MC.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S122 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Conclusions: The intermuscular coherence in the low frequency
band in DYT11 MC with predominant dystonia is in line with the
previously described coherence in other types of dystonia and
suggests that the pathophysiology of M-D shares common pathophysiological features with (inherited forms of) dystonia. The absence of EEG-EMG coherence in the 15-30 Hz frequency band
during weak isometric contraction in DYT11 MC may reect abnormal motor activation caused by an altered cortical drive due to
basal ganglia dysfunction.
396
Clinical and poly-electromyographic diagnostics of facial movement disorders and their treatment with botulinum toxin
G. Reichel, A. Stenner, W. Hermann (Zwickau, Germany)
Objective: In most of the cases, differentiation between hemifacial
spasm (HS), postparetic facial synkinesia (PFS), blepharospasm (Bl) and
tics (Ts) can be accomplished clinically: Spontaneous synchronous contraction of primarily the mm. orbicularis oculi (OOc) and frontalis is a
typical feature of HS; PFS only appears during involuntary or voluntary lid
movements; in Bl, contractions are always limited to the OOc, and Ts or
voluntary movements do not have effects on the frontal muscle.
Background: There will often be doubts about the respective diagnosis, which is of great importance for the treatment with botulinum
toxin (Btx). Is it possible to differentiate these four illnesses via
synchronous electromyographic recordings from mm. frontalis, OOc
and m. orbicularis oris?
Methods: Electromyographic activities from the three muscles mentioned above were recorded synchronously on both sides via surface
electrodes, during voluntary movements, at rest, and during spontaneous eye closing. Respectively eight patients with HS, PFS, Bl and Ts
were examined. Each patient with HS, PFS and Bl was treated with Btx
(Botox, Dysport, Xeomin).
Results: At rest, all patients with PFS showed myokymia-like action
potentials of motor units (MUAP), whereas only three patients with
HS. and none of the patients with Bl. or Ts. showed activities at rest.
During voluntary eye closing, only Bl showed MUAP, although only in
the m. orbicularis oculi. In PFS, there were always groups of MUAP,
synchronous to these discharges, appearing in the m. frontalis and m.
orbicularis oris on the same side, as well as in the OOc of the opposite
side. Spontaneous groups of MUAP were observed in PFS only
during involuntary eye closing, which was also visible clinically at the
unaffected eye. In HS, spontaneous discharge groups were found, not
depending on involuntary lid movements, always affecting the frontal
muscle, although the m. orbicularis oris did not show synchronous
activities in three patients.
Conclusions: In addition to an exact clinical analysis of movement
patterns and lateral spread response, synchronous EMG-recordings of
mm. frontalis, orbicularis oris and OOc will provide relevant information for the differential diagnosis of the most important movement
disorders in the facial area. Treatment with Btx will produce excellent
results in all cases.
397
Knowledge and perception of dystonia: A comparison among various religions and ethnicities in the state of Florida
V. Gosein, M.P. Silverstein, C.E. Jacobson IV, M.S. Okun,
R.L. Rodriguez, H.H. Fernandez (Gainesville, Florida, USA)
Objective: To determine differences in knowledge and perception of
dystonia between religions and ethnic groups.
Background: Dystonia is a neurological disorder characterized by
involuntary, sustained, muscle contractions causing twisting movements and abnormal postures. The prevalence of dystonia has been
estimated to be 30 in 100,000 and the syndrome strikes many
backgrounds and ethnicities. There remains a scarcity of knowledge
and perception on how different religions and ethnicities perceive
dystonia.
Methods: A survey was administered to various and diverse religious
and ethnic groups in the state of Florida to determine differences in

Movement Disorders, Vol. 22, Suppl. 16, 2007

knowledge and perception of dystonia. A two-page questionnaire was

distributed to all members, age 18 or older, of religious and ethnic
organizations without prior introduction to dystonia.
Results: There were 148 participants in the study. The survey consisted of 66.2% (98/148) Caucasian, 16.2% (24/148) Asian, 9.5%
(14/148) African American and 2.7% (4/148) mixed race. Religions
included Protestant 60.1% (89/148), Catholic 12.3% (19/148), Jewish
8.1% (12/148), BahaI 4.7% (7/148) and Hindi 5.1% (6/148). Responses to notions about dystonia: Dystonia is a disease of the muscles:
33.3% Asian, 22.4% White and 21.4% African American; 50.0%
Hindi, 41.7% Jewish, 28.1% BahaI, 22.5% Protestant and 21.1%
Catholic. Dystonia is a psychiatric disease: 37.5% Asian, 23.5% White
and 21.4% African American; 50.0% Hindi, 28.6% BahaI, 23.6%
Protestant, 21.1% Catholic and 16.7% Jewish. Dystonia initially presents with muscle cramps: 45.8% Asian, 42.9% African American and
26.5% Caucasian; 50.0% Hindi, 28.6% BahaI, 31.6% Catholic, 28.1%
Protestant and 25.0% Jewish. There is a cure for dystonia: 12.5%
Asian, 7.1% Black and 4.1% White; 33.3% Hindi, 8.3% Jewish, 5.3%
Catholic and 4.5% Protestant. It is difcult to be a dentist with dystonia: 29.2% Asian, 23.5% Caucasian, 21.4% African American and
50.0% mixed race; 33.3% Hindi, 33.3% Jewish, 28.6% BahaI, 26.3%
Catholic and 24.7% Protestant.
Conclusions: The responses from this survey reveal that there may
be wide variability in the perception and knowledge of dystonia based
on religion and/or ethnicity. More research is needed to determine how
this difference inuences willingness to seek treatment.
398
Sensorimotor cortex hypoactivation during writing in writers
cramp: An event-related fMRI study
P. Havrankova, R. Jech, N.D. Walker, J. Vymazal, E. Ruzicka
(Prague, Czech Republic)
Objective: We searched for cortical dysfunction during handwriting
in patients with writers cramp in regard of the mode of graphic
expression and the presence of visual feedback.
Background: Previous imaging studies of writers cramp present
ambiguous ndings at cortical and subcortical levels while it is unclear
if such abnormities are related to writing alone and if they depend on
visual control.
Methods: We examined 12 patients with writers cramp (age:
43.4(SD)8.6 years) and 11 control subjects (43.98 years). All
subjects were right-handed and all patients suffered from right-hand
dystonia. In the fMRI experiment, they were writing using a specially
adapted joystick. In each trial of the task, the subjects were randomly
instructed to write either one block letter or to perform free scrawling.
The line they drew was displayed on the screen in only half the trials.
Event-related fMRI was examined on a 1.5 T scanner using echo-planar
imaging. Random effect analysis was used for statistical processing.
Data from patients and controls were compared for each factor and
interaction with compensation for the drawn line length. The results of
2nd level analysis were thresholded at P0.05 level with family-wise
error correction.
Results: In the patients, all joystick movements were accompanied by signicantly lower activation in the bilateral primary sensorimotor cortex than in the healthy controls. Additionally, the
patients exhibited higher activation in the contralateral posterior
temporal cortex. The presence of visual feedback was related to
bilateral occipital activation in both groups of subjects. Conversely,
there were no signicant differences in writing/scrawling or visual
control/no-control interactions between patients and controls.
Conclusions: Patients with writers cramp were found to have primary sensorimotor cortex dysfunction presenting as bilateral hypoactivation, thus proving simultaneous involvement of both hemispheres.
The dysfunction appeared regardless of the nature of graphic expression or the presence of visual control. Moreover, the patients showed
motion-related activation in the posterior temporal cortex, possibly
reecting involvement of aberrant or compensatory activation in the
associative cortex.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Study support: IGA MZ CR 1A/8629-5, NR8937-4 and Research
Program MSM0021620849.
399
Is it always necessary to apply botulinum toxin into the lower facial
muscles in hemifacial spasm? A randomized, single-blinded, crossover trial
B. Donmez Colakoglu, R. Cakmur, F. Uzunel (Izmir, Turkey)
Objective: The rst aim of the study was to evaluate the necessity of
botulinum toxin (BTX) treatment of lower facial region in hemifacial
spasm (HFS). If it is necessary, the second aim was to determine the
criteria for BTX injection of lower facial muscles in HFS.
Background: BTX injections have been accepted as a safe and
efcacious modality for the treatment of HFS. The clinical improvement and side effects by local administration in orbicularis oculi
muscle is well known. Nevertheless, the treatment of lower facial
muscles is debated.
Methods: A randomized, single-blind, crossover, clinical trial was
conducted from June 2003 to December 2004. A total of 23 patients
with HFS, aged 39 to 87 years, were randomly allocated to the 2
treatment groups. The rst group received BTX type A into orbicularis
oculi muscle and serum physiologic into lower facial muscles (zygomaticus major, zygomaticus minor, levator anguli oris, risorius). The
second group received BTX type A into both orbicularis oculi and
lower facial muscles. Response to treatment was evaluated just before
and one month after the administration of BTX by both the patients and
the doctor with severity and frequency scales.
Results: All the patients beneted from BTX treatment. In group of
patients with severe lower facial muscle involvement, the result was
better for the application of BTX into both orbicularis oculi and lower
facial muscles (p0.003). In group of patients with mild involvement
of lower facial muscles, there was no difference between two application methods (p0.05). The patients with higher spasm frequency
beneted more from BTX application to lower facial muscles
(p0.013).
Conclusions: Our data suggest that BTX treatment of the lower facial
region is necessary only in group of HFS patients with severe lower
facial muscle involvement.
400
Short intracortical inhibition (SICI) during different phases of
movement in patients with focal hand dystonia
S. Beck, S. Pirio Richardson, M. Hallett (Bethesda, Maryland, USA)
Objective: To evaluate the role of SICI in the pathogenesis of focal
hand dystonia and its contribution to surround inhibition in the primary
motor cortex, different phases of movement were assessed using single
and double pulse transcranial magnetic stimulation (TMS) in patients
with focal hand dystonia and healthy volunteers.
Background: The coordination of nger movement plays an important role in ne motor skills. Surround Inhibition, rst described in the
retina, is one possible mechanism to focus neural activity and has been
observed in the motor system as well (Sohn et al. 2004). There is
evidence that short intracortical inhibition (SICI), mediated through
GABA-A-ergic transmission, could aid the selective execution of desired movements in humans (Stinear et al. 2004). A reduction of SICI
may be associated with focal, task-specic dystonia and contribute to
the lack of surround inhibition that has been observed in these patients.
Methods: We tested eight patients with focal hand dystonia (FHD)
and eight healthy controls during an isolated index nger exion. MEP
size and SICI were tested in the adjacent abductor pollicis brevis
muscle (APB) at rest, 50 ms before EMG onset (premotor), during the
rst peak of the electromyogram (EMG) (phasic) and during the
maintenance of contraction (tonic, after 1800 ms).
Results: While MEP size is signicantly reduced during the initiation
of movement (for premotor and phasic) in controls, FHD patients show
no signicant modulation. In contrast to previous ndings, there was no
increase of SICI during the premotor or the phasic phase in controls.

S123

Tonic activation did not lead to changes in any of the parameters in

patients or controls.
Conclusions: We conclude that surround inhibition is observed in
healthy subjects and lacking in FHD during movement initiation, but
SICI is most likely not the underlying relevant mechanism for its
production.
401
A Multi-center, open-label, multiple-dose, dose-escalation, safety
and tolerability study of Botulinum Toxin Type B in patients with
cervical dystonia
E.J. Pappert (San Antonio, Texas, USA)
Objective: This was an outpatient, open-label, within-patient, forced
dose-escalation study designed to evaluate the safety and tolerability of
increasing doses of botulinum toxin type B (BoNT-B) in patients with
cervical dystonia (CD).
Background: BoNT-B effectively reduces the severity of abnormal
head position and neck pain associated with CD. BoNT-B is often used
for long-term treatment of CD with escalating doses. This study addresses BoNT-Bs long-term safety in subjects with CD, including the
use of BoNT-B doses above 10,000 U.
Methods: This multicenter, outpatient, open-label study evaluated
escalating BoNT-B doses within CD subjects. Subjects who were
responsive or non-responsive to botulinum toxin type A were included
in the study. After initial treatment with 10,000 U of BoNT-B, subjects
received a second (12,500 U) and third (15,000 U) BoNT-B injection
when CD symptoms returned to baseline (based upon clinician assessment) and if no signicant adverse events (AEs) had occurred. Subjects
were evaluated at baseline and 2, 4 and every 4 weeks after each
injection until CD symptoms returned to baseline. Effectiveness was
determined by the Toronto Western Spasmodic Torticollis Rating Scale
(TWSTRS), 3 visual analog assessments (Patient Global, Investigator
Global and Patient Pain) and by determining clinical benet. Safety
was assessed by reports of AEs, vital signs and clinical laboratory
evaluation.
Results: 145 subjects with CD [mean age 53.1 years; predominantly
Caucasian (95%) and female (65%)], participated in this study. 113
(78%) of subjects were BoNT-A responsive and 32 (22%) of subjects
were BoNT-B resistant. Most (86%) subjects received all 3 doses. An
increasing mean number of weeks between injections (i.e., return of
baseline CD symptoms) was observed with escalating BoNT-B dose
(12.1, 12.9, and 13.9 weeks after 10,000, 12,500 and 15,000 U, respectively). For all doses, TWSTRS-Total scores and the 3 visual analog
assessments improved as early as 2 weeks post BoNT-B injection. Dry
mouth, dysphagia and injection site pain were the most commonly
reported AEs. 13 subjects withdrew from the study because of AEs. No
treatment-related serious AEs were reported.
Conclusions: BoNT-B was shown well-tolerated and efcacious in
CD subjects in this sequential three-injection, forced titration study at
doses up to 15,000 U.
402
Experience of treatment focal dystonic hyperkinesis with botulinic
toxin in center neurology and neurorehabilitation FSE Siberian
Regional Medical Center
D.V. Pokhabov, V.G. Abramov (Krasnoyarsk, Siberia, Russian
Federation)
Objective: To analyze our experience of treatment of focal dystonic
hyperkinesis with botulinic toxin.
Background: The injections botulinic toxin are effective method of
treatment of focal dystonias.
Methods: In our clinic, 45 cervical dystonia(CD) patients, aged
22-69 years, 22 men, 23 women, have received botulinic toxin (Dysport) treatment, average length of disease 3,81,8 years. 31 patient had
an isolated form of CD, 14 CD combined with facial paraspasm, or
including muscles of torso and limbs. Medicament treatment was
ineffective. Decrease of symptoms severity by 50% was considered
signicant improvement, 50 % to 25 % - moderate improvement, less

Movement Disorders, Vol. 22, Suppl. 16, 2007

S124 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

than 25% - insignicant. The Dysport dose contained 500-1200UA.
Spacing between the injections was 14 104 weeks (23,82,5). 18
patients with essential blepharospasm, 15 combined oromandibular
dystonia with blepharospasm, 4 facial hemispasm received botulinic
toxin (Dysport) treatment also. Average length of disease 3,81,6
years. The Dysport dose contained 60-380 UA (for combined cases).
Spacing between the injections was 16 38 weeks (19,63,2).
Results: In all patients signicant clinical improvement was marked.
The effect of toxin was shown in during of 1-14 days (5,61,5 day).
Duration of the maximal clinical effect changed from 4-24 weeks
(14,25,6 weeks). The Dysport was kept general clinical effect till 104
weeks (2 case) (16,26,2 weeks), the side effect wasnt observed. For
the patients with blepharospasm there had been side effect of light
ptosis which lasted for 520 days. Good effect was marked by all
patients. Time between injection and visible effect varied from 1-7 days
(4,62,4). Maximal effect duration varied from 6 to 22 weeks
(13,44,5). General effect lasted for 16 42 weeks (21,64,2 weeks).
Conclusions: For the cases with signicant improvement the opportunity to increase spacing between injections of botulinic toxin appeared in both groups. On background of regular use the severity of
hyperkinesis hadnt come back to initial level, the opportunity to
decrease the dose of botulinic toxin for repeated injections appeared.
For the patients with CD to reach maximal and prolonged effect the
doses of 1000-1500UA were required. In our opinion, botulinic toxin
medications should be a rst choice for treatment of such patients.
403
Wilsons disease facies a distinctive clinical sign
A. Aggarwal, A. Nagral, M. Bhatt (Mumbai, Maharashtra, India)
Objective: 1. To characterize the facial features of Wilsons disease
(WD). 2. To establish that they are a distinct clinical entity. 3. To
examine the clinical correlates of WD facies.
Background: WD is an inherited metabolic disorder affecting
mainly the liver and brain. A combination of dystonia, other Movement Disorders, behavioral abnormalities and cognitive decline
constitute the heterogeneous neurological presentation. This is indistinguishable from other childhood extrapyramidal and neuropsychiatric disorders. Wilsons facies are typical but poorly characterized feature of the disease.
Methods: We prospectively studied the facial features of WD
patients (n25) and compared them to a control group comprised of
normal siblings (n12) and parents of the WD patients and patients
with non Wilsonian Movement Disorders (n12) and non Wilsonian liver (n12) diseases. We dened WD facies and evolved a
grading (from 0-4) for them. This grading was apart of our Global
Assessment Scale for WD (GAS for WD) 1. We then correlated WD
facies with various neurological and other systemic clinical manifestations of WD.
Results: We propose that WD facies are characterized by a variable
combination of open mouth, drooling saliva, facetious smile, pseudoptosis, expressionless face, decreased eye contact, decreased exploratory
eye movements and a dull look. Our study showed that this facial
appearance is restricted to patients with neuro-WD. It is not seen in WD
patients without neurological involvement or any of the control population. The degree of dull look correlates with severity of neurological
involvement. Pearsons correlations show that scholastic backwardness
is a major contributor to the WD facies followed by Parkinsonism,
depression, axial symptoms and dystonia. There is resolution of the
WD facies with treatment of WD. In our study there was good internal
consistency (alpha0.94) and inter and intra-rater reliability (95%)
in identifying and grading WD facies.
Conclusions: We propose that Wilsons Disease facies are an important, easily identiable and semi-quantiable clinical sign of the
disease. They are a unique marker of brain involvement by WD,
specically cognitive decline. WD facies thus aid in recognition and
tracking of patients with neuro-Wilsons Disease.
1 Bhatt et al. A novel Progressive 3 Tier (P3T) scale for Wilsons
Disease. Mov Disord 2006. 21;S15:432.

Movement Disorders, Vol. 22, Suppl. 16, 2007

GENE AND CELL-BASED THERAPIES

404
Normalization of 6-hydroxydopamine-induced rotational behavior
by transplantation of dermal broblasts
J.P.M. Finberg, Y. Feld, Z. Gluzman, S. Marom, O. Mohsen,
M. Reshef (Haifa, Israel)
Objective: To assess the ability of dermal broblasts transplanted to
the GPi and SNr to modulate rotational behavior following unilateral
6-hydroxydopamine (6-OHDA) lesion in rats over a period of 6
months.
Background: Motor dysfunction in Parkinsons disease results
from decreased striatal dopaminergic input, leading to increased
neuronal activity in striatal output pathways, primarily GPi, SNr and
STN. In the attempt to develop alternative therapies to systemic
drug administration, alleviation of PD-associated motor symptoms
has been shown by ablation, high frequency stimulation, and local
drug implantation to the hyperactive area. As an alternative strategy,
we are developing autologous broblast transplantation in these
areas of neuronal hyperactivity.
Methods: Primary cultures of rat cortical neurons were prepared and
plated on multi-electrode arrays. Mature broblasts were added to the
cultures and electrical recording was taken 3-4 days later. Unilateral
6-OHDA lesion in medial forebrain bundle was carried out in Fischer rats.
Six rats were transplanted with 200,000 syngeneic dermal broblasts at
both GPi and SNr and 4 rats were sham-operated. Apomorphine-induced
rotational behavior (0.25 mg/kg) was evaluated before transplantation
(baseline) and 2, 4, 8, and 26 weeks following transplantation.
Results: Discharge rate of cortical neurons cultured on multi-electrode
array was reduced to 325 % control at 3 days following broblasts
seeding. In the unilateral 6-OHDA lesion rat model, apomorphine-induced
rotational behavior was reduced to 229 , 2812 and 3011 % control
at 2 , 4 and 8 weeks respectively following transplantation (n 6, P0.01
at each time point; Fig 1), and to 247% at 26 weeks (P0.01, n4). In
histopathological analysis performed 72 days following transplantation the
transplanted cells were located at the site of transplantation without signs
of signicant inammation.
Conclusions: The results of this study indicate that broblasts can
be used to modify neuronal electrical activity. We suggest that this
technology can serve as a broad platform for the treatment of
various neurological disorders in which local areas of inappropriately high neuronal activity exist, and specically for Parkinsons
disease.

FIG. 1 (404).

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

405
Preliminary results of an open-label, dose-escalation, safety study
of AADC gene transfer therapy for Parkinsons disease
M.J. Aminoff, C.W. Christine, K. Bankiewicz, P.A. Starr, P. Larson,
R. Mah, J.L. Eberling, W.J. Jagust (San Francisco, California, USA)
Objective: To assess the safety and tolerability of a gene therapy
given by infusion into the putamen in patients with Parkinsons disease
(PD) to improve levodopa responsiveness.
Background: As PD advances, increasing loss of the enzyme aromatic L-amino acid decarboxylase (AADC) leads to a declining response to levodopa therapy.
Methods: We performed an open-label, dose-escalation, safety, and
tolerability study of bilateral AAV-AADC (adeno-associated-virus
vector encoding human AADC) infusion into the putamen of patients
with moderate to advanced PD. The subjects were evaluated at baseline
and then monthly for 6 months postoperatively using the UPDRS,
motor state diaries, and pre- and post-operative PET imaging with
6-[18F]uoro-L-meta-tyrosine (FMT), a tracer more specic than uorodopa for AADC activity.
Results: Four women and one man were treated with 9 1010
vector genomes of AAV-AADC in the rst-dose cohort. Mean age
was 63 years (range, 60-67) and mean duration of PD was 13 years
(9-26). Therapy was well tolerated. Two serious adverse events
occurred, unrelated to the gene therapy: one subject had an asymptomatic venous infarction, and another underwent DBS 18 months
after treatment due to incomplete benet and disease progression. At
six months after therapy, dopaminergic therapy was reduced in three
subjects and amantadine was started in two. Common adverse
effects were self-limited headache and surgical site discomfort that
resolved shortly after surgery. At six months, PET imaging showed
an average increase in FMT uptake of 30%. At baseline, the average
UPDRS-III score was 41 off and 16 on medication (total UPDRS: 69
off and 33 on). At six months the average corresponding numbers
were 27 and 11 (total, 50 and 22, respectively). The UPDRS-IV
scores showed improvement in each subject from an average of 8.6
at baseline to 4.8 at six months. Motor diaries showed an average
increase in on-time while awake from 61% at baseline to 76% at six
months.
Conclusions: Thus far, AAV-AADC gene transfer therapy appears to
be safe and well tolerated in PD. Efcacy data should be interpreted
cautiously since this is an open-label study with a small sample size.
Continued testing of this vector at higher doses is warranted.
(Supported by Genzyme Corp.)
406
Suicide-gene mediated ablation of Oct4 expressing cells for ES-cell
based cell replacement therapy in Parkinsons disease
J. Schindehuette, P.C. Baier, T. Kuhlmann, C. Trenkwalder,
W. Paulus, A. Mansouri (Goettingen, Lower Saxony, Germany)
Objective: The aim of this study was to evaluate the contribution of
remaining Oct4 expressing cells within ES cell derived grafts in tumorigenesis after transplantation into the rat model of PD and a
reduction in tumorigenesis by transplanting Oct4 negative grafts.
Background: Cell replacement might represent a therapeutical option
to treat Parkinsons disease (PD) in the future. One problem using
dopaminergic transplants derived from embryonic stem cells (ES cells)
is the risk of tumor induction.
Methods: Two different ES cell lines were used to generate dopaminergic transplants and grafted into the unilateral 6-OHDA rat model
of PD. One ES cell line (HT2) was genetically modied to express
thymidinkinase (TK) driven by the Oct4 promotor. The second ES cell
line (MPI II) was not genetically manipulated. Both cell lines were
differentiated on PA6 feeder cells for 14 days. Six days before transplantation ganciclovir was added to the medium to ablate Oct4 expressing cells. Immunocytochemical and histological analyses were performed with differentiated cells before and with grafted animals ve
weeks after transplantation.

S125

Results: After differentiation and ganciclovir treatment MPI II ES

cell derived grafts contained 19.5% of Oct4 expressing colonies
whereas in HT2 derived grafts in only 4% of the colonies Oct4
positive cells were found. Immunosuppressed animals that obtained
ES cell derived grafts generated from the MPI II cell line developed
ve weeks after transplantation teratocarcinomas in approximately
32% identied by morphological criteria and Lu5 staining. Using
grafts generated from HT2 cells reduced tumorigenesis to 10%.
Conclusions: Suicide-gene mediated ablation by Oct4 promotor
driven TK expression can reduce remaining Oct4 positive cells during
the differentiation process. Transplantation of differentiated and ganciclovir treated HT2 cells reduces tumorigenesis, but is not suitable to
prevent it. Additional strategies are needed to avoid tumor induction by
ES cell derived transplants in cell replacement therapy.
407
Transplantation of bone marrow stromal cells containing the neurturin gene in rat model of Parkinsons disease
M. Ye, X.J. Wang, Y.H. Zhang, S.D. Chen (Nanjing, China)
Objective: The experiment was to evaluate the therapeutic benet of
transplanted bone marrow stromal cells (BMSCs) transfected with a
kind of neurotrophic factor gene, neurturin (NTN) gene, in treating the
rat model of Parkinsons disease (PD).
Background: PD symptoms is the degeneration of DA neurons,
transplantation of embryonic stem cells into the substantia nigra or
striatum presents a rational treatment for PD. However, the availability of this treatment is restrained by several factors, including a
limited availability of donors and the difculty in promoting the
long-term survival of transplanted embryonic stem cells. In recent
years, research has begun to focus on gene therapy approaches to
treat PD, and indeed PD is considered one of the best candidates for
gene therapy because of its clear pathogenesis and focused alterations in DA levels.
Methods: Gene engineering BMSCs by the adenoviral vector with
NTN gene were intrastriatal transplanted into PD rat models.BMSCs
with NTN gene, BMSCs with mock gene and physiological saline
group were injected into right striatum of PD rat respectively. Brain
frozen sections were carried out immunouorescence double staining after one month of BMSCs transplantation, meanwhile mmunohistochemical staining of the TH-immunoreactive(TH-IR) neurons
and counting the number of TH-IR were done from the rst month
to the six month after transplantation. NTN mRNA and protein
expression was detected by in situ Hybridization and Western blot.
Results: BMSCs with transferred NTN gene had better results of the
treatment on PD model than that of BMSCs without NTN gene during
the rst three months. BMSCs with transferred NTN gene and BMSCs
with mock vector had better effect than that of physiological saline
gruop during the six months. Immunouorescence double staining cells
of GFP/NSE, GFP/GFAP and GFP/NTN were found in the striatum of
PD rat after one month of BMSCs transplantation. There was no
signicant difference among the percent of survival TH-IR neurons in
the three groups.
Conclusions: BMSCs with transferred NTN gene had better cure
effect than that of BMSCs without NTN gene and physiological saline
gruop.

GENETICS
408
Low prevalence of PANK2 mutations in Brazilian cases of neurodegeneration with brain iron accumulation
S. Camargos, F. Cardoso, J.G. Giannetti, A.L. Teixeira, Jr,
D.P. Maia, M. Cunninham, A.J. Lees, J. Hardy, A. Singleton (Belo
Horizonte, Minas Gerais, Brazil)
Objective: Phenotypic, genotypic, image studies and prevalence of
pantothenate kinase degeneration in a movement disorder reference
center.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S126 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: Mutations in PANK2, encoding pantothenate kinase 2
are a cause of neurodegeneration with brain iron accumulation (NBIA),
formerly called Hallervorden Spatz disease. The symptoms include
dystonia, parkinsonism, mental retardation and the neuroradiologic
hallmark eye of tiger sign characterized by bilateral areas of hyperintensity within a region of hypointensity in the median globus pallidus
(T2-weighted).
Methods: We identied consecutive patients with early onset generalized dystonia and basal ganglia changes identied by imaging,
during the year 2005 in the UFMG Movement Disorders Clinic. We
performed a detailed family history assessment, neurological exam and
the application of the Fahn Marsden Scale. DNA was extracted from
peripheral lymphocytes according to routine procedures. Amplication
of the coding exons of PANK2 by polymerase chain reaction was
performed as previously described, followed by sequencing. Patients
were recruited with their prior consent and ethical committee approval.
Results: We examined 576 patients of whom 195 had a diagnosis of
dystonia. From the dystonia positive group, 14% had early onset
(14.87.7) and 6 exhibited image changes in the basal ganglia compatible with those seen in NBIA. The age at onset was 11.2 4.6 years
in these patients. Sequence analysis revealed that only a single patient
of these six had a PANK2 gene mutation. It was a homozygous
mutation (N294I), in a patient, born of a consanguineous marriage,
presenting at the age of 16 with pure parkinsonism and a relatively slow
progression.
Conclusions: Previous studies identied PANK2 mutations in the
majority of typical and one third of atypical cases. In this series there
were no mutations in patients without the eye of the tiger sign. In line
with this nding, we identied the N294I mutation in one patient with
typical eye of tiger sign on MRI. In contrast, we couldnt nd any
mutations in one patient with the typical sign and in four with pallidal
sign abnormalities. PANK2 mutations are rare within our series including typical patients with eye of tiger sign.
409
New insights into SNCA duplication in a French Parkinsons disease
pedigree. Relevance for genetic and phenotypic evaluations
E. Le Rhun, L. Larvor, K. Dujardin, A. Emptaz, E. Mutez,
V. Mouroux, J. Andrieux, F. Lepretre, M. Steinling, L. Defebvre,
A. Destee, M.-C. Chartier-Harlin (Lille, France)
Objective: To pinpoint relationships between genotype and clinical
phenotype in the P59 family presenting with a duplication of the
-synuclein gene locus (SNCA) responsible for autosomal dominant
forms of Parkinsons disease.
Background: Multiplications of the SNCA locus identied in several
families revealed a correlation between the SNCA copy number and the
severity of clinical symptoms. Inclusions of MMRN1 in the multiplicated locus might contribute to the observed cognitive decits in some
patients.
Methods: The presence and the size of the duplication were determined by FISH and real-time quantitative PCR on light-cycler and
conrmed by an analysis on 244K CGH Genome microarrays. SNCA
mRNA levels in lymphocytes were determined by real-time quantitative PCR. Ingenuity Pathways Knowledge Base analysis was performed. Eleven subjects had clinical assessments, 7 of them also
underwent a detailed neuropsychological evaluation and cerebral imaging using DAT-Scan.
Results: A duplication around 5Mb was found in 4 patients. This
duplication was anked by long interspersed repeat sequences (LINE
1) suggesting a non homologous recombination between LINE 1 elements. This region contained at least 30 known or putative genes. Ten
of them including SNCA and MMRN1 might be biologically connected.
Four subjects presented cognitive impairments principally disturbed
executive functions and working memory. However, 2 of them did not
carry the mutation. Among duplicated patients, abnormalities of tracer
xation in DAT-Scan and increased SNCA mRNA expression were
correlated with the severity of clinical symptoms.
Conclusions: Correspondence between the severity of cognitive deficits and gene dosage of MMRN1 has been evidenced in this family.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Furthermore, DAT-Scan combined with genetic analysis revealed its

utility to identify subtle abnormalities early on the preclinical stage.
ELR and LL equally contributed to this work.
410
Glucocerebrosidase mutations in a patients with sporadic Parkinsons disease from Taiwan
S.G. Ziegler, U. Gutti, M.J. Eblan, K. Hruska, O. Goker-Alpan,
E. Sidransky (Bethesda, Maryland, USA)
Objective: To determine whether mutations in the gene for glucocerebrosidase are a risk factor in sporadic Parkinsons disease.
Background: An association between glucocerebrosidase, the enzyme decient in Gaucher disease, and the synucleinopathies has been
suggested both by the development of parkinsonism in Gaucher probands and carriers, as well as by the presence of mutations in the gene
for glucocerebrosidase (GBA) in different series of subjects with
synucleinopathies. GBA mutations have been identied in several
Ashkenazi Jewish and Caucasian cohorts with parkinsonism. In this
study, an open access Parkinson repository was used to establish the
incidence of GBA alterations in a different ethnic cohort with sporadic
Parkinson disease (PD).
Methods: The glucocerebrosidase gene was sequenced in samples collected from 92 Chinese Parkinson disease patients from Taiwan along with
92 clinically screened controls, matched for age and ethnicity.
Results: The frequency of GBA mutations among the Chinese PD
probands was 4.3%, in contrast to 1.1% in Chinese controls. Mutant
alleles identied included two known mutations, L444P and D409H,
and two novel mutations, L174P and Q497R.
Conclusions: These results, ascertained in a Taiwanese cohort in a
standardized and clinically rigorous open access Parkinson disease
repository and screened by direct sequencing of GBA, demonstrate that
GBA mutations are also encountered in Chinese subjects with sporadic
PD at a higher frequency than many other known PD genes. The study
demonstrates that the association of GBA mutations with the development of parkinsonian pathology is not related to ethnic origin.
411
High prevalence of LRRK2 mutations in familial and sporadic
Parkinsons disease in Portugal
J.J. Ferreira, L. Correia Guedes, M.M. Rosa, M. Coelho,
M. van Doeselaar, D. Schweiger, A. Di Fonzo, B.A. Oostra,
C. Sampaio, V. Bonifati (Lisbon, Portugal)
Objective: To study the frequency and associated phenotype of four
recurrent LRRK2 mutations (R1441C, R1441G, R1441H, and G2019S)
in familial and sporadic Portuguese PD patients and controls.
Background: Mutations in the Leucine-Rich Repeat Kinase 2 (LRRK2)
gene are the most frequent known cause of Parkinsons disease (PD), but
their prevalence varies markedly between populations.
Methods: Patients were consecutively recruited from the Movement
Disorders outpatient clinic of the Lisbons University Hospital. Controls
were unrelated subjects from the surroundings of cases. PD was diagnosed
according to accepted criteria and familial PD was dened as patients who
reported other PD cases among their rst- or second-degree relatives.
Patients and controls were interviewed and examined by a specialist in
movement disorders. Genomic DNA was isolated from peripheral blood
using standard protocols.
Results: Among the 138 recruited unrelated PD probands, we
identied nine heterozygous G2019S carriers (6.52%) and one heterozygous R1441H carrier (0.72%). The G2019S mutation was
present in four of the 107 sporadic (3.74%) and in ve of the 31
familial probands (16.1%). Mutations were not found among the
101 recruited controls. The G2019S mutation was present on a
single haplotype and displayed reduced penetrance. Heterozygous
parkin gene mutations were also found in two G2019S-positive
probands. The clinical phenotype in patients with LRRK2 mutations
was indistinguishable from that of typical PD, including impaired
sense of smell.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Conclusions: The G2019S mutation is a very common genetic determinant among Portuguese PD patients, and the R1441H mutation is
also present in this population. These data have important implications
for the diagnostic work-up and genetic counselling of PD patients in
Portugal.
412
Spastic paraplegia 5: Locus renement, candidate gene analysis
and clinical description
S. Klebe, A. Durr, N. Bouslam, D. Grid, C. Paternotte, C. Depienne,
S. Hanein, A. Bouhouche, N. Elleuch, H. Azzedine, S. Poea-Guyon,
S. Forlani, E. Denis, C. Charon, J. Hazan, A. Brice, G. Stevanin
(Paris, France)
Objective: Renement and description of the SPG5 locus and the
associated phenotype.
Background: Thirty-three different loci for hereditary spastic
paraplegias (HSP) have been mapped, and 14 responsible genes
have been identied. Approximately 40% of dominant cases are
linked to two major loci (SPG3A and SPG4). Less is known about
ARHSP, since the mapped loci have been identied in few, often
single, families and account for only few percent of patients. ARHSP is usually associated with clinically complex phenotypes, but
SPG5, SPG24 and SPG28 are considered to be pure forms of the
disease. Ten families have already been mapped to the SPG5 locus
on chromosome 8q12. Recently, the region was narrowed to an 11
centimorgan (cM) interval.
Methods: We performed a genome-wide scan in a large French
family. Fine mapping of the rened SPG5 region on chromosome 8q12
was performed in another 17 ARHSP families with additional microsatellite markers.
Results: After exclusion of known ARHSP loci the genome-wide
screen provided evidence of linkage with a maximal multipoint lod
score of 2.6 in the D8S1113-D8S1699 interval. This interval partially
overlapped SPG5 and reduced it to a 5.9 megabase (Mb)-region between D8S1113 and D8S544. In a family of Algerian origin from a
series of 17 other ARHSP kindreds, linkage to the SPG5 locus was
supported by a multipoint lod score of 2.3. The direct sequencing of the
coding exons of seven candidate genes did not detect mutations/
polymorphisms in the index cases of both linked families. The phenotype of the two SPG5-linked families consisted of spastic paraparesis
associated with deep sensory loss. In several patients with long disease
durations, there were also mild cerebellar signs.
Conclusions: The frequency of SPG5 was 10% (2/18) in our series
of ARHSP families with pure or complex forms. We have rened the
SPG5 locus to a 3.8 cM interval and extended the phenotype of this
form of ARHSP to include slight cerebellar signs.
413
Case-control analysis of glucocerebrosidase gene mutations in Parkinsons disease and dementia with Lewy bodies
I.F. Mata, S.H. Schneer, A. Samii, J.W. Roberts, A.F. Grifth,
B.C. Leis, J.B. Leverenz, G.D. Schellenberg, E. Sidransky,
D.W. Tsuang, C.P. Zabetian (Seattle, Washington, USA)
Objective: To further elucidate the role of Glucocerebrosidase (GBA)
gene mutations as risk factors for Parkinsons disease (PD) and dementia with Lewy bodies (DLB).
Background: Gaucher disease is an autosomal recessive lysosomal
storage disorder that results from mutations in the glucocerebrosidase
(GBA) gene. Though over 200 pathogenic mutations have been described, two of these (N370S and L444P) account for approximately
70% of the disease alleles among affected individuals of (non-Jewish)
European ancestry. Clinical observations indicated that parkinsonism
might be more prevalent in patients with Gaucher disease. This
prompted case-control association studies of GBA mutations in PD and
DLB which have yielded conicting results.
Methods: We have screened a total of 661 PD patients, 59 DLB
patients, and 555 controls of European origin for N370S, L444P, and a

S127

subset of recombinant mutations (Rec 1) by TaqMan assay and resequencing.

Results: The frequency distribution of N370S, L444P, and Rec 1 in
the sample is displayed in table 1. There was a signicant excess of
mutation carriers in both the PD (odds ratio [OR], 7.7; 95% condence
interval [CI], 1.8-33.5; p0.001 [Fishers Exact Test]) and DLB
groups (OR, 14.8; CI, 2.4-90.6; p0.007). We also detected two new
variants of unknown signicance by sequencing in two PD patients
(T410T and H422T). The clinical characteristics of the PD patients who
were mutation carriers versus non-carriers appeared similar.
Conclusions: Our results suggest that while GBA mutations might
alter susceptibility to PD and DLB, the population attributable risk is
likely no more than 1%. Our data also indicate that N370S and L444P
are much less frequent in DLB than previously reported. Analyses of
larger samples will be required to denitively assess the role of GBA
mutations as risk factors in PD and DLB.

Number of GBA mutation carriers and frequencies

N370S
L444P
Rec1
Total

PD Cases (n661)

DLB cases (n59)

controls (n555)

11 (1.7%)
6 (0.9%)
1 (0.15%)
18 (2.7%)

1 (1.7%)
1 (1.7%)
1 (1.7%)
3 (5%)

1 (0.2%)
2 (0.36%)
0
3 (0.5%)

414
LRRK2 mutations in patients with Parkinsons disease in southern
Spain
P. Mir, L. Gao, F. Diaz, F. Carrillo, M. Carballo, A. Palomino,
J. Diaz-Martin, R. Mejias, P.J. Vime, E. Pintado, M. Lucas,
J. Lopez-Barneo (Seville, Spain)
Objective: To determine the frequency of leucine-rich repeat kinase
2 (LRRK2) G2019S and R1441C/G/H mutations in patients with
Parkinsons disease (PD) in southern Spain.
Background: PD is the second most common neurodegenerative
disease. Although in most cases the mechanism of neuronal death of
PD is sporadic, a small portion of PD is familiar. Recently, LRRK2
gene has been linked to autosomal-dominant, late-onset PD. A common
missense mutation (G2019S) in exon 41 of LRRK2 gene accounts for
about 5-6% of familiar PD and 1-2% of sporadic PD in a number of
European populations and the frequency seems to be dependent on the
ethnic group. In addition, another missense mutation in exon 31
(R1441G) was originally found in Basque Country, a region of north
Spain.
Methods: We included 151 PD patients (63.7 10.7 years old, 89
male and 62 female, including 133 sporadic and 18 familiar PD
patients) and 144 control subjects (62.7 10.2 years old, 64 male and
80 female) from a region in southern Spain. The mean age at onset of
the disease was 55.7 12.4 years, and the mean duration time was
8.0 6.6 years. G2019S and R1441C/G/H mutations were screened
with restriction digestion and conrmed by direct sequencing when a
mutation was found.
Results: One familiar PD patient (5.6 %) was found to contain
G2019S mutation. Four sporadic PD patients were found to contain
mutations (2 G2019S 1.5%; 2 R1441C/G/H 1.5%). The mean age at
onset of the disease in the PD patients with mutations was 50.2 13.1
years, and the mean duration time was 7.8 2.6 years. In addition, one
young female control subject (0.7%, 43 years old) was found to be
positive to G2019S substitution.
Conclusions: G2019S and R1441C/G/H are common LRRK2 mutations in PD patients in southern Spain with a frequency similar to
previous studies.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S128 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

415
DJ-1 protects against dopamine toxicity by increasing its vesicular
sequestration: Implications for Parkinsons disease
N. Lev, N. Pilosof, H. Cohen, D. Offen, E. Melamed (Petah-Tikva,
Israel)
Objective: To examine whether the protective effect of DJ-1 is
mediated through its effect on VMAT2 enabling more efcient clearance of dopamine from the cytoplasm.
Background: Mutations in DJ-1 lead to early onset autosomal recessive Parkinsons disease (PD). Recently, changes in DJ-1 protein have
been demonstrated in patients with sporadic PD. DJ-1 is protective
against oxidative stress, but the mechanism of action is unknown. The
metabolism of dopamine induces formation of toxic reactive oxygen
species (ROS) and their accumulation is implicated in the special
sensitivity of midbrain dopaminergic neurons to oxidative stress and
degeneration in PD. One of the most powerful strategies used by
dopaminergic neurons to protect themselves against dopamine toxicity
is its concentration within intracytoplasmic vesicles by vesicular monoamine transporter 2 (VMAT2).
Methods: Human neuroblastoma SH-SY5Y cells were stably transfected with plasmids inducing overexpression of DJ-1 or inhibiting
DJ-1 expression through SiRNA for DJ-1. Cells were exposed to
dopamine and to the antioxidant N-acetyl cysteine. Protein and mRNA
levels were quantied by Western blot and real-time PCR, respectively.
Viability and intracellular ROS accumulation were monitored by the
MTT and DCF assays. VMAT2 activity was evaluated using radioactive dopamine as a substrate. Chromatin immunopercipitation was used
to demonstrate that DJ-1 is attached to VMAT2 promotor region.
Results: Dopamine-exposure led to rapid upregulation of DJ-1, followed by upregulation of VMAT2. DJ-1 activated VMAT2 promotor at
the genomic level. Overexpression of DJ-1 increased cell resistance to
dopamine toxicity, reduced intracellular reactive oxygen species, and
markedly increased VMAT2 expression and function. Contrary effects
were achieved when DJ-1 levels were reduced by siRNA.
Conclusions: This is the rst report that DJ-1 controls the sequestration of dopamine into the synaptic vesicles by upregulating VMAT2.
These observations suggest a novel mechanism in which ROS, generated by increased cytoplasmic dopamine, lead to rapid upregulation of
DJ-1, which in turn protectively augment the sequestration of dopamine
into the synaptic vesicles through upregulation of VMAT2. This mechanism may explain how mutations in DJ-1 prompt PD.
416
Association of MAPT haplotype-tagging SNPs with Parkinsons disease
J. Vandrovcova, A.M. Pittman, E. Malzer, N.W. Wood, A.J. Lees,
R. de Silva (London, United Kingdom)
Objective: To investigate whether genetic variation within MAPT
contributes to Parkinsons disease predisposition.
Background: MAPT, the gene encoding for microtubule associated
protein tau, has been found mutated in several families with frontotemporal dementia with parkinsonism linked to chromosome 17. In
addition, the MAPT H1 clade specic subhaplotype (H1c) has been
strongly associated with tauopathies, progressive supranuclear palsy,
corticobasal dementia, and Alzheimer disease. In Parkinsons disease
(PD), which is clinically classied as an alpha-synucleinopathy, the
association between MAPT H1 clade has also been examined, but with
rather conicting results.
Methods: To investigate the importance of genetic variation in
MAPT in patients with PD, a large case-control study was performed in
588 clinically diagnosed and/or pathologically conrmed PD cases and
480 controls from UK. Six MAPT haplotype tagging SNPs were genotyped and both single locus and haplotype frequencies were compared.
Results: In the single locus association analysis, the H1 haplotype,
dened by the intron 9 insertion/deletion, was associated with an
increased risk of PD (p0.032; odds ratio (OR) 1.265, 95% condence
interval (CI) 1.020-1.569). A moderate association was also observed
for the SNP rs2471738 (p0.032; OR 1.271, 95% CI 1.020-1.584). In

Movement Disorders, Vol. 22, Suppl. 16, 2007

the htSNP haplotype association analysis, the sole H2-derived haplotype was underrepresented in all of the PD cases compared to controls
(p0.03; OR 0.769, 95% CI 0.594-0.995), conrming the results from
the single SNP analysis. There was no signicant difference in the
distribution of any of the frequent H1-clade specic haplotypes between cases and controls. Among the rarer haplotypes (frequency less
than 2%), there was an increased risk of PD for the H1L haplotype
(p0.03; OR 2.619, 95% CI 1.064-6.446).
Conclusions: Our study supports the hypothesis that genetic variability in the MAPT gene confers a susceptibility to Parkinson disease.
However, the effect is not strong and compared to tauopathies the H1C
specic haplotype is not involved.
417
Screening for POLG1 mutations in a Southern Italian ataxia population
C. Criscuolo, P. Mancini, S. Ammendola, D. Cicale, G. De Michele,
A. Filla (Naples, Italy)
Objective: To evaluate the prevalence of W748S and A467T mutations in a Southern Italian ataxic population.
Background: POLG1 encodes the catalytic subunit of polymerase
gamma, which is responsible for mtDNA replication and repair.
POLG1 mutations are responsible of an extraordinary spectrum of
clinical phenotypes. Among them, W748S and A467T have been
reported to cause mitochondrial recessive ataxia (MIRAS). MIRAS is
a neurodegenerative disorder with usually early onset (range from 5 to
50 years) characterized by ataxia, epilepsy, headache, neuropathy,
ophthalmoplegia, and myoclonus.
Methods: We selected 66 index cases with ataxia and clinical (decreased or absent ankle reexes and decreased vibration sense) or
neurophysiologic signs of peripheral neuropathy from a larger series of
Italian ataxic patients with onset below 50 years. Forty-eight patients
were sporadic and 18 were index cases from families compatible with
a recessive transmission. Pathologic GAA expansions in the Friedreich
ataxia gene were excluded in all and mutations in Aptx and Setx were
not found in the 38 studied patients. After informed consent, A467T
and W748S were detected by a combination of dHPLC and direct
sequencing analysis.
Results: Mean age at onset was 2115 years (range 2-50 years). All
patients had ataxia and peripheral neuropathy, 28 had an ataxia plus
syndrome features frequent in MIRAS or in other mitochondrial diseases (myoclonus, epilepsy, hypogonadism, ophthalmoplegia). Ataxia
was associated with myoclonus in 17 (6 of them had also epilepsy),
with hypogonadism or premature menopause in 6, and with ophthalmoplegia in 5 patients. None of the 66 patients carried the studied
mutations neither in homozygous nor in heterozygous state.
Conclusions: W748S has been reported as the most common genetic
cause of ataxia in Finland with a carrier frequency in general population of 0.8%. MIRAS patients with W748S have been reported also in
Norway, United Kingdom, Belgium. Haplotype analysis revealed that
the ataxic W748S allele is of European origin, having descended from
a common founder. According to our analysis A467T and W748S are
not a frequent cause of ataxia in Southern Italy. Routine screening of
these mutations in ataxia patients of Italian patients seems to be of
limited clinical value.
418
Clinical and molecular analysis of carriers of GAG deletion in
DYT1 gene among Polish patients with primary dystonia
K. Szczaluba, J. Bal, B. Kadziolka, A. Szolna, A. Friedman,
T. Kmiec, T. Mazurczak (Warsaw, Poland)
Objective: To investigate the prevalence of GAG deletion in the
DYT1 gene in Polish patients with primary dystonia. To establish
genotype-phenotype correlation in those with the deletion conrmed at
the molecular level. To investigate presence of other changes of potential signicance within the gene in mutation carriers.
Background: One of the more frequent types of primary dystonias is
DYT1 dystonia. It follows an autosomal dominant mode of inheritance

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

with a deletion of three nucleotides GAG in exon 5 in the DYT1 gene
conrmed as a pathogenic change.
Methods: The molecular analysis for GAG deletion was carried out
in a group of 68 probands with generalized dystonia (n39) and
focal/segmental of various location (n29). Further molecular studies
were then done in probands families for presence of GAG deletion as
well as three other changes within the DYT1 gene (D216H, 567delG
and 191GT).
Results: Five probands (7%) in the analyzed group carried the GAG
deletion in the DYT1 gene. All had dystonia starting in a limb (in a leg
in 3 of 5 cases), with age of onset from 7 to 14 years. In probands
families, additional 11 carriers of the mutation, including 9 asymptomatic, were identied. Generalized, early-onset symptoms were observed
in one of symptomatic carriers. In the other, writers cramp was the
only manifestation. None of fteen mutation carriers (4 probands 11
relatives) had D216H change. A 567delG change was present only in
the mutation carrier with the writers cramp. None of the other mutation
carriers, either affected or unaffected, had this change. A substitution
191GT was present in one of the asymptomatic mutation carriers but
in none of the other carriers in this family.
Conclusions: Molecular analysis for presence of GAG deletion of
DYT1 gene is especially indicated in individuals with generalized type
dystonia of early onset in a limb with or without positive family history.
No conclusions can be drawn as to potential signicance of other
analyzed changes within the gene as they were present in only single
cases in our families. However, it is possible that 567delG change may
have weakened the clinical expression of dystonia in the mutation
carrier manifesting focal dystonia.
419
Clinical features of movement disorders in hemochromatosis
D.A. Hall, S. Ringel, K. Howard, J. Jankovic (Denver, Colorado,
USA)
Objective: To describe a series of patients with hemochromatosis
and movement disorders.
Background: Hemochromatosis has been associated with neurological symptoms to include parkinsonism, cerebellar ataxia, dementia,
gait difculties, and extrapyramidal dysfunction. Hereditary hemochromatosis (HH) is one of the most common genetic disorders in the
population and is caused by two different mutations in the HFE gene.
Some studies have shown an increased frequency of C282Y homozygote mutation carriers in individuals with idiopathic Parkinsons disease, but the signicance of this is controversial. This series describes
the clinical features of patients with hemochromatosis, HH, and movement disorders.
Methods: Seven patients with both hemochromatosis and Movement
Disorders collected from two Movement Disorders clinics are described. Five patients had testing for the C282Y and H63D mutations.
Results: The average age was 54 4.9 years. Three patients had the
C282Y mutation and one was heterozygous for the H63D mutation.
Patients had a variety of movement abnormalities, with most patients
having bradykinesia (71%) and/or tremor (71%) with action tremor
more prevalent. Other neurological signs in order of frequency include:
rigidity, gait ataxia, shufing gait, dysmetria, postural instability, dystonia, and dyskinesia. Only one patient met criteria for Parkinsons
disease. Three patients had abnormal signal on MRI in the basal
ganglia. Two patients responded to dopaminergic medications and
another to mysoline.
Conclusions: This case series suggests a potential association between hemochromatosis and Movement Disorders. Affected patients
are younger than expected for idiopathic Parkinsons disease or essential tremor, but may respond to medications used for other movement
disorders. Movement disorders associated with hemochromatosis are
postulated to be related to iron accumulation in the basal ganglia,
however, this nding has not been consistently seen in imaging studies.
Prospective epidemiological studies and genetic studies with larger
sample sizes would better clarify whether a true association exists and
could be used to delineate the clinical features in these disorders.

S129

420
Parkinsonian spectrum associated with glucocerebrosidase mutations
O. Goker-Alpan, G. Lopez, M. Hallett, E. Sidransky (Bethesda,
Maryland, USA)
Objective: To dene the neurologic spectrum among subjects with
parkinsonism carrying glucocerebrosidase mutations.
Background: Gaucher disease (GD), the inherited deciency of glucocerebrosidase, can present with a wide range of symptoms, affecting
the skeletal, hematologic and nervous systems. Clinical, pathologic and
genetic studies suggest that mutant glucocerebrosidase is associated
with a phenotype characterized by parkinsonism and progressive neurologic deterioration. Subjects have been reported with mild GD and
early-onset, L-Dopa refractory parkinsonian manifestations, often associated with cognitive decline. Moreover, GD heterozygotes have
been identied with parkinsonian syndromes.
Methods: In a prospective study, nine subjects (6M:3F), six with
mild GD and 3 GD carriers, were followed up to 36 months. Cognitive
function, oculomotor and motor decits were tested by the same team.
Olfactory evaluation was done using University of Pennsylvania Smell
Identication Test. Genotypes were conrmed by DNA sequencing.
Results: The N370S mutation was the most common GD allele.
Others included L444P, c.84insG and a recombinant allele. The mean
age of onset was 50 (40 -65) and disease duration was 7.4 years (1.2
-16). At presentation, four subjects had tremor, 5 had symptoms related
to bradykinesia and rigidity, and one had apraxia. Six were diagnosed
with classical PD, three with the akinetic-rigid type. Three subjects
were considered to have parkinson plus syndrome because of early
cognitive changes and hallucinations. All, but one were L-Dopa responsive. Other atypical manifestation included myoclonus with EEG
abnormalities, and clinical seizures. Autonomic dysfunction was observed in three male subjects. Five of 6 subjects tested had olfactory
loss. In half, cognitive changes were reported later in the disease
course, often accompanied by depression.
Conclusions: Among subjects with parkinsonism carrying glucocerebrosidase mutations there is a spectrum of parkinsonian phenotypes,
often with loss of olfaction. This ranges from classic PD, mostly the
akinetic type, to a less common phenotype characteristic of Lewy Body
Dementia.
421
Familial Parkinsonism with double mutations of Parkin and
PINK1
M. Funayama, L. Yuanzhe, H. Yoshino, T.-H. Tsoi, C.-W. Lam,
T. Obi, E. Uyama, Y. Imamichi, H. Takahsima, Y. Mizuno,
N. Hattori (Bunkyo, Tokyo, Japan)
Objective: To screen PINK1 mutations among young-onset parkinsonism with parkin mutations.
Background: Parkin and PTEN-induced putative kinase 1 (PINK1)
have been identied as causative genes for autosomal recessive parkinsonism (ARP). The similar clinical features of both groups of ARP
patients indicate that both causative gene products share a common
pathway in causing nigral degeneration. Recently, several studies using
Drosophila melanogaster models have suggested that Parkin acts downstream of PINK1 in a common pathway to maintain mitochondrial
integrity and function. Based on this concept, we proposed the presence
of a common pathway in both gene products. Here, we report the
results of mutation analyses of PINK1 in ARP patients with parkin
mutations.
Methods: We investigated PINK1 mutation in 90 patients with
parkin mutations using PCR, direct sequencing, and gene dosage analyses using TaqMan probes.
Results: We detected two sibling pairs carrying parkin homozygous
or compound heterozygous mutation as well as PINK1 heterozygous
mutation (parkin; p.T175PfsX2 and PINK1; p. R58_V59insGR, parkin;
p.C441R/exon4del and PINK1; p.R407Q). The age at onset of patients
with both mutations was more than 10 years less than those with only
the same parkin mutation.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S130 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Conclusions: Identication of patients with parkin and PINK1 mutations with clinically modied features including young age at onset,
supports the ndings of studies of parkin or PINK1 knockout Drosophila, and suggests that PINK1 and Parkin act in a common pathway in
maintaining mitochondrial function and their mutations cause nigral
degeneration of ARP.

ratio0.47, 95% CI 0.039, 4.201, p0.05). No subjects carried the

homozygous genotype.
Conclusions: We provided evidence that the LRRK2 Gly2385Arg
variant is present in non-Chinese Asians. However, its frequency
appears relatively less common compared to Chinese, and hence its role
as a genetic risk factor in non-Chinese is uncertain.

422

424

Do Ashkenazi Jew patients with Parkinsons disease want to be

acquainted about their G2019S mutation status in the LRRK2
gene?
O. Moore, C. Shifrin, S. Levin, J. Knaani, N. Giladi (Tel Aviv,
Israel)

APOe alleles in Parkinsons disease: Relation to cognitive decline

and hallucinations. A longitudinal study
M.W. Kurz, G. Dekomien, J.P. Larsen, D. Aarsland (Stavanger,
Norway)

Objective: To assess the interest and response rate of PD patients

regarding their status as carrier of the common G2019S mutation in the
LRRK2 gene.
Background: About 15% of the Ashkenazi patients with Parkinsons
disease (PD) in Israel have recently been identied as carriers of the
disease causing mutation G2019S in the LRRK2 gene. These results
based on a research program approved by the Israeli National Ethics
Committee. Initially, we did not plan to provide the results to the
participants, as was stated in the consent form. As a result of this
unexpected nding, together with the ethical committee of the Tel Aviv
Sourasky Medical Center, it was decided to provide the information
regarding the presence or absence of the G2019S mutation to those
patients who will request this information. We have sent letters to all
participants informing them in general about the new observation and
informed them about the possibility to receive the results of the
G2019S mutation analysis upon replying and signing a letter of request.
Methods: 554 letters were sent to all patients who participated in the
PD genetic study until the end of January 2007.
Results: After 2 months, 226 patients (40.8%) requested the information regarding their status as G2019S mutation carriers. We did not
observe any difference between men and women in regards to their
interest to get the genetic information. Those who requested information were somewhat younger (p0.073), and with signicantly shorter
duration of motor symptoms (p0.047).
Conclusions: The present observation is the rst one ever to provide
the actual rate of interest of PD patients to be informed about their
genetic status even when the mutation is present in a very high
frequency in Israel. Future genetic counseling programs should take
this information in account when planning to provide any counseling to
the general PD population.
423
LRRK2 Gly2385Arg in Parkinsons disease patients of non-Chinese Asian ethnicity
E.K. Tan, Y. Zhao, L. Tan (Singapore, Singapore)
Objective: To analyze the LRRK2 Gly2385Arg variant in NonChinese Parkinsons disease patients.
Background: A common LRRK2 missense variant, Gly2385Arg, has
been found to be a genetic risk factor for Parkinsons disease (PD) in
ethnic Chinese. However, the signicance of the variant in non-Chinese
Asian population is unclear.
Methods: Allelic discrimination using the 5 nuclease activity assay
was adapted to detect the G2385R variant. Primers and probes were
designed using the Primer Express 1.5 software. The TaqMan MGB
probes contain minor groove binder, nonuorescent quencher (NFQ)
and a reporter dye. The amplication reactions were carried out in an
ABI Prism 7700 Sequence Detection System (Applied Biosystems).
Sequencing was carried out to conrm positive samples.
Results: DNA samples from a total of 249 subjects comprising of
145 PD and 104 controls were analyzed. The mean age, age at onset of
PD, and age of controls was 66.0 10.7 (36-86), 60.4 11.1 (32-80),
and 59.7 14.1 (21-84) years, comprising of 63% and 53% men
respectively. About 3% of cases reported a positive family history of
PD. The frequency of the heterozygous Gly2385Arg genotype was not
signicantly different in PD compared to controls (1.4% vs 2.9%, odds

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To explore the role of Apo e alleles in the progression of

cognitive decline and hallucinations in PD.
Background: Apolipoprotein e (Apo e) is a polymorphic proteinwith
three major alleles: e2, e3, and e4, yielding six possible genotypes and
translating into three major isoforms of the protein: ApoE2, ApoE3,
and ApoE4. The e2 allele is considered protective in Alzheimer disease
(AD). The e4 allele is associated with an increased risk of developing
AD and a lower age at disease onset. Alzhemer-type changes contribute
to dementia in Parkinsons disease (PD), but evidence for the role of
APO E in PD has been inconclusive. Some studies have shown associations of the e4 allele with PD and development of dementia or
hallucinations in PD, but conicting results have been reported.
Methods: Patients were recruited from an ongoing population based
study in Rogaland, Norway. 245 patients were followed prospectively
and assessed with MMSE, Neuropsychiatric Inventory and UPDRS. A
subgroup of the patients has come to autopsy and showed brain chances
compatible with PD. Blood from 96 patients were screened and the Apo
e allele-type characterized. 56 of the patients were followed for up to
12 years and the Apo e allele type correlated with the longitudinal
clinical data.
Results: Neither e2-alleles nor e4 alleles were associated with age at
onset of PD nor the longitudinal course of cognition, hallucinations or
parkinsonism.
Conclusions: We cannot conrm an association of Apo e-alleles with
cognitive decline or hallucinations in PD.
425
Are parkin mutation carriers better candidates for deep brain
stimulation?
E. Lohmann, M.L. Welter, V. Fraix, P. Krack, S. Laine, J.-L. Hueto,
V. Mesnage, P. Pollak, A. Durr, A. Brice (Paris, France)
Objective: In order to determine the effects of high-frequency STN
stimulation in early-onset parkinsonism, we have compared the follow-up after surgery ( 12 months) of a series of 7 patients with one
parkin mutation, 7 patients with homozygous or composite heterozygous parkin mutations and 39 patients without parkin mutations.
Background: High-frequency stimulation of the subthalamic nucleus
(STN) is increasingly accepted as an adjunct therapy in Parkinson
disease (Kleiner-Fisman et al. 2006). The usual clinical features of
carriers of parkin mutations are early onset typical parkinsonism with
a slow clinical course, excellent response to low doses of levodopa with
frequent treatment-induced dyskinesias and the absence of dementia.
Neuropathological examination of parkin cases showed severe generalized loss of dopaminergic neurons in the substantia nigra pars compacta (Mori et al. 1998).
Methods: Clinical presentation was evaluated with the UPDRS (I to
VI), neuropsychological evaluations were made with the Mini Mental
State Examination, Mattis dementia rating scale and frontal scores.
Results: Clinical presentation, neuropsychological evaluation and
daily dose of anti-parkinsonian treatment at time of surgery were
similar in all patients, despite the signicantly lower age at onset of
patients with two parkin mutations (26.4years9.6 vs 38years9.2,
p0.05). At least 12 months after neurosurgery, there were no differences in UPDRS scores or neuropsychological states between the two
groups of patients. Nevertheless, the doses of daily equivalent dopa

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

were signicantly lower in parkin mutation carriers in comparison to
non carriers (193mg108 vs 534mg439, p0.05).
Conclusions: These results indicate that carriers of parkin mutations
are very good candidates for STN stimulation and surgery may produce
more durable results.
426
Narrowing down a dystonia gene in a family with translocation
t(7;18): New locus or independent dystonia occurrence?
K. Szczaluba, J. Pietrzak, J. Noskowska, A. Friedman, E. Bocian
(Warsaw, Poland)
Objective: To determine the etiology of dystonia in a familial case.
Background: Dystonia is a rare feature of chromosome 18p deletion
(also known as de Grouchy syndrome) with DYT7 locus originally mapped
to this region by genomewide linkage analysis. However, dystonia has not
been described yet in literature in an apparently balanced translocation
carrier involving chromosome 18p. We present a 21-year old female
proband with mental retardation and, rather unusual for this syndrome,
generalized dystonia. The mother and the sister of her grandmother also
have dystonia. In the proband and her mother a chromosomal aberration
involving chromosome 18p was detected.
Methods: Chromosome analysis using GTG banding and uorescence in situ hybridization (FISH) with probes for chromosome 18p
were performed on peripheral blood lymphocytes from the proband and
her mother.
Results: G-banding analysis of the probands chromosomes revealed
a deletion of the whole short arm of chromosome 18. The abnormal
chromosome is a product of the balanced translocation t(7;18)(p22.3;
p11.1) present in the mother. FISH with BAC clones mapped the
chromosome 18p breakpoint to the pericentromeric region 18p11.21,
proximal to BAC RP11-411B10.
Conclusions: Presence of generalized dystonia in both the proband
and her mother points to common etiology of this phenotypical trait.
Although the feature may occur independently from the detected chromosome abnormality in this family, it may also suggest presence of a
new dystonia locus in the pericentromeric region of chromosome 18p.
427
A detailed clinical study of early-onset Parkinson patients
E. Lohmann, S. Thobois, S. Laine, S. Tezenas, E. Broussolle,
P. Pollak, L. Mallet, B. Dubois, Y. Agid, A. Brice (Paris, France)
Objective: In order to establish pheno-genotype correlation in earlyonset Parkinsonism, we have compared in a detailed clinical study a
series of 21 patients with and 23 patients without Parkin mutations.
Background: PARK2 gene mutations were rst identied in autosomal
recessive juvenile-onset Parkinsonism (Kitada et al. 1998). The usual
clinical features are described as early onset typical parkinsonism with
slow clinical course, excellent response to low doses of levodopa with
frequent treatment-induced dyskinesia and the absence of dementia. We
expected to nd more severe cognitive impairment and/or a particular
comportemental and/or psychiatric pattern in Parkin mutation carriers
because neuropathological studies of Parkin patients have shown severe
generalized loss of dopaminergic neurons in the substantia nigra pars
compacta (Takahashi et al. 1994) that would greatly decrease dopaminergic efferents to the sensory-motor as well as to the limbic system.
Methods: Clinical presentation was evaluated by using UPDRS (I to
VI) in on and off state. Neuropsychological investigations were
accomplished by Mattis dementia rating scale, Mini Mental State
Examination, battery of executive tests (GREFEX), Modied Wisconsin Card Sorting Test, Grober and Buschke test and frontal scores.
Psychiatric examination used Montgomery-Asberg Depression Rating
Scale, Comprehensive Psychopathological Rating Scale and standardized Mini International Neuropsychiatric Interview.
Results: Clinical, neuropsychological and psychiatric evaluations were
similar in all patients, except for the signicant lower daily dose of
antiparkinsonian treatment in Parkin patients after similar disease durations, that caused a signicant more important delay in the development of
levodopa related dyskinesia. No further signicant differences were found.

S131

Conclusions: These results indicate that carriers of Parkin mutations

are clinically indistinguishable from other young onset PD patients.
Severe generalized loss of dopaminergic neurons in the substantia nigra
pars compacta in these patients is associated with an excellent response
to low doses of dopa-equivalent, but cognitive impairment and/or
special comportemental and/or psychiatric symptoms were not more
severe than in other patients.
428
Investigation of the molecular aetiology of South African patients
diagnosed with Parkinsons disease
J.A. Carr, S. Bardien (Tygerberg, South Africa)
Objective: To determine the role of genetics in the etiology of
Parkinsons disease (PD) in South African patients of Afrikaner, Xhosa
(South African black) and mixed ancestry patients.
Background: A wide variety of mutations that include amino acid
substitutions and exon rearrangements have been identied worldwide
in ve genes that cause autosomal dominant and recessive forms of PD.
The aim of this study was to investigate the molecular aetiology of PD
in a cohort of South African patients.
Methods: After obtaining informed consent, a total of 30 patients of
different ethnic backgrounds were recruited from the movement disorders clinic at Tygerberg Hospital. The genomic DNA of these patients
was screened for mutations in the PARK2 and DJ-1 genes using single
stranded conformation polymorphism (SSCP) analysis and direct sequencing. Moreover, exonic deletions and insertions in the PARK2
gene, which could be missed with the SSCP method, were analyzed
using a real-time quantitative PCR technique on a Lightcycler system.
In addition, the presence of the common G2019S mutation in the
LRRK2 gene was also investigated in our cohort using the PCRrestriction fragment length polymorphism technique.
Results: The mean age of the group was 45 years. The study sample
incuded patients from South African black (Xhosa), mixed ancestry and
Afrikaner populations. In the PARK2 gene, 4 mutations were detected.
There were two homozygous exon deletions: exons 3 and 4 in one
proband and exon 4 in two affected siblings. In addition, two missense
mutations were identied: R402C and E310D that were each detected
in one proband but were not observed in controls. In the DJ-1 gene, two
novel variants were detected: a 16-bp deletion over the transcription
start site in one proband and an A56T substitution in one South African
Black patient, which was not observed in controls. A single patient had
the LRRK2 G2019S mutation.
Conclusions: It is apparent that mutations in the PARK2 and DJ-1
genes are not a major cause of early onset PD in the South African
population. Similarly, the LRRK2 mutation appears to be infrequent in
this population.
429
Is the G2019S LRRK2 mutation common in all southern European
populations?
S. Papapetropoulos, N. Adi, L. Shehadeh, N. Bishopric, C. Singer,
A.A. Argyriou, E. Chroni (Miami, Florida, USA)
Objective: To determine the relative frequency of common LeucineRich Repeat Kinase 2 (LRRK2) mutations in a sample of familial and
sporadic Parkinsons disease (PD) patients from the region of southwestern Greece, a region where the majority of alpha-synuclein G201A
positive patients have been reported.
Background: Mutations in the LRRK2 gene (PARK8) are associated
with idiopathic PD. The most frequently reported mutation, G2019S
located in exon 41 of LRRK2, has been associated with both familial
and sporadic PD in North American, European, North African, and
Middle Eastern populations, with a markedly increased frequency in
North African Arabs (39%) and Ashkenazi Jews (18.3%). Due to the
geographic and historic vicinity of Greece with areas of high prevalence of LRRK2 mutations we studied the frequency of the G2019S
mutation in a well-characterized cohort of familial and sporadic PD
patients of Greek origin from mainland Greece.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S132 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: Our sample was comprised of sporadic and familial PD
subjects cases. All patients were of Greek origin. In our cohort there
were 6 cases with G201A alpha-synuclein mutation and 3 patients with
deletions in the parkin gene. DNA was obtained from blood for all
subjects, using standard protocols. DNA was genotyped for the
G2019S, R1441C mutations and the G2385R Asian polymorphism
using ABI by-design probes.
Results: We studied a total of 134 PD patients (mean age at onset
61.012.6 years and mean disease duration 6.76.1 years). Sixty-nine
(51.5%) were male and 65 (48.5%) were female. Forty-ve patients
(33.6%) had family history of PD and 89 (66.4%) were sporadic. None
of our patients carried LRRK2 disease-causing mutations (G2019S,
R1441C) or the disease-associated Asian LRRK2 polymorphism
(G2385A).
Conclusions: Very low prevalence of the LRRK2 G2019S mutations
has been reported in other southern European populations. LRRK2
mutations appear to be limited in certain populations and different
ancestry and founder effects may explain the reported variability.
Accurate estimations of frequencies and penetrance of different
LRRK2 mutations are essential for correct and cost-efcient use of
genetic testing and proper genetic counseling of PD patients.

430
Analysis of glucocerebrocidase mutations in Parkinsons disease
G.R. Clark, P.F. Chinnery, D.J. Burn, C.M. Morris (Newcastle upon
Tyne, Tyne & Wear, United Kingdom)
Objective: To investigate the possible association of glucocerebrocidase (GBA) gene mutations and an increased risk of PD.
Background: Parkinsons disease (PD) is the second most common
neurodegenerative disease after Alzheimers disease, and affects approximately 1% of the over 50 population.In recent years, mutations in
-synuclein, Parkin, DJ-1, PINK1 and UCH-L1 have been associated
with familial forms of PD, and the LRRK2 G2019S mutation has been
associated with both familial and idiopathic PD. More recently, associations with glucocerebrosidase deciency and Parkinsonism in Gaucher disease (GD) patients have been demonstrated. GD is the most
common lysosomal storage disorder, which results from deciencies in
the lysosomal enzyme glucocerebrosidase. Recent studies have supported the hypothesis that PD and GD share clinical and neuropathological phenotypes, whilst others have shown strong genetic associations. These ndings have prompted researchers to examine GBA gene
sequence alterations in individuals diagnosed with PD. Previous research has shown that approximately 150 distinct GBA mutations were
associated with GD, of these mutations, 5 were found to account for
70-98% of all GD cases. Several of these common mutations, along
with various novel mutations, have been found in idiopathic PD cases.
The studies to date, have shown that GBA mutations are present in
between 4-21% of PD subjects, which suggests a possible increased
susceptibility to PD in GBA mutation carriers.
Methods: In order to test this potential increased risk, we directly
sequenced all 11 exons of GBA, in order to identify both common and
novel mutations. In total, 100 dementia with Lewy bodies (DLB)/
Parkinsons disease dementia (PDD) subjects, 130 idiopathic PD subjects and 100 controls were screened for mutations.
Results: Our results show the presence of both the common GBA
mutations, and novel mutations. We found an increased incidence in
the L444P mutation in the PDD/DLB cohort.
Conclusions: Our initial results appear to support those found previously by others, GBA mutations are associated with the incidence of
PD. This suggests that variations in the GBA gene may confer a rare
susceptibility to PD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

431
Obstructive sleep apnea and searching for a genetic cause looking
for inuences by polymorphisms of endothelin receptor subtype-a
D. Buck, K. Diefenbach, I. Fietze, T. Penzel, U. Malzahn, I. Roots
(Berlin, Germany)
Objective: To detect genetic inuences of endothelin-receptor subtype-a polymorphisms in obstructive sleep apnea (OSA).
Background: OSA with its characteristic breathing pauses by obstruction of the upper airway and the following arousal results in sleep
fragmentation, non-recreative sleep and excessive daytime sleepiness.
Typical traits are male gender, obesity, craniofacial abnormalities,
accompanying diseases (e.g.cardiovascular domain) and elevated intrafamilial occurrence of OSA. By searching for a genetic cause of OSA
we focused on polymorphisms of the endothelin receptor subtype-a as
a part of the endothelin system. Interactions between endothelin system
and characteristic traits for OSA were the reason for this line of vision.
Methods: We recruited 378 patients referred to our sleep laboratory,
older than 18 years, of caucasian origin and with polysomnographically
diagnosed OSA (AHI5, central apneas 10%). Genotyping of the
four EDNRA-polymorphisms E335E, H323H, G-231A and C70G
was performed by standard methods.
Results: The supposition of inuences in severity of OSA by the
EDNRA-polymorphisms could not be approved. Multiple linear regression revealed a main effect of BMI on AHI as well as a signicant
interaction term for BMI with gender. Thus, an increase in BMI is
associated with an increased AHI and this AHI increase is more
pronounced in males than in females.
Conclusions: We determined a substantial effect of BMI, modied
by gender, on OSA severity: a well-known nding. Further effort will
be made to elucidate genetic determinants of susceptibility to OSA
especially with regard to the endothelin-system. Because of excessive
daytime sleepiness OSA is an important differential diagnosis for
restless legs syndrome (RLS) and periodic limb movement (PLM). As
for OSA, for RLS a hereditary component was described whereas for
PLM no such association is known. However, a combined occurrence
of OSA and PLM is described - as an inverse relationship: patients
suffering from mild OSA have a high PLM-index in comparison to
patients with severe OSA and low PLM-index. Although no basic
dependencies could be revealed between RLS and OSA, methodology
and results of genetic-cause-research regarding OSA could be interesting for such a research in RLS or PLM patients.
432
Analysis of SCA2 and SCA3 in Parkinsons plus syndromes
P. Ratnagopal, Z. Yi, E.K. Tan (Singapore, Singapore)
Objective: To conduct genetic analysis of SCA2 and SCA3 in
patients with atypical Parkinsonism and Parkinsons plus syndromes.
Background: Some carriers of SCA2 and SCA3 mutations may
present clinically with atypical Parkinsonism and Parkinsons plus
symptoms. It is unclear whether routine genetic screening of these
patietns is worthwhile.
Methods: Consecutive patients diagnosed with parkinsons plus syndromes and atypical Parkinsonism based widely accepted criteria from
the Movement Disorder Clinic of Singapore General Hospital were
included. A Movement Disorder neurologist examined all the study
subjects. Our institutional ethics committee approved this study and
informed consent was obtained from all study subjects. DNA was
extracted from lymphocytes as per standardised protocol. The following sets of forward and reverse primers were utilized respectively: 5 GGGCCCCTCACCATGTCG-3, 5-CGGGCTTGCGGACATTGG-3
for SCA2; 5-CCAGTGACTTTGATTCG-3, 5-TGGCCTTTCACATGGATGTGA-3 for SCA3. PCR were carried out under the optimized
conditions. Electrophoresis of the PCR products was carried out on agarose gel under optimized conditions. The CAG repeat size was also
screened using standard size markers and positive SCA2 and SCA3
samples on the Genescan.
Results: A total of 70 patients with Parkinsonism plus syndromes
were analyzed. The mean age of the patients was 69.7 8.7 (52-85)

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

years. We could not detect any SCA2 and SCA3 mutations in our study
cohort.
Conclusions: SCA2 and SCA3 mutations are unlikely to be associated with Parkinsons plus syndromes in our population and hence
routine screening for these mutations is not cost effective.
433
Worldwide prevalence of Leucine-Rich Repeat Kinase 2 gene mutations in Parkinsons disease: A systematic review
L. Correia Guedes, J.J. Ferreira, M.M. Rosa, M. Coelho,
V. Bonifati, C. Sampaio (Lisbon, Portugal)
Objective: To evaluate the geographic and ethnic prevalence distribution of all known LRRK2 mutations in Parkinsons disease (PD)
patients.
Background: (PD) is the second most common neurodegenerative
disorder affecting 2% of people over 65 years of age. Mutations in the
Leucine-Rich Repeat Kinase 2 (LRRK2) gene have recently been
shown to be signicantly associated with familial and sporadic forms of
PD in different populations.
Methods: We conducted a systematic review of studies on the
prevalence of known LRRK2 mutations. Search strategy: electronic
searches of MEDLINE (2004-2007) were conducted. Grey literature
was hand searched and the reference lists of identied studies and
reviews examined. Selection criteria: studies on the prevalence of
LRRK2 mutations in populations with homogeneous ethic origin. Outcomes: prevalence of LRRK2 mutations on familial and sporadic PD,
methodological characteristics and quality of studies.
Results: Thirty nine prevalence studies on LRRK2 mutations in PD
were included. The studies emanated from 21 countries in 4 continents.
Most studies consecutively recruited unrelated patients and ethnically
matched controls from outpatient clinics. PD was diagnosed according
to specied clinical criteria. Denition of familial PD varied among
studies. Major methodological differences are specied. Prevalence of
G2019S mutation on familial and sporadic PD was higher in North
Africa, reaching 42% in Tunisian familial PD patients, followed by
Ashkenazi Jews and South West European countries. G2019S prevalence diminished as we approached North European, North American
and Asiatic countries. Other known LRRK2 mutations had lower
prevalence in the different populations studied.
Conclusions: LRRK2 gene mutations are a signicant cause of familial
and sporadic PD with heterogeneous prevalence in different world populations. It can not be excluded that methodological aspects, as differences
in the denition of familial PD, do not inuence results. There are still no
prevalence studies on LRRK2 mutation from Central or South Africa and
South America and scarce information in black Africans. These ndings
highlight the importance of using multiple population screenings for genetic association studies and genetic counselling.
434
ATP13A2 missense mutations in juvenile parkinsonism and young
onset Parkinsons disease
A. Di Fonzo, H. Chien, M. Socal, S. Giraudo, C. Tassorelli,
G. Iliceto, G. Fabbrini, R. Marconi, E. Fincati, G. Abruzzese,
P. Marini, F. Squitieri, M.W.I.M. Horstink, P. Montagna,
A. Dalla Libera, F. Stocchi, S. Goldwurm, J. Ferreira, G. Meco,
E. Martignoni, L. Lopiano, L. Jardim, B.A. Oostra, E. Barbosa,
V. Bonifati (Rotterdam, Netherlands)
Objective: To assess the prevalence, nature, and associated phenotypes of ATP13A2 gene mutations among patients with juvenile parkinsonism (onset 21 years) or young onset (between 21 and 40 years)
Parkinsons disease (YOPD).
Background: Loss-of-function mutations in the ATP13A2 gene were
recently identied in two kindreds affected by a juvenile multisystemic
neurodegenerative disease (Kufor-Rakeb syndrome) characterized by
akinetic-rigid parkinsonism, pyramidal tract dysfunction, supranuclear
gaze paresis, and dementia.
Methods: We studied 46 patients, mostly from Italy or Brazil,
including 11 with juvenile parkinsonism and 35 with YOPD. Thirty-

S133

three cases were sporadic and 13 had positive family history compatible with autosomal recessive inheritance. The whole ATP13A2 coding
region (29 exons) and exon-intron boundaries were sequenced from
genomic DNA.
Results: A novel homozygous missense mutation (Gly504Arg) was
identied in one sporadic Brazilian case with juvenile parkinsonism
(onset age 12). This patient had levodopa-responsive severe akineticrigid parkinsonism, levodopa-induced motor uctuations and dyskinesias, severe visual hallucinations, and supranuclear vertical gaze paresis, but no pyramidal decit nor dementia. Brain CT scan showed
moderate diffuse atrophy. Two Italian YOPD cases carried each a novel
missense mutation (Thr12Met, Gly533Arg) in single heterozygous
state.
Conclusions: We conrm that ATP13A2 homozygous mutations are
associated with human parkinsonism, and expand the associated genotypic and clinical spectrum, by describing a homozygous missense
mutation in this gene in a patient with a phenotype milder than that
initially associated to ATP13A2 mutations (Kufor-Rakeb syndrome).
435
Fibroblast growth factor 20 gene and Parkinsons disease in the
Japanese population
T. Toda, W. Satake, I. Mizuta, M. Watanabe, A. Takeda,
K. Hasegawa, M. Yamamoto, N. Hattori, M. Murata (Suita, Osaka,
Japan)
Objective: To test whether the broblast growth factor 20 (FGF20)
gene is a susceptibility gene for Parkinsons disease.
Background: A genetic association between the broblast growth
factor 20 (FGF20) gene and Parkinsons disease has been found by the
pedigree disequilibrium test. However, this association was not replicated by a case-control association study.
Methods: In order to clarify the association between the FGF20 gene
and Parkinsons disease, we attempted to replicate this association by
a case-control association study using a large number of Japanese
samples (1388 patients and 1891 controls).
Results: rs1721100 exhibited a signicant difference in allele C
versus G (P0.0089), and in genotype CCCG versus GG
(P0.0053). Haplotype association analysis showed that haplotype 2
was the protective haplotype for Parkinsons disease (permutationP0.0075).
Conclusions: These results suggest that the FGF20 gene is a susceptibility gene for Parkinsons disease in the Japanese population.
436
Parkinsons disease in Turkish patients: Molecular analyses of
parkin and LRRK2 genes in familial and isolated cases
C.S. Pirkevi, S. Lesage, A. Brice, A.N. Basak (Istanbul, Turkey)
Objective: The aim of this study is to identify the frequency and
distribution of parkin mutations/ rearrangements and the presence of
the LRRK2 G2019S mutation in Turkish patients with Parkinsons
disease (PD).
Background: Parkin is responsible for 39% of autosomal recessive
forms of early onset parkinsonism. In a recent paper by Lesage et al.
(2006), the frequency of the LRRK2 G2019S mutation has been shown
to reach 41% in the North-African population compared to 3% in the
European population.
Methods: For recessively inherited PD, we have performed dHPLC
analysis on 48 Turkish patients and their 29 relatives prior to sequencing. Exon rearrangements in the parkin gene were investigated in the
same patient population by a semi-quantitative multiplex PCR assay.
The presence of the G2019S mutation was analyzed in 72 Turkish PD
patients and their 43 relatives by direct sequencing of LRRK2 exon 41
(47 familial and 25 isolated cases).
Results: A great majority of the population under study revealed an
abnormal dHPLC prole for several exons of the parkin gene. On the
other hand, investigations of exon rearrangements in the parkin gene in
the same patients, resulted in the identication of seven heterozygous
duplications in exon12, six heterozygous deletions in exon11, three

Movement Disorders, Vol. 22, Suppl. 16, 2007

S134 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

heterozygous deletions in exon4, three heterozygous deletions in
exon3, three heterozygous deletions in exon2 and one heterozygous
duplication of exon 7, 8 and 9. Direct sequencing of the LRRK2 gene
exon 41 in 115 Turkish PD patients and their relatives revealed a
heterozygous G2019S mutation in a 69-years-old female patient, with
disease onset at 62 years.
Conclusions: Parkin gene: The high percentage of abnormal dHPLC
proles (56%) observed in the patient population is rather surprising
and has to be further investigated by gene sequencing for mutations and
polymorphisms. LRRK2 gene: The results obtained so far in 72 patients
revealed that the frequency of the LRRK2 G2019S mutation is not very
high in Turkey, which is in accordance with the European populations.
437
Sporadic and familial Parkinsons disease in Uruguay
V.E. Raggio, E.M. Dieguez, R. Aljanati, O. de Medina,
A. Scaramelli, R. Ventura, R. Buzo (Montevideo, Uruguay)
Objective: Asses epidemiology and familial aggregation of Parkinsons Disease.
Background: Parkinsons disease (PD) is caused by the complex
interaction of genetic and environmental factors. Several loci linked to
early onset PD have been found as well as genetic polymorphisms
inuencing PD risk and prognosis. Population aging, new diagnostic
techniques and medical awareness determine that epidemiological data
on PD are highly dynamic and favors the recognition of familial
aggregation in PD.
Methods: Epidemiological questionary and family history interview.
Results: We have established that the prevalence of PD in the
population of Uruguay is 1.36/ 1000, media of age is 72 years, predominates in females (1.1/ 1.000 males vs 1.66/ 1.000 females). Related disorders are also frequent: essential tremor (2.34/1.000), dementia (4.03/1.000), 60.5% being degenerative dementia and 8.1% being
mixed vascular and degenerative dementia. Familial forms of PD are
less than 10% of cases. Among familial cases, bradykinesia and rigidity
or even solely tremorous forms predominate. Age of onset is the best
indicator of genetic etiology: more than 60% of familial cases were of
early onset, vs less than 5% in sporadic cases. Moreover, the peak of
onset for familial forms is between 40 to 50 years while for sporadic
forms is between 60 to 70 years. Among familial cases, we have
detected several families segregating PD or related disorders compatible with dominant, recessive and multifactorial inheritance models.
Conclusions: PD prevalence is relatively high in Uruguay and familial
cases are a not negligible minority. On an attempt to integrate clinical
neurology and medical genetics, genetic counseling was offered and a
project aimed to develop molecular genetics diagnosis was initiated.

Results: Only the G2019S mutation was identied among our PD

patients, 14.8% in the Ashkenazi and 2.7% in the non-Ashkenazi
patients, and in 26% and 10.6% of the Ashkenazi familial and apparently sporadic cases, respectively. The carrier frequencies in the Ashkenazi and non-Ashkenazi control samples were 2.3% and 0.4%, respectively. The common shared haplotype was detected in all nonAshkenazi and half-Ashkenazi carriers and in all full-Ashkenazi
carriers, except two. Women and patients with a positive family history
of PD were signicantly over represented among the G2019S mutation
carriers, relative to their representation in the cohort of PD patients.
Age at disease onset was similar in carriers and non-carriers.
Conclusions: Our data suggest that the LRRK2 G2019S mutation plays
an important role in the causality of familial and sporadic PD in Israel and
that family history and gender affect its frequency among patients.
439
Mutation spectrum and clinical features of SPG4 HSP
C. Depienne, E. Fedirko, B. Bricka, E. Denis, S. Forlani, A. Durr,
A. Brice (Paris, France)
Objective: We investigated the spectrum of SPG4 mutations, the
clinical phenotypes and the mechanisms accounting for reduced penetrance and disease variability in a large series of 543 patients with
either pure or complex spastic paraplegia.
Background: Mutations in SPAST/SPG4, the gene encoding spastin,
are the most frequent cause of autosomal dominant hereditary spastic
paraplegia (AD-HSP).
Methods: The SPAST/SPG4 gene was screened by denaturing high
performance liquid chromatography (DHPLC) and/or multiplex ligation-dependent probe amplication (MLPA).
Results: We identied 164 (30%) positive families, 133 (25%) of
which have substitutions or small deletions/insertions detected by DHPLC and 31 (6%) which have larger exonic deletions ranging from one
exon to the whole gene, detected by MLPA. The clinical picture of
patients with deletion was comparable to that of patients with point
mutations. The great majority of the mutations were associated with
pure HSP in the families. However, few families also showed additional features including cognitive impairment, dementia, mental retardation, peripheral neuropathy or extra-pyramidal signs. A high proportion of mutations, especially of missense type, were found in sporadic
patients. Two different variants were identied in several patients,
suggesting that they could have an additive effect. Finally, we also
identied a de novo mutation associated with mosaicism in one patient.
Conclusions: Our ndings support the hypothesis of several mechanisms underlying the penetrance and age at onset variability including
genetic modiers.

438

440

The frequency of the LRRK2 G2019S mutation in Ashkenazi and

non-Ashkenazi Jews with Parkinsons disease in Israel
A. Orr-Urtreger, C. Shifrin, U. Rozovski, S. Rosner, D. Bercovich,
T. Gurevich, H. Yagev-More, A. Bar-Shira, N. Giladi (Tel Aviv, Israel)

Multiregional high-throughput gene expression proling of Braak

regions in Parkinsons disease
S. Papapetropoulos, D.M. Maraganore, N. Adi, J. Ffrench-Mullen,
D. McCorquodale, D.C. Mash (Miami, Florida, USA)

Objective: To assess the occurrence of the leucine-rich repeat kinase 2

(LRRK2) G2019S, I2012T, I2020T and R1441G/C/H mutations in a cohort of Jewish Israeli Parkinsons disease (PD) patients, and to determine
the LRRK2 haplotypes in G2019S-carriers and in non-carriers.
Background: Mutations in several genes have been identied in PD,
supporting an important role for genetic factors in this disease. Mutations in LRRK2 are among the most common genetic determinants of
PD identied to date, and have been implicated in both familial and
sporadic forms of disease. The G2019S change in LRRK2 exon 41 has
been associated with disease at varying frequencies in Asian, European,
North American and North African populations, and is particularly
prevalent among Ashkenazi Jews.
Methods: Until November 2006, we tested 472 PD patients of
Ashkenazi and non-Ashkenazi origin, and 1536 control samples. Mutation screening was performed by dHPLC and sequencing. Six microsatellite markers, which span the entire LRRK2 gene, and GeneScan
were used for haplotype analysis.

Objective: To identify genes and pathways contributing to the pathogenesis of Parkinsons disease (PD).
Background: Braak and Braak rst described the topographically
predictable sequence of the intracerebral formation of abnormal proteinaceous Lewy bodies and Lewy neurites in presymptomatic and
symptomatic PD. Based upon their neuropathological observations,
they developed a neuropathological staging for PD. To date, gene
expression proling studies of PD brain tissue has been largely limited
to the substantia nigra.
Methods: We performed multiregional gene expression analysis of
brain areas affected by Lewy body pathology in PD (Braak regions:
substantia nigra, ventral tegmental area, dorsal motor X nucleus, dorsal
raphe, locus coeruleus, reticular formation, and amygdala). We conducted high density gene expression analysis utilizing the Affymetrix
HG U133 Plus 2.0 gene chips (fold change 1.3, t-test p-value 0.05
with present calls 75% and RNA 5/3 GAPDH and b-actin 0.2) in
post-mortem brain specimens taken from PD cases (n22) and aged

Movement Disorders, Vol. 22, Suppl. 16, 2007

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

controls (n23). A total of 176 chips meeting strict RNA and microarray criteria from 7 Braak regions (PD: 98 and controls:78) were
included in the analysis. Brain samples were taken from patients who
met clinical and neuropathological criteria (Braak stage 4-6) for PD.
Results: We identied 169 genes whose pattern of expression was
signicantly regulated in 5 out of 7 regions studied. Eighteen genes
were signicantly regulated in all 7 regions. Among the top candidates
were two genes implicated in PD: (a) septin 4-a known component of
the Lewy bodies and (b) lysosomal-associated membrane protein 2,
involved in the autophagic degradation of alpha-synuclein. Several
genes involved in neuronal development, axonal repair, and apoptosis
were signicantly regulated in six or more Braak regions.
Conclusions: Gene expression proling of affected Braak regions
suggests the involvement of multiple pathogenetic processes in advanced PD.
441
Familial Parkinsonism and early onset Parkinsons disease: Phenotypic and genotypic characterization in a Brazilian Movement
Disorders clinic
S.T. Camargos, F. Cardoso, L.O. Dormas, P. Momeni,
A.L. Teixeira, Jr, D.P. Maia, M. Cunningham, A.J. Lees, J. Hardy,
A. Singleton (Belo Horizonte, Minas Gerais, Brazil)
Objective: 1 To evaluate the prevalence of familial Parkinsonism and
early onset Parkinsons disease in a Movement Disorder reference center.2
Phenotypic and genotypic studies of above-mentioned population.
Background: Idiopathic Parkinsons disease (IPD) is the most frequent cause of Parkinsonism, being diagnosed in up to 85% of cases.
Early onset PD (EOPD), dened by age of onset between 20 and 40
years of age, accounts for 4-10% of all patients with PD. Recent
epidemiologic studies have indicated a more evident Mendelian component at EOPD than in IPD. There is also evidence that etiologic
factors may vary depending on the geographic and ethnic background
of the studied population. To our knowledge there is no comprehensive
study of genetic factors in a cohort of Brazilian IPD patients.
Methods: Careful pedigree evaluation and a detailed neurological
exam including the application of the UPDRS. Patients were recruited
with their prior consent and ethical committee approval. DNA was
extracted from peripheral lymphocytes according to routine procedures.
Amplication of the genes PRKN, PINK1, SNCA and LRRK2 by
polymerase chain reaction was performed as previously described,
followed by sequencing reaction.
Results: During the period of the study we examined 576 patients of
whom 230 met the diagnosis of Parkinsonism. IPD was recognized in
89.5% of them with 13 probands of familial PD and 45 patients with
EOPD. The age at onset of the rst group was 52.312.2 years; six had
a pattern of autosomal recessive inheritance and seven autosomal
dominant. Only one patient had a mutation of the screened genes: it was
a novel LRRK2 mutation (Q923H). The age at onset of the second
group was 34.85.42 years; 11 had positive familiar history for the
disease in which 8 showed an autosomal recessive pattern. We have
found ve known mutations in PRKN (P153R, T240M, 255Adel,
W54R, V3I) and one novel mutation in PINK1, homozygous deletion
of exon 7.
Conclusions: To our knowledge this is the rst complete genetic
analysis in a cohort of Brazilian patients with Parkinsonism. We have
found two novel mutations at genes related to the etiology of Parkinson
disease (LRRK2 and Pink1).
442
Phenotypes and genotypes in myoclonus-dystonia: Signicance of
deletions of the entire SGCE gene
A. Grunewald, A. Djarmati, K. Lohmann-Hedrich, K. Farrell,
C. Klein (Lubeck, Germany)
Objective: To investigate the frequency and type of epsilon-sarcoglycan gene (SGCE) mutations with particular emphasis on gene dosage alterations and to explore the associated phenotypes in a preclassied group of myoclonus-dystonia (M-D) patients.

S135

Background: M-D is an autosomal dominant movement disorder

caused by mutations in SGCE in a subset of tested cases. Gene dosage
alterations have only recently been described for SGCE, although they
are common in other genes.
Methods: We tested 34 M-D index patients (typical M-D with a
positive family history: n11; typical M-D without a positive family
history: n13; possible M-D: n10) and available family members for
gene dosage alterations in SGCE by quantitative PCR methods and for
other mutations by genomic sequencing. The extent of the deletions
was further dened by haplotype analysis and quantitative PCR.
Results: Mutations were found in 27% (9/34) index cases, all with
typical M-D and a positive family history in 7 of them. Mutations
included two different heterozygous deletions of the entire SGCE gene
(c.Ex1_12del) and anking DNA as well as one deletion of exon 2 only
(c.Ex2del). Thus, gene dosage alterations accounted for 9% (3/34) of
M-D in our sample and for 33% (3/9) of the mutations found. Segregation of the mutations was demonstrated in 2/3 families. The size of
the two whole gene deletions comprised at least 0.54 Mb, as demonstrated by hemizygosity at relevant DNA markers. Both of them contained the collagen type I alpha 2 gene (COL1A2) and were associated
with skin and joint problems in addition to the motor syndrome. A
correlation between the genotype and phenotype was only found in
patients with deletions encompassing additional genes.
Conclusions: A rigorous preselection of patients for clinical features
and careful accounting for non-motor signs should precede the mutational screening. Gene dosage studies should be included in routine
SGCE genetic testing. A combination of these considerations has led to
a high mutation detection rate in our study with implications on both
the clinical and the research setting.
443
Caffeine intake and CYP1A2 variability in Parkinsons disease
E.K. Tan, E. Chua, L. Tan, Y. Zhao (Singapore, Singapore)
Objective: We investigated the potential interaction of a CYP1A2
genetic variant with the quantity of coffee and tea intake and their
relationship with the risk of Parkinsons disease (PD).
Background: The cardinal endogenous system responsible for caffeine
metabolism in humans is the cytochrome P450 1A2 (CYP 1A2), accounting for more than 90% of caffeine clearance. Genetic variability of
CYP1A2 results in a wide individual variation of caffeine metabolism.
Methods: PD and control subjects were recruited from hospital
clinics. Exposure information on coffee and tea intake was collected.
Genotyping of a CYP1A2 polymorphic variant was carried out using
standardized methods.
Results: A total of 400 subjects with 200 PD and 200 race, gender
and age matched controls were included. A multivariate analysis of the
variables including the CYP1A2 genotypes, age of onset, gender, and
the quantity of tea and coffee intake, interaction of the CYP1A2
genotypes with coffee intake, interaction of CYP1A2 genotypes with
tea intake demonstrated the quantity of caffeine intake to be signicantly associated with PD in both fast and slow caffeine metabolizers
(p 0.001).
Conclusions: A dose dependent protective effect of coffee intake was
observed in both fast and slow caffeine metabolizers in PD.
444
Focal dystonia as an important feature of X-linked mental retardation caused by ARX gene duplication
K. Szczaluba, M. Nawara, K. Poirier, J. Pilch, M. Gajdulewicz,
K. Spodar, J. Chelly, J. Bal, T. Mazurczak (Warsaw, Poland)
Objective: To assess frequency of ARX gene duplication in Polish
men with X-linked mental retardation. To establish genotype-phenotype correlation in mutation-positive cases.
Background: Mental retardation (MR) has a frequency of 2%. It is
more prevalent in men than in women suggesting a crucial role of genes
on X chromosome in its etiology. One of the genes of interest is
Aristaless-related homeobox (ARX) located on the short arm of chromosome X (Xp22). A duplication c.428_451dup24 in exon 2 of ARX is

Movement Disorders, Vol. 22, Suppl. 16, 2007

S136 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

responsible for characteristic phenotypes of Partington syndrome, West
syndrome with infantile spasms and subsequent MR with epilepsy, MR
with seizures, and non-syndromic forms of X-linked MR.
Methods: We screened 165 mentally retarded Polish patients for
ARX gene duplication by PCR and DHPLC methods.
Results: Eighteen individuals from ve families were found to carry
the duplication, and all had MR. Twelve individuals from all mutationpositive families presented with focal hand dystonia. In addition, one of
them had foot dystonia. Four other patients, including the three with
apparently no hand dystonia, showed signicant spasticity over upper
or lower limbs. Other observed features, except speech difculties,
were less consistent throughout the mutation-positive families.
Conclusions: Focal dystonia with or without accompanying spasticity is an important clinical sign in evaluation of mentally retarded men.
In X-linked families it strongly points to causative duplication in the
ARX gene. It is therefore reasonable to request molecular analysis for
the presence of the duplication in dystonic men from MR families
consistent with X-linked inheritance.

MYOCLONUS
445
Gabapentine induced myoclonus: Case report
F. Ege, Y. Kocak, A.P. Titiz, S. Ozturk, S. Ozbakir (Ankara, Turkey)
Objective: To review and discuss the potential relations between
myoclonus and Gabapentine.
Background: Myoclonus is a phenomenon, which is classied in the
hyper-kinetic movement disorders. So many factors are described in its
etiology so far. Gabapentine is a new anti-epileptic drug, which is
considered to be well tolerated. It may however give rise to involuntary
movements rarely.
Methods: 87 years old male patient has been accepted to our clinic
for multi-focal myoclonus starting approximately three hours after he
took one dose of 600 mg Gabapentine. In his medical history he had not
experienced any movement disorder. Other probable causes of myoclonus were excluded by laboratory, imaging and electrophysiological
examinations.
Results: The patient has been diagnosed as iatrogenic myoclonus.
Conclusions: Although Gabapentine is considered to be safe drug in
the elderly patients it may induce myoclonus.
446
Two cases of segmental myoclonus: Primary spinal myoclonus and
radicular myoclonus induced by cervical physiotherapy stretching
J. Vaamonde, G. Martin-Palomeque, J.M. Flores, R. Ibanez (Ciudad
Real, Spain)
Objective: Spinal myoclonus has been associated with various spinal
cord insults. Anyway only few cases of peripheral nervous system-generated myoclonus have been reported in patients with spinal root lesion.
Background: Myoclonus results from abnormal activity in many
different parts of the central nervous system.
Methods: We present the case of a 22- year-old woman with dorso
lumbar spinal myoclonus and normal magnetic resonance imaging.We
also report a 24 -year-old woman who developed segmental myoclonus
immediately after prolonged cervical stretching in an attempt to reduce
neck tension related to exion-extension neck movement (trafc accident).
Results: In the rst patient clinical and electromiographic ndings
pointed to the segment D12-L2 as the origin of the myoclonus. No
structural lesion was found in MRI and laboratorial examinations were
normal. In the second patient EMG recording revealed contraction of
several muscles all supplied by left C6/C7 roots. Cervical MRI was
normal. The two patients improved with levetiracetam and valproico.
Conclusions: The pathophysiology of spinal myoclonus remains
speculative (loss of inhibitory function of local dorsal interneurons, loss
of inhibition from suprasegmental descending pathway. . . ) and in
primary myoclonus the etiology is unknown. In the second patient the
segmental distribution of jerks involving C6/C7 myotomes, the normal
spinal MRI, the absence of any excitability change in cerebral cortex,

Movement Disorders, Vol. 22, Suppl. 16, 2007

brainstem or spinal cord suggest a peripheral level origin. It is likely

that the cervical stretching had determine a transient mechanical irritation of the roots.
447
Propriospinal myoclonus: Report of seven new cases and literature
review
P. Bounolleau, E. Apartis, D. Ducreux, Y. Beaugendre,
M.-C. Lavallard-rousseau, F. Bourdain, P. Dupont, L. Carluer, L. Verdure,
M. Vidailhet, E. Roze (Paris, France)
Objective: To further delineate the clinical, neurophysiological and
neuroradiological spectrum of propriospinal myoclonus (PSM).
Background: In 1991, Brown et al. provided the rst description of
PSM and reported afterwards the only large case series involving 8
patients. Literature on the subject is poor and there is no available
systematic review of all published cases to date.
Methods: We prospectively investigated all patients seen in our
movement disorders clinic with a rm diagnosis of PSM between 2003
and 2006. The patients had detailed clinical examination, large laboratory investigation, comprehensive neurophysiological examination
including polymyography, C-reex recording, EEG jerk-locked-backaveraging and multimodal evoked potentials, conventional MRI and
diffusion tensor imaging with ber tracking (DTI-FT) of the spinal
chord. In addition, we provided a systematic review of the literature
focusing on the phenomenology of this disorder and its etiology.
Results: Mean age was 39 years (range 17-51), six patients were
male and one female, possible cause was history of back trauma (2),
myelopathy associated with anti-thyroid antibodies (1), lymphocytic
myelo-meningitis of unknown etiology (1). PSM were increased by
lying position in all patients. Most patients had premonitory sensations
before jerks and PSM worsening in presleep relaxation period. Interestingly, one patient had dramatic post-coital bouts of PSM. Neurophysiological examination conrmed the diagnosis with slow estimated
conduction velocity, absence of bereitschaft potential, long loop C
reex or short latency potential. Myoclonus bursts duration ranged
from 400 to 1500 ms. Generator level was thoraco-lumbar in all
patients. Conventional MRI of the spinal chord was normal in 5/7
patients whereas DTI-FT showed abnormalities in all patients.
Conclusions: This new series of 7 patients shows that the clinicophysiological spectrum of PSM is homogenous. Etiology is difcult to
identify, and thus a wider etiological screening may be required.
DWI-FT appears more sensitive than conventional MRI to detect
associated microstuctural abnormalities of the spinal chord.
448
Steroid responsive propriospinal myoclonus associated with antithyroid antibodies
E. Roze, E. Apartis, M. Vidailhet, V. Cochen, Y. Beaugendre,
J.-M. Trocello, P. Lasjaunias, D. Ducreux (Paris, France)
Objective: To describe a patient with propriospinal myoclonus
(PSM) and associated antithyroid antibodies that dramatically respond
to steroid administration.
Background: PSM is a rare Movement Disorder characterized by
involuntary spinal-generated muscular jerks that spread rostrally and
caudally to other spinally innervated muscles. Most patients have no
clear etiology, and conventional MRI of the spinal cord is generally
normal. Symptomatic pharmacological treatment are disappointing.
Methods: The patient had detailed clinical, biological and MRI
examination including diffusion tensor imaging (DTI) and ber tracking (FT). Electrophysiological analysis included polymyography, Creex recording and EEG jerk-locked-back-averaging. MRI was performed with sagittal T2-STIR sequences, followed by Diffusion tensor
imaging and ber tracking.
Results: A 51-year-old man spontaneously developed involuntary repetitive
shock-like jerks of the upper limbs and trunk and, to a lesser extent, of the
lower limbs and neck. Neurophysiological studies found that the rst muscles
to be activated were those controlled by low-thoracic spinal level and the
conduction velocity was estimated at 6.7 m/s and 4.5 m/s from T10 to C6 and

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

from T10 to S1, respectively. Whereas conventional MRI was normal, colorcoded tracts analysis showed asymmetry and thinning of the posterior lemniscal tracts and corticospinal posterolateral tracts from spine levels C6 to T10,
that predominate on the left side. Biological investigations were normal, except
for a positive anti-thyroperoxidase antibody titer (114 U/mL; normal values
35 U/ml). The patient received steroid bolus therapy followed by a tapering
oral therapy. It resulted in disappearence of the jerks after ve days. Every
trial to decrease the dose below 0.5 mg/day of prednisone led to reappearence of the jerks.
Conclusions: PSM may be related to myelopathy associated with
antithyroid antibodies. This possibility should be considered in the
diagnosis work-up of patients with PSM. In addition, MR-DTI-FT may
be useful for detecting spinal cord abnormalities in patients with
propriospinal myoclonus.
449
Psychogenic propriospinal myoclonus
M. Cowey, K. Tuck, B. Day, D. Williams (Melbourne, VIC, Australia)
Objective: To describe the clinical and electrophysiological features
in a case of psychogenic propriospinal myoclonus (PSM).
Background: PSM is characterized by jerking that involves muscles
innervated by many different segments of the spinal cord. Characteristic electrophysiological ndings show selective recruitment of truncal
and proximal limb muscles. Similar patterns of muscle activity have
been demonstrated in subjects mimicking PSM (Kang, 2006).
Methods: In the case reported, surface electrodes were used to record
the electromyogram (EMG) from sternomastoid, pectoralis, 7-8th intercostal space, and upper abdominal muscles. Epochs were studied for
burst duration and order of activation. A jerk locked back averaged
EEG and median nerve SEPs were performed.
Results: A 65-year-old woman received soft tissue injuries to her right
orbit after tripping on the pavement, requiring reconstructive blepharoplasty and a subsequent browplasty. The facial surgery was complicated by
chronic head and neck pain and anxiety related to the surgical procedure.
Following a period of severe pain, 18 months after the fall, she developed
disabling paroxysms of axial, exor jerks, occurring in bouts lasting up to
an hour, and most severe when lying. Brain and cord MRI were normal.
Routine EEG was normal. An order of activation study of representative
1-second epochs showed variable muscle activation (most frequently propogating from pectoral to abdominal muscles at 9-15 m/s). EMG burst
length was 50-150ms. Median nerve SEPs were normal. A jerk locked
back averaged EEG was negative. The ndings were suggestive of PSM.
Psychiatric evaluation did not identify features of somatization. She incompletely responded to anticonvulsant and benzodiazepine therapy, and
became progressively disabled over 2 years. Three surgical screws used in
the blepharoplasty were removed at the patients insistence and the axial
jerks coincidentally resolved completely. She returned to normal function
and has been symptom free for 2 years. The unusual mode of onset with
associated anxiety, the uctuating clinical course and spontaneous resolution make psychogenic PSM likely.
Conclusions: The electrophysiological features said to be characteristic
of PSM do not adequately differentiate organic PSM from voluntary axial
jerks (Kang, 2006) or, in the present case, psychogenic PSM. In the
absence of a cord lesion, psychogenic aetiology must be considered.

NEUROIMAGING

S137

visceral organs and in the brain. The pathogenesis of brain involvement

in aceruloplasminemia is unclear.
Methods: Diffusion tensor imaging (DTI) were used to determine the
mean diffusion (MD) and fractional anisotropy (FA) in the brains of the
proband with full phenotype, the 31-year-old sister of the proband with
mild phenotype, the 47-year-old healthy sister of the proband with
heterozygote at the same position in comparison to 16 female healthy
subjects. Voxel-based analysis (VBA) of both the diffusion and anisotropy values was performed using statistical parametric mapping (SPM).
Results: Diffused increases in MD were identied in the cerebral cortex
and cerebellar cortex in full phenotype case, but slight abnormalities in the
milder phenotype one. Reductions in MD were identied in several structures of basal gangalion in the full phenotype case, while only slight in the
left dentate nucleus and left putamen in the milder case. Reductions in FA
were identied in the several areas of basal gangalion in the full phenotype
case, while only in the corpus closum in the milder case. Several areas of
FA increase were also identied. No abnormalities of MD and FA were
identied in the healthy sister of the proband.
Conclusions: We present previously unreported brain abnormalities
due to aceruloplasminemia with voxel based analysis. These abnormalities correlate with clinical presentations and were slight in milder one.
This suggests that the development of neurological symptoms caused
by the damage following iron deposit in the central nervous system
may take a long duration. In contrast, hypoceruloplasminemia found in
the heterozygote state do not provoke a measurable disturbance of the
central nervous system.
451
Diffusion tensor imaging of two unrelated Chinese men with hereditary spastic paraplegia associated with thin corpus callosum
H.-F. Shang, Q. Chen, J.-G. Wang, Q.-Y. Gong, D. Zhou,
J.-M. Burgunder (Chengdu, China)
Objective: To investigate and localize abnormalities in the brains of
two Chinese patients with HSP-TCC.
Background: Hereditary spastic paraplegia associated with thin corpus
callous is a rare autosomal recessive neurodegenerative disorder, characterized by abnormally thin corpus callosum, normal motor development,
slowly progressive spastic paraparesis and mental deterioration.
Methods: Diffusion tensor imaging (DTI) was used to determine the
mean diffusion (MD) and fractional anisotropy (FA) in the brains of
patients in comparison to 20 healthy subjects. Voxel-based analysis
(VBA) of both the diffusion and anisotropy values was performed
using statistical parametric mapping (SPM).
Results: Signicant MD increases and FA reduction in the already
known lesions (such as corpus callosum, cerebellum and thalamus) and
normal appearing regions in conventional MRI (such as brain stem,
internal capsule, cingulum and spreaded subcortical white matter including superior longitudinal fascicle and inferior longitudinal fascicle)
were found in both cases. No FA increases and MD reduction were
detected in the brain.
Conclusions: Our study provides a clear in vivo MR imaging evidence of diffuse brain involvement of HSP with TCC. Increases in MD
in thalamus and subcortical white matter is more sensitive than FA in
detecting lesions, suggesting MD could be a better marker of the
disease progression.
452

450
Diffusion tensor imaging nding in a Chinese family with aceruloplasminemia
Q. Chen, X.-P. Chen, Q.-Y. Gong, J.-M. Burgunder, D. Zhou,
H.-F. Shang (Chengdu, China)
Objective: To investigate and localize abnormalities in the brains of
members of a Chinese family with aceruloplasminemia resulting from
a novel homozygous mutation (W283S) of ceruloplasmin gene.
Background: Aceruloplasminemia is a rare autosomal recessive neurodegenerative disorder, characterized by iron accumulation in the

fMRI correlates of foot movement relative to gait dysfunction in PD

P. Schwingenschuh, P. Katschnig, S. Ropele, F. Gorani, F. Ebner,
E. Ott, F. Fazekas, C. Enzinger (Graz, Austria)
Objective: To investigate the central mechanisms of gait control in PD.
Background: Gait dysfunction represents a key feature of PD. Recently,
fMRI paradigms to study central mechanisms of gait control have been
proposed and already applied to multiple sclerosis and stroke patients.
Here we used an ankle dorsiexion paradigm to characterize differences in
activation patterns between patients with PD relative to controls and to
identify fMRI correlates of gait dysfunction within the patient group.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S138 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: 20 PD patients (mean age 66.8 years, mean disease duration
6.2 years) and 20 healthy age-matched controls underwent both fMRI
scanning at 3.0T and extensive gait testing. All subjects were right-handed
and had a mild to moderate gait disorder related to PD. Patients were
investigated in a practical off state. Clinical examination included UPDRS, Schwab and England and Hoehn&Yahr scores. The fMRI experiment included blocks of active and passive foot movement in a specic
wooden manipulandum with interspersed rest periods. FMRI Data were
analyzed using FEAT, part of FMRIBs software library FSL. Motion
parameters were included as covariates of no interest. Higher-level group
analyses were performed in mixed effects models.
Results: We observed consistent activation of expected supraspinal
sensorimotor networks associated with ankle movement both in patients and controls. Active movements elicited a more extensive brain
response than passive movements. However, non-volitional passive
movement also was associated with signicant activation within the
contralateral primary sensorimotor cortex (cSMC) as expected by somatopy (paracentral lobule). Further areas of activation comprised the
supplementary motor area (SMA), cingulate motor areas (CMA), and
ipsilateral cerebellum. There were distinct differences between the
activation patterns of PD patients relative to controls, scaling with
disease severity as reected by the functional scales used.
Conclusions: This study demonstrates the clinical feasibility of an
ankle dorsiexion MRI paradigm to study neocortical changes relative
to gait dysfunction in PD. Distinct activation differences from controls
in a cortico-subcortical network suggest the potential of fMRI to further
elucidate neural correlates of parkinsonian gait in vivo.
453
fMRI in gene-positive myoclonus-dystonia
R.J. Beukers, E.M. Foncke, J.N. van der Meer, A.J. Nederveen,
M.B. de Ruiter, L.J. Bour, D.J. Veltman, M.A. Tijssen (Amsterdam,
Netherlands)
Objective: To study cerebral activation patterns with functional
magnetic resonance imaging (fMRI) in the autosomal dominantly inherited movement disorder myoclonus-dystonia (M-D).
Background: M-D is a movement disorder clinically characterized by
myoclonic jerks and dystonic postures or movements of the upper body,
often combined with psychiatric symptoms such as depressed mood or
anxiety. M-D is autosomal dominantly inherited and is frequently caused
by mutations in the epsilon-sarcoglycan gene (SGCE) on chromosome
7q21 (DYT11). Penetrance of M-D is highly dependent on the parental
origin of the disease allele, assumed to result from maternal imprinting.
Based on current neuronal models of myoclonus and dystonia, we hypothesised that MC patients would exhibit abnormal activation patterns in basal
ganglia and cortical sensorimotor areas compared with healthy controls.
Furthermore, based on the maternal imprinting mechanism, we hypothesized that NMC would not show these abnormalities.
Methods: Nine manifesting (MC), 8 non-manifesting (NMC)
DYT11 mutation carriers, and 11 control subjects were studied in a 3.0
Tesla MRI. A motor task and the serial reaction time task (SRT) were
performed in a block design.
Results: In MC signicant hyperresponsiveness in contralateral inferior parietal cortical areas, ipsilateral premotor and motor cortex, and
ipsilateral cerebellum as well as hyporesponsiveness in contralateral
insula and anterior cingulate cortex were observed during the motor
task compared to healthy controls. NMC showed similar but less
pronounced abnormalities. During the SRT hyperresponsiveness was
detected in the right putamen and caudate nucleus in MC and NMC
compared to controls.
Conclusions: The cortical and subcortical activation patterns in MC
and NMC during both tasks point to a disorganised sensorimotor
integration in DYT11. The observed abnormalities in NMC are not
consistent with a maternal imprinting mechanism and suggest the
involvement of additional genetic or environmental factors in the
phenotypic expression of M-D.

Movement Disorders, Vol. 22, Suppl. 16, 2007

454
Major depression and brain perfusion images in Parkinsons disease
I.U. Isaias, G. Marotta, D. De Gaspari, C. Siri, R. Benti, R. Cilia,
M. Canesi, G. Pezzoli, A. Antonini (Monza, Italy)
Objective: To determine whether Parkinsons disease (PD) patients
with major depression exhibit differential brain activity compared to
non-depressed PD patients.
Background: The frequency of depression in PD is about 40% and
the rate of depression is twice that seen in other disabling diseases.
However, the pathophysiology of depression in PD remains obscure.
Regional brain changes have been demonstrated in non-PD patients
with depression with the core perfusion decit found in bilateral
parietal regions.
Methods: We studied 9 right-handed PD patients who fullled diagnosis of depression according to DSM-IV criteria (dPD: 3 females,
mean age 59 8SD years, disease duration 11 2SD years) and 15
right-handed non-depressed PD patients (ndPD: 2 females, mean age
59 8SD years, disease duration 10 4SD years). Diagnosis of PD
was made according to the UK Parkinsons Disease Brain Bank criteria. All dPD and ndPD patients were at Hoehn and Yahr stage 2 or 3
and matched for age, disease duration and severity (UPDRS-III). We
excluded patients with dementia, cognitive and/or executive functions
decits assessed by the Mini Mental State Examination, the Frontal
Assessment Battery and the Wisconsin Card Sorting Test. The Beck
Depression Inventory (BDI) was used to quantify the severity of
depressive symptoms. All subjects underwent resting state brain perfusion SPECT on-medication using 99mTc-ethylcysteine dimer. Statistical Parametric Mapping (SPM2) was used for data analysis
(p0.001, uncorrected).
Results: We found hypoperfusion in the left and right inferior frontal
gyrus and in the left middle frontal gyrus; we also found two areas of
hyperperfusion: a rst one in the right thalamus and right hippocampus and
a second in the posterior cingulum, cerebellar tonsil and posterior lobe of
left cerebellum. BDI score was 21 3SD for dPD and 3 2SD for ndPD
(p0.01, Mann-Whitney test); UPDRS-III mean score did not differ
between PD subgroups (dPD: 34 10SD; ndPD of 33 12SD).
Conclusions: Our analysis outlined that major depression in PD is
associated with hypoperfusion of inferior and middle frontal gyri. The
ndings indicate that loss of dopaminergic innervation in PD might
cause a frontostriatal circuits impairment and play a key role in the
manifestation of depression in PD.
455
Flair sequences in Wilsons disease MRI. A case report
V. Sanchez, C. Coca, M. Fernandez, J. Chacon, L. Redondo (Seville,
Spain)
Objective: To describe the brain abnormalities in Wilsons disease as
depicted at FLAIR imaging in one of our patients, and to suggest this
sequence to be included in diagnostic protocols.
Background: Its widely known that in patients with Wilsons disease with neurologic manifestations a brain MRI may show characteristic symmetrical signal abnormalities in basal ganglia, cerebellum,
thalami and brainstem. Its been recommended to use MRI as a diagnostic tool, mainly T2WI and DWI sequences.
Methods: We present a case of the 26 year old woman with elevated
liver enzymes and mood disorders. She was admitted with upper and
lower extremities distal tremor, hypophonia and drooling. Neurologic
examination revealed upper lip dystonia and lower extremities, bradykinesia, ataxia and left foot extensor plantar response. Kayser-Fleischer
rings were observed. Serum ceruloplasmin and urine copper studies
conrmed the diagnosis of Wilsons disease. MRI, DaT-Scan and brain
SPECT were also performed.
Results: T2-weighted images revealed symmetric involvement of the
brainstem and basal nuclei. The characteristic face of the giant panda
appeared. FLAIR sequence showed the same lesions with higher sensitivity: symmetric hyperintensities involving pons, cerebellar peduncles, midbrain and corticospinal tracts, while globi pallidi were hypointense.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Conclusions: Lately one study 1 has demonstrated that there is
clinical correlation between DWI sequences and evolution of the disease; in this same study, FLAIR sequences were more sensitive, showing characteristic lesions in all syntomatic patients. Although there are
many descriptions of T2 and DWI imaging2, FLAIR images are only
now beginning to be used in characterization on Wilsons disease. We
think that a case like ours may show the utility of such sequences, as
it depicts clearly and in a comprehensive way the affected areas.
1
Clinical correlates of cerebral water diffusion in Wilson disease.
Favrole P, Chabriat H, Guichard JP et al. Neurology 2006;66:384
389

S139

Wilsons disease: cranial MRI observations and clinical correlation. Sinha S, Taly AB, Ravishankar S. et al. Neuroradiology
2006; 48: 613 621.

456
Altered brain response in PD patients with hallucinations during a
face perception task. An fMRI study
B. Ramirez-Ruiz, C. Junque, C. Falcon, N. Bargallo, M.-J. Marti,
F. Valldeoriola, T. Eduard (Barcelona, Spain)
Objective: To investigate brain regions involved in visual face processing in patients with Parkinsons disease (PD) and visual hallucinations
(VH) by means of functional Magnetic Resonance Imaging (fMRI).
Background: Several prospective studies indicated that PD patients
with visual hallucinations showed decits on tests of recognition and
memory for faces in comparison with non-hallucinating PD patients.
To our knowledge, the visual cortex response during a face perception
task has not been explored.
Methods: Twenty people suffering from idiopathic PD gave informed consent to take part in the study. Ten PD patients who had
experienced VH in the previous year and 10 PD patients without any
history of VH were explored. Exclusion criteria for both groups were
clinical depression and dementia. To evaluate face recognition ability
we used the Benton Facial Recognition Test (BFRT). Subjects were
explored with fMRI while performing a visual task. During this task,
participants viewed alternating blocks of abstract patterns and pictures
of human faces. Data were analyzed using SPM2 software. We restricted our analyses of group differences to voxels that showed greater
activation in the face condition relative to abstract pattern condition.
Threshold for statistical signicance was set at p0.001 (uncorrected).
Results: In comparison to non-VH PD patients, the performance in
Benton Facial Recognition Test was impaired in hallucinating PD patients
(p0.002). Neuroimaging data showed that hallucinating PD patients,
when compared to non-VH PD subjects, had lower BOLD signals in right
prefrontal areas (BA 8, 10, 47) and posterior cingulate gyrus (BA 31).
Conclusions: In comparison to non-hallucinating PD subjects, PD
patients with VH showed lesser activity in prefrontal regions and in the
posterior cingulate gyrus in response to human faces. Our results
suggest that alterations in different parts of cortical face perception
network can lead to impairment in face recognition with can contribute
to the development of complex VH in PD patients.
457
Amyloid deposition and glucose metabolism in Parkinsons disease
dementia (PDD): An [11C]PIB and [18 F]FDG PET study
I. Ahmed, P. Edison, N.Q. Quinn, Z. Walker, D.J. Brooks (London,
United Kingdom)

FIG. 1 (455).

Objective: To correlate regional brain amyloid load with glucose

metabolism in Parkinsons disease with dementia.
Background: Neuropathological studies have reported varying
amounts of amyloid pathology associated with PDD and dementia with
Lewy bodies (DLB). 11C-PIB PET is a marker of brain amyloid load.
Regional glucose metabolism reects synaptic activity and FDG PET
studies have shown reduced glucose utilisation in cortical association
areas in Parkinsons disease with dementia. The relationship between
amyloid load and brain metabolism in PDD is unclear.
Methods: 8 control subjects and 7 PDD patients were recruited. Each
subject had an MRI and two PET scans (PIB and FDG) .Amyloid load
was quantitated as 60-90 target:cerebellar uptake ratios (RATIO).
Object maps were created using a standard probabilistic atlas. Cortical
PIB and FDG uptake were assessed using region of interest analysis.
Results: (Please refer to attached gure) None of the 7 PDD patients
showed any increase in amyloid levels but all had reduced glucose
metabolism in frontal, temporal, parietal and occipital association areas
compared to controls (p value0.03).
Conclusions: This pilot study conrms signicant hypometabolism
in the frontal, temporal, parietal and occipital cortex in PDD. In these
subjects amyloid deposition did not contribute signicantly to the

Movement Disorders, Vol. 22, Suppl. 16, 2007

S140 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

pathogenesis of their dementia which is likely to arise from a combination of cortical Lewy body disease and neurotransmitter changes.

111 had PD - Hoehn and Yahr (HY) stage 2-4, 8 patients had parkinsonian variant of multiple system atrophy (MSA-P) - HY stage 3-4, 10
patients had vascular parkinsonian (VP) syndrome - HY 2-4, 10 patients had ET, 8 patients had neuropathic or drug induced tremor, 2
patients had focal dystonia. As control group we examined 60 patients
without any extrapyramidal symptoms.
Results: The enlarged (equal or more than 0,25 cm2) hyperechogenic
SN was detected in 84.7% patients with PD (p0.05), 50% patients
with MSA-P (p0.05), 27.3% patients with VP, 20% patients with ET,
30% patients with other tremors, one of two patients with focal dystonia and 0% patients without extrapyramidal symptoms.
Conclusions: TCS could be used in differentiation between PD, APS
and other extrapyramidal symptoms. Enlarged hyperechogenic SN
seems to be a marker of structural involvement of this nucleus in PD or
APS.
460
White matter damage is more severe in PSP than MSA or PD, a
diffusion tensor MRI study
C.R.V. Blain, G.J. Barker, X.A. Chitnis, R.G. Brown, J.M. Jarosz,
D.K. Jones, S.C.R. Williams, N. Leigh (London, United Kingdom)

458
In vivo qualitative and semi-quantitative analysis in basal ganglia
disorders: A [(123)I]-FP-CIT (Ioupane) study
L. Pierguidi, N. Tambasco, F. Fabiani, C. Menichetti,
M. Sebastianelli, P. Calabresi, A. Rossi (Perugia, Italy)
Objective: To investigate (FP)-CIT binding pattern of two in basal
ganglia pathological conditions, as PD and dystonia.
Background: Iodine-123 uoropropyl-CIT (FP-CIT) is an analogue
of -CIT, and has been shown to achieve peak trace uptake in the brain
within hours postinjection and to provide greater selectivity for the
dopamine transporter.
Methods: 14 parkinsonian and 13 dystonic patients were studied with
(FP)-CIT. For analysis of striatal FP-CIT binding, Region of interest
(ROI) were constructed manually in area corresponding to the whole
striatum, caudate, putamen nuclei and cerebellum. For the SPECT
imaging analysis both methods, qualitative and semi-quantitative, were
used.
Results: PD group showed signicant decrease in striatal (FP)-CIT
uptake when compared with dystonic group. Indeed, both qualitative
and quantitative analysis were comparable regarding reductions of
(FP)-CIT uptake in whole striatum and caudate/putamen alone, using
double blind method.
Conclusions: Analysis of (FP)-CIT SPECT revealed a widespread
decline of monoamine transporter availability in Parkinsons Disease
group than in Dystonia group, reecting differences in the underlying
pathology. We suggest that the qualitative analysis of caudate/putamen
[123I] uoropropyl (FP)-CIT, as well as the semi-quantitative measurement, is able to enhance the diagnostic accuracy in basal ganglia
dopaminergic disorders.
459
Substantia nigra evaluation in atypical parkinsonian syndromes
and Parkinsons disease
P. Bartova, D. Skoloudik, T. Fadrna, P. Ressner, P. Kanovsky,
R. Herzig (Ostrava, Czech Republic)
Objective: The aim of study was to test an ability of Transcranial
sonography (TCS) in differentiation of Parkinsons disease (PD), essential tremor (ET) and atypical parkinsonian syndromes (APS) patients.
Background: Differentiation among idiopathic PD, ET and APS
using clinical criteria may be difcult, especially in early stage of
disease. TCS is able to detect structural abnormalities of substancia
nigra (SN).
Methods: SN echogenicity and area were examined using TCS
(Philips HDI 5000, 2-4 MHz probe). Out of the 211 examined patients

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To evaluate the distribution and severity of regional

damage to white matter tracts in patients with progressive supranuclear
palsy (PSP), multiple system atrophy (MSA) and Parkinsons disease
(PD) using diffusion tensor MRI.
Background: Axonal degeneration within white matter tracts is described pathologically in the parkinsonian disorders, but the distribution and extent of white matter damage has not been directly compared.
Automated analysis of diffusion tensor MRI (DTI) data provides a
method of comparing disruption of white matter tracts across the whole
brain in vivo in these disorders.
Methods: Sixteen patients with PSP, 18 with MSA, 14 with PD and
19 healthy volunteers were recruited. All subjects underwent whole
brain diffusion tensor MRI in a 1.5 tesla system (same scanner and
protocols). Following calculation of the diffusion tensor, fractional
anisotropy (FA) maps were constructed. The white matter was masked
from the rest of the brain and white matter FA in each patient group
compared to controls using a voxel-based analysis technique.
Results: In patients with PSP, white matter FA was signicantly
reduced in brainstem regions including the pons, midbrain (decussation
of the superior cerebellar peduncle) and cerebral peduncles. In the
cerebral hemispheres, FA was reduced in the internal capsule, corona
radiata, genu of the corpus callosum and frontoparietal association
tracts. In patients with MSA, there was an extensive cluster of reduced
FA within pontocerebellar tracts and the cerebellar white matter. There
was a further smaller cluster in the left occipitotemporal region. There
were no clusters of reduced white matter FA in PD patients.
Conclusions: Our ndings demonstrate widespread damage to white
matter tracts in PSP with bilateral degeneration of motor projection
tracts and commissural and association bres within the prefrontal
cortex. In contrast, white matter damage was conned to pontocerebellar projections in MSA patients, with none identied in patients with
PD. This study suggests that whole brain analysis of white matter
damage may be a useful diagnostic marker in these disorders. In
addition the extent of prefrontal white matter damage in PSP suggests
that disconnection of key cortical regions may contribute to cognitive
dysfunction.
461
Functional 3 Tesla MRI for target identication before deep brain
stimulation (DBS)
T.M.-L. Loennfors-Weitzel, C. Kiefer, C. Ozdoba, A. Kaelin-Lang,
A. Stibal (Bern, Kanton Bern, Switzerland)
Objective: Our aim is to improve neuroimaging methods for individual targeting based on morphological MRI combined with functional MRI (fMRI).
Background: DBS is an expanding sector of functional neurosurgery
and has been well established as a therapy for Parkinsons disease.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

S141

However, the current neuroradiological methods do not always enable

a unequivocal identication of a particular target. Individual variations
and disease associated degeneration complicate target denition.
Methods: 5 right-handed volunteers were examined at 3 Tesla Trio
(Siemens Medical Solutions, Erlangen, Germany) with high-resolution
T2-3D MRI for morphological imaging. The fMRI was performed 4
times in succession with a self-initiated motor task of the hand in a
block-paradigm. We rst performed a paradigm with nger tapping,
and secondly a paradigm with wrist rotation involving tense proximal
extremity musculature.
Results: The 3 Tesla MR imaging allowed a reliable identication of
the nucleus subthalamicus, the globus pallidus internus and the main
thalamic targets in all subjects. FMRI visualised summational functional activation of particular targets, notably in Globus pallidus internus, Nucleus ventrointermedius and Nucleus subthalamicus during
wristrotation.
Conclusions: The multimodal approach with 3Tesla MRI enables an
exact targeting by direct visualization of the individual morphology and
by including an estimation of functional activation. This multimodal
approach could improve the selection process and the postoperative
outcome after DBS.
462
Diffusion tensor MR imaging for evaluation of fronto-subcortical
neural pathway changes in Parkinsons disease with dementia
M.Y. Park, H.U. Kang, G.Y. Jung (Daegu, Kyungbuk, Korea)
Objective: The purpose of this study was to evaluate the frontosubcortical neural pathway changes in PDD patients by using diffusion
tensor magnetic resonance imaging (DTI).
Background: The prevalence of the Parkinsons disease with dementia (PDD) is more than 40% in Parkinsons disease (PD) patients. It is
known that clinical chracterization of PDD is frontal subcortical dysfunction, and different from that of Alzheimers disease. However,
there has not been reported abnormalities of frontal subcortical changes
in PDD patients.
Methods: Six PDD patients, six PD patients and six normal control
group were enrolled, and the fractional anisotrophy (FA) of eight
regions of interest (ROIs: globus pallidus, white matter of frontal,
parietal and temporal lobe, the genu and splenium of corpus callosum,
pericallosal area, and caudate nucleus) were evaluated.
Results: Signicant differences of FA in the pericallosal area (F1)
and frontal white matter (F2) and globus pallidus were observed among
the three groups (p0.05).
Conclusions: Reduced FA in the frontal (F1, F2) white matter and
globus pallidus suggests the neural pathway changes from the basal
ganglia to frontal subcortical areas in PDD patients. And this also
suggests dementia in PD, which is characterized by frontal lobe dysfunction, may be attributable to nigro-striato-cortical pathway abnormality.
463
Unexpected MRI changes in younger carriers of premutation in the
FMR1 gene
L.D. Litewka, D.Z. Loesch, M. Cook, E. Storey, F. Tassone (Fitzroy,
VIC, Australia)
Objective: The aim of the study was to ascertain if neurological or
MRI changes may occur in younger premutation carriers.
Background: The fragile X-associated tremor/ataxia syndrome (FXTAS) is a new late-onset disorder caused by a premutation in the FMR1
X-linked gene, where intention tremor/ataxia and cognitive decline are
associated with characteristic changes on magnetic resonance imaging
(MRI).The earlier ndings indicate that FXTAS may be a signicant
cause of tremor/ataxia in older males.

FIG. 1 (463).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S142 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: Comprehensive MRI investigations were conducted in an
unbiased sample of ve FMR1 premutation carriers aged between 35
and 52 years, ascertained through their affected relatives. MRI volumetric protocols for the hippocampus, cerebrum and cerebellum consisted of two principal methods: 1) a region-of-interest approach, and 2)
a semi-automated extraction of intracranial structures based on the use
of morphological matrix operations on binary data.
Results: None of the participants showed any neurological or cognitive impairments. However, two (aged 30 and 52) showed typical
MRI changes manifesting as symmetrical T2 hyperintense signals
within the middle cerebellar peduncles, and one (aged 49) had periventricular white matter changes.The results of MRI volumetric measures
in each carrier, and the means from normal controls in corresponding
age categories showed a striking reduction in cerebellar volume, and he
also had the most typical changes of MCP involvement.
Conclusions: This study, based on a small sample of non-selected
carriers of a premutation in the FMR1 gene, reveals a discrepancy between
obvious MRI changes and a lack of clinical and neuropsychological
impairments. This is important for early diagnosis of such carriers, and also
suggests that the true penetrance of premutations in the FMR1 gene may
have been underestimated if this described endophenotype is considered.
The sole presence of characteristic MRI anomalies may represent either the
initial stages of FXTAS, or a subclinical form of fragile X associated
neurodegeneration manifesting only by MRI changes, which we term
FXN. Apart from the implications of our ndings for early treatment and
preventative measures, they have an impact on the studies of penetrance of
the FMR1 premutation alleles.

464
Gray matter abnormalities in dementia with Lewy bodies (DLB)
and Parkinsons disease with dementia (PDD)
C. Sanchez-Castaneda, B. Ramirez-Ruiz, R. Rene, J. Gascon,
M. Calopa, J. Campdelacreu, M. Juncadella, C. Junque (Barcelona,
Spain)
Objective: The purpose of this research was to compare DLB and
PDD patients in both their neuropsychological characteristics and brain
structure.
Background: There is controversy regarding whether DLB and PDD
are in fact different manifestations of the same disorder.
Methods: We examined 40 subjects (11 patients with DLB, 13
patients with PDD and 16 control subjects; see table 1). MRI data
were acquired on a 1.5 T Philips Master Intera machine. We
obtained 110 overcontiguos slices 1.3 mm thick (TR: 40 ms; TE:
1.79 ms; ip angle: 35; voxel size: 0.98 0.98 1.3 mm).
Voxel-based morphometry and statistical comparisons were performed according to previously described methods (Woermann,
1999). Each scan was spatially normalized, segmented into the
tissue classes, modulated with the Jacobian determinants, and
smoothed with an 8 mm isotropic Gaussian kernel. Data modulated
by the Jacobian determinants were used to assess gray matter
volume differences. The thresholded for statistical signicance was
set at puncorrected 0.001.
Results: A voxel-wise SPM analysis of cortical gray matter, in which
each patient was compared to a distribution of healthy control subjects,
revealed signicantly abnormal regions (DLB PDD, p corrected0.047) in the medial temporal lobe (comprising hippocampus, parahippocampus and amygdala) in 54.5% of the DLB patients, whereas only
15.4% of the patients in the PDD group showed such differences (table 2,
gure 1). As well as a decrease in the cingulate gyri in a 36.4% of the DLB
patients, where none of the subjects in the PDD group have it (DLB
PDD, p corrected0.020) (table 2, gure 2).
Conclusions: Our data suggest that DLB patients showed greater
gray matter reduction in the medial temporal lobe and in the cingulated
gyri than PDD patients with a similar degree of dementia.

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (464).

Table 1. Demographic and clinical characteristics of the

sample

Sex (M:F)
Age
Education
GDS
Hoeh and Yahr
Total UPDRS-III
(max 108)
MMSE (max 30)
Duration of
illness

PDD
(n13)

DLB
(n11)

controls
(n16)

10:3
71.87.6
6.15.6
4.20.9
2.80.8

8:3
70.511.1
10.76.1
4.21
2.80.6

8:8
71.97.6
7.76.5
1.00

36.313.2
21.64.2

27.311
19.26.5

22.819.7

32.716.1

28.62

p-value
NS1
NS2
NS2
p0.0012 CNTPDD, DLB
NS2
NS2
p0.0012 CNTPDD, DLB
NS2

Values expressed as mean SD. NS not signicant. 1Pearsons

Chi-square. 2U-Mann Whitney.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Table 2. Localization of gray matter reduction detected in
individuals with DLB and PDD compared with healthy
normal subjects
Subject
DLB Group (n11)
1
2
3
4
5
6
7
8
9
10
11
Total
PDD Group (n13)
1
2
3
4
5
6
7
8
9
10
11
12
13
Total

Medial temporal lobe

Cingulate gyri

Y
N
N
N
Y
Y
N
Y
Y
Y
N
6 (54.5%)*

N
N
Y
Y
N
N
N
N
Y
Y
N
4 (36.4%)

N
Y
N
N
N
N
N
N
Y
N
N
N
N
2 (15.4%)*

N
N
N
N
N
N
N
N
N
N
N
N
N
0 (0%)

S143

Conclusions: Preliminary data from this ongoing multi-center study

suggest that L-dopa and pramipexole do not have a short-term effect on
DAT imaging in early PD patients. Additional subjects will be studied
to answer this question more denitively.
466

Group comparison were performed by Pearsons Chi-square,

*P0.05. Y reduction; N not reduction.
465
InSPECT: Investigating the effect of short-term treatment with
pramipexole or levodopa on [123I]-CIT and SPECT imaging
D.L. Jennings, R. Tabamo, J.P. Seibyl, K. Marek (New Haven,
Connecticut, USA)
Objective: To evaluate the effect of short-term treatment with
pramipexole or levodopa on dopamine transporter (DAT) imaging
using [123I]-CIT and SPECT.
Background: DAT imaging has developed as a tool to monitor the
progressive loss of DAT density in PD. Two studies comparing levodopa with dopamine agonists (CALM-CIT and REAL-PET) have demonstrated slowing in the rate of loss of [123I]-CIT (40% over 4 years)
or [18F]Dopa (30% over 2 years) uptake in early Parkinsons disease
(PD) patients. The ELLDOPA study showed an increased rate of loss
of [123I]-CIT uptake in patients treated with 600 mg l-dopa compared
to placebo, over the 9-month study period. While these data suggest
that l-dopa may increase the rate of loss of dopaminergic neurons in
PD, alternatively pharmacologic mechanisms that may regulate the
DAT might inuence the results of these studies. Resolving this controversy is critical to the design of future studies using DAT imaging.
Methods: In this multi-site study, subjects undergo baseline [123I]CIT SPECT imaging and randomization to l-dopa (600 mg),
pramipexole (3 mg) or no treatment. Following 12 weeks of treatment
a second scan is completed. After an 8-week withdrawal from treatment
a third scan is obtained. Uptake of [123I]-CIT in scan 2 compared to
scan 1 determines the short-term effects of l-dopa or dopamine agonist
on the DAT imaging outcome measure.
Results: 112 subjects completed baseline imaging. The mean age at
baseline is 64.9 years (range 30 86) and mean duration of PD from
date of diagnosis is 12 months. The mean total UPDRS at baseline is
29.7, mean motor UPDRS is 20.4, median Hoehn and Yahr is 2. The
mean % change (SD) in striatal [123I]-CIT uptake between baseline
and 12 weeks (on treatment) was 1.22 (10.55) for L-dopa (n31), -2.03
(8.71) for pramipexole (n33) and -1.53 (9.38) for untreated (n30).
There was no signicant change in uptake from baseline to 20 week in
any treatment group.

Cerebral activation patterns in psychogenic paralysis

M. Van Beilen, B.M. de Jong, E.W. Gieteling, K.L. Leenders
(Groningen, Netherlands)
Objective: To assess cerebral activation patterns during performances and imagining of movements in conversion paralysis en fakers.
Background: Conversion paralysis involves an unconscious psychological mechanism which results in the inability to move. Although no
structural abnormalities are found, functional imaging studies have
postulated the hypothesis that representations of motor action might be
inhibited by abnormal activation of the striato-thalamo-frontal brain
regions. The gyrus cinguli anterior and orbitofrontal regions appear to
be of particular interest in these patients.
Methods: Cortical activity of conversion paralysis patients was measured with functional magnetic resonance imaging (3T fMRI). During
scanning both performance and imagining of standardized exion and
extension movements of the wrist was randomised for both left and
right. 8 patients and 12 fakers who imitate conversion paralysis were
investigated. 18 healthy controls are studied previously. Statistical
analyses was performed with SPM2.
Results: Gyrus cinguli anterior was commonly activated in conversion patients compared to healthy controls during movement. Also,
different activation patterns were found for fakers and conversion
paralysis patients.
Conclusions: Conversion paralysis is characterized by abnormal
activation patterns during movement, compared to healthy controls and
fakers who imitate conversion paralysis. Abnormal activation patterns
may be specic for conversion paralysis and not for fakers.
467
Camptocormia in Parkinsons disease: Magnetic resonance image and
near-infrared spectroscopy (NIRS) study of paraspinal muscles
K.-Y. Murata, K. Hama, T. Kihira, H. Miwa, T. Kondo (Wakayama,
Japan)
Objective: The aim of this study was to identify features of Parkinsonian camptocormia, and to clarify the main physiological mechanism
of patients with Parkinsons disease who develop camptocormia.
Background: Camptocormia, characterized by extreme forward exion of the thoracolumbar spine and severe stooping in the supine
position, seems to be prevalent in Parkinsons disease. The conditions
principal organic aetiologies include affections of the central nervous
system (of which the most common are Parkinsons disease and amyotrophic lateral sclerosis), muscle disease (muscular dystrophy, nemaline myopathy, inclusion body myositis) and other disordres.
Methods: An extensive range of clinical, biochemical and imaging
data were gathered for 36 patients with Parkinsons disease with or
without camptocormia. We analyzed the volume and fatty inltration of
the paraspinal muscles using magnetic resonance imaging (MRI). We
also used near-infrared spectroscopy (NIRS) to measure tissue blood
volume and its oxygenation in the paravertebral muscles at the L3 level
in different trunk postures (exed at 20 degrees, neutral posture of the
lumber spine). Aged-matched controls were also analyzed with the
same MRI and NIRS studies.
Results: Camptocormia occurred in severe Parkinsons disease with
axial predominance and motor uctuations. The MRI study showed
paraspinal muscle atrophy, especially longissimus muscles, and severe
fatty inltrations in 40% of Parkinsons disease compared to 20% of
normal controls. Total-Hb, Oxy-Hb and oxygen saturation of the
paraspinal muscles in Parkinsons disease patients with normal muscle
tonus increased when lumber spine was extended to neutral posture
from exed at 20 degrees.
Conclusions: Various mechanisms may contribute to the development
of Parkinsonian camptocormia. Our study show that exed posture of the

Movement Disorders, Vol. 22, Suppl. 16, 2007

S144 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

trunk could induce muscle hypoxic conditions, which lead to progressive
muscle atrophy and fatty inltrations, resulting camptocormia. The screening and early management of paraspinal musles is likely to be important
for preventing camptocormia in Parkinsons disease.
468
Phenotype and genotype driven changes of basal ganglia structure
in primary dystonia
B. Draganski, S.A. Schneider, K. Stefan, K.P. Bhatia,
R.S.J. Frackowiak (London, United Kingdom)
Objective: The aim of the present study is to test the hypothesis
supporting the existence of a unique pattern of structural brain abnormalities in primary dystonia depending on the inheritance mode (i.e.
DYT1 gene status) and the presence of abnormal behaviour (e.g.,
involuntary movements).
Background: Challenging the assumption of normal brain structure
in primary dystonia, recent morphometric studies have described cortical as well as subcortical structural grey and white matter alterations
in dystonic patients.
Methods: The morphometric analysis on 12 clinically manifesting and
11 asymptomatic DYT1 gene mutation carriers, 13 DYT1 negative lateonset idiopathic dystonia patients and 37 healthy controls was carried out
using voxel-based morphometric (VBM) analysis of high-resolution T1weighted and diffusion tensor magnetic resonance images.
Results: We demonstrate in a whole brain analysis a signicant
interaction between genotype and phenotype in DYT1 mutation carrying dystonic patients consisting of structural change restricted to the
posterior part of the putamen and internal globus pallidus bilaterally.
Exploring the simple effects underlying the interaction we found expanded grey matter density of basal ganglia regions in asymptomatic
DYT1 carriers compared to healthy subjects and in late-onset idiopathic compared to DYT1 mutation carrying dystonic patients. Additionally, we observed a differential effect of disease duration on basal
ganglia structure in the two cohorts of symptomatic patients. The
manifesting DYT1 mutation carriers demonstrated a negative correlation between grey matter density and disease duration, opposed to a
positive correlation in late-onset idiopathic patients. Using fractional
anisotropy (FA) as a measure of axonal bre coherence we found a
correlate of the interaction between genotype and phenotype in frontostriato-pallidal connections bilaterally.
Conclusions: The present results in primary dystonia allow the
assumption that a mutated DYT1 gene alterates phenotypic expression
of dystonic symptoms as manifested in brain structure. We suggest that
this is the result of a dynamic process based on gene dependant
modulation of the capacity for plastic change at different stages of brain
development that in turn determines clinical expression.

470
Myocardial 123I-MIBG scintigraphy is a sensitive tool with which
to differentiate Lewy body disorders from the other neurodegenerative disorders
K. Kashihara, M. Nakashima, M. Ohno, S. Kawada, T. Imamura
(Okayama, Japan)
Objective: To evaluate the usefullness of myocardial 123I-MIBG
scintigraphy to differentiate Lewy body disorders from the other neurodegenerative diseases.
Background: Evidence indicates that cardiac uptake of 123I-MIBG is
reduced in idiopathic Parkinsons disease (IPD), dementia with Lewy
bodies (DLB), pure autonomic failure (PAF), and idiopathic REM sleep
behavior disorder (RBD). These diseases are known to show Lewy bodies
and Lewy neuritis inside nerve cells. Therefore, the reduction may occur in
common with diseases presenting Lewy body pathology.
Methods: We investigated cardiac uptake of 123I-MIBG in 186 patients
with IPD, 37 DLB, 12 PAF, 4 RBD, 15 multiple system atrophy (MSA),
11 progressive supranuclear palsy (PSP), 9 corticobasal degeneration
(CBD), and 34 controls. Planar images of the chest were obtained after 15
min for the early image and after 4 h for the delayed one. Average counts
per pixel in planner images of the heart and mediastinum were used to
calculate the heart-mediastinum (H/M) ratio. We also reviewed the reports
of myocardial 123I-MIBG scintigraphy results on patients with familial
Parkinsons diseases with or without Lewy body pathology.
Results: Resulting H/M ratios were signicantly low in patients with
IPD, DLB, PAF, and RBD as compared with rates for MSA, PSP,
CBD, and the controls (Fig.1). H/M ratios reportedly are low in Park1
which has Lewy body pathology, whereas they are not low in Park2
which shows no Lewy bodies.
Conclusions: Myocardial 123I-MIBG scintigraphy may be a sensitive
tool with which to differentiate neurodegernerative diseases with Lewy
body pathology from ones without Lewy bodies.

469
Magnetic resonance imaging in Wilsons disease
M. Behari, J. Garg, G. Shukla, S. Singh, V. Goyal (New Delhi, India)
Objective: To characterized neurospectral pattern with absolute
quantication of metabolites and its clinical correlation in symptomatic
and pre-symptomatic Wilsons disease (WD) patients and compare
with age and sex matched healthy controls.
Background: The potential of magnetic resonance spectroscopy (MRS)
in providing clinically useful pathochemical information in (WD) patients
is in its infancy. Though there is some information on MRS of ganglionic
region, information on cortical and brainstem regions is scanty.
Methods: Volume localized proton MRS of left basal ganglia, right
frontal region and pons was performed in 23 WD patients, three
presymptomatic patients and eight age matched healthy controls.
Results: Signicant reduction of absolute values of NAA and choline in
basal ganglia (p0.000) and frontal region (p0.001) in symptomatic patients
compared to pre-symptomatic patients and control patients was observed.
Conclusions: MRS reveals sub-clinical involvement of basal ganglia,
cortical and other brain areas in absence of clinical signs and symptoms. It can be utilized to evaluate pre-symptomatic or asymptomatic
siblings of WD patients.

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (470).

471
Brain perfusion SPECT analysis using NEUROSTAT in multiple
system atrophy a comparison between MSA-C and MSA-P
Y. Osaki, Y. Morita, N. Akagi, M. Fukumoto, T. Kuwahara, C. Mori,
Y. Doi (Nankoku, Kochi, Japan)
Objective: The objective was to compare SPECT scans between
MSA-C and MSA-P and to investigate whether cases with MSA-C
without parkinsonism in their early disease course present hypoperfusion in basal ganglia.
Background: Most patients with MSA-C eventually develop parkinsonism in their disease course. In order to have earlier correct diagnosis
of MSA, brain perfusion SPECT scans are particularly helpful if they
can detect any abnormality in basal ganglia when patients with MSA-C
do not yet show parkinsonism.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

S145

Methods: The subject comprised 14 patients, including eight patients

with MSA-C and six patients with MSA-P, who underwent brain
perfusion SPECT scan within three years after onset of the disease. In
MSA-C, the mean age at onset and the mean disease duration were 55.4
years old and 8.5 years, respectively. All patients with MSA-C had the
SPECT scan when they did not have parkinsonism. Whereas in
MSA-P, the mean age at onset and the mean disease duration were 64.5
years old and 7.8 years, respectively. Four of six patients with MSA-P
showed mild cerebellar sign(s) at the time of SPECT scan. We performed anatomical standardisation and comparison to normal control
using NEUROSTAT. Then we analyzed Z scores in putamina, and
considered hypoperfusion was present if Z score was larger than 4.
Results: Seven of eight patients with MSA-C presented hypoperfusion
in either putamen when they did not have parkinsonism. The follow up
showed that ve of them later showed clinical parkinsonism. On the other
hand, four of six patients with MSA-P presented cerebellar hypoperfusion.
Conclusions: This method may be helpful to have earlier correct diagnosis of MSA. However, as putamen locates in deep brain, careful interpretation is needed to avoid errors during anatomical standardisation.

Results: In 16 subjects (21%), TCS was unsuccessful due to insufcient

acoustic temporal bone windows. The mean area of bilateral SN hyperechogenicity was signicantly greater in the PD patients than that in the
controls (0.68 0.43 and 0.05 0.19 cm2, respectively, p 0.01). The
mean ratio of the area of bilateral SN hyperechogenicity to the transverse
midbrain area was also greater in the PD patients than that in the controls
(0.15 0.09 and 0.02 0.04, respectively, p 0.01). In the PD patients,
the area of SN hyperechogenicity and its ratio to the individual midbrain
area were correlated with the PD duration (r 0.526 and r 0.536, p
0.01, respectively) but not with the age, UPDRS motor scores or H-Y
stage. There was no difference in the SN hyperechogenicity between the
tremor-dominant, akinetic-rigid and mixed-type PD patients.
Conclusions: Midbrain TCS is an effective diagnostic tool for detecting PD in the Korean population, even in the preclinical stage.
However, it does not reect the severity or phenotypes of parkinsonism. The relatively high failure rate due to an insufcient acoustic
temporal bony window may be a limitation to the broad application of
TCS in Asian patients. To enhance the validity of TCS, further standardized data for Asian populations is needed.

472
Test re-test reliability of ALTROPANE SPECT in Parkinsons
disease patients
K. Marek, D. Jennings, G. Zubal, J. Seibyl (New Haven,
Connecticut, USA)
Objective: To assess the test-retest reliability of ALTROPANE
([123I]-E-IACFT or [123I]-2-carbomethoxy-3-(4-uorophenyl)-N-(3iodo-E-allyl) nortropane), a radiotracer targeting the dopamine transporter.
Background: During the past decade, in vivo neuroimaging targeting
the dopamine transporter (DAT) has emerged as a reliable diagnostic
tool for Parkinsons disease (PD) and related parkinsonian syndromes
(PS). ALTROPANE is under development as a tool for PD diagnosis. It has the advantage of rapid kinetics enabling imaging within 40
minutes of injection. ALTROPANE has the potential for quantitative
assessment of dopamine transporter density.
Methods: Eleven PD patients mean age 65.1 yrs, disease duration 5.2
yrs, total UPDRS 27.8, treated with carbidopa and/or dopamine agonists underwent ALTROPANE SPECT. Images were acquired on a
Picker PRISM 3000XP, LEHR collimators following 8 mCi injection
of ALTROPANE . Images were reconstructed using 1, 2, or all 3
camera heads to simulate effects of lower injected dose on the outcome
measure. Time-activity data were analyzed to determine the uptake and
washout of specic striatal counts. Striatal binding ratios were obtained
during a period of secular equilibrium occurring between 13 and 40
min post injection. In this ongoing study eight subjects have undergone
repeat Altropane SPECT.
Results: The mean ALTROPANE striatal:occipital ratio for these PD
subjects was 1.34 (0.07 SD). Studies assessing the performance of Altropane in either two or three heads of the Picker PRISM showed no difference
in the ALTROPANE striatal:occipital ratio. Test re-test comparison
demonstrated a 4.9% (1.2% SD) change in scans 1-3 weeks apart.
Conclusions: In this ongoing study, data demonstrates that ALTROPANE uptake is reduced in Parkinson disease patients with moderate
PD with excellent test re-test reliability. ALTROPANE may be a
useful tool to assist in PD diagnosis and to monitor PD progression.

474
Dopa responsive sub-acute parkinsonism with pallidal T1 hyperintensity on MRI of brain
M. Molaie, N. Molaie (San Pedro, California, USA)
Objective: Evaluation of L-Dopa therapy in parkinsonism associated
with liver failure in a single case study.
Background: In chronic liver failure, manganese and copper accumulations in the basal ganglia are most likely reponsible for parkinsonian features observed in some of the patients (1). However, the
differential diagnosis from the ideopathic Parkinson disease includes
lack of response to L-Dopa therapy.
Methods: We observed rather rapid development of parkinsonian features in a 44 year old man with history of liver cirrhosis. Cognitive
impairment, bradyphrenia, braykinesia, and high amplitude asymmetrical
predominently action tremore were the high lights of his parkinsonism. He
had signicant elevation of serum manganese level of 6.9 ng/ml (normal
range 0.0 to 0.9 ng/ml). MRI of brain with and without contrast studies
showed symmetrical non-enhancing dense increased signal intensity of
bilateral globus pallidus on T1 weighted images (g 1).
Results: Treatment with moderate dosage of L-dopa, 400 mg a day,
improved both motor and non-motor manifestations of parkinsonism
within few days along with improvement of liver function tests. On a
trial basis, L-dopa was temporarely discontinued, and the return of
motor symptoms and signs were observed, followed by bradyphrenia in
period of few days. He has remained on maintenece L-dopa therapy
with minimal parkinsonian symptoms.

473
Midbrain transcranial sonography in Korean patients with Parkinsons disease
J.Y. Kim, W.Y. Lee, S.T. Kim, S.H. Jeon, E.J. Chung, W.T. Yoon
(Seoul, Republic of Korea)
Objective: To investigate the validity of transcranial sonography
(TCS) in Korean Parkinsons disease (PD) patients and its correlation
with the clinical features.
Background: TCS is potentially useful for the diagnosis of PD. However, studies on TCS have so far been restricted to European populations.
Methods: We carried out midbrain TCS in 43 PD patients and 35
normal controls and evaluated the area of the substantia nigra (SN)
hyperechogenicity and its ratio to the area of the whole midbrain.

FIG. 1 (474).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S146 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Conclusions: This case illustrates that some the patients with parkinsonism associated with liver failure respond to L-dopa therapy
similar to ideopathic parkinson disease. Magnesium and copper accumulation affecting nigro-striatal projections may be responsible for
L-dopa responsiveness of these patients.
1-Klos KJ, Ashlskog JE, KumarN et al: Brain metal concentrations
in chronic liver failure patients with pallidal T1 MRI hypersensitivity.
Neurology 2006;67:1984-1989.
475
Assessment of the feasibility of midbrain sonography in a population-based study
H. Stockner, K. Seppi, S. Kiechl, C. Schmidauer, J. Schwaiger,
M. Sawires, J. Willeit, W. Poewe (Innsbruck, Austria)
Objective: Assessment of the prevalence of adequate acoustic temporal bone window for TCS investigation in a population-based cohort
aged 55-94 years.
Background: Transcranial sonography (TCS) shows characteristic
increases in the area of substantia nigra (SN) in 90% of patients with
Parkinsons disease (PD). Midbrain hyperechogenicity was also seen in
10% of healthy controls and has been suggested as a potential riskmarker for PD in these subjects. To apply TCS of the midbrain in a
population screening, it is important to establish the feasibility of this
method.
Methods: A population-based cohort of 562 caucasian men and
women aged 55-94 years (Bruneck study) underwent TCS investigation
(2.5MHz,GE) from both sides using the acoustic temporal bone window. For the purpose of the present study, the acoustic window was
categorized as adequate(1) or inadequate(0) by the quality of TCS
image: adequate was dened as clear demarcation - inadequate as
absence of clear demarcation of midbrain strucutures.
Results: 84.5% of total individuals were found to have an adequate
acoustic temporal bone window. There was no difference between right
and left side in both groups. The prevalence of an inadequate window
was signicantly increased in females (22.6%) compared to males
(5.4%)(p0.01). There was a signicant increase of inadequate acoustic window with age in both genders: the proportion of subjects with
inadequate acoustic window increased in male subjects from
1.8%(55-64 years) to 12%(85-94 years) and in female subjects from
6%(55-64 years) to 45.8%(85-94 years).
Conclusions: This is the rst study to assess feasibility of TCS
specically for midbrain structures in a population-based cohort. In
15.5% of the individuals the echogenic signal of SN was not detectable
because of an inadequate acoustic bone window. Similar to studies
performed by conventional transcranial doppler sonography this study
showed an increase of inadequate window with age, in females greater
than in males. TCS has been proposed as a screening tool for PD risk,
but its application may be limited due to an inadequate window,
especially in postmenopausal women. One explanation may be hyperostosis that is thought to affect the transmission of ultrasound due to an
increase in the density of inner table of the skull.
476
Substantia nigra T2 measurements in PD patients and healthy
controls
K.J. Schweitzer, A. DiSanto, T. Brussel, J. Godau, D. Berg, U. Klose
(Tuebingen, Germany)
Objective: A multi-slice study was performed with high spatial
resolution, which allows the examination of differences within the
T2-distributions of affected volumes around the substantia nigra.
Background: The substantia nigra of Parkinsons disease (PD) patients accumulates ferritin, but several studies on the expected reduction of T2, T2* or T2 within the substantia nigra showed inconsistent
results.
Methods: 13 PD patients (mean age: 54 years (y) 5 y, mean
disease duration: 8 y) and 15 healthy controls were measured with a
triple-echo turbo spin echo sequence at a 1.5T scanner (Avanto, Siemens, Erlangen, Germany, TE 18, 98, 160ms, TR 6800 ms).

Movement Disorders, Vol. 22, Suppl. 16, 2007

T2-maps were calculated using the rst and second echo images.
Irregular ROIs were marked on successive slices by surrounding the
area of an increased signal decay near the substantia nigra as identied
in maps of the quotient of the second and rst echo after interpolation
of the data to a 0.3*0.3*2 mm3 resolution (Fig.1). In further evaluations, circular ROIs (diameters of 5mm / 15 mm) were drawn centered
within the substantia nigra in a representative slice.
Results: In Fig. 2a the histograms of T2-values for both groups and
for the three chosen ROIs are shown. Additionally the relative cumulative histograms are given in Fig. 2b. The number of pixels with
T2-values lower than 80 ms are only slightly increased in the patient
group for all chosen ROI shapes. A signicant difference was found in
the number of pixels in the irregular regions in patients compared to
controls.
Conclusions: The expected increased number of pixels with reduced
T2 values could be shown, but it was not signicant in all three selected
ROIs. The largest difference was found to be the size of the irregular
ROIs drawn on quotient maps, which highlight the effect of reduced
T2-values. The extent of the iron-containing area seems to be larger in
the PD patient group vs. healthy controls. Therefore, rather the extended region of inreased iron levels than the amount of iron itself
seems to be specic for PD.

FIG. 1 (476).

477
The occurrence of typical MRI ndings in progressive supranuclear palsy and multiple system atrophy: A retrospective study
Z. Chovancova, P. Hlustik, P. Kanovsky (Olomouc, Czech Republic)
Objective: To evaluate the neuroimaging results in patients with PSP
and MSA for typical signal changes and signs reported to be associated
with these diseases.
Background: Conventional MRI is usually normal in patients with
Parkinsons disease, whereas it is believed to show characteristic abnormalities in patients with other parkinsonian syndromes. This fact
provides the potential for using objective neuroradiological criteria in
the differential diagnosis of Parkinsonian syndromes.
Methods: MRI examinations of the brain were retrospectively assessed in 7 patients (5 females, 3 males: aged 59-71, mean 65 4.2
years) suffering from PSP (4 patients) and MSA (3 patients). The
diagnosis was conrmed with clinical, laboratory, electrophysiological
and CSF ndings. The areas of midbrain tegmentum and pons were
particularly inspected and the following typical MRI signs were
sought: morning glory sign, hot cross bun sign, panda bear face
sign and standing penguin silhouette sign, and also hypo- or hyperintensities in the posterolateral putamen (in T2 and FLAIR images).
Results: In all the patients with PSP, we have found the standing
penguin silhouette, only in one patient with PSP we have observed the
morning glory sign. In contrast, all patients with MSA had hypo- and
hyperintensities in the posterolateral putamen in T2 and FLAIR scans.
The hot cross bun sign and panda bear face sign were not found in
any patient.
Conclusions: The presence of midbrain atrophy seems to be a typical
neuroimaging feature (standing penguin silhouette) in PSP. This
standing penguin silhouette is probably more common than morning
glory sign. Patients with MSA instead manifested signal changes in
the posterolateral putamen in T2 and FLAIR images. On the contrary,

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

the hot cross bun sign has not been found in any patient suffering
from MSA.
478
Modulation of cerebral functional interactions by cortical dopamine in health and Parkinsons disease
G. Garraux, L. Talagala, R. Carson, M. Hallett (Liege, Belgium)
Objective: To investigate the role of cortical dopamine in human
motor control.
Background: Evidence from electrophysiological recordings in Parkinsons disease patients (PDP) suggests a role for dopamine (DA) in
modulating functional cerebral interactions, particularly those involving the supplementary motor area (SMA).1 Whether this effect might
be mediated, at least in part, by cortical dopamine remains unknown.
Methods: We used perfusion functional magnetic resonance imaging
(perfMRI)2,3 to measure brain activity in 16 healthy subjects (HS) and
3 PD patients (off-medication) under 2 main experimental conditions:
a visually-triggered (VT) sequential nger movement (SFM) task and
a visual baseline task (VIS). All individuals also underwent an 18FDOPA positron emission tomography (PET) scan. Parametric images
of tracer uptake constant (Kiocc)4 were created using PMOD (www.
pmod.com). Perfusion time series and Kiocc maps were analyzed separately using SPM2 (www.l.ion.ucl.ac.uk/spm). A psychophysiological analysis interaction (PPI) analysis5 was applied to perfusion data to
test for brain regions showing a stronger coupling of their activity with
SMA during VT SFM as compared to VIS.
Results: Mean reaction time during perfMRI was 42170 and
56734 ms in HS and PDP, respectively. In HS, we found a relationship between the individual level of Kiocc in SMA and the strength of
coupling of activity between SMA and some other regions engaged in
the task, including the lateral premotor cortex contralateral to the
moving hand. Compared with HS, PDP showed signicantly lower
Kiocc uptake in SMA and weaker coupling of activity between SMA
and lateral premotor cortex during VT SFM.
Conclusions: The nding in HS provides for the rst time evidence
suggesting a role for DA in SMA in modulating functional cerebral
interactions within motor circuits involving the SMA. Preliminary
results in PDP suggest that impaired DA function in SMA may have
functional implications. This should be conrmed in a larger patient
sample.
References:
1. Williams et al. Brain 2002; 125:1558.
2. Talagala et al. Magn Reson Med 2004; 52:131.
3. Garraux et al. Neuroimage 2005; 25:122.
4. Brooks DJ, et al. Brain 1990; 113:1539.
5. Friston et al. Neuroimage 1997; 6:218.
479
Involuntary psychogenic movement increases limbic activity and
decreases voluntary movement-related activity in an fMRI study of
psychogenic movement disorder
V. Voon, N. Hattori, C. Gallea, M. Bruno, M. Hallett (Bethesda,
Maryland, USA)
Objective: To determine the BOLD activation associated with an
involuntary psychogenic movement by comparing psychogenic tremor
with voluntary mimicked tremor.
Background: Psychogenic movement disorders (PMD) are characterized by involuntary movements which are not due to a neurological
cause. Functional imaging studies of psychogenic paralysis, characterized by lack of movement, have suggested impairments in motor
initiation (dorsolateral prefrontal cortex), motor execution (caudate,
putamen) and potentially, inhibition of motor processes (medial frontal
cortex). Functional imaging studies in PMDs, characterized by positive
movements, have not yet been reported.
Methods: Patients with clinically denite PMD (Fahn and Williams criteria) with postural upper extremity tremor were included.
Patients with psychogenic symptoms at rest were excluded. Over 25
second blocks interspersed with 25 second rest periods, subjects either

S147

placed their upper extremity in a posture that triggered the psychogenic

tremor (T) or mimicked (M) the tremor symptoms in the same posture.
Scanning was performed in the 1.5T. A preliminary analysis was
performed with spm2 using the general linear model with group analysis using xed effects. The contrasts of T M and M T were
modeled.
Results: The right upper extremity (UE) of seven PMD patients (4
male; mean age 55.7 /- 23.1; 6 R handed; 6 unilateral RUE and 1
bilateral UE tremor) were scanned. Results of four patients were
excluded due to excessive motor artifact or tremor that was not sufcient prolonged. The T M contrast revealed right ventromedial
prefrontal cortex and right amygdalo-hippocampal activations. The M
T contrast revealed bilateral premotor and right cerebellar activations.
Conclusions: Involuntary psychogenic movements were associated with
the novel nding of activity in limbic regions implicated in emotion and
arousal along with impairments of the prefrontal regulation of amygdala
activation. Decreased activation of preparatory and executory voluntary
motor-related regions may reect the involvement of implicit procedural
motor regions in keeping with the loss of the sense of agency. Aberrant
limbic-motor interactions may play a role in PMD.

480
Unexpected extra-striatal 123 I-FP-CIT uptake in a case of Parkinsonism
J. Hernandez-Vara, M. Boronat-De Ferrater, C. Lorenzo-Bosquet,
F. Miquel-Rodriguez, J. Castell-Conesa, J. Alvarez-Sabin
(Barcelona, Spain)
Objective: To describe an unexpected extra-striatal dopamine transporters overexpression by using 123I-FP-CIT SPECT in a patient with
parkinsonism and atypical diseases course.
Background: Dopamine Transporters availability could be in vivo
assessed by using a wide variety of radioligands, such as 123I-FP-CIT
and SPECT techniques. The most typical nding in Parkinsons disease
and related disorders is a striatal decrease of dopamine transporters.
Cases of an abnormal and ectopic extra-striatal 123I-FP-CIT uptake
have not been reported previously.
Methods: A 78-year-old, right-handed woman with a previous history of bilateral upper limb rest tremor and bradykinesia ve years
before admission, was referred to our department with a three month
history of rapidly progressive cognitive impairment and generalized
myoclonus. Brain SPECTs with 20 mCi of 99mTc-ECD and 4 mCi of
123I-FP-CIT were performed. A Magnetic Resonance Imaging was also
performed taking into account ndings of Brain SPECTs.
Results: 99mTc-ECD: Perfusion brain SPECT showed a strongly
decreased uptake in the left temporoparietal area that partially affected
the ipsilateral thalamus, basal ganglia nuclei and parietal lobe. 123I-FPCIT: SPECT images revealed not only a decreased level of dopamine
transporters in both striatal nuclei (suggesting an organic parkinsonism)
but also a strongly and ectopic increase of 123I-FP-CIT uptake in the
left temporal lobe. The (ectopical) extremely abnormal hyperactive
focus, placed in the left temporal area, perfectly matched the lack of
99m
Tc-ECD uptake region displayed by perfusion brain SPECT. This
exceptional pattern made it necessary to dismiss a dopamine transporters expressing brain tumour, so a Magnetic Resonance Imaging was
performed. Magnetic Resonance Imaging showed a left frontotemporal
expansive lesion with signicant vasogenic oedema. Other supra and
infratentorial lesions could be displayed. Cerebral biopsy could not be
performed due to technical issues.
Conclusions: This is, to our knowledge, the rst case-report of an
abnormal extra-striatal 123I-FP-CIT uptake due to a brain tumour. So
certain cerebral malignant growths could express dopamine transporters and could be detected by using 123I-FP-CIT SPECT.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S148 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

481
Susceptibility-weighted imaging of the substantia nigra in patients
with Parkinsons disease
T. Maeda, H. Toyoshima, K. Nagata (Akita, Japan)
Objective: To investigate; (1) whether susceptibility-weighted imaging (SWI) is useful to detect the substantia nigra (SN) in patients with
Parkinsons disease (PD), (2) whether SWI is possible to evaluate the
degenerative change at the SN in patients with PD.
Background: PD becomes clinically evident when 60-70% of dopaminergic neurons in the SN are lost. Visualization of these degenerative
changes can aid in the early diagnosis and clinical assessment of PD.
However, the SN is not well dened on conventional MR images. SWI
is a method to enhance the contrast of tissue iron. It has been suggested
that the tissue iron in the SN results in several times higher contrast in
SWI than T2 star-weighted imaging (T2*WI), clinically utilized to
small and asymptomatic hemorrhage in the brain by detecting tissue
iron, and hence better denition in SWI images compared to those
acquired with T2*WI.
Methods: We recruited outcome PD patients and obtained their
clinical proles. We used 1.5 Tesla Siemens Magnetom and obtained 2
mm-thick axial SWI images of the midbrain. Volume of the SN was
measured on the computer analyzing system and statistically analyzed.
Results: SWI was performed in 37 patients. Their age was 67.0
10.5 (mean SD) and disease duration was 8.3 6.8. Hoehn-Yahr
stage (HY) was 2.7 1.1 and unied PD rating scale part 3 score
(UPDRS3) was 17.9 14.1. More than 6 slices including the SN were
given by our scanning protocol. The SN was imaged as a low signal
complex of the SN pars compacta and reticlaris, and was easily separated from relatively high signal surroundings. Tomographic artifacts
were observed in many frontal lobes and in some temporal lobes.
Therefore, the SN volume measurement has been performed in 35
patients. Total SN volume was 1112 313 l. The SN volume of the
symptomatically dominant side was 571 168 l and that of the
opposite side was 569 157 l. Total SN volume was correlate with
the increase of HY and UPDRS3.
Conclusions: SWI using 1.5 Tesla MRI devices is possible to clarify
the SN as a low signal substructure. It was suggested in our study that
total SN volume could be decreased with the clinical progression of
parkinsonism. Further studies in more PD patients and normal controls
are recommended to conrm whether SWI can be useful to estimate the
early diagnosis and clinical assessment in PD.
482
Cerebral and cerebellar activation during postural and kinetic
self-paced task in essential tremor: A f-MRI study
C. Menichetti, O. Presciutti, L. Pierguidi, W. Di Iorio, V. Rossi,
A. Rossi, P. Calabresi, N. Tambasco (Perugia, Italy)
Objective: The present study was aimed to identify cerebral and
cerebellar fMRI activation during maintained posture and complex
nger task in patients with ET.
Background: Essential tremor (ET) is the most common Movement
Disorder with a prevalence of 1.2%. Its pathophysiological mechanisms are not completely understood. A recent fMRI study demonstrated that, in patients with a postural tremor, a contralateral activation
of primary motor and sensory areas, globus pallidus, and thalamus, as
well as a bilateral activation of nucleus dentatus, cerebellar hemispheres and red nucleus occur.
Methods: Functional-MRI (f-MRI) was used to study blood oxygen
level dependent (Bold) brain signal changes associated with hand movements. Eleven patients fullled criteria for ET and 10 age-matched controls were included. Patients and controls were studied using 1.5 Tesla MR
system (Signa LX, GEMS) with epibold sequence (TR/TE 3s/40ms). For
both sides, maintained posture of the arm and a nger tapping were
separately performed, during six activation blocks (30s each block), interleaved with six rest periods (30s each period). Elaboration of data and
statistical analysis were performed using SPM2 software.
Results: Patients with ET showed increased activation in contralateral primary sensorimotor, lateral premotor cortex and supplementary

Movement Disorders, Vol. 22, Suppl. 16, 2007

areas during arm maintained posture compared to controls. During

complex self-paced nger movements, a signal increase was observed
in cerebellar hemisphere bilaterally, lateral premotor bilaterally and
supplementary motor areas was observed.
Conclusions: In ET patients more cortical and cerebellar areas are
recruited in arm postural maintenance and in performing kinetic movements. This may be the result of increasing cortico-cortical activity due
to cerebello-thalamic-cortical dysfunction. We hypothesize that different cortical motor centers are involved in the genesis of tremor and
might, therefore, play a role in cortical motor planning.
483
Diagnolstic usefulness of 3T magnetic resonance imaging in multiple system atrophy
J.-Y. Lee, J.-Y. Kim, D.G. Na, B.S. Jeon, J.-W. Cho (Seoul, Republic
of Korea)
Objective: To determine sensitivity, specicity of putaminal abnormalities on 3T MRI in distinguishing MSA-P from Parkinson disease (PD).
Background: Brain MRI has been used in differential diagnosis of
MSA. Previous studies were done using 0.5 tesla (T) or 1.5T MRI, and
showed that striatal abnormalities have relatively low sensitivity and
high specicity. Increased level of iron in the putamen is an important
biochemical abnormality in MSA, and a main contributor of MRI
ndings. So, 3T MRI is thought to show more prominent low signal
intensity of iron containing deep nuclei than 1.5T MRI. The usefulness
of 3T MRI in MSA has not yet been investigated.
Methods: Parkinsonian patients with HY stage 2.5-3 who desired or
needed MRI were scanned with 3T MRI at Seoul National University
Hospital. Diagnosis was based on the last visit by criteria of NINDS
and Quinn. Sixteen patients with possible or probable MSA-P without
cerebellar symptoms (age 44 - 77 years, mean 65 years old), and 26
patients with PD (age 44-70 years, mean 60 years old) were included
in the study. Two neuroradiologists blinded to the diagnosis independently rated putaminal abnormalities on axial T2 weighted, FLAIR, and
gradient echo (GE) images. Putaminal low signal intensity was dened
as a ratio to signal intensity of globus pallidus (GP) and red nucleus
(RN). Abnormal hyperintense rim was dened as complete or partial
obscuration, or irregular thickening of hyperintense signal in the outer
margin of putamen.
Results: The sensitivity of putaminal hypointensity in T2 weighted,
FLAIR, GE were 13%, 25%, and 31% with ratio to GP, and 50%, 56%,
and 44% with ratio to RN. In FLAIR, 25%, 98%, and 83% to GP, and
55%, 89% and 68% to RN. The sensitivity of abnormal putaminal
hyperintense rim was 50%, and of atrophy of putamen was 25%. The
specicities of all these parameters were 100%. Rating of hypointensity
as a ratio to signal of RN rather than that of GP increased the
sensitivity.
Conclusions: In this study, the striatal changes on 3T MRI was very
specic, but due to relative low sensitivity, it was not better than 1.5T
MRI in distinguishing MSA-P from PD. Hyperintense rim, the most
specic parameter in 1.5T MRI, was seen in all patients with MSA and
PD, while abnormalities of hyperintense rim in 3T MRI, the novel
pattern of abnormality, was found in 50% of MSA-p.
484
Demonstration of the neuroanatomy of the midbrain using high
eld MRI
L.A. Massey, M. Miranda, H. Parkes, S.-W. Po, T. Revesz,
J.L. Holton, A.J. Lees, T. Yousry (London, United Kingdom)
Objective: To describe the neuroanatomy of the midbrain using high
eld MRI of post mortem pathological specimens.
Background: To date conventional MRI techniques have not been
able to depict the detailed anatomy of the midbrain and even the
anatomical boundaries of well-studied regions such as the substantia
nigra (SN) remain controversial. A more detailed MRI evaluation of the
midbrain might enable better discrimination between the different
causes of neurodegenerative Parkinsonism, and the development of a
reliable surrogate marker of disease progression in these maladies. We

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

have imaged pathological specimens at high eld (9.4T) with the aim
of dening midbrain structures in more detail.
Methods: Formalin xed specimens were obtained from the QSBB
for Neurological Disorders, Institute of Neurology, UCL, London.
Half-brain sections of the midbrain were prepared which were then
imaged using a 9.4T MRI machine using a multimodal MR protocol.
Results: High resolution images showed clear identication of many
brainstem structures including the red nucleus, periaqueductal grey,
subthalamic nucleus and substantia nigra. In Parkinsons disease we
have seen signal change on T2 weighted images in the lateral portion
of the substantia nigra. Images will be displayed.
Conclusions: High eld MRI of pathological specimens at 9.4T
enables visualisation of many midbrain structures with greater clarity
than conventional MRI during life. High eld MRI will lead to a clearer
in vivo window into the pathological processes underlying Parkinsons
disease and related disorders.
485
Patterns of cerebral blood ow reduction in patients with vascular
parkinsonism: Perfusion MRI study
S.H. Kim, S.H. Lee (Chuncheon, Kangwon-do, Republic of Korea)
Objective: White matter hypoperfusion may play a part in the pathogenesis of vascular parkinsonism.
Background: Although chronic ischemia is presumed to cause the
subcortical white matter lesions, the pattern of perfusion abnormalities
and hemodynamic reserve in vascular parkinsonism, particularly within
the white matter, are not well known.
Methods: Ten patients with vascular parkinsonism, and ten agematched control subjects, underwent exogenous contrast-based quantitative perfusion MRI. Cerebral blood ow (CBF), cerebral blood
volume(CBV), and mean transit time (MTT) were calculated. Regions
of interest were placed at standard positions in the white and gray
matter and mean values of CBF, CBV, and MTT were compared
between the two groups.
Results: Mean white matter CBF was signicantly reduced in patients (13.43.8 vs 20.24.2 ml/min/100g, p0.01). Signicant reductions in CBF were seen in all white matter regions. CBF in gray
matter did not differ signicantly between groups. CBV were also
decreased in patients groups in white matter lesions (1.310.36 vs
1.710.39 ml/100g, p0.01). MTT values did not differ signicantly
between the two groups.
Conclusions: MTT values did not differ signicantly between the
two groups. A quantitative perfusion MRI showed reduced white
matter CBF and CBV in patients with vascular parkinsonism. Hypoperfusion may play a part in the pathogenesis of vascular parkinsonism.
486
[123]-FP-CIT SPECT imaging of dopamine transporters in patients with recurrent sudden falls
R. Djaldetti, M. Lorberboym, S. Yust-Katz, I. Ziv, E. Melamed
(Petah Tiqva, Israel)
Objective: To investigate the integrity of the striatal dopamine transporters using [123I]-FP-CIT single-photon emission computed tomography (SPECT) imaging in patients with recurrent sudden falls.
Background: Recurrent falls in the elderly are commonly associated
with extrapyramidal syndromes, but also in abnormalities that involve
other parts of the central nervous system. It is difcult to differentiate
between the various causes of recurrent falls, especially if extrapyramidal symptoms are part of the clinical presentation.
Methods: 21 patients who were referred to the Movement Disorders
Unit for evaluation of sudden recurrent falls, without a denite neurological diagnosis, were included in a prospective cohort study. A
thorough evaluation of the neurological signs and postural responses
was performed. SPECT with the dopamine transporter (DaT) ligand
was performed 180 minutes after injection of 185 MBq [123I]-FP -CIT
using a dual-head gamma-camera.
Results: DaT SPECT was normal in 15/21 patients (71%). Of those
73% had abnormal MRI imaging suggestive of artherosclerotic lesions.

S149

Eleven patients with a normal DaT SPECT had mild parkinsonian

symptoms. Two patients with no parkinsonian symptoms had abnormal
SPECT values. There was no correlation of the SPECT results with
patient age, fall duration, or fall frequency, and no signicant difference in the relative distribution of SPECT ndings among patients with
and without extrapyramidal symptoms or vascular risk factors.
Conclusions: The diagnosis of recurrent falls cannot be accurately
conrmed on the basis of clinical features alone. [123I]FP -CIT SPECT
imaging may be useful for determining whether or not recurrent falls in
individual patients, regardless of the presence or absence of extrapyramidal symptoms, are due to degeneration of the nigrostriatal system.
487
A comparison of white matter changes in patients with Parkinsons
disease and Alzheimers disease relative to cognitively-healthy elderly controls using diffusion tensor imaging
M.A. Trivedi, C.M. Murphy, J.M. Pagonabarrago, J.G. Goldman,
L. DeToledo-Morrell, G.T. Stebbins (Chicago, Illinois, USA)
Objective: To examine group differences in white matter (WM)
integrity in Alzheimers disease (AD) and Parkinsons disease (no
dementia) (PD) relative to cognitively-healthy older control subjects
(ONC) using diffusion tensor imaging (DTI).
Background: AD and PD are progressive neurodegenerative diseases
that appear to impact different brain regions. These neuroanatomical
changes are thought to directly inuence many of the behavioral,
cognitive, and physical disturbances that are observed in these disorders. Previous studies have reported reduced WM integrity in posterior
WM regions including medial temporal regions in AD. The few studies
examining DTI-derived WM changes in PD have reported decreased
WM integrity in corticospinal regions.
Methods: The present study included 13 patients with AD (mean
age 75, mean MMSE 21), 17 non-demented patients with PD
(mean age 70 years, mean MMSE 29), and 21 ONC subjects
(mean age 72, mean MMSE 30). DTI imaging was performed on
a 1.5T MRI scanner using an echo-planar DTI pulse sequence with
whole brain coverage. Fractional anisotropy (FA), a measure of WM
integrity, was derived from these scans, normalized to a common
template, and masked for any WM abnormalities. These images were
then entered into a second-level ANOVA to determine the linear
contrast of ONC PD AD. The analysis was followed by pair-wise
t-tests to determine regions where WM integrity was reduced in patients with AD and PD relative to the ONC group.
Results: The linear contrast analyses revealed reduced WM integrity in
medial temporal regions adjacent to the hippocampus for the ONC
PD AD linear analysis. The follow-up t-test of ONC AD revealed a
similar pattern of reduced WM integrity in the region of the left temporal
stem lateral to the hippocampus in patients with AD. The follow-up t-test
of ONC PD revealed reduced WM integrity, bilaterally, in the corticospinal tract of the PD patients relative to the ONC group.
Conclusions: These results conrm previous ndings of reduced
WM integrity in the medial temporal regions of AD patients, and in the
corticospinal tract of non-demented PD patients.
488
Cardiac sympathetic denervation in Parkinsons disease is correlated with age at onset and severity of specic motor symptoms
J.-S. Kim, K.-S. Lee, I.-U. Song, H.-T. Kim (Seoul, Korea)
Objective: To investigate prospectively the relations between myocardial 123I-metaiodobenzylguanidine (MIBG) uptake and age at onset,
disease severity and disease duration in patients with Parkinsons
disease (PD).
Background: In idiopathic PD, myocardial MIBG uptake is signicantly reduced even without apparent autonomic failure, which enables
early diagnosis of the illness. Several studies have suggested that
duration and severity inuence MIBG uptake in PD.
Methods: MIBG uptake was examined in 51 patients with PD. MIBG
uptake was assessed using the ratio of the heart to the upper mediastinum (H/M) according to planar scintigraphic data and correlated with

Movement Disorders, Vol. 22, Suppl. 16, 2007

S150 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

age at onset, age at examination, and disease severity and duration. PD
severity was assessed by the Hoehn and Yahr stage and Unied
Parkinsons Disease Rating Scale.
Results: There was a signicant negative correlation between H/M
ratio and age at onset and between Hoehn and Yahr stage. Bradykinesia, posture, gait, and other midline symptoms, such as speech and
facial expression, compared with rigidity, seem to be more closely
related to cardiac MIBG uptake. By contrast, neither the severity of
parkinsonian rest nor of action tremor is related to the degree of cardiac
sympathetic denervation as measured by MIBG scintigraphy.
Conclusions: This study suggests that late onset, specic motor
symptoms is associated with myocardial sympathetic dysfunction.
489
Nociceptive brain activation in painful patients with Parkinsons
disease
C. Brefel-Courbon, P. Payoux, F. Ory, C. Thalamas, O.O. Rascol
(Toulouse, France)
Objective: We compare pain threshold before (OFF) and after (ON)
administration of levodopa in patients with Parkinsons disease (PD)
suffering from PD related neuropathic pain and in PD patients without
pain and we assess the effect of levodopa on cerebral activity with
Positron Emission Tomography (PET) during nociceptive stimulation
in these two groups.
Background: PD patients frequently experienced painful sensations.
It has been suggested that the occurrence of painful symptoms could be
in part due to central modication of nociception in PD.
Methods: Pain threshold was determinated using thermal stimulation
(cold pressor test) during two randomized periods: OFF and ON. Using
H215O PET, we studied the brain activation on subjects while they
received alternate randomized noxious (dened as pain threshold) and
innocuous (noxious value plus 10C) cold stimuli during OFF and ON
periods. Signicance : p0.01. As control group, nine age matched
volunteers were included.
Results: We studied nine PD patients with neuropathic pain (617
years) and nine PD patients without pain (658 years). In OFF period,
pain threshold in the two groups of PD patient (with and without pain)
was signicantly lower than in controls (84C and 83C vs 44C,
p 0.03). Administration of levodopa signicantly raised pain threshold in PD patients with pain (84C vs 64C, p 0.007) and without
pain (83C vs 53C, p 0.007) but not in controls. During OFF
period, there was a signicant increase in pain induced-activation in
right insula in the two groups of PD patients compared to controls. PD
patients with pain had a signicant increase in pain induced-activation
in mesial frontal area compared with pain free PD patients. Levodopa
signicantly reduced pain induced-activation in right insula in both
groups of PD and there was no signicant difference in pain induced
activation between the two PD groups in ON condition.
Conclusions: Pain threshold was lower in PD patients with and
without pain and levodopa improved this abnormality. All PD patients,
regardless of the fact that they complained or not of neuropathic pain
had higher pain induced-activation in the lateral discriminative nociceptive pathway (insula), while only PD patients who experienced
neuropathic pain exhibited a greater activation in medial affective
nociceptive area.

NEUROPHARMACOLOGY
490
Levodopa as a rst-line treatment for the neuroleptic malignant
syndrome
G. Mihailescu, C. Mihailescu, S. Nica (Bucharest, Romania)
Objective: To prospectively evaluate the inuence on outcome of a
L-Dopa-based treatment in patients with neuroleptic malignant syndrome.
Background: The neuroleptic malignant syndrome still represents a
daily threat, mainly concerning psychiatric patients even after the
occurence of the so-called low-risk atypical neuroleptics. Its course
covers a wide range of clinical possibilities and depends on the pre-

Movement Disorders, Vol. 22, Suppl. 16, 2007

sentation pattern, including presence or absence of the most feared

complications like renal failure and rhabdomyolysis. The syndrome
bears a poor prognosis and, if left untreated, could lead to fatalities. The
treatment should be instituted as early as possible and it elicits individual responses in each patient. The classical pharmacological treatment approach didnt result so far in a dramatically improvement of the
prognosis. Recently, new data has emerged on the role played by
L-Dopa and its combinations amongst the other classes of drugs used,
including benzodiazepines, dopamine agonists and peripheral myorelaxants, conrming the physiopathological basis for the involvement of
the dopaminergic blockade.
Methods: We compared outcomes in different treatment groups used
in a series of patients with this pathology.
Results: The discussion of the cases submitted in this work accounts
for the differences in their pharmacological treatment and evolution.
The dispersible formulations, when available, seem to t better the
goals and increase the administration ease of the treatment in this
difcult-to-treat group of patients.
Conclusions: Based on the positive ndings resulted from these
recent cases, we advance the hypothesis that L-Dopa (including combinations) should be considered rst-line treatment, not only in the
most severe cases but irrespective of the severity at presentation. Thus,
together with increasing the awareness about this syndrome and the
likelihood of an early diagnosis, we could attempt to improve its
prognosis even by preventing the occurence of its complications.

491
Analgesic effect of botulinum toxin type A in experimental models
of pain
C. Favre, M. Auguet, P.-E. Chabrier (Les Ulis, France)
Objective: To gain further insight into the analgesic effect of botulinum toxin type A haemagglutinin complex we investigated the effect
of Dysport in a neuropathic pain model and in an inammatory pain
model in comparison with Botox in rats.
Methods: Male Sprague-Dawley rats were used. Neuropathic pain
was induced by four loose ligatures around the common sciatic nerve
(above the trifurcation) of the right hind paw. Inammatory pain was
induced by a sub-plantar (s.p.) injection of carrageenan (2 mg/0.1ml) in
the right hind paw. Hyperalgesia was assessed by measurement of the
withdrawal threshold of each hind paw in response to mechanical
stimulus (Randall & Selitto pressure test). In the neuropathic model,
mechanical hyperalgesia was measured before (D0) and on Day 13
(D13) after surgery. Dysport was then injected (20 U/Kg; s.p.) into
the injured paw and pressure test performed one, three and six days
after treatment. In the inammatory model Dysport (7.5 and 15 U/kg)
or Botox (5 and 10 U/kg) was injected (s.p.) three days before the
carrageenan test. Hyperalgesia was measured before treatments (D0),
prior to carrageenan administration on Day 3 (baseline) and three hours
after carrageenan injection.
Results: The neuropathic model Dysport induced a statistically
signicant analgesic effect at D3 and D6 after treatment. The inammatory model of the sub-plantar administration of Dysport or Botox
had no effect on the locomotion performance of the rats as assessed by
the rotarod test or on the withdrawal nociceptive reex of the rats on
D3 before carrageenan injection. However, both compounds induced a
statistically signicant anti-hyperalgesic effect when assessed three
hours after carrageenan injection. Statistical comparisons indicated that
the effects of 15 U/kg Dysport were not signicantly different from
those of 10 U/kg Botox but were higher than those of 5 U/kg Botox.
Conclusions: A single sub plantar injection of Dysport exhibits an
analgesic effect in experimentally-induced inammatory and neuropathic pain models. Comparison with Botox suggests a dose ratio
efcacy close to 1.5 in this inammatory model.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

492
Effect of some naturally occuring antioxidants (vitamins) in the
treatment of chronic lead intoxication-interaction Parkinsons disease in rats
N. Djebli (Mostaganem, Algeria)
Objective: The present investigation is aimed at delineating the
effect of vitamins C or E in the treatment of lead poisoning. The
purpose of this study was to explore the relations between lead levels
of brain and blood on learning and memory, and to determine the role
of vitamins on lead toxicity in brain and blood.
Background: The toxic effects of lead are well-known. The nervous
system and the heme synthesis are the two main points where the
symptoms present themselves rst. During the last two decades, advances in behavioural, cellular and molecular neuroscience have provided the necessary experimental tools to begin deciphering the many
and complex effects of lead on neuronal processes and cell types that
are essential for synaptic plasticity, learning and memory in the mammalian brain.
Methods: Male Wistar rats were exposed to 1000ppm lead acetate in
drinking water for seven weeks and treated thereafter with ascorbic
acid (vitamin C); 500mg/kg orally once daily or alpha-tocopherol
(vitamin E); 100mg/kg intramuscularly for 5 days.
Results: The effects of these treatments in inuencing the leadinduced alterations in blood, locomotor activity, stereotypic exploratory behavior, brain oxidative stress and lead concentration from
blood. Oral administration of 1000 ppm of lead acetate to young rats for
seven weeks caused a reduction in locomotor activity and stereotypic
exploratory behavior during a 20 minute testing period. This locomotor
Hypoactivity induced by lead was accompanied by a reduction in
stereotypic behavior (stress, snifng, lickings, biting and grooming)
compared to the control groups and groups treated by vitamin E.
Conclusions: These outcomes suggested that lead may interfere with
catecholaminergic and particularly dopaminergic neurotransmission. The
results showed thad lead disturb heme synthesis of exposed rats compared
to controls. However the treatment of vitamin C was effective in decreasing Pb levels in rats and help Pb detoxication. Thus, the administration of
vitamin E during treatment could be recommended with combination
therapy for achieving optium effects of chelation therapy.
493
Topiramate as a monotherapy in essential tremor. Comparison of
a new drug to prior golden standard medication
M. Arnaoutoglou, V. Costa, G. Spanos, A. Tychalas, S.A. Kapsali,
E. Avdelidou, E. Kalliolia, N. Arnaoutoglou, S.J. Baloyannis
(Thessaloniki, Greece)
Objective: The aim of our study was to evaluate topiramate efcacy
in the treatment of essential tremor and to compare it with propanolol,
which for many years was considered a gold standard medication for
essential tremor.
Background: Classical essential tremor could be described as a
monosymptomatic, predominately postural and action tremor that is
usually hereditary and tends to progress slowly over the years. When
tremor interferes with activities of daily living, medication is administered.
Methods: We evaluated 33 patients with classical essential tremor
according to the (Bain et al, 2000) diagnostic criteria. The patients were
devised into two groups: group A, consisted of fteen patients, 8 male
and 7 female, mean age 46 11, and group B consisted of eighteen
patients, 12 male and 6 female, mean age 48 6. Tremor was quantied and monitored as moderate or intense according to interference
with daily activity. Group a patients were treaded with propanolol
(60 40mg/day) whereas group B patients with low dose topiramate
(100 50 mg/day). Neuroimaging (brain MRI and DAT-scan) was
normal.
Results: Group A patients showed improvement on relatively high
daily doses (120 mg/ day), whereas group B patients reported marked
improvement on low topiramate doses (100 mg/day).No serious side

S151

effect was reported to both groups whereas symptomatic side effects of

topiramate (drowsiness, cerebellar signs) were dose dependent.
Conclusions: Topiramate appears to be an efcacious drug in essential
tremor treatment. Further investigation would suggest whether topiramate
possess a long-term efcacy, compared to 2 blockers, whose initial good
response is maintained in only 25% of patients in two years.
494
Effects of pramipexole on the motor response to levodopa infusion
in Parkinsons disease: A double-blind, placebo controlled crossover study
M.A. Brodsky, M.C. Barnard, B.S. Park, J.G. Nutt (Portland,
Oregon, USA)
Objective: To examine the effect of a dopamine agonist (DA) on the
motor response to levodopa (LD) using a 2 hour LD infusion model in
patients with Parkinsons disease (PD).
Background: Pharmacologic treatment of PD commonly includes
LD and DAs. While LD remains the most potent drug, initial treatment
with a DA has become common as this delays onset of motor complications. It is unproven if DAs enhance motor performance during ON
periods in PD patients taking LD, or if continuing a patient on a DA
once LD is started has any effect on LD-induced dyskinesias.
Methods: Subjects already on oral LD were randomly assigned to
receive either treatment with pramipexole (PPX) or placebo for 4
weeks. They were admitted to our General Clinical Research Center
(GCRC), PD medications were held overnight, and they underwent
intravenous LD infusion at 0.5 mg/kg/hr and 1.0 mg/kg/hr on subsequent days, the order of which was randomized and blinded. Motor
function was assessed at 30 minute intervals starting 2 hours prior to
LD infusion and continuing until 3 hours after LD infusion was
stopped. Subjects were then crossed over to the alternate therapy (PPX
or placebo) for another 4 weeks, and again assessed at the two infusion
rates in the GCRC.
Results: 11 subjects underwent randomization, and 9 subjects completed the protocol. Mean baseline tapping score in the OFF state prior
to LD infusion was 116.1 in the placebo group and 130.8 in the PPX
group (SE4.47), a signicant improvement (p0.0025). Estimated
area under the curve for nger tapping response to LD infusion was
125.5 in the placebo group and 142.9 in the PPX group (SE8.73,
p0.001), and motor response to LD improved with PPX irrespective
of LD infusion rate. Overall mean dyskinesia scores were greater with
PPX (1.86) versus placebo (0.46) with LD infusion (SE0.31,
p0.014).
Conclusions: Using LD infusion to precisely measure effect on
motor response, PPX enhanced the improvement of mobility seen with
LD. PPX also improved baseline bradykinesia in the OFF state. LD
infusion induced dyskinesia was greater with PPX versus placebo,
although it remained mild in all cases. Consideration should be made to
start or continue DA treatment in PD patients requiring LD therapy to
enhance motor function, weighing this benet versus the risk of worsening dyskinesia.
495
Effect of early use of amantadine on the development of motor
uctuations and dyskinesias during levodopa treatment in Parkinsons disease
A. Kishore, S. Gopinathan (Trivandrum, Kerala, India)
Objective: To examine the effect of early use of amantadine on the
development of motor complications in Parkinsons disease (PD).
Background: Currently amantadine has a limited role in the management of early stage of PD. However, its early use prior to L-dopa
treatment was not unusual in India until recently, because of issues of
cost of dopamine agonists (DA) and unavailability of newer DA. Such
cases are frequently referred to our Movement Disorders clinic for the
management of motor complications of PD.
Methods: A retrospective study, from June 2006 to December
2006, of patients with PD from the Movement Disorders clinic of a
tertiary referral hospital. Cases of idiopathic PD with age of onset of

Movement Disorders, Vol. 22, Suppl. 16, 2007

S152 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

illness less than 55 years and experiencing motor uctuations and
peak dose L-dopa-induced dyskinesias were screened and two
groups of patients were identied. Group A which had received
amantadine before starting levodopa treatment and continued it or
had received concurrent treatment with both the drugs from the start
of therapy. Group B, had patients who never received amantadine
prior to or concurrent with L-dopa treatment until they developed
motor complications. The clinical and treatment characteristics of
the patients were collected from the Movement Disorders clinic les
and supplemented by patient interviews and examination of prescriptions in all cases. Data were analyzed using ANOVA, chisquare test and Fishers exact test.
Results: Group A consisted of 40 patients (including 10 patients on
concurrent early therapy) and Group B had 50 patients. The 2 groups
did not show a signicant difference in the age of onset of illness, type
of parkinsonism, duration of levodopa treatment, mean starting dose of
levodopa or mean total dose of levodopa at the onset of dyskinesias or
uctuations (p0.05 in all comparisons). There was a signicantly
greater delay in the onset of motor uctuations (Group A mean 7.1
years 3.3, Group B mean 2.7years 2.1) and onset of LIDs in
group A (Group A mean 7.8years 3.8 , p0.0001 for both
comparisons).
Conclusions: The data from this retrospective study suggest that
amantadine may delay the onset of dyskinesias and uctuations, if
initiated prior to levodopa therapy. This observation needs to be veried in prospective, double blind, placebo- controlled trials.
496
Does an infusion of apomorphine at low concentrations inhibit
motor function in Parkinsons disease?
S.A. Gunzler, M. Pavel, C. Koudelka, N.E. Carlson, S. Crocker,
P. Kirchhoff, J.G. Nutt (Portland, Oregon, USA)
Objective: (1) To evaluate whether low concentrations of dopaminergic medication cause an inhibitory effect in patients with Parkinsons disease (PD). (2) Secondarily, to explore foot tapping rate as an
outcome measure in PD.
Background: A substantial body of preclinical research shows
that low concentrations of dopaminergic medications may inhibit
motor function in PD. Anecdotally, some patients report worsening
of motor symptoms soon after taking levodopa, or after levodopa
effect has worn off. We sought to explore the entire dose-response
to apomorphine (APO), a D1/2 dopamine agonist, in order to
determine if low plasma concentrations of drug will worsen motor
status in PD.
Methods: This was a randomized, double-blind, placebo-controlled
crossover study of 14 subjects with PD. On 3 consecutive days,
subjects received a subcutaneous placebo infusion on one day, lowdose (subthreshold) APO on a second day, and higher-dose (threshold
to suprathreshold) APO on a third day. The order of the days was
randomized. Rates of nger tapping and foot tapping were measured at
30-minute intervals. Repeated measures ANOVA was performed by
mixed models, after subtracting the preinfusion baseline tapping rate.
Results: The infusions were well-tolerated. The foot tapping task
clearly differentiated the response to high-dose APO from the response
to other infusions, with a greater motor response to high-dose APO than
to either placebo (p0.01) or low-dose APO (p0.01). The nger
tapping task showed a trend toward a greater motor response to highdose infusion than to either placebo (p0.15) or the low-dose infusion
(p0.08). There was no signicant difference in response to low-dose
infusion compared to placebo. There was a trend in some individual
patients toward a decline in motor function during low-dose APO
infusion, relative to placebo.
Conclusions: This study nds no evidence that subthreshold doses of
dopaminergic medication worsen Parkinsonism, although an inhibitory
effect may be present in subpopulations. Further analysis will be
necessary to determine if a subtle or transient decline below baseline
motor function occured with low-dose APO. Foot tapping was a less
variable and more sensitive measure of motor function in this study,
compared to nger tapping.

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (496).

497
Reversible posterior leukoencephalopathy syndrome in a patient
with multiple system atrophy: A possible association with oral
midodrine treatment
J.S. Kim, K.S. Lee, I.U. Song, H.T. Kim (Seoul, Korea)
Objective: To describe reversible posterior leukoencephalopathy
syndrome as an adverse effect associated with supine hypertension
after receiving oral midodrine treatment in a patient with multiple
system atrophy.
Background: Oral midodrine is an effective and well tolerated management option for patients with refractory orthostatic hypotension.
The major effect of midodrine is to increase standing systolic blood
pressure. Hypertensive encephalopathy following midodrine ingestion
has not been previously described.
Methods: We describe a 51-year-old man with a three year history of
multiple system atrophy who developed a hypertensive encephalopathy
after receiving prescription midodrine for therapeutic treatment of
orthostatic hypotension. Brain MR images disclosed diffuse symmetric
high signal intensities in both the cerebellum, the basal ganglia and the
parieto-occipital lobe. The possible causal mechanisms are discussed.
Results: During follow-up the supine arterial pressure dropped from
180/100 to 130/80 mmHg after cessation of the midodrine. A follow-up
MRI showed improved signal changes in the cerebellum, basal ganglia
and parieto-occipital lobes.
Conclusions: Typical reversible MRI ndings, following treatment
with midodrine, suggested a possible relationship between midodrine
treatment, supine hypertension and reversible posterior leukoencephalopathy syndrome, although a cause-and-effect relationship cannot be
conrmed.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

498
Naturalistic long term follow-up of psychotic Parkinsons disease
patients treated with either clozapine or quetiapine
C. Klein, T. Prokhorov, E. Dobronevsky, A. Miniovich, J.M. Rabey
(Zerin, Israel)
Objective: The objective of this study was to evaluate the long-term
outcome of quetiapine (QTP) and clozapine (CLOZ) use in psychotic
Parkinsons Disease patients (PPDP).
Background: The management of patients with advanced Parkinsons Disease (PD) presents a clinical challenge, since patients on
chronic dopaminergic therapy eventually manifest in addition to motor
uctuations also non-motor ones. Among them psychosis (hallucinations and delusions) is quite difcult to treat, as classical neuroleptics
are forbidden. Atypical antipsychotics have been used with variable
results. CLOZ and QTP seem to be the most appropriate considering
their effectivity and tolerance.
Methods: Thirty ve PPDP (mean age 76, mean disease duration
10.3 y; 19 of them with dementia DSM IV criteria) received QTP
(mean daily dose 9374 mg) and were followed up for 2 years. Thirty
two PPDP (mean age 73, mean disease duration 12.2 y; 14 with
dementia) received CLOZ (mean daily dose 20 mg) and were followed
for 5 years.
Results: In the QTP group, 11 patients (31%) continued treatment for
2 years (7 of them with dementia). Complete resolution of symptoms
was observed in 7 patients and partial resolution in 4. Treatment was
stopped in 24 patients: 15 (42%) due to lack of effect; 3 (9%) resolution
of symptoms; 1 severe somnolence; 3 personal reasons; and 2 patients
died for reasons unrelated to QTP. In the CLOZ group, 19 patients
(59%) continued treatment for 5 years (14 with dementia). Thirteen
patients (40%) stopped treatment: 9 (28%) because of resolution of
symptoms; 3 (9%) somnolence; and 1 for personal reasons. No correlation was found in either group between age, sex, duration and severity
of disease, presence of dementia and LD dose.
Conclusions: In this study, CLOZ was more effective and better
tolerated. However, due to the necessity of continuous blood monitoring there is still a tendency in clinical practice to rst try QTP. In
resistant patients, CLOZ is undoubtedly a useful alternative.
499
Pramipexole treatment reverses motor decits with low risk of
dyskinesia in MPTP-treated common marmosets
P. Jenner, M.J. Jackson, K. Tayarani-Binazir, S. Rose, C.W. Olanow
(London, United Kingdom)
Objective: Dyskinesia limits the use of short-acting dopamine agonists for treating Parkinsons disease whereas, long-acting dopamine
agonists such as pramipexole may reduce the risk of dyskinesia. This
study compared the effect of treatment with pramipexole and L-dopa
alone and also the effect of adding L-dopa to pramipexole treatment in
the MPTP-treated common marmoset.
Methods: Behavioural decits were induced by administration of
MPTP (2.0mg/kg sc) for 5 days. When stabilized the animals were
assigned to 1 of 3 treatment groups (n6/group); (1) L-dopa (1012.5mg/kg po BID) days 1 to 63 (2) pramipexole (0.04-0.3mg/kg po
BID-SID) days 1 to 63 (3) pramipexole (0.04-0.3mg/kg po BID-SID)
days 1 to 63 with additional L-dopa (3.125-6.25mg/kg po BID) for days
36 to 63. All animals were challenged with L-dopa (12.5mg/kg) on 3
occasions following the end of the 63 days of treatment.
Results: L-dopa treatment induced a reversal of motor decits for the
63 days with mild dyskinesia present at day 7 becoming severe from
day 21 onward. Pramipexole (0.04-0.3mg/kg) produced a similar reversal of motor decits with only mild dyskinesia from day 42 onward.
The addition of L-dopa (3.125-6.25mg/kg) to pramipexole treatment
produced a further slight reversal of motor decits, but dyskinesia,
which was greater than that of the prior pramipexole treatment, was
induced rapidly. However, this remained moderate compared to the
severe dyskinesia induced by L-dopa alone. Subsequent, acute L-dopa
challenge indicated that animals treated with pramipexole alone expressed the lowest dyskinesia.

S153

Conclusions: Pramipexole produced a good reversal of motor

decits with low risk of dyskinesia. The addition of low-dose
L-dopa treatment did not markedly improve this benet but did
produce a rapid onset of moderate dyskinesia. This suggests that the
addition of L-dopa treatment should be delayed and when introduced, the lowest possible dose should be used to minimise the risk
of inducing dyskinesia.
500
Quality of post-marketing drug surveillance EMEA regulatory
restraints and reality of drug safety monitoring exemplied with
tolcapone prescription in Parkinsons disease in Germany
M.M. Unger, W.H. Oertel, K.M. Eggert (Marburg, Germany)
Objective: To evaluate the congruency between the high standards
aspired by the European Medicines Agency (EMEA) and every-dayreality of post-marketing drug-surveillance exemplied with tolcapone
in Parkinsons disease (PD).
Background: In 1998, the EMEA suspended the approval for the
COMT inhibitor tolcapone in PD with motor complications due to
the drugs implication in fulminant liver failure and consequent
death of three patients. Clinical data obtained by ongoing use of
tolcapone in other countries proved that adequate safety can be
guaranteed under strict liver enzyme monitoring. In 2005, tolcapone
was relaunched in the EU under the prerequisite of bi-weekly liver
enzyme monitoring during the rst year of therapy. Here we report
the situation the prescribing physician faces, when trying to fulll
the EMEA demands.
Methods: 21 patients receiving tolcapone for advanced PD with
motor uctuations were enrolled in this observational study. Prior to
the rst intake of tolcapone, patients were informed about possible
side effects and the necessity of regular blood tests. If patients did
not report the results automatically, the patients and their general
practitioner were contacted and asked to perform the tests and to
provide the results. All obtainable test results were included in the
analysis.
Results: Tolcapone was well-tolerated, no severe adverse events
were reported and all patients reported a clinical benet under tolcapone treatment. Regular results (i.e. more than 2/3 of all mandatory
values) were obtainable from 8 of 21 patients. 7 of 21 patients provided
less than two thirds and 6 of 21 patients even less than one third of all
expected values (see Fig. 1).
Conclusions: Our data depicts a general problem in post-marketing drug-surveillance with an impact for physicians, manufacturers
and legal authorities: despite repeated information and close contact
to the patients and their general practitioners, only a minor part of
PD patients receiving tolcapone regularly performed and reported
the required laboratory tests, i.e. complied with the discussed safety
procedure. Our data demonstrates the incongruity between the high

FIG. 1 (500).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S154 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

standards in post-marketing drug-surveillance aspired by the EMEA
and every-day-reality. Practical, legal and ethical aspects will be
discussed.
501
The new neuroprotective agent; BN83026 reduces L-DOPA-induced dyskinesia in hemiparkinsonian rats
B. Spinnewyn, C. Charnet, S. Cornet, M. Auguet, P.E. Chabrier (Les
Ulis, France)
Objective: Chronic L-DOPA pharmacotherapy in Parkinsons disease is often accompanied by motor complications known as dyskinesia. Although the underlying mechanism of dyskinesia remains to be
claried, recent ndings suggest that excitotoxicity, oxidative stress
and mitochondrial dysfunction may play a role. Since BN83026 acts on
neuronal excitotoxicity oxidative stress and possesses mitochondrial
protective properties its symptomatic effects have been tested on LDOPA-induced dykinesia in rats.
Methods: Unilateral intrastriatal injection of 6-hydroxydopamine
(26g) was chosen as a model of Parkinsons disease (Bjorklund
et al, 1997). Four weeks after this lesion 80-90% of animals were
parkinsonian. The selected rats received a daily treatment with
escalating dose of L-DOPA plus benserazide for four weeks. During
this period, about 80% of the rats gradually developed abnormal
involuntary movements, lasting for 2-3 h following each L-DOPA
dose. Abnormal involuntary movements (AIMs) were classied in
four subtypes: locomotive dyskinesia, axial dystonia, orolingual
dyskinesia and forelimb dyskinesia. For quantication of L-DOPAinduced dyskinesia, rats were observed individually every 30 minutes from 30 to 180 minutes after the injection of L-DOPA.
BN83026 was administered orally just after the rst AIMs scoring
(30 min baseline).
Results: BN83026 markedly and dose dependently (up to 30
mg/kg) reduced the severity of dyskinesia. In the same conditions,
the NMDA-receptor antagonist, amantadine at 40 mg/kg (i.p.) reduced AIM score while the glutamate release inhibitor, riluzole at 8
mg/kg (i.p.) did not.
Conclusions: In addition to its neuroprotective properties, BN83026
may be potentially benecial to alleviate motor complications due to
L-DOPA therapy.
502
In vitro pharmacological prole of the A2A receptor antagonist istradefylline on adenosine receptors
M. Saki, K. Sasaki, M. Ichimura, T. Kanda (Shizuoka, Japan)
Objective: To evaluate the in vitro pharmacological prole of istradefylline as an A2A receptor antagonist.
Background: Long-term use of dopaminergic therapy to treat
Parkinsons disease (PD) is often limited by the development of
motor response complications. Adenosine A2A receptors, highly
expressed on medium-sized spiny neurons in the striatopallidal
(indirect) pathway, are promising non-dopaminergic targets for the
treatment of PD. Istradefylline has been reported to have antiparkinsonian activity in rodents, non-human primate models of PD, and
PD patients.
Results: We used a radioligand binding assay to investigate the
potency and selectivity of istradefylline for A2A receptors from human,
marmoset, dog, rat, and mouse. Istradefylline showed high afnity for
A2A receptors in these species and a Ki value of 12 nmol/L for the
human A2A receptor. Afnity for the A1 receptor was lower in each
species and was especially low (IC50: 1000 nmol/L) in humans.
Selectivity for A2A over A1 receptors was higher in all species, but the
difference in afnity for A2A and A1 receptors in dogs was lower than
that for the other species.Istradefylline showed no signicant afnity
for 54 other receptors. To characterize the binding of istradefylline to
the A2A receptor, we investigated the kinetics of [3H]-istradefylline
binding to human A2A receptor. Binding of istradefylline to A2A
receptor was fast and reversible (half-life, 0.32 min). Antagonistic
activity of istradefylline was determined in a model evaluating cyclic

Movement Disorders, Vol. 22, Suppl. 16, 2007

adenosine monophosphate (cAMP) accumulation in PC12 cells induced by the A2A receptor selective agonist CGS21680. Istradefylline
produced a shift to the right of the CGS21680 concentration-response
curve without affecting maximal response of the agonist and the KB
value was 0.74 nmol/L. Istradefylline alone had no effect on basal
cAMP levels.
Conclusions: Our results indicate that istradefylline is a potent,
selective, and competitive A2A receptor antagonist in some species.
Selectivity for the A2A over the A1 receptor was especially high in
humans. The in vitro pharmacological prole of istradefylline is helpful
to explain the in vivo prole of istradefylline and may be useful for
clinical pharmacokineticpharmacodynamic considerations of efcacy
and safety.

NON-MOTOR ASPECTS OF
MOVEMENT DISORDERS
503
Rest-activity rhythm and sleep-wake cycle are altered in PD patients with hallucinations
D.L. Whitehead, A.D.M. Davies, J.R. Playfer, C.J. Turnbull (London,
United Kingdom)
Objective: To assess the association between hallucinations, cognition, sleep and rest-activity rhythm breakdown in Parkinsons disease.
Background: Hallucinations affect around one-third of patients with
Parkinsons disease (PD) and have been associated with night-time
disturbances and excessive daytime sleepiness, and also with a more
rapid decline in cognitive abilities and dementia. Circadian rhythm
indexed by rest-activity rhythm is a correlate of cognitive decline and
neuropsychiatric symptoms in Alzheimers Disease, but has rarely been
assessed in Parkinsons Disease.
Methods: 77 patients with PD and 31 healthy older adults were
monitored over 5-7 days using actigraphy and sleep diaries. Mean age
of PD patients was 74.4 (7.9) years, with a mean disease duration of
5.7 (5.3) years. Circadian rhythm indices were calculated using
Non-Parametric Circadian Rhythm Analysis (NPCRA; Sleep Analysis
98, Cambridge Neurotechnology Ltd). Sleep characteristics of hallucinators (n35) were compared to those of non-hallucinators (n28) and
with controls.
Results: Hallucinators demonstrated greater levels of nocturnal sleep
fragmentation (F4.13,p.05) and daytime sleep than non-hallucinators (F5.21,p0.01), independently of disease severity. Rest-activity
rhythm variables were associated primarily with disease-related factors
disease duration and severity of motor uctuations, but were not
associated with cognitive decline. The PD group as a whole showed a
more disrupted circadian rhythm than controls, and hallucinators
showed lower interdaily stability across the period of monitoring,
independently of motor uctuations and disease duration than nonhallucinators (F4.40,p.02).
Conclusions: PD patients with hallucinations show a greater degree
of breakdown in rest-activity rhythms non-hallucinators, independently
of cognitive decline. Degeneration of the suprachiasmatic nucleus
underlies circadian rhythm dysfunction in Alzheimers disease. However it is likely that changes in the sleep-wake cycle in PD are driven
by degeneration of brain stem sleep centers as is characteristic of the
-synucleinopathies, destabilizing mechanisms which maintain steadystates of sleep and wakefulness (Saper et al, 2005). Instability in brain
stem sleep centers particularly those generating REM sleep may also
contribute to pathogenesis of hallucinations in PD.
504
Anxiety in Parkinsons disease relates to limbic and brainstem
alpha-synuclein pathology
M.E. Kalaitzakis, R.K.B. Pearce, M.B. Graeber, S.M. Gentleman
(London, United Kingdom)
Objective: To investigate the relation of anxiety to focal brain
pathology in Parkinsons disease (PD).

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Background: Anxiety is commonly encountered in the Lewy Body
disorders of PD, PD with dementia and dementia with Lewy bodies but
the underlying pathological basis for this clinical feature remains
unclear.
Methods: Through retrospective analysis of medical case notes, 81
cases from the UK Parkinsons Disease Society Tissue Bank at Imperial College, were screened and the clinical severity of anxiety was
graded on a global impression scale (0-3, absent to severe). We
identied a group of 6 PD patients with moderate to severe anxiety
without concomitant neuropsychiatric complications and a group of 9
PD patients with no anxiety and no other neuropsychiatric features.
Using immunohistochemistry the deposition of -synuclein (aSN) in
the temporal, cingulate and entorhinal cortices as well as in the hippocampus CA2 sector, subiculum, amygdala, Nucleus Basalis of Meynert (NBM), locus coeruleus and raphe nuclei was assessed semiquantitatively (0-3, absent to severe).
Results: PD cases with anxiety demonstrated an increased aSN
burden in the amygdala (p0.004), CA2 sector of the hippocampus
(p0.008) and locus coeruleus (p0.04) when compared to those PD
cases without.
Conclusions: The present study indicates that aSN pathology in
limbic and brainstem regions may be linked to the pathogenesis of
anxiety in PD. These ndings reinforce the involvement of several
anatomical systems in relation to psychiatric abnormalities in PD.
505
Neuropsychological early detection and the characteristics of Parkinsons disease associated with mild dementia
K.S. Lee, I.U. Song, J.S. Kim, J.Y. An (Seoul, Republic of Korea)
Objective: We investigated the characteristics of the cognitive impairments in mild PDD.
Background: Parkinsons disease (PD) patients, even those with the
mild form, may exhibit patterns of cognitive decits that include
decrements in planning, sequencing, concept formation and the working memory. Yet physicians are currently lacking operational criteria to
dene the clinical boundaries between PD and PD dementia (PDD).
Methods: We prospectively recruited thirty patients with PDD,
twenty patients with PD without dementia, and age- and educationmatched thirty-three controls. All subjects were evaluated with a battery of detailed neuropsychological tests.
Results: Our results showed that most of the cognitive functions of
the PDD were more severely impaired than those of the non-dementia
group. Visual memory, visuospatial functions, naming and calculation
demonstrated more marked impairment than did the other domains for
the PDD group. A portion of the frontal executive tests, verbal memory
and some of the language function tests revealed more severe impairment in the PD and PDD groups than those in the control group.
Conclusions: Our result indicates that adding cognitive dysfunctions
of cortical type to the early symptoms of PD can help predict the
development of dementia because most of the PD patients already have
subcortical dementia features.
506
Reduced nocturnal heart rate variability in Parkinsons disease: A
polysomnographic case-control study
M. Vaillant, N.J. Diederich, J. Tiete, J.P. Lobreau (LuxembourgCity, Luxembourg)
Objective: To study nocturnal heart rate variability (HRV) in patients
with Parkinsons disease (PD).
Background: Reduced cardiac uptake of iodine-123 metaiodobenzylguanidine ([123I]MIBG-SPECT) demonstrates sympathetic denervation even in at an early stage of the disease. It has been proposed that
reduced HRV in PD patients could be partially due to this denervation.
However, nocturnal HRV is also dependent on aging, the sleep stages
(NREM versus REM), and it varies momentarily with body movements
or apnea events.
Methods: Retrospective, polysomnography (PSG)-based study comparing PD patients and controls. 38 PD patients were matched accord-

S155

ing to age, gender and apnea/hypopnea index with 38 controls that had
PSG for suspicion of sleep apnea syndrome. PSG was a one-night study
with a 16-channel- montage, with PD patients and controls taking their
usual medications. RR intervals were extracted from the PSG data of 34
PD patients and 36 controls that had PSG for suspicion of sleep apnea
syndrome. A random coefcient model was used to model the mean RR
interval with sleep stage and group (PD or control) as factors. A
random intercept was included in the model with unstructured covariance matrix blocks for each subject. A post hoc comparison of least
squares means was performed using the Tukey-Kramer adjustment for
multiplicity.
Results: Mean age was 63 (13) yrs in the PD group and 65 (9)
yrs in the control group. There were 80% men and 20% women in both
groups. AHI was 11.5 (15.7) in the PD and 9.9(14.0) in the
controls. The distribution of RR intervals within the sleep stages was
different between both groups (p0.0001). There were also signicant
differences in the mean length of RR intervals in all the sleep stages
between both groups (p0.0001). Thus the average length of RR
intervals in sleep stages 1, 3& 4 and REM was longer in controls
whereas in sleep stage 2 it was longer in PD patients.
Conclusions: Our study conrms reduced disease inherent nocturnal HRV in PD patients in most sleep stages. However, further analyses, possibly including random slopes in the random coefcients
model or spectral analysis, are required to conrm these results. It also
remains open whether vagal and/or sympathetic denervation is primarily responsible for these ndings.
507
Striatal amyloid plaques relate to dementia in Parkinsons disease
M.E. Kalaitzakis, M.B. Graeber, S.M. Gentleman, R.K.B. Pearce
(London, United Kingdom)
Objective: To determine clinicopathological associations between
dementia, visual hallucinations (VH) and focal brain pathology through
retrospective analysis of case notes and histological study.
Background: Dementia and VH are common complications in Parkinsons disease (PD). However, their anatomical and pathological
bases remain unclear. Recently, extensive striatal pathology in Lewy
body diseases has become apparent although its clinical relevance has
not been studied.
Methods: The clinical histories of 81 cases were examined and the
severity of dementia and VH was rated blind to the neuropathological
diagnosis. Cases with conrmed PD were grouped by clinical phenotype as follows: 1) cases with no dementia and no VH (n9); 2) cases
with severe dementia and no VH (n4); 3) cases with severe VH and
no dementia (n5); and 4) cases with both severe dementia and VH
(n12). Using immunohistochemistry the extent of -synuclein (aSN),
tau and amyloid plaques (A) burden in the Caudate Nucleus (CN),
Putamen (Put) and Nucleus Accumbens (NAcc) was assessed semiquantitatively (0-3, absent to severe).
Results: Striatal aSN and tau deposition was sparse to mild in all
regions under investigation with no statistical differences detected
between differing PD clinical groups. In contrast, compared to the
cognitively intact group, A burden was signicantly greater in the
NAcc of the cases with dementia but no VH and in the CN, Put and
NAcc of cases with concurrent dementia and VH. As A deposition
was minimal in cases with VH but no dementia, the increased A
pathology observed in the group with co-existing dementia and VH
may contribute to the dementing phase of PD.
Conclusions: The present study demonstrates that the occurrence of
dementia in PD is strongly associated with A plaques in the striatum.
508
Depressive disorders in early-onset Parkinsons disease
A. Kummer, S.T. Camargos, M.C. Cunningham, D.P. Maia,
F. Cardoso, A.L. Teixeira (Belo Horizonte, Brazil)
Objective: To systematically assess depressive disorders in earlyonset Parkinsons disease (PD).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S156 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: Early-onset PD is dened as an idiopathic parkinsonism with onset before age 50 years. Depressive disorders affect more
frequently PD patients than the general population. Interestingly, depressive symptoms can even precede the onset of PD. However, only
few studies investigated these disorders in early-onset PD.
Methods: Forty-eight consecutive patients (M/F: 28/20; mean age
SD, 50.1 8.0) with early-onset PD were recruited for this study. They
were submitted to a structured clinical interview for psychiatric diagnosis according to DSM-IV (MINI-Plus). The Beck Depression Inventory (BDI) and the Hamilton Depression Scale (Ham-D) were used to
assess the severity of depressive disorders.
Results: The prevalence of major depression and dysthymia in earlyonset PD was 31.3% and 12.5%, respectively. These disorders were
associated with female gender, higher UPDRS scores and increased
impairment of daily living. The mean Ham-D and BDI scores for all
patients were 14.2 (SD 9.0) and 17.7 (SD 11.9), respectively. For
patients with major depression, Ham-D and BDI scores were, respectively, 21.4 (SD 8.2) and 26.1 (SD 13.1). The mean of Ham-D and
BDI in dysthymia was 16.3 (SD 7.5) and 21.1 (SD 8.9), respectively. There was a direct correlation between BDI and UPDRS scores.
Receiver operating characteristic curve (ROC curve) showed that a
cut-off score (equal or higher than) of 14 for BDI and of 13 for Ham-D
yielded the best sensitivity and sensibility with regard to diagnose
depressive disorders (either major depression or dysthymia). In addition, the areas under the ROC curve were 0.86 for BDI and 0.89 for
Ham-D, suggesting good discriminative property.
Conclusions: Major depression and dysthymia are frequent diagnosis
in early-onset PD. Of note, twenty-six patients (54.1%) had a BDI score
higher than 14, while twenty-ve (52.1%) scored higher than 13 in
Ham-D. This suggests that many patients may display relevant depressive symptoms without fullling DSM-IV diagnostic criteria.
509
Effects of motor and cognitive tasks on hemodynamic parameters
in orthostatic hypotension
B. Shihman, L. Grundlinger, J.M. Hausdorff, N. Giladi, T. Gurevich
(Tel Aviv, Israel)
Objective: To assess the relationships between hemodynamic
changes (mainly blood pressure), gait, and cognitive performance under single or dual tasking.
Background: Orthostatic hypotension (OH) is a frequent and often
underdiagnosed disorder that is known to affect cognitive and motor
performance. The effects of OH on cognitive function while walking
have not yet been studied.
Methods: The study group included 10 subjects with OH (age 71.5
10.6 years, 3 females) and a group of 16 age and gender-matched
controls. Blood pressure (BP) and heart rate (HR) were monitored
continuously during the entire investigation using a pulse plethysmograph (Finapres Medical Systems), and gait parameters were measured
using force-sensitive insoles (Infotronic Ultraex).
Results: In the OH group, on tilt table examination, systolic blood
pressure (SBP) declined by a mean of 31.210.4 mmHg (range 20-46
mmHg), and diastolic BP (DBP) declined by 8.2 8.4 mmHg. During
baseline walking, mean SBP in the OH group was similar to levels
measured during the supine condition, while in controls SBP increased
by 11.317.8 mmHg (p0.03). HR signicantly increased in both the
control and OH groups. Cognitive challenge while walking caused
signicant increase by 1617 and 106 mm Hg respectively in mean
SBP and DBP measures in the control group, as compared to baseline
walking (p0.05). In the OH group, mean DBP increased by 87 mm
Hg, while mean SBP was essentially unchanged, rising by 417 mm
Hg. HR was unchanged in both groups. The effects of dual tasking on
gait parameters included a reduction in speed and an increase in gait
variability, which were similar in both groups.
Conclusions: Subjects with OH respond differently than controls to
a 2-minute walk with regard to BP and HR. The dual tasking condition
further separated OH subjects from age matched controls. Patients with
documented OH on tilt-table testing, have reduced hemodynamic ad-

Movement Disorders, Vol. 22, Suppl. 16, 2007

aptation to continuous motor and cognitive demands. This observation

may have important clinical implications.
510
The predictors of fatigue in Parkinsons disease: Results from a
UK-German study of 135 cases
Y. Naidu, P. Martinez-Martin, R. Brown, P. Odin, A. Russemann, K.
Ray Chaudhuri, International Parkinsons Disease Non Motor
Group (London, England, United Kingdom)
Objective: What are the clinical factors that may predict occurrence
of fatigue in a consecutive cohort of Parkinsons disease (PD) patients
and how does it affect health related quality of life (Qol)?
Background: Fatigue may occur in over 40% PD cases and is a
prevalent non motor symptom of PD. Fatigue can be assessed using a
pragmatic approach by asking the patients directly about the severity of
perceived fatigue by means of a visual analogue scale (VAS)0 (worst
fatigue) to 100 (no fatigue at all). This scale has been useful in
assessment of fatigue in PD.
Methods: Consecutive PD cases (8.89% drug nave) attending outpatient clinics in London and Bremerhaven were studied as part of the
non motor scale validation protocol with several motor and non motor
variables and fatigue VAS.
Results: 135 PD cases (mean age 69.710.5 yrs (35-88), duration of
disease 5.775.1(SD)yrs (1-32)) were studied and had mean fatigue
score of 62.519.9 (10-100). Fatigue was not different between sexes,
had a trend towards lower scores in akinetic PD (56.222.3 vs
65.0318.3 (tremor dominant) and signicantly increased with severity of PD based on Hoehn and Yahr (HY) staging (Krusakll-Wallis
p0.004). Fatigue was associated to impaired mood, as measured by
the Hospital Anxiety and Depression Scale (r0.40, anxiety, 0.42,
depression; both p0.0001), non-motor questionnaire (NMSQ) total
score (r0.31; p0.0003 including sleep domain (p0.0009)), and
Qol (PDQ-8, r 0.37; p0.0001).
Conclusions: Anxiety, depression and sleep problems appear to be
strong predictors of fatigue in PD which correlates strongly with health
related quality of life.
511
Prevalence of behavioural symptoms in PSP and MSA: Evidence
from NNIPPS (Neuroprotection and Natural History in Parkinsons Plus Syndromes)
B.R. Stanton, P.N. Leigh, G. Bensimon, Y. Agid, A.C. Ludolph,
J.R. Hodges, T.H. Bak, T. Bak, J. Noth, L. Lacomblez, B. Dubois,
I. Uttner, R.G. Brown, The NNIPPS Consortium (London, United
Kingdom)
Objective: To determine the prevalence of behavioural symptoms in
Progressive Supranuclear Palsy (PSP) and Multiple System Atrophy
(MSA).
Background: Behavioural symptoms are an important cause of disbability and care-giver distress in neurodegenerative disorders. Neuropsychiatric symptoms are a recognised feature of PSP, but studies of
their prevalence are limited by small sample size. The frequency of
behavioural symptoms in MSA is unknown.
Methods: The NNIPPS study recruited patients with PSP and MSA
to a multicentre double-blind randomised controlled trial of riluzole. At
baseline, behavioural symptoms were assessed by a structured interview with a carer (the Neuropsychiatric Inventory, NPI) alongside
clinical and cognitive assessments. Complete data was available for
314 PSP and 273 MSA patients.
Results: At least one behavioural symptom was present in 80% of
patients with both PSP and MSA. Depression was the most common
symptom (59% PSP, 60% MSA) followed by apathy (54% PSP,
38% MSA), anxiety (36% PSP, 37% MSA), agitation (30% PSP,
18% MSA) and irritability (27% PSP, 24% MSA). Aberrant motor
behaviour, disinhibition and euphoria were uncommon (15%) and
delusions and hallucinations were rare (5%). The prole of behavioural symptoms was similar in the two disorders, but apathy and
agitation were more common in PSP (Figure 1). Figure 1 In general,

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

behavioural symptoms were not associated with age, disease duration or the clinicians global impression of disease severity. Cognitive impairment (MMSE26) increased the risk of psychotic
symptoms in both disorders and of disinhibition and aberrant motor
behaviour in PSP. Apathy in PSP was associated with frontal
cognitive change.
Conclusions: The evidence from NNIPPS suggests that depression is
more common in PSP than previously reported and that behavioural
symptoms are also very common in MSA. These ndings have important implications for all clinicians caring for patients with Parkinsons
Plus syndromes.

S157

(p0.05). Simple audio- and visuomotor reactions time was longer in

patients with PSP (0.560.06 sec) than in patients with PD (0.410.05
sec) (p0.05). Complex audio- and visuomotor reaction time was
longer in PSP than in MSA and PD due to choice of colour time and
choice of hand time. Patients with PSP made more impulsive mistakes
in carrying out of simple and complex sensomotor reactions in comparison with the patients with MSA and PD (p0.05).
Conclusions: Thus psychomotor dysfunction in MSA and PSP manifested as inhibition of sensomotor reaction time due to both motor
disorders and problems in choice strategy of motor action. Psychomotor dysfunction in MSA and PSP was more severe than in PD.
513
A prospective study of mood in Parkinsons disease: The PROMS-PD
study
R.G. Brown, D. Burn, J. Hindle, C.S. Hurt, S. Landau, J. Playfer,
M. Samuel, K. Wilson (London, United Kingdom)

FIG. 1 (511).
512
Correlation of psychomotor dysfunction in patients with Parkinsons disease, multisystem atrophy and progressive supranuclear
palsy
N. Amosova, O.S. Levin (Moscow, Russian Federation)
Objective: To investigate the features of psychomotor dysfunction in
multysystem atrophy (MSA) and progressive supranuclear palsy (PSP)
in comparison with Parkinsons disease (PD).
Background: Psychomotor impairments to a variable degree of expressiveness are a common feature in neurodegenerative disorders of
CNS. A movement is a compound functional complex of motor periphery and interactive regions of brain (A.R. Luria, 1969).
Methods: We observed 18 patients with MSA (according to
criteria N. Quinn, 1994) (mean age 60.25.4 years; mean duration
of the disease 2.81.3 years); 11 patients with PSP (according to
criteria of NINDS-SPSP, 1994) (mean age 62.56.4 years; mean
duration of the disease 3.62.04 years); 21 patients with PD
(according to criteria of UKDRS, 1988) (mean age 63.75.5
years; mean duration of the disease 4.12.4 years). Psychomotor
functions (quantitative assessment of oculomotor and visuomotor
responses, counting and denition of type of mistakes) were assessed using rhythmic tapping. Complex sensomotor reactions were
performed by using an element of choice (a colour of visual signal
or tone of sound signal).
Results: There were no signicant differences of simple sensomotor
reaction time between patients with MSA and PSP. Complex visuomotor choice reaction time was longer in MSA (0.150.1 sec) because
of choice of hand time in comparison with PD (0.060.12 sec)

Objective: To present the design, methods and aims of a recently

initiated prospective study of mood in Parkinsons disease (The
PROMS-PD Study).
Background: Symptoms of depression are typically reported in a third of
patients with Parkinsons disease (PD) and have been shown to impact
negatively on aspects of both the patients and caregivers lives. Identication and treatment remain suboptimal. Research is currently constrained
by a limited understanding of the nature of depression in PD and its
possible clinical heterogeneity.
Methods: Five hundred patients diagnosed with idiopathic PD will be
recruited from three UK centres. A detailed clinical assessment will be
performed using standardised measures covering a number of main areas:
basic demographics, current general medical health and use of health care
services, PD history, symptoms and management, psychiatric and cognitive symptoms, health outcome and psychosocial factors. Clinical assessment will be repeated annually over the ve years of the study.
Results: The information gathered from clinical assessments will be
used to develop and test clinical models of depression in PD. These
models will be derived from both existing criteria and patterns found in
the data. The ability of each model to account for relationships between
risk factors and depression, and depression and health outcomes, will
be used as a measure of the clinical utility of each model.
Conclusions: The results of the study will provide important insights
into the nature of depression in PD and provide important foundations for
future research into the identication and treatment of depression in PD.
514
The effect of nighttime levodopa and entacapone on subjective and
objective sleep measures in Parkinsons disease
I. Itin, C.L. Comella, J.A. Jaglin, M. Park, L. Benson, W. Fan,
S. Lergans (Cleveland, Ohio, USA)
Objective: To assess the effect of levodopa and entacapone on
nocturnal sleep disturbance due to recurrent symptoms in treated Parkinsons disease (PD) patients using both subjective assessments and
objective measures of sleep and daytime sleepiness.
Background: Sleep fragmentation due to recurrence of PD symptoms is
a common cause of sleep disruption and contributes to daytime sleepiness.
Methods: This is an open-label study to assess the effect of levodopa
(150mg)/carbidopa (37.5 mg)/entacapone (200 mg) combination (Stalevo
150) at bedtime in PD patients with sleep fragmentation (greater than 3
awakenings occurring at least 3 nights per week due to recurrent PD
symptoms). All subjects took their routine PD medications with the last
dose no later than 7 p.m. The subjects were excluded due to depression,
dementia or a diagnosed sleep disorder. The subjects were assessed with an
array of subjective and objective tests before and after treatment with
Stalevo 150 at bedtime for twelve weeks. Subjective evaluations included
the Pittsburgh Sleep Quality Index (PSQI) and Epworth Sleepiness Scale
(ESS). Objective measures included polysomnography (PSG), the Multiple Sleep Latency Test (MSLT) and the Maintenance of Wakefulness Test
(MWT). Values for each outcome measure were summarized as means

Movement Disorders, Vol. 22, Suppl. 16, 2007

S158 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

and SDs, with changes between baseline and end-of-study evaluated by
two-tailed Wilcoxons signed rank test.
Results: There were 19 men and 7 women included with mean age
64 yrs (SD 9.4), and mean total UPDRS score 22.5 (SD 11.1). At
baseline, the mean total PSQI was 18.8 (SD 6.5) and ESS was 11.4 (SD
5.3). PSG at baseline showed a mean total sleep time of 473 (SD 30.9)
minutes, sleep efciency of 69 (SD 18.7) percent, PLM index of 29.8
(SD 49.1), and apnea/hypopnea index of 9.5 (SD 15.5). The MSLT
showed a mean sleep latency of 7.8 (SD 5.6), and the MWT a mean
sleep latency of 32.8 (SD 8.0). Following treatment, the PSQI showed
a mean decrease of 3 points (SD6.6, p0.0389). There were no
signicant changes in the other outcome measures.
Conclusions: Stalevo 150 at bedtime improved subjective sleep
quality, but did not change objective sleep measures in PD patients.

trode. The electrogastrographic (EGC) signal was registered, sampled,

ltered by a dedicated system. We have studied three groups of patients
(total 20): a)de novo b)with motor uctuations c)without motor uctuations. The protocol of study was: 1) stopping L-dopa therapy and have an
empty stomach in the last 12 hours before EGC evaluation 2) two sessions
of EGC registration, each one lasting 45 minutes, before and after LD
intake. In the same patients we evaluated in three different days the LD
plasma levels.
Results: The EGC frequency at rest and after LD intake was normal and
does not change in the three clinical groups. The LD plasma levels showed
a marked intraindividual and interindividual variability in all three groups.
Conclusions: In PD the gastric motility at rest is normal and is not
inuenced by LD intake. L-dopa plasma levels are not correlated with
gastric motility at rest.

515
A guided interview to assess behavior in Parkinsons disease
C. Ardouin, I. Chereau, P.M. Llorca, F. Durif, A. Pelissolo, M. Schupbach,
A. Funkiewiez, K. Dujardin, P. Krystkowiak, C. Arbus, F. Tison,
J.L. Houeto, P. Auquier, P. Pollak, P. Krack (Grenoble, France)

517

Objective: To develop an assessment tool allowing to detect typical

behaviors that can be specically related to either Parkinsons disease
(PD) or dopaminergic treatment.
Background: In PD there are a large spectrum of behaviors that can
be associated either with a decrease (disease-related) or on the contrary
with an increase (treatment-related) in dopaminergic activity. A tool
specically assessing hyper- and hypo-dopaminergic behaviors would
be helpful in both research and the management of PD.
Methods: An expert panel including neuropsychologists, psychiatrists
and neurologists developed a guided interview assessing different aspects
of parkinsonian and hyperdopaminergic behaviors. Different relevant emotional, behavioral and cognitive items were identied. Frequency and
severity of individual items were rated from 0 (absence) to 4 (severe) and
the items were integrated in different structured domains.
Results: The guided interview evaluates four different domains : I) a
general section including depressive mood, (hypo)manic mood, anxiety,
irritability/aggressiveness, emotional lability, hallucinations/delusions; II)
global functioning on an apathetic way; III) psychic and behavioral uctuations and IV) hyperdopaminergic behaviors (nocturnal hyperactivity,
diurnal somnolence, binge eating, creativity, hobbyism, punding, risk
behavior, compulsive shopping, pathological gambling, hypersexuality,
addiction to dopaminergic drugs and global functioning on an appetitive
way). This interview takes from 30 minutes in a patient without any
behavioral abnormalities up to 3 hours in patients with severe pathological
hyperdopaminergic behaviors. There was good inter-rater agreement
among participants of the panel, based on ratings from video documented
interviews. The validation of this scale is in progress.
Conclusions: We have developed a tool to detect and quantify typical
behaviors that can be specically related to either PD or dopaminergic
treatment. This scale is sensitive for changes in behavior related to
dopaminergic or surgical treatment (see accompanying poster by E
Lhommee & al). In our experience it has become a useful guide in the
management of PD.
516
Features of gastric motility at rest in Parkinsons disease
G. Albani, N. El Assawy, S. Cattaldo, A. Mauro (Piancavallo,
Verbania, Italy)
Objective: Aim of this study was to evaluate the myoelectrical
gastrointestinal activity in patients with Parkinsons disease (PD) by
means of an electromyographic (EMG) surface evaluation.
Background: In PD various clinical gastrointestinal dysfunctions are
well-known, also supported by anatomopathological evidences since
rst studies of FH.Lewy, till more recent studies of H.Braak. It has also
been hypothesized a crucial role of these dysfunctions on the absorption of L-dopa (LD), and thus on LD plasma levels and , at the end, on
the genesis of motor uctuations.
Methods: For the EMG study, we placed on the abdominal skin two
active recording electrodes, one reference electrode and one ground elec-

Movement Disorders, Vol. 22, Suppl. 16, 2007

Neuroanatomical loci of subjective panic-like phenomena in OCD

DBS subjects
I.U. Haq, W.K. Goodman, K. Foote, C. Jacobson, M.S. Okun
(Gainesville, Florida, USA)
Objective: To compare the lead location at which panic-like phenomena were induced by deep brain stimulation (DBS) for obsessivecompulsive disorder (OCD).
Background: DBS has become a potentially important but experimental treatment for medication resistant obsessive-compulsive disorder. While stimulating in a relatively unexplored human target, the
anterior limb of the internal capsule and nucleus accumbens region
(ALIC-NA), we encountered stimulation-induced panic like phenomena: tachycardia, ushing, a sensation of heat, fear, uneasiness, and a
subjective sense of panic.
Methods: Six subjects with medication refractory OCD enrolled in
an NIMH study underwent test stimulation during intra-operative implantation of their bilateral DBS devices in the ALIC-NA. Each contact
of the DBS was stimulated intra-operatively from 0 to 8 volts and
active contact locations that elicited panic-like phenomena were recorded. CT-MRI fusion was used to calculate the x,y,z coordinates of
the center of each contact that elicited panic-like phenomena
(xlateral, yanteroposterior, zdepth, all in relation to the midcommissural point).
Results: Two groups were identied: those with panic-like phenomena (2 subjects, 3 leads), and those without (4 subjects, 9 leads). The
average age of the panic group was 40 years of age (SD12.12) and the
average age for the non-panic group was 34.89 (SD8.34). The average disease duration in the panic group was 28.33 years (SD7.51)
while average disease duration in the non-panic group was 19.44
(SD8.00). Panic-like phenomena occurred at average coordinates of
x9.93mm, y14.52mm, and z-0.90mm (SD4.56, 4.79, 6.24).
The contact locations which failed to produce panic responses on
activation had an average location of x12.10mm, y18.26, and
z6.00 (SD3.99, 4.04, 7.24).
Conclusions: The regions of activation that elicited panic-like phenomena seemed to be more medial, posterior, and deep. We suspect
that these coordinates reect the location of ber bundles important to
the panic-like phenomena such as the accumbens-amygdala connections. More data will be needed to conrm these results. Understanding
the location of these ber pathways may prove useful for safe targeting
of specic neuropsychiatric disorders.
518
Sleep and circadian changes in early stage Huntingtons disease
A.O.G. Goodman, J. Morton, M. King, J. Shneerson, R.A. Barker
(Cambridge, United Kingdom)
Objective: To assess changes in sleep patterns and circadian rhythms
in early stage patients with Huntingtons disease (HD).
Background: HD is a debilitating autosomal dominant, neurodegenerative disease with a fatal prognosis. Classical symptoms include
motor disturbances, subcortical dementia and psychiatric symptoms
and although striatal cell loss is the dominant pathology in HD, as the

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

disease progresses, neurodegeneration becomes increasingly widespread and even extends outside the central nervous system. As a result,
further features emerge, in particular sleep disturbances.
Methods: We elected to study circadian rhythms and sleep disturbances using actigraphy and polysomnography.
Results: We found that patients had an overall loss of form and
denition in their rest-activity actograms, suggesting a disturbed circadian rhythm as well as markedly disturbed sleep. This included sleep
cycles that were poorly consolidated, fragmented and irregular with the
majority of patients having longer sleep latencies, reduced sleep efciency, more time spent awake during the sleep period, frequent arousals and reduced slow wave and rapid eye movement sleep.
Conclusions: We have shown that in early HD there are disturbances in
sleep and circadian rhythms suggesting a possible breakdown of hypothalamic driven processes as part of the disease process. Longitudinal studies
to investigate this further are now being undertaken to assess the extent to
which these abnormalities change with disease progression.
519
Trajectory control and motor learning in pre-symptomatic Huntingtons disease (pHD)
F. Ghilardi, A. Feigin, C. Moisello, F. Battaglia, A. Di Rocco,
D. Eidelberg (New York, New York, USA)
Objective: To verify whether trajectory formation and sequence
learning precede the appearance of motor symptoms in pHD.
Background: Working memory and attentional functions might be
impaired before the occurrence of motor symptoms in pHD subjects.
Motor sequence learning captures both explicit, visual, and implicit,
motor, aspects of learning.
Methods: Subjects were eleven right-handed pHD gene carriers (six
women, ve men; CAG repeat length 38) and eleven age-matched
neurologically normal controls. All patients had undergone genetic
testing. All subjects moved their dominant hand on a digitizing tablet
to eight targets displayed on a computer screen. Tasks were: CCWReaching for predictable targets in a timed-response task; RAN-Movements to random unpredictable targets in a reaction time task; SEQLearning to anticipate a repeating sequence of eight targets in two trial
blocks; VSEQ-Learning sequence by attending to the display without
moving for one block and by reaching in a second block. Ten pHD
carriers were also tested a second time, eighteen months later, and eight
a third time, three years later.
Results: In RAN, both reaction times and spatial errors of pHD
carriers were not signicantly different than controls at baseline, but
increased signicantly at the second and third time point
(p0.0001). Spatial errors of CCW movements were signicantly
higher in pHD than normal controls at all the three testing points.
Moreover, errors increased in the second and third sessions
(p0.05). At baseline, both implicit (acquisition of motor skill) and
explicit (acquisition of the order) aspects of sequence learning in
SEQ were altered in pHD compared to controls (p0.0001). When
sequence was rst learned visually in VSEQ, the explicit, but not the
implicit learning component improved. Learning performance did
not further worsen in the second and third testing sessions.
Conclusions: Both implicit and explicit decits of sequence learning
are apparent early on. These symptoms appeared to have a slow
progression. Aspects of trajectory controls may be impaired before the
appearance of clinical motor symptoms and seem to progress rapidly.
Supported by: K08 NS01961; R01 NS7564.
520
Disturbance of automatic auditory change detection in dementia
associated with Parkinsons disease: A mismatch negativity
study
K. Bronnick, H. Nordby, J.P. Larsen, D. Aarsland (Stavanger,
Norway)
Objective: To investigate whether automatic auditory change detection, as measured by the mismatch negativity (MMN) event-related
potential waveform, differs in dementia associated with Parkinsons

S159

disease (PDD) and dementia with Lewy-bodies (DLB) as compared to

Alzheimers disease (AD), Parkinsons disease (PD) without dementia
and healthy control subjects (HC).
Background: Attentional decit is a feature of both DLB and
PDD, two syndromes with shared clinical features, and with cortical
and brain-stem Lewy-body pathology as neuropathological substrate. However, studies on attentional phenomena in PDD and DLB
have used visual stimuli, possibly representing a confound, as both
disorders are characterized by visuospatial decits. Most research
has also focused on controlled, selective attention, and there is a
lack of research on automatic, involuntary attentional phenomena in
the auditory modality. The MMN is therefore used to compare
patients with PDD to patients with DLB.
Methods: Seventeen DLB, 15 PDD, 16 PD, 16 AD patients and 18
HC subjects participated. The dementia patients were matched on
overall MMSE score. A passive MMN paradigm with compound
sinus-tones was used. 1800 tones with duration of 75 milliseconds
(ms) and 200 deviant tones with duration of 25 ms and an interstimulus interval of 350 ms were presented. In addition, the subjects
completed an oddball-distractor task in which they had to selectively respond to a frequency-deviant tone, while ignoring a frequent standard tone and a distractor stimulus consisting of white
noise.
Results: The PDD patients had reduced MMN area and amplitude
compared to the DLB group, and the PD, AD and the HC groups.
The MMN area correlated signicantly with number of missed
target stimuli in the oddball-distractor task, and the PDD group
missed targets signicantly more often than the DLB group.
Conclusions: The MMN and behavioral results taken together
indicate that PDD patients may have a decit of automatic auditory
change detection that contribute to impairment in their ability to
selectively attend and respond to deviant auditory stimuli. The
results indicate that attentional functions may differ in the auditory
modality for PDD vs. DLB.
521
A novel paradigm to assess mental fatigue in Parkinsons disease
F. Battaglia, D. Crupi, A. Di Rocco, A. Quartarone, F. Ghilardi
(New York, New York, USA)
Objective: To assess objectively the presence and severity of mental
fatigue in patients with untreated Parkinsons disease (PD) with a novel
combined TMS and imagery paradigm.
Background: Mental fatigue is a very common non-motor symptom
in PD patients with prevalence from 40% to 56%. Currently, the
presence and severity of mental fatigue is assessed with questionnaires.
Here we introduce a new paradigm using TMS and mental imagery task
for objective assessment.
Methods: We tested 10 untreated patients in the early stages of
PD (Hohen & Yahr stage I and II) and 10 age-matched normal
controls. Mental fatigue was assessed with motor evoked potentials
(MEP) measurement elicited with TMS stimulation during a repeated motor imagery task. TMS results were correlated with subjective scores of mood (Beck Depression Inventory, BDI), daytime
sleepiness (Epworth Sleepiness Scale, ESS) and fatigue (Multidimensional Fatigue Inventory Scale, MFI) scales.
Results: Motor imagery induced decreases in motor threshold and
increase in MEP amplitude (both p0.05) without difference between PD and control groups. However, in PD patients the TMS
correlate of mental endurance measured during repetitive motor
imagery was signicantly reduced compared to controls (p0.05)
but, like in normal controls, it recovered after a 30-minute rest. This
TMS mental endurance index was inversely correlated with BDI and
MFI but not with ESS sleepiness scores.
Conclusions: Untreated PD patients in the early stage of the disease
display normal kinesthetic motor imagery and exhibit increased mental
fatigue. The correlation we found between these two ndings further
suggests that mental fatigue and mood disturbance might share common bases. This objective evaluation of mental fatigue could be useful

Movement Disorders, Vol. 22, Suppl. 16, 2007

S160 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

in monitoring the efcacy of therapeutical interventions in PD and
other neuropsychiatric diseases.
522
Major depression and cognitive dysfunctions in non demented
parkinsonian patients
S. Gabriella, C. Vitale, M.E. De Martino, T. Luigi, G. Dario,
P. Barone (Neaples, Italy)
Objective: The present study explores the relationship between
major depression and cognitive impairments in non demented Parkinsons disease (PD) patients and the association between affective
symptoms of major depression (depressed mood, apathy and anhedonia) and cognitive functions within a group of depressed PD
patients.
Background: The relationship between depression and cognitive
dysfunctions in Parkinsons disease (PD) was investigated in previous
studies but the ndings are still discordant.
Methods: 123 consecutive PD patients without dementia
(DSM-IV criteria and MMSE 23) were examined. The presence
of major depression was assessed using DSM-IV criteria for major
depressive disorder. Motor symptoms, depressive symptoms, anhedonia and apathy were evaluated using Hoehn & Yahr stage (H&Y),
Unied Parkinsons Disease Rating Scale (UPDRS-III), Hamilton
Rating Scale (HDS), Snaith Hamilton Pleasures Scale (SHAPS) and
Apathy Evaluation Scale (AES), respectively. Cognitive functions
were examined by means of semantic (Flu-S) and phonological
uency (Flu-F) tasks, Stroop Test (ST), Frontal Assessment Battery
(FAB) and copying of gures tasks (CT).
Results: Sixty-three (51.2%) patients were depressed (dPD) and
sixty (48.8%) patients were not (nPD). The two groups did not
signicantly differ for demographic and clinical characteristics.
dPD had signicantly higher scores than nPD on Ham-D (p0.001),
SHAPS (p0.002) and AES (p0.001). dPD had signicantly
lower performances than nPD on Flu-S (p0.019), ST-interference
(p0.047), FAB (p0.003) and CT (p0.001). Flu-F neared signicance (p0.060). Within the dPD group, three sub-groups were
identied according to DSM-IV criterion 1 (depressed mood) and
criterion 2 (apathy/anhedonia): 20 dPD responded afrmatively to
criterion 1 (dPD-1), 20 dPD to criterion 2 (dPD-2), 23 dPD to both
criterion 1 and criterion 2 (dPD-12). The three subgroups were
compared with nPD patients: dPD-2 scored signicantly lower than
nPD on CT, whereas dPD-12 scored signicantly lower than nPD
on CT and FAB.
Conclusions: The present study supports the association between
major depression and cognitive dysfunctions in non demented PD
patients. The combination of apathy and anhedonia in depressed
patients is associated with frontal and visuoconstructional dysfunctions.
523
Diagnosis of dementia in Parkinsons disease with FDG PET
I. Liepelt, M. Reimold, W. Maetzler, G. Reischel, K. Schweitzer,
A. Gaenslen, J. Godau, T. Gasser, H.-J. Machulla, R. Bares,
D. Berg (Tuebingen, Germany)
Objective: To determine the accuracy of FDG PET in the differentiation between PD and PDD and to verify whether hypometabolism
reects primarily cognitive deterioration.
Background: In non-demented patients with Parkinsons disease
(PD), PET studies have shown either normal glucose metabolism or
widespread reductions to levels between controls and demented
patients with Parkinson (PDD). Relation to cognitive deterioration
in demarcation to motor decits are not clear. Neither, has the
accuracy in seperating PD and PDD been analyzed.
Methods: We investigated 24 (9 female) patients, 13 with PD
(median age67.5 7.1), 11 with PDD (median age70.0 4.5)
and 7 age matched healthy controls. All patients were assessed with
a neuropsychological test battery and underwent PET with
[18F]FDG. Using SPM2, we assessed group differences in FDG

Movement Disorders, Vol. 22, Suppl. 16, 2007

uptake between patients (PD n7; PDD n7) and controls (n7).
A mask was generated comprising voxels with decreased FDG
uptake in patients (p0.05). For each patient we calculated a ratio
of mean FDG-uptake within the mask over that in the remaining
gray matter.
Results: The FDG ratio correlated closely with an index of
severity of neuropsychological decits (rho0.83; all groups,
n31) and with the MMSE (rho0.75; n31). In contrast, age
(rho-0.36, N31), disease duration of PD (rho-0.18, not including controls, n24) and daily dose of L-Dopa (rho-0.09; n24)
was not associated with the calculated FDG ratio. The correlation
between the UPDRS-Motor Score and the FDG ratio was only
moderate (rho-0.51, n24). Although PDD and PD patients were
equally represented when the mask was initially dened, the derived
ratio correctly separated PDD from PD with the exception of only
one PDD patient, who was misclassied as PD. There was no
overlap between PDD and controls (sensitivity 91%; specicity
100%; positive predictive value 100%; negative predictive value
95%).
Conclusions: FDG PET is able to separate PDD from PD and
controls with very high accuracy. Reduced glucose metabolism in PDD
may reect cognitive rather than motor dysfunction.
524
Assessment of odor identication and discrimination in Dutch
Parkinsons disease patients
S. Boesveldt, D. Verbaan, D.L. Knol, J.J. van Hilten, H.W. Berendse
(Amsterdam, Netherlands)
Objective: To determine the prevalence of odor identication and
discrimination impairments in a large population of Dutch Parkinsons disease (PD) patients, and to develop optimal discrimination
criteria for olfactory tests to distinguish between PD patients and
controls.
Background: Hyposmia is among the rst signs of PD and is probably related to early stage extranigral pathological changes in the
olfactory bulb and tract.
Methods: Olfactory function was assessed using two components
of the Snifn Sticks test battery in 127 control subjects (50 women;
mean age 59.4 years, range 45-78 years) and in 380 PD patients (140
women; mean age 61.5 years, range 40-90 years, Hoehn and Yahr
stages I-V). Odor identication was measured by presenting 16
odorants in a multiple (4)-forced choice format. In the odor discrimination test, subjects were asked to select the odd odor out of
three odorants presented. In both tests, olfactory scores were dened
as the number of correct answers (0-16).
Results: The mean identication score ( SD) was 12.7 2.3 for
control subjects, and 7.4 3.0 for PD patients. The odorant that
best separated patients from control subjects was aniseed (37.5%
vs. 92.1% correctly identied). The mean discrimination score was
11.4 2.4 for control subjects, and 8.1 2.6 for PD patients. For
each of the olfactory tests, cut-off values were based upon the 95%
lower bound of the individual prediction interval of the linear
regression lines of the control subjects plotted against age. Based on
these values, 241 PD patients (64.1%) were impaired on the odor
identication test, whereas 158 PD patients (41.9%) were impaired
on the odor discrimination test. Receiver operating characteristic
(ROC) curves based upon sensitivity and specicity estimates for
each test will be presented at the meeting.
Conclusions: In this population of Dutch PD patients, the overall
prevalence of olfactory decits was lower than that reported previously for other populations. Considering the difference in prevalence between odor identication impairments (64%) and odor
discrimination decits (42%), odor identication appears to differentiate better between PD patients and controls than odor discrimination.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

525
Towards a better understanding of the relationship between the
Mariana dementia and the Parkinsonism/dementia complex of
Guam (Lytico-bodig): Evidence from a longitudinal study
T.H. Bak, J.R. Hodges, J.C. Steele (Edinburgh, Scotland, United
Kingdom)
Objective: To characterise the relationship between different
types of dementia and Parkinsonism/dementia complex (PDC) of
Guam (Lytico-bodig) using longitudinal and cross-sectional
design.
Background: The ALS/Parkinsonism/Dementia Complex (ALS/
PDC) of Guam can be associated with different phenotypes, including amyotrophy, parkinsonism and dementia. In recent years we
have identied progressive dementia without parkinsonism in a
large number of islands elderly subjects. We refer to it is Mariana
dementia (MD). Many MD patients come from families whose other
members have suffered ALS or PD. In a cross-sectional study of 14
patients with PDC and/or Dementia, conducted in 2005 and presented at the 10thInternational Congress of Parkinsons disease and
Movement Disorders in Kyoto, Japan in November 2006, we concluded that MD is a phenotype of ALS/PDC rather than of Alzheimers Disease (AD).
Methods: In 2006, one year after the rst evaluation, we reassessed 7 of the original 14 patients using similar neurological,
neuropsychological and psychiatric assessment tools, including
evaluation of motor function, orientation, different aspects of memory, frontal-executive functions, social cognition, visuospatial
skills, mood and behaviour.
Results: We observed a pronounced change in the severity and
pattern of motor, cognitive and behavioural symptoms in three out
of seven patients. In two, the initial picture was characterized by
mnestic and frontal-dysexecutive symptoms. One year later they
showed pronounced behavioural abnormalities typical of frontotemporal dementia including stereotypic, disinhibited and challenging behaviour. The third patient, presenting in 2005 with a cognitive-behavioural picture without any motor involvement, developed
parkinsonism by 2006.
Conclusions: All patients examined in 2005 and 2006 show a distinct
and consistent pattern of amnestic and frontal-executive symptoms,
which is different from that observed in AD. Morover, our longitudinal
data document that the amnestic and frontal-behavioral presentations of
dementia as well as parkinsonism can occur in the same patients as
consecutive stages of the same disease. We suggest that the dementia
associated with ALS/PDC should be treated as a distinct entity, referred
to as MD.
526
An acceleration of rhythmic nger tapping in patients with Parkinsons disease
I.S. Smolenteva, L.A. Batukaeva, O.S. Levin (Moscow, Russian
Federation)
Objective: To investigate dynamic characteristics of psychomor
functions with spontaneous and induced nger tapping in patients with
Parkinsons disease and factors which inuence on it.
Background: Close interplay of cognitive and motor functions in
Parkinsons disease (PD) can be studied with the model of psychomotor functions. Instrumental methods of studying of psychomotor impairments by using the rhythmic nger tapping allow to reveal disturbances of attention and processes of planning, programming and the
executive control.
Methods: Seventy-seven patients with PD (male/female 42/35,
age 59.29.6 years, duration 5.02.7 years) were observed. The
assessment of psychomotor functions in patients with PD was performed in comparison with 15 healthy persons. The severity of parkinsonian symptoms were assessed by using Hoehn and Yahr scale and
part III (motor) of Unied Parkinsons Disease Rating Scale (UPDRS).
Cognitive functions was assessed by using comprehensive neuropsychological battery.

S161

Results: Mean duration of intra-tapps intervals in patients with PD

was statistically longer than the one in healthy persons in realization of
simple rhythmic nger tapping. However during the test we revealed
paradoxical gradual decreasing in patients with PD in induced and
spontaneous nger tapping. Decreasing of intra-tapps interval was
correlated with verbal uency test (r0,41, p0,01) and perseverative
responses in Wisconsin Card Sorting Test (WCST) results (r-0,38,
p0,05), but not with the severity of motor disturbances.
Conclusions: Patients with PD had tendency to decreasing of intratapps intervals during performing of nger tapping with xed rate. As
similar features (such as propulsion in walking and tahyphemy in
speech) this phenomenon probably reects loss of the inhibitions
control. The acceleration correlates with dysexecutive syndrome and
may be explained by frontal lobes dysfunction. It helps to assess the
severity of the disease and also allows making an assessment of the
efciency of antiparkinsonian drugs more objective.
527
Knowledge of disease among patients with hemifacial spasm
W.S. Shahul Hameed, T.E. King (Singapore, Singapore)
Objective: We conducted a prospective questionnaire survey to
assess the level of knowledge of HFS among patients suffering from
this condition.
Background: Patients with hemifacial spasm(HFS) frequently felt
embarrassed and emotionally disturbed about their condition and this
can affect their quality of life. There is currently limited information on
HFS patients degree of knowledge of their condition. Such data may
assist in clinical management and help patients cope better with their
condition.
Methods: Consecutive patients diagnosed with HFS were recruited
from a movement disorders clinic. The clinical severity of their condition was assessed and their demographics tabulated. All the patients
answered a questionnaire that assessed the level of knowledge regarding the etiology, association, and treatment options of HFS.
Results: A total of 100 HFS patients were recruited, consisting of
predominantly females with mean age of 56.0 years of age. The vast
majority of HFS patients (68.9%) did not know the etiology of HFS and
its associations. Some were of the opinion that HFS can lead to
blindness(16.1%) and stroke(0.9%). 53.8% were aware that microvascular decompression surgery is an option of treatment, and younger
patients seemed more knowledgeable about this option than older
patients(62.5% vs 41.7%,p0.04).
Conclusions: Our survey highlights the general lack of knowledge
among HFS patients regarding their condition. A greater effort will be
needed to incorporate intensive education and counselling on the etiology, treatment options and compilcations of HFS as part of routine
care for this distressing condition. Such measures may reduce patients
misconceptions and improve quality of life.
528
Symptomatic REM sleep behavior disorder in patients with Parkinsons disease over eight years
M.D. Gjerstad, B.B. Boeve, T. Wentzel-Larsen, D. Aarsland,
J.P. Larsen (Stavanger, Norway)
Objective: To examine the occurrence and clinical and demographic
correlates of REM sleep behavior disorder (RBD) in patients with
Parkinsons disease (PD) in a community based cohort over eight years.
Background: 231 patients with PD were included in a populationbased prevalence study in 1993 in the Stavanger area, southwest of
Norway. The patients were then followed prospectively and reexamined after four and eight years.
Methods: Information upon clinical and demographic data was collected through semi-structured interviews at all study visits. Standardized rating scales of parkinsonism, depression, and cognitive impairment were used. The diagnosis of probable RBD (pRBD) was based on
a sleep questionnaire and the minimal diagnostic criteria of the ICSD
(The International Classiction of Sleep Disorders). Caretakers or a
close relatives were asked for additional information if available and

Movement Disorders, Vol. 22, Suppl. 16, 2007

S162 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

necessary. A proportional-odds ordinal logistic regression model for
clustered data was used to study the relationship between pRBD and
various demographic and clinical variables.
Results: 231 patients were evaluated for RBD in 1993 and 142 and
89 patients were available for re-evaluation after four and eight years.
The frequency of pRBD varied from 14.6% to 27% during the eight
year study period and was related to male gender, higher dopaminergic
treatment and less severe parkinsonism.
Conclusions: The frequency of pRBD varies over time and is associated with male gender, less parkinsonism and higher levodopa equivalent dose. Our ndings indicate that dopaminergic therapy may contribute to the expression of RBD and that RBD is symptomatic in
earlier stages of PD.
529
Psychiatric comorbidities in a Brazilian sample of early-onset Parkinsons disease
A. Kummer, S.T. Camargos, M.C. Cunningham, D.P. Maia,
F. Cardoso, A.L. Teixeira (Belo Horizonte, Brazil)
Objective: To investigate the psychiatric comorbidities in patients
with Parkinsons disease (PD) that started before age 50 years.
Background: Early-onset PD refers to an idiopathic parkinsonian syndrome with onset before age 50 years. Psychiatric disorders are frequently
reported in PD and they are associated with signicant impact on clinical
control and quality of life. However, studies that systematically assess
psychiatric symptoms in early-onset PD are lacking in the literature.
Methods: Forty-eight consecutive early onset PD patients (M/F:
28/20; mean age SD, 50.1 8.0) were included in this study. A
structured clinical interview for psychiatric diagnosis according to
DSM-IV (MINI-Plus) and neurologic examination, including Unied
Parkinsons disease Rating Scale (UPDRS), Hoehn-Yahr scale (HY)
and Schwab-England activities of daily living scale (SEADL), were
performed in all patients.
Results: Major depression was present in 31.5% of the patients
with early onset PD; dysthymia in 12.5%; bipolar disorder in 4.2%;
generalized anxiety disorder in 31.3%; panic disorder in 8.3%;
social phobia in 54% and obsessive compulsive disorder in 10.5%.
Psychotic disorders occurred in 3 patients (6.3%), but in 2 they were
possibly related to medication. Depressive disorders ever in life
were diagnosed in 58.3% of patients. Depressive disorders were
associated with female gender, higher UPDRS scores and increased
impairment of daily living. There was no signicant association
between depression diagnosis and age, HY scores, length of disease,
levodopa dosages, and incidence of dyskinesias or uctuations.
General anxiety disorder was associated only with diseases length,
and psychosis occurred more frequently in those patients with
higher UPDRS scores.
Conclusions: Psychiatric disorders seem to be very common in
early-onset PD with frequencies similar to older-onset PD.
530
Cognitive and motor mediators of the changes in gait stability
during dual tasking in healthy older adults
J.M. Hausdorff, T. Herman, N. Inbar-Borovsky, M. Brozgol,
G. Yogev, N. Giladi (Tel-Aviv, Israel)
Objective: To test the hypothesis that in older adults changes in gait
variability are related to cognitive function, motor function, and single
walking abilities.
Background: Dual tasking may reduce swing time, a measure reecting balance during gait, and increase swing time variability, a
measure of consistency.
Methods: 172 older adults (mean: 76.5/-4.6 yrs; 62% women)
free of pathologies likely to directly affect gait were evaluated.
Subjects walked while wearing footswitches for 2 minutes under 4
conditions (random order): single task walking, while subtracting
serial 3s, while subtracting 7s, and while phoneme monitoring
(PM). The Berg Balance Test (BBT) and the Dynamic Gait Index
(DGI) were also used. Memory, executive function (EF), attention,

Movement Disorders, Vol. 22, Suppl. 16, 2007

and visual spatial abilities were quantied. The dual tasking decrement of the average % swing time (DTD-A) and swing time variability (DTD-V) were ranked (each in turn); subjects in the top 3rd
and bottom 3rd were compared.
Results: BBT (54.0/-2.4) and DGI (22.9/-1.5) scores were
near perfect. The DTD-A (e.g., mean decrement1%) and DTD-V
(e.g., mean changes1%) were small, but signicant (p0.009) for
all 3 cognitive tasks. For each of the 3 dual tasks, cognitive function
measures were not different in subjects with low vs. high DTD-A,
but subjects with a low DTD-V had higher (better) EF (p0.009).
Subjects with a low DTD-V had higher BBT and DGI scores,
compared to those with a high DTD-V (e.g., p0.004 PM task), but
these scores were not related to the DTD-A. 4) Single task walking
swing time and swing time variability were not related to both
DTDs.
Conclusions: Among relatively healthy older adults, usual walking does not predict the effects of dual tasking and may not be
optimal for fall risk identication. The decreased swing time during
a dual task is also apparently not strongly related to cognitive
function or to measures of gait or balance. Conversely, the dual
tasking effect on the consistency of the stepping pattern (e.g.,
DTD-V) is associated with balance (BBT) and EF. These ndings
underscore the differences in the factors required for balance
during walking and those needed to maintain a steady gait, and
demonstrate that to meet the everyday challenges of dual tasking, a
consistent gait pattern depends on EF.
531
Constipation in Parkinsons disease: A literature review
K.M. Mahawish (Prescot, Merseyside, United Kingdom)
Objective: A comprehensive literature review of bowel disorders in
Parkinsons disease (PD).
Background: Constipation is the most common non-motor feature of
PD, is associated with considerable morbidity and an adverse effect on
quality of life.
Methods: Fourteen databases (including Medline, Cochrane, BIND,
CINAHL & PEDro) were searched for literature relating to bowel
disorders in PD. Search terms included: bowel, constipation, parkinson,
faecal, incontinence. Additionally, all reference lists of identied papers were checked for other possible studies.
Results: 50 papers were identied of which 44 contained original
data. Studies included observational studies, uncontrolled trials and
reviews. Mean age in studies were 56.3-73.9 years and mean Hoehn
& Yahr scores 2.1-3.1. 4 papers investigating 66 subjects describe
degeneration of enteric and autonomic neurons. Further studies
identify a central mechanism contributing to constipation. 3 papers
found signicant levels of constipation in 298 patients according to
the Rome criterion. Incontinence was often the result of overow
around impacted faeces. Colonic Transport Time (CTT) (4 papers,
128 patients) was found to be signicantly prolonged. Defecatory
studies in 287 patients performed using manometric, electromyographic and radiological techniques demonstrate changes in pressures and paradoxical contractions of the anal sphincter and puborectalis. Changes were independent of duration or severity of
disease. Improvement in these parameters was demonstrated in 6
papers investigating the effect of apomorphine. The stepwise management of constipation in PD is well described in the literature. A
randomised single blinded trial demonstrated improvement in bowel
symptoms with psyllium, however no associated changes in CTT or
anorectal function. A further study investigating high bre intake,
demonstrated additional benets in motor function. New therapies
such as abdominal massage produces signicant improvements in
CTT and defecation, but this has not been investigated in PD.
Finally, small studies using the new 5-HT4 receptor agonists show
signicant improvements in CTT and bowel symptoms in PD and
are well tolerated.
Conclusions: Constipation is a common problem in PD and is a
signicant problem for patient and physician. Optimal care is by a

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

multi-disciplinary approach. New treatments appear promising, however need assessment in larger trials.
532
Safe and efcacious treatment of sialorrhea related to Parkinsons
disease, parkinsonism and other neurological diseases with blind
single transdermical botulinum toxin injections into the parotid
glands: Clinical experience with 58 patients
O. De Fabregues, G. Ribera, D. Coll, J. Martinez, D. Canovas,
M. Viguera, M. Marco, F. Miquel, J. Gamez (Sabadell, Barcelona,
Spain)
Objective: To report our experience in treating sialorrhea (S) related
to Parkinsons disease (PD), parkinsonism and other neurological diseases with a one-site transcutaneous retromandibular injection (OSTRInj) of different doses of botulinum neurotoxin type A (BoNT-A)
and B (BoNT-B) into the parotid glands (PG) and to analyse its safety
and efcacy.
Background: S is a common and disabling symptom of PD and
parkinsonism, as well as in other neurological disorders. BoNT has
been reported to be an effective alternative for treating S. The
optimal dose and type of BoNT, best salivary gland, technique and
sites of injection and patient selection, still remain unknown.
Methods: Fifty-eight patients with S related to PD(21), parkinsonism(11), amyotrophic lateral sclerosis (ALS)(12) and other neurological diseases(14) mean age 67,5 years, were enrolled in an
open-label, nonrandomized, prospective, four-stage dose scalation
study. Patients received 20 MU BoNT-A per gland, in rst stage; 40
MU BoNT-A to previous stage nonresponders (PSNR); 1250 MU
BoNT-B to PSNR and 2500 MU BoNT-B to PSNR; through a
OSTRInj into the mass of the PG. Patients were blindly assessed at
baseline and at 1, 3, 6 months to determine the efcacy (by Drooling
Severity and Frequency Scale) and safety (by testing any adverse
effect (AE)).
Results: We performed one hundred and sixty-four treatments:
eighty-nine with 20 MU BoNT-A per gland with 74% decrease of
drooling (DD) and 13% AE; forty-nine with 40 MU BoNT-A to
PSNR: 67% DD 25% AE; nineteen with 1250 MU BoNT-B to
PSNR: 75% DD; and seven with 2500 MU BoNT-B to PSNR: 100%
DD. There was a remarkable benecial response starting at two
weeks and lasting for four months, with sufciently minimal AE for
patients to continue BoNT treatment, except for four who declined
further treatment.
Conclusions: OSTRInj of BoNT into the PG is an easy, safe and
effective technique treating S in neurological disorders. Patients with
parkinsonism and PD have better response to this minimally invasive
treatment of S compared to those with ALS. BoNT-B reduces S in
patients who do not respond to BoNT-A.
533
Intimate partner abuse in movement disorders and the role of
health care professionals
J. Posen, N. Giladi (Tel Aviv, Israel)
Objective: To report our experiences with partners abuse of patients
with movement disorders.
Background: Intimate partner abuse occurs at all levels of society.
Types of abuse often overlap and include physical (hitting, kicking,
throwing objects) sexual (non-consensual relations or relating to partner as sexual object) psychological/emotional (continuous verbal
threats or degrading partners abilities), economic (preventing access to
nances or misuse affecting partners well-being, stalking (obsessive
tracking of partner), isolating (controlling external contacts) and neglect (preventing daily-activities care). People with disabilities are at
risk for partner abuse especially when cognitively impaired or socially
isolate. Premorbid relationship and reduced cognitive, physical or
emotional functioning of partner also affect vulnerability. Victims
present frequently to health care facilities with unexplained injuries,
chronic uncharacterized pain, vague complaints, stress related sypmtoms and depression. Shame, guilt, and fears of abandonment and

S163

barriers to screening by health professionals lead to underreporting of

this serious problem within the family.
Methods: Our experences with 20 families: 13 with Parkinson
disease (PD), six with Huntingtons disease (HD) and one with
Multiple System Atrophy (MSA) are reported. All families were
treated at our center between October 2005 and December 2006 by
the social worker.
Results: 70% (n14) abusers were partner, 30% (n6) patients.
Abuse was rarely the proferred reason for referral and its extent was
revealed only in individual interviews. Among the families with HD,
75% of patient and partner abuse was physical, and with PD, 69% was
emotional. Abuse development risk factors included: severe cognitive
impairment 85% (n17), partners reduced emotional or physical functioning 50% (n10), drug inuenced sexual/gambling behaviours 15%
(n3).
Conclusions: Intimate partner abuse exists in families with movement disorders. It can signicantly affect the familys disease burden and necessitates diagnosis and treatment. Specied questions in
non-judgemental atmosphere and seperate intervies are recommended.

534
Dopamine dysregulation syndrome, impulse control disorders and
subthalamic nucleus deep brain stimulation: A case series and
literature review
S.S. OSullivan, D.A. Gallagher, A.H. Evans, S. Tisch, P. Limousin,
A.D. Lawrence, A. Schrag, A.J. Lees (London, United Kingdom)
Objective: To describe three cases of patients with the dopamine
dysregulation syndrome (DDS) before being treated with subthalamic nucleus deep brain stimulation (STN-DBS), and to review the
current literature on STN-DBS in DDS and impulse control disorders.
Background: DDS is a phenomenon that occurs in patients with
PD who take amounts of dopamine replacement therapy (DRT) in
excess of those required to treat motor symptoms. It is associated
with addictive behaviours regarding DRT, and the development of
signicant psychological symptoms and impulse control disorders
(ICDs). STN-DBS is an effective treatment for the motor symptoms
in advanced Parkinsons disease. Data on STN-DBS as a treatment
for DDS or ICDs are limited, with conicting results.
Methods: Three patients with DDS before treatment with STNDBS are described, and we review the current literature on STNDBS in DDS and impulse control disorders, using a PubMed literature search.
Results: Our three patients ICDs and DDS did not improve postSTN-DBS, despite demonstrating good motor responses to treatment.
21 cases of patients with PD treated with STN-DBS are reviewed from
available literature; 13 cases describe the improvement of known ICDs
post-STN-DBS, including 7 cases of DDS; while 8 cases describe the
development of new ICDs in patients post STN-DBS, including one
patient developing DDS. Individual patient data regarding doses of
dopaminergic medications pre- and post-STN-DBS are available in
eight cases, three demonstrating an improvement of pre-STN-DBS
ICDs or DDS, and ve cases showing a development or exacerbation
of ICDs or DDS post-STN-DBS. Comparing these two sub-groups,
including our three patients, there was no signicant difference in
pre-DBS L-Dopa equivalent units (LEU) amounts per patient, in the
percentage of reductions in total LEU per patient post-DBS, and in the
interval between STN-DBS surgery and the reductions of dopaminergic
medications. Other possible mechanisms of the impact of STN-DBS on
DDS or ICDs are discussed, including the position of electrode placement within the subthalamic nucleus.
Conclusions: Further research into DDS and ICDs in PD is required
to clarify which patients are likely to benet from STN-DBS.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S164 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

535
The behavioural changes in progressive supranuclear palsy (PSP)
are not just a mild form of frontotemporal dementia (FTD)
T.H. Bak, J.R. Hodges (Edinburgh, Scotland, United Kingdom)
Objective: To compare the severity and pattern of the neuropsychiatric symptoms of progressive supranuclear palsy (PSP) with that of the
frontal variant of the frontotemporal dementia (fvFTD).
Background: Since the seminal work of Albert et al on the subcortical
dementia of PSP in 1974 the behavioural changes of PSP have been
considered to reect frontal dysfunction, similar in pattern to frontotemporal dementia (FTD), in particular its frontal variant (fvFTD). However,
the two diseases have never been compared directly in terms of the severity
and pattern of neuropsychiatric symptoms.
Methods: 185 caregivers of patients with PSP and 47 caregivers of
fvFTD patients were administered CBI - Cambridge Behavioural Inventory, a comprehensive behavioural questionnaire assessing different
aspects of emotion, thinking and behaviour such as mood, challenging
and stereotypic behaviour, abnormal beliefs, sleep, eating habits, motivation, disinhibition etc. The data in PSP was additionally analyzed as
a function of disease duration.
Results: The frequency of the behavioural changes was overall significantly lower in PSP than in fvFTD. A common feature in both groups was
apathy. In PSP, it is present already in the early stages of the disease,
within the rst two years after the appearance of the rst symptoms.
Disnihibition and aggression were common in fvFTD but rare in PSP. In
the few cases in which it was observed it did not show any relationship to
disease duration. The only symptom which was more common in PSP than
in FTD was sleep disturbance. Overt psychotic symptoms, such as delusions and hallucinations, were rare in both groups.
Conclusions: Although PSP and fvFTD do share some common
features, notably the pronounced apathy, the pattern of behavioural
disturbance in PSP is not simply a milder form of fvFTD. The relative
rarity of disinhibition and challenging behaviour in PSP, in early as
well as in the advanced stages of the disease, is of particular importance
in reassuring the relatives and carers and advising them about the
possible evolution of the disease.
536
Pain in Parkinsons disease pharmacoepidemiological research
into the consumption of analgesic drugs
C. Brefel-Courbon, S. Grolleau, J. Micaleff, C. Thalamas, O.O. Rascol,
J.L. Montastruc, M. Lapeyre-Mestre (Toulouse, France)
Objective: To describe the pain frequency in patients with Parkinsons disease (PD) using analgesic prescription histories and to compare with that observed in the general population and two other samples
of painful patients: diabetics (neuropathic pain) and osteoarthritis patients (musculoskeletal pain) in two regions in France (Midi-Pyrenees
[MP] and Provence-Alpes-Cote dAzur-Corse [PACA]).
Background: PD patients frequently experienced painful sensations which
could be related to neuropathic pain or/and musculoskeletal pain. Nevertheless,
there are no epidemiological data about frequency of pain in PD.
Methods: PD patients were identied from health insurance system
databases. We compared drug use of this sample during 2005 to randomly
selected controlled population (containing general population, diabetic and
osteoarthritic patients) matched on demographic factors. Pain was dened
by the use of at least one analgesic drug and chronic pain by more than
three deliveries of one specic analgesic drug. Analgesic drugs were
opiates, other analgesics, some antiepileptics and antidepressants and nonsteroidal anti-inammatory drugs [NSAIDs].
Results: The study included 4,162 PD patients in MP and 7304 in
PACA. Sex ratio M/F was 0.8 and the mean age was 77 11 years.
PD patients received signicantly more analgesics than general
population (82% vs 77%) whatever the kind of analgesic drugs.
Their global consumption of analgesic drugs was not signicantly
different from diabetics (82% vs 81%). PD patients received signicantly more antiepileptics and antidepressants than diabetics
whereas NSAIDs were less used. Compared to osteoarthritis patients, PD patients used signicantly less analgesics (82% vs 90%)

Movement Disorders, Vol. 22, Suppl. 16, 2007

and particularly NSAIDs. Concerning chronic pain, PD patients

were signicantly more to receive at least three prescriptions than
general population (61% vs 53%).
Conclusions: The prevalence of pain estimated by analgesic drug
consumption in PD patients was signicantly more important than in
general population. It was similar in diabetics but less than in
osteoarthritis patients. Finally, characteristics of analgesic drug use
were closely related to that observed in diabetics suggesting that
neuropathic pain could be predominant in PD patients.
537
Searching for subtle sleep abnormalities by polysomnography in
early Parkinsons disease: A prospective case-control study
N.J. Diederich, M. Vaillant, O. Rufra, S. Blyth, V. Pieri
(Luxembourg-City, Luxembourg)
Objective: To search for subtle sleep abnormalities by polysomnography (PSG) and sleep questionnaires in early stages of Parkinsons
disease (PD).
Background: According to Braaks theory of ascending degeneration in PD, there is early involvement of sleep regulating centers,
such as locus coeruleus and nucleus pedunculopontinus, even at
premotor stages of the disease. Concordantly, REM sleep behavior
disorder (RBD) has been identied by clinicians as a precursor
syndrome of PD. We hypothesized that there may be other, not yet
detected sleep abnormalities, that could be found at a premotor stage
of the disease, and - a fortiori- at early motor stages of the disease.
Methods: We prospectively recruited 16 PD patients with a short
disease duration (cut-off: 4 years; mean 2.5 1.3 years) and
compared them with 10 age-matched, healthy volunteers. A onenight PSG using 16 channels was performed, with patients and
controls taking their usual medication. In addition the following
questionnaires were used: Epworth Sleepiness Scale (ESS) and
Parkinsons Disease Sleep Scale (PDSS).
Results: There was no difference in terms of age, gender distribution and BMI between both groups. PD patients scored less well
than the controls on ESS, PDSS, and all the selected PSG parameters (see table). However, the difference was only signicant for the
percentage of REM sleep atonia (RSA) and the amount of deep sleep
time (p 0.001, respectively p 0.01). There was no observable
RBD event in any subject. RSA was still 99% in two PD patients.
Conclusions: At an early stage PD patients show subtle abnormalities on numerous PSG parameters. However, as a group, they can
reliably be differentiated from age-matched controls only by reduced
RSA. In some individual patients, RSA can still be complete. The value
of the sleepiness scales for differentiation is questionable. We caution
that PSG is probably only useful within a broader clinical test battery
when searching for premotor stages of PD.

Results of polysomnography and sleep questionnaires in

16 PD patients and 10 age-matched controls
Variable

total sleep time (min)

sleep latency (min)
stage 3&4 (min)
sleep efciency (%)
stage 3&4 (%)
apnea/hypopnea index (n/hour
mean O2 saturation(%)
minimal O2 saturation(%)
REM sleep with atonia (%)
REM density
microarousals (n)
Parkinsons Disease Sleep Scale
Epworth Sleepiness Scale

Controls (n10)

PD patients
(n16)

value

(SD)

value

(SD)

351.8
26.6
67.1
78.6
15.85
1.9
94.1
83.1
98.6
287.5
68.7
64.61
6.8

(53.97)
(25.40)
(41.79)
(11.27)
(9.55)
(2.69)
(1.20)
(13.80)
(1.54)
(84.99)
(48.33)
(9.32)
(3.85)

308.8
36.94
27.06
68.13
6.34
7.69
94.81
84.19
83.35
249.1
80.38
69.89
7.88

(56.56)
(28.79)
(28.28)
(14.83)
(6.67)
(10.49)
(2.01)
(14.51)
(22.76)
(135.1)
(59.48)
(8.04)
(4.49)

p-value

0.07*
0.19
0.01$
0.07*
0.22
0.10
0.32*
0.25$
p 0.001*
0.43*
0.54
0.0516*
0.54*

* Anova on raw values; Anova on log-transformed values; $

Mann-Withney test

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

538
Neuropsychological predictors of hallucinations in Parkinsons disease: Cognitive and perceptual biases
D.L. Whitehead, A.D.M. Davies, J.J. Downes, C.J. Turnbull,
J.R. Playfer (London, United Kingdom)
Objective: To investigate specic neuropsychological correlates of
hallucinations in Parkinsons disease.
Background: Hallucinations in Parkinsons disease are associated
with increased motor severity and global cognitive decline. Less is
known about the specic cognitive prole of hallucinating patients, and
which decits are strongly and independently associated with hallucinations.
Methods: 78 patients were interviewed about their experience of
hallucinations and assigned to a non-hallucinators group (N35) and
a visual hallucinators group (N28). All participants underwent a
comprehensive battery of neuropsychological tests tapping domains
including executive function, memory and visual perception.
Results: The hallucinators demonstrated both greater disease severity (UPDRS-III) (p.01) and greater global cognitive decline
(MMSE) than their non-hallucinating counterparts (p.001). They
also showed decits in delayed prose recall, verbal uency, and
visual closure and visual object recognition and visual interference
independently of age, disease severity and estimated premorbid IQ
(all at p.05). In addition to failure to produce correct answers
hallucinators also showed a tendency to produce erroneous and
task-irrelevant responses in two key areas of verbal and visual
performance. Hallucinators produced more confabulatory responses
during prose recall, deviating from the storys original theme and
more intrusions of irrelevant words on the verbal uency task.
They also demonstrated a greater number of erroneous misperceptions on visual object recognition tasks. Hallucinators scored signicantly higher on two derived measures of verbal intrusions
(p.01) and visual misperceptions (p.05) independently of age,
premorbid IQ and disease severity.
Conclusions: Hallucinating PD patients display decits in source
monitoring on simple prose recall and verbal uency tasks, giving
greater salience to internally-produced and non-veridical responses.
Compromised function of dopaminergic pathways ascending to the
dorsolateral prefrontal cortex (DLPFC) may underlie errors of source
monitoring. Against a backdrop of visual decits, such a bias may lead
patients to favor top-down modes of visual processing where expectancy plays a key role and errors of perception or hallucinations result.
539
Prevalence of non-motor symptoms in idiopathic Parkinsons disease. The PRIAMO study
C. Colosimo, PRIAMO Study Group, Italy (Rome, Italy)
Objective: To describe the prevalence of nonmotor symptoms
(NMS) and their impact on quality of life (QoL) in a large sample of
Italian patients with Idiopathic Parkinsons disease (IPD) from the
PRIAMO study.
Background: NMS often remain under-diagnosed and under-treated
during clinical practice. Recent studies highlight the relevance of NMS
in PD quality of life and may have consequences for patients clinical
management.
Methods: PRIAMO is a 2-yrs ongoing longitudinal observational
study that enrolled 1072 IPD patients from 58 centers widely distributed throughout Italy. Clinical diagnosis of IPD was performed according to the current diagnostic criteria. All patients underwent a clinical
examination. NMS were grouped in following domains: gastrointestinal, pain, urinary, balance, sleep, fatigue, apathy, attention/memory,
cutaneous, psychiatric, respiratory. Prevalence was calculated for each
NMS group.QoL was measured by PDQ-39, cognitive impairment by
FAB and MMSE, depression and anhedonia by HDRS and SHAPS
respectively.
Results: Demographics of IPD study population were: meanSD
age 679 yrs; 60% males; average disease onset 6111 yrs; Hoehn
& Yahr median stage 2 (Q1-Q3:1.5-2.5). On average each patient

S165

presented 5.52.5 NMS. The more prevalent (50%) NMS were:

psychiatric (67%; mainly anxiety and depression), sleep disorders
(64%), gastro-intestinal (61%), pain (61%), fatigue (58%), urinary
(57%). The more frequent NMS at PD onset were pain (28%),
anxiety (25%), fatigue (24%) and depression (22%). For each NMS,
prevalence increased with disease severity (UPDRS-III p0.01).
QoL (PDQ-39) was inversely related with increased prevalence of
NMS (p0.01). FAB mean score was lower (p0.05) in patients
with vs without attention/memory symptoms. Mood assessment
showed higher HDRS and SHAPS scores in patients complaining
for attention/memory symptoms (p0.01).
Conclusions: PRIAMO study shows that the most frequent NMS are
anxiety/depression and pain both at disease onset and during the course
of the disease. NMS are related to disease severity and motor disability
and signicantly affect QoL.
540
Improvement of hyperdopaminergic behaviors by subthalamic nucleus (STN) stimulation
E. Lhommee, C. Ardouin, H. Klinger, J. Xie, A. Kistner, S. Thobois,
P. Pollak, P. Krack (Grenoble, France)
Objective: To evaluate hyperdopaminergic behaviors (nocturnal hyperactivity, diurnal somnolence, binge eating, creativity, hobbyism,
punding, risk behaviour, compulsive shopping, pathological gambling,
hypersexuality, addiction to dopaminergic drugs) and mood uctuations in Parkinsons disease (PD) patients before and 12 months after
bilateral STN stimulation.
Background: STN stimulation improves levodopa-responsive motor
symptoms in Parkinson disease (PD). Hyperdopaminergic behavioral
disorders are frequent in PD patients treated by dopaminergic drugs.
There are no prospective studies evaluating the effect of STN stimulation on these behaviors.
Methods: Twenty-seven patients operated in two centers were prospectively evaluated, preoperatively and 12 months after STN stimulation. Their motor and dyskinesia score were respectively evaluated
with the UPDRS III and IV (items 323334). Mood and behavior
were assessed using a newly developed scale (see accompanying poster
by C Ardouin & al).
Results: Motor score improved from 3813 off medication at
baseline to 168 (off drug on stimulation) at 12 months and
dyskinesia decreased by 75%. The levodopa equivalent dose was
reduced by 78%. Non-motor uctuations almost completely
disappeared with a decrease in both on-period (hypo)mania
(p0.0001) and off period depression (p0.003). There were a
signicant decrease in nocturnal hyperactivity (p0.01), creativity
(p0.02), hobbyism (p0.001), and dopaminergic drug addiction
(p0.03).
Conclusions: Hyperdopaminergic behaviors were less prominent one
year after STN stimulation. We speculate that this is largely related to
the drastic reduction in dopaminergic treatment. STN DBS may be
useful in patients with pathological behaviors that can be attributed to
dopaminergic treatment.
541
Validation of the CAMCOG neuropsychological assessment in Parkinsons disease
P.J. Hobson, J.R. Meara (Rhyl, Denbighshire, United Kingdom)
Objective: To evaluate the validity, reliability, sensitivity and specicity of the CAMCOG the cognitive screening instrument of the
Cambridge Examination for Mental Disorders of the Elderly, to detect
dementia in Parkinsons disease (PD).
Background: Parkinsons disease patients have around a 5-fold
risk of developing dementia compared to the general population. For
that reason, the psychometric properties of neuropsychological assessments should be valid, reliable, and have acceptable sensitivity
and specicity to detect true cases of cognitive impairment or
dementia. Although the CAMCOG has been employed in several

Movement Disorders, Vol. 22, Suppl. 16, 2007

S166 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

patient populations, its psychometric properties in PD has not been
established.
Methods: A sample (n 42) of clinically probable PD patients
randomly drawn from a community based register participated in
this investigation. We compared the CAMCOG against a battery of
standardized neuropsychological tests: Rivermead orientation test;
Boston naming test; WAIS-R (Comprehension test, Block Design;
Arithmetic); Camden Memory tests; Digit span; Weigl Colour
Form Sorting Test; Ravens Coloured Progressive Matrices; Star
Cancellation test; Letter cancellation. Demographic, disease specic and pre-morbid intellectual functioning details were also recorded.
Results: The internal reliability for the overall CAMCOG and its
individual domains exceeded the 0.7 standard (0.82, Cronbachs
alpha). The test-retest reliability of the CAMCOG was found to be
excellent (r 0.88, p 0.0001). Comparisons between the individual domains of the CAMCOG and the corresponding neuropsychological assessments revealed signicant correlations (p 0.05).
The CAMCOGs optimum cut point to detect dementia was 82/105,
giving a sensitivity and specicity 90% and 92% respectively (gure). Higher pre-morbid intellectual function (p0.001) was found
be predictive of better CAMCOG test performance.
Conclusions: This investigation has established the utility of the
CAMCOG assessment to detect dementia in PD patients compared
to existing standardized neuropsychological measures. It was found
to be brief in its administration, easy to collate and score and well
tolerated by the patients. Furthermore, it had very good psychometric properties in terms of its test/retest reliability, validity, sensitivity and specicity, and was relatively independent of confounding
factors.

Background: Pain is a frequent non-motor symptom in PD, that

can not always be explained by peripheral mechanisms and may
therefore be at least partly attributed to altered central pain processing. Of areas involved in degeneration, the basal ganglia are suggested to play a role, locus coeruleus is established as area of
descending pain inhibition and the limbic system acts on the affective component of pain.
Methods: Twenty consecutive painfree patients with PD and
predominant affection of one side and 20 sex- and age-matched
controls underwent thermal quantitative sensory testing (QST) on
six sites of the body (both sides within dermatomes of maxillary
nerve, C6 and L5). Thresholds for warmth and cold perception
(WPT, CPT), heat and cold pain perception (HPPT, CPPT), heat and
cold pain tolerance (HPTT, CPTT) were assessed and compared.
Results: No difference could be detected for WPT and CPT between
patients and controls or comparison within the group. HPT was significantly lower on the more affected side in face and upper extremity of
patients compared to the less affected side and to controls. HPTT and
CPTT were signicantly lowered in PD patients compared to controls
all over the body.
Conclusions: Thermal thresholds reect the different sites of alteration in PD: Perception thresholds, which are attributed to peripheral
nerve function, are unaltered. HPT is lowered on the more affected
side, probably because of degeneration in basal ganglia or loss on
neurons within the locus coeruleus. Lowered tolerance thresholds on
both sides refer to an altered affective component of pain sensation in
PD, probably associated with known degeneration within the limbic
system.

543
Executive functioning in early-onset Parkinsons disease
A. Kummer, S.T. Camargos, M.C. Cunningham, D.P. Maia,
F. Cardoso, A.L. Teixeira (Belo Horizonte, Brazil)

FIG. 1 (541).

542
Altered pain and pain tolerance thresholds in Parkinsons disease
a result of multifocal degeneration?
D. Samal, D. Haubenberger, T. Sycha, E. Auff (Vienna, Austria)
Objective: We sought to assess different thermal thresholds in Parkinsons disease (PD) patients, map them comprehensively and compare them to controls.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To investigate the performance of early-onset Parkinsons

disease (PD) patients in the Frontal Assessment Battery (FAB).
Background: The FAB is an easily administered bedside test developed to assess executive functions. PD patients frequently have dysexecutive syndromes. Moreover, dementia in PD is the prototype of
subcortical dementia. Early-onset PD is supposed to have a long course
of disease with less cognitive decline. However, few studies have
investigated executive functioning in early-onset PD.
Methods: We administered the FAB and the Mini-Mental State Examination (MMSE) to 48 subjects with PD started before age 50 years.
Neurologic examination, including Unied Parkinsons disease Rating
Scale (UPDRS), Hoehn-Yahr scale (HY) and Schwab-England activities
of daily living scale (SEADL), was also performed. Only patients with
MMSE scores higher than 20 were included in this study.
Results: The mean age and the mean age of onset of PD were 50.1
(SD 8.0) and 40.5 (SD 7.4), respectively. The mean BAF score
was 12.3 (SD 3.6). Seventeen patients (35.4%) scored under 11 in
the FAB, a cut-off point for signicant dysexecutive syndrome. We
found no correlation between FAB scores and the variables gender, age
of onset, UPDRS score, HY stages and SEADL score. There was a
positive correlation between FAB and MMSE performance as shown
by linear regression analysis (p0.001).
Conclusions: The present sample of non-demented early-onset PD
patients had a very low performance in the FAB. This result suggests
a signicant impairment of executive functioning in early-onset PD
what it is not in line with the traditional view that these patients are less
cognitively compromised.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

PARKINSONS DISEASE
544
Continuous levodopa duodenal infusion in advanced Parkinsons
disease: Long-term follow-up
M. Canesi, I. Isaias, M. Zibetti, F. Mancini, L. Manfredi,
M. Del Fante, L. Lopiano, G. Pezzoli, A. Antonini (Milano, Italy)
Objective: Evaluate long-term treatment with Ldopa/carbidopa by enteral infusion via a portable pump in advanced Parkinsons disease (PD).
Background: Motor uctuations are a common and disabling complication in the treatment of PD. Apomorphine infusion and deep brain
stimulation are available strategies to alleviate these complications. The
ideal pharmacological treatment would be continuous dopaminergic
stimulation.
Methods: We evaluated prospectively open-label 12 PD patients
(H&Y3) who fullled UK brain bank diagnostic criteria. All patients
presented with motor uctuations and dyskinesia that could not be
controlled with Ldopa and dopamine agonist oral treatment. None of
them had dementia, active hallucinations or neuroleptics treatment.
Results: Two patients dropped out. In the remaining 10, Ldopa/
carbidopa infusion rate ranged between 40 and 120 mg/hr and bolus
doses of Ldopa ranged from 100 mg to 200 mg. There was no
signicant difference in the total daily doses of Ldopa administered by
infusion at 6 and 12 months compared with equivalents at baseline. The
mean of off periods during the 14 hrs of observation were signicantly reduced at 12-month treatment period (p0.01). At 12 months,
the time in off state had reduced 9.5-fold. The time with disabling
dyskinesia was signicantly reduced by 4-fold at the end of the study
(p0.01). There was no signicant change compared to baseline in the
UPDRS-III score evaluated in either the on or off condition. There
were signicant improvements in on UPDRS-II and IV (p0.02).
Improvements were also found in 4 of the 8 subscales of the PDQ-39,
including mobility (p0.008), activities of daily living (p0.008),
stigma (p0.031) and bodily discomfort (p0.031) after 12-month
treatment.
Conclusions: This study demonstrated a long-standing signicant reduction in off time and a signicant reduction of dyskinesia despite
overall unchanged total Ldopa doses. Ldopa/carbidopa duodenal infusion
improved motor conditions and signicantly reduced disabling dyskinesia
in patients with advanced PD, resulting in signicant benet in quality of
life measures. Pulsatility of oral administration rather than Ldopa itself
could be responsible for the development of motor uctuations.
545
Correlations of initial mentation rating in UPDRS and progression of motor and cognitive functions in Parkinsons disease
J.G.G. Hou, E.C. Lai (Houston, Texas, USA)
Objective: To investigate the reliability of the rst item (mentation)
in the Unied Parkinsons Disease Rating Scale (UPDRS) as correlatedwith the progression of motor and cognitive functions in patients
with Parkinsons disease (PD).
Background: The mentation item of UPDRS allows raters to assess
patients cognitive and memory dysfuntion. Some studies revealed that
cognitive impairment was correlated with the progressions of PD motor
symptoms. Using this single item as an indicator to predict such
correlation may be useful in everyday clinical practice, though the
reliability is not tested.
Methods: 210 idiopathic PD patients were seen by movement disorder specialists. The initial and most recent scores were collected.
Mean duration of follow ups were about 30 months. Patients were
divided into four groups: A) no dementia, mentation score 0; B) mild,
1; C) Moderate, 2; D) Severe, 3 & 4. UPDRS III (motor), subtotal
scores of gait (items 27 to 31), H&Y scores and Mini Mental Status
Exam (MMSE) were assessed for each group. The differences between
groups were determined by analysis of variance.
Results: Sixty patients were in group A); 102 in B); 35 in C); and 13 in
D). At initial visits, mentation score is highly correlated with the mean
UPDRS III: A) 21.6; B) 29.5; C) 33.8; D) 38.2 and subtotal scores of gait:

S167

A) 5.7; B) 7.4; C) 9.0; D) 11.2; and H&Y scores A) 2.3; B) 2.6; C) 3.0; D)
3.7. After 30 months, neither UPDRS III, gait subtotal, nor H&Y showed
signicant progression in all groups. However, 75% (45/60) of group A)
reported worsening of mentation (37 worsened by only 1 scale), with 28%
in B), 20% in C), and none in D). Mean MMSE in the latest visits were A)
28.2; B) 27.7; C) 24.1; D) 13.0.
Conclusions: Our study shows that mentation rating has a high
correlation with the severity of PD motor symptoms. After 30-month
follow-ups, the motor symptoms remained fairly stable, probably due
to specialized care from our Parkinsons Center. In spite of this,
patients subjective cognitive functions deteriorated. It is especially
notable for those who initially were normal in cognition but later
reported mild progression. Still, mentation rating correlates with their
cognitive functions reliably, as measured by MMSE. We conclude that
UPDRS item mentation is a reliable predictor of the motor and
cognitive functions for PD patients.
546
Risk factors of falls related to balance and gait disorders in Parkinsons disease prospective study
M. Rudzinska, J. Stozek, W. Chwala, S. Bukowczan,
K. Banaszkiewicz, A. Szczudlik (Krakow, Poland)
Objective: To nd which parameters of the clinical and objective
tests of balance and gait assessment are predictors of falls in Parkinsons disease (PD) patients.
Background: Balance and gait disorders typically accompany Parkinsons disease and might be risk factors or causes of falls.
Methods: 105 consecutive moderately advanced PD patients (II-IV
H&Y stage, mean age 6710 years, mean disease duration 6.53.5
years) underwent a multidisciplinary examinations to exclude those
with falls not related to PD including visual or orthopaedic problems
severe enough to interfere with balance. Balance and gait were tested
in all patients with UPDRS, Functional Reach Test (FRT), Tinetti
Balance Test (TBT), Tinetti Gait Test (TGT), Up and Go Test (U&GT),
10 meter walk test, Freezing of Gait-Questionnaire and computerised
static posturography. The Vicon 250 gait analysis (length of steps,
cadence, walking speed, single and double support time, angle of gait
instability in coronal plane, etc.) was performed additionally in 44
patients (22 fallers). The number of falls was assessed during one year
prospective observation.
Results: 102 patients completed one year observation and 52(51%)
of them reported 243 falls. In univariate regression analysis the following results of UPDRS part II and III subscore freezing (OR2.5),
walking (OR4.5), arising from chair (OR2.4), posture (OR2.5),
postural stability (OR3.3), as well as FOG-Q(OR1.2), U&GT
(OR1.2) and TBT (OR1.3), TGT (OR1.6), FRT (OR1.2),
higher number of steps in walk test (OR1.2), and posturographic
parameters: higher mean AP and lateral sway during quiet standing on
the soft ground (OR1,6) and on the hard ground (OR1.5) and low
mean velocity in dynamic tests (OR1.2) were risk factors of falls.
Standard spatiotemporal and kinematic parameters of Vicon gait analysis were not related to the occurrence of falls. In multivariate regression analysis older age (OR1.1), UPDRS falling (OR3.1) and
waking (OR3.2), FOG-Q (OR1.2), FRT (OR1.1), postural stability (OR3.4) and higher mean AP sway during quiet standing on the
soft ground (OR1.5) were independent risk factors of falls.
Conclusions: The simple clinical tests of balance and gait assessment
as well as some posturographic parameters are useful in predicting falls
in PD patients.
547
Elevated prevalence of malignant melanoma in Israeli patients
with Parkinsons disease
R. Inzelberg, J.M. Rabey, R. Djaldetti, A. Reches, S. Badarny,
S. Hassin-Baer, H. Trau, J. Aharon-Peretz, M. Huberman, L. Gilead,
N. Giladi (Kfar Saba, Israel)
Objective: To assess the frequency of melanoma in Parkinsons
disease (PD) patients by active dermatological screening.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S168 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: Recent retrospective studies have demonstrated a 2-5
fold higher risk of melanoma among patients with PD than in the
age-matched general population (Olsen, JH et al., 2005, 2007).
Methods: Consecutive PD patients in 12 Israeli centers were evaluated, using a detailed questionnaire, for medical history, current medications, and previous skin disorders. Patients were actively screened
by a dermatologist, and suspicious lesions were biopsied and evaluated
by a central laboratory. Observed prevalence of melanoma in the PD
population was compared to age- and sex-matched patients in the Israel
National Cancer Registry (5-year limited-duration prevalence).
Results: Of 1395 patients who participated in the survey, 62.5% were
male, 97.8% were Caucasian, 70.0% were greater than age 65, and 67.5%
were never smokers. Average PD duration was 7.3 years and mean Hoehn
and Yahr (H&Y) stage was 2.50.9. Dopaminergic agents were used by
95.3% of patients (79.1% on levodopa). A history of melanoma was
reported by 14 patients (1.0%), 6 occurring before and 8 after PD diagnosis. Biopsies were performed on 85 suspicious lesions from 71 patients.
Eight of these patients (0.6%) were diagnosed with in situ melanoma and
1 (0.1%) with invasive melanoma. Since 3 newly diagnosed patients also
had a history of melanoma, the total number of patients with current or
prior melanoma was 20 (1.4%). This prevalence in PD patients was 4.4
times greater (95% CI 2.6-7.6) than expected from melanoma prevalence
in an age-and sex-matched cohort from the Israel National Cancer Registry. The difference was accounted for by higher prevalence of in situ
melanoma. No relationship was seen between melanoma and PD duration,
H&Y stage, or levodopa treatment.
Conclusions: These results, showing a higher prevalence of melanoma in patients with PD than expected from the general population,
support the suggested association between melanoma and PD. Active
screening may be responsible for some of the increase, yet it does not
appear related to levodopa use.
548
The effect of mechanical vibration in Parkinsons disease (PD): A
randomized blind controlled trial
A.R. Bayes, M.V. Cosculluela (Barcelona, Catalunya, Spain)
Objective: To compare the effect of a program of physiotherapy (PT)
training on the motor symptoms of Parkinsons disease, combined or
not with the application of mechanical vibration (MV).
Methods: Subjects: 34 PD from Parkinson Unit (n17) and Aragon Parkinson Association (n17) recruited and after collecting their
informed consent. Inclusion criteria: Idiopathic PD (Brain Bank criteria), stage H&Y I-III, no cognitive impairment (Minimental State24),
not attending physiotherapy at least a month before beginning the study
Protocol evaluation: pre and post intervention: UPDRS, gait test,
Bergs Balance Scale, H&Y, Life Quality scale PDQ-39; Minimental
State (pre-intervention), patients satisfaction assessment (post-intervention). Procedure: Subjects were randomly assigned to a trained
(PT MV) or control group (PT). Treatment: PTMV group: 12 PT
sessions (45) x 3days/week 5 MV training [individualized sessions
6 turns (45-60) x 4-5Hz with 15 pause]; PT group: 12 PT sessions
(45) x 3 days/week. Pre and post treatment blind evaluation had had
following the protocol; Analysis: Descriptive statistic analysis with
SSPP/WIN (V.12) is used. There was applied Wilcoxon test in order to
compare start evaluations and nal evaluations.
Results: N34 PD; 50% men; average age 66a /-9 (min 42; max
80). Average of years of evolution: 8/-4 (mn 1; max 21).The patients
were randomized in two groups. Signicant differences between the
two samples are not observed. Initial motor subscale (MS) UPDRS
group PT (n15) 29,73(/-9,07); group PTMV (n19) 28,42
(/-14,93). At the end of the treatment signicant statistical improvement is registered in the UPDRS (part III) in both groups (PT group
P0,036; PTMV group P0,010) Although, clinically, improvement
is better in the MV group than in the PT group, differences between
both groups are not statistically signicant. Quality of life, changes had
not been appreciated. 91% of the participants value as good or very
good their degree of satisfaction.
Conclusions: PT combined or not with MV training improves PDs
motor symptoms. Even though a greater clinical improvement in the

Movement Disorders, Vol. 22, Suppl. 16, 2007

treatment of PT combined with MV training is observed, it would be

necessary to increase the sample to corroborate this clinical impression.
549
The neuropathological substrate of the dementia of Parkinsons
disease
T. Voss, M.S. Forman, J. Duda, H. Hurtig (Philadelphia,
Pennsylvania, USA)
Objective: To investigate the extent of Alzheimer and Lewy body
pathology in a cohort of patients with Parkinsons disease and dementia
(PDD).
Background: The impact of Alzheimer versus Lewy body pathology
in dementia in Parkinsons disease remains unclear. We evaluated the
extent of Alzheimer and Lewy body pathology in a cohort of patients
with PDD.
Methods: 172 of the patients who presented to the Parkinsons
Disease and Movement Disorders Center at the University of Pennsylvania from 1985 to 2006 were autopsied. Forty-eight of these were
clinically diagnosed with PDD. Patients in this cohort whose dementia
began within 1 year of motor symptoms were considered to have
dementia with Lewy bodies and were excluded. Alzheimers disease
(AD) pathology was assessed by standard criteria: CERAD for senile
plaques (SP) (None 0; mild A; moderate B; severe C); Braak
and Braak for neurobrillary tangles (NFT) (I-II, III-IV, V-VI). Subjects were also staged for the probability of a post-mortem diagnosis of
AD, using the National Institute of Aging-Reagan (NIA-R) criteria
(low, intermediate, or high probability). Lewy body (LB) pathology
was classied as brainstem predominant, limbic/transitional or diffuse/
neocortical, as per recent consensus criteria (Neurology, 2005).
Results: Staging for SP revealed 50% (24/48) with a CERAD score
of 0 or A, 23% (11/48) with a score of B and 27% (13/48) with a score
of C. Staging for NFT revealed 44% (21/48) with a Braak and Braak
score of I-II, 40% (19/48) with a score of III-IV and 17% (8/48) with
a score of V-VI. By NIA-R criteria, 44% (21/48) met intermediate or
high probability for AD, while 56% (27/48) were staged as no or low
probability. Staging of LB pathology showed that 23% (11/48) and
77% (37/48) had limbic/transitional and diffuse/neocortical pathology,
respectively. No patients were staged as brainstem predominant.
Conclusions: In this population of patients with PDD, limbic/transitional and diffuse/neocortical LB pathology correlated more strongly
with dementia than did AD pathology, which was present in a minority.
550
Outcome of acute ischemic stroke in hospitalized Parkinsons disease patients
M. Niethammer, H.C. Schumacher, B.T. Bateman, C. Henchcliffe
(New York, New York, USA)
Objective: To assess the prevalence of risk factors for ischemic
stroke as well as outcomes in Parkinsons disease (PD) patients hospitalized for acute ischemic stroke (AIS).
Background: AIS and PD are common age-related disorders. Previous studies have suggested a lower rate of stroke risk factors in the PD
population, but little is known about the impact of PD on outcome after
AIS.
Methods: We utilized the Nationwide Inpatient Sample, a discharge
database of 20% of all U.S. hospitalizations, for the years 1998-2003.
Patients age 45 years hospitalized with the primary discharge diagnosis AIS were selected using appropriate ICD-9-CM codes. In this
cohort, 4,801 patients also diagnosed with PD were compared to
234,338 AIS hospitalizations without PD (controls). Chi-square and t
tests were used, where appropriate. Binary logistic regression analysis
was used to determine whether PD was associated with predened
outcomes. Results are presented as odds ratios and 95%-condence
intervals (OR [CI]).
Results: PD patients with AIS were less likely than controls to have
hypertension (57.1 vs. 65.9%, OR 0.69 [0.65-0.73]), atrial brillation
(21.6 vs. 23.4%, OR 0.91 [0.85-0.97]), valvular heart disease (3.6 vs.
4.4%, OR 0.80 [0.69-0.93]), metabolic disorder dened as either dia-

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

betes, obesity or hyperlipidemia (34.6 vs. 42.6%, OR 0.72 [0.67-0.76]),
or smoking (3.3 vs. 9.7%, OR 0.32 [0.27-0.37]). The prevalence of
coronary artery disease (27.0 vs. 28.1%, OR 0.95 [0.89-1.01]), congestive heart failure (14.7 vs. 14.0%, OR 1.06 [0.98-1.15]), and chronic
renal failure (0.2 vs. 0.3%, OR 0.68 [0.38-1.24]) was similar in both
cohorts. When adjusted for age, gender, race and stroke risk factors, PD
patiens had a lower in-hospital mortality rate (OR 0.84 [0.75-0.94]), but
were less likely to be discharged home compared to controls (OR 0.68
[0.64-0.72. Adjusted length of stay (6.217.2 vs. 6.017.4 days) and
total charges (US$ 15,05123,925 vs. 16,61323,825) were similar in
both cohorts.
Conclusions: Despite a more favorable stroke risk factor prole PD
patients suffering an AIS are less likely to be discharged home compared to controls despite having lower adjusted in-hospital mortality.
This suggests that AIS has a higher impact on stroke-related disability
in PD versus non-PD. Prospective studies are needed to validate these
results.
551
Side and symptom of disease onset is related to lifespan in Parkinsons disease
B.K. Scanlon, H.L. Katzen, D.A. Nation, R.A. Rodriguez,
S. Papapetropoulos, J.A. Ledon, A. Capano, D. Wilensky, J. Zitser,
C. Singer, B.V. Gallo, J.A. Jagid, B.E. Levin (Miami, Florida, USA)
Objective: To investigate whether specic motor phenotypes are
differentially related to lifespan in Parkinsons disease (PD).
Background: Research has linked different disease parameters to
mortality in PD; however, the contribution of initial symptom presentation has yet to be determined.
Methods: The study sample consisted of 104 participants enrolled in
a prospective study of idiopathic PD initiated in 1985. Participants were
grouped according to side (right vs. left) and type of symptom (tremor
vs. rigidity/bradykinesia) at disease onset. Twenty years later, the
United States Social Security Death Index was utilized to gather
mortality information; death date was conrmed in 45 participants.
Mean age of the deceased sample at baseline evaluation was 67.4 years
(SD7.6), mean Hoehn and Yahr stage was 2.2 (SD.8), mean disease
duration was 7.4 years (SD5.7), and mean Mini Mental Status Exam
(MMSE) score was 26.2 (SD2.5); 53% were male. Mean age at death
was 78.2 years (SD7.2) and mean years from diagnosis to death was
18.4 (SD6.8).
Results: 22 ANCOVA was performed to examine the effects of
side and symptom at onset on mortality. Using age of disease onset as
a covariate, a main effect for side of disease onset emerged. Participants with right-sided onset lived longer (p.031). No main effect for
symptom at onset was found. Although no interaction between side and
symptom was observed, cell means indicated that participants who
developed right-sided tremor at onset (RTO) lived longer than participants who developed left-sided tremor (LTO), right-sided bradykinesia/rigidity (R B/R), or left-sided bradykinesia/rigidity (L B/R). Participants with RTO had an average lifespan of 82.1 years, 6.7 years longer
than the other three subgroups combined (p.001).
Conclusions: These ndings demonstrate an intricate relationship
between motor symptom, side of onset, and mortality. Participants who
initially develop right-sided tremor may represent a distinct subgroup
of PD participants who have a longer lifespan.
552
Weight loss and disease progression in Parkinsons disease
Y. Tsuboi, Y. Baba, H. Inoue, T. Kobayashi, T. Yamada (Fukuoka,
Japan)
Objective: To determine the temporal course of weight change and
progression of motor symptom in Parkinsons disease.
Background: Weight loss commonly occurs in the course of illness
of patients with Parkinson disease (PD). Cause of weight loss in PD
patients remains obscure. The weight loss in PD starts years before the
onset of PD motor symptoms, indicating that it is not caused by energy

S169

intake or energy expenditure. It is well documented that recovery of the

weight is seen after surgical treatment of PD such as STN DBS.
Methods: Height and weight data were collected around the onset by
using retrospective chart review in 28 consecutive PD patients. Series
of the weight from ve years before and after the onset were analyzed.
We divided cases into two groups; weight loss group (WL) who shows
decreased weight of over 2 kg /year, and no weight loss group (NWL)
who has no weight change or weight loss of less than 2 kg /year around
the onset. BMI was calculated as weight in kilograms. We compare the
BMI changes and disease progression between the two groups.
Results: Among 28 patients, WL include 17 cases (M:6, F:11), while
NWL include 11 cases (M:5, F:6). Groups were similar in regard to age
(WL:67.5, NWL:64.6 yrs, p0.35), disease duration (WL:5.7, NWL:
6.3 yrs, p0.67). BMI at onset in patients with WL was not signicant
difference from that of NWL (21.6 vs. 22.2, p0.77). In contrast, BMI
in WL at the latest evaluation was signicantly lower than that in NWL
group (19.9 vs. 22.4, p0.015). Hoehn-Yahr stage at the latest evaluation was 3.0 in WL group and 2.2 in NWL group, showing greater
progression of motor symptom in WL compared with NWL. Considering with disease duration, progression of PD symptoms was faster in
WL than that of NWL. Weight loss to a greatest extent in WL was seen
in 2 years (4 cases), one year (2 cases) before the onset, and after PD
diagnosis in 11 cases.
Conclusions: BMI in PD was on average 11% lower in WL than in
NWL. There was difference regarding to disease progression between
WL and NWL. Weight loss starts prior to diagnosis of PD. Our results
suggested that weight loss at diagnosis of PD may partially predict the
clinical course of PD. It is also speculated that autonomic dysfunction
preceding motor symptoms may inuence metabolism in central nervous system affecting parkinsonism as well as peripheral system affecting BMI.

553
Sleep disturbances in Parkinsons disease patients
D. Dobi, M. Kapisyzi, B. Kokona, J. Kruja (Tirana, Albania)
Objective: The aim of our study is to evaluate the frequency and
nature of sleep disturbances in Parkinsons disease (PD) patients.
Methods: We studied 120 (68 females, 52 males) consecutive PD
patients attending the movement disorders unit of the University Hospital Mother Theresa, Tirana and the Movement Disorders unit of
regional hospital of Gjirokastra and 112 age-matched healthy controls.The diagnosis of PD was established from a Movement Disorder
specialist. UPDRS and Hoehn and Yahr score were performed to all
patients. Sleep assessment was done using a 23-question, validated
sleep questionnaire.
Results: Mean age of PD patients and control group were 58,3 years
old (SD_ 10,45) and 5,7 years (SD_ 3,85) respectively.(p0.05)
Mean duration of the disease was 5,7 years (SD_ 3,8) Sleep problems
was seen in 51 patients (41,5%) compared to 12% at healthy controls.
Insomnia was observed in 32% of patients and 12% of controls Nightmares in 32% of patients and 5% of healthy controls, Excessive day
time sleepiness in 15% of PD patients and 6% of controls (p0.025)
The presence of nightmares was signicantly associated with higher
Hoenh and yahr score(p0,002), high UPDRS part I score(p0,001)
and levodopa daily taking medication(p0,004) The sleep latency was
longer in PD patients as compared to controls (p0,001).
Conclusions: Multiple logistic regression anlysis showed association
of sleep disturbances with UPDRS score, levodopa dose and Hoenh and
Yahr stage of the disease. Sleep disturbances are much more common
in PD patients compared to age matched controls and correlate with
increased severity of the disease.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S170 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

554
Training and placebo effects outweigh effects of single session
conventional rTMS and theta burst stimulation in PD patients
H. Rothkegel, M. Sommer, T. Rammsayer, C. Trenkwalder,
W. Paulus (Goettingen, Germany)
Objective: We sought to determine which of several conventional
rTMS and theta burst stimulation protocols is most suitable for therapeutic application in Parkinsons disease (PD).
Background: Focal single session rTMS of the primary motor cortex
has been shown to be capable of improving motor function in PD,
however these effects have been rather weak so far.
Methods: 22 PD patients received ve different rTMS-protocols on
ve consecutive days in a pseudorandomized and counterbalanced
order either in the dened-OFF condition or in addition to their usual
medication. The protocols tested in the present study included two
conventional rTMS-protocols (0.5 Hz and 10 Hz) as well as the
recently introduced theta burst stimulation (cTBS, iTBS) and a sham
condition. Cortical excitability, motor performance (pointing movement, pronation-supination, Purdue Pegboard test, walking) and mood
were assessed before and after each session.
Results: In most tests we found strong training and placebo effects,
especially in the ON-group. These effects might have masked or
prevented therapeutic effects of the rTMS protocols. Only for the
Purdue Pegboard test active stimulation yielded slightly stronger effects than sham stimulation.
Conclusions: We conclude that even theta burst stimulation is not
sufciently effective to induce therapeutically relevant improvement of
motor performance in Parkinsons disease patients after single session
rTMS.
555
Diversity in Parkinsons disease prevalence among elder South
Carolinians
K.J. Bergmann, J.K. Rodgers, V.L. Salak, V.K. Hinson (Charleston,
South Carolina, USA)
Objective: We calculated the prevalence and geographic distribution
of Parkinsons disease (PD) among South Carolinians (SC) who have
Medicare health insurance.
Background: The bare requirements of Medicare, a government
health insurance for elder Americans, eliminate potential barriers to
healthcare. This inclusiveness is valuable when investigating diversity
in disease prevalence rates.
Methods: The participants in this IRB approved study were coded as
ICD-9 332.0 (PD) from 1996 through 2001 in an identity protected
state database of all SC Medicare encounters. The outcome measure
was the crude prevalence rate for PD by county of residence for
Americans of African and European origin.
Results: The races are equally distributed across the state with the
numbers of African Americans and whites in a county strongly correlated. (Spearman rank r 0.7, p 0.0000). There were 9,336 individuals coded as ICD9 332.0 in the database: 1,289 African Americans
and 7,907 whites. The crude prevalence rate for PD in SC was 246 / 105
(range 96 to 395); African American prevalence rate 119 / 105 (range
37 to 302) and American of European origin rate 331 / 105 (range 107
to 523). African Americans have proportionately lower rates of PD than
whites and the rates by county correlate closely (Spearman rank r
0.4546, p 0.0015).
Conclusions: The prevalence of PD in elder African Americans is
one third of that found for Americans of European origin. Our Medicare data suggests that there is a different but closely linked susceptibility to PD between Americans of African and European origin. It may
be that genes originating in Europeans which confer increased risk for
PD have become admixed with the genome of African Americans,
increasing the even lower risk for PD found in their African ancestors.
Parra and colleagues suggest that a simulation of continuous gene
ow best models the complex continuous admixing of European genes
with African American genes over many generations (Am J Hum Genet
1998;63(6):1839-51). This could explain why elder African American

Movement Disorders, Vol. 22, Suppl. 16, 2007

prevalence for PD correlates so closely with that of elder whites in the

same county.
556
Parkinsons disease: Atypical improve of gait disturbances. Case
report
E.M. Dieguez, R.M. Buzo, A. Scaramelli, R. Aljanati, O. de Medina,
R. Ventura, V. Raggio, M. Pebet (Montevideo, Uruguay)
Objective: To report the clinical nding of a patient with Parkinson
disease (PD), after 12 years of treatments with severe gait disorders,
who improved his march with the aid of a ball while walking.
Background: History Case : A 39 year old man, who presents a PD
during the last 12 years. At present he suffers from different long term
therapy complications, under L-dopa and cabergoline during the last 8
and 5 years respectively.
Methods: History and clinical examination, video recording.
Results: He typically has bradykinesia (with all the components
bradykinesia, akinesia and hipokynesia), rigidity, tremor, balance and
gait disorders. His neuropsy-chological tests were normal. The balance
and gait disorders, are his most important problems since the patient is
young and he must work. Under this current situation he discovered a
very interesting form to facilitate his gait by walking and playing with
a ball, as it was mentioned above. Through this trick, he improves the
amplitude, velocity and blockage of the gait.
Conclusions: This special method appears to be very interesting and
we will discusses its pathophysiological mechanism, which is very
close to the response of PD in front of external triggers such as music,
the walking stick with a laser light, and other individually-selected
methods. A video of the patient will be shown, including different
moments of the day, ON-OFF oscillations, as well as the clear-cut
inuence of the use of the ball as a complex action interfering with the
motor control mechanisms.
557
Statin use and the risk of Parkinsons disease
A.D. Wahner, J.M. Bronstein, Y.M. Bordelon, B. Ritz (Los Angeles,
California, USA)
Objective: To investigate the association between statin (3-hxydroxy-3-methylglutaryl-conenzyme A reductase inhibitors) medication
use and Parkinsons disease (PD) in a population-based case control
study.
Background: Some biologic evidence exists that statins may be
neuroprotective through mechanisms such as nitric oxide synthase
regulation, anti-oxidant effects, and the regulation of pro-inammatory
mediators. On the other hand, recently it has been postulated that low
levels of low-density lipoprotein (LDL) might cause PD and that statin
use might be detrimental. Yet, to date, no epidemiologic study has
evaluated the inuence of statin use prior to onset on PD occurrence.
Methods: We recruited 312 incident PD cases and 342 matched
population controls from three rural California counties between January 2001 and January 2007. All diagnoses were conrmed in examinations by our study neurologist. Participants completed a medical
questionnaire providing information on statin use prior to disease onset.
We calculated odds ratios for ever versus never use of statins, stratied
by duration of use in years, age at diagnosis (60 yrs, 60 yrs), and
gender, and also excluded use 5 years immediately prior to diagnosis/
interview; all adjusted for age at diagnosis, gender, smoking, education,
race, and county of residence.
Results: PD risk was found to be 55% reduced among statin users
(OR0.45, 95% CI 0.29, 0.71). We also noted a strong dose-response
relation, with subjects having used statins for 5 years showing the
greatest reduction in risk (OR0.38 95% CI 0.18, 0.78). Results were
similar when excluding any use 5 years prior to diagnosis/interview
(ever/never use: OR0.33, 95% CI 0.14, 0.79). Effect estimates did not
differ by gender, and the protective effective was slightly stronger in
younger (60) statin users (60 OR0.25, 95% CI 0.07, 0.89; 60
OR 0.47, 95% CI 0.29, 0.77). When evaluating use of specic types

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

of statins we noted similar size (60-70%) risk reductions for all statins
except for Pravastatin (OR1.81 95%CI 0.44, 6.52).
Conclusions: We observed an inverse association between statin use
prior to disease onset and PD risk. Yet it is too early to ascribe causality
to this association because of the complex relationship between statin
use and cholesterol levels; i.e. statins may act as proxy indicators for
high cholesterol and LDL levels.
558
Biceps brachii myoelectric manifestations in medicated Parkinsons disease patients
M. Kankaanpaa, S.M. Rissanen, M.P. Tarvainen, J. Nuutinen,
I. Tarkka, P.A. Karjalainen, A. Meigal, O. Airaksinen (Kuopio,
Finland)
Objective: To characterize the biceps brachii electromyographic
(EMG) signals in medicated Parkinsons disease (PD) patients by using
visual inspection, and linear and non-linear analysis tools. We also
aimed to dene the best muscle loading level that would most prominently reveal typical PD induced myoelectric characteristics.
Background: Due to overactivity of striatum and basal ganglia, burst
and syncronized motor unit activity has been associated with PD.
Methods: 25 medicated PD patients and 22 young healthy controls
participated in the study. Bipolar surface EMG was measured over the
biceps brachii muscles bilaterally while subjects were standing and
holding isometrically their elbows in 90 degree angle with 0, 12, 1 and
2 kg loads on hands. Surface EMG was analyzed by linear [amplitude
and spectral 3-15 and 16-30 frequency bands] and non-linear [kurtosis
of histogram (KURT), approximate entropy (ApEn), correlation dimension (CD), RQA recurrence (REC) and determinism (DET)] analysis
tools. UPDRS was obtained prior to the measurements.
Results: Visual inspection of the EMG signals exhibited clear PD
related features, i.e. EMG amplitude was higher and burst activities
were evident. Linear EMG analysis indicated that EMG amplitude was
higher in PD patients than controls at 0 kg loading, whereas frequency
bands were similar between the groups. Non-linear EMG parameters,
KURT, ApEn, CD, REC and DET were able to detect clear between
group differences at most affected side. However, only CD and REC
showed group differences also at 12 and 1 kg loading conditions in most
affected side.
Conclusions: The biceps EMG amplitude, structure and, especially,
nonlinear characteristics differed between healthy controls and medicated PD patients, i.e. medication does not completely restore the
abnormal electrophysiological muscle function in PD patients. These
results may indicate that non-linear EMG analysis may have potential
in characterizing the PD induced myoelectric manifestations. The best
loading conditions to reveal these EMG manifestations were observed
when minimal muscle loading was used (0 and 12 kg). Further studies
regarding the non-linear EMG analysis in PD patients are suggested.
559
Clinical evaluation of asymptomatic G2019S LRRK2 mutation
carriers and assessment of factors inuencing LRRK2 related
Parkinsons disease expression (the ASAP LRRK2 study)
C. Gaig, E. Tolosa, M. Ezquerra, A. Rojo, M. Aguilar, J. Hernandez,
F. Miquel, M. Calopa, P. Pastor (Barcelona, Spain)
Objective: To describe a collaborative study designed to detect
genetic and environment factors that modify disease expression in
Parkinsons disease (PD) patients with G2019S LRRK2 mutation and
to characterize non-motor symptoms and nigrostriatal abnormalities in
asymptomatic mutation carriers.
Background: The G2019S LRRK2 gene mutation is found in a significant proportion of PD patients. The penetrance of the G2019S mutation
has been estimated to be from 30 to 70% and may be inuenced by other
genetic and environment factors. In PD, it is suspected that -synuclein
pathology starts in the lower brainstem and olfactory structures (causing
non-motor symptoms), with involvement of the substantia nigra occurring
later. Disease progression in G2019S LRRK2 PD may follow the same
caudorostral pattern that occurs in PD.

S171

Methods: 1200 consecutive PD patients attending four spanish

Movement Disorders Clinics, and their affected and unaffected relatives will be screened for G2019S mutation. Families with LRRK2
mutations will be studied by linkage covering all the genome. The
results will be analyzed taking into account age at onset to perform
quantitative traits analysis. Questionnaires about environment factors
and a validated Spanish Food Frequency Questionnaire will be administered. In asymptomatic mutation carriers and in age-sex matched
controls without mutations, the presence of non-motor symptoms and
the nigrostriatal pathway integrity will be assessed as follows: medical
and neurological history-examination, constipation (Bristol Stool
Scale), smell test (UPSIT), sleep studies (Pittsburgh sleep quality and
Epworth questionnaires), neuropsychological and psychiatric assessments (HADS, SCL-90, standarized neuropsychological battery) and
DAT imaging (123I-Ioupane SPECT).
Results: It is expected that non-motor symptoms will be more
frequent in asymptomatic mutation carriers than in controls. Decreased
striatal tracer uptake on DAT imaging will be present in a high
proportion of asymptomatic carriers. Genetic and environment factors
that inuence disease expression will be identied.
Conclusions: The ASAP LRRK2 Study will provide relevant information on the onset and progression of LRRK2 related PD and factors
that inuence disease expression.
560
Parkinsons disease in boxers: A link or a myth?
P. Lolekha, K. Phanthumchinda, R. Bhidayasiri (Bangkok, Thailand)
Objective: To determine the prevalence of Parkinsons disease (PD)
in Thai boxers.
Background: Pugilistic Parkinsonism (PP) refers to a parkinsonian
syndrome that result from long-term cumulative consequences of subclinical concussions to the head. Although trauma has long been linked
to PD, the concept of posttraumatic Parkinsonism is far from denite,
partly due to study design limitations, lack of radiologic documentation, and often time-lag between injury and the onset of Parkinsonism.
In Thailand, boxing remains a very popular sport for more than a
century; hence, offering a unique group of population to study the
prevalence of parkinsonism as well as to determine if posttraumtic
Parkinsonism is identical to PD.
Methods: Two standardized screening questionnaires (Mutch and
Tanner) were completed by Thai boxers and control subjects. All
demographic data were collected, particularly a history of head trauma,
and the occurrence of head trauma before the onset of Parkinsonism.
All boxers were asked about the presence of alteration of consciousness, amnesia, other neurologic complications, together with details on
the duration of their boxing career, number of professional ghts, and
unsuccessful bouts. Subjects who answered positively were invited for
clinical examination by two independent neurologists (one movement
disorders neurologist). Probable PD was diagnosed based on UKPDSBB Clinical Diagnostic Criteria.
Results: Among 979 boxers received the questionnaires, 525 boxers
(53%) participated in the study. Forty sets of questionnaires (4%) were
return because of incorrect address. Among those answered, all of them
were retired boxers in which 110 boxers (21%) answered positively.
Those who answered positively had older mean age, compared to those
with negative answers (60.2 vs. 55.9 years, SD 10.3, p 0.0001).
Currently, they are invited for clinical examination.
Conclusions: Approximately 20% of boxers who participated in this
study reported some symptoms of Parkinsonism. At present, they are
invited for clinical examination in order to determine the prevalence
and the nature of their Parkinsonism. Certain variables entailing the
severity and extent of head injury are being analyzed. We also plan to
follow the subjects longitudinally to study if their Parkinsonism is
identical to PD. Detailed statistical analysis is undertaken and we
expect that more results will be available at the time of the meeting.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S172 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

561
The mutant C-allele of the variant MTHFR c.1298A>C (E429A) is
associated with later age of onset in Parkinsons disease and multiple system atrophy and provides resistance against oxidative
stress in vitro
M. Linnebank, U. Wullner (Bonn, Germany)
Objective: To investigate the role of methionine/homocysteine metabolism in Parkinsons disease (PD).
Background: Glutathione, a dipeptide generated from cysteine and
cystine via the methionine and homocysteine metabolism provides
important defence against oxidative stress and glutathione deciency
has been found in PD midbrain. We have observed previously that
patients with Alzheimer disease (AD) carrying the CC genotype of the
gene 5,10-methylenetetrahydrofolate reductase (MTHFR) variant
c.1298AC (E429A) had a later onset of disease (log rank 4.75;
p0.029).
Methods: We performed a case control association study of 342 patients
with PD, 28 patients with multiple system atrophy (MSA) and 342 control
subjects. Five functional polymorphisms impairing the homocysteine metabolism and apolipoprotein E genotypes were investigated: methylenetetrahydrofolate reductase (MTHFR) c.677CT (A222V) and c.1298AC
(E429A), methionine synthase c.2756AG (D919G) and cystathionine
beta-synthase c.833TC (I278T) and 844ins68 (splice alteration). To test
the hypothesis that the MTHFR variants indeed modify oxidative stress,
we incubated human broblasts of different MTHFR genotypes
(c.1298AAwildtype, c.1298CC, and MTHFR decient residual activity only) with different concentrations of H2O2 and measured cell viability
and glutathione content.
Results: Onset of disease was later in patients with PD carrying
the AC or CC (sample 1; log rank 6.0; p0.014) or the CC genotype
(sample 2, log rank 3.5; p0.063 for trend) and in MSA patients
carrying the CC genotype (log rank 5.6; p0.018), suggesting that
presence of the C of MTHFR c.1298AC serves as a genetic
modier and protects against neurodegenerative disorders associated with oxidative stress. Fibroblasts with the MTHFR c.1298CC
genotype retained the highest levels of total glutathione after 30min
incubation. After 48h incubation with 500 mol/l H2O2 the MTHFR
c.1298AA wildtype broblasts showed a viability of 35%30%
meanSD (relative to non-H2O2-incubated wildtype cells) in comparison to 73%41% for MTHFR c.1298CC cells (T7.86;
p0.001).
Conclusions: Our data suggest that MTHFR and thus folate and
homocysteine metabolism are promising candidates in the search for
modiers of neurodegenerative disorders.
562
Is there a correlation between clinical presentation and body mass
index in Parkinsons disease?
R.P. Munhoz, H.A. Teive, C.B. Ribas (Curitiba, PR, Brazil)
Objective: To study the clinical prole of patients with Parkinsons disease (PD) and higher body mass index (BMI) looking
for phenomenological differentials that may be particular to this
group.
Background: During the past decade several studies showed objectively that patients with PD have lower BMI in comparison with
controls. However, the etiologies and implications of this nding
remain inconclusive and controversial.
Methods: Thirty-two patients (group I) with PD and BMI above a
cutoff of 25.6 Kg/m (mean BMI for our whole PD population) were
compared with 28 age-matched controls with PD and BMI below the
proposed cutoff (group II). Subjects were studied using a standardized
protocol, including anthropometrical assessment, clinical data and disease staging.
Results: Mean (SD) BMI for group I was 31,8 (6) Kg/m, mean age
(SD) 66,2 (11,2) years, age of onset 58,7 (12.1). Mean (SD) disease
duration 7,5 (4,5) years and H&Y scale score 2,6. Three subjects
(9,3%) had rigid akinetic (RA) parkinsonism and two (6,2%) had
dementia. Group II had mean BMI 22,4, mean (SD) age 65,3 (10,6)

Movement Disorders, Vol. 22, Suppl. 16, 2007

years and age of onset 55,9 (12,9). Mean disease duration 9 (6) and
mean H&Y score 2,6. Eleven (39,2%) subjects had RA parkinsonism and seven (25%) patients had dementia. Data comparison shows
statistical signicant differences for the presence of dementia and a
RA form of parkinsonism, both more prevelent in the group with
lower BMI. All other parameters tested were not signicantly different.
Conclusions: Our study demonstrated that onset and staging of motor
symptoms in PD are similar among subgroups differing in regards to
BMI. On the other hand, dementia and the RA phenotype of PD were
signicantly more common among subjects with lower BMI. Based on
these ndings, we cannot exclude the possibility that preserved cognition and a PD phenotype that includes resting tremor may interact
positively with body weight maintenance.
563
Enhanced lymphocyte apoptosis in treated and untreated Parkinson patients
J. Bas, M. Calopa, M. Mestre (Barcelona, Spain)
Objective: To assess the inuence of antiparkinsonian treatment and
clinical parameters on lymphocyte apoptosis.
Background: Apoptosis is a putative mechanism in neurodegenerative diseases. In former studies we have shown that Parkinson
patients present an increase in lymphocyte activation and apoptosis
that causes a decrease in circulating lymphocytes, mainly
CD4CD45RA.
Methods: We included 85 Parkinson patients (55 treated and 30
untreated) and 30 healthy controls of similar age. Selected lymphocyte surface markers were studied. Both spontaneous and mitogeninduced apoptosis were evaluated in several T lymphocyte subsets
by incubation with annexin-V / propidium iodide. Samples were
analyzed by ow cytometry.
Results: In both, treated and non treated patients, it was observed
a signicant increase in spontaneous and mitogen-induced apoptosis
of nave (CD4CD45RA) and memory T helper cells (CD4CD45RO)
and in activated helper cells (CD4CD25), compared with healthy
subjects. No differences were found between treated and untreated
patients. Fas (CD95) expression on circulating T helper cells (CD4),
activated helper cells (CD4CD25), nave and memory T helper cells
(CD4CD45RA, CD4CD45RO), T cytotoxic/suppressor cells (CD8,
CD8CD45RA) was not correlated with age of patients, years of
disease, age at onset and severity of the disease as assessed by
UPDRS (OFF) and HY scores. The degree of in vitro spontaneous
and mitogen-induced apoptosis of all studied subsets did not correlate with any clinical parameter.
Conclusions: Our results indicate that CD4 T lymphocyte subsets
from Parkinson patients do present an enhanced susceptibility to apoptosis irrespective of clinical status and treatment. This suggest the
existence of a primary, systemic alteration in apoptotic mechanisms in
Parkinsons disease.
564
Diagnostic accuracy of SPECT in parkinsonian syndromes: A
meta-analysis
A.M.M. Vlaar, M.J.P.G. Kroonenburgh Van, A.G.H. Kessels,
S.C. Tromp, W.E.J. Weber (Maastricht, Netherlands)
Objective: Performing a meta-analysis of all the existing literature on
the diagnostic accuracy of both pre- and post-synaptic Single Photon
Emission Computer Tomography (SPECT) in the differential diagnosis
of idiopathic Parkinsons disease (IPD).
Background: IPD is the second most common neurodegenerative
disorder. One of the most employed techniques to diagnose IPD is
SPECT imaging to visualize the integrity of the dopaminergic pathways in the brain. However, discussion remains on the value of SPECT
in the differential diagnosis of IPD.
Methods: Relevant studies were searched in Medline, EMBASE
and Cochrane databases with back-searching of their reference lists.
We limited our analysis to studies with a clinically relevant meth-

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

odology: i.e. when they assessed the ability of the SPECT to provide
an early diagnosis of IPD in patients with little signs and symptoms,
its ability to discriminate IPD from the other parkinsonian syndromes, both in follow-up and in cross-sectional studies.
Results: The search gave 1503 hits, of which we deemed 33
suitable for our analysis. From these we recalculated the diagnostic
power of SPECT for the following clinical problems: 1. diagnosis of
IPD in an early phase vs. normalcy; 2. diagnostic differentiation
between IPD and essential tremor; 3. distinguishing between IPD
and vascular parkinsonism; 4. delineation of IPD from atypical
parkinsonian syndromes (APS) like Multiple System Atrophy and
Progressive Supranuclear Palsy.
Conclusions: 1) SPECT with presynaptic radiotracers is relatively
accurate to differentiate patients with IPD in an early phase from
normalcy and patients with PD from those with essential tremor or
vascular parkinsonism. 2) The accuracy of SPECT with both presynaptic and postsynaptic tracers to differentiate between IPD and
APS is relatively low.
mean odds ratio (95% condence interval)

early IPD vs. normalcy

IPD vs. essential tremor
IPD vs. vascular parkinsonism
IPD vs. APS

presynaptic tracer

postsynaptic tracer

60 (13-277)
210 (79-562)
105 (32-348)
2 (1-4)

2 (0.5-5)
8 (2-30)
19 (9-36)

565
Treadmill training improves functional ability and cardiopulmonary capacity in stable Parkinsons disease
E. Pelosin, E. Faelli, F. Lofrano, L. Marinelli, M. Bove, P. Ruggeri,
G. Abbruzzese (Genova, Italy)
Objective: To test whether the treadmill training can improve functional outcome of patients with stable Parkinsons disease (PD).
Background: Gait impairments are common in patients with PD and
are associated with an increased risk of falls and loss of independence.
Walking ability may be conditioned by the occurrence of fatigue or
inadequate cardiopulmonary responses. Treadmill training, with or
without body weight support, has been successfully used in the rehabilitation of neurological patients, but only few studies reported the
effect of treadmill training in PD patients.
Methods: 7 patients with idiopathic PD (age: 61-79 years; H&Y
stage: 1-3) participated in this study. They were under stable drug
treatment (without uctuations or dyskinesias) and did not present
cardiovascular diseases. Patients with cognitive impairment and orthopaedic problems were excluded. All subjects performed 10 gait trainings (3 times per week) of 30 minutes walking on treadmill without
weight support. Treadmill speed was increased by 0.5 Km/h every 3
days of treatment. Patients were evaluated by means of: UPDRS,
PDQ-39, timed Up and Go test, 10-meters and 6-minutes walking tests
ant the Fatigue Severity Scale; cardiopulmonary functional capacity
was measured by a maximal oxygen uptake exercise test (VO2 max).
Patients were tested at the beginning of treadmill training (T0), at the
end (T1) and after 30 days (T2).
Results: All walking parameters (including speed and step frequency) improved signicantly (p0.05) after treatment (T1); the
improvement persisted after one month (T2). Functional cardiopulmonary capacity was signicantly increased.
Conclusions: Treadmill training is effective in improving walking
motor performance and quality of life in PD patients. Cardiopulmonary
performance beneted from treadmill training and PD patients showed
a similar response to normal controls with regard to mobility energy
costs. These results suggest that treadmill without weight support may
be a useful rehabilitative approach in Parkinsons disease.

S173

566
Clinical predictors of on-road driving performance in patients with
Parkinsons disease
D.P. McCarthy, C. Garvin, D.N. Lanford, M.S. Okun,
R.L. Rodriguez, J. Romrell, W.C. Mann, S. Bridges, H.H. Fernandez
(Gainesville, Florida, USA)
Objective: To determine what clinical tests administered by neurologists and occupational therapy driving rehabilitation specialists (DRS)
are predictive of on-road driving performance in patients with Parkinsons disease (PD).
Background: Driving is a complex task that requires integration of
visual, motor, and cognitive abilities which are often impaired by PD
and its treatment. Previous research is inconclusive as to what clinical
tests administered by neurologists are informative when deciding if a
patient should stop driving. This project examines the relationship of
tests commonly administered by physicians and driving specialists to
predict safe driving ability.
Methods: Nineteen random subjects with idiopathic PD, recruited
from the University of Floridas Movement Disorder Center (MDC),
were referred to a DRS to determine safe driving ability, regardless of
the clinicians perception of their driving competence. Unied Parkinson Disease Rating Scale (UPDRS) motor scores in the on and off
state and visual, cognitive and other motor evaluations were performed,
followed by a comprehensive driving evaluationincluding an on-road
driving test on a standardized route of progressive difculty.
Results: Nineteen PD patients (78% males) with a mean age of 75
years (SD 6.07), UPDRS mean motor score of 25.9 (6.9) on medication
and 34.2 (9.9) off medication were analyzed. The comparatively high
failure rate of this group (44% vs. 20% for those without PD) is
consistent with the literature. Of the tests administered by neurologists,
only the UPDRS off meds score was signicantly correlated (r
-.640, p .008) with driving performance. Of the tests administered by
the DRS, signicant moderate correlations were found for visual contrast sensitivity (r 723, p .004), Trailmaking Test, Part B (r
-.643, p .004), and the Clock Drawing Test (r .544, p .029) with
the driving test outcome.
Conclusions: Our results suggest the possibility of incorporating
additional psychometric tests to a bedside battery and also they emphasize the importance of collaboration between neurologists and
driver rehabilitation specialists in assessing medical tness to drive.
567
Is there a morphological substrate of parkinsonian rest tremor a
voxel-based morphometry study
D. Benninger, S. Thees, C.L. Bassetti, S.S. Kollias, D. Waldvogel
(Zurich, Switzerland)
Objective: To investigate morphological substrates of rest tremor in
patients with Parkinsons disease (PD).
Background: Rest tremor is a hallmark of PD and its pathogenesis
remains incompletely understood. Nigrostriatal dopaminergic deciency correlates with bradykinesia, but not with rest tremor. A central
oscillator originating in an interaction of basal ganglia- and cerebellothalamo-cortical ciruits is postulated to cause tremor. Tremor may be
disabling and refractory to dopaminergic therapy. Better understanding
of the pathogenesis helps to develop new treatment strategies.
Methods: We investigated 24 men (median, range [years], age 62,
55-70, at onset 55, 42- 67, disease duration 7, 3-20) with mild to
moderate PD (Hoehn Yahr [HY] 2 and 3). Exclusion criteria were
advanced PD (HY 4 and 5), dementia and other signicant brain
pathology. Patients with (n14) and without rest tremor (akinetic-rigid
patients; n10) were compared. Demographical and clinical prole,
including detailed neuropsychological and psychiatric evaluation,
were similar, except for higher prevalence of motor uctuations (90%
vs 14%, p0.01) and impaired balance (100% vs 50%, p0.01) in
akinetic-rigid patients. Voxel-based morphometry of a high-resolution
3 Tesla T1-weighted MRI image, pre-processed according to an optimized protocol using SPM2, was performed. The resulting normalized

Movement Disorders, Vol. 22, Suppl. 16, 2007

S174 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

grey matter volume distributions of the two groups were tested for local
differences by a voxel-wise ANCOVA.
Results: Grey matter density is decreased in the right left posterior
part of the quadrangular lobe and declive of the cerebellum in PD with
tremor compared to akinetic-rigid patients (PFDR 0.05).
Conclusions: These results demonstrate for the rst time in vivo
morphological changes in the cerebellum in rest tremor in PD and
underscore the involvement of the cerebellum and cerebello-thalamocortical circuit in the pathogenesis of parkinsonian rest tremor.
568
Non motor symptoms in untreated patients with Parkinsons disease in Tanzania
C.L. Dotchin, R.W. Walker (North Shields, Tyne and Wear, United
Kingdom)
Objective: To determine the non motor symptom burden in patients
with Parkinsons disease (PD) in Tanzania, the majority of whom were
previously untreated.
Background: Non motor symptoms have come to the fore in the management of (PD). They are a source of much distress for patients and
contribute to a reduced quality of life. They have previously been poorly
identied by both physicians and patients. The majority of identied
patients in a prevalence study in rural Tanzania[ii] had not received
treatment for PD. We report on their non motor symptom burden.
Chaudhuri KR, Martinez-Martin P, Schapira AHV, Stocchi F, et al International multi-centre pilot study of the rst comprehensive self-completed non motor symptoms questionnaire for Parkinsons disease: The
NMS Quest study Movement Disorders 2006; 21(7): 916-23 [ii] Hood
CL, Walker RW The prevalence of Parkinsons disease in Hai, Tanzania
Movement Disorders 2006 Vol 21(Suppl 15) S461.
Methods: 33 patients were seen by the research doctor in their own
home in the company of a caregiver and PD nurse specialist. Local
interpreters assisted with translation. The Non Motor Symptoms (NMS)
rating scale was used to identify and quantify non motor symptoms. The
Hospital Anxiety and Depression (HAD) scale was used to measure mood.
Hoehn and Yahr stage of each patient was recorded.
Results: Three of the 33 patients were unable to fully complete the
questionnaires because of cognitive impairment. The remaining 30 (22
were male) had a mean age of 73.4 years (range 37- 94 years), mean
disease duration 5 years (0.25-19 years) and Hoehn and Yahr range
from 2-5. Mean total NMS score was 59 (range 11-209) (domains 5 and
10 were omitted from the questionnaire) out of a possible 312. HAD
results showed patients were not depressed (14), possibly depressed
(11) or probably depressed (5). Anxiety scores showed that 21 were not
anxious, 4 possibly and 5 probably anxious.
Conclusions: These patients, who were largely on no treatment for
PD, did suffer non-motor symptoms, but their scores were less than
those included in the pilot NMS testing (mean 247). A wide range of
patients with a variety of disease durations and stages were included.
As far as we are aware these scales have not been used in Kiswahili
before and cultural and translational problems could account in part for
lower scores.
569
Use of clozapine in Brazilian patients with Parkinsons disease
L. Gomide, A. Kummer, S.T. Camargos, M.C. Cunningham,
D.P. Maia, F. Cardoso, A.L. Teixeira (Belo Horizonte, Brazil)
Objective: To investigate the indications, tolerability and safety of
the use of clozapine in Brazilian patients with advanced Parkinsons
disease (PD).
Background: Levodopa is the most effective drug to control motor
symptoms in PD. However its chronic use can lead to complications
such as dyskinesia, clinical uctuation and psychiatric symptoms.
Atypical neuroleptics, especially clozapine, have been employed in the
management of some of these complications.
Methods: A systematic chart review of PD patients followed at our
Movement Disorders Clinic was performed. Clinical and demographic
data were carefully collected.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Results: Clozapine was prescribed for 25 patients (M/F, 11/14; mean

[range] age, 60 [39-87]) with advanced PD. The mean [range] score of
the UPDRS for these patients was 65 [30 121] while the mean time
of diagnosis was 9.7 years. Clozapine was indicated for severe dyskinesias in 17 patients and for psychosis in 8. Ten patients did not even
start the use of medication. Some of these refused to perform weekly
blood tests. From the remaining 15 patients, clozapine was interrupted
in 5 cases due to adverse side effects (agranulocytosis and excessive
sedation) and in 3 cases for deterioration of the motor symptoms. Seven
patients used clozapine for a period longer than 2 years with a significant clinical improvement in 5.
Conclusions: Clozapine may play a role in the management of motor
and psychiatric complications in advanced PD. However clozapine is
frequently associated with low tolerability and/or serious adverse effects in PD. Further studies are needed to dene which patients can
have few side effects with maximum clinical benet.
570
Therapeutic effect of neuropeptide PACAP27 on MPTP-induced
parkinsonism in mice
G. Wang, Y.Y. Tan, X.K. Sun, R.J. Ren, H.Y. Zhou, S.D. Chen
(Shanghai, China)
Objective: To investigate the effects of different doses of pituitary
adenylate cyclase-activating polypeptide (PACAP) on the functional
and morphological outcome in a mice model of PD rendered by MPTP.
Background: Pituitary adenylate cyclase activating polypeptide(PACAP) play a important role during the development of the nervous
system and in the regeneration following nervous injuries. Our previous
studies and others in vivo and in vitro showed the neuroprotection of
PACAP against neuronal insults induced by rotenone, 6-OHDA, glutamate, beta-amyloid and human prion protein.
Methods: Male mice were treated with PACAP 0.02,0.2 or 2g by
i.v bolus for 7 days after MPTP administration, and were compared
with saline-treated mice. Immunohistochemistry and western blot were
used to detect the alterations of PD biomarker including tyrosine
hydroxylase (TH), dopamine transporter (DAT) and vesicular monoamine transporter2(VAMT2). In addition, monoamine neurotransmitters in the striatum of mice were measured by the high performance
liquid chromatography (HPLC).
Results: TH immunohistochemistry indicated that the number of
dopaminergic neurons in the substantia nigra was increased in all
PACAP-treated mice compared with saline-treated mice. Western blot
demonstrated the similar TH protein expression in striatum. Dopamineassociated transporters including DAT and VMAT2, were markedly
decreased in NS-treated mice compared with the normal mice, but the
different reactions were found in PACAP-treated mice. The concentrations of striatal dopamine and its metabolite DOPAC compared to the
normal mice were decreased signicantly in other groups. Only
PACAP-treated mice (0.02g/d) minimized the reduction of DOPAC
compared with the NS-treated mice.
Conclusions: These results showed that PACAP is able to rescue the
dopaminergic neurons in different doses and reverses the loss of
DOPAC at dose of 0.02g/d, and it may be a promising therapeutic
agent in PD.
571
Sleep disturbances in patients with Parkinsons disease
T. Smiljkovic, S. Kostic, V. Dedic, J. Potic, V. Nikolic (Belgrade, Serbia)
Objective: To estimate the frequency of sleep disturbances and its
correlation with disease, therapy and demographic factors in patients
with idiopathic Parkinsons disease.
Background: Sleep problems, common in Parkinsons disease, are
consequence of neurodegenerative process as well as neurochemical
changes on one side, and of the drug intake, on the other side.
Methods: In the study there were enrolled 65 consecutive patients who
fullled criteria for PD. Original questionnaire was performed to obtain
demographic, disease and treatment data. Patients were tested with standardized scales UPDRS, H-Y scale, Schwab and England scale. MMSE

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

was used for evaluation of cognitive status and Hamilton depression rating
scale for depression. For assessment of the sleep problems. the Parkinsons
disease sleep scale (PDSS) was used, as well.
Results: There were 37 male and 28 female patients, average age 66,
10 years; disease duration 6,2 years; in H-Y stage between I and IV
(average 2, 1); MMSE 27,1; UPDRS 53,45 and total PDSS 9, 94.
Signicant correlations (p0.05) were found between PDSS and total
UPDRS, each part of UPDRS and H-Y stage. There was no difference
in PDSS scores regarding sex. Analyzing each item in PDSS scale, the
lowest score was obtained for item 8 (nocturia), while highest score
was obtained for items 9 and 7. We did not nd difference in total
PDSS score between patients on d-agonist and patients who did not
take d-agonists. Regarding amantadin intake there were differences
between gropes for PDSS4,PDSS5 and PDSS10. We registered significant differences of the PDSS score between tremor versus mixed form
of PD.
Conclusions: Patients in advanced stages of disease and with higher
UPDRS scores have more prominent sleep problems. Some of sleep
problems seems to be inuenced by drug intake. Sleep problems are not
rare in Parkinsons disease and have important impact to life quality in
these patients.
572
Rotigotine transdermal patch as adjunct to levodopa in the treatment of advanced-stage Parkinsons patients
P.A. LeWitt, K.E. Lyons, R. Pahwa (Southeld, Michigan, USA)
Objective: To investigate efcacy and safety of rotigotine (Neupro), a D3/D2/D1 dopamine receptor agonist formulated as a patch,
in advanced Parkinsons disease (PD) adjunctive to levedopa.
Background: Rotigotine transdermal patch (Neupro) is a broadspectrum D3/D2/D1 dopamine receptor agonist, which has been approved in Europe for the treatment of idiopathic Parkinsons disease
(PD). The patch is applied once-daily and achieves stable 24-hour
plasma levels of rotigotine.
Methods: PD subjects inadequately controlled with levodopa were
randomized to placebo or two target doses of rotigotine (either 8 mg/24
h or 12 mg/24 h). This double-blind, multicenter, multinational trial
consisted of a pre-treatment (4 weeks), titration (5 weeks), maintenance
(24 weeks), de-escalation, and safety follow-up. The primary efcacy
measurement was reduced off time in all-day hourly patient diary
home assessments.
Results: Of 351 randomized subjects (111 to rotigotine 12 mg/24 h
and 120 to rotigotine 8 mg/24 h; 120 to placebo), 260 (74%) completed
the maintenance phase. As compared to their baseline diary evaluations, rotigotine-treated subjects receiving 12 mg/24 h had an adjusted
mean decrease of -2.1 hours in off time; and the group receiving 8
mg/24 h had off time reduction by -2.7 hours. Neither group experienced an increase in the time on with troublesome dyskinesias.
Placebo was associated with 0.9 hour decreased off time. The differences between both rotigotine dose groups and placebo were significant (p-values of 0.003 and 0.001, respectively; ANCOVA LOCF).
The most common AEs with rotigotine were application site reactions,
somnolence, nausea, and dizziness.
Conclusions: Rotigotine transdermal system as adjunctive therapy to
levodopa showed clinically relevant and statistically signicant reductions in off time in doses up to 12 mg/24 h. There was no increase in
on time with troublesome dyskinesias. It was well-tolerated for the
majority of subjects in this trial.
573
Quantitative motor tests for early detection of motor dysfunction in
Parkinsons disease
C.A. Haaxma, B.R. Bloem, G.F. Borm, M.W.I.M. Horstink
(Nijmegen, Netherlands)
Objective: To investigate if a battery of quantitative motor tests
(QMTs) can reliably detect early motor dysfunction in patients with
Parkinsons disease (PD) compared to healthy individuals.

S175

Background: Early diagnosis of PD is important as putative neuroprotective therapies should ideally be initiated in the earliest stage of
the disease. In a previous study, we described a QMT battery which
showed good validity, reliability and sensitivity to monitor disease
severity and treatment effects in PD. This QMT battery could possibly
be applied for early detection purposes as well.
Methods: One hundred nine PD patients (disease duration 2 years;
prior to dopaminergic treatment initiation) and 102 healthy, agematched subjects were assessed with nine QMTs based on (a) walking,
(b) writing, (c) single- and double-handed pegboard performance, (d)
nger tapping on one key and alternating on two keys, (e) rapid
alternating forearm movements, and (f) squeezing force. We evaluated
the ability of individual and combined QMTs to discriminate between
patients and controls. Second, we investigated if the QMTs could detect
a signicant defect in motor function of the clinically unaffected limb
in early PD patients with strictly unilateral symptoms, compared to
controls. In addition, the preclinical phase was estimated.
Results: The QMT sumscore reached an area under the curve (AUC)
of 0.82 for discriminating early PD patients from healthy subjects. Of
all individual QMT items, the pegboard dexterity test had by far the
best ROC curve (AUC 0.95). It showed a 92% sensitivity with an 85%
specicity at 9.6 seconds cut-off. Discriminating between motor scores
of the unaffected limb and healthy scores was more difcult but still
acceptable (AUC 0.78). The preclinical phase was estimated at 3.3 to
7.4 years, based on the QMT sumscore. The dexterity test indicated a
duration of 3.4 years.
Conclusions: The QMT battery described here can discriminate well
between healthy and early parkinsonian motor scores, applying either
the QMT sumscore or just the single dexterity test. Adding its stable
metric properties and feasibility as shown previously, this QMT battery
could be a useful diagnostic tool in clinical practice or a screening tool
for early detection of PD signs in a dened population at risk.
574
Detection of increased echogenicity in the substantia nigra in a
large family with homo- and heterozygous PINK1 mutations
J.M. Hagenah, G. Seidel, N. Bruggemann, A. Djarmati, K. Lohmann,
A. Sprenger, C. Klein (Luebeck, Germany)
Objective: To investigate substantia nigra echogenicity in a family
with PINK1 parkinsonism with transcranial sonography (TCS).
Background: The substantia nigra (SN) shows a distinct hyperechogenic pattern on TCS in about 90% of patients with Parkinsons disease
(PD). An increased area of echogenicity in the substantia nigra (aSN)
has also been found in symptomatic homozygous and asymptomatic
heterozygous carriers of Parkin mutations, suggesting the increased
aSN as a potential preclinical marker.
Methods: We investigated 20 members of a PINK1 family including
four homozygous and eleven heterozygous mutation carriers and ve
individuals without mutation. All four homozygous mutation carriers
had clinically denite PD, six of the heterozygous mutation carriers had
signs of possible or probable PD, and one mutation-negative family
member had a secondary hypokinetic syndrome. The TCS examination
was performed using a phased-array ultrasound system with a 2.5-MHz
sector transducer. A standardized axial mesencephalic plane was used
to localize the maximum extent of the hyperechogenic signal from the
ipsilateral SN. Data analysis was carried out by two independent
investigators in a blinded fashion.
Results: The mean area of the larger of the two aSNs of each
individual (aSNmax) was 0.480.12 cm2 in the homozygous,
0.360.12 cm2 in the heterozygous mutation carriers, and 0.280.07
cm2 in the family members without mutation. Compared to the mean
aSNmax of a healthy control group of 18 subjects (mean aSNmax:
0.070.05cm2), a signicant difference was found for all subgroups,
regardless of their clinical or mutational status (p0.001). A signicant
difference of aSNmax was also found between the homozygous mutation carriers and family members without a mutation (p0.01) but not
between the other subgroups of the family.
Conclusions: In this rst study on TCS in PINK1 parkinsonism, an
increased aSN was found in homozygous and heterozygous carriers of

Movement Disorders, Vol. 22, Suppl. 16, 2007

S176 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

a PINK1 mutation, comparable to ndings in carriers of Parkin mutations and nongenetic PD. The increased aSN in family members without a mutation suggests an additional contributing factor independent
of the PINK1 mutation.

found (r 0.503, p 0.014). No statistically signicant correlation

was found between the tau-protein levels and the PD duration.
Conclusions: The CSF levels of tau-protein may serve as a potential
marker of level of neurodegeneration in PD.

575

577

Metric attributes of a quantitative motor test battery for practical

and scientic use in Parkinsons disease
C.A. Haaxma, B.R. Bloem, G.F. Borm, M.W.I.M. Horstink
(Nijmegen, Netherlands)

Levodopa therapy is associated with reduced body weight in advanced Parkinsons disease patients
C.G. Bachmann, A. Zapf, E. Brunner, C. Trenkwalder (Goettingen,
Germany)

Objective: To investigate the metric attributes of nine quantitative

motor tests (QMTs) in Parkinsons disease (PD).
Background: The most widely used scale currently available for the
clinical evaluation of motor dysfunction in PD- the Unied Parkinsons
Disease Rating Scale (UPDRS)-III - is time-consuming, subjective and
has suboptimal sensitivity. A brief, quantied motor test (QMT) battery
could possibly overcome these drawbacks.
Methods: 204 PD patients (disease duration 3.1 years; prior to
dopaminergic treatment initiation) were assessed with the UPDRS-III
and also received nine QMTs based on (a) walking 11 m, (b) writing a
standard sentence, (c) single- and double-handed pegboard performance, (d) nger tapping on one key and alternating on two keys, (e)
rapid alternating forearm movements, and (f) squeezing force. We
investigated factor structure, internal consistency, test-retest reliability
and sensitivity to change.
Results: Completing the entire QMT battery took approximately 5
minutes. Two factors explained 61% of the QMT variance, the rst
represented mainly upper extremity function, the second mainly axial
function. Cronbachs alpha was 0.82 for the QMT compared to 0.79 for
the UPDRS-III. Test-retest reliability of the QMT sumscore was 0.93 to
0.89 for measurements separated by 3 up to 24 months, whereas
UPDRS-III correlations were 0.88 to 0.84. Based on the QMTs sensitivity at group level, a trial using change from baseline as endpoint
would require only 75% of the patients needed with the UPDRS-III. At
patient level, QMT and UPDRS-III were equally responsive.
Conclusions: The QMT battery described here is valid, reliable and
feasible. Compared to the UPDRS-III, it is more objective and more
sensitive to change. Therefore, it could be a useful tool for both
practical and scientic purposes.

Objective: The aim of this study is to investigate whether PD patients

in the advanced stages display body weight alterations and to observe
any correlations between medication and other putative determinants of
body weight.
Background: Several studies have suggested that Parkinsons disease
(PD) patients in the advanced stage with or without dyskinesias show
a too low body weight when compared with age-matched, healthy
subjects. However, the etiology of the observed body weight changes in
PD is still unresolved.
Methods: Charts of 210 PD patients, with uctuations and dyskinesias, admitted within 6 months to a German movement disorders clinic,
were investigated for body mass index (BMI), age at onset, disease
duration, UPDRS motor score, eating coordination and medication
such as levodopa, dopamine agonists, neuroleptics, amantadine, budipine, antidepressants, anticholinergics. Follow-up BMI was carried out
again two years later. Control population data were taken from a survey
of the German Federal Ofce for Statistics.
Results: 4.2 % of PD patients showed slight underweight (BMI
18.5), 46.4 % were normal (BMI 18.5-25); 33.7 % overweight (BMI
25-30); 15.7 % obese (BMI 30). Dyskinesias, daily levodopa dosage
per kg body weight and total levodopa dopaminergic dosage (levodopa/
entacapone/dopaminagonist) were negatively correlated with BMI.
Overall, patients BMI had not signicantly changed within 2 years of
follow-up.
Conclusions: Patients with advanced idiopathic PD in a German
movement disorders clinic show a reduced BMI compared with the
general German population of similar age. Our ndings suggest that
levodopa may be responsible for weight loss in PD and that patients
with a lower body weight receive a higher cumulative levodopa dosage.

576
The evaluation of tau-protein in the CSF in patients suffering from
Parkinsons disease
H. Vranova, P. Kanovsky, M. Nevrly, J. Mares, P. Hlustik (Olomouc,
Czech Republic)
Objective: The aim of our study was to assess the tau-protein CSF
levels in patients suffering from PD and to compare the tau-protein
levels with both the PD duration and severity of motor manifestation of
PD.
Background: Sporadic PD is a progressive degenerative illness of the
nervous system manifesting itself clinically after the underlying pathology already has reached an advanced stage. According to the Braaks
staging of brain pathology the severity of involvement of the brain is
expressed in the severity of motor manifestation of the disease. Tauprotein is a component of neuronal cytoskeleton. During neurodegeneration the cells decay and tau-protein is released into the CSF. The
severity of neurodegeneration should correlate with tau-protein CSF
levels.
Methods: The tau-protein CSF levels were assessed in 25 patients
suffering from PD without dementia (18 males, 7 females, aged 37-73,
mean 58.8 /- 11.52 years). The tau-protein CSF levels were correlated
with PD duration and the severity of motor manifestation of PD.
Hoehn-Yahr scale was used for the assessment of the severity of motor
manifestation. Correlation analysis and t- test were used when assessing statistical signicance of the results.
Results: A statistically signicant positive correlation between the
tau-protein CSF levels and the severity of motor manifestation was

Movement Disorders, Vol. 22, Suppl. 16, 2007

578
The relationship between antiparkinsonian therapy and sleep disturbances in patients with moderate stage of Parkinsons disease
M. Boczarska-Jedynak, B. Jasinska-Myga, G.A. Klodowska-Duda,
M. Arkuszewski, G. Opala (Katowice, Poland)
Objective: The aim of our study was to evaluate the relationship
between antiparkinsonian therapy and sleep disturbances in patients
with moderate stage of idiopathic Parkinsons disease (PD).
Background: Sleep disturbances are one of the most frequent nonmotor symptoms of PD affecting 60% - 98% of patients. One of the
main causes of sleep disorders in PD is dopaminergic therapy.
Methods: Two groups of PD patients of Movement Disorders Outpatient Clinics in Katowice, Poland, with moderate stage of PD
(Hoehn&Yahr staging: 2-3, 15-65 points in motor section of UPDRS),
without severe depression and dementia were included into the study.
Sleep disturbances were estimated basing on a positive (Group I,
n51) or negative (Group II, n51) answer to the question: Do you
have sleep problems? and basing on the Parkinsons Disease Sleep
Scale (PDSS). Types and dosages of antiparkinsonian medications in
both groups were compared. The results were statistically analyzed. P
values of 0.05 were considered statistically signicant.
Results: Patients in Group I achieved signicantly lower mean result
in PDSS than patients in Group II (73.1 vs 114.7 points, p0.0001).
Both groups didnt differ with relation to the type of antiparkinsonian
therapy. Daily L-dopa dose in Group I was signicantly higher than in
Group II (811.5 vs 512.2 mg/d, p0.001). There were no signicant
differences in both groups taking into account the total daily dose of
DA, selegiline, amantadine and Biperidene.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Conclusions: In our study the higher dose of L-dopa had a signicant
negative correlation with the sleep quality.
579
The pesticide of rotenone selectively enhances voltage-gated potassium channels and endogenous PACAP mRNA expression in neuronal differentiated PC12 cells
G. Wang, H.Y. Zhou, S.D. Chen (Shanghai, China)
Objective: Neuronal differentiated PC12 cells were used to investigate the role of voltage-gated potassium (Kv) channels in rotenoneinduced neurotoxicity. Additionally, the possible adaptive roles of
endogenous pituitary adenylate cyclase activating polypeptide
(PACAP) and its receptor, PAC1, were probed.
Background: As an important environmental pollutant with features
of inhibition of mitochondrial complex I, rotenone, that has been
implicated in the pathogenesis of Parkinsons disease (PD). Our previous studies have demonstrated that rotenone induced apoptosis and
exogenous PACAP27 has a neuroprotective role against rotenoneinduced neurotoxicity in neuronal differentiated PC12 cells.
Methods: Quantitative RT-PCR was utilized to elucidate mRNA
expression of Kv channels, PACAP and PAC1 receptor following
rotenone treatment.
Results: A total of six Kv channels family members were detected,
including Kv1.2, Kv1.3, Kv2.1, Kv3.1b, Kv3.2 and Kv4.2. Gene expression analysis revealed there were no signicant differences among
groups(p0.05)except Kv2.1 . Compared to untreated controls, rotenone treatment had no effects on Kv gene expression except Kv2.1,
which showed a signicant 3.70 fold increase following rotenone
treatment (p0.05). No signicant differences were noted between
rotenone-treated and PACAP cells treated with rotenone cells. There
was a signicant difference in PACAP gene expression proles, compared to controls, with increases noted in both the rotenone-treated
group and the PACAP-treated group with rotenone in the presence of
PACAP6-27(p0.01). No signicant differences were seen between
the rotenone-treated group and PACAP-treated group with rotenone(p0.05). Further analysis revealed that rotenone treatment resulted in a 1.93-fold increase in PAC1 gene expression (p0.05),
although the increase was not signicant in comparison to rotenonetreated cells and the PACAP-treated cells with rotenone.
Conclusions: Our results indicated that Kv2.1 expression was more
sensitive to both apoptosis and chemical hypoxia compared to other Kv
channels and at least at mRNA level, Kv2.1 was not an effective target
for the neuroprotective effects of exogenous PACAP27. Endogenous
PACAP was shown to contribute to self-defense and compensatory
mechanisms by regulating PACAP expression itself.
580
Genetic polymorphism of thymidylate synthase enhancer region
(TSER) in patients with idiopathic Parkinsons disease
W.-C. Kim, K.-K. Kim, H.-S. Kim, Y.-H. Koo, O.-J. Kim, M.-S. Lee
(Seong-Nam City, Gyeong-Gi Do, Korea)
Objective: To indentify the effect of thymidylate synthase (TS) on
idiopathic Parkinsons disease (IPD).
Background: Recently, several studies have suggested that hyperhomocysteinemia is associated with pathogenesis of neurodegenerative
disorders such as Alzheimers disease and IPD. The level of plasma
homocysteine (pHcy) is determined by vitamine supplementation and
activities of related enzymes such as 5,10-methylenetetrahydrofolate
reductase (MTHFR) and TS. Because TS competes with MTHFR for
their common cofactor, 5,10-methylenetetrahydrofolate, activity of TS
might elevate pHcy level. Such activity of TS is modulated by 28bp
tandem repeat polymorphism in thymidylate synthase enhancer region
(TSER). Alleles of TSER polymorphism are 2R, 3R and more copies of
28bp tandem repeat. The 3R allele tends to increase translational
efciency in vitro, then, the 3R3R genotype of TSER polymorphism
decreases serum folate level and elevates pHcy level as 677TT genotype of MTHFR dose.

S177

Methods: Since levodopa is known to increase pHcy level, we

enrolled de novo IPD patients. 41 patients with IPD and 161 healthy
individuals were included and their fasting pHcy and serum folate
levels were measured. The genotypes of TSER and MTHFR were
identied with the PCR-RFLP methods.
Results: Mean pHcy level was signicantly higher in IPD patients
(12.062.95mol/l) than controls (9.042.66mol/l)(p0.05). Frequencies of MTHFR C677T genotype and TSER 28bp tandem repeat
genotype were not different between the patients and the controls.
PHcy levels were not signicantly different between 3R3R and 2R3R
genotype in population as a whole (p0.698), nor in subsets of patients
with IPD (p0.391). However, in individuals with TSER 3R3R genotype, pHcy level was signicantly higher in patients
(13.161.94mol/l) than in controls (8.951.89mol/l)(p0.01).
Conclusions: Our ndings suggest that TSER genotype is not a
major determinant of pHcy levels neither a risk factor for IPD in
Korean patients. Larger polupation based study may be required to
conrm this ndings.
581
The PARS study: Reaching for pre-motor Parkinsons disease
D.L. Jennings, A. Siderowf, M.B. Stern, K. Marek (New Haven,
Connecticut, USA)
Objective: The PARS study will develop and test a strategy to
identify a large-scale cohort at risk for Parkinsons disease (PD) using
combined olfactory testing and dopamine transporter (DAT) imaging in
rst-degree relatives of PD patients.
Background: There is a lengthy pre-motor phase of PD during which
extra-nigral neurodegeneration precedes the onset of motor symptoms.
Pathological changes in the anterior olfactory region may be the earliest
site for neurodegeneration in PD. Profound decits in olfaction are
documented in early PD and olfactory loss in asymptomatic relatives of
PD has been associated with a 20% risk of DAT decit and a 10% risk
of developing clinical PD within 2 years (Ponsen, et al 2004). In a pilot
study, the validity of administering the University of Pennsylvania
Smell Identication Test (UPSIT), a test of olfactory performance, by
mail survey was assessed. 213 1st degree relatives of PD patients
returned valid self-administered UPSITs. 43 (20%) respondents had an
UPSIT score 15th percentile for age and gender. 25 subjects completed the UPSIT at home and in clinic with mean scores of 32.5
(home) and 31.8 (clinic) (ICC0.90). This pilot study demonstrates the
reliability of the UPSIT at home approach and suggests that it is
feasible to identify 1st degree relatives with clearly abnormal UPSIT.
Methods: The ongoing PARS study aims to identify 7,500 PD from
20 US PD research centers to recruit their 1st degree relatives to
participate. Relatives who agree will complete a 40-item UPSIT and
return it by mail. Based on our pilot data we would anticipate 4,320
valid returned UPSITs and about 860 to have substantial olfactory loss
(15th percentile for age and gender). Individuals with olfactory loss
and 10% of those with normal olfaction will undergo longitudinal
clinical follow-up, [123I]-CIT/SPECT imaging and collection of biological samples.
Conclusions: The PARS study is a multi-center observational study
of individuals at-risk for PD. It will test a practical strategy to identify
a cohort of individuals in the pre-motor phase of PD. Once assembled,
the PARS cohort will provide an opportunity to study clinical, imaging
and biochemical markers of the immediate pre-motor stage of PD.
Ultimately, these insights will guide the design of clinical trials to test
interventions that may prevent the onset of motor features of PD.
582
Early-onset Parkinsons disease and depression
D.C. Bertucci Filho, H.A.G. Teive, R.P. Munhoz, N. Becker,
L.C. Werneck (Curitiba, PR, Brazil)
Objective: In this study we aimed to dene the frequency of depression in patients with early-onset Parkinsons disease (EOPD) and to
identify possible differences between the groups with and without
depression.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S178 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: Parkinsons disease (PD) is the second most common
neurodegenerative disease and affects 1 to 2% of the population over
65 years of age. Patients who develop the clinical manifestations of PD
at an earlier age are called early-onset PD (EOPD) and represent 5 to
10% of the total. The incidence of depression in EOPD is believed to
be greater than in the late-onset form of the disease.
Methods: Patients diagnosed with EOPD according to the international criteria of Parkinsons disease (presence of at least two of the
following signs or symptoms: bradykinesia, tremor, muscular rigidity,
and postural instability), with good response to levodopa treatment (
30%); progressive disease, with the onset of symptoms before the age
of 45 years.were studied. Patients were considered to have major
depression if they had 5 or more of the 9 symptoms of the criteria of the
DSM-IV. We used the Hamilton scale with 17 items to quantify the
severity of the depression.The DSM-IV and Mini Mental State Examination (MMSE) were used as criteria to diagnose dementia. The
severity of PD was quantied using the Hoehn-Yahr scales, the Unied
Parkinson Disease Rating Scale (UPDRS) motor examination and the
Schwab and England activities of daily living. Forty-ve patients were
found to fulll the above criteria of EOPD.
Results: Depression was diagnosed in 16 (35.5%) of the patients, a
higher incidence than in the population at large but similar to the gure
for late-onset PD; 8 (50%) of the patients had mild depression, 4 (25%)
moderate depression and 4 (25%) were in remission. There was no
relationship between depression and any of the clinical characteristics
of the disease, although the EOPD presented earlier levodopa-related
complications and were more affected on the Hoehn-Yahr, UPDRS and
Schwab-England scales.
Conclusions: The incidence of depression observed in EOPD patients in our study was greater than that of the population at large,
although similar to gures reported in studies of patients with late-onset
PD, and had no relationship with any clinical characteristic of the
disease.
583
Is there an increased risk of young onset Parkinsons disease in
patients with a history of poliomyelitis?
P. Agarwal, A.L. Diamond, V.R. Segro (Englewood, Colorado, USA)
Objective: To report a possible increased risk of young onset Parkinsons disease (PD) in patients with a history of poliomyelitis.
Background: Poliomyelitis is caused by an enterovirus which causes
acute lower motor neuron dysfunction often resulting in chronic paralysis of the extremities, bulbar muscles are less frequently affected.
Poliomyelitis is still prevalent in some eastern hemisphere countries.
Post-polio syndrome consists of progressive weakness in a patient
stable for 10 years after the initial attack. It is not known if any other
motor pathways such as the extrapyramidal pathways could be affected
several years after onset of acute poliomyelitis.
Methods: A retrospective chart review was conducted of two patients
at our movement disorders center who had a history of acute polio and
then presented with symptoms of PD at a young age.
Results: 47 year old male Mexican patient who suffered from an
attack of polio at age 1.5 years with residual severe bilateral lower
extremity weakness and atrophy, noticed new onset of rest tremor and
difculty using the left arm. Family history was positive for mothers
cousin having PD. On examination, he had left upper and lower
extremity rest tremor, moderate rigidity and bradykinesia. Pull test
could not be performed because of profound weakness of the lower
extremities from polio.He met the clinical criteria for PD, pramipexole
was initiated with clinical bent. 50 year old male with a diagnosis of
poliomyelitis at age 5 years, was evaluated for a 10 year history of rest
tremor, bradykinesia and rigidity in all four extremities. He responded
well to carbidopa/levodopa and developed end of dose wearing off
manifested by dyskinesias and dystonia.
Conclusions: Both our patients had a history of poliomyelitis in
childhood and then developed PD in their 40s. The early onset of their
symptoms raises the question that the co-occurrence of PD and polio
may be more that a chance occurrence. Polio virus mediated immune
mechanism, or stress on the extrapyramidal neurons from the abnormal

Movement Disorders, Vol. 22, Suppl. 16, 2007

gait mechanics may make these patients more prone to developing PD

at a young age. Larger epidemiological studies are needed to determine
incidence and prevelance of PD in patients with a history of poliomyelitis.
584
A study of clinical effect of STN-DBS for Parkinsons disease in
younger and elder patients
H. Saiki, H. Toda, M. Nishida, S. Kaneko, S. Kosaka, M. Ishikawa,
H. Itoh, S. Matsumoto (Osaka, Japan)
Objective: To clarify age-related limiting factor of clinical effect of
subthalamic nucleus deep brain stimulation (STN-DBS) for Parkinsons disease (PD).
Background: STN-DBS is effective for advanced PD patients, especially with severe motor uctuation such as wearing-off, peak-dose
dyskinesia. However, most of facilities usually does not operate patients elder than 70 or 75 y.o. because of risk increment in aged patients
whereas clinical effectiveness tends to diminishes. On the other side, it
is still unclear whether aging itself is independent limiting factor of
prognosis of STN-DBS or not. Here we studied differences of clinical
effect of STN-DBS in younger and elder patients group with same
stimulation parameters in drug off state.
Methods: Nine PD patients(47 to 72 y.o, bilateral operation 7,
unilateral 2) were studied. Within two weeks after DBS operation,
effects of each contact of DBS lead were surveyed with same settings;
unipolar stimulation, cycle 130PPS, pulse width 60uS and battery
current 25uA. The study was conducted in drug-off period; without any
anti-parkinosian drug since the last night. Clinical symptoms were
evaluated with subscales of Unied Parkinsons Disease Rating Scale
(UPDRS). Changes of UPDRS scores between off stimulation and on
stimulation with best contacts both in bilateral and unilateral stimulation were compared between younger (under 60 y.o., n5) and elder
(over 60 y.o., n4) age groups.
Results: Improvement of UPDRS part 3 (motor function score) is
15.34.8 in younger group and 16.96.0 in elder group with unilateral
stimulation, 27.012.5 in younger and 23.015.6 in elder group with
bilateral stimulation. There was no signicant difference between
younger and elder group.
Conclusions: We could not nd age related difference of clinical
effect of STN-DBS with small population of this study, which suggests
further study with larger population is mandatory. Also, we found there
are excellent responders who showed marked improvement with on
stimulation not only in younger but also in elder group. Those excellent
responders in both group suggests un-detailed age-independent factors
play an important role to provide clinical effectiveness of DBS. Clarifying those factors may contribute to better clinical outcome of STNDBS.
585
Worsening of motor symptoms and gynecomastia during spironolactone treatment in a patient with Parkinsons disease and congestive heart failure
H.A. Teive, R.P. Munhoz (Curitiba, Brazil)
Objective: To describe a patient with Parkinsons disease (PD) and
marked worsening of motor symptoms and gynecomastia during spironolactone therapy for congestive heart failure (CHF).
Background: Etiologic recognition in secondary parkinsonism has
always been of particular interest because of its potentially reversible
nature. Among other causes, drug-induced parkinsonism (DIP) due to
an ever-growing list of agents, have received particular attention not
only for its high prevalence but also because most of the same drugs
can also worsen motor symptoms when used in patients with stable PD.
Methods: Single case report.
Results: A 76-year-old male with a 12 year history of PD, stable
from the motor standpoint. CHF was treated with digoxine, furosemide
and aspirin. UPDRS part III score in the ON state was 25. Due to
worsening of CHF symptoms, the patient was started on spironolactone
100mg qd. After a week he came for an early follow-up after abrupt

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

motor deterioration. His ON state UPDRS part III score was 35 and he
complained of painful bilateral gynecomastia. Dopaminergic treatment
was not modied and a work-up was performed to detect potential
malignancies or other secondary causes. Hormonal prole was normal,
except for high prolactine level (91.5 ng/ml, normal 20 ng/ml).
Spironolactone was then withdrawn. On follow-up after 30 days there
was a signicant improvement with remission of gynecomastia and
return of motor function back to baseline. Last prolactine level assessment was 20 ng/ml.
Conclusions: Most of the side effects related to spironolactone reect
its interactive multi-level antiandrogenic effects. This explains gynecomastia in the case presented here. Hiperprolactinemia is the most
frequent hormone change in patients with CHF. Our review of the
literature found no evidence of a clear relation between prolactin level
and either spironolactone use and aldosterone secretion. In regards to
motor function, several studies showed that prolactin levels are inversely correlated with motor function in PD serving as an indicator of
dopaminergic receptor function. Also, testosterone has been shown to
improve motor function and apathy in patients with PD . Although our
patients level of testosterone was normal, spironolactone may have
played its antiandrogen role at testosterone-related target tissue levels.

586
Studying emergence of behavioral changes during one month of
intensive voice treatment (LSVT) in a person with Parkinsons
disease
J. Spielman, A. Halpern, L. Ramig, C. Fox (Denver, Colorado, USA)
Objective: To evaluate weekly behavioral changes in voice and
speech over a 1-month period of intensive voice therapy (LSVT) in a
person with Parkinsons disease (PD).
Background: Individuals with PD can often be cued to modify
behavior, so that some PD-related impairments (e.g. soft voice) appear
to normalize in the clinic. However, these changes are not effectively
acquired through traditional models of speech therapy. Efcacious
speech therapy for PD requires intensive practice and repetition aimed
at generating the internal cues necessary for long-term change and
maintenance. More than 15 years of research has established the LSVT
as a highly efcacious speech therapy program for PD. However, little
is known about how treatment-related changes emerge during the
therapeutic process.
Methods: A subject with PD receiving the LSVT (16 sessions) was
recorded before and during sessions 1, 5, 9, and 13 of treatment (the
rst therapy session of each week). Before treatment, he was recorded
while describing two different pictures under one of two conditions
(doing nothing, or alternately tapping middle and index nger). During
treatment, one of his regular treatment tasks was recorded. Data were
analyzed for changes in vowel articulation and pause time during
tapping and no-tapping conditions, speech behaviors not directly targeted in treatment.
Results: Vowel space (the area created by mapping formant frequencies F1 and F2 for vowels /i/, /u/ and /a/) increased from day 1 (144,437
Hz2) to day 5 (204,508 Hz2) to day 13 (224,515 Hz2), with a dip on day
9 (155,467 Hz2). The relationship between the second formant for
vowels /u/ and /i/ changed in a positive direction on each of the 4 data
collection days (1.57, 1.96, 2.0, 2.02), indicating increasing differentiation among vowels. Percent pause time in the no-tapping condition
decreased from days 1 (47.1%) to 5 (38.6%) to 13 (35.6%). In the
tapping condition, pause time decreased at each session, suggesting
improvements in the subjects ability to multi-task.
Conclusions: Data indicate that this subject made measurable
changes reecting improved performance throughout the 16 sessions of
therapy. We propose that such interim measures can be valuable for
improving efciency of therapy programs, and studying learning in
patient populations.

S179

587
Dopamine dysregulation syndrome: Impact of subthalamic chronic
stimulation
T. Witjas, J. Regis, M. Delphini, J.C. Peragut, J.P. Azulay
(Marseille, France)
Objective: To report the outcome of 7 patients with Dopamine
Dysregulation Syndrome (DDS) who underwent STN DBS.
Background: DDS is a rare syndrome occurring in young Parkinsons disease (PD) patients. It is characterized by behavioural disorders
associated with excessive use of dopaminergic replacement therapy(DRT). The effect of DBS on this syndrome is matter of debate (Voon
et al 2006). After the long-term improvement of 2 patients who suffered
from this syndrome, we evaluated prospectively patients diagnosed
with DDS and candidate for STN DBS.
Methods: Patients with selection criteria for DDS(Lawrence et
al.2003) were evaluated before and 12 to 24 months after surgery. The
UPDRS, Mattis Dementia Rating scale, Beck Depression Inventory,
Hospital Anxiety and Depression Scale, questionnaire for non motor
uctuations and psychiatric interview were assessed.
Results: 85 patients were selected for STN DBS between May 2003
and June 2006. Among them, 7 patients (6 men, mean age at surgery:
52yo, mean disease duration: 9,7y) who suffered from DDS were
implanted bilaterally in the STN. All the patients had preoperative
history of DRT abuse associated with behavioural disorders: hypomania: 7, hypersexuality: 5, psychosis: 5, pathological gambling: 4, craving: 3, punding:2. After surgery, the motor and dyskinesia improvement at 1year was 72 and 85% respectively. The LEDD was reduced
signicantly(71%). The behavioural disorders were dramatically reduced as no psychosis, hypomania or hypersexuality was reported after
chronic STN stimulation. One patient had a transient episode of depression 6months after DBS and one patient complained of apathy. For
one patient, the LEDD, especially dopamine agonist was not decrease
rapidly enough and he had one episode of hypomania with hypersexuality, which resolved after massive reduction of DRT without any
change in stimulation parameters.
Conclusions: From these cases, STN DBS may be a useful treatment
for DDS. However, patients should be well-selected and detected
before surgery in order to propose a rigorous postoperative management.
588
Does somatization have a role in the manifestation of the nonmotor symptoms of Parkinsons disease?
H. Murck, M. Stacy, K. Kroenke (Hanover, New Jersey, USA)
Objective: To investigate the recognition of motor and non-motor
symptoms of wearing off in patients with Parkinsons disease (PD).
Background: There is increasing awareness of the non-motor symptoms of PD. This goes in parallel with the recognition that dopamine is
not only involved in motor function, but also in sensory mechanisms,
such as pain threshold. Somatization is a term which refers to medically
unexplained symptoms and involves an alteration of pain perception.
Phenomenologically there is an overlap between typical symptoms of
patients with somatization and those with non-motor symptoms of
wearing off, in particular the complex of pain/aching, anxiety and
mood changes. Moreover, primarily psychiatric disorders have typical
psychomotor and vegetative expressions, such as tremor, bradykinesia and gastrointestinal complaints.
Results: We examined the presence of the motor and non-motor
symptoms of wearing off using the new self-report screening tool
Wearing-off Questionnaire (WOQ)-9. This differentiated the presence
of the symptoms and their sensitivity to the next dopaminergic drug
administration. Among patients who reported non-motor symptoms
(anxiety, pain, mood-changes), 4350% of patients experienced an
improvement with the next dose. This gure was only slightly lower
than that for motor symptoms (slowness, reduced dexterity), which was
in the range of 48 66%. However, the reporting of single non-motor
symptoms was much lower (25 48%) in comparison to motor symptoms (56 80%). This discrepancy between the reported presence of

Movement Disorders, Vol. 22, Suppl. 16, 2007

S180 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

motor versus non-motor symptoms despite similar reported improvements in all symptoms, leads to the hypothesis that non-motor symptoms might be attributed by the patient to motor symptoms and are,
therefore, not recognized as separate symptoms.
Conclusions: The conceptual framework of PD as a primarily motor
disease might conceal the recognition of non-motor symptoms. Their
importance as symptoms in their own right should be stressed and
further explored.
589
Parkinsons disease; relationship between neuroinammation and
cognition
A. Bora Tokcaer (Ankara, Turkey)
Objective: To evaluate the effects of proinammatory cytokines on
P300 activity in patients with Parkinsons disease (PD).
Background: There are some studies which suggest autoimmune
disorders play a role in the pathogenesis in PD. In PD microglia in SNc
and not elsewhere in the brain have been shown to be activated. These
activated microglia cluster around the dopaminergic neurons may cause
cell death via phagocytosis. The role of proinammatory cytokines in
PDs pathogenesis is poorly understood despite of the evidence of the
concentration of proinammatory cytokines such as TNF and interleukin-6 (IL-6) increase in the plasma of the patients with PD. On the
other hand, the presence of certain inammatory cytokines in the
cerebrospinal uid of demented patients was demonstrated. Frontal
type cognitive decits are frequently described in PD. In advanced
stage of PD almost 30% of patients have dementia. To investigate
whether neuroinammation is a causative factor of dementia associated
PD, we decided to study the correlation between TNF, IL-6 and
cognitive tests. In this study, event related potentials (ERPs) which
provide a means of measuring cognitive processing that is independent
of motor speed and disability were prefered as a marker cognitive
abilities.
Methods: In this study we examined 30 patients with PD and 30
healthy controls. TNF and IL6 were measured in sera by using ELISA
method. P300 potentials were recorded by using auditory oddball
paradigm by being given in two different frequency stimuli.
Results: The mean of age was 52 6.7 in the PD group, and 497.2
in the control group. TNF and IL-6 levels in sera of patients with PD
were signicantly higher than control subjects (19.16.8 pg/ml and
18.67.1 pg/ml vs 5.82.7 pg/ml and 6.75.9 pg/ml, respectively)
(p0.001, p0.01). Latency of P300 in PD was longer than control
group (487159 msec vs 348147.2 msec) (p0.001). After 3 months
treatment with levodopa, TNF and IL-6 levels decreased at the similar
levels of control subjects (p0.05). The latency of P300 was signicantly shorter than before treatment value (p0.01).
Conclusions: We suggest the correlation between neuroinammation
and cognitive dysfunction in PD. Levodopa may repair the cognitive
status by reducing neuroinammation.
590
Diplopia in Parkinsons disease: A clinical and optometric study
L. Bye, K. Ray Chaudhuri (London, United Kingdom)
Objective: To explore the cause of unexplained diplopia in a group
of Parkinsons disease (PD) patients declaring diplopia in the non
motor questionnaire (NMSQuest)1.
Background: To explore the cause of unexplained diplopia in a group
of Parkinsons disease patients declaring diplopia in the non motor
questionnaire (NMSQuest)1.
Methods: A subset of treated PD patients who had declared diplopia
on NMSQuest were invited to take part in detailed clinical and ocular
examination including measurement of accommodation using Royal
Air Force (RAF) rule.
Results: 8 patients with moderate to severe diplopia (mean age 70 yrs
(range 59-84 yrs), F6, mean disease duration 9.9 yrs (range 1-22 yrs)
were studied. Mean duration of diplopia was 12.5 mnths range (2 mnths
2 yrs) with gradual onset in all except one case. Diplopia was
intermittent in all and horizontal in 6 (mean distance, range 6-20cm).

Movement Disorders, Vol. 22, Suppl. 16, 2007

Convergence insufciency on RAF rule was present in 6/8 cases. None

had abnormality of external ocular movements, anti-acetylcholine receptor antibodies and MRI brain imaging. In comparison 4 control PD
subjects with no diplopia had normal convergence. Diplpoia was not
linked to motor response uctuations.
Conclusions: Diplopia in PD appears to be common, mostly horizontal in direction and appears to be linked to convergence insufciency.
1. Chaudhuri KR, Martinez-Martin P, Schapira AHV, Stocchi F,
Sethi K, Odin P et al. (2006) Mov Disord 21(7):916-923.
591
End-of-dose blepharospasm and its relationship to the dose of
levodopa: Observation in a patient with Parkinsons disease
P.Kr. Pal, A. De Souza, P.S. Bindu (Bangalore, Karnataka, India)
Objective: To report an unusual end-of-dose phenomenon in a patient of Parkinsons disease (PD) with motor uctuations.
Background: In addition to the common peak-dose dyskinesias,
patients with long standing PD may have disabling dyskinesias or
dystonia at the end-of-dose period. Focal dystonia in the form of
blepharospasm and its temporal relationship to levodopa has not been
well documented.
Methods: Case description with Video.
Results: Case Report: A 76 year old woman, diagnosed to have PD
since 5 years, started having motor uctuations in the form of end-ofdose wearing off and peak dose dyskinesias since one year. She was on
levodopa and ropinirole. However recently she noted spontaneous
closure of her eyes about 3 hours after a dose of levodopa (levodopa
(LD)/carbidopa (CD): 250/25), lasting for about 212 hours and spontaneously improving before the next dose, which she usually took after
6 hours. We admitted her in the ward to document (clinical examination
and video) the phenomenon. Examination (baseline observation) 612
hours after a dose of LD/CD 250/25 showed moderately severe (grade
3 severity on Unied Parkinson Disease Rating Scale (UPDRS)) bradykinesia and rigidity, but no tremor, dyskinesia or blepharospasm. She
was then given another dose of LD/CD 250/25, and the following
observations were made: (a) between 1-2 hours: peak dose dyskinesias,
grade 1-2 bradykinesia and rigidity, no tremor or blepharospasm; (b) at
312-4 hours: onset of blepharospasm, gradual increase in bradykinesia
and rigidity, reduction in dyskinesia, occasional tremor; (c) at 412-5
hours: severe blepharospasm, grade 2-3 bradykinesia and rigidity, more
persistent tremor, axial and nuchal dystonia, occasional dyskinesias;
and, (d) 512 -6 hours: grade 2-3 bradykinesia and rigidity, but no
blepharospasm, tremor, or dyskinesias. Increasing the dose of ropinirole, and more frequent (4 hourly) administration of a lower dose of
levodopa, reduced the blepharospasm signicantly. Six months later
she again started having blepharospasm just before the next dose of
levodopa, but it responded completely to levodopa within 15 minutes.
Conclusions: Disabling dystonia may occur during the declining
phase of serum levodopa. In selected patients, more frequent dosing
may be useful in managing such complications.
592
Factors that impact quality of life in patients with Parkinsons
disease
T. Simuni, D. Breslow, L. Vainio, S. Miskevics, C. Zadikoff,
F. Weaver (Chicago, Illinois, USA)
Objective: To assess what factors correlate with disease related
quality of life (QOL) in Parkinsons disease (PD) subjects receiving
care in a multidisciplinary center where patients are seen by a physician, social worker, physical therapist and other professionals.
Background: Based on recent data depression is one of the major
factors that impact QOL in PD. Depression remains under recognized
in PD. Limited data is available on the impact of multidisciplinary care
on the non-motor symptoms of PD.
Methods: 72 participants were recruited from patients seen at a
university PD center. Subjects were mailed a package that included:
consent form, demographics questionnaire, the PD Sleep Scale (PDSS),

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

the Epworth Sleepiness Scale (ESS), the PDQ-39, the Geriatric Depression Scale-15 (GDS), and the Lubben Social Support Network
Scale (LSNS). H&Y scale was obtained from the medical records.
Results: The mean age of the cohort was 68.9 (range 41-83), mean
disease duration-6 years (range 1-24), 63% had mild-moderate disease
(H&Y1-2.5) and 37% had more advanced disease (H&Y 3). 83% of
the subjects lived at home; 25% were employed; 39% of subjects
attended a PD support group. Mean GDS-15 score of the cohort was 3.8
though only 48/72 completed that scale; 16 subjects (33%) had GDS-15
score 5 consistent with the diagnosis of depression. Mean ESS score
was 8.9; half (33/63) of those who completed ESS scale had score10
consistent with excessive day time sleepiness (EDS). Mean PDSS score
was 52.4. There was a signicant correlation between depression and
worse scores in each domain of PDQ-39 except social, as well as with
PDSS and ESS scores. Individuals with sleep problems (ESS10) had
poorer scores on each domain of PDQ-39 except emotional and social,
as well as PDSS and GDS. Sample size was insufcient for multivariate
analysis. Neither depression nor EDS correlated with age or disease
duration.
Conclusions: Depression and excessive day time sleepiness have
high prevalence in PD even in a multidisciplinary center where patients
are routinely screened for non-motor co morbidities. Both factors have
a signicant impact on disease related QOL impairment. Healthcare
providers should continue to be vigilant in screening for depression and
EDS. Future studies will demonstrate if treatment of these co morbidities leads to improvement of QOL.
593
PRODEST study: Depressive symptoms in Parkinsons disease
P. Barone, C.G. Goetz, J. Houben, K. Juergen, A.F.G. Leentjens,
W. Poewe, O.O. Rascol, H. Reichmann, A.H. Schapira, E. Tolosa
(Naples, Italy)
Objective: Explore depressive symptoms in Parkinsons disease
(PD) in a large cross-sectional, multinational survey.
Background: Depression is recognized as one of the key non-motor
elements of PD, but the symptom complex, overall prevalence and
cross-cultural patterns have not been carefully explored.
Methods: PRODEST (PROle of DEpressive SympToms in Parkinsons Disease) is a multinational, multicenter, observational study from
24 centers in 8 European countries.
Results: We enrolled 1023 patients with Parkinsons disease according to the UKBB criteria; the full analysis set included 1016 patients
who met the predened MMSE 24 inclusion criterion. Within the
analysis set 58.8 % were men, 41.2 % women; mean age 65.5 yrs (SD
9.9). Patients with uctuations were 48.3% of the study population.
Mean age of PD onset was 59.0 yrs (SD 10.6) and mean PD duration
was 6.85 yrs (SD 5.35). Depression was reported by 284 patients
(27.8%). Antidepressant therapy was administered in 20.8% of the
overall population. Among patients with depression on antidepressants,
71.8% still showed current depressive symptoms (BDI). DSM IV major
depressive disorder occurred in 10.1%. UPDRS I item 3 (depression)
was scored as 0 (no depression present) in 55.6%, mild 31.9%, moderate 7.9%, marked 4.4%, severe 0.3%. Mean HAMD-17 score in the
total study population was 7.5 (SD 5.7). Mean FAB total score 15.3
(SD 2.9). HADS anxiety 5.6 (SD 3.8), HADS depression 5.3 (SD 3.8).
BDI 9.8 (SD 7.4). Correlation and factor analysis will be done on full
analysis set to determine items most characteristic for depressive symptoms in Parkinsons disease and cross-cultural patterns.
Conclusions: Depressive symptoms are common in PD patients.
Study is supported by Boehringer Ingelheim
594
Cognitive function assessment in idiopathic Parkinsons disease
patients. Control study with normal subjects
M.R. Piovezan, H.A.G. Teive, E.J. Piovesan, M.J. Maeder,
L.C. Werneck (Curitiba, PR, Brazil)
Objective: Our aim was to determine the cognitive behavior of the
Parkinsons disease (PD) patients.

S181

Background: In Idiophatic PD occurs the lost dopaminergic tonus of

the nigrostriatal and cortical brain regions. These structural changes
generally produce movement disorders and latter on, behavior and
cognitive disturbs.
Methods: We studied 30 patients with PD, with mean age 64,23
11,24 years old and compared with a control group (CG) of 30
patients, mean age age 64,13 11,27 years old. All patients were
submitted to: motor assessment utilizing the UPDRS scale; and HoehnYahr modied scale; depression assessment utilizing the MontgomeryAsberg scale; assessment of attention compromising; verbal inuence
tests (FAR), animals; cognitive function utilizing the Mini Mental State
(MMS); assessment of visuospatial and executive functions (house
drawing) and test of clock drawing.
Results: The UPDRS scale showed 21.43 7.20 points and HoehnYahr modied 2,43 0.92 points for the PD; depression assessment
utilizing the Montgomery-Asberg scale, for PD was 12.80 6.86 points
and for CG 6.27 6.45 points (p0.0013); assessment of attention
compromising for PD 4.83 10.11 and for CG 0.53 2.03
(p0.0003); verbal inuence tests (FAR) for PD was 15.4011.06
words and for CG 23.50 11.15 words (p 0.0093), (animals) for PD
was 9.43 4.25 words and for CG 11.97 3.01 (p0.0121); cognitive
function utilizing the Mini Mental State (MMS) for PD 23.10 4.89
points and CG 27.80 2.01 (p0.0001) points; assessment of visuospatial and executive functions for PD during the house drawing was
4.17 1.26 points and for CG 4.97 0.18 (p0,0015); test of clock
drawing for PD 6.07 2.99 points and CG 4.97 0.18 (p0,0002).
Conclusions: PD patients showed motor and cognitive disabilities,
furthermore executive and verbal functions, visuospatial and attention
disturbs. Depression is more signicant in PD than control group.
595
Hypersexuality in Parkinsons disease: Differences in associated
factors may lead to the expression of different impulse control
behaviors
V. Voon, T. Thompsen, J.M. Miyasaki, M. de Souza, M. Zurowski
(Bethesda, Maryland, USA)
Objective: To determine factors associated with pathological hypersexuality (HS) in Parkinson disease (PD).
Background: Medication-related incentive-based and repetitive behaviors in PD include HS, pathological gambling (PG), compulsive
shopping, eating, medication use and punding. PG is associated with
high novelty seeking, alcohol use, young PD onset, medication-induced
mania and right sided PD onset(1).
Methods: HS patients that fullled our working diagnostic criteria(2)
were compared with PD patients without compulsive behaviors.
Results: 18 HS patients (without PG; 18 males; age 58.6/-11.6y;
age of PD onset 49.7/-12.2y; LEDD 972.5/-548.6mg/d; dopamine
agonist dose 187.3/-96.9mg/d; right sided PD onset 8/18; HY
2.5/-0.4; UPDRS III 22.2/-6.6; MMSE 25.2/-1.2) were compared
to 42 PD controls. Male gender (p0.001), current depression
(p0.0001), younger PD onset (p0.007), and increased novelty seeking (p0.006) predicted HS at 87.2% (R20.67) in a logistic regression model. To increase power, the types of dopamine agonist in HS
patients were compared with published dopamine agonist proportions
prescribed in the same clinic population(2). HS patients were more
likely to be on pramipexole 13/17 (HS 76.5% versus PD controls 46%;
p0.02) compared to ropinorole 3/17 (17.6% versus 34.2%) or pergolide 1/17 (5.9% versus 20%). One patient was on levodopa monotherapy. There were no signicant differences in LEDD, dopamine
agonist dose or side of PD onset.
Conclusions: HS patients are more likely to be male and have a current
depression. PG , in contrast, is not associated with gender but is associated
with medication-induced mania and right-sided PD onset(1). Both PG and
HS are associated with novelty seeking and young onset PD. Whereas PG
does not appear to be related to any specic dopamine agonist(1,2), HS
may be more likely to be associated with pramipexole implicating a D3
mechanism. The results suggest that differing associated factors may lead
towards the expression of specic behaviors. Despite potential similarities,
PG and HS should be studied separately.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S182 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

References:
1. Voon V et al. Factors associated with dopaminergic drug-related
PG in PD. Arch Neurol, 2007
2. Voon V et al. Prevalence of reward-seeking and repetitive behaviors in PD. Neurology, 2006.
596
Decreased plasma alpha synculein levels in patients with Parkinsons disease
B. Neumayer, M. Reindl, K. Seppi, E. Wolf, S. Boesch, H. Stockner,
M. Kogler, A. Zangerl, K. Mair, G.K. Wenning,
M. Stampfer-Kountchev, W. Poewe (Innsbruck, Tirol, Austria)
Objective: To examine if alpha synuclein could be a biomarker for
Parkinson s disease (PD) we measured the blood concentration of
alpha synuclein in PD patients (n125) and healthy controls (n50).
Background: Pathological aggregation and intraneuronal depositon
of alpha synuclein is a pathological halmark of PD. Recent studies have
also reported decreased plasma levels of alpha synuclein in PD as
compared to controls.
Methods: We recruited 125 patients with PD from our MD outpatient
clinic. All patients were diagnosed with Hughs criteria for PD by at
least two Movement Disorder specialists. PD patients, aged between 37
and 86 (mean 64,3 /- 12,0), are compaired with controls with an mean
age of 51,0/- 9,2. To measure the alpha synuclein blood level we used
an ELISA system with a horseradish peroxidase labeled anti-rabbit IgG
. Sensitivity and specicity values were calculated for alpha synuclein
concentration, using optimal cut-off values determined by receiver
operating characteristics (ROC) curve analysis.This curve plots sensitivity (i.e., relative number of patients with PD identied by a abnormal
test result) vs specicity (i.e., probability of not having PD given the
absence of the abnormal test result) for every possible cut-off point.
Results: PD patients had a signicant (p0.001) lower alpha
synuclein concentration (mean 10.3 /- 22.6ng/ml) compared to the
healthy control group (mean 29.4 /-11.6 ng/ml). Maximal discrimination between patients and controls is reached at the cut-off level that
has the highest sum of sensitivity and specicity. This optimal cut-off
level (with an area under the curve of 0.93) for blood alpha synuclein
levels to discriminate between PD and healthy controls was 12.5ng/ml
implying that a alpha synuclein level of 12.5ng/ml or lower is indicative of a diagnosis of PD. Sensitivity for the cut-off level of 12.5ng/ml
was 83%, specicity 92%. The cut-off level at 7 ng/ml had optimal
specicity (100%) with a reasonable sensivity of 66%.
Conclusions: Concentrations of alpha-synuclein are decreased in
blood samples of PD patients compared to healthy controls, further
studies should include patients with non-PD synucleopathies like MSA
and Lewy body negative parkinsonian disorders.
597
Evaluation of a multidisciplinary daycare unit for patients with
Parkinsons disease (PD)
M.A. van der Marck, B.R. Bloem, M.L. van Nimwegen,
M.A.M. Schmidt, M. Munneke (Nijmegen, Netherlands)
Objective: To evaluate the quality of care as perceived by patients
and carers of a dedicated multidisciplinary Daycare Unit (MDU) for
PD.
Background: The Parkinson Center Nijmegen is a dedicated clinical
and research centre for PD. A core element is the MDU, which offers
PD families a comprehensive multidisciplinary assessment tailored to
individual needs, as prioritized by patients prior to consultation using a
specically developed questionnaire covering all domains of PD. Additional attention is given to problems experienced by carers. After a
3-day assessment, a detailed health plan is formulated that is then
implemented within the direct vicinity of the patients home by regional networks of specialized health professionals (ParkNet).
Methods: Perceived quality of care was evaluated through questionnaires and semi-structured interviews with patients and carers 6 months
after visiting the MDU.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Results: Thirty-eight patients (25 men, mean age 62.8 years (40-84),
mean Hoehn and Yahr score 2.6 (range 2-4)) and 35 carers participated.
Patients had PD (n24) or atypical parkinsonism (n14). 79% of
patients and carers were satised about the assessment and referrals.
Frequency of consultations, referrals and treatment are indicated in
Table 1. All patients were routinely seen by a social worker. Positive
elements were the specic expertise of health professionals, the comprehensive multidisciplinary assessment, and the attention given to
both patient and carer. Participants also gained more knowledge and
awareness about their disease. The questionnaire completed by patients
before admission to the MDU was also scored positively, since health
professionals were made aware of perceived problems before consultation. Suggestions for improvement included a request for receiving a
nal report of the overall assessment and more information about the
nature of subsequent referrals in regional ParkNet networks. When
asked about the overall quality of their visit on a scale from 1 (low) to
10 (high), high scores were given by patients (7.9, range 5-10) and
carers (8.2, range 6-9).
Conclusions: Perceived quality of care in a MDU is high among
parkinsonian patients and their carers. The effectiveness of this approach will be evaluated in a controlled study.

Table 1. Frequency of consultation within MDU, referral for

treatment and treatment delivered

Social worker
Physical therapist
Occupational
therapist
Speech therapist
Parkinson nurse
Sex therapist
Dietician
Neuropsychologist
Psychiatrist
Rehabilitation
medicine
Sleep specialist

% Consultation within
MDU (n38)

% Referral for
treatment (n38)

% Treatment delivered
(n38)

100
97

32
76

13
66

87
66
66
26
26
21
8

66
37
13
8
0
5
8

47
34
3
5
0
0
3

5
3

3
0

3
0

598
Adult human stem cells differentiated into astrocytes: Therapeutic
potential for Parkinsons disease
M.B. Stroomza, Y. Barhom, Y. Levy, O. Karpov, S. Bulvik,
E. Melamed, D. Offen (Petah-Tikva, Israel)
Objective: We aimed to differentiate human bone marrow-derived
mesenchymal stromal cells (hMSC) into astrocyte-like cells, capable of
generating NTFs.
Background: An alternative strategy to restore the surviving dopaminergic neurons is by treatment with neurotrophic factors (NTFs).
These proteins regulate the survival, functional maintenance and phenotypic development of neuronal cells.
Methods: hMSC were treated with our novel astrocyte differentiation
medium, and then transplanted into the striatum of 6-hydroxydopamine
(6-OHDA) lesioned rats.
Results: The induced cells presented astrocyte-like morphology and
express astrocyte markers. These cells secreted signicant amounts of
glial cell line-derived neurotrophic factor, nerve growth factor and
brain-derived neurotrophic factor. The cells produced behavioral improvement in motor tests. Histological assessments revealed that the
engrafted cells survived and expressed astrocytes and regenerated the
damaged dopaminergic nerve-terminal system.
Conclusions: This nding indicates that our novel procedure to
induce neurotrophic factors producing astrocyte-like cells from hMSC
might become a promising and feasible autologous transplantation
strategy for Parkinsons disease.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

599
The inuence of walking speed on lower extremity JOINT
TORQUE asymmetry in persons with early Parkinsons disease
J. Song, G.M. Petzinger, B. Fisher, J. Gordon, G.J. Salem (Los
Angeles, California, USA)
Objective: To determine the effect of walking speed on lower extremity joint torque asymmetry in persons with early-stage PD.
Background: Early-stage Parkinsons disease (PD) is characterized
by a predominantly unilateral appearance of clinical symptoms1. Muscle performance studies demonstrate that persons with early stage PD
have asymmetrical lower extremity weakness and that this asymmetry
is accentuated with increased movement speed2. To date, however, no
one has assessed the inuence of speed modication during functional
activities (e.g. walking) on lower-extremity motor performance asymmetry. With increased asymmetry at higher speeds, individuals with PD
may be at greater risk for falls, musculoskeletal injury, and freezing
episodes3.
Methods: Thirty participants with PD, diagnosed within 3 yrs, performed three 6-meter walking trials at two movement speeds: 1) self
selected (SS), and 2) as fast as possible (AFAP). An 8-camera motion
analysis system (60 Hz), ground reaction forces (1560Hz), and inverse
dynamics equations of motion were used to quantify segment kinematics and peak net joint torques (PNJT). Lower Extremity Asymmetry4
(LEA) was calculated as 100*( ln (RightPNJT/LeftPNJT)) at the hip,
knee and ankle joints. Paired t-tests were used to assess the differences
across the joints between movement speeds (p 0.05).
Results: Across joints, asymmetry was greatest at the knee (27.7
17.8; 21.6 16.8) followed by the hip (11.3 7.9; 11.28.1) and the
ankle (5.13.6; 9.17.2) during both SS and AFAP walking, respectively. Regarding the inuence of walking speed, the LEA was greater
at the ankle joint during AFAP walking; whereas, differences were not
evident at the hip or knee between walking speeds.
Conclusions: Our results suggest that asymmetry at the hip and knee
joints in early PD are not inuenced by movement speed. Contrastingly, joint torque asymmetry at the ankle becomes more exaggerated
with higher walking velocity. Future studies will be required to better
understand the effects of PD-related joint-torque asymmetry on other
functional tasks (e.g. stair climbing), falling behaviors, and the development of musculoskeletal injury.

S183

Conclusions: The intrastriatal infusion of fosB antisense and 5-amino7-(2-phenylethyl)-2-(2-furyl)-pyrazolo[4,3-]-1,2,4-triazolo[1,5-c]pyrimidine (SCH58261) have been reported for the correction of these molecular
mechanisms. Prevention of these changes is hoped to prevent L-DOPA
induced dyskinesia.Fig: This elaborate the normal (A) and dyskinetic (B)
neurotransmitter release from different parts of the brain. Smooth lines
show excitation (GREEN) while dashed one shows inhibition (RED). The
lines without arrow show blocked neurotransmission (BLUE). The
strength of the each output is indicated by the width of arrow. In g A
prodynorphin mRNA and glutamic acid decarboxylases mRNA (GAD67)
function in physiological limits while in g B it is enhanced. The neurotransmission of S.N to GP ES & to Thalamus, of GP external segment to
globus pallidus internal segment is shown in physiological (A) and pathological (B). SN (Subthalamic Nucleus), GP (globus pallidus)[ ES (external
segment) IS (Internal Segment)], Glu (Glutamate), KOP (kappa opioid
receptor), GAD67 (glutamic acid decarboxylase).

600
Molecular mechanism of levo-dopa induced dyskinesia in Parkinsons disease
F.U.H. Subhani (Quetta, Balochistan, Pakistan)
Objective: To study the mechanism of levodopa induced dyskinesia
in Parkinsons disease.
Background: Parkinsons disease is one of the common and complicated movement disorders. Successful therapeutic management is
available since decades in the form of L-DOPA. The most complicated
adverse effect of this drug is induction of dyskinesia after chronic
treatment.
Methods: It is a review to study the up to date mechanism for the
induction of L-DOPA induced dyskinesia in Parkinsons disease with
alternate therapeutic regimes to prevent it.
Results: One of the common and complicated adverse effects of
L-DOPA is induction of dyskinesia after chronic treatment for 4-10
years. After the induction of dyskinesias the positive effects of the drug
almost disappear. In the present review, mechanisms, which cause the
induction of L-DOPA induced dyskinesia (DID), are elaborated. The
two most important factors, which cause L-DOPA induced dyskinesia,
are increased activity of the kappa opioid receptor by dynorphin and
increased GABA formation by glutamic acid decarboxylase. Both these
chemical changes are caused by upregulation of prodynorphin mRNA
and glutamic acid dehydrogenase mRNA, respectively, after chronic
L-DOPA therapy as a result of gene expression. The ultimate effect is
to reinforce the inhibitory output from the internal segment of globus
pallidus to thalamus, which results in increased excitatory output from
the thalamus to the cerebral cortex causing dyskinesia.

FIG. 1 (600).
601
Clinical impact of fatigue in Parkinsons disease
V.K. Metta, A. Matouskova, R. Mitchell-Hay, A. Deshraj, J. Roomi,
K. Ray Chaudhuri (London, United Kingdom)
Objective: How common is clinically important fatigue in an unselected Parkinsons disease (PD) patients population attending outpatient clinics.
Background: Fatigue complicates over 40% of patients with PD but
is poorly recognised and treated. The presence and severity of fatigue
may be independent of other non motor symptoms such as excessive
daytime sleepiness and recently tools such as the Parkinson Fatigue
Scale (PFS-16) and the non motor questionnaire (NMSQuest) enables
the clinician/nurses to address fatigue in clinics.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S184 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: In an ongoing survey, 30 consecutive non depressed PD
patients completed the PFS-16 (scores 8 have signicant fatigue), the
Epworth Sleepiness Scale (ESS) and completed demographic forms
and NMSQues routinely used in the clinic.
Results: In 30 non depressed patients (mean age 69.6 /- 9.9,
duration of disease 9.1 /- 5.0, Mean Hoehn and Yahr (HY) score 2.8
/- 0.73), mean PFS score was 8.7/- 5.38. 56% had signicant
fatigue (mean age 72.7 /- 10.1). Duration of PD, HY scores were not
different between PD with and without fatigue. ESS was in normal
range in those with fatigue (mean 9.92 /- 2.67). PD patients with
older age (72.7 /- 10.1 vs 65.7 /- 8.6 yrs) and akinetic disease had
signicantly more fatigue than those with tremor dominant PD.
Conclusions: The ongoing study highlights that fatigue is an important clinical non motor symptom of PD across all stages and can occur
independent of disease severity, duration, depression and daytime
sleepiness and appears to be more prevalent in akinesia dominant PD.
1. Brown RG, Dittner A, Findley et al. The Parkinson fatigue scale.
Parkinsonism Relat Disord. 2005 11(1):49-55.
602
Types of driving errors committed by patients with Parkinsons
disease
D.P. McCarthy, C. Garvin, M.D. Justiss, D.N. Lanford, M.S. Okun,
R.L. Rodriguez, J. Romrell, W.C. Mann, S. Bridges, H.H. Fernandez
(Gainesville, Florida, USA)
Objective: To determine what types of driving errors made by
patients with Parkinsons disease (PD) differ from those made by
drivers without PD.
Background: Although evidence of driving safety among drivers
with PD is inconsistent, the literature suggests that this group differs in
the types of driving errors made compared to their age-matched
non-PD counterparts. This study is among the rst to utilize an occupational therapist driving rehabilitation specialist (DRS) to determine
the types of errors made by drivers with PD.
Methods: Patients with idiopathic PD were randomly recruited from
the University of Floridas Movement Disorder Center (MDC) and
referred to a DRS to determine safe driving ability, regardless of the
clinicians perception of their driving ability. Driving-related data,
including driving errors that occurred during typical driving maneuvers
(e.g., lane changes), were collected by the DRS during a comprehensive driving evaluation. Results from the PD group were compared to
an age-matched control group of drivers without PD.
Results: Nineteen PD patients (78% males) with a mean age of 75.0
years (SD 6.08), UPDRS mean motor score of 25.9 (6.9) on meds and
34.2 (9.9) off meds were analyzed and compared to a control group of
62 older drivers without PD (56.5% male, mean age 77.7 (5.85). The
comparatively high failure rate of the PD group (44% vs. 20% for those
without PD) is consistent with the literature. Signicant differences
between groups, with drivers with PD performing more poorly, were
found for vehicle positioning (p.03) and yielding to other trafc
(p.001). The ability to maintain lane position during driving maneuvers and the ability to appropriately respond to driving situations
approached signicance (p .08 and .06, respectively).
Conclusions: Results of this study indicate that the types of driving
errors among those with PD differ from drivers without PD. This
nding has implications for physicians working with clients with PD,
emphasizing the need to consider safe driving ability among these
patients. Additionally, results suggest the need for DRS to include these
maneuvers into their on-road driving assessment when evaluating a
subject with PD.
603
Metabolomic analysis in LRRK2 Parkinsons disease
K.K. Johansen, W. Matson, F. Beal, J.O. Aasly, M. Bogdanov
(Trondheim, Norway)
Objective: To develop biomarkers for the diagnosis of Parkinsons
disease (PD) using metabolomic analysis.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Background: In the recent years various hereditary forms of PD have

been recognized, one of the most frequent is LRRK2-G2019S substitution. Up to date there are no established diagnostic biomarkers to
conrm the disease and follow up its progression.
Methods: Metabolomic proling is a quantitative measurement of a
large number of low molecular weight molecules which reects both
genetic and environmental factors in a given time. Liquid chromatography electrochemical array detection (LCECA) was used to create a
database representing about 2000 metabolites. Multivariative data analysis and frequency distribution analysis were performed to determine
the metabolites. Plasma samples were acquired from 15 patients with
sporadic PD, 11 patients with LRRK2-G2019S substitution, 11 family
members without the mutation and 11 controls.
Results: The plasma metabolic proles of sporadic PD patients were
different from the proles of the G2019S patients. The analysis of the
data of the 3 subgroups: controls, family members without mutation
and the LRRK2-G2019S PD patients provided statistically signicant
separations as well.
Conclusions: Metabolomic analysis in this study signicantly separated PD patients from controls and even LRRK2 from sporadic PD.
This method should be performed in a larger population and it could
provide insight into pathogenesis of the disease. Metabolomics may
offer a non-invasive way to conrm PD diagnosis and follow up its
progression.
604
Effect of L-type Ca2 channel antagonist, Isradipine, in the abnormal involuntary movement rat model of dyskinesia
S. Schuster, A. Berthet, E. Doudnikoff, C. Ittrich, B. Bloch,
B. Hengerer, E. Bezard (Biberach, Germany)
Objective: It has recently been shown that striatopallidal neurons
lose their spines and glutamatergic synapses (Day et al., Nat. Neurosci.
2006) in PD. This loss of connectivity is triggered by a dysregulation
of intraspine Cav1.3 L-type Ca2 channels. We hypothesized that the
loss of connectivity is important in the development of adverse events
related to L-Dopa therapy as the system loses its ability to reconnect the
cortex and basal ganglia.
Background: In Parkinsons disease dopaminergic (DA) neurons that
innervate the striatum were lost. The major striatal target of dopaminergic innervation is the medium spiny neuron (MSN). These principal
neurons form two major efferent pathways. MSNs that project to the
external segment of the globus pallidus express DA D2 receptors,
whereas those projecting to the internal segment of the globus pallidus
and substantia nigra pars reticulata express DA D1 receptors. The
prevailing model of PD asserts that dopamine depletion elevates the
activity of striatopallidal neurons and lowers the activity of striatonigral
neurons, which leads to an imbalance in the control of basal ganglia
outow to the thalamus and an inability to move effectively in response
to higher motor system commands (Mallet et al, J. Neurosci., 2006).
Methods: By blocking the Cav1.3 L-type CA2 channels using
Isradipine (subcutaneous pellets at 0.05-0.1-0.2 mg/kg/day), we should
prevent the loss of spines and prevent development of L-Dopa-induced
(L-Dopa 6 mg/kg-benserazide 15 mg/kg, p.o.) abnormal involuntary
movements (AIMs)in the 6-OHDA rat model of PD. Extent of lesion
was checked with tyrosine hydroxylase immunohistochemsitry.
Results: Our results show a signicant dose-dependent effect of
Isradipine treatment in reducing the severity of AIMs. Blocking the
spine retraction in the 6-OHDA rat model shows a behavioural effect
and no blood-pressure-effect of Isradipine as ascertained by the rotation
test.
Conclusions: Further experiments are in progress to link the behavioural effects to the actual prevention of loss of spines on MSNs. D2
immunoreactive spines will be tested by peroxidase EM. The effect of
L-Dopa in the striatum will be investigated by testing the D2 receptor
trafcking and localization by combination peroxidase and immunogold EM.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

605
Chocolate consumption is increased in Parkinsons disease
M. Wolz, A. Kaminski, L. Matthias, S. Alexander, H. Reichmann
(Dresden, Germany)
Objective: To assess the consumption of sweets and chocolate in
patients with Parkinsons disease (PD) in comparison to their partners.
Background: In our Movement Disorder Clinic, we observed increased chocolate consumption in patients suffering from Parkinsons
disease. Furthermore, chocolate contains high amounts of various biogenic amines related to the dopamine and serotonin metabolism. There
are no structured data available on sweet and chocolate consumption in
PD.
Methods: We assessed the consumption of sweets and chocolate in
498 PD patients of our movement disorders outpatient clinic and their
partners using a structured questionnaire including the Becks Depression Inventory (BDI).
Results: 272 patients and 226 partners returned their questionnaires,
the return rate for PD patients was 55%. Consumption of chocolate was
signicantly higher in PD patients (132102 grams per week) compared to controls (9181 grams per week; P0.0001). Although PD
patients had signicantly higher BDI scores (2.3 versus 1.3;
P0.0001), there was no correlation between chocolate consumption
and depressive symptoms as measured by BDI (P0.128).
Conclusions: Our questionnaire study suggests that chocolate consumption is increased in PD patients independent from depressive
symptoms rated by BDI. The reasons for increased chocolate consumption in PD remain elusive, but might be a consequence of high contents
of various biogenic amines such as -phenylethylamine.
606
Nonmotor symptoms of Parkinsons disease: Prevalence and
awareness of patients and families
S.-M. Cheon, H.M. Jeong, S.M. Jun, J.W. Kim (Busan, Republic of
Korea)
Objective: The aim of this study is to know the prevalence of the
non-motor symptoms in PD and the awareness of patients and family
members on these symptoms.
Background: Nonmotor symptoms of Parkinsons disease (PD) are
known to be very common and have great impacts on the quality of life.
Appropriate management along with dopaminergic treatment can alleviate much of the symptoms. However, many of patients or families are
not aware that the nonmotor symptoms are associated with PD.
Methods: We interviewed 74 parkinsonian patients and 54 of their
family members on nonmotor symptoms of PD based on the questionnaire recently published by nonmotor symptoms questionnaire
(NMSQuest) study. The questionnaire consisted of 30 items on nonmotor symptoms of PD and we also asked whether the patients and
their family members were aware that the non-motor symptoms were
associated with PD.
Results: All patients except one (28 men, age 64.98.5 years) had
more than one symptom (mean 12.45.5 symptoms). Most common
symptoms were nocturia, constipation, restless legs, feeling sad, in the
order of frequency. Fecal incontinence and hallucination were least
common symptoms. Men had nocturia and restless legs while women
had feeling sad and memory disturbance most frequently. Only the
quarter of patients were aware that sialorrhea, nocturia, constipation,
feeling sad and feeling light headed, most frequently known symptoms,
were associated with PD. Hallucination and fecal incontinence were
least frequently known symptoms to the patients. Awareness of the
non-motor symptoms in the patients was correlated with duration of
disease and education, and number of non-motor symptoms they had.
On the other hand, memory disturbance, feeling sad and sleep disturbance were most frequently known symptoms to the family members,
and sexual dysfunction, nausea and hallucinations were least frequently
known symptoms.
Conclusions: Patients and their family members are not well aware
that non-motor symptoms are associated with PD, although they are

S185

very common in PD. In order that PD is properly managed, non-motor

symptoms should be looked for comprehensively.
607
Impact of young onset parkinsons disease on employment
C.L. Wielinski, C. Erickson-Davis, S.A. Parashos (Golden Valley,
Minnesota, USA)
Objective: To assess employment status of persons with young-onset
Parkinsons disease (PD) and the inuence of PD symptoms, severity,
and disability on employment.
Background: Employment status is impacted by PD. Loss of employment and employability in young-onset PD has not been described.
Methods: Persons who attended the 2006 National Parkinson Foundation Young Onset Network Conference were asked to complete
surveys distributed at registration. Surveys consisted of demographic
and clinical data, PD symptoms, current employment status, and selfreported Hoehn and Yahr stage (HYS), Activities of Daily Living
(ADL) of the Unied Parkinson Disease Rating Scale (UPDRS), quality of life (PDQ-39), Schwab and England (SE) disability scale, and the
Multidimensional Health Locus of Control (MHLC). The effect of
demographics, disease characteristics, degree of disability, and patients
locus of control beliefs on employment status were assessed.
Results: Of 118 surveys distributed, 85 were returned wth 8 excluded
due to PD onset after age 55. The remaining 77 had an average age of
50.6 years, with average onset age of 42.7. 34 (44%) were men. 44
(57%) had a HYS of 1 or 2, 32 (42%) a HYS of 3, and 1 (1%) of 4. 40
(52%) were still working with 9 (23%) having changed occupation
since the diagnosis of PD, and 16 (40%) making changes in their career
plans due to PD. Five of those working (13%) felt that PD did not affect
their job performance, while 18 (45%) had mild and 13 (33%) moderate
impact of PD on their job performance. Among the 37 (48%) patients
currently not working, 22 (59%) were on disability income, 7 (19%)
were retired, and 6 (16%) were unemployed. Twenty-nine were working at the time of PD diagnosis and continued to work for an average
of 3.9 years thereafter. Thirty-one (83%) felt that they had to stop
working due to PD related symptoms. Working patients had signicantly lower HYS, higher income, lower UPDRS-ADL scores, better
PDQ-39 scores in the mobility, ADL, social support, and communications domains, better SE scores, and higher MHLC scores compared to
non-working subjects.
Conclusions: Loss of employment is a frequent outcome in PD of
young onset. Patients who continue to work experience career plan
changes and reduced job performance due to PD. Employment status
correlates to disease severity, social support and an internal locus of
control.
608
Striatal dopamine transporter function and apathy in Parkinsons
disease
Z. Katsarou, S. Bostantjopoulou, G. Gerasimou, G. Kourtesi,
V. Tsipropoulou, A. Kafantari, E. Peitsidou (Thessaloniki, Greece)
Objective: Evaluation of apathy in Parkinsons disease (PD) and
correlation with [123]I-FP-CIT SPECT (DAT SPECT) measurements.
Background: Apathy is a frequent symptom in various degenerative
disorders including Parkinsons disease (PD) and although it is strongly
related to depression or dementia it is probably an independent non
motor symptom.It is attributed to either a direct lesion of the frontal
cortex or damage to frontal-subcortical circuits.Various hypothesis
implicate dopaminergic and non dopaminergic dysfunction in apathy
but its pathophysiology is still unclear.
Methods: Seventy consecutive PD patients were evaluated with DAT
SCAN as a diagnostic procedure.A subgroup of 43 PD patients non
demented ,non depressed ,was screened for apathy by means of an
abridged form of the Apathy Evaluation Scale (12 items; cut-off score
12) modied and adapted for the Greek population (AES).They were
also clinically evaluated by means of the Unied PD Rating Scale
(UPDRS) and classied in Hoehn and Yahr stages.All were under
treatment with levodopa or dopaminergic agonists.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S186 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Results: All patients had abnormal DAT SCAN ndings consistent
with parkinsonism.Their mean UPDRS score was 19.8 (SD 9.7) and the
mean stage 1.6 (SD 0.68). Mean AES score was 6.6 (SD 5.0).Seven
patients had signicant high AES score (16. 3%).Correlation between
AES scores and DAT SCAN measurements showed a negative correlation between AES score and radiologand uptake in the striatum and
caudate of the most affected side (r-.333, p.025 and r-.388,
p.008, respectively).However multiple regression analysis revealed
that reduced caudate radioligand uptake was the only predictor of
apathy (beta coefcient -.388, p .008).
Conclusions: Our ndings indicate that apathy is present in PD
patients even in the absence of depression or dementia.The inverse
relationship between AES scores and dopaminergic function in the
caudate indicates that dysfunction in non motor frontal-subcortical
circuits may play a signicant role in the pathophysiology of apathy.
609
Dementia and hyperhomocysteinemia in Parkinsons disease
M. Menendez, R. Ribacoba, G. Jimenez, J.R. Virgili, C. Huerta,
V. De la Vega (Mieres, Asturias, Spain)
Objective: Evaluate the presence of hyperhomocysteinemia as risk
factor for dementia in patients suffering from Parkinsons disease (PD)
treated with L-Dopa
Background: Hyperhomocystinemia is associated with Alzheimers disease.
Methods: We reviewed the records of 88 patients (30 men and 58
women) suffering from PD who underwent blood test to determine
plasma homocysteine level and the MTHFR gen polymorphisms. We
checked the presence of dementia after being put on L-Dopa treatment.
PD diagnosis was made according to the Brain Bank of London
Criteria. Normal homocysteine level was 12 milimol/L, high homocysteine level was 15 milimoles/L; between 12 and 15 it was
considered as undened. The mean time in treatment with L-Dopa was
84 months and the mean dose 650 mg.The presence of dementia was
assessed with the MMSE of Folstein, the seven minute screening test,
the Tower of Hanoi test and the trail making test.
Results: Dementia was present in 11 patients with hyperhomocysteinemia (29,5%) (polymorphisms: 5CC, 2TT, 4 CT, 21,05% patients
with undened homocysteine level (3 CT , 1CC) and 15,6% with
normal homocysteine level (4 CT, 1 CC).
Conclusions: Hyperhomocysteinemia is associated with dementia in
PD patients treated with L-Dopa.
610
Does gait analysis quantify the efcacy of PPN-DBS in Parkinsons
disease patients?
A. Peppe, A. Stefani, P. Mazzone, A. Gasbarra, M. Pierantozzi,
D. Crovato, C. Caltagirone, P. Stanzione (Rome, Italy)
Objective: To assess unequivocally the effects promoted by PPNDBS on Gait.
Background: Deep Brain Stimulation (DBS) is a well established
strategy to ameliorate advanced Parkinsons disease (PD) patients.
Since some axial PD signs as gait disturbances and freezing seem
poorly responder to STN-DBS, we have been looking to alternative
approaches and demonstrated the benecial effect of PPN-DBS on gait
(UPDRS subitems; Mazzone et al., 2005; Stefani et al., 2007). Aim of
this study is to evaluate the efcacy of PPN-DBS using the Gait
analysis as objective tool.
Methods: Six patients (mean age: 55.4 years) were bilaterally implanted in both STN and PPN. The Gait analysis was performed
postsurgery, at least one month after the switching ON the stimuli.Gait
analysis included an optoelectronic system (SMART system, BTS
Padova, Italy) to measure the three coordinates of retro-reective
markers. Six video cameras placed along an 8m walkway were used for
the study. The working volume (2 3x 6 m2) was calibrated by
sweeping it with the three markers wand provided. After three-dimensional (3D) calibration, the spatial accuracy of the system was less than
0.5 mm. For correct positioning of markers a common Davis protocol

Movement Disorders, Vol. 22, Suppl. 16, 2007

was used. Patients were evaluated in OFF-Ther/OFF-DBS (in CAPIT);

in OFF-Ther/ON-PPN-DBS; in OFF-Ther/ON-STN-DBS; in OFFTher/ON-STIM (PPN STN). Moreover the patients were evaluated
during stable antiparkinsonian therapy in ON-Ther/OFF-DBS; in ONTher/ON- PPN-DBS; ON-Ther/ON-STN-DBS; ON-Ther/ON STIM
(PPNSTN).
Results: Data from PD group were compared to a group of healthy
subjects matched for gender and age. All variables showed the effect of
PPN and STN in recovering the kinetic and kinematic alterations
detected in OFF-Ther-OFF-DBS, by reducing the percentage differences with healthy subjects. This reduction is particularly important
when both nuclei are simultaneously ON, and even more pronounced
when the PPN is added to STN and to levodopa therapy, thus suggesting that PPN may induce a better utilization of endogenous dopamine.
Conclusions: These data conm the impact of PPN-DBS in modulating the neuronal circuits of Gait and encourage further studies in
DOPA-resistant extrapyramidal disorders.
611
The effectiveness of cabergoline vs levodopa in early Parkinsons
disease: Results of a three year follow up
N.S. Oztekin, M.F. Oztekin (Ankara, Turkey)
Objective: Cabergoline is an ergoline derivative which has the potential for more continuous stimulation of dopaminergic receptors. The
aim of this study was to evaluate whether a more sustained dopaminergic effect of cabergoline would translate into delayed onset of motor
complications, compared with levodopa, in patients with newly diagnosed Parkinsons disease.
Background: Long term levodopa therapy is associated with the
development of motor complications. Dopamine agonists can reduce
the duration of off periods and the need for levodopa therapy while
improving motor impairment and only minimally increasing dopaminergic adverse events.
Methods: 50 patients were enrolled to the study who had early idiopathic Parkinsons disease (Hoehn and Yahr stages 1 to 3) and had
received no previous treatment with levodopa. Patients were randomly
assigned to receive either cabergoline (0.25 to 4 mg once daily) or
levodopa (125 to 625 mg/day) titrated over a maximum period of 24
weeks. Once the optimum or maximum tolerated dose was achieved, it
was maintained up to the end-point (development of motor complications
conrmed at 2 consecutive 3-month visits) or up to a minimum of 3 years
treatment. Open labelled levodopa was added in both treatment arms when
the improvement in motor disability [Unied Parkinsons Disease Rating
Scale (UPDRS) factor III] decreased below 30% vs baseline.
Results: Motor disability, decreasing UPDRS factor III scores and
factor II scores for activities of daily living improved in both arms of
treatment.The primary end point was the development of motor complications and it was signicantly less frequent in patients treated with
cabergoline compared to levodopa treated patients(12% VS
29%,p0.05).The relative risk of developing motor complications during treatment with cabergoline was more than 50% lower than with
levodopa. Serious adverse events, either drug related or not, were
slightly more frequent in cabergoline-treated patients (23 %)than in
those treated with levodopa (15%).
Conclusions: To conclude, our study revealed that in patients with early
Parkinsons disease, cabergoline is effective either as monotherapy or
combined with levodopa. Moreover, starting treatment with cabergoline
signicantly delays the development of motor complications.
612
Neuropsychological assessment of mild to moderate stage Parkinsons disease without dementia: Comparison with mild stage of
Alzheimers disease (AD) a preliminary study
Y.D. Kim, J.E. Kim, J.H. Kim (Daejeon, Republic of Korea)
Objective: We sought to characterize cognitive prole in PD without
dementia patients compared with early AD.
Background: Patients with Parkinsons disease(PD) also show a
wide spectrum of nonmotor manifestations due to varied patterns of

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

degeneration in dopaminergic (mesolimbic, mesocortical, retinal) and
nondopaminergic(e.g.,cholinergic, noradrenergic, serotonergic) neuronal systems. Cognitive dysfunction is common, ranging from subtle
disturbances in early PD to dementia in late PD. However, there were
few reports about cognitive dysfunction of mild to moderate stage PD
without dementia compared with early stage AD.
Methods: The PD group consisted of 38 patients (25 men and 13
women) with mild to moderate disease severity who were recruited from
the department of neurology, Konyang University Hospital. The neuropsychological battery test results (attention, memory, visuospatial function,
frontal executive function and language) of two subject groups (38 PD
patients, 30 AD and 10 Normal) were reviewed and analyzed. They were
all matched for age and education period. AD patients were recruited as
mild dementia(K-MMSE18) were selected.
Results: Compared with the normal control group and PD without
dementia, mild AD patients showed worse cognitive decline with
signicant memory dysfunctions and impairment on language(p
0.05). However, neuropsychological tests involved in visuospatial
function and frontal executive function revealed statistically signicant
worse score in PD without dementia compared with mild AD and
control(p 0.05).
Conclusions: Patients with mild to moderate Parkinsons disease
showed impaired visuospatial function and frontal executive function
compared with elderly control and early AD patients.Visual dysfunction and frontal executive dysfunction contributes to parkinsonism
disability such as postural instability, reduced spatial contrast sensitivity and bradyphrenia through its inuence on locomotion and bradykinesia.
613
Evolution of patients with Parkinsons disease chronically treated
with continuous subcutaneous apomorphine infusion. A multicenter study
P.J. Garcia Ruiz, M. Alvarez, J.A. Burguera, M. Calopa,
M. Carballo, A. Castro, J.R. Chacon, J. Hernandez Vara,
E. Lezcano, F. Miquel, P. Mir, J. Obeso, M.C. Rodriguez Oroz,
A. Sesar, J. Vaamonde, J.M. Arbelo (Madrid, Spain)
Objective: To study the evolution of patients with PD and severe
motor uctuations chronically treated with Continuous Subcutaneous
Apomorphine Infusion (CSAI).
Background: CSAI is an alternative for those patients with Parkinsons disease (PD) and severe motor uctuations.
Methods: We have reviewed data from 50 patients with PD (mean
age 6511,8, evolution of disease 14,45,9) and severe motor uctuations referred to 10 tertiary centers in Spain. These patients were
chronically treated (at least during three months) with CSAI and
tolerated the procedure without serious side effects. We compared
baseline data of these 50 patients (before CSAI treatment) with those
obtained from the last visit of each patient.
Results: The mean duration of CSAI was 16,214 months (58-3
months). We found a statistically signicant reduction in off hours,
according with self scoring diaries (6,93,6 vs 1,11,4 hours/day,
p0.0001); Total and motor UPDRS scores (for total scores:
74,618,8 vs 46,122,6, p0.0001; for motor scores 43,613,3 vs
27,914,7 p0.0001), dyskinesia UPDRS score (1,60,9 vs 1,10,7
p0.001) and equivalent dose of antidopaminergic drugs (1410541
vs 759499 mg of levodopa equivalent units p0.0001). 20/50 patients were treated with levodopa and CSAI, the remaining still needed
another antidopaminergic drug. 9/50 patients did not report any side
effect, the remaining had at least one side effect, including subcutaneous nodules (68%), hallucinations (36%), somnolence (26%) and confussional state (12%). No case of hemolytic anemia was detected.
Conclusions: Continuous subcutaneous apomorphine infusion is a
reasonable alternative for those patients with PD and severe uctuations poorly controlled with conventional treatment. Although several
side effects can be expected, most patients achieve important motor and
functional improvement.

S187

FIG. 1 (613).

614
Anti-apoptotic mechanisms by D2/D3 receptor agonist ropinirole
against rotenone-induced cell death in dopaminergic cell line
S. Chen, X.-J. Zhang, W. Le (Shanghai, China)
Objective: The objective of this study is to determine whether D2/D3
agonist ropinirole possesses anti-apoptotic property against oxidative
stress-induced cell death.
Background: Dopamine receptor D2/D3 agonists are effective drugs
for the treatment of Parkinson disease (PD). Several lines of evidence
suggest that D2/D3 agonists may also have neuroprotective effects but
the underlying mechanisms are not clear.
Methods: We applied human dopaminergic cell line SHSY-5Y
which was exposed to mitochondria complex I electron transport chain
inhibitor rotenone (1-20M) to establish apoptotic model. We pretreated the SHSY-5Y cells with ropinirole for neuroprotection. Hoechst
staining was used to detect apoptotic cells and western blot was used to
assess the expression of p-Erk, p-JNK, p-P38, cleaved caspase 9,
cleaved caspase 3, cleaved PARP, p-Akt, bcl-2 and HSP 70, which are
related with apoptosis.
Results: Ten M Rotenone induced cell apoptosis at high percentage
and increased the expression of p-P38, p-JNK, cleaved caspase 3,
cleaved caspase 9 and cleaved PARR. Pretreated with ropinirole at dose
of 100M signicantly inhibited cells apoptosis and suppressed the
activation of p-P38, p-JNK, cleaved caspase 3, cleaved caspase 9 and
cleaved PARR. In addition, expression of anti-apoptotic proteins such
as bcl-2, p-Akt and p-Erk but not HSP 70 were markedly elevated after
the treatment of ropinirole in a time (0.5h, 4h, 8h, 12h)- and dose (10,
50, 100M)-dependent manner.
Conclusions: Selective D2/D3 agonist ropinirole can protect
SHSY-5Y cells against rotenone induced apoptosis through inhibiting
the expression of apoptosis-related proteins (p-P38, p-JNK, cleaved
caspase 3, cleaved caspase 9 and cleaved PARR) and enhance the
expression of anti-apoptotic proteins (bcl-2, p-Akt and p-Erk).
615
Inuence of red and green laser lights on freezing of gait (FOG) in
persons with Parkinsons disease (PD)
M. Suteerawattananon, D.H. Rintala, E.C. Lai, J.G.G. Hou,
E.J. Protas (Houston, Texas, USA)
Objective: To investigate the effect of red and green light beams on
freezing of gait.
Background: Laser light has been shown to help overcome freezing
of gait in PD. However, inuence of color of laser light on freezing is
not known.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S188 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: Three participants with PD performed walking tests while
on and off antiparkinsonian medications. A cane with a green light
beam and another with a red light beam were used. The participants
performed a timed 360 turn, a 50-foot walk, and a provocation of
freezing test. Time and number of steps and freezes were recorded for
each test. Their gait speed, cadence, and step length were recorded by
using the GaitRite walkway (GAITRite, CIR Systems Inc., Havertown,
PA). Case I is an 84-year-old man, 7 years PD, UPDRS III (off/on;
30/22), H &Y III. He could not perform the 50-foot walk while off
medication. Case II is a 62-year-old man, 5 years PD, UPDRS III
(off/on; 35/26), H&Y III. Case III is a 68-year-old man, 16 years PD,
UPDRS III (off/on; 34/24), H&Y III. All subjects had a freezing of gait
problem rated as at least a 3 (occasional freezing) on item 14 (freezing
when walking) of the UPDRS.
Results: While off medication, the green light reduced freezing more
than the red light during the turn in 2 of 3 patients. Green light also
increased step length in 2 of 3 patients while off medication. While on
medication, the green light reduced the time to turn in 2 of 3 patients.
There was no effect of either color cue on the straight walk. Gait speed
was reduced with both color cues in 1 patient while off medication and
2 patients while on medication. One patient increased his step length
with both colors while off and on medications.
Conclusions: It seems that green laser light helped with freezing of
gait better than the red light, especially during turing. This study is
ongoing to further elucidate the efcacy and differences of the red and
green laser beams on freezing of gait.
616
Vascular parkinsonism: A case of lacunar infarction localized to
mesencephalic substantia nigra
A. Akyol, U.O. Akyildiz (Aydin, Turkey)
Objective: Vascular parkinsonism presents with a marked, rapidly
progressive bradykinesia of the lower extremities of sudden onset, with
little or no response to dopamine therapy. It is known that lacunar
infarctions in basal ganglia, diffuse ischemic lesions of subcortical
white matter and infarctions localized to the frontal lobe present with a
clinical picture of parkinsonism [2,3]. We thought that it would be
appropriate to present a case of vascular parkinsonism secondary to
lacunar ischemic lesion localized to the substantia nigra in the mesencephalon, since it is rarely encountered.
Background: An 80-year-old male case, with right hand dominance,
presented with slowness of movement, rest tremor of his left hand and
tremor of the lower limbs at upright position, particularly in the left leg.
The patient stated that the symptoms had begun suddenly. He reported
that the tremor of the lower limbs was most conspicuous at rest. On
neurological examination the cranial nerves were intact, and there was
no visual disturbance. Bradymimia was not evident. Resting tremor and
mild rigidity were observed in the left hand. Postural tremor was
present in the lower limbs, particularly in the left leg at standstill. The
patient was walking with short steps and had no arm-swing, particularly
on the left side. He was unsteady on turning and postural instability was
evident. Cranial MRI revealed a lacunar infarction localized to the
substantia nigra on the right side (Fig. 1).
Conclusions: The incidence of Parkinsonism after infarction in basal
ganglia varies between 9 and 38% [5]. It has been reported that the rate
of parkinsonism in lesions of the nucleus lentiformis is 25% [6].
Vascular lesions secondary to diffuse subcortical infarctions could
result in vascular parkinsonism; however, it could also present as
Biswangers disease without causing parkinsonism [7]. The association
of a single lesion located at the mesencephalon with vascular parkinsonism has been demonstrated in a recent report [11]. In our case, the
association between the localization of MRI lesion and the sudden
onset clinical picture is of particular interest. Involvement of this site
may be consistent with the clinical picture of parkinsonism, and such an
infarction specically targeting substantia nigra relating to parkinsonism is rarely encountered.

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (616).

617
The contribution of Jules Froment to the study of parkinsonian
rigidity
E. Broussolle, P. Krack, S. Thobois, J. Xie-Brustolin, P. Pollak,
C.G. Goetz (Lyon, France)
Objective: To demonstrate the contribution of Jules Froment to the
study of parkinsonian rigidity during the 1920s.
Background: Rigidity is commonly dened as a resistance to passive
movement in opposing muscle groups. In Parkinsons disease (PD), two
types of rigidity are classically recognized which may coexist, leadpipe
and cogwheel. Charcot was the rst to investigate parkinsonian rigidity
during the second half of the 19th century, whereas Negro and Moyer
described cogwheel rigidity at the beginning of the 20th century.
Methods: Jules Froment, a French neurologist from Lyon, investigated rigidity of the wrist at rest in a sitting position, as well as in stable
and unstable standing postures, both clinically and with myographic
recordings. We present his experiments which were conducted step by
step following a scientic approach.
Results: With Gardere, Froment described enhanced resistance to
passive movements of a limb about a joint that can be detected
specically when there is a voluntary action of another contralateral
body part. This has been designated in the literature as the Froments
maneuvre and the activation or facilitation test. In addition, Froment
showed that parkinsonian rigidity diminishes, vanishes, or enhances
depending on the static posture of the body. He proposed that in PD
maintenance stabilization of the body is impaired and that reactive
stabilization becomes the operative mode of muscular tone control. He
considered rigidication as compensatory against the forces of gravity. Froment also demonstrated that parkinsonian rigidity increases
during the Romberg test, gaze deviation, and oriented attention.
Conclusions: In their number, breadth, and originality, Froments
contributions to the study of parkinsonian rigidity remain currently
relevant to clinical and neurophysiological issues of PD.
618
Steady state administration of istradefylline does not affect the
pharmacokinetics of levodopa/carbidopa
N. Rao, K. Allenby, T. Uchimura, A. Mori, P. Chaikin (Princeton,
New Jersey, USA)
Objective: To evaluate the effect of a supra-therapeutic (80 mg QD),
multiple-dose regimen of istradefylline on the pharmacokinetics (PK)
of levodopa/carbidopa.
Background: Istradefylline is a novel adenosine A2A receptor antagonist under evaluation as adjunctive therapy in patients with Parkinsons disease taking levodopa/carbidopa.
Methods: The study was conducted as an open-label, single-center,
xed-sequence design. Twenty-four healthy adult subjects (17 men, 7
women; 18-55 years of age) each received a single dose of levodopa/
carbidopa (200 mg/50 mg) alone or after 14 days of 80 mg QD
administration of istradefylline. Serial plasma samples were collected

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

through 12 hours after administration of the levodopa/carbidopa dose
and were analyzed for levodopa and carbidopa with a validated highperformance liquid chromatography method that uses uorescence
detection. Plasma samples were collected at three time points to document istradefylline exposure but were not subjected to formal PK
analysis. PK analysis was conducted on levodopa and carbidopa using
non-compartmental methods. Relevant PK parameters were compared
using ANOVA, in which day was a xed effect and subject was a
random effect. Ninety-percent (90%) condence intervals (CIs) were
constructed on the ratio of least-square mean (LS mean) levodopa/
carbidopa PK parameters, with levodopa/carbidopa administration
alone as the reference treatment and levodopa/carbidopa in the presence of istradefylline as the test treatment.
Results: The administration of levodopa/carbidopa alone, istradefylline alone, and levodopa/carbidopa in the presence of istradefylline was
safe and well tolerated. Ratios of LS mean AUC and Cmax (test/
reference) were within 12% of each other, and corresponding 90% CIs
were within the bioequivalence window of 80% to 125%. Istradefylline
did not have an effect on the elimination of levodopa/carbidopa.
Conclusions: These data indicate that steady state istradefylline does
not affect the PK of levodopa/carbidopa, and the combination treatment
can be administered safely.
619
Accuracy of patient versus spousal reports of disability in Parkinsons disease
H. Tanji, A.L. Gruber-Baldini, K.E. Anderson, S.G. Reich,
P.S. Fishman, W.J. Weiner, L.M. Shulman (Baltimore, Maryland,
USA)
Objective: To compare the accuracy of subjective patient vs. spousal
reports of disability using physical performance measure ratings of
patient disability.
Background: Previous studies show that family members report
greater disability than patients, however it is unknown whether patient
or family reporting, more accurately reects disability.
Methods: 52 Parkinsons disease (PD) patients (HY I-IV) and their
spouses individually rated the patients disability with the Older Americans Resources and Services ADL subscale (OARS). Patients were tested
with 3 physical performance measures: the modied Physical Performance
Test (mPPT), Short Physical Performance Battery (SPPB), and Timed Up
and Go (TUG). The correlations between both patient and spousal disability ratings and performance measure scores were examined.
Results: Greater disability was reported by spouses than patients
(p0.033), particularly on dressing (p0.011), eating (p0.031), traveling (p0.046), preparing meals (p0.015), and doing housework
(p0.023). Performance on the mPPT showed better correlation with
spousal than patient reports (r-0.553 vs. -0.426). The SPPB and TUG
correlated similarly with both patient and spousal reports (r-0.583 vs.
0.558) and ( 0.337 vs. 0.322).
Conclusions: Greater disability was reported by spouses than patients
with PD. Spousal reports may be more accurate than patient reports, based
on results of one of the three objective performance measures. The mPPT
includes simulations of daily activities involving both upper and lower
extremity function (e.g. dressing, lifting, bending, and gait) while the
SPPB and TUG rely solely on gait and balance tasks. Therefore, observation of daily activities by the spouses may result in more accurate reporting
of disability on activities of daily living.
620
A comparison of MMSE to MoCA in identifying cognitive decits
in Parkinsons disease
C. Zadikoff, S.H. Fox, D.F. Tang-Wai, T. Thomsen, R.M.A. De Bie,
S. Duff-Canning, P. Wadia, T. Steeves, A.E. Lang, C. Marras
(Toronto, Ontario, Canada)
Objective: Determine if the Montreal Cognitive Assessment(MoCA)
is a more sensitive screening tool for identifying cognitive decits in
Parkinsons disease (PD) than the Mini-Mental State Examination(MMSE)

S189

Background: Twenty to eighty percent of PD patients will develop

dementia. Neuropsychological testing, the standard for assessment of
cognition, is time consuming and not widely available. The MMSE, a
convenient and popular screening instrument, often fails to detect early
cognitive decline. The MoCA is a rapid and easily administered screening tool developed for the detection of mild cognitive impairment(MCI), a stage prior to the development of dementia. Because it uses
more numerous and demanding tasks to assess domains frequently
affected in PD, it may be a better screening tool than the MMSE.
Methods: English speaking patients with idiopathic PD of at least 5
years duration and without signicant depression(score2 on UPDRS
I(Q1.3)) were assessed. All patients were examined in the ON state.
Items common to both tests were asked only once. The order of items
similar in both tests was alternated among subjects to avoid order
effects when comparing MMSE to MoCA scores.We measured the
proportion of patients who scored less than 26, a cutoff used in previous
studies, on either the MMSE or the MoCA.
Results: Eighty-eight subjects participated(mean age of 65, mean
disease duration of 9.5 years, and mean UPDRS III score of 20.7).
More subjects were classied as having MCI by MoCA(43%) than by
MMSE(11%)(p .000002). No subject without MCI by MoCA had
MCI by MMSE while 36% of those without MCI by MMSE had MCI
by MoCA(p .0001). The range and standard deviation of scores
was larger with MoCA(7-30, 4.26) than with MMSE(16-30, 2.55). A
pronounced ceiling effect was observed with MMSE(27 patients with
score of 30 on MMSE;4 with score of 30 on MoCA). Both MMSE and
MoCA scores correlated with UPDRS I Q1, but MMSE more so
(Spearman correlation coefcients -0.34vs-0.26).
Conclusions: Compared to the MMSE, the MoCA may be a more
sensitive tool to identify early cognitive impairment in PD. Additional
studies validating the MoCA in PD against formal neuropsychological
testing should be undertaken.
621
No sleep-dependent consolidation of an implicit learning task in
patients with Parkinsons disease
S. Wailke, M. Fehlau, G. Deuschl, J. Volkmann (Kiel, Germany)
Objective: To compare encoding and off-line (sleep dependent)
consolidation of an implicit learning task (ILT) in Parkinsons disease
(PD) patients and healthy controls.
Background: Sleep is necessary to consolidate newly acquired implicit knowledge. This has been proven using the serial reaction time
task (SRTT) in which a hidden ten number sequence is embedded in
two random number sequences. During the task performance the reaction time (RT) is shortened as a sign of implicit learning (IL). After
sleep the initial RT are equal or better than during the last sequences of
the rst day indicating overnight consolidation of IL. IL is impaired in
PD. Here we address the question whether this IL decit is related to
problems during the encoding and/or consolidation.
Methods: 10 patients with PD and 16 healthy age matched subjects
underwent a SRTT. Patients were tested after a levodopa challenge and
received levodopa over night. The next morning the SRTT was performed again under the same drug condition. Outcome measure was the
delta-power, as dened by the difference between the mean reaction
time in the random task and the mean reaction time of the last two
sequences of the SRTT. Another outcome measure was the difference
between the mean reaction time of the last two sequences on day one
and the two rst sequences on the second day. Analyses were made by
repeated ANOVAs.
Results: The learning success during encoding was equal in both
groups (12 % improvement of RT in the PD group, 14% in controls),
although the RT of the patients were generally slower than those of the
controls (see gure). In the control group the shortened RT at the end
of day 1 were retained on day 2. In contrast, PD patients worsened
signicantly in their RT from day 1 to day 2 (p0,018). The initial RT
at day 2 increased by 12% from the nal RT of day 1 and returned to
the baseline value (see gure).
Conclusions: Medicated PD patients and controls exhibit a similar
degree of learning during encoding. In contrast to controls, the PD

Movement Disorders, Vol. 22, Suppl. 16, 2007

S190 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

patients did not show consolidation of the ILT. The sleep related
memory consolidation in normal controls is known to depend on a
physiological sleep architecture with a certain amount of REM sleep. A
further analysis has to prove, whether sleep fragmentation and a reduced amount of REM periods in PD relate to the impaired consolidation.

urine volume per void and an increase in maximum detrusor pressure

for about 80 minutes after the drug administration (a short-term effect),
and a decrease in number of micturition, an increase in urine volume
per void and a decrease in maximum detrusor pressure for from 80 to
230 minutes after the drug administration (a long-term effect). High
dose administration caused more signicant change than middle dose
administration.
Conclusions: Anti-parkinsonian drug (apomorphine) has biphasic
effects on normal micturition reex time-course dependently. This may
account for the previously conicting reports on the effects of antiparkinsonian drugs on micturition.
623
Weight change in Parkinsons and Alzheimers disease patients
taking atypical antipsychotic drugs
O. Sitburana, S. Rountree, K. Dat Vuong, W.G. Ondo (Houston,
Texas, USA)

FIG. 1 (621).
622
The short- and long-term effects of a single dose of apomorphine on
micturition function in conscious and free moving rats
T. Uchiyama, R. Sakakibara, Z. Liu, T. Yamamoto, T. Ito, T. Hattori
(Chiba, Japan)
Objective: To investigate the short- and long-term effects of a single
dose of apomorphine, which is one of anti-parkinsonian drugs, on
micturition function in awake and free moving rats.
Background: The effect of anti-parkinsonian drugs on micturition
has been controversial, because previous reports showed that antiparkinsonian drugs aggravated and alleviated micturition disturbance in
patients with Parkinsons disease (PD), and accelerated and inhibited
micturition reex in experimental models. These studies did not, however, consider any changes in pharmacological characteristics with
given dose, time, and concentration, and these may account for the
conicting ndings.
Methods: Experiments were performed on adult male Sprague-Dawley rats. 7 days before studies, a polyethylene tube was inserted into the
bladder. 3 days before studies, animals were attached on harness with
external tube, and kept in metabolic cages. Number of micturition,
urine volume per void, and cystometrograms were measured continuously. A single dose of apomorphine (low dose: 0.01, middle dose:
0.05, high dose: 0.5 mg/kg) or saline was given subcutaneously, and
recording was continued for over 4 hours after drug administration.
Results: In saline-administrated control rats, the number of micturition, urine volume per void, and cystometrograms almost unchanged
during the study. In apomorphine-administrated rats, a low dose induced a decrease in number of micturition, an increase in urine volume
per void and a decrease in maximum detrusor pressure. A middle and
high dose induced an increase in number of micturition, a decrease in

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To determine body weight and body mass index (BMI)

change in Parkinsons disease (PD) taking atypical antipsychotic (AA)
drugs compared to PD controls and Alzheimers disease (AD) patients
on AA.
Background: Weight loss in PD is a chronic process that is observed
in both early and late stages of PD. Atypical antipsychotic drugs are
associated with marked weight gain and new onset diabetes mellitus
(DM). This may result in increased cardiovascular morbidity and
mortality.
Methods: Sixty-six eligible consecutive PD subjects started on AA
who had accurate (same scale) weights for more than 6 months prior to
initiate AA and more than 6 months after, with up to 3 years when
available. We compared weight change in these PD subjects before and
after AA use, against a control group of sixty-one sex matched PD
subjects, and against twenty-eight AD subjects taking AA. We tabulated demographic information, Hoehn and Yahr stage, UPDRS part I.2
(thought disorder), L-dopa and dopamine agonist dose equivalent and
AA dosage. A linear regression model was created to compare weight
change in PD subjects before and after starting AA, against PD controls, and against AD on AA adjusted for age, sex, height and observational duration.
Results: Fifty-nine PD subjects had complete data; quetiapine (n
53, 89.8 %) and clozapine (n6, 10.2%). The mean actual weight and
BMI loss in the period before starting AA was 0.00 kg/month, 0.00
kg/m2/month over a duration of 1.95 1.41 years. After starting AA
subjects lost 0.10 kg/month and 0.03 kg/m2/month over 1.44 0.8
years. (p 0.001) In 61 PD controls, the mean weight and BMI loss
were 0.02 kg/month and 0.01 kg/m2/month for follow-up of 4.14
2.71 years (p 0.009, PD on AA vs. PD controls). The 28 AD on AA
gained 0.02 kg/month and 0.01 kg/m2/month over 1.491.07 years
(p 0.001, PD on AA vs. AD on AA). There were no documented
cases on new onset DM in PD subjects.
Conclusions: AA did not cause weight gain in PD population. This
should not be a concern when administering these medications. Conversely they are not effective as weight gaining drugs when this is
desired. The different effects in PD and AD suggest uniquely altered
weight homeostasis in PD, perhaps due to degeneration of histaminergic cells.
624
Molecular screening of the LRRK2 and parkin genes in a large
cohort of Russian patients with Parkinsons disease
S.N. Illarioshkin, S.A. Klyushnikov, P.A. Slominsky, M.I. Shadrina,
G.K. Bagyeva, E.V. Bespalova, T.B. Zagorovskaya, E.D. Markova,
S.A. Limborska, I.A. Ivanova-Smolenskaya (Moscow, Russian
Federation)
Objective: To estimate the frequency of mutations in the LRRK2 and
parkin genes in patients with predominantly sporadic and familiar cases
of Parkinsons disease in Russian population.
Background: Parkinsons disease (PD) is a progressive age-dependent neurodegenerative disease. At present, 11 chromosomal loci

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

linked to familial PD and eight causative genes have been identied.
Among them, parkin and LRRK2 genes seem to be most frequently
involved in the development of PD.
Methods: We performed mutation screening of the LRRK2 and
parkin genes in a consecutive series of 345 Russian PD cases, including
331 sporadic and 14 familial (autosomal dominant) cases. Patients with
juvenile parkinsonism (i.e. age at onset before 20 years) were excluded
from analysis. We analyzed a common mutation G2019S in the LRRK2
gene and parkin gene rearrangements (exonic deletions and multiplications) using a real-time TaqMan PCR system which allows to evaluate quantitatively parkin dosage.
Results: Of the 345 patients screened, we found four with heterozygous G2019S mutations: three G2019S carriers did not have a family
history of PD and one case was familial. Thus, in our series the
frequency of the LRRK2 G2019S mutation was 0.9% among the
sporadic patients and 7.1% among familial PD. Screening for the
parkin gene rearrangements showed heterozygous exonic deletions or
duplications in 5.8% of patients in the total cohort, including 10.7%
patients with early-onset PD (before 45 years) and 1.7% patients with
late-onset PD. One sporadic patient with severe and early PD was
found to be a double mutant carrying both the LRRK2 G2019S and
heterozygous duplication of the parkin exon 5.
Conclusions: Identication of mutations in the LRRK2 and parkin
genes in a subset of PD patients without an overt family history has
notable implications for genetic counseling.

625
The SCOPA motor scale in Latin-America: Metric properties
M.J. Forjaz, F.J. Carod, J. Virues, G. Galeano, G. Meza-Rojaz,
C. Velazquez, F. Micheli, G. Reina, M. Serrano-Duenas, B. Calero,
P. Martinez-Martin (Madrid, Spain)
Objective: To study the metric properties of the SCOPA motor scale
(S-MS) in a cross-national sample of Parkinson disease (PD) patients.
Background: As a new UPDRS version is currently being developed
by the Movement Disorders task force, the SCOPA motor scale
(SCOPA-MS) offers a valid, reliable and shorter alternative. Up to date,
there are only two known validation studies of the SCOPA-MS, both
performed with European samples.
Methods: This study used a cross-sectional, one-point in time
assessment design. The study sample was 323 PD patients (mean
age63.18.5, 54.1% males), from 4 Latin-American countries:
Argentina (n61); Brazil (n132); Ecuador (n60); and Paraguay
(n70). The neurologist-based assessments were: Hoehn and Yahr
stage (HY); Clinical Impression of Severity Index for PD (CISIPD); comorbidity questionnaire (CIRS-G); and S-MS. Several metric characteristics were analyzed for each of the S-MS sections
(Examination, Disability-ADL and Complications): acceptability,
scaling assumptions, internal consistency, precision and discriminative validity.
Results: The S-MS lacks oor and ceiling effects (0.17-0.93%)
except for Complications (oor effect: 37.46%). Skewness was appropriate (0.53-0.71). All items expect two (right hand rest and postural
tremor) showed good item-total corrected correlation coefcients
(Spearman r0.37-0.80). Cronbachs alpha was 0.91 for ADL and 0.88
for the other two sections. Homogeneity (inter-item correlation mean)
was 0.33-0.64 and SEM ranged from 1.07 to 2.62. The S-MS sections
displayed tight correlations with CISI-PD (r0.66-0.82, p0.05) and
HY (r0.52-0.78, p0.05); moderate correlations with PD duration
(r0.41-0.58, p0.05); and low correlations with CIRS-G (r0.090.22). S-MS scores were signicantly related to HY stage (see Figure)
in a dose-dependent fashion (Kruskal-Wallis, p0.001).
Conclusions: The results of this study are in line with previous ones,
showing that the S-MS displays satisfactory metric characteristics,
stable across different socio-cultural contexts.

S191

FIG. 1 (625).

626
Medication review in patients on anti-parkinson medication in the
community
E.J. Newman, K. Grosset, E. Thomson, M. Liddle, D. Grosset
(Glasgow, United Kingdom)
Objective: To dene the pattern of medication intake for Parkinsons
disease (PD) in a community setting.
Background: Therapy choice in PD is inuenced by patient age
and co-morbidity, physician preference and tolerability. An additional factor may be the type of clinical service that the patient
attends.
Methods: Prescribing databases and case records in Primary Care
were searched to identify all patients on anti-PD medication. Current
and previous prescribed drugs, and out-patient clinic attendance were
recorded. Patients on anti-parkinson therapy for alternative diagnoses
(eg. pituitary tumour, restless legs syndrome) were excluded. Dosage as
levodopa equivalents was calculated by standard formula. Data are
presented as median (interquartile range, IQ).
Results: In 33 general practices (population 207,677) there were 335
patients on therapy for PD (187 male, 148 female) giving a prevalence of
161 per 100,000 and an incidence of 20.7 per 100,000. Patient age was
76.8 years (IQ 71.6-82.9) and age at time of diagnosis was 71.6 years (IQ
65.0-77.8). Daily medication intake was 330 levodopa equivalents (IQ
200-520). 203 patients (60.6%) attended a specialist PD clinic, 112
(33.4%) did not attend a specialist PD clinic, and clinic attendance was
unknown in 20 patients (6.0%). There was no signicant difference in
patient age, disease duration, or levodopa equivalents intake between those
attending and not attending specialist clinics. More patients not attending
specialist clinics were on monotherapy (92.0% of 112 versus 49.3% of
203, p0.0001). More patients not attending specialist clinics were treated
with levodopa (95.5% of 112 versus 82.8% of 203, p0.001). More
patients attending specialist clinics were treated with dopamine agonists
(39.9% of 203 versus 8.9% of 112, p0.0001).
Conclusions: In the area studied, a large proportion of patients on
therapy for PD do not attend specialist clinics. There was evidence that
geography and service availability affected specialist clinic attendance.
The variation in drug therapy approach in the two clinical settings,
despite similar age and disease duration, merits further evaluation.

Table 1
Number of
Patients
Male

187

Female

148

Total

335

Patients attending
clinics
Patients not attending
clinics

203
112

Age, years
76.9 (71.883.4)
76.3 (71.381.7)
76.8 (71.682.9)
76.8 (71.583.3)
77.5 (72.081.9)

Age at
diagnosis, years

Daily dose in levodopa

equivalents

71.6 (66.4-79.0)

300 (200-520)

71.6 (64.2-77.0)

400 (200-520)

71.6 (65.0-77.8)

330 (200-500)

70.5 (62.5-79.0)

315 (200-520)

72.0 (64.2-77.4)

300 (200-520)

Data are presented as: median (interquartile range)

Movement Disorders, Vol. 22, Suppl. 16, 2007

S192 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Table 2

Attending specialist clinic

(n203)
Not attending specialist clinic
(n112)
Total (n315)

Patients on
monotherapy

Patients on
levodopa

Patients on dopamine agonist

therapy

100 (49.3%)

168 (82.8%)

81 (39.9%)

103 (92.0%)
225 (67.2%)

107 (95.5%)
275 (82.1%)

10 (8.9%)
96 (28.7%)

627
Transcranial sonography (TCS) of the substantia nigra (SN)
biological and clinical signicance in patients with Parkinsonian
syndromes (PS)
D. Weise, R. Lorenz, M. Schliesser, K. Reiners, J. Classen
(Wuerzburg, Germany)
Objective: To assess the biological and clinical signicance of hyperechogenicity of the SN in PS as revealed by TCS.
Background: Abnormal spatial extension of SN-echogenicity as assessed by TCS has been found previously in idiopathic Parkinson
disease (IPD). Spiegel et al. (1) failed to detect correlation of SN-area
to striatal activity of pre-synaptic dopamine reuptake transporters as
assessed by Single-Photon-Emission-Computed-Tomography (SPECT)
suggesting that SN-echogenicity may be sensitive to pathomechanisms
different from degeneration of SN dopaminergic projection neurons.
Methods: The area of the echogenic SN-signal was measured by
TCS in 30 patients clinically diagnosed with IPD (5913 y), 27 with
atypical PS (APS; 648 y) and 10 with clinically confounding disease
(CCD; 6016 y). SN-area 0.20 cm2 was considered abnormal. 45
patients underwent both TCS and I-123-2-beta-carbomethoxy-3-beta(4-iodophenyl)-tropane (-CIT) SPECT. In 22 of these and in an
additional 28 patients post-synaptic D2-dopamine receptor density was
assessed using I-123-Iodobenzamid (IBZM). Radiotracer uptake was
assessed quantitatively in bilateral striatum.
Results: TCS could be evaluated bilaterally in 57 of 67 patients. In
these, SN-area was abnormally extended in 21 of 27 (78%) IPDpatients, 11 of 23 (48%) APS, and 3 of 7 (43%) CCD (P0.056).
Overall, SN-area was signicantly correlated with -CIT uptake in
ipsilateral putamen (r-0.253; P0.037; linear regression analysis). In
a subgroup analysis, this correlation was absent in APS (N8;
r0.116; P0.670). -CIT-SPECT was abnormal in all patients with
PS. In IPD, IBZM-SPECT was abnormal in 1 of 14 (7%), and in APS
in 18 of 20 (90%). In PS, abnormal SN-area increased the positive
predictive value of a normal IBZM-SPECT to diagnose IPD from 87 to
90%.
Conclusions: Correlation of SN-area with radioimaging of pre-synaptic dopamine re-uptake does not support the notion that these methods assess pathophysiologically different phenomena. In the presence
of post-synaptic dopaminergic dysfunction, -CIT-SPECT may not
provide reliable information about degeneration of pre-synaptic neurons. TCS appeared to be less sensitive and less specic to the diagnosis of IPD than previously reported.
References:
(1) Brain 129, 1188 1193, 2006.
628
Impaired information processing as a key determinant of akinesia
in Parkinsons disease
B. Christe, C.-A. Hauert, A.J. Pegna, P.R. Burkhard (Geneva,
Switzerland)
Objective: This study focused on a detailed, instrumental assessment
of akinesia, one of the most complex and invalidating motor features of
Parkinsons disease (PD).
Background: Because motor repertoire is highly diversied, we
believe that only a comprehensive analysis of motor processes underlying any overt motor response is likely to provide accurate information
regarding global motor functioning.
Methods: We evaluated PD patients (N28) and control (CTRL)
subjects (N56) using a digitizing tablet-based instrument with distinct

Movement Disorders, Vol. 22, Suppl. 16, 2007

quantitative measurement levels. Temporal, spatial and sequential constraints underlying the experimental conditions allowed the intricate
motor processes of planning, programming and execution to be dissociated.
Results: Our data suggest that the diversied aspects of akinesia,
including bradykinesia, hypometria and altered sequential movements,
can be specically measured. Analyses of movement time in a typical
speed-accuracy trade-off setting showed a signicant slowing of PD
patients movements compared to CTRL subjects, conrming the typical parkinsonian bradykinesia. Results concerning constant error show
a general tendency toward target center overshoot and a relative, but
not absolute, hypometria, with a signicantly weaker overshoot tendency in PD patients compared to CTRL subjects. Sequencing difculties associated with PD were exemplied by a signicant difference
of effective capacity between CTRL subjects and PD patients in the
planning condition and was found to be the best discriminatory factor
between both populations as demonstrated by the largest area under the
curve in the receiver operating characteristics (ROC) analyses. In
addition, the effective capacity in the planning condition was the only
data showing, with our limited population samples, a signicant difference between early PD (N11) and advanced PD (N17). Finally,
we found that the rate of failure in the planning condition was as high
as 36% for PD patients, with 18% for early and 47% for advanced PD,
compared to a rate of 2% only in the CTRL subjects.
Conclusions: These results strongly highlight the pivotal role of
altered planning processes, and therefore impaired information processing, at the basis of akinesia in PD.
629
Efciency of physiotherapy in Parkinsons disease: The ParkNet
trial
M. Munneke, M.J. Nijkrake, S.H.J. Keus, I.I.K. Lim, G.F. Borm,
G.J. van der Wilt, R.A.C. Roos, G. Kwakkel, H.W. Berendse,
B.R. Bloem (Nijmegen, Netherlands)
Objective: To evaluate the efciency of physiotherapy care in Parkinsons disease (PD).
Background: Physiotherapy according to evidence-based guidelines
may be effective in improving mobility decits in PD. We developed
a multifaceted ParkNet strategy aiming to implement the guideline
into clinical practice. ParkNet is a regional network of physiotherapists
and neurologists. The physiotherapists are continuously educated in
treating patients according to the guidelines. The neurologists are
instructed to refer PD patients to dedicated ParkNet therapists, according to objective referral criteria as dened in the guidelines. The
efciency of this ParkNet strategy is evaluated in the ParkNet Trial.
Methods: We designed a cluster-randomised trial in which 16 general hospitals were allocated to either ParkNet or Usual Care. In all
eight ParkNet clusters a ParkNet was implemented. We aimed to
include 50 patients for each cluster. Inclusion criteria were idiopathic
PD, Hoehn & Yahr stage 1 to 4, able to speak Dutch, no severe
co-morbidity and living independently in the community. Patients were
included two weeks before a routine outpatient visit to their neurologist
and were measured at home (week 0 and 16) and by mail (week 8 and
24). The MACTAR-PD (a PD-specic Patient Preference rating scale)
was used as primary outcome measure. Secondary outcome measures
included the PDQ-39, costs, falls, and the quality of physiotherapeutic
care. Tertiary measures included health benets within all domains of
the guideline and satisfaction of patients, therapists and neurologists.
Results: Regional ParkNets were successfully implemented in eight
clusters. PD specic knowledge and competence of participating therapists increased signicantly (P 0.05). After screening 3543 patient
records, 1256 patients were found to be eligible for inclusion, and were
invited to participate. A total of 708 patients gave informed consent to
the study (gure 1). No important differences were found between the
clinical proles of both groups.
Conclusions: Implementing ParkNet into everyday care of regional
hospitals is feasible. We are now awaiting the results of the ParkNet
trial (available in 2008) in order to evaluate the efciency and health
benets of this strategy.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

S193

FIG. 1 (629).
630
Frequency and phenomenology of motor and non-motor features
of wearing-off phenomenon in Brazilian Parkinsons disease patients
F. Cardoso, B.P. Cesar, A.L. Teixeira, Jr, S.T. Camargos,
D.P. Maia, M.C. Cunningham (Belo Horizonte, MG, Brazil)
Objective: To evaluate the frequency and phenomenology of wearing off phenomenon in a cohort of Brazilian Parkinsons disease (PD)
patients.
Background: Dopaminergic therapy-related uctuations are characterized by emergence of PD symptoms and signs before the next dosage
of the medication. Wearing off is a predictable uctuation consisting of
shortening of duration of action of the medication. Fluctuations are
thought to be common although the reported frequency vary according
to many factors. Recent data show that the phenomenology of uctuations include motor and non-motor features and a signicant proportion of uctuations remain unrecognized by physicians (Neurology
2002;59:408, Mov Disord 2005, 20:726). This important aspect of PD
has been little studied in non-caucasian populations.
Methods: We performed a structured interview of 83 consecutive PD
patients on dopaminergic (levo-dopa and/or dopamine agonists) followed up at the UFMG Movement Disorders Clinic. The interview

used a modied version of EODWO, selecting 19 of the most sensitive

items of the original 32 questions developed by Stacy et al (Mov Disord
2005, 20:726).
Results: All patients of the study, except for 6 on agonist (5,
pramipexole; 1, bromocriptine) monotherapy, were on levodopa.
Fluctuations were identied in 78 (94%) of patients, including 4/5
on agonist monotherapy. The percentage of patients with uctuations of the screened features is: tremor 78.3%, difculty in speech
49.3%, tiredness 36.1%, balance problems 55.4%, slowness of
movement 55.4%, reduced dexterity 40.9%, general stiffness
51.8%, muscle cramping 25.3%, difculty in getting out the chair
56.6%, anxiety 45.7%, mood changes 39.7%, panic attacks
7.2%, slowness of thinking 37.3%, abnormal sensations: numbness 27.7%, abdominal discomfort 8.4%, abnormal sensations:
hot/cold 9.6%, pain 30.1%, abnormal sensations: aching 9.6%,
sweating 8.4%.
Conclusions: Similarly to North American and European PD patients, in our non-caucasian population seen in a tertiary center, uctuations are common both in levodopa- and dopamine agonist-treated
patients. Motor symptoms are the most common features of uctuations
but cognitive, behavioral, sensory and autonomic manifestations are
also very common.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S194 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

631
Postural instability in persons with Parkinsons disease: Comparison among disability scales of the Hoehn and Yahr
M. Suteerawattananon, S. Kamolsawat, V. Hiengkaew, C. Akamanon,
N. Poungvarin (Houston, Texas, USA)
Objective: To compare postural instability in individuals with PD
among the three Hoehn and Yahr (H&Y) disability stages on and off
medications and to age-matched controls.
Background: Postural instability is one of the common clinical
hallmarks in individuals with PD. Little is known about differences in
postural instability in each stage of the disease, its responses to medications, as well as its comparison to age-matched controls.
Methods: Twenty-seven individuals with PD (age 65.0 1.73 years)
and nine healthy elders (age 62.2 3.67 years) participated. Nine
subjects with PD were in each stage of the H&Y. Static and dynamic
balance were measured with posturography (Neurocom International
Inc, Clackmas, OR, US). Static balance was measured with the Sensory
Organization Test including average sway velocity, percentage of maximum stability, and percentage of ankle strategy. Dynamic balance was
measured with the Limit of Stability Test including reaction time,
movement velocity, and endpoint excursion. Subjects with PD were
measured both on and off antiparkinsonian medications.
Results: No differences were found between stage I and controls on
static balance variables. For dynamic balance, persons with stage I
showed slower movement velocity to the left both off and on medications and lower endpoint excursion to the left-front while off medication than controls. When comparing static balance among the stages
while off medication, those with stages I or II maintained static balance
by integrating visual, proprioceptive and vestibular information better
than stage III. For dynamic balance, persons with stage III had slower
movement velocity in one direction than stage I. Reaction time and
endpoint excursion were not different among the three stages. After
taking medications, persons with stage III increased average sway
velocity, decreased percentage of maximum stability and decreased
percentage of ankle strategy. For dynamic balance, stage III improved
reaction time and movement velocity after taking the medications.
Conclusions: Persons with Stage I PD showed dynamic balance
impairment when compared to age-matched controls, but not static
balance. Antiparkinsonian medications improved movement velocity
and reaction time in several directions in persons with stage III.
632
Biopsychosocial needs of people living with PD and their caregivers
S. Giles, L. Johnston, C. Marras (Toronto, Ontario, Canada)
Objective: To identify and describe the biopsychosocial needs of
patients (PT) with PD and their caregivers (CG) at a tertiary care
movement disorders centre.
Background: In order to provide better comprehensive clinical care
we assessed PT and CG biopsychosocial needs.
Methods: A questionnaire assessing biopsychosocial needs related to
PD was self-administered by consecutive PT and CG pairs at the
movement disorders center.
Results: PT: N148 CG: N119 Gender: CG: 40% men, PT 57%
men. Mean Age: CG 60.3 years, PT 65.8 years. Mean Disease Duration: 7.7yrs. Preferred Service Medium 56.1% of PT and 37.5% of CG
reported more support services were not needed at this time. The
support service medium requested most commonly by PT were support
groups (15.8%) and home based physical help (13.7%). CG prefer
educational pamphlets (20.5%), home based physical help (15.2%).
Information Needs: 40.4% of PTs and 37.5% of CGs felt they did not
require information at this time. PT want medication information
(29.1%), updates on latest research (28.4%). CG want information on
stress management (26.8%), medication information (24.1%). Need for
Increased Clinical Care: 43.5% of PT and 38.5% of CG felt they did not
need an increase in clinical care. PT would benet from more physiotherapy (41.3%); physician time (18.8%) and speech therapy (17.4%).
CG feel PT would benet with more: physiotherapy (45.9%), psychosocial counseling (22.0%) and physician time (16.5%). Qualitative

Movement Disorders, Vol. 22, Suppl. 16, 2007

data: Main theme: CG experience a restriction of many daily activities.

Minor theme: being a CG was seen a normal part of life, but it did not
seem to improve quality of their life. PT want healthcare services for
them and their CG extended beyond medical to include allied health
care.
Conclusions: PT and CG needs and preferred service media differ,
illustrating the need to ask both patients and caregivers about their
needs. However, both PT and CG would like more medication-related
information and home-based physical support. Physiotherapy was most
commonly stated as an unmet need in clinical care by both patients and
caregivers. A self-administered questionnaire is a feasible and useful
method to understand the unmet needs of patients and caregivers, and
can serve as a basis for improving care for patients with PD. The results
of this study will be used to advocate for better access to comprehensive biopsychosocial care for PT and CG.
633
Socio-demographic characteristics of the caregivers of patients
with Parkinsons disease
P. Martinez-Martin, S. Arroyo, B. Frades, J. de Pedro, The ELEP
Group (Madrid, Spain)
Objective: To analyze the socio-demographic characteristics of the
caregivers looking after patients with Parkinsons disease (PD).
Background: There is interest in knowing which factors are inuencing the burden and quality of life of people caring of PD patients.
However, there is a lack of studies focused on the characteristics of
caregivers as a population group.
Methods: Socio-demographic data were collected from PD patients
and respective caregivers taking part in the ELEP study (an observational, multi-centre, longitudinal study). Included variables were: age,
sex, years of study, habitat, marital status, and work status. In addition,
number of years caring of the patient, their relationship, rotation of
care, and antidepressant and ansiolitic medication were recorded.
Results: 289 caregivers were studied. In the patients group, 53.8%
were men, 64.910.9 years of age, and 9.95.6 years of study.
Duration of PD was 7.58.8 years and 24.1% were in moderate or
advanced stages; 63.1% were retired, and 96.6% were living at home.
Concerning the caregivers, 66.2% were women (p0,0001),
59.313.6 years old (p0.0001), with 10.75.8 years of study
(p0.03); 75% were spouses and 14%, children of patients; 85.2%
were living with the patient and only 3.1% of them did care rotation;
31.7% were working full-time and 5.5% part-time; 29.7% were retired.
Patients were signicantly more depressed than their caregivers
(5.80.2 vs. 4.50.2; p0.0001), but no signicant difference was
found for anxiety. 10% of caregivers received antidepressant and 14%
anxiolytic treatment. EuroQoL tariff and perceived health status were
signicantly worse in patients, but caregivers tariff was signicantly
lower than normative mean value for Spanish population. Factors
linked to disease severity (e.g., duration of disease) and caregiver mood
disorder were related to caregivers burden.
Conclusions: Most of caregivers were patients wives, without employment and no care rotation. Caregivers perceived health status was
impaired and their mood disorders increased the burden.
634
Handwriting in Parkinsons disease: The effects of disease severity
and acute levodopa dosing
M. Contin, P. Martinelli, R. Shrairman, C. Scaglione, A. Landau,
F. Albani, R. Riva, A. Baruzzi (Bologna, Italy)
Objective: To assess disease severity and acute levodopa dosing
effects on handwriting dynamics in patients with Parkinsons disease
(PD) by a new biometric instrument for quantitative measurement of
ne motor control (NeuroskillTM).
Background: Handwriting is commonly affected in PD patients. The
main impairments include a shift from an automatic to a controlled
pattern of movement generation, leading to slower handwriting movements and micrographia. Previous studies on the effects of disease

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

severity and dopaminergic therapy on handwriting in PD patients have
given conicting results.
Methods: Fifty-seven patients (22 women, 35 men), aged 35-79
years, H&Y stage 1-4, and 13 healthy volunteers enrolled in the study.
Each patient was examined with the NeuroskillTM device before levodopa dosing (8 a.m.) and at hourly intervals up to 3 hours after the
intake of a fasting dose of levodopa/benserazide (100/25 mg). The
controls underwent three repeated examinations at 1 hour intervals.
NeuroskillTM consists of an instrumented pen attached to a computer,
and a software package for motor control analysis. The device translates the handwriting dynamics of acceleration and pressure into digitized data, every sample being taken in 2.5 msec intervals. Five samples
of handwriting signature were collected per patient and control at each
repeated trial. Criterion of stability (reproducibility) of movements
was chosen as the main outcome variable. Patients motor response to
acute levodopa dosing was also evaluated at xed times by the nger
tapping test.
Results: Basal, pre-levodopa stability values of handwriting signicantly decreased with the advancing of H&Y stage (r - 0.347, p
0.007); basal average handwriting stability values in each patient group
were signicantly lower compared with controls (p0.001). A statistically signicant (p0.02) positive effect of acute levodopa dosing on
handwriting stability has been appreciated in patients of groups II and
III, while in groups I and IV it was less clear-cut.
Conclusions:
Objective assessment of handwriting dynamics by
TM
Neuroskill seemed suitable to detect impairment of ne motor skill
also at the early stages of PD and might be useful in the evaluation of
acute levodopa effects.
635
Continuous and low-dose infusion of erythropoietin into the brain
shows neuroprotective effect and induces neurogenesis without
hematogenesis in the parkinsonian rat model
A. Kondo, T. Shingo, T. Kadota, T. Yasuhara, Y. Miyoshi, T. Matsui,
I. Date (Okayama, Japan)
Objective: To examine whether intracerebroventricular continuous
and low-dose infusion of erythropoietin (EPO) is useful for the treatment of Parkinsons disease (PD) without any side effects.
Background: PD is a neurodegenerative disorder characterized by
the death of midbrain dopaminergic (DA) neurons. Meanwhile, EPO,
the principal regulator of erythroids progenitor cells, has been shown to
be neuroprotective against cerebral ischemia and brain injury in rodents. However, we have little information available about protective
effect of EPO on nigral DA neurons in rat model of PD.
Methods: 6-hydroxydopamine (6-OHDA), the DA neurotoxin, was
injected into the striatum of rats. Thirty minutes later, either EPO
(n6) or vehicle (n6) was continuously infused into the ipsilateral
lateral ventricle with a osmotic infusion pump for a week. Amphetamine-induced rotational behavior was investigated one week and one
month after 6-OHDA lesioning. The rats were sacriced a month after
pump infusion and their brain tissue were exmamined immunohistochemically.
Results: There were no signicant differences between EPO-treated
or control rats in hematocrit value and hemoglobin concentration.
EPO-treated rats signicantly reduced behavioral asymmetries compared to control rats. In immunohistochemical analysis, EPO-treated
rats showed the remarkable protection of the tyrosine hydroxylase (TH)
positive bers in the striatum and TH-positive neurons in the substantia
nigra (SN) than the control. The number of BrdU/PSA-NCAM positive
cells in the subventricular zone of EPO-treated rats increased and some
of those cells migrated toward the lesioned striatum. Additionally, the
EPO treatment induced the decrease of the number of TUNEL positive
cells and increase of phospho-Akt positive cells in the ipsilateral SN to
the lesioned side.
Conclusions: These results demonstrate that local and low-dose
infusion of EPO prevents the apoptosis through the Akt signaling in the
DA neurons and can be a neuroprotective agent without side effects.

S195

636
Correlation between depressive symptoms and nocturnal disturbances in Japanese patients with Parkinsons disease
K. Suzuki, Y. Okuma, N. Hattori, S. Kamei, F. Yoshii, H. Utsumi,
Y. Iwasaki, M. Iijima, T. Miyamoto, M. Miyamoto, K. Hirata (Mibu,
Tochigi, Japan)
Objective: The aim of this study was to determine the correlation
between depressive symptoms and nocturnal disturbances in patients
with Parkinsons disease (PD) in Japan.
Background: Depression and nocturnal disturbances are frequent in
patients with PD.
Methods: 188 PD patients (85 men and 103 women, 66.48.7 years)
and 144 age matched controls (64 men and 80 women, 65.16.8 years)
were included. The study was carried out between April 2005 and
December 2005 at eight university hospitals (Dokkyo Medical University, Juntendo University Shizuoka Hospital, Juntendo University
School of Medicine, Nihon University School of Medicine, Tokai
University School of Medicine, Tokyo Medical University, Toho University Omori Hospital, Tokyo Womens Medical University) and
afliated facilities in the Kanto area of Japan. This multi-center crosssectional study was performed using semi-structured questionnaires
which were Parkinsons disease sleep scale (PDSS) and Zung SelfRating Depression Scale (SDS).
Results: 122 patients (64.9 %) had depressive symptoms. The total
PDSS score of patients with depression was signicantly lower than
those of patients without depression and controls (Bonferroni test).
Stepwise regression model with SDS index identied PDSS (p0.001)
and UPDRS I (p0.002) as signicant determinants of SDS index.
Also, in PD patients, stepwise regression analysis showed that item 15
(daytime sleepiness) (p0.002), item 13 (early morning tremor)
(p0.008), item 12 (nocturnal dystonia) (p0.015), item 3 (sleep
maintenance insomnia) (p0.026) were signicant predictors of the
SDS index, while in controls, item 4 (nocturnal restless legs)
(p0.001), and item 11 (nocturnal cramps) (p0.016) were signicant
predictors of the SDS index.
Conclusions: In our study, there was a signicant correlation between patients with depressive symptoms and nocturnal disturbances.
Daytime sleepiness, dystonia, tremor and sleep fragmentation seem to
be the crucial nocturnal disturbances in patients with depression.
637
A simple model of dopamine-related neurotoxicity Parkinsons?
L.R. Baxter, H.H. Fernandez, M.S. Okun, S. Janssen, K. Williams,
S. Constance, J. Riley, Y. Liang, M. Boules, E. Richelson
(Gainesville, Florida, USA)
Objective: Examine rotenone in planaria as a model of Parkinsons
disease.
Background: Planaria have the simplest nervous system with bilateral symmetry and cephalic enlargement. Pharmacological-behavioral
similarities to vertebrates are established for many serotonin (5-HT)
and dopamine (DA) drugs. Similar animals were ancestral to more
complex cephalids. Here development of bradykinesia-like behavior,
and its drug-treatment, along with DA depletion and CNS degeneration, are shown on exposure to rotenone (mitochondrial complex I
inhibitor) in the planarian, Dugesia tigrina.
Methods: Animals were exposed to rotenone for dose-response vs.
days. We measured motivated locomotor speed: time to travel 5 cm. in
a light gradient. Loss of heads and deaths were noted. Effects of
anti-Parkinsonian drugs were examined. Levels of DA, 5-HT and
norepinephrine (NE) were done via HPLC.
Results: Rotenone vs. vehicle gave signicant slowing at 100 and
300 nM; all died at 1000 nM. At 300 nM slowing was signicant by
day 1 and progressed to 300% of vehicle by day 2. All living
animals responded briskly to pin prick. Head loss in 300 nM
rotenone began day 2 (40%; day 3 75%), but no deaths until day
4 (21%). Rotenone reduced DA levels to 6% of controls (p0.05),
NE to 30% (p0.05), 5-HT to 80% (ns). Rotenone removed, animals with heads did not recover speed by day 4. By day 5, rotenone-

Movement Disorders, Vol. 22, Suppl. 16, 2007

S196 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

headless animals had light response, but were still slow by day 10,
compared to head-amputated animals. Rotenone animals had increases in speed to acute L-DOPA (1 mM) (mean 61 sec/5 cm 26
SD before drug, to 4017 after) vs. non-treated animals (5116 to
5212), p0.05. Non-rotenone animals had no L-DOPA speed
change (242 prior, 255 after). Bromocriptine (50 nM) improved
speed in rotenone treated animals (207%), vs. no drug (08%),
P0.05. Spiperone gave bradykinesia at 100nM; 1000 nM gave
marked dystonia. Anti-muscrinic benztropine (1 nM) reversed dystonia, but not bradykinesia.
Conclusions: Planaria exposed to rotenone seems to have adequate
face, construct and predictive validity to warrant further study as a
simple model of Parkinsons disease. Besides use as a possible screen
for anti-parkinsonian drugs, this model may be useful to study mechanisms of DA-system neurotoxicity.

Conclusions: In these four trials rotigotine transdermal patch improved nocturnal and early morning PD symptoms without increasing
daytime sleepiness, probably as a result of a benecial effect on
dopaminergic nocturnal symptoms of PD.
640
Plasma ceruloplasmin ferroxidase activity correlates with substantia nigra sonographic area in Parkinsons disease patients
R. Martinez-Hernandez, S. Montes, J. Higuera-Calleja, C. Rios
(Mexico City, DF, Mexico)
Objective: To evaluate (1) the utility of Transcraneal Ultrasound
(TCUS) in the diagnosis of Parkinsons (PD), (2) the utility of Ceruloplasmin (Cp) ferroxidase activity in plasma as a biochemical markers
and (3) to establish a relationship between the two markers.

638

639
Rotigotine transdermal patch as a treatment of nocturnal symptoms in patients with idiopathic Parkinsons disease
K.R. Chaudhuri, J. Jankovic, C. Trenkwalder, B. Boroojerdi
(London, United Kingdom)
Objective: Given the stable 24-hour plasma levels achieved with this
rotigotine transdermal system, benecial effects on Parkinsons disease
(PD) symptoms during night and day are expected. Effects of rotigotine
treatment on nocturnal and early morning PD symptoms (motor function, overall quality of sleep, nocturia, early morning akinesia and
daytime sleepiness) are reported.
Background: Rotigotine transdermal patch (Neupro) is a broadspectrum D3/D2/D1 dopamine receptor agonist, which has been approved in Europe for the treatment of idiopathic Parkinsons disease
(PD).
Methods: The effects of rotigotine on sleep was analyzed based on 3
double-blind, placebo-controlled trials and one open-label trial in patients with advanced PD. The following questionnaires were used: the
15 item Parkinsons Disease Sleep Scale (PDSS), Epworth Sleepiness
Scale (ESS) and UPDRS Part IV.
Results: A total of 1032 patients were included in this analysis, of
whom 727 were treated with rotigotine transdermal patch and 305 with
placebo. Compared to baseline, the status on awakening, measured as
mean change from off to on without troublesome dyskinesias,
improved by 24.1%, 15.6%, and 25.5% with rotigotine and by 8%,
4.3% and 11.5% with placebo in the 3 trials after up to 6 months of
treatment. In the open-label trial, the mean change from baseline (9.4)
in overall PDSS score was 11.6 (p 0.001)(including non-dopaminergic therapy responsive domains) after 3 months of treatment. ESS sum
score improved from 7.3 to 6.1. A total of 58% of patients, who had
sleep disturbances at baseline according to UPDRS Part IV were free of
symptoms at the end of the 3 month treatment phase. Furthermore,
frequency of nocturia decreased from 2.12 to 1.41 after 3 months of
treatment with rotigotine.

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (640).

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Background: TCUS is a recent tool contributing to PD diagnosis that
may be helpful in the detection of pre-morbid states and in follow-up
of disease progression. Berg et al. has shown that a measure of
.25cm2 in the area of substantia nigra (SN) has a 100% PPV for PD
diagnosis. An increase in echogenicity has been related with iron
deposits or with a change in the proteins related to its metabolism
(Hochstrasser et al. Neurology 2004; 63:1912-17). Cp ferroxidase
activity plays an important role in the CNS iron transport and a its
malfunction may be implicated in PD pathophysiology. (Boll et al.
Neurosci Let 1999;265:155-8).
Methods: We conducted a cross-sectional study with PD patients. SN
area was measured with TCUS and plasma Cp ferroxidase activity; we
also applied H-Y and UPDRS scales.
Results: Twenty-one patients and 13 controls were included; mean
age of 61.6 and 58y respectively (p0.10), 53% cases were females vs.
61% controls (p .844); H-Y has a mean of 2.45 and UPDRS off was
29.9. Sonographic areas have a mean of .31cm2 in right and .32cm2 in
left sides for cases and .12cm2 and .11cm2 for controls (p 0.001
right, p 0.001 left); no difference between SN area and presentation
type or use of L-dopa. Symptoms predomination in left body have an
increase in right SN (p .05) not for right SN. Plasma ferroxidase
activity was reduced in PD (p .001) L-dopa did not altered this
activity (p .123). Plasma ferroxidase activity and size of SN showed
a signicant correlation of R2.24 (gures 1 and 2).
Conclusions: This is the rst Mexican PD series that corroborate the
utility of TCUS in measurement of SN. We observe a higher difference
between cases and controls than that previously reported and showed
that plasma Cp ferroxidase activity is decreased in PD and had an
important correlation SN size. This was a pilot study with some
limitations for denitive conclusions, the sample is short and without
follow-up, a prospective study will be the next step.
641
Effects of subthalamic nucleus stimulation and levodopa on freezing of gait in Parkinsons disease
M. Ferraye, B. Debu, V. Fraix, J. Xie-Brustolin, S. Chabardes,
A.-L. Benabid, P. Pollak (Grenoble, France)
Objective: To assess the efcacy of subthalamic nucleus (STN)
stimulation versus levodopa therapy on freezing of gait (FOG) in a
large group of patients with Parkinsons Disease (PD).
Background: FOG in PD is disabling and poorly understood. Because STN stimulation usually mimics the effects of levodopa on most
motor parkinsonian symptoms including FOG, it is indicated in patients
with levodopa responsive symptoms. However, some patients are operated on, although their FOG or other axial features are not fully
levodopa responsive. Moreover, in few patients STN stimulation has a
deleterious effect on axial symptoms. Therefore, we studied the effects
of STN stimulation and levodopa on FOG occurrence and gait parameters in a consecutive series of patients.
Methods: This study included the rst 123 patients with bilateral
STN stimulation operated in Grenoble. They all performed the Stand
Walk Sit Test (SWST) as part of the CAPIT protocol before and one
year after surgery both off and on levodopa and off and on stimulation.
We analyzed the ability to perform the SWST, the occurrence of FOG,
a UPDRS composite gait score, step length and SWST duration.
Results: Before surgery, 48 patients were unable to complete the
SWST and 25 displayed FOG episodes when off levodopa. These
symptoms disappeared under levodopa. After surgery, STN stimulation
failed to replicate the levodopa-induced improvement in only 5 patients
unable to walk and 2 others with FOG. In 11 patients, FOG or inability
to perform the test rst occurred after surgery, in 9 of them under the
untreated condition only. In addition, the composite gait score was
worse under STN stimulation than under levodopa before surgery in all
patients. Finally, in patients able to perform the SWST without FOG,
STN stimulation decreased step length and increased SWST duration in
comparison with levodopa before surgery, whereas gait parameters did
not change in patients with FOG whatever the condition.
Conclusions: STN stimulation improved levodopa-responsive FOG
in most patients. However, our results suggest that stimulation is less

S197

effective than levodopa for gait impairments and FOG. The fact that
STN stimulation only inuences gait parameters in patients without
FOG suggests that the pathophysiologies of FOG and gait impairments
are independent.
642
Tremor related neuronal activity correlated with segregated somatotopy in the human subthalamic nucleus
P. Zhuang, M. Hallett, J. Li, Y. Zhang, Y. Li (Beijing, China)
Objective: To explore whether parkinsonian rest tremor in different
muscle groups is generated by a single central oscillator or by multiple
independent oscillators in subthalamic nucleus (STN) in patients with
Parkinsons disease (PD).
Background: There is evidence that the cortico-basal ganglia-thalamic-cortical circuits have a functional segregated somatotopic organization.
Methods: Ten medically intractable PD patients (M:6/F:4) with their
mean age 59(6.8) years at the time of operation were studied. Their
average duration of the disease was 10.6(4.6) years and the Hoehn
and Yahr score at off -state was from 2 to 5. In addition to predominantly symptoms of rigidity and akinesia/bradykinesia, all of them had
rest tremor. Microelectrode recording was performed in the STN in
patients who were undergoing surgery. Spontaneous neuronal activity
was recorded. Electromyogram (EMG) was simultaneously recorded in
selected muscle groups of upper and lower limbs (muscles of extensor
carpi radialis (ECR), exor carpi radialis (FCR) and tibialis anterior
(TA)) contralateral to surgery. Single unit and power spectral analysis
was used. Raster display and cross correlation analysis were carried out
to study neuronal activity in relation to parkinsonian tremor.
Results: One hundred and fty-six neurons were obtained from 11
trajectories. The majority of neurons (59%, n92) had irregular neuronal activity, and 15% neurons (n23) showed tonic neuronal discharge. Twenty-six percent of neurons (n41) red at same frequency
as tremor (4-6 Hz), dened as tremor-related neuronal activity, and
these neurons were investigated further. It was found that along the
tract, different tremor-related neuronal activity correlated with different
muscles measured by ECR, FCR or TA. When cells showed coherent
activity to upper limb muscles, the neuronal activity was not signicantly correlated to the lower limb tremor. When cells showed correlations with tremor in the lower limb, they did not correlate with the
upper limb tremor. Raster displays of neuronal activity illustrated these
relationships on many occasions.
Conclusions: PD rest tremor in different body parts is mediated
independently by different tremor oscillators in STN, likely a result of
the segregated somatotopy in the basal ganglia circuits.
643
Taste threshold is abnormal in Parkinsons disease and suggests
cortical spread
M. Shah, J. Deeb, M. Fernando, E. Visentin, A. Noyce, L.J. Findley,
C.H. Hawkes (Romford, Essex, United Kingdom)
Objective: To determine whether taste is signicantly involved in
classical Parkinsons disease (PD).
Background: There is strong evidence for olfactory involvement in
PD but it is unclear whether taste is involved as well. Pathologically
there is sparing of the rst and second order taste neurones (solitary
tract and nucleus; nucleus ventralis posteromedialis in the thalamus)
but the frontal operculum which carries taste bers, is involved in the
later stages. Sienkiewicz-Jarosz et al (2005) found normal taste threshold in PD. We wished to verify this and whether there was a correlation
between taste and the known smell defect.
Methods: Taste threshold was estimated from the tongue tip (fungiform papillae) with a Rion electrogustometer and olfaction by the
University of Pennsylvania Smell Identication Test (UPSIT) in 75
patients and 74 controls. PD patients were in Hoehn and Yahr stages
1-3 and scored 27 or more on the Minimental Status Examination.
Results: Patient taste thresholds were greater than controls by 6.9dB
(95% CI: 3.9, 9.9; P0.001), allowing for age, gender and smoking.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S198 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Twenty of the 75 PD patients (27%) scored outside the 95% reference
range. Taste threshold increased marginally with age in patients but not
controls (p0.039). PD-UPSIT score was signicantly impaired (PD
mean 19.5; range 4-35; Control mean 33, range 13-40; p0.001).
There was no correlation between taste and smell, adjusted for subject
status, age and gender (partial correlation coefcient -0.056 (p
0.505).
Conclusions: In our 75 patients with classical PD there was significant impairment of taste threshold that occurred independently of their
smell abnormality and affected 27% of this sample. Age and smoking
had minimal effect. The rst taste structure to show Lewy pathology is
the frontal operculum. Hence taste impairment may indicate disease
that has advanced beyond the brainstem into the frontal lobes (Braak
stage 5). While smell impairment is an early sign of PD, taste impairment is probably a late feature.
644
Levodopa, DDCI and entacapone has minor long-term effects on
dyskinesia while signicantly decreasing OFF-time in Parkinsons
disease (PD) patients with wearing off
M. Kuoppamaki, M. Vahteristo, H. Nissinen, J. Ellmen (Turku,
Finland)
Objective: To analyze dyskinesia duration and severity with levodopa, DDCI and entacapone vs levodopa and DDCI in PD patients.
Methods: We present a retrospective pooled analysis of four
6-month, double-blind, placebo-controlled, Phase III studies, in which
808 uctuating PD patients were randomized to receive entacapone or
placebo in addition to their existing levodopa and DDCI therapy.
Dyskinesia was evaluated at baseline and end of study using UPDRS
Q32 (duration) and Q33 (severity).
Results: At baseline, 64% of patients randomized to entacapone and
68% randomized to placebo had dyskinesia (Q32 1). At 6 months,
24% of patients treated with levodopa, DDCI and entacapone had an
increased duration of dyskinesia vs 14% receiving levodopa and DDCI.
Importantly, there were no differences between the two groups in the
change in severity of dyskinesia. The daily levodopa dose or dosing
frequency did not affect the impact of levodopa, DDCI and entacapone
therapy in this respect. In patients with no dyskinesia at baseline, 71%
and 79% of patients treated with levodopa, DDCI and entacapone, and
levodopa and DDCI, respectively, remained dyskinesia-free at 6
months, while 21% and 18% of patients, respectively had a Q32 score
of 1 (125% of day). Dyskinesia that developed during the study was
generally non-disabling (Q330 in 50% of both arms); 25% of
patients in the levodopa, DDCI and entacapone and 10% in the levodopa and DDCI arms had mildly disabling dyskinesia (Q331), and
18% and 19%, respectively, had moderately disabling dyskinesia
(Q332 4) at 6 months. Only 1.7% of patients receiving levodopa,
DDCI and entacapone discontinued treatment due to dyskinesia. In
patients with or without dyskinesia at baseline, OFF-time was signicantly decreased by 0.7 0.8 h (p0.05) with levodopa, DDCI and
entacapone vs levodopa and DDCI.
Conclusions: In PD patients with wearing-off symptoms, levodopa,
DDCI and entacapone therapy had only a minor impact on dyskinesia
while signicantly improving daily OFF-time during the 6-month follow-up. If present, dyskinesias were non-disabling in the majority of
patients. This is clinically important as patients usually prefer being
ON with mild dyskinesia compared with being under-treated and
having troublesome parkinsonian symptoms.

Background: There is a wide range of AS in PD, but the full

spectrum has never been evaluated with a validated instrument and in
comparison with control subjects. In this study a reliable and valid
instrument, the SCOPAAUT, was used.
Methods: A cohort of 420 patients with PD was evaluated for the
occurrence of AS, motor and non motor symptoms, as well as for
demographic and diseaserelated characteristics. Results were compared with those of 150 control subjects. In patients, associations
between AS and demographic and clinical characteristics were also
studied.
Results: For all autonomic domains, patients with PD reported more
symptoms compared to control subjects, with the greatest differences in
the gastrointestinal and urinary domain. Higher age, greater disease
severity and higher doses of dopaminergic medication were related to
more autonomic problems. Autonomic symptom severity was associated with more motor dysfunction, depressive symptoms, cognitive
dysfunction, psychiatric complications, nighttime sleep disturbances
and excessive daytime sleepiness (all pvalues 0.01).
Conclusions: AS are an important feature of PD and increase with
age, disease severity, and medication use. The prominent presence of
AS warrants increased clinical awareness and highlights the need for
efcacious therapies for the wide spectrum of problems related to this
domain of PD.
646
SOS Parkinson: An emergency call service report on ve years of
experience
A.L. Zecchinelli, A. Antonini, M. Barichella, A. Marczewska,
G. Pezzoli (Milano, Italy)
Objective: To evaluate the role and usefulness of an emergency call
center for parkinsonian patients.
Background: The SOS Parkinson service was founded in 1997 to
meet the demand for emergency information during holidays and on the
weekend, when patients with Parkinsons disease (PD) nd it difcult
to contact their neurologist. When they experience a problem with their
disease because of the occurrence of a concomitant disorder or for other
reasons, and they cannot discuss it with their neurologist, they often
inappropriately go to a hospital emergency room, not knowing what to
do. Physicians working in emergency rooms are not trained to manage
the highly individualized therapy of a chronic disorder, such as PD, and
the absence of a specialist is a source of anxiety for the patient.
Methods: A group of neurologists specialized in movement disorders
decided to set up a call center for patients, caregivers and also for non
specialized colleagues, who need advice that can be imparted by
telephone. The usefulness of the service was assessed by collecting
information on the reasons for the calls and the identity of the caller, as
well as information on their frequency and on the main issues that
emerged during the telephone conversations.
Results: On average we received 541 calls per year, 4.7 calls per day,
37% of them were judged as urgent (i.e. necessitating an immediate
answer). Most of the calls regarded PDs therapy (39%) concomitant
medications/comorbidity (25%), psychiatric complications (8%), as
well as general information (23%).
Conclusions: Based on the number of calls the SOS service is a
useful service than can provide a rst aid to patients when they cannot
reach their neurologist. In some cases the service was a good source of
advice even for the physician not trained in the eld of movement
disorders.
647

645
Patient-reported autonomic symptoms in Parkinsons disease
D. Verbaan, J. Marinus, M. Visser, S.M. van Rooden,
A.M. Stiggelbout, J.J. van Hilten (Leiden, Netherlands)
Objective: To evaluate the occurrence of autonomic symptoms (AS)
in a large cohort of patients with Parkinsons disease (PD) and control
subjects and to assess the relations with demographic, diseaserelated
and clinical variables in patients.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Glucose-6-Phosphate dehydrogenase in the peripheral blood of

patients with Parkinsons disease
P. Mir, L. Gao, F. Diaz, R. Mejias, F. Carrillo, P.J. Vime,
J. Diaz-Martin, A. Palomino, M. Carballo, E. Pintado, M. Lucas,
J. Lopez-Barneo (Seville, Spain)
Objective: To determine whether reduced glucose-6-phosphate dehydrogenase (G6PD) activity is a risk factor for Parkinsons disease
(PD).

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

Background: G6PD is a key cytoprotective enzyme that provides
NADPH, a major reductant to maintain intracellular redox status.
G6PD deciency is a common enzymopathy, causing hemolytic anemia in some extreme cases as a result of decreased oxidative defense in
red blood cells (RBCs). Oxidative damage to dopaminergic neurons is
involved in the pathogenesis of PD. The role of reduced G6PD activity
in PD is still uncertain.
Methods: We included 159 PD patients (64.1 10.7 years old, 91
male and 68 female) and 190 control subjects (62.8 10.6 years old,
108 male and 82 female) in this study. The mean age at onset of the
disease was 56.0 12.3 years old, and the mean disease duration time
was 7.9 6.5 years. Peripheral blood sample was obtained from each
subject for measuring G6PD activity in RBCs and lymphocytes, pentose-phosphate pathway activity in RBCs, and routine blood analysis.
Results: The activity of G6PD was similar between PD patients and
control subjects in both RBCs and lymphocytes. The activity of the
pentose-phosphate pathway in RBCs, where G6PD is the rst ratelimiting enzyme, was also similar between PD patients and controls.
When the RBCs were challenged with 50 M cumene hydroperoxide,
the activity of the pentose-phosphate pathway increased, but the increase was similar between the two groups. Furthermore, no clear
association was observed between hemolytic anemia and PD as determined by measuring hemoglobin, hematocrit, and erythrocytes levels.
Conclusions: Our results argue that although oxidative stress plays
an important role in the pathophysiology of PD, reduced G6PD activity
is not associated with PD at least in our sample.
648
Establishing a model of Standards of Care for patients with
Parkinsons disease using a comprehensive database Interdisciplinary Florida Registry and Movement Disorders Database (INFORM)
R.L. Rodriguez, C. Jacobson, C. Garvan, R. Stephen, K.D. Foote,
C. Nugent, J. Romrell, P. Zeilman, M.S. Okun, S. Vaidyanathan,
H.H. Fernandez (Gainesville, Florida, USA)
Objective: To formulate key questions and criteria that should be
considered as the minimum comprehensive care offered to PD patients.
Background: Quality of care (QOC) is dened as excellence in
delivery of care that maximizes the probability of benecial health
outcome. PD is a complex disorder often requiring comprehensive
multi-disciplinary, pharmacological and non-pharmacological treatment. Formal assessments of QOC standards in PD do not exist.
INFORM is a database that contains motor, functional, QOL, behavioral and cognitive scales used in the initial and follow-up assessment
of all PD patients seen at the Univeristy of Florida MDC.
Methods: A preliminary list of questions assessing the recognition
and treatment of motor, functional and non-motor aspects in PD was
formulated. INFORM was used to randomly screen for PD patients. A
chart review was performed by a MDS to determine if: a) the problem
was recognized by the clinician (agged as yes, no or unclear)
and b) the problem was addressed in a timely manner (agged yes,
no, unclear or not applicable). For a questions to be considered
as good/useful, less than 25% should be agged as unclear . The nal
list was comprised of good/useful questions to assess QOC. A Comprehensive PD Care Center should aim to have less than 25% no
assessments on each question in the nal list.
Results: Our preliminary list comprised 33 questions. INFORM
identied 60 random PD patients. No questions were agged as unclear more than 25% of the time. The questions regarding swallowing,
family/caregiver problems, use of anticholinergics, troublesome dyskinesia, wearing off, and surgery were applicable to less than 10 patients
and therefore might not be useful questions for this small sample.
While our Center scored in the passing range for most criteria, we
failed to recognize depression and driving problems and to document
treatment of speech, gait, cognitive, behavioral and driving problems.
Conclusions: Developing models to assess QOC standards in PD will
be important for PD centers worldwide.

S199

649
Inter-individual variations of plasma concentrations of amantadine
hydrochloride in patients with Parkinsons disease
M. Nagai, M. Kubo, N. Nishikawa, H. Yabe, M. Nomoto (Toon,
Ehime, Japan)
Objective: To evaluate the inter-individual variations on pharmacokinetics of amantadine in patients with Parkinsons disease (PD).
Background: Nowadays relatively high dose of amantadine is used
for the treatment for levodopa-induced dyskinesias in patients with PD.
Unchanged form of amantadine mainly excretes in urine via kidney. It
appears to be a relationship between plasma levels of amantadine and
central nervous system (CNS) adverse drug reactions. However, interindividual variations of amantadine and expected its concentrations
associated with CNS adverse drug reactions have not been fully dened.
Methods: Fifty-seven of PD patients taking amantadine orally more
than two weeks were enrolled in this study. Blood samples were
collected at three hours after taking amantadine. Plasma levels of
amantadine were measured using gas-chromatography. Creatinine
clearance (Ccr) was calculated by Cockcroft-Gault equation.
Results: The mean value on plasma concentrations of amantadine,
which converted equivalent dosage as amantadine 100mg, was 650ng/
ml. The levels of amantadine in the patients who had enough renal
function (Ccr 75ml/min) varied between 107 and 837ng/ml. CNS
adverse events (myoclonus, hallucinations, and dysarthria) occurred in
three patients. Plasma concentrations of amantadine in all of them were
more than 3000ng/ml. These symptoms were reversible and improved
totally after discontinuation of amantadine.
Conclusions: The plasma concentrations of amantadine showed approximately eightfold difference among individuals. CNS adverse
events appeared when plasma concentrations of amantadine were over
3000ng/ml.
650
Environmental and genetic factors in Parkinsons disease. The
FRAGAMPP case-control study
A. Nicoletti, P. Pugliese, G. Nicoletti, G. Arabia, G. Torchia,
M. De Mari, P. Lamberti, L. Grasso, R. Marconi, A. Epifanio,
L. Morgante, L. Cozzolino, P. Barone, A. Quattrone, M. Zappia
(Catania, Italy)
Objective: We carried out a large multicenter case-control study (the
FRAGAMPP Study) to evaluate the possible role of environmental and
genetic factors in Parkinsons disease (PD).
Background: Importance of genetic factors in the pathogenesis of PD
has been recently understood after the identication of monogenic
familial parkinsonisms. However 90% of cases are apparently sporadic
and probably due to a complex interaction between genetic and environmental factors.
Methods: Cases and controls were enrolled from six Italian Research
Units located in Bari, Catanzaro, Cosenza, Grosseto, Messina and
Naples during the study period 2004-2005. All patients affected by PD
who fullled the diagnostic criteria proposed by Gelb et al. in 1999
were consecutively enrolled in the study. Two different control groups
were selected. The rst control group consisted on the partner, when
available, of each PD patient enrolled in the study, in order to evaluate
the role of genetic factors; healthy controls matched by age (/- 5
years) and area were selected from the general population and represented the second control group. A standardized questionnaire was
administered to both cases and controls to record demographic, epidemiological and clinical data. All cases and controls underwent a standard neurological examination. Presences of depression, cognitive impairment, hallucinations, and sleeps disturbances were also evaluated
using standardized scales. A blood sample was taken from both cases
and controls for molecular analyses.
Results: Overall 585 subjects affected by PD (237 women and 348
men) were enrolled in the study. The mean age at entry for PD patients
was 66.8 9.7 years with a mean age at onset of 59.7 10.7 and with
mean disease duration of 7.2 5.6 years. Healthy controls selected

Movement Disorders, Vol. 22, Suppl. 16, 2007

S200 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

were 354 women and 200 men, with mean age at entry of 63.79.9
years.
Conclusions: The FRAGAMPP study represents, to date, one of the
largest case-control studies carried out to investigate the possible role
of environmental and genetic factors in PD.
651
Investigation on the prevalence of dyskinesia following the switch
from continuous rotigotine to L-DOPA treatment and vice versa
K. Stockwell, D.K.A. Scheller, S. Rose, M. Jackson, P. Jenner
(Monheim, Germany)
Objective: The study investigates the differences in the prevalence of
dyskinesia, switching from continuous rotigotine treatment to pulsatile
L-DOPA treatment and vice versa in MPTP-treated marmosets.
Background: Rotigotine is a non-ergolinic dopamine agonist developed for the treatment of idiopathic Parkinsons disease. It is administered transdermally to provide stable plasma levels.
Methods: Stable motor decits in common marmosets (Callithrix
jacchus; 325-389g; 5-8years, n6) were induced with MPTP
(2.0mg/kg sc for 5 days). Group 1 (n5) received continuous rotigotine
(0.18mg/kg/day sc osmotic minipump) followed by L-DOPA/carbidopa (12.5mg/kg, po, BID, 3.5h apart); Group 2 (n6) received LDOPA BID followed by continuous rotigotine infusion. Each treatment
period lasted 28 days. Locomotor activity, motor disability and dyskinesia were assessed weekly over 8 hours. The study was performed in
accordance with UK Scientic Procedures Act (1986).
Results: In Group 1, continuous infusion of rotigotine resulted in a
sustained improvement in locomotor activity and motor disability and
induced little or no dyskinesia (no signicant difference to baseline).
The improvement in locomotor activity and motor disability was maintained following the switch to pulsatile L-DOPA; however, a rapid and
persistent increase in dyskinesia with a signicant increase in dyskinesia on-time was observed. In Group 2, treatment with pulsatile
L-DOPA improved locomotor activity and motor disability transiently
but resulted in moderate to marked dyskinesia the duration of which
was signicantly prolonged. Upon switching to continuous rotigotine,
dyskinesia was less severe and its duration was signicantly reduced
with the improvement in motor activity being maintained.
Conclusions: Continuous treatment with rotigotine caused a sustained improvement of the motor disability with mild dyskinesia in a
primate model of PD which, however, could not prevent the induction
of dyskinesia by subsequent L-DOPA administration. By contrast, the
severity of dyskinesia induced by L-DOPA tended to improve after
switching to continuous rotigotine. Thus, pre-existing dyskinesia induced by pulsatile L-DOPA treatment in monkeys can be improved by
a switch to continuous rotigotine administration.
652
Proteome analysis of human substantia nigra in Parkinsons disease
C.J. Werner, R. Heyny-von Haussen, G. Mall, S. Wolf (Aachen,
NRW, Germany)
Objective: To characterize changes in protein composition of substantia nigra (Sn) in idiopathic Parkinsons disease (PD) using proteomics.
Background: Affection of the substantia nigra and its projections is
responsible for some of the most debilitating features of PD. To further
advance an understanding of nigral pathology, we conducted a tissuebased comparative proteome study of healthy and diseased human
substantia nigra.
Methods: Five parkinsonian midbrain samples and ve age- and
gender-matched controls were examined using high-resolution/high
sensitivity proteomics. Thirty gels with a pH 4-7 (24 cm) were created,
stained with Sypro Ruby and analyzed using the PDQuest 7.1 package. Automated spot picking was followed by tryptic in-gel digestion
and MALDI-ToF mass spectrometry. NCBI and SwissProt databases
were queried for matching proteins.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Results: Results showed signicant differences among groups. The

gross number of differentially regulated proteins was 221. In total, we
identied 37 proteins, of which 17 were differentially regulated. Differential proteins comprised elements of iron (ferritin) and redox metabolism (GST M3, GST P1, GST O1). Additionally, many glial or
related proteins were found to be differentially regulated in PD (GFAP,
GMFB, galectin-1, sorcin), as well as proteins of metabolic pathways
not yet or sparsely described in PD, such as methyl glyoxalase 1 (AGE
metabolism), adenosyl homocysteinase (methylation), ADH-1 and cellular retinol-binding protein 1 (aldehyde metabolism). Further proteins
included annexin V, beta-tubulin cofactor A, coactosin-like protein and
V-type ATPase subunit 1. Similarly expressed proteins comprised
classical housekeeping proteins such as COX5A, Rho GDI alpha, actin
gamma1, CK-B, LDH-B, disulde isomerase ER-60, Rab GDI beta and
glutamine synthetase, but also DJ-1 and UCH-L1. Furthermore, proteins believed to serve as internal standards (such as 14-3-3 epsilon and
hCRMP-2) were found to be expressed in a constant manner.
Conclusions: Using high sensitivity/high-resolution proteomics, we
show that alterations of Sn in PD include many more proteins than
previously thought. Differentially expressed proteins point towards a
heterogeneous aetiopathogenesis of PD, including alterations of known
metabolic pathways, but also highlighting many new and potentially
promising targets.
653
Elevated C-reactive protein in Parkinsons disease: Are they associated with neuropsychiatric complications?
S. Hassin-Baer, O.S. Cohen, B.-A. Sela, Z. Nitzan, E. Vakil,
R. Schwartz, J. Chapman, T. David (Ramat-Gan, Israel)
Objective: To assess whether plasma levels of C-reactive protein
(CRP) are associated with clinical characteristics or neuropsychiatric
manifestations in patients with Parkinsons disease (PD).
Background: A chronic inammatory reaction may take part in the
neuronal death in PD as activated microglia and increased levels of
inammatory mediators have been detected in the striatum of PD
patients. CRP is a plasma protein that participates in the systemic
response to inammation and its concentration is regarded an important
vascular risk factor and is associated with cognitive impairment and
depression. The role of CRP in PD or its neuropsychiatric complications is unknown.
Methods: Idiopathic PD patients were evaluated using the UPDRS,
Hoehn & Yahr stage, Parkinson Psychosis Rating Scale (PPRS), Beck
Depression Inventory (BDI), Frontal Assessment Battery (FAB), Minimental Status Examination (MMSE) and several tests for frontal type
cognitive functions and memory. Blood samples were collected and
assessed for CRP levels.
Results: Consecutive PD patients (n73; 46 males, average age
68.711.6yrs, average disease duration 7.04.7yrs) were recruited.
The average level of CRP was 3.9 4.1 umol/L. Patients with elevated
CRP levels (3 umol/L, n40, 55%) did not differ in gender, disease
duration, levodopa dose, motor UPDRS score and Hoehn and Yahr
stage, but tended to be slightly older (71.49.2 vs. 66.712.9;
p0.09) than those with CRP levels 3 umol/L. Coronary artery
disease (CAD) was more prevalent in the elevated CRP group (36% vs.
10% p0.05; age and sex adjusted odds ratio 5.6, 95%CI 1.526.8).There were no differences in crude or adjusted scores of MMSE,
FAB, BDI score, PPRS, or apathy score or in prevalence of hallucinations or delusions between the 2 groups. The prevalence of depression
was higher in the elevated CRP group (55% vs. 25%, p0.05; age and
sex adjusted odds ratio 4.4, 95%CI 1.6-13.9). No differences in several
tests for frontal-type cognitive impairment or memory were found.
Conclusions: Elevated CRP levels in our patients with PD are
associated with a higher prevalence of CAD and depression, but not
with disease duration or severity, nor with most neuropsychiatric complications such as dementia, frontal-type cognitive decline or psychosis.

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

654
Brain permeable iron chelators M-30 and AK-28 rescue degenerated nigral dopamine neurons in animal model of PD
W. Zhu, T. Pan, W. Xie, J. Jankovic, H. Zheng, M. Youdim, W. Le
(Houston, Texas, USA)
Objective: To test our newly-developed brain permeable iron chelators M-30 and VK-28 in vivo in an ubiquitin-proteasome system (UPS)
impairment-induced animal model of PD for their rescuing effects on
degenerated nigral dopamine (DA) neurons.
Background: There is a signicant body of evidence from animal and
human brain studies that dysregulation of iron and UPS collapse have
a pivotal role in DA neurodegeneration. Our previous studies in vivo
demonstrated the ability of proteasome inhibitor, lactacystin, when
directly injected in nigra to inactivate UPS locally, cause accumulation
of iron in substantia nigra (SN) and degeneration of DA neurons. In a
previous study, we demonstrated that M-30 and VK-28 can prevent the
process of neurodegeneration if given before lactacystin. In this new
study we test the ability of M-30 and VK-28 to rescue degenerated DA
neurons.
Methods: We applied direct stereotactic injection of proteasome
inhibitor lactacystin (1.2 ug/2ul) in the SN of C57BL/6 mice. We
demonstrated a progressive loss of nigral DA neurons and DA contents,
ubiquitin-positive intracytoplasmic aggregation, and high levels of iron
in SN, which resembles some cordial neuropathological features of PD.
One week after lactacystin injection, we administrated M-30 (5 mg/kg
daily, i.p) and VK-28 (5 mg/kg daily, i.p) for 21 days till the animals
were sacried for immunohistology and biochemistry analyses.
Results: Systemic administration of M-30 and VK-28 showed
marked rescuing effects on DA neurons in SN against lactacystininduced injury by 78% and 67% respectively. Furthermore, M30 and
VK-28 signicantly attenuated the lactacystin-induced depletions of
striatal DA by 88.3% and 76.1%, respectively. In additon, M-30 and
VK-28 showed the ability to attenuate the lactacystin-induced iron
increase in SN.
Conclusions: Both M-30 and VK-28 has strong DA neurons recurring effects in the UPS-impairment induced animal model of PD, and
multiple functional drug M30 seems to be more potent than VK-28 in
neuroprotection. The results of this study may warrant further preclinical and clinical tests for their potential in the treatment of PD.

655

S201

Conclusions: Using the Foot Scan System (RS scan international

cooperation) measuring the plantar pressure distribution, we showed
that F/R ratio and time percentage of propulsion phase are reduced
signicantly in patients with IPD, so we can be easy to understand the
features of gait in IPD patients.
656
Risk factors for heavy burden among family caregivers of Parkinsons disease patient
O. Benavides, P. Chana, D. Alburquerque, T. Parrao, C. Juri,
C. Kunstmann (Santiago, Chile)
Objective: The object of this study is to explore the factors that are
generating a burden on caregivers, with the goal of discovering a
method of intervention to improve the quality of living not only for the
patient, but for their caregiver as well.
Background: Parkinsons disease (PD) patients often are assisted
daily by others. These caregivers are exposed to a decrease in their
quality of life.
Methods: Fifty-nine patients with Parkinsons disease were included:
32 men and 27 women; with an average age of 6712 years, with an
average time of evolution of the disease of 85 years to whom the
following were applied: the UPDRS III scale, the Hoehn & Yahr stage,
the Parkinsons minimental test, the neuropsychiatric inventory, and to
the caregivers the Zarit Burden Interview (ZBI) was applied, exploring
the risk factors of those who presented a heavy caregiver burden and in
those who presented light or non-existent burden.
Results: Forty-ve percent (15 out of 33 cases) of the patients, whose
caregivers presented light or no burden, presented a depression according to the Beck Depression Inventory (BDI). 77.8% (14 out of 18 cases)
of the patients, whose caregivers presented heavy burden, had depression according to the BDI (p0.01). The caregivers that presented
heavy burden, according to the ZBI, accompany the patient 984.6%
of the day. Those who present light or no caregiver burden accompany
the patient 8627.9% of the day (p0.05). When correlating the ZBI
with age, BDI and axial compromise in the UPDRS-III (R0.4
p0.005; R0.6 p0.001; R0.46 p0.005 respectively).
Conclusions: In this study, like many others, the motor disorders, the
cognitive deterioration or the behavioral disorders are related to the
burden, never the less, the depression appears as the most relevant
factor. It is interesting mentioning the variable of time that the caregiver dedicates to his/her patient, which in our study resulted close to
100% generating an insufcient time alone for the caregiver.

Dynamic foot pressure measurement in Parkinsons disease with

foot scan system
C.N. Lee, D.H. Lee, K.W. Park, B.J. Kim, K.M. Oh (Seoul, Korea)
Objective: We studied the parameters of foot scan system between
normal controls and IPD patients before and after levodopa administration.
Background: Foot Scan System(RS Scan International Cooperation)
measuring the plantar pressure distribution is simple and inexpensive
tool for gait analysis. And the analysis of plantar pressure distribution
of the feet during gait maybe helps the assessment of motor symptoms
or gait stability of Idiopathic Parkinsons disease (IPD).
Methods: We compared the parameters of foot scan system between
34 normal controls and 23 IPD patients before and after levodopa
administration. Data of plantar force distribution and time of stance
phase were collected using pressure-sensitive insoles as parts of the
Foot Scan System.
Results: There were signicant difference between normal controls
and IPD patients in fore foot peak pressure/rear foot peak pressure
ratio, time percentage of heel strike phase, mid stance phase, propulsion
phase in stance phase. But in these parameters there were no signicant
difference between IPD patients before dopamine with after dopamine
medication.

FIG. 1 (656).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S202 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

657

658

What is dorso-lateral in the STN? An anatomical consideration on

the ambiguous nomenclature of todays principle target for DBS
surgery
V.A. Coenen, A. Prescher, T. Schmidt, F.I.H. Gielen (Aachen,
Germany)

Discharge properties of human subthalamic nucleus neurons in the

parkinsonian and non-parkinsonian state
F. Steigerwald, P. Stangenberg, M. Potter, J. Herzog, M. Pinsker,
H.M. Mehdorn, G. Deuschl, J. Volkmann (Kiel, Germany)

Objective: To describe and to clarify the ambiguity of naming of

what today is still called the dorso-lateral STN (subthalamic nucleus). To dene a new anatomical description according to stereotactic
coordinates three-dimensional character of the STN that represents the
sensory-motor target of STN DBS (deep brain stimulation).
Background: Classically, the target for stereotactic Neurosurgery in
advanced Parkinsons Disease (PD) is called the dorso/lateral STN,
representing its sensory-motor part. Ambiguities arise, as to what the
true meaning of dorsal (superior or posterior?) and ventral (inferior or
anterior?) with respect to the STN is. Possibly, because of this uncertainty stereotactic neurosurgeons around the world do not all speak the
same language.
Methods: We conducted a review of the classical anatomical literature and compared planning data from our STN DBS procedures.
Results: The STN is located at the junction of the upper brain stem
(mesencephalon) and the forebrain. Anatomically two axes cross at this
junction with approximately a 120 degree angle: Forels axis of the
forebrain that runs through the roof of the third ventricle, and Meynerts axis of the brain stem which runs through the base of the
rhomboid fossa. These axes dene directions in the brain differently [1,
Figure 1]. For this reason the ambiguity of naming arises, since it is not
clear to which of those axes dorso-lateral refers. Direct targeting for
STN DBS targets lateral to the anterior border of the Red nucleus (RN)
in the axial slice representing its widest cross section in T2W MRI.
With the direction of the trajectory and a double oblique and precoronal approach the nal active electrode contact will be located at the
superior border of the STN. This is the lateral and anterior but superior
part of the STN.
Conclusions: We propose a renement of target denition nomenclature. In our understanding the classical anatomical term dorsolateral STN does not reect the complex three-dimensional nature of
the nucleus and is misleading. A proper denition of the target area for
STN DBS would be anterior lateral and superior STN.
References:
ber Lage- und Formveranderung des Hypothalamus
1 Diepen R: U
und des Infundibulums in Phylogenese und Ontogense. Deutsche
Zeitschrift f Nervenheilkunde, 1948 (159):340-358.

FIG. 1 (657).

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To assess abnormalities in single cell and ensemble

coding of the basal ganglia by comparing the discharge properties of
human subthalamic nucleus (STN) neurons in Parkinsons disease (PD)
to patients in a non-parkinsonian state.
Background: Based on neural recordings - mainly in the MPTP monkey
model - a disinhibition of neurons in the STN with a predominance of
oscillatory and/or bursting discharge patterns and an increase of interneuronal spike synchronisation within the basal ganglia have been proposed as
pathophysiological hallmarks of PD. Microelectrode guided implantation
of deep brain stimulation (DBS) electrodes for treating patients with
essential tremor (ET) allow to explore the subthalamic area in humans not
affected by PD and to test these hypotheses.
Methods: We used a Ben-Gun approach to simultaneously record
neuronal activity along up to 5 parallel microelectrode trajectories
during the implantation of DBS electrodes into the STN (for PD) or the
ventrolateral thalamus/subthalamic area (for ET). A threshold detection
and template matching procedure in combination with a principal
component analysis was used to isolate the activity of single neurons.
The ring pattern of each neuron was classied according to the shape
of the inter-spike interval histogram. Auto- and crosscorrelations were
analyzed for oscillations and synchrony.
Results: Recordings of 65 PD-patients and 9 ET-patients were
screened and a total of 351 SUA in PD and 32 in ET could be isolated
from the STN. A stereotactic analysis of the recording positions conrmed the overlap of the neurons within the STN region. STN neurons
of PD patients exhibited a signicantly higher discharge rate (40Hz vs.
18Hz, p 0.001) and a predominance of bursting discharge patterns
(82% vs. 56%, p 0.001) compared to STN neurons in ET. Oscillations and synchrony in the bandwidth 11 - 30Hz were a unique feature
in PD, while low-frequency oscillations (1 - 10Hz bandwidth) were
present in both ET and PD.
Conclusions: We provide rst evidence in human recordings for an
increased discharge rate of the STN in PD, a shift towards a bursting
discharge pattern and an increased synchrony of single cell oscillations
in the bandwidth of 11 - 30Hz as compared to a non-parkinsonian
control-group.
659
Rotigotine transdermal patch in early stage Parkinsons disease:
Results of a placebo- and ropinirole-controlled trial
N. Giladi, A. Korczyn, B. Boroojerdi (Monheim, Germany)
Objective: Neupro (rotigotine transdermal patch) has been approved in the EU for treatment of early and advanced stage Parkinsons
disease (PD).
Background: Rotigotine for the treatment of early stage PD was
compared to placebo and ropinirole in a randomized, multinational,
double-blind, double-dummy trial. This abstract outlines results of post
hoc comparisons of rotigotine and ropinirole study arms based on
maintenance periods of equal duration.
Methods: In this trial, starting at 2mg/24h rotigotine (10cm2), earlystage PD patients were randomized to rotigotine (up to 8mg/24h, 40
cm2), ropinirole (up to 24mg/day) or placebo. The primary efcacy
measure was the change in UPDRS subtotal (Parts IIIII) score. The
titration period to the maximal dose was 13 weeks for ropinirole and 4
weeks for rotigotine. Corresponding maintenance periods were 24 and
33 weeks. In this analysis, active study arms were compared based on
maintenance periods of equal duration of 24 weeks (FAS, LOCF,
ANCOVA) for different ropinirole maintenance dose subgroups. Although dosing was allowed up to 24 mg/day, ropinirole doses for early
PD are generally about 12-15 mg/day.
Results: After 24 weeks of maintenance period, the UPDRS subscores showed an improvement of -8.00.7 (LS MeanSEM; rotigotine up to 8mg/24h, N213) as compared to -10.90.6 (ropinirole up

POSTER SESSION II, WEDNESDAY, JUNE 6, 2007

to 24mg/day, N227; p0.002). In comparison, ropinirole group
showed an improvement of -8.71.0 (n104; p0.5) up to a dose of
12mg/day and -9.81.7 (n134; p0.08) up to a dose of 15mg/day.
Adverse events at the planned doses were consistent with stimulation of
dopamine receptors and the use of a transdermal system, including
nausea (27% vs. 35% for rotigotine and ropinirole, respectively), vomiting (11% vs. 10%), somnolence (22%vs. 28%), and application site
reactions (35% vs. 7%).
Conclusions: Rotigotine transdermal patch represented a safe and
effective option for the treatment of patients with early-stage PD in this
trial. Ropinirole showed the greatest reduction in UPDRS when all
doses were evaluated. The efcacy of rotigotine (up to 8mg/24h) was
comparable to that of ropinirole up to 15mg/day.
660
Two novel missense mutations were found in a Parkin2 gene and
may be related to the development of the early onset parkinsonism
M. Kasap, G. Akpinar, E. Ergul, H.A. Idrisoglu, A. Sazci (Kocaeli,
Turkey)
Objective: We aimed to reveal Park2 mutations by studying fulllength cDNA generated from leukocytes of a 33-year-old healthylooking individual and a 35 year-old Parkinsons disease (PD) patient.
Background: Parkin protein, a RING-type E3 ubiquitin ligase with
an amino-terminal ubiquitin-like (Ubl) domain and an RBR signature
consisting of two RING nger domains separated by an IBR/DRIL
domain. Naturally occurring mutations in Parkin2 gene are thought to
cause the autosomal-recessive juvenile parkinsonism.
Methods: Total RNA was isolated from human leukocytes using a
commercial kit. After conrming RNA integrity, using gene specic
primers and two step reverse transcriptase PCR, a 1.4-kb Park2 cDNA
was synthesized and cloned into pDuet vector. The identity of the gene
product was conrmed by sequencing (NCBI accession number
EF375726.
Results: Three silent and two missense mutations were found in
Park2 gene of an early onset PD patient. The silent mutations detected
at base number 429, 513 and 667 were causing C to T, G to A and C
to T changes, respectively. The missense mutation at base number 932
(G to A) causes replacement of glutamine with arginine and the
missense mutation at base number 1111 (G to A) causes replacement of
alanine with threonine. The 1111 mutation was also detected in the 33
year-old healthy looking individual. Function-wise and structure-wise,
both mutations are radical and may affect protein stability and function.
Additionally, the mutation at base number 932 creates a BsrBI cut-site
which can be used to screen other patients for a possible parkinsonismrelated polymorphism.
Conclusions: We propose that these missense mutations may affect
critical domains of the Parkin protein and should be studied in detail to
reveal their relationship with the disease. We are in the process of
understanding the effect of these mutations on the function of Parkin
protein.
661
Impaired attention: A risk factor for falls in Parkinsons disease?
L.M. Allcock, E.N. Rowan, K. Wesnes, R.A. Kenny, D.J. Burn
(Newcastle upon Tyne, Tyne and Wear, United Kingdom)
Objective: To determine whether impaired attention independently
predicts falls in a cohort of subjects with Parkinsons disease (PD)
studied prospectively over a one year period.
Background: Falls are common in PD resulting in injury, loss of
independence and reduction in quality of life. Cognitive decits, in
particular decits of attention and executive function, affect postural
sway and balance in PD. We prospectively studied attention as a risk
factor for falls in PD, hypothesising that greater impairments would be
associated with more falls.
Methods: Patients meeting UK PD Society Brain Bank Criteria were
assessed in the morning, in a fasting state and off all medications from
midnight. Assessment included UPDRS III and a computerized cognitive battery (CDR) from which power of attention, continuity of atten-

S203

tion, cognitive reaction time and reaction time variability were derived
(Wesnes K, Neurology 2005; 65(10):1654-6). Falls were assessed
prospectively using monthly fall diaries returned over 12 months following baseline assessment.
Results: Of 176 study participants, four died during the 12 month
observation period and eight withdrew from the study, leaving 164
completed fall diary datasets. Of those patients completing the fall
diaries, 103 (63%) fell one or more times during the year. A total of 736
falls were logged (mean 4.5 falls per patient, median 1.0). Comparison
of fallers and non-fallers revealed that disease severity (p0.004), a
history of previous falls (p 0.002) and continuity of attention
(p0.03) were the factors most signicantly associated with falls.
Logistic regression revealed that the association of reduced continuity
of attention with increased fall frequency was retained even after
correcting for UPDRS III scores (table 1).
Conclusions: Decreased continuity of attention is associated with
increased fall frequency in PD. This is has implications for the identication of those most at risk of falling, and for the management and
prevention of falls in this patient group.

Logistic Regression: Factors Associated with Falls

Outcome

Explanatory Variable

95% CI

Fall Frequency

UPDRS III
Age
Power of Attention
Continuity of Attention
Cognitive Reaction Time
Reaction Time Variability

-4.32
-0.48
-1.68
2.28
-1.42
-1.87

0.89-0.96
0.96-1.03
1.00-1.00
1.00-1.05
1.00-1.00
0.03-1.08

0.001
0.63
0.09
0.02
0.16
0.06

662
Age-dependent changes in glial cells from parkin null mice
R.M. Solano, M.J. Casarejos, J. Menendez-Cuervo,
J.A. Rodrguez-Navarro, J. Garca de Yebenes, M.A. Mena (Madrid,
Spain)
Objective: To study the age-dependent changes in glial cultures from
wild type and parkin null mice
(PK-/-).
Background: Parkin mutations produce Parkinsons disease in humans and behavioral abnormalities and disfunction of nigrostriatal
dopamine neurons in mice. Abnormal dopamine processing in young
PK-/- mice is compensated by increased levels of GSH (Itier et al.
Human Mol. Gen. 2003; 12, 2277-2291). Since the glial cells play an
important role in the metabolism of GSH, we have investigated the role
of glia in PK-/- mice.
Methods: Glial mesencephalic cells, from 129SV/C57BL6 WT and
PK-/- mice were cultured for different periods of time (since 15 days to
9 months in culture) in DMEM-FCS.
Results: The study of the glial cultures shows an enhancement of the
proliferation rate of PK-/- cells with respect to WT and an enhancement
of the initial number of microglial cells present in both types of
cultures. The increase of GSH that was observed in the younger glial
cultures (18 DIV to 3 month in culture) disappeared in the older PK-/glial cells (6 - 8.5 months in cultures). Moreover, older PK-/- glial
cultures were more sensitive than old WT glial cultures and PK-/young cells to hydrogen peroxide-induced loss of viability.
Conclusions: The lack of parkin function induces glial dysfunction
that were increased with the time of the culture.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S204 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

POSTER SESSION III

Thursday, June 7, 2007
9:00 AM 4:00 PM
Rumeli Hall, Lower Level
Poster Numbers 663973
Authors Present: 12:30 PM2:30

PM

OTHER CLINICAL
663
High-level gait disturbance is a manifestation of the pyramidal,
cortico-pontine and thalamic-demyelination in elderly people. MRI
pilot study
Y. Balash, M. Kafri, E. Sasson, O. Eizenstein, Y. Assaf,
J.M. Hausdorff, N. Giladi (Tel Aviv, Israel)
Objective: To compare evidence of ischemic microvascular changes
and degree of demyelinative disintegration of white matter at the
subcortical brain areas in patients with HLGD vs. healthy controls.
Background: High Level Gait Disorder (HLGD) is a common disturbance of elderly patients characterized by cautious gait with small
steps, llings of posture and gait instability, fears of falling, and urge
to have a support. HLGD result in severe functional disability. The
pathophysiology of this syndrome is not clear; vascular etiology is
suggested to be basic, whereas leucodystrophy of another origin is
underestimated. The Q-space diffusion MRI technique, which has been
shown to be highly sensitive to changes of brain white matter metabolism, could elucidate the disorder.
Methods: 13 community living non-demented patients with HLGD
(mean age 76.53.6; 9 females) and 9 healthy controls (mean age
74.84.6; 6 females) without history of cerebro-vascular disease were
studied. All patients were neurologically assessed to diagnose HLGD,
and than underwent both a regular MRI and the Q-space diffusion MRI
(high-b value DWI) in diverse sessions. Visual assessment of vascular
brain changes in MRI scans and displacement parameter of the Q-space
intensities in the white matter structures was used for comparison
between the two groups.
Results: Patients with HLGD had signicant white matter hyperintesities presented as bilaterally increased Q-space displacements
(p0.01) along the both pyramidal tracts mostly in the posterior and
anterior horns of internal capsula, cortico-pontine axons from frontal
and prefrontal cortex, and projections from dorso-medial thalamic
nuclei. Subcortical ischemic foci in the MRI imaging were occasional
and scanty, without signicant distinctions in both groups.
Conclusions: These results suggest that HLGD may be a form of
neurodegeneration affecting myelin metabolism in the pyramidal tract,
cortico-pontine bundle and thalamic locomotor regions. Ischemic
changes seem to be lesser important for development of HLGD.
664
Substantia nigra and olfactory function in idiopathic REM sleep
behaviour disorder: A pilot study
V. Gschliesser, H. Stockner, B. Hogl, B. Frauscher, C. Schmidauer,
W. Poewe (Innsbruck, Austria)
Objective: We performed SN echogenicity and olfactory function in
10 patients with idiopathic RBD and compared ndings to data obtained in 100 consecutive normal controls.
Background: REM sleep behaviour disorder (RBD), hyperechogenicity of the substantia nigra (SN) in transcranial sonography (TCS)
and olfactory dysfunction have been suggested as early diagnostic
markers of Parkinsons disease (PD). Olfactory dysfunction has been
reported in patients with idiopathic RBD, but so far no study has
assessed midbrain sonographic ndings in such patients.
Methods: Ten patients with idiopathic RBD conrmed by polysomnography (mean age 62.3 9.8 years) underwent TCS and olfactory

Movement Disorders, Vol. 22, Suppl. 16, 2007

testing. TCS was performed using a 2.5 MHz transducer from both
sides using the temporal approach to display the echogenic signal in the
area of SN. The side of greater value of SN echogenicity of each
subject was used for statistical analysis. The cut-off value dening SN
hyperechogenicity was 0.24 cm2, corresponding to 2 standard deviations from the mean of echogenic area of 100 normal individuals
studied in our ultrasound laboratory (Stockner et al., Mov Disord
2007). Olfactory function was studied using standardized Snifn
Sticks and presented as a TDI sum score, including tests for odor
threshold (T), discrimination (D) and identication (I). Based on an
investigation of more than 1000 subjects TDI score of 30 is dened
as hyposmia (Kobal et al., Eur Arch Otorhinolaryngol 2000).
Results: Hyperechogenicity was found in 2 of 10 RBD patients
compared to 3 of 100 control subjects. Mean SN echogenicity in the
RBD patients was 0.19 0.08 cm2, whereas SN echogenicity of the
control subjects 0.11 0.06 cm2 (p0.005, Wilcoxon test). Furthermore six RBD patients showed an olfactory impairment. Mean TDI
score in the patients with hyposmia was 26.5 2.8. All patients with
SN hyperechogenicity showed also olfactory dysfunction.
Conclusions: Our study conrms previous ndings of olfactory
dysfunction in idiopathic RBD. While impaired olfaction was found in
6/10 patients, SN hyperechogenicity was present in 2/10. The patient
ndings support the notion that hyperechogenicity of the midbrain is
more common in patients at increased risk for PD as compared to
healthy controls.

665
Ipsilateral upper limb asterixis and contralateral parkinsonism
related to putaminal hemorrhage
M.J. Kim, J.K. Kim, B.G. Yoo, K.S. Kim (Busan, Korea)
Background: Involuntary movement such as chorea, dystonia, asterixis,
and tremor, may occur as a consequence of stroke, most often due to
involvement of the basal ganglia or thalamus and subthalamus. We present
a interesting patient who developed asterixis on right upper extremity and
left side parkinsonism after right putaminal intracerebral hemorrhage. Case
Description : A 68-year-old patient with hypertension developed acute left
upper extremity clumsiness and dysarthria on June 7, 2005. Brain MRI
showed huge hemorrhage located on the right putamen. 1 week later,
abnormal movement was developed to the right wrist and ngers, it
consisted of frequent arrhythmic loss of extensor muscle tone on instruction to maintain the wrists and ngers extended, producing intermittent
brief lapses in the assumed posture, and simultaneously, resting tremor and
bradykinesia developed on the right upper extremities. The right side
abnormal movement was corresponed with asterixis. Metabolic screening,
including liver enzyme values and electroencephalogram, was normal. The
right side asterixis disappeared 8 days later.
Conclusions: The unilateral asterixis is seen most commonly in association with ischemic or hemorrhagic disorders, especially those involving
the contralateral ventrolateral or lateroposterior thalamus. An unusual
feature of our patient was that asterixis is developed on the ipsilateral limb
of the putaminal lesion. A previous report of ipsilateral asterixis in patient
with cerebellar lesion was explained by crossing cerebellar-rubral bers at
the superior cerebellar peduncle. The occasional occurrence of bilateral
asterixis and the transient nature of the symptoms suggesting that the
system regulating posture maintenance is not strictly unilateral. The pathogenic mechanism for asterixis remains elusive, and we guessed that an
unknown pathway is related with ipsilateral asterixis. We guessed another
possibility, though the asterixis transiently appeared bilaterally, left side
asterixis was masked or overlooked by prominent resting tremor. Asterixis
is not an uncommon poststroke involuntary movement, and there seems to
be a need for more concern about this symptom.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

666
Botulinum toxin treatment is effective for epilepsia partialis continua
J.-S. Kang, K. Krakow, H. Steinmetz, R. Hilker (Frankfurt/Main,
Germany)
We report on a 60-year-old patient suffering from epilepsia partialis
continua who improved remarkably after botulinum toxin type A
(BTX-A) treatment of the affected arm. Two weeks prior to the rst
injection, the patient developed continuous painful myoclonic jerks of
the right hand resistant to antiepileptic drugs. T2-weighted brain magnetic resonance imaging showed a small hyperintense lesion within the
left precentral gyrus suspicious of low-grade glioma. Combined anticonvulsive treatment with levetiracetam, clobazam and valproate remained without benet. Therefore, a total dose of 200 mouse units
(MU) BTX-A (BOTOX, Allergan, Inc.) was injected into the most
affected right upper limb muscles (60 MU each into M. exor digitorum supercialis and M. exor digitorum profundus, 50 MU into M.
extensor carpi radialis, 30 MU into M. adductor pollicis). Marked
improvement of pain and motor function developed three days after
injection reaching a maximum effect after seven days already. Whereas
motor function and ne nger movements of the right hand worsened
again after a week due to progressive denervation, the painful jerks
completely resolved within eleven days. We suggest that BTX-A
treatment of muscle hyperactivity is an effective therapy of painful
myoclonic jerks in patients with otherwise treatment refractory epilepsia partialis continua.
667
Appearance of Kleine-Levin Syndrome (KLS) after acute infection
K. Dimitrios, K. Konstantinos, B. Aggeliki (Athens, Greece)
Objective: In this study we examine the possibility of KLS appearance after an acute infection.
Background: The KLS is a disturbance of eating behavior, with a
characteristic hyperphagia, periodic hypersomnolence, hypersexuality
and exhibitionism by episodes of hyperphagia. We tried to detect
probable anatomic disorders in relation with post infection KLS patients.
Methods: An 18 years old woman appeared KLS after a pneumonic
infection. A thorough neurological, neurophysiological and neuroradiobiological examination, including MRI and SPECT, was made.
Results: The results certify new ndings, to the basal ganglia and in
temporoparietial lobe. After an anti-inammatory and immunosuppressive treatment the clinical symptoms of KLS backpedaled.
Conclusions: The ndings show that one inammatory illness is
possible to cause the onset of KLS. The fact that the symptoms
recovered after treatment, shows that it concerns to a temporary hypothalamic disturbance.
668
A system for syncronized recording and automatic processing of
video- and biodata les in movement disorder evaluation
E. Nordh, M. Larsson, M. Johansson (Umea, Sweden)
Objective: To create a computerised system for automated video/
data recording and ling processes, as well as for secured digital data
storage.
Background: An important task in Movement Disorder research is to
document the motor performance for later reviewing. This is usually
done through video recordings, sometimes joined with kinematic and
bioelectric recordings, resulting in large amounts of material to be
stored. The video tapes also require considerable physical archiving
space, and structured editing, cataloguing, and retrieval work.
Methods: The system operates with a digital video camera, linked to
a computer together with optional kinematic and bioelectric recording
systems. Descriptive key parameters are entered manually. With a
combined start of all hardwares, data are temporarily stored on the local
computer. Recorded video is then automatically compressed and linked
to other les, and stored in an open source SQL database. This may

S205

reside on a local disk or on a remote central server, to which data are

sent in an encrypted form over a secure network connection.
Results: The system provides easy and time effective collection,
automatic compression and secure storage of patient video records,
automatically linked to optional data les. As all recordings are controlled from the sampling computer, the amount of collected excess
video data is minimised, eliminating the need for raw cutting of the
material in editing softwares. Data can be recalled for further analyses
through the dened key parameters, and new or edited data and les
can also later be re-stored in the database.
Conclusions: The described system provides an efcient tool for
reducing the time needed to edit and organise video information, and
also enables retrieval and modication of the recorded clinical data. It
also provides immediate and secure video storage, eliminating the need
for physical storage of video cassettes. As the material is stored in a
database, data can also be automatically backed up, to prevent data
losses, and can be reached from other digital patient record systems.
The system is being used in a one-center longitudinal prospective study
of kinematics in Parkinsons disease, with requirements of a 5 year
follow-up period, but may easily be adapted for use in decentralised
multi-center studies, with centralised data storage and analyses.
669
High Prevalence of tremor, parkinsonism and neuropsychological
impairment induced by mercury in handmade miners. Andacollo,
IV region, Chile
F. Pancetti, G. Lam, P. Lillo, D. Saez, D. Moraga, S. Corral
(Coquimbo, Chile)
Objective: To determinate if exists higher prevalence of neuropsychological impairment, tremor and parkinsonism in handmade gold
miners exposed to mercury.
Background: Mercury produces broad damage to the nervous system, and this is proportional to the time of exposition. Andacollo is a
chilean comunity of handmade gold miners. They obtain the gold by
amalgam with mercury. Then, the mercury is evaporated to take out the
gold. The inhalation of this vapour is the main way of income to
organism by this metal.
Methods: We selected a population of handmade gold miners exposed to mercury by means of a survey poll. People were subject to
neurological examination, neuropsychological tests to measure executives functions, memory and attention. Also we measured the mercury
level in blood and hair. We compared this population with other people
with similar demographic features and no exposure to mercury.
Results: The data actually obtained reveals a high prevalence of
neuropsychological impairment (54%), tremor (14,2%) and parkinsonism (14,2%) in handmade miners exposed to mercury and high levels
of this metal in their organism. People of Andacollo have a high
prevalence of tremor (13%) and cognitive impairment (8,7%) and
mercury level was higher, too. Control group does not have tremor or
parkinsonism, they have a cognitive impairment in 8 %, but the level of
mercury in these people is not high.
Conclusions: Our data suggests that people exposed to mercury have
a higher prevalence of neuropsychological impairment, parkinsonism
and tremor. This data was correlated with high level of mercury in their
organism.
670
An acquired neuromyotonia case non-associated to thymoma with
signicant clinical improvement after thymectomy
M.A. Sierra-Beltran, U. Rodrguez-Ortiz, J.A. Nader-Kawachi,
M.S. Rodr`guez (Mexico City, DF, Mexico)
Objective: To present an Acquired Neuromyotonia case without any
association to either a Neoplastic disease nor even Thymoma with a
signicant improvement after Thymectomy.
Background: A 27 years old woman suffering Neuromyotony is
presented. The current disease began in 2002 (at age 22) with the
developement of sudden episodes of myotonic movments, which initially lasted aprox. 10 minutes, involving upper limbs distal muscles. In

Movement Disorders, Vol. 22, Suppl. 16, 2007

S206 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

2004, these myotonic episode became incresingly more frequent and
generalized. Some of the episodes resulted in Opistotonus. She was
then admitted to Medica Sur Hospital, image studies were requested
and discarded any cranial pathology as well spinal pathology. Both a
neoplastic disease and a thymoma were discarded. By this time, this
myotonic movements were only responsive to Biperidene and forearms
hypertrophy became also evident. She was medicated with corticoesteroids, however no signicant clinical improvement was yield these
episodes worsened their frequency. For the next 12 months she was
readmitted 11 times because of refractory myotonic episodic recurrent
movements. In October 2005 a thymectomy was performed.
Methods: Image studies (CT cranial, chest and abdominal, MRI
cranial and spinal scans), an electromyography, a muscle and thymic
biposies were requested. Additionaly a determination of serum spec
antibodies titres were required.
Results: The Electromyography showed myokimic discharges. The
ndings of the electromyography leaded to a muscle biopsy and another series of image studies were requested (including CT chest and
abdominal scans). Subsequent studies showed antibodies against muscle cell membrane high voltage gated K channels. The Pathologic
Anatomy reported no sign of Thymic hiperplasia nor even maignant
foci. The patient underwent a signicant clinical improvement after the
Thymus was removed.
Conclusions: A great percentage of Neuromyotonia are mostly acquired. It has been described associated to auto-immume disease, a
thymoma and to Neoplastic state (a s a para-neoplatic syndrome) as
well. This association is evident in this case. It isnt unfrequent to nd
this syndrome without a Thymoma, which doesnt imply hat there is no
thymic involvement. This afrmation is supported by the factof the
post-surgicl clinical remission.

671
Movement Disorders at university hospital emergency room; an
analysis of clinical pattern and etiology
P.H. Lee, J.H. Yoon, H.Y. Park, T.S. Lim, J.Y. Choi (Suwon,
Gyeonggi, Republic of Korea)
Objective: To evaluate the clinical features and etiologies of all
common categories of Movement Disorder seen in the emergency room
(ER) of an urban university hospital.
Background: Movement Disorders in the context of the emergency
room (ER) have been under-recognized and underestimated, despite the
disabling features of the disorders
Methods: We prospectively enrolled adult patients (age 15 years)
who presented with Movement Disorders at ER Ajou University Hospital in Suwon over a period of 12 months. We divided Movement
Disorders according to the presenting phenomenology likely to dominate the clinical presentation, that is, gait disorder, tremor, dystonia,
myoclonus, and acute akinetic crisis and classied a specic etiology in
the individual phenomenology.
Results: In one year, there were 60,002 ER visits; of these, 58
(0.09%) were diagnosed as a primary Movement Disorder. Among
them, the most common clinical presentation was gait disorder (n21,
36.2%), followed by myoclonus (n16, 27.6%), dystonia (n10,
17.2%), tremor (n8, 13.8%), and acute akinetic crisis (n2, 5.2%).
Comparing the Movement Disorders, the mean age of the patients with
dystonia was signicantly lower than that of patients with other Movement Disorders (P 0.001). 37 of the patients (63.8%) had drugrelated Movement Disorders. The contribution of drugs was signicantly higher in patients with dystonia compared with the other
Movement Disorders (P 0.01).
Conclusions: Our study showed that a large proportion of the Movement Disorders seen in the ER are drug-related. Therefore, careful
selection of drugs while prescribing would decrease Movement-Disorder-related visits to the ER.

Movement Disorders, Vol. 22, Suppl. 16, 2007

672
Mirror movements in different neurological diseases
S. Sahin, S. Ayalp, S. Karsidag (Istanbul, Turkey)
Objective: We aim to dene mirror movements (MM) in different
neurologic diseases and try to describe the meaning of this sign.
Background: MM are involuntary movements during the voluntary
movements of the contralateral homologous body parts. Previous studies have suggested that MM may occur in various congenital or
acquired disorders such as Kallmanns syndrome, stroke and hemiparkinsonism.
Methods: We evaluated MM in all patients who were admitted to our
neurology outpatient clinic into the six months period. To investigate
the MM, each hand was tested separately in the four tasks performed 10
times as rapidly as possible with the widest amplitude. Selected items
of the Unied Parkinson Disease Rating Scale (UPDRS) were used for
the evaluation of the regional components of parkinsonism. Barthel
index was used to evaluate of patients with stroke. Also, MMs amplitudes were scorred according to MM assessment scale which is
improved by Espay et al.
Results: We found MM in 10 patients with difrent neurological
conditons such as, Parkinsons disease, essential tremor, stoke and
polymicrogyria. MM have been especially observed in non-affected
hand during the movements of the rigid or paratic hand.
Conclusions: MM were found especially in patients with asymmetric neurological involvement. It may be evidence for increased
ipsilateral primary motor cortex activity and/or reduced transcallosal inhibition. Further investigations including neuroimaging
methods are needed.
673
Three cases of bilateral striatopallidodental calcinosis
E. Lobsien, A. Bick-Sander, S. Eibach, K.-T. Hoffmann,
T. Trottenberg, A. Kupsch (Berlin, Germany)
Objective: To show the clinical variability in bilateral striatopallidodental calcinosis (BSPDC).
Background: BSPDC is characterized by calcication of basal
ganglia (BG) and cerebellum. It can present with different types of
Movement Disorders. Combination with other neurological disorders (seizures, cerebellar signs, cognitive impairment) is common.
Diagnosis is made by examination, imaging and exclusion of symptomatic causes. The cause of idiopathic BSPDC is unknown. We
report three cases of BSPDC, with one unusual early onset and
severe presentation.
Methods: Clinical case series.
Results: CASE 1 (see g.): Male 19y; disease onset at age of 2
with 4 epileptic seizures; Age 4: mild impairment of ne motor
skills; Age 6: mild Ataxia; in the course of the disease development
of generalized dystonia and dysphonia requiring constant care.
Imaging showed calcication of brainstem, hemispheres, BG, thalamus and cerebellum. Therapy for dystonia with tiaprid and levodopa showed no effect. CASE 2: Male 49 y; focal epilepsia since
childhood; Age 48: asymmetric parkinsonism and focal dystonia of
lower extremities as well as impaired postural stability not responsive to levodopa. Imaging showed calcication of BG and cerebellum. CASE 3: male 63 y; Age 57: progressive gait disorder, no
further movement disorders. Imaging showed bilateral calcication
of BG, thalamus and cerebellum. Levodopa had no effect. In all
cases familiy history was negative and sympomatic causes were
excluded.
Conclusions: Idiopathic BSPDC can present with a broad range of
symptoms. In our cases progressive movement disorders led to
further differential diagnosis revealing calcications in BG and
cerebellum. Leading symptom was generalized dystonia in CASE 1,
parkinsonism in CASE 2 and gait disorder in CASE 3. CASE 1 had
an unusual early onset and severe clinical presentation. All cases did
not respond to common treatments. Our cases underline the diversity of clinical manifestations in BSPDC. Further research is needed
to understand the pathophysiological processes of BG dysfunction

POSTER SESSION III, THURSDAY, JUNE 7, 2007

induced by BSPDC and clarify the linkage to a broad clincal variety
of symptoms.

S207

posterior cerebral artery occlusion. The levitating hand disappeared

under penicillin treatment.
Conclusions: Levitating hand accompanied by fever should raise the
suspicion of a posterior lesion of infectious origin such as septic
embolus or abscess.
675
Methylphenidate improves cognition and reduces fall risk in older
adults with cognitive decline: Single dose, placebo controlled, double-blind study
R. Ben-Itzhak, J.M. Hausdorff, E.S. Simon, N. Giladi (Tel-Aviv,
Israel)

FIG. 1 (673).

674
Posterior alien limb phenomenon as presenting symptom of bacterial endocarditis
A. Glik, R. Inzelberg (Kfar Saba, Israel)
Objective: To report an unusual case of a posterior alien hand
syndrome as the presenting symptom of bacterial endocarditis.
Background: Neurological complications of bacterial endocarditis
include septic emboli with focal ischemia or hemorrhage due to the
formation of mycotic aneurysms. The majority of these events occur
in the carotid territory. Other patients show meningitis or toxic
encephalopathy. The neurological presentation often precedes the
cardiologists diagnosis. Posterior alien hand syndrome, sometimes
coined as levitating hand, is a rare phenomenon described in the
present of posterior parietal lesions.
Methods: We describe the clinical details of a patient with acute
leviating hand.
Results: Case report: A 55 year old right handed immunocompetent Caucasian man presented with right limbs weakness and fever.
He had been somnolent for the last three days. On admission he was
disoriented for situation. General physical examination showed normal cardiac sounds and no signs of systemic infection. Neurological
examination revealed dense right heminaopia accompanied by mild
right limb weakness. The reexes were brisk on the right limbs and
the plantar response was extensor on the right. In addition, his right
upper limb showed involuntary levitation. The movements were
purposeless and consisted of elevation of the whole upper limb at
the level of the right shoulder. The patient was aware of their
existence but denied performing them voluntarily. When relocated
at knee level, the hand was quiet, showing normal praxis and no
groping activity. The next day he developed color anomia and alexia
without agraphia. An echocardiograph disclosed aortic valve vegetation. Streptococcus viridans was isolated in several blood cultures.
The patients fullled the major Dukes criteria for denite bacterial
endocarditis. Brain MR disclosed acute infarction in the left temporooccipital area and splenium of the corpus callosum due to left

Objective: To test the hypothesis that a single dose of methylphenidate (MPH) enhances usual walking stride and swing time variability
(markers of fall risk), improves cognitive function, and reduces the
deleterious effect of dual tasking on gait stability.
Background: Impaired executive function (EF) and attention have
been associated with fall risk and gait instability, especially while dual
tasking during walking. Improved cognition may reduce this dual
tasking effect and reduce fall risk.
Methods: A randomized, double-blind, placebo-controlled, crossover study was conducted in 17 non-demented patients (mean age: 75
yrs) with impaired performance in EF or attention, i.e., scores of 90 or
below (2/3 of 1 SD below the age-normed means) on a standardized
computerized neuropsychological battery, and a history of falls or near
falls. Gait and cognitive function were evaluated before and two hours
after a single dose of MPH (20 mg) or placebo, in sessions separated by
about 1 week.
Results: MPH treatment signicantly improved (reduced) stride
time variability and swing time variability, both during usual walking and when subjects walked while performing serial 3 subtractions
(dual tasking). For example, in response to MPH, swing time
variability decreased from 4.40.9% to 3.90.7 % during usual
walking (p0.047) and from 6.82.5% to 3.70.5% during serial
3 subtractions (p0.002). The placebo did not signicantly effect
stride time or swing time variability during usual walking or during
dual task walking. MPH also signicantly improved EF (from
88.92.6 to 95.72.3; p0.001) and attention (from 86.14.4 to
99.82.8; p0.006), while having no effect on memory (p0.92).
Improvement in EF in response to MPH correlated with improvement in gait variability (e.g., r0.60; p0.015 for usual walking
swing time variability and r0.77; p0.001 during serial 3 subtractions).
Conclusions: These results demonstrate the potential of using cognitive-enhancing pharmacologic agents to improve gait and reduce fall
risk.
676
Two cases of primary progressive freezing gait with different
chronologic progression and different imaging
M. Kim, E. Son, S. Choi, K. Lee, S. Lee, M. Park, K. Cho (Gwangju,
Korea)
Objective: Primary progressive freezing gait disorder (PPFG) is a
rare gait disorder characterized by early gait freezing and a stereotyped
progression.
Background: We report two cases of PPFG with different chronologic progression and different imaging.
Methods: A 72-year-old man developed gait ignition failure at age
69. By age three, he developed postural instability with falling and
wheelchair-bound state. He also had mild bradykinesia and rigidity at
left hand. Brain MRI showed diffuse atrophy and Brain SPECT showed
diffuse perfusion decrease, especially at frontal area. A 68-year-old
man presented gait ignition failure three months ago followed by
postural instability and wheelchair-bound state within one year after
rst symptom. He did not have parkinsonian feature. Brain MRI &
SPECT was normal.
Results: The symptoms of the two patients were unresponsive to
dopaminergic medication.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S208 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Conclusions: PPFG might not be a distinct disorder but a syndrome
with diverse causes.
677
The Dynamic Gait Index provides insight into stair climbing and
fear of falling in healthy elderly men and women
T. Herman, M. Brozgol, N. Inbar-Borovsky, N. Giladi, G. Yogev,
L. Grundlinger, J.M. Hausdorff (Tel-Aviv, Israel)
Objective: The purpose of this study was to better understand the
Dynamic Gait Index (DGI), and its relationship to other tests and fear
of falling.
Background: The DGI has been developed as clinical tool to assess
gait, balance and fall risk. Because the DGI evaluates not only usual
steady-state walking, but also walking during more challenging tasks
such as while rotating ones head, while accelerating, and when climbing stairs, it may be an especially sensitive test.
Methods: 222 older adults (mean:76.5/-4.7 yrs; 60% women) were
studied. Measures included the Dynamic Gait Index (DGI), the Berg
Balance test (BBT), the Timed Up and Go (TUAG), the Mini-Mental
State Exam (MMSE), the Unied Parkinsons Disease Rating Scale
(UPDRS) motor portion, the Activities-Specic Balance Condence
(ABC) scale and the # of falls in the past year.
Results: The DGI was moderately correlated with BBT (r0.53;
p0.001), the TUAG (r-0.42; p0.001) and the ABC (r0.49;
p0.001). Scores on the DGI were near perfect in men, but among
women, there was a small, but signicant (p0.001) decrease in the
DGI (22.7/-1.6). Whereas pivoting, head turning, and other DGI
items were not different in men and women, this reduction in the total
DGI score in women was due primarily to stair climbing performance
(p0.001), with many women choosing to walk while holding a
handrail. Scores on the BBT and the TUAG were similar. UPDRS and
MMSE scores were also similar in the two groups. On the other hand,
ABC scores and fall history were different. Still, the gender-difference
in DGI scores persisted if we adjusted for ABC scores or when subjects
were stratied by fall history.
Conclusions: Given its ability to identify subtle changes in performance, the DGI appears to be an especially appropriate tool for
assessing function in healthy older adults. Application of the DGI
indicates that among relatively healthy older adults, men and womens approach to stair climbing is different. While fear of falling and
fall history contribute to this gender-specic attitude towards stair
climbing, other as yet unidentied factors apparently also play a
role. This should be considered when applying the DGI and when
evaluating functional independence and fall risk in older adults.
678
Associated conditions and clinical signicance of awake bruxism
Y.T. Kwak, S.-H. Suk, W.-J. Kim (Yongin-shi, Kyeongki-do, Republic
of Korea)
Objective: Conrming the possible related anatomy and the clinical
signicance of awake bruxism in geriatric hospital is the aim of study.
Background: Awake bruxism is dened as an oral parafunctional
activity that includes clenching and grinding of teeth during wakefulness.
Methods: We analyzed the medical records of 503 patients who were
admitted in hospital from April to June 2005. After the recognition of
bruxism, the clinical, brain imaging studies and statistical parametric
mapping (SPM) of Brain SPECT were done.
Results: (1) In each disease group, 5 among 125 Alzheimers disease(AD) patients(4.0%, 5/125), 3 among 11 frontotemporal dementia(FTD) patients(27.3%; 3/11), 7 among 230 stroke patients(including
two patients related with citalopram, 3.0%; 7/230), 1 among 45 Parkinsons disease patients(2.2%; 1/45), and 4 hydrocephalus patient(23.5%; 4/17) had bruxism. (2) Even though awake bruxism occurred early after stroke onset, it occurred late after AD and FTD onset.
This occurred in far advanced stage of AD, while it occurred in
moderately advanced stage of FTD and stroke. (3) SPM analysis in AD
and FTD patients with awake bruxism revealed signicant hypoperfu-

Movement Disorders, Vol. 22, Suppl. 16, 2007

sion in frontotemporal and other subcortical structures.(4) Surface

EMG recordings from the masseter muscle showed rhythmic regular
motor activity at a rate of 1-2/second.
Conclusions: This study suggests that awake bruxism is encountered
in various diseases in geriatric hospital. It is closely related frontotemporal lobe dysfunction in degenerative dementia like FTD and AD.
Therefore it can be regarded as one of marker of frontal neurological
sign in degenerative dementia.
679
Nordic walking improves mobility in Parkinsons disease
F. Vereijkeren, R. Reijmers, A. Minten, J.P. ter Bruggen
(sHertogenbosch, NB, Netherlands)
Objective: Whether a standardized training program, according to
the Nordic walking (NW) principles, was safe and able to ameliorate
locomotion and improve quality of life. To our knowledge no such
evidence-based studies were reported.
Background: The usefullness of physiotherapy in IPD is questionable. Evidence based studies are rare. NW might be a suitable clinimetric method for magnifying armswing, steplength and less postueral
problems.
Methods: A cohort of nine Dutch Parkinson patients (IPD), was
included in a pilot study. The group consisted of six males and three
females. Age (59-76 yrs, mean 67 yrs.) and severity varied (HY 1-3
, mean 1.6). Cognitive function and condition were within normal
limits. Therefore, two physiotherapists, members of the ParcNet
group, were educated and qualied as NW instructor. According to
a standardized training protocol they instructed the group NW
exercises, twice a week during one hour sessions. The same physiotherapist scored the patients. Parkinson medication was unchanged during the testing period. At T1 (entry) and T2 (after six
weeks) IPD patients were investigated as follows. PDQ, six minutes
walking test, ten minute m walking test, Time to Get up Go were all
scored on both occassions. At the end, a self-rating questionnaire
was performed.
Results: NW seems to be safe and use of the sticks attibutes to
prevent falling. The mean walking distance over six minutes even
substantially improved by 30% from 388 in 531 meters. The change in
10 meters walking was not that impressive. Some improvement was
even measured in time to stand-up. The improvement in QoL (PDQ 39)
was remarkable as well. Before mean 20.5, afterwards mean 18.5. One
patient suffering motor complications was trained to tap her stick as a
trick, to overcome freezing. Arm swing intensied after NW. On
self-rating NW group training is believed to be superior to previous
individual based physiotherapy programs.
Conclusions: Our data suggest that after a six-week training course,
using a standardized NW method, mildly affected IPD patients were
able to improve their mobility especially on longer distances. Time to
stand-up seems to improved too. This resulted in a better QoL. Whether
we might achieve long-term positive locomotion effects, persisting
changes in motivation or lifestyle or perhaps changes in motor processing, remains to be investigated.
680
A progressive, fatal dystonia-parkinsonism syndrome in a patient
with primary immunodeciency receiving chronic IVIG therapy
S. Papapetropoulos, J. Friedman, C. Blackstone, G.I. Kleiner,
C. Sengun, C. Singer (Miami, Florida, USA)
Objective: To describe and present a video of a 14-year old patient
with Brutons X-linked agammaglobulinemia (XLA) that developed a
fatal dementing, dystonia-parkinsonism syndrome after 13 years of
continuous IVIG therapy.
Background: The primary immunodeciency disorders reect abnormalities in the development and maturation of cells of the immune
system. Neurologic complications of agammaglobulinmia are primarily
related to CNS enteroviruses infections due to the lack of neutralizing
antibodies. Recent studies have identied a growing number of XLA
patients that developed unexplained central nervous system (CNS)

POSTER SESSION III, THURSDAY, JUNE 7, 2007

deterioration with extrapyramydal clinical manifestations without evidence of enteroviral infection.
Methods: The patient had been on a monthly IVIG (500mg/kg) for
13 years. During year one he developed behavioral changes, progressive speech difculties and gait disturbance. By year two he required
help with all activities. At that time saccades were slow, there was
eyelid opening apraxia, hypokinetic articulation/marked hypophonia
with unintelligible speech, left-sided torticollis, left dystonic foot inversion, bilateral upper limb rigidity and bradykinesia, start hesitation,
frequent gait freezing and lower limb spasticity (left-sided predominance-see video) . Trials with L-dopa and benztropine failed to control
symptoms.
Results: Genetic screening, serum and CSF studies for inammatory,
metabolic, infectious and neurodegenerative disease markers were negative. EEG was relatively normal with occasional slow waves. Brain
MRI revealed bilateral brain atrophy more prominent in the temporal
lobes and showed bilateral non-specic T2 pallidal hyperitensities.
Asymmetric hemispheric glycolytic activity, diffusely decreased on the
right compared to the left was noted on uorodeoxyglucose-PET. The
patient died of disease-related complications six years after symptom
onset. For unknown reasons no autopsy was performed on the patient
that died outside the USA.
Conclusions: The etiology of the neurodegeneration associated with
primary immunodeciency and chronic IVIG use is unknown. We
suggest that physician awareness of this syndrome and close neurological monitoring of patients receiving IVIG may lead to its better
characterization and management.
681
New assessment concerning movement signs/symptoms in atypical
movements diseases consulted in units-setting. Treatment rationalizing
S.G. Echebarria (Spain)
Objective: Consider useful and day-to-day assessment in atypical
movement disorders in proper movement disorders units and treatment
considerations.
Background: Recent standpoints around movement disorders typologies and classication have been based on categorical and / or axial
work-up steps: -Non-typical tremor,emerging myoclonus and pre/post
treatment dyskinesias in Plus diseases -Painful/painless limb movements with/without peripheral nerve myokymia /fasciculation/conduction signs -Spasm/contraction denitions :Phasic/dynamic character
-Axial /truncal/head-spine exion and bent pre- and post-movement
conditions.
Methods: Latent Trait theory - IRT applied to rule induction and
response-based segmentation.Chi square applied to categorical-variables.
Results: Peripheral,limb,diaphragmatic myoclonus, Focal myoclonus , Spasm RSD I-II , Tonus expression, Behcets/Sjog., Cortical
Myoclonic expression ,myoclonus ,expanded Jerky condition ,
forms , PLMT -myokymia, carpopedal spasm Contractures PD
subtypesoscillant amplitude assoc.movs, Plus types ( MND)
*_tauopathies, Treatment segmental groups :, DDD - Dosage / ind /day
*10 5.
Conclusions: Typical /atypical spectrum:Segmental Dyst. plus/Essential myoclonus/Overow hyperkinetic movements
Hypertrophy/Myokymia/Pseudomyotnia
Peripheral neurop.
682
EEG comparison in early AD, LBD, PDD patients with a 2-year
follow-up
L. Bonanni, A. Thomas, L. Manzoli, M. Onofrj (Pescara, Italy)
Objective: The primary aim of the present study was to determine
whether the EEG alterations historically described in dementia or AD
are differentiated in the three clinical entities (AD, LBD, PDD) The
second endpoint was to evaluate whether these differences are merely
statistical or might express a cut-off level.

S209

Background: In the last revision of criteria for the diagnosis of Lewy

body disease (LBD), EEG abnormalities with transient slow waves or
sharp waves were also reported as supportive features for the diagnosis
of LBD. However, a close examination of the literature cited in the
paper revealed that the few papers on EEG recordings in LBD included
anecdotal reports or inconclusive ndings.
Methods: In 40 AD, 36 LBD, 35 PDD patients with initial cognitive
decline (MMSE 20-25/30,ADAS-cog 37/70) EEGs were recorded
and analyzed by visual inspection and several quantication methods.
Patients were assessed for the presence of cognitive uctuations, neuropsychiatric symptoms, REM-sleep-behavior-disorder. Clinical and
consensus-based diagnoses were conrmed by a 2 year follow-up.
Results: The most signicant differences were observed in EEGs
from posterior derivations, separating LBD from AD patients(p0.001). Dominant frequencies were 7.4 (1.6) in LBD patients,
8.3 (0.6) in AD patients, and in 88% of LBD patients a frequency band
at 5.6-7.9 Hz was identied. DF variability was also different in
LBD(1.81.2 Hz) in comparison with AD (1.10.4 Hz). Differences
were best evidenced when DF, and DFV were represented in Compressed-Spectral-Arrays showing EEG changes across time, with visually interpretable salient patterns. At follow-up abnormal EEG on
posterior leads was prevalent in LBD (100%) and increased in PDD
(74.3%). Absent alpha and 5.6-7.9 DF with DFV differentiated early
LBD from AD. In early PDD these ndings were observed in a
minority of patients, but became prevalent at follow-up.
Conclusions: The denite presence of EEG abnormalities early in the
course of LBD, with a core feature evidencing marked variability of
dominant frequencies in posterior derivations, occurring every few
seconds, or with the substitution of alpha with frequencies at 5.6-7.9
Hz, suggests that centers regulating EEG rhythms in parieto-occipital
areas are affected in the early course of this disease.
683
Atypical PKAN, a broadening clinical spectrum: Case report and
video
N. Lubarr, S. Frucht, S.K. Westaway, A. Gregory, S.J. Hayick (New
York, New York, USA)
Objective: Pantothenate kinase-associated neurodegeneration
(PKAN) refers to the form of neurodegeneration with brain iron accumulation (NBIA) associated with PANK2 gene mutations. It has a
pathognomonic MRI nding of globus pallidus central hyperintensity
with surrounding hypointensity on T2-weighted images, known as the
eye-of-the-tiger (EoT) sign. Patients with PANK2 mutations can be
classied as having either classic or atypical disease. The classic
phenotype has early age of onset and rapid progression of disease, and
the atypical phenotype has a later age of onset, slower progression of
disease, and a heterogeneous spectrum of clinical presentations. We
report a case of atypical PKAN in a 49 year-old man, with onset at age
14 of balance difculties, followed by dysarthria in his early 20s. By
his early 20s he was unable to walk independently due to spontaneous
retropulsion, and the dysarthria ultimately progressed to near anarthria.
He also had signicant dysphagia, dystonic spasms, mild facial and
limb dystonia, mild Parkinsonism, and hyperreexia, with no cognitive
or psychiatric symptoms. He had been treated with clonazepam with
improvement of the dystonic spasms, and a prior trial of baclofen had
not been benecial. We started trihexyphenidyl, which gave some
improvement to the dysphagia and retropulsion. MRI showed the EoT
sign. Sequencing of PANK2 showed a previously reported missense
mutation (1253C-T) and a novel nonsense mutation (606T-A). The
clinical phenotype of atypical PKAN continues to be broadened. Our
patient had several features consistent with prior descriptions of atypical PKAN, including severe dysarthria, Parkinsonism, and a notably
long duration of disease, but also had loss of independent ambulation
within 10 years of onset, and presented with gait impairment, both
considered more characteristic of classic PKAN. A primary clinical
feature was dysphagia, which has not been noted in previous patients.
The presence of a PANK2 nonsense mutation is rare in atypical PKAN,
and could perhaps account for the features that are more common in
classic disease. Trihexyphenidyl, which has been used to treat dystonia

Movement Disorders, Vol. 22, Suppl. 16, 2007

S210 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

in classic PKAN, provided some benet to our patients dysphagia and
gait disorder, and should be considered as a treatment option in all
PKAN patients.
684
Normal interhemispheric inhibition in persistent developmental
stuttering
M. Sommer, K. Knappmeyer, E.J. Hunter, A. Wolff von Gudenberg,
W. Paulus (Goettingen, Germany)
Objective: To explore the interhemispheric inhibition in patients
with persistent developmental stuttering (PDS).
Background: PDS affects about 1 % of the postpubertal population,
predominantly males. Imaging studies suggest a right hemispheric
overactivity of motor and premotor areas in patients with PDS. The
interhemspheric inhibition studied with transcranial magnetic stimulation is an established tool to study the interplay of motor areas.
Methods: We studied 15 male patient with PDS (average age 26.7
(standard deviation 8.9) years) and 13 male control subjects (26.3 (4.8)
years), excluding patients with cluttering or control subjects with a
previous or family history of stuttering or cluttering. We studied the
interhemispheric inhibition using (1) a one-coil technique over the hand
area of one motor cortex during voluntary activation of the ipsilateral
abductor digiti minimi muscle. Here, stimulus intensity was adjusted to
yield MEP amplitudes of about 2 mV contralaterally, and the duration
of the induced ipsilateral silent period obtained from 30 rectied trials
was measured and compared between groups using unpaired, twotailed t-test. In addition (2), we used a two-coil technique with the
conditioning pulse over the hand area of one and the test pulse over the
hand area of the other hemisphere while the hand muscles were at rest.
The test pulse was adjusted to yield amplitudes of about 1.0 mV, and
the conditioned pulse to yield amplitudes of about 1.5 mV. We studied
interstimulus intervals of 2, 5, 6, 8, 10, 20, 50, and 80 ms 10 times each,
and unconditioned test stimuli 20 times. Here, the amplitude of the
motor evoked potential (MEP) recorded in the abductor digiti minimi
muscle contralateral to the test pulse was measured and normalized to
the unconditioned amplitudes.
Results: There was no signicant between-group difference for interhemispheric inhibition either in the one-coil design or in the two-coil
design.
Conclusions: These data suggest that the interplay between the motor
cortices is normal in patients with PDS. This is consistent with a normal
intracortical inhibition previously described in PDS patients. The abnormal right motor and premotor activity observed in imaging studies
on PDS are not likely to reect altered primary motor cortex excitability, but are likely to have a different origin.
685
Patients with hyperhidrosis treated with botulinum toxin have
changed grip force, coefcient of friction and safety margin
T. Zackrisson, B. Eriksson, N. Hosseini, B. Johnels, A.L. Krogstad
(Gothenburg, Sweden)
Objective: We wanted to evaluate if subjects with pathologically
increased sweating in the palms (primary focal hyperhidrosis) have
functional problems with handgrip caused by the wet slippery surface
of palm and ngertips. To this aim we measured the muscle strength
using a object equipped with force sensors and calculated the coefcients of friction and safety margin of the precision grip before and
after treatment with botulinum toxin. We used two different dosages of
botulinum toxin to explore the impact on sweating and on the muscle
strength in the hand.
Background: The ability to grasp, hold and handle objects of different sizes, structures and weight is important for many activities of daily
life. It is also a prerequisite for identication of small objects. Smaller
objects are handled by the precision grip between the thumb and the
index nger. When an object is lifted up, the grip force is automatically
tuned to the lifting force of the hand so that fragile objects can be
handled without crushing them (e.g. picking a berry). When handling
objects or transporting them in space, tactile information from glabrous

Movement Disorders, Vol. 22, Suppl. 16, 2007

skin is essential both for rened manual manipulation, but also for the
explorative function of the hand. Primary focal hyperhidrosis is a
common and often hereditary condition with excessive sweating especially on the palms, soles and/or in the axillae. To have sweat literally
dripping from the hands may present considerable social, psychological
and occupational problems as well as give rise to great difculties in
many daily activities such as holding a pen or manipulating small
objects.
Methods: Repeated measurements of evaporation, grip force and
safety margins were made on 13 patients before and 2, 4, 6, 8 10-12
weeks and 6 months after treatment with botulinum toxin.
Results: We found a signicant decrease in evaporation and a parallel reduction of grip force in the dominant hand of the patients. The
safety margin used by the patients was signicantly higher before than
after the treatment, and increased gradually when sweating reappeared.
Conclusions: These measurements showed for the rst time that
hyperhidrosis of the palms may cause an objective perturbation of the
hand function which may partially be corrected by botulinum toxin
treatment.
686
Long-term follow-up of deep brain stimulation: Timing of generator replacement
M. Takanashi (Sapporo, Hokkaido, Japan)
Objective: Deep brain stimulation (DBS) has widespread in the
treatment of various movement disorders and the other diseases. However, treated patients need the surgical replacement of the pulse generator because of the battery depletion. Here, we examine the relationships between the timing of generator replacement and the changes in
functional outcome as well as changes in stimulation parameters.
Methods: Twenty-nine patients received DBS for 25 Parkinsons
disease (PD), 2 dystonia and 2 intractable pain (IP) and were followed.
Seven of 29 patients underwent generator replacement and were included in this study (5 PD, 1 dystonia and 1IP). Mean time between
generator implantation to replacement was 52 months (range 27 to 66
months).
Results: In 3 out of 7 patients, symptomatic deterioration occurred
before generator replacement and their batteries were exhausted almost
completely. Stimulation using double electrode contacts as the cathodes rapidly depleted the batteries (within 27 and 37 months). All
patients recovered after replacement. Four patients needed to reduce the
stimulation voltage and 3 patents required higher power output.
Conclusions: Various stimulation parameters (electrode contact, frequency, pulse width, voltage) participate in the depletion of the pulse
generator. And the exhaustion of the generator result in the symptomatic deterioration of treated patient. DBS with the higher power output
rapidly exhausts the battery and requires surgical replacement of the
generator in a short interval.
687
The other babinski sign in hemifacial spasm
W.P. Stamey, J. Jankovic (Houston, Texas, USA)
Objective: To assess the frequency of the other Babinski sign among
patients with hemifacial spasm (HFS), and determine its use in differentiating HFS and blepharospasm (BSP).
Background: The other Babinski sign, described by Joseph Babinksi
in 1905, is manifested by simultaneous eye closure and eyebrow
elevation. The utility of the sign in differentiating HFS and BSP has not
been systematically studied. We sought to characterize the frequency,
sensitivity or specicity of this nding.
Methods: Patients presented to the Baylor College of Medicine
Movement Disorders Clinic were diagnosed with HFS (n75; 45
women) or BSP (n73; 50 women), and videotaped. HFS was clinically diagnosed with the presence of unilateral involuntary facial muscle contractions affecting one or more muscle groups innervated by the
ipsilateral facial nerve. Patients with BSP had bilateral contraction of
periorbital muscles without other associated facial or oromandibular
dystonia. A patient was considered positive for the presence of the

POSTER SESSION III, THURSDAY, JUNE 7, 2007

other Babinski sign if the videotape showed unilateral contraction of
the frontalis muscle, causing eyebrow elevation, with concurrent contraction of the ipsilateral orbicularis oculi muscle, causing eyelid closure. Patients with unilateral frontalis contraction but not concurrent
eye closure were excluded, as such contraction may have been compensatory (voluntary) as in patients with BSP, rather than involuntary
as typically observed in patients with HFS.
Results: The other Babinski sign was present in none of the BSP
patients but was evident in 19 (25.3%) of the HFS patients (10 female).
The sensitivity of the other Babinski sign as a test of the presence of
HFS was 25.3%; whereas specicity for the presence of the sign,
compared with BSP, was 100%.
Conclusions: The other Babinski sign, found in 25% of our cases of
HFS, but not in any of the cases of BSP, is an under-recognized
physical sign which can be used to distinguish HFS from BSP.

FIG. 1 (687).
688
Electroconvulsive therapy for depression in a patient with rightsided VIM DBS
V.C. Chang, D. Hardesty, B. Ford, P. Greene (New York, New York,
USA)
Objective: To describe a case of a patient with a right ventral
intermediate thalamic deep brain stimulator for Parkinsons disease
tremor who required ECT treatment for major depressive disorder.
Background: Electroconvulsive therapy is an established and effective treatment for severe and medication-resistant depression. To date
there are only 2 case reports of ECT treatment in patients who also have
deep brain stimulators one with essential tremor and the other with
advanced Parkinsons disease. The number of patients with deep brain
stimulators for various movement disorders is growing and the percentage of movement disorders patients with depression is signicant.
A recent study found that 48% of PD patients, 37.3% of dystonia
patients, and 34% of ET patients were depressed. Treatment options for
these patients who have symptoms refractory to medications are limited.
Methods: A 63 year-old man with a history of recurrent major
depressive disorder without prior suicide attempt or psychotic features,
and also a diagnosis of Parkinsons disease for which he received right
VIM DBS in July 2005 presented with worsening depression refractory
to medication adjustment. He had a history of a successful response to
ECT which had been performed in 2000 prior to his deep brain
stimulator placement. Impedance of each contact was tested to ensure
integrity of the electrode, the device parameters were set to zero, and

S211

the stimulator was turned off. Ultra-brief right unilateral ECT was
administered. One ECT paddle was placed approximately 5 centimeters
anterior to the exit of the DBS electrode from the skull, and the other
was placed more medially than normal to avoid the electrode as it
traveled around the ear. He received a total of 6 ECT treatments over
a period of 2 weeks and had signicant improvement in his mood
allowing him to return to work shortly thereafter.
Conclusions: ECT appears to be a safe and effective treatment option
for severe depression in Parkinsons patients who have deep brain
stimulators.
689
Clinical and electrophysiological features of 12 patients with painful legs and moving toes
M.V. Alvarez, V.G. Evidente, E.D. Driver-Dunckley, J.N. Caviness,
C.H. Adler (Scottsdale, Arizona, USA)
Objective: To characterize the clinical and electrophysiological features in 12 patients with painful legs moving toes (PLMT) and variants.
Background: PLMT was rst described by Spillane etal., which later
expanded to include hand movements (Painful Hands Moving Fingers,
PHMF). Painless variants were later identied: Painless Legs Moving
Toes (P-LMT) & Painless Hands Moving Fingers(P-HMF). Descriptions of PLMT are scarce, with a few case reports & a case series.
Methods: A retrospective review of the Movement Disorders database at Mayo Clinic Arizona from 1996-2006 for patients diagnosed
with PLMT & variants.
Results: Of 4,780 database patients with movement disorders, we
identied 12 cases (ve men, seven women): eight PLMT, two PLMT/
PHMF, one PLMT/P-HMF, one P-LMT. nine bilateral & three unilateral movements consist of exion-extension of toes/ngers, & sometimes exion-extension of foot. 5/12 lumbosacral MRI showed
degeneration. 8/12 electromyography(EMG): 5 polyneuropathy, 2
L5-S1 radiculopathy & 1 normal (had P-LMT). 12 patients had neuropathic/radiculopathic symptoms/signs, with presumed etiologies: 4
lumbar stenosis, 3 Sjogrens, 2 diabetes (1 also had IgG monoclonal
gammopathy), 1 perphenazine (presumed tardive), 1 B12 deciency &
1 presumed lupus. 6/12 underwent surface EMG studies. These showed
semi-continuous irregular EMG bursts lasting 80-1000 ms in the extensor digitorum brevis, tibialis anterior & medial gastrocnemius, with
cocontracting pattern most commonly. There were semi-rhythmic or
pseudo-rhythmic brief runs of 2-9 Hz frequency. Movements could be
partially suppressed at will & were diminished but persistent during
light stages of sleep. Three had improvement with gabapentin, three
others with pregabalin. The patient with P-LMT opted to have no
treatment & the presumed tardive PLMT switched to quetiapine.Other
treatments tried unsuccessfully include antidepressants, analgesics, antiepileptics, steroids, & TENS.
Conclusions: This current series of 12 PLMT patients & variants
represents the second largest in literature. Most common triggering
factor preceding PLMT was pain due to neuropathy/radiculopathy
(7/12.) Surface EMG studies show semi-rhythmic or pseudo-rhythmic
brief runs of 2-9 Hz frequency that persist during light stages of sleep.
Gabaergic agents are most effective in treating pain & concurrent
movements.
690
A study on the effects of botulinum toxin A, Botox and Dysport,
in patients with hemifacial spasm
N. Wan Yahya, N. Mohamad Ibrahim, R. Sahathevan, H. Basri,
R. Azman Ali (Kuala Lumpur, Malaysia)
Objective: To determine and compare the efcacy, duration and
safety of two preparations of botulinum toxin A, Botox and Dysport, in
the treatment of hemifacial spasm in the Malaysian population.
Background: Botulinum toxin is an established treatment for hemifacial spasm. The two commercially available preparations differ in
manufacture, units of measure and potency.
Methods: Single-blind, randomised study over 16 weeks. Forty-eight
patients with hemifacial spasm attending the Hospital University Ke-

Movement Disorders, Vol. 22, Suppl. 16, 2007

S212 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

bangsaan Malaysia outpatients clinic were randomised to receive either
Botox or Dysport at a standardised dose and at predened sites. A
conversion ratio of 1:5 of Botox to Dysport was used. Patients were
assessed with the Burke-Fahn-Marsden (BFM) dystonia rating scale at
two, four, eight and sixteen weeks, and with the EQ-5D quality of life
questionnaire and the visual analogue scale (VAS) at baseline and at
sixteen weeks.
Results: Forty-eight patients were enrolled. The mean age was 57.2 (SD
10.24) years and the median duration of symptoms was 3.0 (IQR 2.75)
years. The mean BFM scores improved signicantly compared to baseline
in both groups with a peak response at two weeks (Botox, p0.0005 and
Dysport, p0.02). This was sustained for up to 16 weeks. There were no
signicant differences in the BFM scores between the Botox and Dysport
groups at any time. The EQ-5D and VAS did not show any signicant
difference before and after treatment at 16 weeks. Ptosis was the most
common adverse event, followed by facial paresis (33% and 17%, respectively). Although these appeared to be more common in the Dysport group
(60% compared to 39% reported by the Botox group), it was not statistically signicant (p0.248). Facial paresis occurred in seven patients in the
Dysport arm compared to only one in the Botox arm. This difference
veered towards signicance (p0.05).
Conclusions: Botox and Dysport, in a conversion ratio of 1:5, were
equally effective in the treatment of hemifacial spasm in the Malaysian
community, with a peak response at two weeks and the duration of
benet lasting up to 16 weeks. The higher incidence of adverse events
in the Dysport group limits further increase in the conversion ratio and
perhaps should be maintained at 1:5 or lower, at least in the Asian
population.
691
New and associated motor signs in movement disorders units case
sampling
S.G. Echebarria (Spain)
Objective: Considering different Health Providers in altered movements assessment (VA ,Medicaid , OOP) , it would be possible to
obtain growing percentages in typical and atypical rigid series descriptions, with increasing gures in hyperkinetic / hypokinetic typologies
(new hillmarks would be obtained if we describe complementary or
associated movements in these casuistics (myotonia, myokymia, conduction signs, trophism,contraction, twitchs, head-trunk-limb isolated
movements).
Background: Partial percentages have been obtained in grouping and
sizing stepping methods in r Spearman ranks and multivariant logistic
methods.
Methods: Ranking and ruling observational and unobserved repeated
events. Multilevel loglinear models. Discrete choice models.
Results: Segmental motor paresias, Limb / facial myokymia, Parkinsonism / cramp segmental types, Axial spasms, Pseudomyotonia, Sustained muscle ber activity laryngeal spasm, Dyst-plus subtypes,
Overow segmental myoclonus and essential myoclonus.
Conclusions: Perspectives and considerations are being obtained in
new diagnosis and treatment applications in case sampling including
motor evolving types from usefulness standpoint in movement disorders units.
692
Prevalence of movement disorders in Orhangazi district of Bursa,
Turkey (a population-based door to door study) (Bursa, Turkey)
M. Zarifoglu, S. Erer, N. Karli, A. Ozcakir, A. Semra, N. Caliskan,
D. Aslan (Bursa, Turkey)
Objective: To determine the prevalence of movement disorders
among in 40 years and older population in Orhangazi district of Bursa
in Turkey.
Background: The prevalence rate of movement disorders have been
reported mostly variable according to prevalence studies.
Methods: This population based study was planned in 3 phases. In
phase 1; door to door home interviews were performed by neurology
and family medicine residents using a short questionnaire, in phase 2;

Movement Disorders, Vol. 22, Suppl. 16, 2007

suspected participants in the rst were examined and videotaped. In this

phase cases were evaluated according to the scales for tremor, RLS,
Parkinsons disease and hemifacial spasm by movement disorders
experts and a diagnosis has been made. In the phase 3 video records of
all diagnosed cases were reviewed and nal diagnosis were made by
agreement of all three experts.
Results: This study has been performed between May 2004 and August
2006 in Orhangazi district of Bursa, Turkey. A total 1388 subjects were
planned for screening. Of the target population has been screened according to the study questionnaire. 510 cases with suspectful symptoms of
movement disorders and parkinsonism have been detected in phase 1
(tremor; 247, trembling of hands: 274, bradykinesia: 32, bradymimia; 17,
rigidity: 28, dystonia; 2, hemifasial spasm; 6). 131 of suspectted cases
could not be evaluated in the second phase for various reasons. We dened
that 60 cases with RLS, 57 cases with tremor, 13 cases with PD, 6 cases
with HS, 2 cases with dystonia in phases 2 and 3.
Conclusions: Although ET is the most common movement disorders,
RLS had a higher prevalence than ET in our study. The prevalence of
ET was 2,25 %, RLS was 6,428 %, PD was 1,393 %, HS was 0,428%,
dystonia was 0,214 % respectively.
693
Miraxion treatment for Huntingtons disease a 2 year follow-up
A. Clarke, B.R. Leavitt, M. Manku, A. Rosenblatt (Oxford,
Oxfordshire, United Kingdom)
Objective: (1) To examine the longer-term (24 months) effects of
Miraxion in the treatment of Huntingtons disease (HD) and (2) to
examine the effects of switching placebo-treated patients to Miraxion
treatment.
Background: Miraxion is ultra-pure ethyl-eicosapentaenoic acid
(ethyl-EPA). The effects of 6 and 12 months Miraxion treatment (2g
per day) on motor function measured using the Total Motor Scale 4
(TMS-4) sub-scale of the Unied Huntingtons Disease Rating Scale
(UHDRS) were previously determined in a placebo-controlled doubleblind study involving 135 patients with HD.
Methods: After 12 months double-blind therapy, patients were
given the option to continue with open-label treatment: those previously treated with placebo were switched to Miraxion. Patients
were followed up after 18 and 24 months. Efcacy was measured
using the TMS-4.
Results: From 135 patients randomized in the original study, 121
completed 12 months double-blind treatment. Of these, 120 elected
to continue treatment. One hundred and ten (110) patients completed a further 12 months of treatment (53 received Miraxion
throughout and 57 switched from placebo to Miraxion). Progressive
improvements from baseline of up to 11.2% were seen in TMS-4
over 24 months of Miraxion treatment with no evidence of a
deterioration of response over this timescale. Placebo-treated patients showed no signicant change in TMS-4 over 12 months.
However, once switched to Miraxion, these patients demonstrated
an improvement (8.9%) similar to that of the group treated with
Miraxion throughout.
Conclusions: Our results suggest that the shorter-term benets of
Miraxion in HD are sustained in the longer term. The response seen in
placebo patients after switching to Miraxion, support earlier ndings
that Miraxion improves motor function within 6 to 12 months.
694
A new test to measure upper limb apraxia (TULIAS): A reliability
study
B. Van Hemelrijk, T. Vanbellingen, A. Van de Winckel,
W. De Weerdt, S. Bohlhalter (Tschugg, Switzerland)
Objective: The aim of the study was to assess inter-rater reliability of
a comprehensive clinical test of upper limb apraxia (TULIAS) recently
developed in our clinic.
Background: Apraxia is increasingly recognized as a disabling feature of various Movement Disorders, making a reliable assessment
important for both clinical evaluation and research protocols. Cur-

POSTER SESSION III, THURSDAY, JUNE 7, 2007

rently, few clinical scales are available and they do not cover all motor
and behavioural aspects of praxis production.
Methods: A cohort of fty-two patients with either left or right
hemispheric stroke was prospectively included in the study. Patients
were evaluated by their non-paretic arm. The TULIAS test consists of
48 items including imitation and pantomime of transitive (tool use),
intransitive (communicative) and meaningless gestures corresponding
to 6 subtests. The gestures were balanced for proximal and distal,
simple and repetitive and for movements towards and away from the
body. The assessment required about 15 min. All movements were
recorded on video and scored by two investigators independently. A six
point scoring method was used. The maximum score for the complete
test was 240, the patients reaching a mean total score of 171 41
(standard deviation). Inter-rater reliability was calculated by intra-class
correlation coefcient (ICC) and weighted Kappa statistics. Cronbachs
Alpha was used to measure internal consistency.
Results: The ndings demonstrated a good to excellent inter-rater
reliability for total score and the 6 subtests (ICCs 0.80). For interrater reliability at item level (n48) all but 5 items had acceptable to
excellent degrees of agreement (weighted kappas 0.60). Internal
consistency was good for 4 subtests (alpha values 0.70), and moderate (0.65) to less consistent (0.58) for 2 subtests.
Conclusions: These results of the present study show that the TULIAS test is a reliable instrument to systematically assess upper limb
praxis, justifying further validation. The test can be easily applied and
is therefore useful in clinical practice and for research purposes. The
test will be further evaluated in healthy subjects to determine cut-off
scores for apraxia.
695
Clinical trial participation in Movement Disorders: Why do patients accept or reject?
M.P. Silverstein, C.E. Jacobson IV, M.S. Okun, R.L. Rodriguez,
H.H. Fernandez (Gainesville, Florida, USA)
Objective: To evaluate the reasons which encourage or discourage
patient participation in clinical trials.
Background: Knowing the factors that encourage and discourage
participation in clinical trials may improve the recruitment of subjects
especially as more agents are developed for the treatment of movement
disorders.
Methods: All patients seen and offered to participate in any clinical
trial (i.e. drug, device or surgical) at the University of Florida Movement Disorders Center were asked to ll out a questionnaire. The
questionnaire was comprised of 36 items looking at demographics and
factors deemed important in their decision to participate in clinical
trials.
Results: 39 consecutive movement disorders patients (26 males and
13 females) over 6 months participated in this survey. 71.8% (28/39)
decided to participate in the clinical trial, 17.9% (7/39) said they would
think about it and 10.3% (4/39) chose not to participate in the clinical
trial. Of the trials offered: 55.3% were drug trials, 39.5% were device
trials and 5.3% were surgical. 73.1% of the patients that accepted and
75% of patients who rejected participation stated their doctor spent
10-30 minutes offering the trial. 64% of the patients who accepted and
75% of the patients who rejected claimed they were very satised
with their doctors explanation of the trial. Of the patients in agreement
to participate, 78.6% strongly agreed that the hospitals reputation
was a factor in deciding to participate, 64.3% strongly agreed the
doctors reputation, 60.7% strongly agreed means of a cure and 50.0%
were neutral about clinical care at no charge. Of the patients who
rejected participation, 25% strongly agreed they need immediate
treatment and cannot wait for treatment and 50% agreed their disease
is not severe enough to want to participate, 50% agreed distance was
a factor and 25% agreed they thought they will be a guinea pig.
Conclusions: The amount of time spent and the satisfaction of the
clinicians explanation was not a major factor in accepting or rejecting
clinical trial participation. Willingness to participate in a clinical trial
relied upon the hospital and doctors reputation; while those who

S213

rejected participation stated distance from study center, feeling like a

guinea pig and their disease not severe enough to participate.
696
Analysis of survival in patients with Huntingtons disease in Serbia
I.N. Petrovic, M. Svetel, T. Pekmezovic, N. Dragasevic, V.S. Kostic
(Belgrade, Serbia)
Objective: The objective of this study was to estimate cumulative
probability of survival of Huntingtons disease (HD) patients in Serbia
as a function of CAG repeat length and selected demographic variables.
Background: HD is an autosomal dominant progressive neurodegenerative disorder, caused by an unstable expanded CAG trinucleotide
repeat in exon 1 of the huntingtin gene on the tip of the short arm of
chromosome 4.
Methods: This follow-up study was carried out at the Institute of
Neurology, Clinical Centre of Serbia, Belgrade, 1982-2004. After
providing informed consent, 112 Serbian patients with clinically and
genetically veried HD were included in the study. We dened HD
onset as zero time and death as the end point. The cumulative survival
probability was calculated by life table method, while predictive value
of different variables was assessed by Cox proportional hazard model.
Results: Male to female ratio in this study was 0.9, without the
statistically signicant gender differences regarding the age at HD
onset (t0.391, p0.697), the age at death (t1.293, p0.203), and
the CAGn (t0.376, p0.708). There was no signicant difference in
age distribution between males and females (20.032, p0.857). The
difference in residence (home vs. institution) between male and female
patients was not signicant (20.130, p0.719). The signicant
inverse correlation was found between CAG repeat length and age at
onset of HD (r-0.732, p0.001) and age at death (r-0.760,
p0.001). The cumulative probability of 15-year survival was
10.35.5%, 3.14.3% for men and 18.74.2% for women. The
lowest value of 15-year survival (0%) was registered in the group with
onset of HD before 20, and the highest (45.711.3%) in the oldest
group. The 15-year survival was higher in HD patients with CAG
numbers 46 (26.617.4%) than in those with 47 (14.517.3%).
The Cox regression analysis showed that signicantly poorer outcome
of HD in our population was related to younger age at onset (HRhazard ratio1.9; p0.047), and larger CAG numbers (HR2.4;
p0.071). The female sex was statistically signicantly associated with
longer survival (HR0.4; p0.007).
Conclusions: These data might be of some importance for further
exploration of natural history and prognosis of HD.
697
Motor neuron disease associated with copper deciency in a case of
Wilsons disease
A. Foubert, A. Kasadi, M. Rouanet, A. Lagueny, F. Tison (Pessac,
France)
Objective: To describe an exceptional case of ALS-like presentation
of copper deciency in (Wilsons disease) WD.
Background: Symptomatic copper deciency is a rare but serious
adverse event of zinc and/or chelating therapy in WD. Copper deciency often presents as myeloneuropathy and pancytopenia. More
atypical and rare forms such as amyotrophic lateral sclerosis (ALS)like presentations have been recently described.
Methods: Single case study.
Results: This 41-year-old man presented at age 16 a history of
psychosis, dystonia, and parkinsonism leading to the diagnosis of WD
in 1989. He was treated with zinc acetate then trientine (1998). His
clinical status was stabilized by using zinc acetate (120 mg/d), trientine
(900 mg/d), a low copper diet and olanzapine (15 mg/d). In September
2004, he progressively developed a mild leucopenia, with a myelodysplasic bone marrow. Treatment was not modied, leucopenia was
monitored and found mild and stable. In June 2006, he presented
progressively bilateral foot drop with falls. In September 2006 he
displayed distal weakness of the 4 limbs (MRC grade 3 to 4) with
foreleg and intrinsic hand muscle amyotrophy without sensory signs,

Movement Disorders, Vol. 22, Suppl. 16, 2007

S214 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

fasciculations and preserved tendon jerks. Electromyography showed
acute denervation in 4 limbs with preserved sensory nerve action
potentials. Subacute axonal denervation was found at the musculocutaneous nerve biopsy. Brain and spinal cord MRI were normal.
Serum copper (0,5 mol/l, N 12 25 mol/L), ceruleoplasmin ( 0.1
g/l; N 0.22-0.61 g/l) urinary copper (1.75 mol/24h; N1.9 mol/
24h) and CSF copper (0.05 mol/L; N 0.9-0.38 mol/L) were low.
Liver biopsy also showed low copper content (1.8 mol/g; N 4.0
mol/g). Zinc acetate was withdrawn, low copper diet stopped and
trientine dosage lowered at 300mg/d after a 10 days withdrawal. The
condition stabilized but did not improved.
Conclusions: Hypocupraemic myeloneuropathy is a severe disorder
that might, in rare cases, mimic ALS. It is hypothesized that lower
motor neuron disease may be due to alteration in various copperdependant enzymes such as SOD. Usually it occurs in patients with
gastrectomy, total parenteral nutrition or excess zinc ingestion. Movement disorders specialists should be aware that it might occur in zinc
and/or copper chelator-treated WD patients.
698
Benecial effect of Piracetam on obstructive sleep apnea syndrome
in patients with multiple system atrophy
Y. Nakamura, I. Yamada, H. Sakamoto (Sakai, Osaka, Japan)
Objective: Patients with multiple system atrophy (MSA) are frequently
known to develop obstructive sleep apnea syndrome (OSA) and/or laryngeal stridor with disease process and the tracheostomy or nasal continuous
positive air pressure is often needed. Recently EMG investigation showed
that the mechanism for stridor is dyskinesia or dystonia of laryngeal
muscles. Evidence of the laryngeal dystonia could suggest a new choice of
treatment for laryngeal narrowing in MSA. Piracetam is a low molecular
weight derivate of GABA and have a signicant benecial effect of
anti-myoclonus drug. The purpose of this report is to evaluate the effect of
piracetam on OSA /laryngeal narrowing in MSA.
Methods: Three patients with MSA were enrolled. They were all male
and their average age was 60.95.7 years old and the duration of disease
was 3.31.2 years. They had probable cerebellar type (MSA-C). All
patients had the polysomnographic study (PSG) and laryngeal endoscopy.
In PSG, routine record including EEG, respiration and oxyhaemoglobin
saturation (SaO2) were measured. The average number of apnea-hypopnea
episodes per hour of sleep was apnea-hypopnea index (AHI). OSA was
dened as a cessation of airow for over 10 seconds despite persistent
respiratory effort with an AHI5. The criteria of piracetam treatment was
an AHI above 20/h. They had 12g/day of piracetam for two weeks. They
were evaluated before and after treatment.
Results: 71-year old male had showed stridor during night. His PSG
showed OSA with 46/h of AHI and 86% in average SaO2. After
piracetam treatment, effect of piracetam on apnea was observed (AHI
was 16/h and average SaO2 89%). In case 2, his PSG showed 54.3/h of
AHI and 75 % in average SaO2. After treatment, effect of piracetam on
apnea was also observed (29.2/h in AHI and 88% in average SaO2).
Case 3 also showed improvement after piracetam (23/h to 12.7/h in
AHI, 86% to 89% in average SaO2).
Conclusions: Piracetam therapy was demonstrated to be effective on
OSA and was well tolerated in patients with MSA. To our knowledge,
the benecial effect of piracetam on OSA was not previously reported.
OSA in MSA could be caused by laryngeal narrowing with dystonia of
laryngeal muscles. Our ndings suggested piracetam has a potential
effective drug on OSA and/or laryngeal narrowing in MSA.
699
Electroconvulsive therapy for a patient with lethal catatonia-neuroleptic malignant syndrome
V.C. Chang, D. Hardesty, M. Pietro (New York, New York, USA)
Objective: To describe a case of a patient with lethal catatonia-NMS
who recovered after receiving electroconvulsive treatment.
Background: Catatonia refers to a neuropsychiatric syndrome with
multiple etiologies and a highly variable clinical presentation. This
includes a malignant form, known as lethal catatonia, characterized by

Movement Disorders, Vol. 22, Suppl. 16, 2007

labile blood pressure, hyperthermia, and rigidity that require emergent

treatment. The life-threatening end of this spectrum can be indistinguishable from neuroleptic malignant syndrome as it often occurs in the
context of neuroleptic treatment. The relationship between catatonia
and NMS remains inconclusive.
Methods: A 33 year-old woman with a history of panic attacks and
no other past medical or psychiatric history was treated as an outpatient
with quetiapine for insomnia and minor psychotic symptoms. Two
weeks later she developed confusion and disorganized behavior and
was treated for psychosis with risperidone, haloperidol, benztropine,
and lithium. She developed progressive rigidity, fever, tachycardia, and
mildly elevated creatine kinase levels (800-900 IU/L) and was subsequently treated for neuroleptic malignant syndrome. Her mental status
was notable for absence of response to deep pain and lack of following
of verbal commands. However, there was also forced eye closure,
active resistance to eyelid opening, intermittent smooth pursuit tracking, absent oculocephalic reex, and intermittent verbalization of short
phrases. Treatment with intravenous lorazepam was not effective. In
the fth week, electroconvulsive therapy was instated and she gradually
improved. Over a period of 8 weeks, she received a total of 22 ECT
treatments with signicant improvement in her muscle tone, responsiveness, and autonomic symptoms. She was discharged 14 weeks after
initial presentation with a normal neurologic exam and a diagnosis of
major depression.
Conclusions: The overlap of lethal catatonia and NMS remains
poorly characterized, leading to uncertainty about treatment approaches. Despite this ambiguity, this patients response to ECT supports that 1.) electroconvulsive therapy can be a life-saving treatment
for benzodiazepine-resistant lethal catatonia and 2.) when NMS does
not resolve with conventional treatments, ECT should be strongly
considered by neurologists as an early treatment option.
700
A rare combination of Klippel-Feil syndrome and pheochromocytoma: Case report
S. Telarovic, T. Bajica, S. Juren, M. Relja (Zagreb, Croatia)
Objective: To present a patient with Klippel-Feil syndrome (KFS),
and tumor of the adrenal gland marrow (pheochromocytoma).
Background: KFS is a rare disorder characterized by the cervical
vertebra fusion, with consequent limitation of movement span, combined with various other anomalies of organs or organic systems
contributing to diverse clinical presentations.
Methods: Case report: A 38-year old male patient was refered for
botulinum toxin treatment of anterocollis, which had occured a year
ago. In neurological status were evident the compulsory neck anteexion, low hair line, markedly limited ante- and retroexion, as
well as tense and painful paravertebral muscles of the cervical
spine.The patient also had convex scoliosis on the right and intensied lumbar lordosis, hypoplasia of both thumbs and divergent
strabism of the left eye. The cervical spine MR presented congenital
block of the C5, C6 and C7 segments, what conrmed the diagnosis
of KFS. A complete radiological, laboratory, electrophysiological
and ultrasound examination was done.
Results: The results veried an asymptomatic tumor of the adrenal
gland marrow- pheochromocytoma, requiring adrenalectomy. Due to
continuous anterocollis, accompanied by pronounced tension of paravertebral muscles and strong painful component that could not be
successfully treated with muscle relaxants, botulinum toxin type-A was
locally applied (BOTOX 100 U). Already after the rst application, the
muscle tension considerably decreased, with the reduction of pain,
what improved the patients quality of life.
Conclusions: All patients refered to botulinum toxin treatment
should have completed neurological examination. Here we present the
rst case of KFS in combination with pheochromocytoma. If there is no
indication for surgical treatment of KFS, therapy is symptomatic with
the aim of ameliorating spasm and pain caused by muscle tension.
Although botulinum toxin is not the rst choice medication in this
indication, in the described patient the local application led to signi-

POSTER SESSION III, THURSDAY, JUNE 7, 2007

cant subjective improvement, with muscle relaxation and complete
regression of the painful component.
701
Safety and efcacy of Hengli and Dysport for primary hemifacial spasm: A randomized controlled trial
Y.W. Wu, L.L. Zeng, S.D. Chen (Shanghai, China)
Objective: The aims of this study were to evaluate the safety and
effectiveness of Hengli for primary HFS and compare the clinical
outcomes with those of Dysport.
Background: Botulinum toxin A (BTX) is the currently preferred
symptomatic treatment for primary hemifacial spasm (HFS). Dysport
is one of the most popular BTX products in the western countries. In
China, only Hengli is available for HFS. Despite many reports
conrming its short-term efcacy, few studies have compared its outcome with that of the Dysport.
Methods: From February 2006 to February 2007, a total of 31
patients with HFS were assessed for entry in this randomized controlled
trial (RCT). Patients were received either the injection of Dysport or
the injection of Hengli. The efcacy was judged by patients selfevaluation scale. The improvements of spasm symptoms were judged
according to the Jancovic Scale by the same neurologist 1,4,12 weeks
after treatment. Dosage of Hengli and Dysport, onset benet, duration of benet and side effects were also studied.
Results: 31 patients with primary HFS took part in the study, 16 in
the Dysport group and 15 in the Hengli group. The response rate
were 90% in the Dysport group and 89.7% in the Hengli group
respectively, however, no signicant difference was observed. The
effectiveness in relieving the symptoms of primary HFS was comparable between these two groups. The average duration of improvement
was 16.094.47 mon in the Hengli group, which was signicantly
shorter than 18.104.1 mon in the Dysport group (P0.01). The
average time of effect-producing in the Hengli group was 4.171.74
d, which was signicantly longer than 3.211.88 d in the Dysport
group (P0.01). The adverse effects between these two groups were
comparable.
Conclusions: Hengli and Dysport are both safe and effective for
primary HFS. The effectiveness of Dysport in relieving the symptoms
of primary HFS is better when compared with that of Hengli.
702
Paroxysmal kinesogenic dyskinesia following ischemic insult
S.R. Daniels, K. Nakamura, G.A. Kang (San Francisco, California,
USA)
Objective: We present a case, along with accompanying video, of a
patient who presented with episodes of hyperkinetic movements of his
left arm and leg, after experiencing two subcortical ischemic events.
Background: Paroxysmal kinesigenic dyskinesias (PKD) is a rare
movement disorder characterized by brief and recurrent hyperkinetic
attacks of chorea, athetosis, dystonia, and ballism. PKD occur typically
in a sporadic or inherited manner. However, there are several cases of
secondary PKD described in the literature.
Methods: Our patient is a 58-year-old male who presented with a
chief complaint of episodes of left lower extremity shaking that began
about one and a half years before presenting to our Movement Disorders Clinic. History is signicant for two ischemic strokes. Approximately four days after his second ischemic event, he developed episodes of left leg shaking of very brief duration. Initially, they occurred
only with signicant exertion and only while walking. Later, symptoms
progressed to also include the arm, and occur with standing, especially
when standing from a sitting position. These episodes were never
associated with altered mentation. Exam was notable for several episodes of rhythmic, high amplitude, 3-4Hz proximal shaking of left leg
lasting about 5-10 seconds. This would occur upon standing and
worsened with challenges of walking, such as tandem gait.
Results: Given history and exam ndings, he was given a diagnosis
of secondary paroxysmal kinesigenic dyskinesias. He was started on an
empiric trial of carbamazipine for symptomatic therapy. Once he began

S215

treatment, he noted immediate decrease in the number of episodes, and

by the third day of taking the anticonvulsant, his episodes resolved
completely.
Conclusions: The pathophysiology of PKD has not been elucidated,
although it is thought to originate from an insult to the basal ganglia.
The extrapyramidal symptoms as well as basal ganglia abnormality
seen on radiologic studies of our patient support this view. Of interest,
our patient responded very well to low dose carbamazepine. This
response is typically seen in patients with idiopathic PKD, but has not
been described in the literature on secondary PKD. Given our patients
robust response to carbamazipine, it seems prudent to give a trial of this
medication in patients presenting with paroxysmal dyskinesias following an ischemic insult.
703
Frontotemporal dementia due to VCP mutations: Clinical and
functional neuroimaging ndings
D. Haubenberger, G. Pusswald, M. Hoffmann, A. Zimprich, E. Auff
(Vienna, Austria)
Objective: To present functional brain imaging and neuropsychological data on a novel type of frontotemporal dementia caused by mutations in the VCP gene.
Background: Mutations in the gene coding for VCP (valosin containing
protein) lead to the autosomal dominant clinical trias (IBMPFD) of inclusion body myopathy, Paget disease of the bone and frontotemporal dementia (FTD). VCP, as ATPase, is involved in the ubiquitin-dependent
protein degradation pathway. Neuropathological studies on brains of patients with IBMPFD revealed a novel type of FTD with intraneuronal
inclusion bodies containing ubiquitin and VCP.
Methods: We previously described an Austrian family of four siblings affected by inclusion body myopathy and Paget disease of the
bone and detected a novel mutation in the VCP-gene (R159H,
688GA; Haubenberger et al, Neurology, 2005). All four patients were
mutation carriers; age of onset was between 37 and 63. During clinical
follow-up over 2 years, one patient (case 1, female, age 65) developed
signs of dementia. Neuropsychological assessment as well as brain
perfusion imaging (99mTc-HMPAO SPECT) was performed on the
affected patient as well as her sister (case 2, age 69), who was also a
mutation carrier but has not presented with signs of dementia until the
time of examination.
Results: Case 1 exhibited signicant reduction in tests of executive
functions, working memory, spatial functions, and problem solving.
Attention and concentration was uctuating. There were no language
difculties; apraxia was not present. Case 2 showed slight decits in
executive functions but no overall signs of dementia. SPECT imaging
of case 1 showed signicant bifrontal hypoperfusion, pronounced in
posterior and temporoparietal portions while frontopolar cortical areas
were spared. SPECT imaging of case 2 was normal.
Conclusions: As shown in previous reports on VCP-mutations (e.g.
R155C) with ubiquitin-positive and tau-negative neuropathology, also
the mutation detected in our kindred (R159H) can lead to the complete
clinical picture with inclusion body myopathy, Paget disease and FTD.
This is the rst report showing functional brain imaging data of an
affected patient on a novel type of hereditary FTD due to impairment
of ubiquitin-based degradation pathways with intraneuronal inclusion
bodies.
704
Wilsons disease: A study of 21 cases from Indian subcontinent
B. Sharma, R.K. Sureka, A. Panagariya, N. Agarwal, V. Agarwal,
A. Dev (Jaipur, Rajasthan, India)
Objective: Wilsons disease is a heredofamilial, progressive multisystem disease of rare occurrence due to inborn error in copper metabolism with protean clinical spectrum. Early recognition of this
potentially treatable disorder needs high index of suspicion.
Background: Aims of the present prospective study was to make
observations of clinical, biochemical and radiological characteristics in
cases of Wilsons disease from Northwestern India and compare it with

Movement Disorders, Vol. 22, Suppl. 16, 2007

S216 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

other parts of Indian subcontinent and Western countries so as to assess
the diversity of symptoms, signs and radiology in different geographic
and ethnic groups.
Methods: Between January 2002-July 2006, twenty-one patients of
Wilsons disease admitted in Department of Neurology of SMS Medical College and Hospital, Jaipur, a tertiary care center of India were
included in the study with prior consent. The diagnosis was based on
detailed history and clinical examination and was conrmed by biochemical parameters (Including presence of Kayser eisher ring, serum
copper level75ug/dl, serum ceruloplasmin 20 ug/dl and 24-hour
urinary copper excretion 100 ug/24 hours as per the accepted diagnostic criteria.
Results: The mean age of onset of disease was 13.2 years with
male preponderance (3.2:1). The most common neurological signs
were tremors (85.7%), speech abnormality (76.1%), dystonia
(57.1%), impaired mental functions and drooling of saliva in 57.5%
patients. Seizures were present in 38% patients in juvenile group
and cerebellar signs were present in 60% of adult group. Presence of
Kayser-Fleisher ring (KF) and increased 24 hours urinary copper in
all cases were in addition associated with low serum ceruloplasmin
in 85% and low serum copper in 57%. Computerized axial tomography revealed Basal ganglia hypodensity in 42.8%, Ventriculomegaly in 23.8% and cerebellar atrophy in 9.5% of cases.
Conclusions: Due to wide clinical spectrum Wilsons disease is
often missed at early stage. A high index of suspicion may help in
improving outcome. This study highlights preponderance of extrapyramidal features in juvenile and cerebellar symptoms at late age
of onset of disease. In addition, this study differs from Western
literature in having male preponderance and earlier age of onset.

705
L-Dopa responsive movement disorder in a young patient with
mixed connective tissue disease (sharp syndrome)
S. Haegele-Link, A. Burrow, T. Hundsberger, B. Tettenborn (St.
Gallen, Switzerland)
Objective: We report a 20 year-old female patient developing a non
kinesiogenic, alternating hemiballistic and choreo-athetotic movement
disorder over a 6 month period involving the upper and lower right
limb.
Background: Except for the Movement Disorder neurological
examination did not reveal any abnormalities. Any drug abuse apart
from occasional cannabis and alcohol consumption was denied. The
patient was known to suffer from a mixed connective tissue disease
(sharp syndrome) with raynaud syndrome and arthralgia treated with
chloroquine. Family history was lacking any evidence of a hereditary disease.
Methods: Extensive diagnostic investigations (MRI of the brain,
CSF, metabolic investigation, electrolytes, copper metabolism, antiphospholipid antibodies, blood count and blood cell investigation) did
not reveal any evidence for a symptomatic metabolic or inammatory
disease. Especially, there was no evidence for an antiphospholipid
syndrome. Electrophysiological (EMG, VEP, SEP) and neurosonological investigation did not show any abnormality. Neuropsychological
investigation presented slight impairment of attention and working
memory possibly due to the persisting Movement Disorder during
testing.
Results: Treatment with L-Dopa up to 300mg per day was followed
by sustained improvement of the symptoms. Discontinuation of the
medication led to a relapse of the symptoms.
Conclusions: L-Dopa responsive dystonia (Sydenham) as well as an
association to the mixed connective tissue disease must be considered
as differential diagnoses.

Movement Disorders, Vol. 22, Suppl. 16, 2007

706
Handtapping as a clinical marker for evaluating disease progression in Huntingtons disease
S.L. Mason, A.O.G. Goodman, A. Michell, R.A. Barker (Cambridge,
Cambridgeshire, United Kingdom)
Objective: To evaluate the utility of a simple and inexpensive handtapping device as an objective marker of clinical phenotype in Huntingtons disease (HD).
Background: HD is a progressive neurodegenerative disease that is
characterized clinically by the progression of motor, cognitive and
behavioral symptoms. Experimental trials for potential disease modifying treatments are hampered by the difculty in objectively measuring the clinical phenotype of the disorder. Currently, this is commonly
measured using structured clinical observations to generate scores on
subjective rating scales such as the Unifed Huntingtons Disease Rating
Scale. There is a clear need for an inexpensive, objective and simple
way of assessing clinical phenotype to compliment the procedures used
at present.
Methods: All patients were recruited from the HD research clinic at
the Cambridge Centre for Brain Repair. On the hand tapping device the
subjects task was to alternately tap one button after the other as rapidly
as possible using rst the right hand, and then using the left hand. Total
number of taps made in 30 seconds was recorded.
Results: The total number of taps in the 30 seconds signicantly
correlated with the motor UHDRS score and independence score in a
large population assessed in an HD research clinic.
Conclusions: The objective assessment of motor function in patients
with HD is easily and rapidly achieved by using a hand tapping device.
We have shown that total number of taps in 30 seconds correlates well
with motor UHDRS. The measurement of hand tapping is proposed as
an objective adjunct to the clinical assessment of patients, not as a
replacement for existing assessments. One of its strengths is that it
removes the subjective rating necessary, for example, to calculate
UHDRS. Eventually it is envisaged that the main use of this device will
be as part of the routine objective assessment of clinical phenotype,
both in the clinic and during clinical trials.
707
The role of proportion of cerebrospinal uid total tau-protein
levels to phosphorylated tau-protein levels in differential diagnosis
of Creutzfeltd-Jacob disease
M. Valis, J. Hort, R. Talab (Hradec Kralove, Czech Republic)
Objective: Diagnosis of Creutzfeld-Jacob disease (CJD) is based on
typical clinical features and can be supported by detection of 14-3-3
protein in the cerebrospinal uid (CSF). To evaluate the role of proportion of CSF total tau-protein levels to phosphorylated tau-protein
levels in differential diagnosis of Creutzfeltd-Jacob disease.
Background: Present study suggests the importance of investigating
the ratio between CSF total tau-protein and CSF phosphorylated tauprotein in differentiating CJD from other dementias.
Methods: Thirty-one patients with Alzheimer disease (AD) of Frontotemporal dementia (FTD) and four patients with denitive diagnosis
of Creutzfeldt-Jacob disease were included into the study. Patients were
of Czech origin. All study subjects underwent MRI scan of the brain
and extended neuropsychiatric examination at baseline to classify the
patients as having Alzheimer dementia (according to NINCDSADRDA criteria) or FTD (according to Neary criteria). Results from
baseline investigations were compared with an age-matched cognitively normal control group. Tau-protein was analyzed using a commercially available ELISA and 14-3-3 protein was assessed by Western
blotting. Three markers were put into comparison: total tau-protein
(cutoff value of 355 pg/ml), phosphorylated tau-protein (cutoff value of
55 pg/ml), and beta amyloid (cutoff value of 458 pg/ml). Due to absent
consensus in current literature regarding reference range for normal
values of all three measured markers, the receiver operating characteristic (ROC) curve has been designed to achieve the best possible
sensitivity and specicity for each marker.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Results: High ratio between CSF total tau-protein and CSF phosphorylated tau-protein has been found in all patients diagnosed by CJD,
even in those with negative 14-3-3 protein blots results. Contrary,
markers analysis in patients with Alzheimer dementia revealed the
highest ratio between CSF beta amyloid and CSF phosphorylated
tau-protein levels.
Conclusions: CSF tau-protein and phosphorylated tau-protein are
valuable diagnostic biomarkers for CJD, especially in patients with
negative 14-3-3 protein ndings.

S217

increase of gamma-1 (32-45 Hz) and gamma-2 (45-55 Hz) bands in the
sensorimotor areas, as well as in the parieto-occipital regions. The
effective DBS during the imagery task led to a decrease of the beta
pattern in the prefrontal and posterior cortical areas.
Conclusions: These data suggest that the improvement in the execution of a complex motor task by therapeutic STN-HFS might be
related to the recruitment of electrical rhythms which may play a role
in the cortical modulation of movement.

708
Alien hand syndrome and dystonia in a pediatric patient
T. Soman, T. Steeves, A.E. Lang (Toronto, Ontario, Canada)
Objective: Report of a pediatric patient with Alien Hand Syndrome
(AHS), a condition when a limb has a will of its own with observable
involuntary activity, in association with dystonia.
Background: AHS has been described in a variety of conditions like
Corticobasal degeneration, tumors, surgical division of the corpus
callosum and frontal lobe infarction. It is usually not seen after infarctions in the parietal lobe, and has not been reported in the pediatric
age-group.
Methods: This is a case report.
Results: The patient had a right middle cerebral artery infarction with
hemorrhagic conversion, of unknown etiology, at the age of 3 years.
Follow-up MRI showed involvement of right parietal lobe, dorsal
putamen and body of caudate. Abnormal signals in thalamus and
posterior limb of the internal capsule was attributed to Wallerian
degeneration. Globus Pallidus and Corpus Callosum were unaffected.
She developed a left-sided hemidystonia and was treated with botulinum toxin. The patient noted an insidious onset of involuntary complex
movements, around 9-10 years age. There were groping activities e.g.
grabbing her grandmothers coat the grocery store and self-destructive
behaviours - when she began to write (using her right hand), the left
hand would reach out for her hair and grip it tightly until it hurt. The
hand would not loosen by itself and would have to be pried loose.
Inter-manual conict, characterisitic of AHS, was also reported e.g.
zipping up a sweater and undoing it with the left (affected) hand. On
examination, there was dystonic posturing of the left hand and arm and
left foot. She was able to follow commands using the affected hand and
did not deny ownership. Mirror movements were noted.
Conclusions: AHS may occur concomitantly with dystonia. Though
rare, it may be seen in the pediatric age group.

PARKINSONS DISEASE

FIG. 1 (709).

709
Effects of subthalamic stimulation on brain electrical activity during a motor task in Parkinsons disease
L. Colloca, F. Benedetti, M. Lanotte, M. Sigaudo, M. Zibetti,
A. Cinquepalmi, S. Vighetti, A. Ducati, L. Lopiano (Turin, Italy)
Objective: We investigate behavioral changes and neurophysiological pattern of electrical activation during motor and imagery task.
Background: Patients with idiopathic Parkinsons disease (PD) show
impairment in executive and planning processes. High frequency stimulation (HFS) of the subthalamic nucleus (STN) induces improvement
of motor symptoms, but its specic effects on motor execution are not
fully understood.
Methods: We recorded scalp EEG from 35 sites in ten Parkinsonian
patients who were instructed rstly, to track a complex motor task and,
then to image the same task. These tasks were performed with therapeutic bilateral deep brain stimulation (DBS) in the on and off conditions. Any medication was stopped 12-18 h before the recording
session. Clinical improvement was assessed by means of Unied Parkinson Disease Rating Scale (UPDRS).
Results: As to motor execution, effective DBS caused a signicant
improvement of both time and accuracy of motor execution. We
observed an increase of activation of alpha-2 band (10-12 Hz) in the
sensorimotor areas. Therapeutic stimulation also induced a signicant

710
Salsolinol decreases expression of the antral but not duodenal and
colonic interstitial cells of Cajal
T.A. Banach, A.T. Krygowska-Wajs, K.M. Gil, D. Zurowski,
P.J. Thor (Cracow, Poland)
Objective: The aim of our study was the evaluation of the effect of
chronic peripheral salsolinol administration on the expression of c-Kit
positive interstitial cells of Cajal (ICC) in antrum, duodenum and
ascending colon.
Background: Gastrointestinal (GI) dysmotility in Parkinsons disease
(PD) has been attributed to both central and peripheral action of the
neurotoxins involved in PD, however, direct neurotoxin effect on
elements of GI myenteric plexus has not been previously evidenced.
We hypothesized that PD neurotoxin salsolinol may decrease ICC
expression in GI wall.
Methods: Twenty rats were injected intraperitoneally with salsolinol
(50 mg/kg/day) for three weeks whereas the equal group served as the
control. On the last day, the animals were sacriced, stomachs and
small and large intestines were removed, and parafn embedded specimens were prepared. ICC were visualised using anti c-Kit rabbit
polyclonal antibodies (Santa Cruz Biotechnology, USA) and the

Movement Disorders, Vol. 22, Suppl. 16, 2007

S218 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

LSAB/HRP (DAKO Corporation, USA) dye set. Antral, duodenal and
ascending colon intramuscular c-Kit positive cells were assessed.
Results: Image analysis revealed the presence of c-Kit positive cells
in the wall of each analyzed segment of GI tract. However, only in the
antrum c-Kit expression was signicantly lower in the salsolinol group
than in the control (p0.01). No differences were visible in duodenum
and ascending colon.
Conclusions: Our results suggest the possibility of the direct impairing effect of salsolinol on the antral, but not duodenal, and colonic
myenteric ICC. A decrease of the antral ICC expression may result in
the decreased GI myoelectrical activity with motility and transit dysfunction.
711
The differential diagnoses of Parkinsonism in outpatient clinics of
PD Northumbria
E. Stone, R. Walker, B. Wood (North Shields, United Kingdom)
Objective: To determine the causes of parkinsonism seen in all
patients known to PD Northumbria.
Background: Parkinsonism is a feature of a number of neurological
conditions and is characterized by four cardinal features which include,
bradykinesia, resting tremor, rigidity and postural instability. Parkinsonism is most commonly caused by idiopathic PD but also has several
other differential diagnoses. This study investigates diagnostic categories of patients with parkinsonism attending a PD service in the North
of England.
Methods: A retrospective review of all medical records held by PD
Northumbria was conducted. Diagnostic categories of all cases were
reviewed both at the time of diagnosis and currently.
Results: Analysis of 627 case records found idiopathic PD to be the
commonest cause of parkinsonism as a current diagnosis (n439,
73%). Cerebrovascular Parkinsonism and Benign Essential Tremor
followed (n29 and n21, 5% and 3% respectively). Drug Induced
Parkinsons disease and LBD were diagnosed in 23 patients (n15 and
n8, 2.5% and 1% respectively). Atypical parkinsonian syndromes
were diagnosed in 12 patients (6 PSP, 4 MSA, 2 CBD, 2% of all cases).
Mixed diagnoses were found in 7 patients (1% of all cases), these
patients had more than one cause for their parkinsonism. Miscellaneous
other causes were found (n62, 11%). This included patients whose
diagnosis remained unclear or presented with atypical features. Diagnoses made at initial clinic visit showed similar results with idiopathic
Parkinsons disease as the commonest cause of parkinsonism, followed
by other causes. Essential tremor and cerebrovascular parkinsonism
followed. This was followed by drug-induced cases, parkinsonian
syndromes and mixed diagnosis.
Conclusions: Idiopathic Parkinsons disease is the most common
cause of parkinsonism seen in clinics of The Northumbria Healthcare
Trust. Differential diagnoses of parkinsonism include vascular pathology, drug-induced parkinsonism and parkinsonian syndromes. PD
Northumbria is run by elderly care trained physicians and the relatively
small number of cerebrovascular parkinsonism cases is somewhat
surprising.
712
Continuous duodenal levodopa infusion (DUODOPA) in advanced
Parkinsons disease: First French experience
M. Hery, F. Lallement, P. Sauleau, S. Drapier, I. Rivier, M. Verin
(Rennes, France)
Objective: To determine both efcacy and safety of this unique
administration system in the daily living of patients with advanced
Parkinsons disease.
Background: Patients with advanced Parkinsons disease suffer from
motor complications (dyskinesias, motor uctuations) in spite of an
optimal oral treatment, causing severe disability. The variability of
motor response is correlated with levodopa plasma level uctuations. It
was demonstrated that continuous duodenal levodopa/carbidopa infusion is able to limit these complications by reducing levodopa plasma
level uctuation. Only few studies however inform on the clinical

Movement Disorders, Vol. 22, Suppl. 16, 2007

benet, quality of life of patients and the safety of this system during
its use.
Methods: Thirteen French patients with severe motor uctuations
and dyskinesias (mean age: 67 8 years, range 53-77) were assessed.
DUODOPA was administered 14 hours per day, only one patient
needing a 24 hours administration. Bolus was available in case of
on/off phenomenon. Clinical Global Improvement (CGI) permitted to
evaluate the global efcacy in daily living. All complications were
notied. Mean follow-up was 10 5 months.
Results: Thirteen French patients with severe motor uctuations and
dyskinesias (mean age: 67 8 years, range 53-77) were assessed.
DUODOPA was administered 14 hours per day, only one patient
needing a 24 hours administration. Bolus was available in case of
on/off phenomenon. Clinical Global Improvement (CGI) permitted to
evaluate the global efcacy in daily living. All complications were
notied. Mean follow up was 10 5 months.
Conclusions: All the 13 patients were improved by DUODOPA.
Continuous duodenal infusion appears like an interesting alternative in
advanced Parkinsons disease notably in case of failure or contraindication of apomorphine or deep brain stimulation.

713
Good treatment compliance in patients with Parkinsons disease on
ropinirole: The Ropi-Park study
F. Valldeoriola, S. Cobaleda, Ropipark Study Research Group
(Barcelona, Spain)
Objective: To retrospectively evaluate the tolerability, the effectiveness and the observance of patients with the treatment with the nonergot dopamine agonist, ropinirole both as monotherapy and add-on
treatment in patients with Parkinsons disease under usual medical care
conditions in a Spanish outpatient setting.
Background: There are only few studies about the compliance with dopamine agonists of parkinsonian patients. A low observance of the prescribed treatment could result in worse clinical
outcomes.
Methods: Fifty-two (52) investigators participated in this study. A
random sample was selected from 643 clinical records of patients
treated with ropinirole in the previous 18 months. The main endpoints retrospectively analyzed were percentage of patients maintaining treatment during the period of study (compliance), reported
adverse reactions and treatment withdrawal (safety), changes in
symptoms as assessed by the Clinical Global Impression (CGI) scale
(effectiveness), and start-to-end mean ropinirole dose.
Results: This study evaluated charts from 418 patients, 68.69.7
years old (meanSD), 58% were male. RPN was administered as
monotherapy (24%) or add-on treatment (76%). The mean maintenance dose reached was 9.545.34 mg/day. Adverse reactions were
reported by 29% patients, being the most frequent somnolence and
sedation (9%), gastrointestinal symptoms (7%), increase in dyskinesia (5.9%), and orthostatic hypotension (4%). Treatment was
withdrawn in 14.4% of patients due to adverse reactions (6%), lack
of efcacy (2%), or other reasons (7%). Most patients (81%) reported symptom improvement according to CGI scale. At the end of
the study, 86% patients were still receiving RPN treatment (CI 95%,
82 - 89).
Conclusions: The low incidence of adverse reactions and low
rate of treatment withdrawal observed in this study support both
the safety and the effectiveness or ropinirole in the treatment of
PD when used as mono- or add-on therapy, at relatively low
doses. The good tolerability and effectiveness of ropinirole promoted good compliance with treatment in usual clinical conditions.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

714
Valvular heart disease in Parkinsons disease patients treated with
bromocriptine
L.C.S. Tan, K.K.C. Ng, N.C.K. Tan, W.L. Au, R.K.K. Lee (Singapore,
Singapore)
Objective: To assess the frequency of valvular heart disease in
Parkinsons disease (PD) patients treated with bromocriptine compared
to PD patients who had not been exposed to any dopamine agonist.
Background: It has been reported that patients on ergot agonists
pergolide and carbergoline are associated with an increased risk of
developing cardiac valve regurgitation. It is uncertain if this is related
to the ergot nature of the drug or its agonist action on 5HT2B receptors.
Bromocriptine is an ergot-derivative but a 5HT2B antagonist.
Methods: Seventytwo PD patients who had been treated on bromocriptine for 6 months or more and who had not been previously
treated on other dopamine agonist were recruited. Controls were 50 PD
patients who had not been exposed to any dopamine agonist. All
participants had no previous history of valvular or ischaemic heart
disease, and had not been on any other ergot or anorectic agents.
Transthoracic echocardiographic studies were performed following
standardized protocols. A blinded cardiologist reviewed all echocardiographic studies.
Results: There was no signicant difference in the presence of mitral,
atrial or tricuspid regurgitation between bromocriptine cases and controls. The risk of developing any regurgitation on any cardiac valve in
bromocriptine cases compared to controls was 1.0 (95% CI: 0.4-2.4,
p1), while the risk of developing any cardiac valvular thickening was
0.6 (95% CI: 0.3-1.4, p0.3). There was also no signicant difference
in the presence of any valvular regurgitation when cumulative doses of
bromocriptine were analyzed by quartiles.
Conclusions: Our data shows that there is no increased risk of valvular
heart disease amongst PD patients treated with bromocriptine compared to
patients who had not been treated with any dopamine agonists.
715
The diagnostic value of transcranial duplex scanning and SPECT
imaging versus clinical diagnosis (clinical prospective study)
S.C. Tromp, A.M.M. Vlaar, M.J.P.G. Kroonenburgh Van,
W.H. Mess, T. de Nijs, A. Winogrodzka, A.G.H. Kessels,
W.E.J. Weber (Maastricht, Netherlands)
Objective: Comparison of the diagnostic value of transcranial duplex
scanning (TCD) and Single Photon Emission Computer Tomography
(SPECT) in patients with an unclassied parkinsonian syndrome using the
clinical diagnosis after follow-up as golden standard.
Background: For idiopathic Parkinsons disease (IPD) and other
parkinsonian disorders diagnostic criteria based on clinical characteristics have been formulated. However purely on clinical grounds,
especially in the early stage of disease it is often difcult to differentiate between the different parkinsonian syndromes. SPECT visualizing
the dopaminergic pathways by using pre- or postsynaptic radiotracers is
one of the most employed techniques in the differential diagnostic of
IPD. Yet, SPECT is only used in the minority of the patients with
suspected IPD, mainly because SPECT would be too costly to be
applied routinely. TCD is less costly and more comfortable for the
patients.
Methods: 95 parkinsonian patients with uncertain diagnosis were
investigated with TCD (SONOS 5500, Philips) in the period 2004 to
2006. The mean (sd) follow-up until clinical re-examination by a
movement disorder specialist was 14 (10) months. In totally 73 of
the 95 patients a clinical diagnosis after follow-up could be established by the movement disorder specialist: IPD 50, atypical parkinsonian syndromes (APS) 4, essential tremor (ET) 2, vascular
parkinsonism (VP) 5, drug-induced parkinsonism (DIP) 3 and the
restgroup of disorders without dopaminergic involvement 7. In 2
patients we doubted between IPD and APS. Only 59 of the 73
patients had SPECT imaging: 30 presynaptic (FP-CIT) SPECT, 4
postsynaptic (IBZM) SPECT, 25 as well FP-CIT as IBZM SPECT.
Results: See Table.

S219

Conclusions: The accuracy to discriminate patients with IPD from

patients with other parkinsonian syndromes or ET is higher for
FP-CIT SPECT than for TCD. However specicity and PPV are
very high for TCD. TCD is a patient friendly test and can, because
of its high PPV be used as screening in unclear parkinsonism: if
TCD is compatible with IPD a SPECT can be considered unnecessary. However larger prospective and inter-observer studies are
needed to assess diagnostic accuracy of TCD.

IPD versus ET
IPD versus VP

IPD versus DIP

IPD versus APS

IPD & APS versus ET, VP,

DIP & restgroup

FP-CIT
TCD
FP-CIT
IBZM
IBZM & FP-CIT
TCD
FP-CIT
TCD
FP-CIT
IBZM
IBZM & FP-CIT
TCD
FP-CIT
IBZM
IBZM & FP-CIT
TCD

odds ratio (95%CI)

PPV

NPV

122 (4-2823)
11 (0.5-233)
219 (8-6224)
5 (0.4-39)
245 (4-14555)
3 (0.5-21)
73 (2-2653)
15 (0.7-304)
3 (0.1-102)
5 (0.4-66)
58 (2-1863)
2 (0.3-17)
451 (26-7801)
0.2 (0.2.5)
100 (3-2989)
11 (2-53)

1.00
1.00
1.00
0.93
1.00
0.94
1.00
1.00
0.92
0.93
0.94
0.94
0.98
0.77
0.94
0.94

0.76
0.13
0.80
0.25
1.00
0.18
0.50
0.18
0.00
0.25
0.00
0.13
0.92
0.06
1.00
0.40

PPV positive predicitve values, NPV negative predictive value

716
Roles of DJ-1, a causative gene product for familial Parkinsons
disease, in dopamine biosynthesis
S. Ishikawa, T. Taira, H. Maita, C. Maita, H. Ariga,
S.M.M. Iguchi-Ariga (Sapporo, Japan)
Objective: To evaluate the function of DJ-1 in dopamine biosynthesis, transcriptional activity of DJ-1 towards the human and mouse
tyrosine hydroxylase genes were examined.
Background: DJ-1 has recently been shown to be responsible for
onset of familial Parkinsons disease (PD), PARK7. We have shown
that DJ-1 plays roles in transcriptional regulation and anti-oxidative
stress, and loss of its function is thought to trigger onset of PD. One of
the target genes regulated by DJ-1 has been reported to be the tyrosine
hydroxylase (TH) gene whose gene product TH is a key enzyme for
dopamine biosynthesis. While the TH activity observed in the dopaminergic neurons and dopamine content in patients with PARK7 were
lower than those of healthy persons, no changes of a number of the
dopaminergic neurons and dopamine content in DJ-1-knockout mice
have been reported, suggesting distinct regulation of the TH gene
expression by DJ-1 in human and mouse.
Methods: Human SHSY-5Y and mouse Neuro2A cells, neuroblastoma cell lines from dopaminergic cell origins, were transfected with
siRNAs targeting human and mouse DJ-1, respectively, and expression
levels of mRNA of TH genes and TH activities were measured by
real-time RT-PCR and HPLC. Promoter regions of human and mouse
TH genes were linked to the luciferase gene and their luciferase
activities were measured after transfection of these reporter plasmids
into SHSY-5Y and Neuro2A cells together with or without co-transfection of the DJ-1 expression plasmid.
Results: Under conditions where more than 90% of mRNA expressions both of human and mouse DJ-1 genes were suppressed, the
expression of the human TH gene but not mouse TH gene were
suppressed. The TH activity in human but not mouse cells was also
found to be suppressed in DJ-1-knockdown cells. Furthermore, the
promoter activity of the human TH gene was activated by human DJ-1
but not mouse DJ-1. The promoter activity of the mouse TH gene was,
however, not activated by mouse DJ-1.
Conclusions: The expression of the TH gene is distinctly regulated in
human and mouse cells. These ndings affect no dopamine loss in
DJ-1-knockout mice.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S220 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

717
Level of movement, cognitive and emotional disturbances and their
correlation in patients with Parkinsons disease
D.R. Hristova, I.S. Grozdev (Plovdiv, Bulgaria)
Objective: The purpose of the study is to establish the level of
movement disability in everyday life, the disturbances of thinking,
behavior and emotions, and to determine their correlation in patients
with Parkinsons disease.
Background: Main clinical symptoms of the Parkinsons disease
are the neurodegenerative movement disorders. Apart from that the
psycho-neurological and autonomous dysfunctions further aggravate the movement disability and worsen the quality of life of the
patients.
Methods: We have studied 922 patients (of them 485 women and
437 men) with clinically proven Parkinsons disease with the criteria of
the Brain Bank London Institute, aged 46 to 96, average age of 73.76
0.25, and suffering from the disease from 1 to 17 years, average period
of 6.68 0.09. The level of disability in everyday activities in all
patients was measured on the Schwab&England scale, and the disturbances of thinking, behavior and emotions by means of part I of
UPDRS. Using correlative analysis we determined the strength of the
relation between the movement and cognitive; movement and emotional; cognitive and emotional disturbances.
Results: Up to 50% limited movement capacity in everyday life
on the Schwab&England scale was established in 54.44% of the
studied group of patients, and in 27.64% of them the movement
limitations were severe, demanding continuous assistance (in wheelchairs or permanently conned to bed). Severe (3 or 4 points on part
I of UPDRS) intellectual changes and disturbances of thinking,
resulting in obvious cognitive decit were noticed in 8.79%, respectively 9.65% of the patients. Disturbed emotional welfare, expressed
in severe and constant depression was established in 20%, and
serious lack of motivation was found in 25.06 of the studied patients. Using correlative analysis we determined the average correlation between movement and cognitive (r 0.510 p 0.01),
between movement and emotional (r 0.500 p 0.01), and
between cognitive and emotional (r 0.550 p 0.01) disturbances.
Conclusions: Parkinsons disease causes serious disturbances of the
movement activity and psychotic condition of the patients. In the
course of the disease the movement and psychic disability are locked in
a vicious circle, which determined their synchronous progression, thus
seriously worsening the quality of life of the patients.
718
The QUICK questionnaire identies wearing-off in Parkinsons
disease patients
P. Martinez-Martin, E. Tolosa, B. Hernandez, X. Badia (Madrid, Spain)
Objective: To assess the validity of the Quick Questionnaire (QQ) to
identify the wearing-off (WO) phenomenon in patients with Parkinsons disease (PD).
Background: WO can be difcult to be recognized when manifested
through a variety of non-motor symptoms. The QQ is the crossculturally adapted Spanish version of the Patient Questionnaire by
Stacy et al (2003).
Methods: Observational, cross-sectional, multicenter study. Patients30 y suffering from PD no longer than 5 years were included.
The gold-standard was WO presence determined by neurologists who
applied the WO Working Group denition. Neurologists were
blinded to the QQ. Patients were classied as without WO or with WO
(mild or moderate/severe). The QQ is self-applied and includes 19
symptoms related with PD. The QQ diagnostic capability to identify
WO was tested by means of ROC curve and determination of main
cut-off points.
Results: Out of 222 enrolled patients, 80 had no WO, 71 experienced mild WO and 71 moderate to severe WO, according to
neurologists diagnosis. Patients mean age (y) was 66.210.5;
52.7% of patients were male. Duration of disease was 3.51.4.
Table 1 shows WO distribution by Hoehn&Yahr staging. The area

Movement Disorders, Vol. 22, Suppl. 16, 2007

under the ROC curve was 0.90 (CI 95%: 0.86-0.94), bearing a high
probability for QQ values to be higher for the patients declaring WO
than for those who do not. To choose a cut-off point, the best results
were obtained when relief or disappearance of two symptoms was
considered (Table 2). Concordance with the gold-standard was
substantial (kappa 0.68).
Conclusions: The QQ is a valid tool to identify WO in PD attending to
the relief/disappearance of two symptoms of the questionnaire. QQ shows
a sensitivity and a specicity over 75% and good predictive values.

Table 1
Without WO
With mild WO
With moderate/severe WO

Stage 1

Stage 2

Stage 3

Stage 4

28.8%
14.1%
2.8%

65%
78.9%
61.4%

6.3%
7.0%
32.9%

0
0
2.9%

Table 2
Number of symptoms
1
Sensitivity
Specicity
Positive Predictive Value
Negative Predictive Value

95.1
65.0
82.8
88.1

%
%
%
%

2
88.0
80.0
88.7
79.0

%
%
%
%

3
82.4
88.8
92.9
74.0

%
%
%
%

719
Dopamine dysregulation syndrome in Parkinsons disease patients:
Preliminary results of a clinical study
A. Gunduz, F. Beskardes, S. Ertan, S. Ozekmekci, G. Kiziltan
(Istanbul, Turkey)
Objective: To investigate clinical features of dopamine dysregulation
syndrome (DDS) in patients with Parkinsons disease (PD) and the
relationship between dopaminergic therapy as well as other psychiatric
disorders and DDS. We present the preliminary results of this study.
Background: DDS includes compulsive use of dopaminergic drugs
and various behavioural phenomena. DDS is reported in approximately
4% to 6% of PD patients with a male preponderance, early onset of
disease, high dose chronic dopaminergic treatment and history of
depression.
Methods: Nineteen patients (15 men, four women) out of 278 PD
patients examined between September 2005 and September 2006
who had DDS were included. Data was compared with age, gender,
disease duration and Hoehn-Yahr stage-matched 13 PD patients who
did not have DDS. All patients were evaluated for sociodemographical data and DDS symptomatology and were underwent Mini Mental State Examination (MMSE), Hamilton depression, SCID depression and SCID psychosis tests and Unied PD Rating Scale
(UPDRS). Patients who scored less than 24 on the MMSE were
excluded. Dopaminergic treatment was calculated as levodopa
equivalent dose.
Results: Rate of DDS was 6.8% and men were predominant
(78.9%). Mean ages of patients with DDS was 49.39.2. Most
frequently encountered symptoms were aggression (30.4%) and
hypersexuality (17.4%). Other common symptoms were pathological gambling (13.1%), compulsive shopping (13.1%) and punding
(13.1%). Cabergoline (37.3%), pramipexole (14.8%) and ropinirole
(14.8%) were the most frequently used dopaminergic agonists in
DDS group, but pergolide (36.4%) and cabergoline (27.2%) were
among controls. Dopaminergic agonist combination was signicantly common among patients with DDS (8/19 vs 1/13, p0.033).
Mean total and motor UPDRS scores of the two groups did not
differ. Levodopa equivalent doses and total dopaminergic therapy
duration were similar between groups. Although mean Hamilton
depression scores did not differ between groups, SCID test disclosed
13 (68.4%) with psychosis and nine (47.4%) cases with major
depression in the DDS group.
Conclusions: Psychosis and depression are encountered more frequently in DDS patients. The most frequent symptoms were aggression

POSTER SESSION III, THURSDAY, JUNE 7, 2007

and hypersexuality. Male gender and combined agonistic therapy seem
to be associated with DDS development.
720
Pathological gambling in Parkinsons disease: An analysis of published case series
D.A. Gallagher, S.S. OSullivan, A.H. Evans, A.J. Lees, A. Schrag
(London, United Kingdom)
Objective: To determine the characteristics of pathological gambling
(PG) in Parkinsons disease (PD).
Background: PG has been reported as a complication of the treatment of PD.
Methods: We examined all published cases of PG (PubMed literature
search, 23 series, N145) for prevalence and risk factors of this
complication, the relationship of PG and use of dopamine agonists
(DA), and the relationship of PG to the dopamine dysregulation syndrome (DDS).
Results: The prevalence of PG in prospective studies of PD patients
using DA has been reported between 2.3 and 8%, compared to approximately 1% in the general population. As in the general population, PD
patients with this complication are often young (57.7 years 9.7), male
(76.6%) and have psychiatric co-morbidity (65.6%). However, the
majority (72.4%) did not gamble before onset of PD and previous
substance misuse was uncommon (16%). Ninety eight percent of patients with PG in PD are on DA (pramipexole 50.3% of cases, ropinirole 21.4%, pergolide 13.8%, bromocriptine 9%, cabergoline 3.4%).
There is a trend for pramipexole and other non-ergot DA to be more
associated with PG. However, overall the difference between treatment
with individual DA does not reach statistical signicance. The pooled
odds ratio (OR) of PG associated with pramipexole compared to
ropinirole is 1.55, condence interval(CI) 0.88-2.73, P0.13 and the
OR comparing total ergot and non-ergot DA is 1.78, CI 0.89-3.54,
P0.10. PG associated with L-DOPA monotherapy is uncommon, but
in the majority of cases L-DOPA is co-prescribed (81%), suggesting
possible cross-sensitization of brain systems mediating reward. PG can
occur with DDS (N17) but often occurs in isolation. In contrast to
dopamine dysregulation, escalation and self regulation of anti-parkinsonian medication are not usually seen.
Conclusions: The prevalence of PG in patients with PD using DA
is higher than PG reported in the general population, however, it
shares similar characteristics and risk factors. PG is predominantly
associated with oral DA, often at high doses. It often occurs in
isolation and may not be associated with DDS, which typically
occurs on treatment with L-DOPA or S/C apomorphine. Sensitisation of dopamine receptors in predisposed individuals may underlie
this often serious complication.
721
Driver safety errors in Parkinsons disease
E.Y. Uc, M. Rizzo, J. Sparks, S.W. Anderson, R.L. Rodnitzky,
J.D. Dawson (Iowa City, Iowa, USA)
Objective: To compare driving safety between drivers with Parkinsons disease (PD) and controls, and to determine the predictors of
driver safety errors in PD.
Background: Cognitive, visual and motor dysfunction have the potential to impair driving safety in PD.
Methods: Licensed, active drivers with mild-moderate PD (n65,
median Hoehn-Yahr stage2 and age66) and controls (n142,
median age67) took a battery of cognitive, visual and motor tests
(e.g, UPDRS, tapping and walking speed) and drove an instrumented vehicle in an experimental 35 mile drive across residential
city streets, suburban commercial strips, rural two-lane highways,
and a four-lane 65 mph speed limit freeway. Secondary tasks such
as route following were interspersed across approximately seven
miles of the drive (on-task segments). At-fault safety errors (e.g.,
erratic steering, lane deviation, speed violations, unsafe intersection
behavior) were determined.

S221

Results: Drivers with PD performed worse on all aspects of the

off-road test battery. Despite driving slower, PD drivers made more
driving safety errors than controls (total errors 15.08.2 vs.
3.63.7, on-task 5.63.7 vs. 1.51.8, baseline segments
9.35.8 vs. 2.12.4, all p0.0001, adjusted for age, gender, education, and previous familiarity with the region). The group difference in on-task safety errors remained signicant even after
adjusting for baseline errors, p0.0007. Within the PD group, worse
scores on tests of attention, memory, visuospatial abilities, and set
shifting, correlated with the error counts, but motor dysfunction did
not. Multivariate analysis showed age, disease duration, and Complex Figure Test-Recall (non-verbal memory) score as the independent predictors of baseline errors. The sole independent predictor of
on-task errors in the PD group was prolonged time to complete on
Trail Making Test (B-A), a set-shifting test independent of motor
speed.
Conclusions: Drivers with PD made more safety errors than
neurologically normal drivers on a road test despite driving slower
as a compensation strategy. The demands of secondary tasks probably increased the cognitive load during driving, which may explain
the higher number of on-task safety errors even after adjusting for
baseline errors. Impairments in cognitive and visual function, rather
than motor severity predicted driver safety errors in PD.
722
Mitochondrial DNA polymorphisms and the risk of Parkinsons
disease in Taiwan
C.-M. Chen, C.-C. Kuan, G.-J. Lee-Chen, Y.-R. Wu (Taipei, Taiwan)
Objective: To investigate whether mitochondrial DNA (mtDNA)
single-nucleotide polymorphism (SNP) 9055G/A, 10398G/A, and
13708G/A are associated with the risk of Parkinsons disease (PD) in
Taiwan.
Background: A critical role of mitochondrial dysfunction has been
implicated in pathogenesis of PD. MtDNA SNPs may affect the function of mitochondrial enzymes by affecting mitochondrial matrix pH
and intracellular calcium dynamics and then confer susceptibility to the
risk of PD. The association of mtDNA SNP 9055G/A, 10398G/A and
13708G/A with PD has been controversial.
Methods: We analyzed mtDNA SNP 9055G/A, 10398G/A and
13708G/A in a cohort of 416 PD cases and 372 ethnically matched
controls.
Results: The allele frequency distribution of any of these three
analyzed polymorphisms was not signicantly different between the
cases and the controls. None of the six haplotypes derived inuences
risk of PD. Notably, after stratication by age, individuals over 70
years of age carrying the haplotype 9055G-10398A-13708G demonstrated a signicant decrease in risk of developing PD (OR 0.44,
95% CI 0.24-0.80, p 0.008).
Conclusions: These results suggest that the mtDNA haplotype
9055G-10398A-13708G plays a role in PD susceptibility among Taiwanese people older than 70 years of age.
723
Social impact on Parkinsons disease caregivers and the impact of
disease duration
J. Loekk (Stockholm, Sweden)
Objective: To investigate the impact of Parkinsons disease (PD) on
social and relationship factors over the time course of disease from the
caregivers (CGs) perspective.
Background: The task of managing care for a person with a chronic
disease, such as PD, often falls upon a family member taking on the
role as a CG. With the progression of PD, inevitable functional decline
might result in increased demands on CGs.
Methods: A simple random sample of CGs from the Swedish PD
Association was interviewed by telephone by four independent interviewers using a structured questionnaire.
Results: 90% response rate (404/451) where the CGs were divided in
three groups regarding to disease duration: 0-4, 5-10, and 11 years.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S222 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Mean age was 68 years, 62 % men and 38 % women, 98% spouses. Of
the 30% of CGs below the age of 65, one third was prepensioned or on
sick leave. About 1/3 was still working of which 40 % reported less
working time due to the PD patient. The relationships to children, work
mates, relatives, patient and friends were reported to be impaired in 6%,
12%, 19%, 26%, and 29%, respectively. There were signicant more
limitations in social activities like seeing friends and relatives (53%),
performing leisure (51%) as well as everyday activities (16%) for the
CGs with the longest PD/CG duration compared to the shorter duration
groups. Mean intake of medication was 4,8, 6,3, and 7,5 times a day in
respective group where CGs reported mandatory reminding of patients
of their medication intake in 27, 40, and 43%, respectively.
Conclusions: Informal caregiving of PD patients is conning with
signicant more connement with longer disease duration in certain
areas. CGs life space is being limited and restraint, which must be
considered when offering and planning health care for PD patients.
724
Antibiotics and Parkinsons disease: Overview on literature and
case reports
K.S. Paulus, V. Agnetti, P. Galistu, G.A. Cocco, G. Sechi (Sassari,
Sardegna, Italy)

Background: Retrocollis is not a clinical feature of idiopathic Parkinsons disease. In Parkinsons disease, malignant syndrome can be
precipitated by drug withdrawal, sodium abnormalities and menstruation. A case of reversible retrocollis induced by hyponatremia precipitated malignant syndrome is being reported in a case of Parkinsons
disease.
Methods: Case description.
Results: A 60-year-old gentleman with 3 year history of Parkinsons
disease was admitted with severe rigidity, fever and altered sensorium
following repeated bouts of vomiting. He was on thrice daily schedule
of half tablet of L-Dopacarbidopa (100mg25mg). He had a temperature of 101.5F and was drowsy. He was akinetic rigid with severe
axial and appendicular rigidity and had pronounced retrocollis. Serum
sodium was 109meq/L CPK was 1500 IU/L and there was leucocytosis
(15,000/mm3). Non-contrast CT brain was normal as were CSF study,
thyroid function test and rest of the metabolic parameters. No obvious
focus of sepsis was detected. Serum sodium was slowly corrected at a
rate of 8 meq/L per 24 hours and bromocriptine 7.5 mg/day was added.
Gradually sensorium normalised. Over a period of 2 weeks retrocollis
reverted back completely.
Conclusions: Hyponatremia may precipitate malignant syndrome in
Parkinsons disease. Retrocollis may be seen in Parkinsons disease
with malignant syndrome, which if treated promptly is reversible.

Objective: We describe ve patients with parkinsonian syndromes in

which antibiotic treatment of infectious comorbidity has caused alterations of the clinical symptoms and discuss possible meccanisms of
drug interferences.
Background: Tetracyclines could have neuroprotective and neurorestorative activity. Some quinolones, such as gatioxacine, cyprooxacine, ooxacine, could be responsible of psicosis; convulsive crisis are
caused by another uoroquinolone, levooxacine. These interaction are
thought to be caused either by alteration of bacterial ora and enteric
drug absorption, or by inhibition of drug elimination. Mechanisms of
interaction have been hypothesized at the level of GABA, glutamate,
and adenosine receptors. Observations have been reported in which
uoroquinolones have a high afnity for the GABA-A receptor, which
blocks the natural ligand GABA, and causes an excessive receptor
stimulation which could be the reason of central adverse events of
antibiotics, such as headache, agitation, dizziness, confusion, sleep
disturbances, psychoses, and rarely, convulsion. Little is known about
antibiotic interferences in patients affected by Parkinsons disease.
Controverse results are reported about the interaction of minocycline in
animals treated with MPTP. Some authors postulated a protective effect
against the MPTP toxicity by inhibition of the microglia, whereas
others hypothesized that the minocycline could increase the toxicity of
MPTP against the dopaminergic neurons by inhibition of the reuptake
of dopamine and MPP. Nakatsuka et al. (2006) observed that clarithromycin could increase the action of cabergoline reducing the drug
metabolism by inhibition of cytochrome P450, and/or inhibition of the
P-glycoprotein-mediated excretion. Fluoroquinolones are thought to
have a direct action on the CNS mediated by inhibition of adenosine
receptors. Diquet E, Fernagut PO, Wei X, Du Y, Rouland R, Gross C,
Bezard E, Tison F. Deleterious effects of minocycline in animal models
of Parkinsons disease and Huntingtons disease. Eur J Neurosci 2004;
19(12):3266-76. Nakatsuka A, Nagai M, Yabe H, Nishikawa N, Nomura T, Moritoyo H, Moritoyo T, Nomoto M. Effect of claritromycin
on the pharmacokinetics of cabergoline in healthy controls and in
patients with Parkinsons disease. J Pharmacol Sci 2006;100:59-64.
725
Reversible Retrocollis in a case of Parkinsons disease with hyponatremia induced malignant syndrome
S. Chandran (Trivandrum, Kerala, India)
Objective: Presentation of a rare case of reversible retrocollis in
Parkinsons disease, due to hyponatremia induced malignant syndrome.

Movement Disorders, Vol. 22, Suppl. 16, 2007

FIG. 1 (725).

POSTER SESSION III, THURSDAY, JUNE 7, 2007

726
Comparison of pharmacokinetics and pharmacodynamics of combined immediate- and extended-release (IRER)/carbidopa/levodopa formulations (VADOVA IRER) with IR carbidopa/levodopa and controlled-release (CR) carbidopa/levodopa in
Parkinsons disease (PD)
J. Nutt, P.A. LeWitt, A. Ellenbogen, S. Wang, T. Nguyen, S. Khor,
A. Hsu (Portland, Oregon, USA)
Objective: Compare VADOVA, a formulation of immediate- and
extended-release carbidopa/levodopa, with current products.
Background: CR carbidopa/levodopa preparations are hampered by
slow onset of clinical benet. IR preparations tend to have a shorter
duration of action when administered in smaller doses to avoid high
plasma peak concentrations and increased dyskinesia. A combination
of IR and ER preparations could reduce these problems with currently
available carbidopa/levodopa preparations.
Methods: Seventeen PD subjects with motor uctuations participated
in a randomized, quadruple-dummy, double-blind, crossover comparison of single doses of immediate-release carbidopa/levodopa (2 tabs of
25/100), controlled-release carbidopa/levodopa (50/200 CR), VADOVA 50-200 (25/100 IR25/100 ER) and VADOVA 62.5-250
(12.5/50 IR50/200 ER). Plasma levodopa concentrations and parkinsonism were measured at 30 minute intervals for up to 8 hours following drug administration after overnight without antiparkinsonian medications. Data were compared by analysis of variance with repeated
measures and pair wise comparisons.
Results: The IR and the two VADOVA IRER preparations yielded
higher plasma levodopa concentration earlier than the CR product
(p0.02 up to 1.5 hour) and the time to turning on, as measured by
walking speed was correspondingly reduced relative to CR product
(p0.074). The area under the plasma levodopa concentration-time
curve (AUC) and the peak concentration (Cmax) were greater with
immediate-release and VADOVA IRER preparations than with CR
product (p 0.002). Likewise, the duration of antiparkinsonian effects
measured with tapping, walking, tremor and dyskinesia scores were
generally greater with IR product and with VADOVA IRER products, although some did not reach statistical signicance due to small
sample size. The maximum dyskinesia scores trended from least with
the CR product to greatest with the IR product (p 0.04).
Conclusions: VADOVA, a combination of immediate- and extended-release carbidopa/levodopa preparation, may offer benets in treating patients with PD and motor uctuations.

S223

controlled study, in 48 non-demented, advanced PD patients, suffering

from major depression, despite their use of optimal dopaminergic
doses.
Results: Compared with placebo, a signicant improvement on the
Montgomery Asberg Depression Rating Scale was observed after 14
days on desipramine and after 30 days on citalopram. Signicant
improvement of the MADRS dysphoric apathy/retardation symptoms
(lassitude, inability to feel, apparent sadness, and concentration difculties) was observed after 14 days in the desipramine group, compared
with the other symptoms and groups. After 30 days, both antidepressants had produced equivalent improvements in terms of depression,
anxiety and quality of life.
Conclusions: The rapid effect of desipramine suggests that noradrenergic system is mainly involved in advanced parkinsonian depression and may be characterized by an effect on dysphoric apathy/
retardation symptoms. Antidepressants should therefore be chosen
according to the depression symptoms and the drug prole.
728
Botulinum toxin treatment for anterocollis in Parkinsons disease
H. Ito, Y. Takanashi (Akishima, Tokyo, Japan)
Objective: To evaluate the efcacy of botulinum toxin type A
(BTX-A) for anterocollis in Parkinsons disease (PD).
Background: Anterocollis appears in some patients with PD, and the
method of its treatment has not established.
Methods: We enrolled four PD patients with anterocollis who exhibited focal dystonia. None of the patients showed improvement of
anterocollis by drug control. We injected BTX-A into the sternocleidomastoid muscles, calenus muscle, and splenius muscle of the head.
Results: Anterocollis showed progressive improvement with continued treatment in all patients. In one patient, anterocollis disappeared
completely after three sessions of BTX-A injections, but dysphagia and
husky voice developed. CT showed expansion of the esophagus. In
addition, poor vocal chord movement and pharynx were also observed
by berscopy. In the other patients, mild signs of bulbar palsy were
noted. These symptoms disappeared without treatment.
Conclusions: Invasion of BTX-A may cause weakness of the esophagus
and pharyngeal muscles. In a patient showing disappearance of anterocollis, BTX-A may have invaded prevertebral muscles located near the
esophagus, vocal chords, and calenus muscle. Anterocollis in PD could be
treated with BTX-A; however, further study is needed to establish a safe
method of BTX-A injection into the prevertebral muscles.

727

729

Rapid efcacy of a noradrenergic reuptake inhibitor in depression

in advanced Parkinsons disease: A double-blind, randomized,
placebo-controlled study
D. Devos, I. Poirot, K. Dujardin, S. Duhem, B. Lucas,
N. Waucquier, C. Moreau, P. Bocquillon, P. Devos, K. Ajebbar,
B. Thielemans, O. Cottencin, P. Thomas, A. Destee, R. Bordet,
L. Defebvre (Lille, France)

Novel LRRK2 mutation in the Roc domain in an Western Australian family with autosomal dominant late-onset Parkinsons disease
(LOPD)
F.L. Mastaglia, Y. Huang, G.M. Halliday, D.B. Rowe, C.M. Sue
(Nedlands, WA, Australia)

Objective: To compare the efcacy between a noradrenergic and a

serotonergic antidepressant in a placebo-controlled study, in advanced
parkinsonian patients suffering from major depression in order to
formulate recommendations for antidepressant use in Parkinsons disease (PD).
Background: Depression in early Parkinsons disease (PD) is probably related to a dopamine decit - explaining the benecial effect of
antiparkinsonian drugs. In advanced PD, specic antidepressants are
needed because depression may be related to noradrenaline or serotonin
pathways. Determining an antidepressants pharmacological prole
(efcacy and onset of action) is thus essential. Recent reviews have
underlined the lack of controlled trials and the ensuing difculty in
formulating recommendations for antidepressant use in PD.
Methods: In three parallel groups, we assessed, after 14 and 30 days,
the efcacy and acceptability of two antidepressants (desipramine,
selective noradrenergic reuptake inhibitor and citalopram, selective
serotonin reuptake inhibitor), in a double-blind, randomized, placebo-

Objective: To report a novel mutation in the Roc domain of LRRK2

in a 3-generation family with LOPD.
Background: Over 20 mutations in LRRK2 have been reported to
cause familial or sporadic Parkinsons disease (PD). The commonest
mutations are in the kinase domain in exon 41 (at G2019S) and in the
Roc domain in exon 31 (at R1441G/C/H).
Methods: We performed PCR-RFLP analysis to screen for mutations in
exon 31 and 41 in a large Western Australian PD cohort (n109). We
subsequently identied mutations by direct sequencing of the PCR product.
Results: We found an A1442P (4324 GC) mutation in a 65-yearold male proband, with clinical features typical of PD. One of his three
sisters, aged 69 years, who on clinical examination had mild unilateral
limb rigidity also had the A1442P mutation. Five other affected family
members, at least three of whom were thought to have PD, were not
available for clinical or genetic study.
Conclusions: We conclude that the A1442P mutation is pathogenic
because: a) the A1442 residue is highly conserved; b) the substitution
of proline for alanine is likely to alter the secondary structure of the
protein; c) it may alter the binding properties of the RabSF3 motif on

Movement Disorders, Vol. 22, Suppl. 16, 2007

S224 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

the surface of Rab GTPase in a similar way to mutations at residue
1441; and d) it has not been identied in any other member of our
Australian cohort or in large numbers of patients or controls in previous
studies. Similar to other mutations in LRRK2, the A1442P mutation
may express variable penetrance. Functional studies are warranted to
assess pathogenic mechanisms.
730
Psychiatric features of caregivers of Parkinsons disease patients
with dopamine dysregulation syndrome: Preliminary results of a
clinical study
F. Beskardes, A. Gunduz, G. Kiziltan, S. Ertan, S. Ozekmekci
(Istanbul, Turkey)
Objective: To nd out whether an association between the dopamine
dysregulation syndrome (DDS) of Parkinsons disease (PD) patients
and mood and anxiety disorders of primary caregivers. We present the
preliminary results of this study.
Background: It has been reported that depression and anxiety of
primary caregivers of PD patients are associated with low scores of
Mini Mental State Examination (MMSE) and high scores of Unied
PD Rating Scale (UPDRS) of their PD patients. But it is unknown
whether DDS is associated with caregivers stress.
Methods: Eighteen caregivers (ve male, 13 female) of non-demented PD patients having DDS examined between September 2005
and September 2006 were included. Eleven age and gender-matched
caregivers of PD patients without DDS who had similar disease duration and Hoehn-Yahr stage as those with DDS consisted the control
group. Caregivers in both groups were evaluated for sociodemographical data and were underwent MMSE, Hamilton depression, Hamilton
anxiety, SCID depression and SCID psychosis tests and all patients
were examined for DDS symptoms and by UPDRS. Participants who
scored less than 24 on the MMSE were excluded.
Results: None of the caregivers in control group had major depression, whereas among those of DDS patients, 3 (16.7%) had major
depression. Mean scores of Hamilton anxiety and depression tests were
in normal ranges and did not differ from control group. However,
according to results of SCID tests, 56.3% of caregivers in DDS group
had generalized anxiety disorder (GAD) in comparison to 18.2% in
control group (p0.048). The most common DDS symptoms in patients whose caregivers had GAD were hypersexuality, aggression and
compulsive eating and compared to caregivers without GAD hypersexuality was the leading.
Conclusions: DDS appears to be associated with GAD of primary
caregivers. Hypersexuality is the most commonly encountered DDS
symptom among those patients whose caregivers have GAD.
731
Can dual-forceplate posturography support the diagnosis Parkinsons disease?
A.C. Geurts, N. Voermans, M.G. Diender, V. Weerdesteyn,
B.R. Bloem (Nijmegen, Netherlands)
Objective: To identify dynamic postural asymmetry in early Parkinsons disease (PD) using dual-forceplate posturography and to determine whether such analysis may support this diagnosis.
Background: PD is primarily a clinical diagnosis based on a combination of hypo- and bradykinesia, rigidity, tremor and postural instability. Distinguishing PD from various forms of atypical parkinsonism
(AP) may be difcult, particularly in early stages. An important supportive criterion is the degree of asymmetry, which is typically pronounced at the onset of PD, but much less so in AP. Asymmetries can
be seen both in the arms and legs, and possibly also in postural control.
Here, we evaluated whether static posturography may be a tool to
identify asymmetries in the kinetic contribution of each leg to equilibrium control.
Methods: Seventeen patients with relatively early stages of PD
(mean total UPDRS score 12.6 during ON phase; mean age 59.4 years)
and 10 healthy subjects (mean age 64.5 years) underwent the same
assessments with a dual-plate force platform. Two series of ve 30-

Movement Disorders, Vol. 22, Suppl. 16, 2007

second quiet-standing tasks were done: (1) with eyes open, (2) with
eyes closed, (3) while performing a concurrent arithmetic task, (4) with
eyes open on foam and (5) with eyes closed on foam. RMS values for
center-of-pressure (COP) velocity were calculated, separately for each
individual foot and for both feet together. Differences between the feet
were expressed in a symmetry index (SI (difference / average)*100).
Results: Although PD patients were generally less stable than the
controls, this effect was not signicant, indicating relatively good
balance. Both groups showed comparable increases in COP velocity
with increasing task complexity. There was no relationship between the
SI and the UPDRS score. Five patients showed a SI outside the 95%
range of the controls. In these patients the kinetically least active leg
corresponded with the most affected arm based on clinical examination.
Conclusions: It is possible to identify dynamic postural asymmetry
in relatively mildly affected PD patients by dual-forceplate posturography, and this balance asymmetry corresponds with upper body asymmetry. Future studies must clarify whether this simple technique can
support the clinical diagnosis of PD and might be used to monitor
progression or evaluate therapeutic interventions in PD.

732
Quality of life improvement in Parkinsons patients after DBS:
Identifying the super-responders
I.U. Haq, M.S. Okun, R. Rodriguez, K. Foote, C. Jacobson,
C. Garvan, H. Fernandez (Gainesville, Florida, USA)
Objective: In this study we directly examined quality of life (QOL)
to determine those factors associated with DBS success.
Background: Most successes of deep brain stimulation (DBS) therapy for Parkinsons disease (PD) have been judged on the basis of
motor improvement.
Methods: We compared the pre- and six-month post-lead implantation PDQ-39 scores of Parkinson patients who underwent DBS surgery
at our institution. The PDQs domains were summed to yield a total
ranging from 0-800 for each patient. QOL improvement was measured
as the absolute difference between the summed post-operative and
pre-operative scores, and as the percent improvement at six months.
The cohort was divided into quartiles; patients in the highest were
named super responders and compared to the other cohorts. Correlation
analyses were also performed on the entire cohort. Variables examined
included demographics, cognition, mood, functional impairment, and
motor function. Categorical data was analyzed using chi-square and
Fisher s Exact tests. Numerical data was analyzed using Spearman
correlations and Wilcoxon rank sum tests.
Results: 44 PD patients had a mean: age of 60 years, disease duration
of 12.7 years, education of 15.4 years, MMSE score of 28.3, DRS score
of 137.6, QOL score of 290.5, and UPDRS off scores of 44.8. Correlation analyses on the dataset between the chosen variables and absolute QOL improvement yielded the following correlates: less education
(p0.0215); lower DRS scores (0.0276); higher UPDRS sensory complaints scores (p0.0142); and higher UPDRS ADL scores
(p0.0142). Only sensory complaints remained signicant on dataset
analysis using percent QOL improvement and when comparing super
responders to poorest responders (p0.028) or to the cohort (p0.044).
Conclusions: In this cohort, the best QOL improvement occurred in
PD patients with the greatest baseline sensory complaints. PD patients
with less education, greater functional impairment, and, surprisingly,
worse cognition at baseline had better QOL improvement at 6 months
post-DBS. However, while our analysis revealed several positive predictors using absolute QOL improvement, these were not supported
when using percent QOL improvement. Future studies may need to be
better powered to determine predictors of QOL super-responders after
DBS.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

733
Increased Synphilin-1 expression in elderly and parkinsonian
brains
A.T. Krygowska-Wajs, T. Lenda, K.I. Ossowska, D. Adamek,
E.A. Gryz-Kurek, J. Kunz (Cracow, Poland)
Objective: We compared the expression of synphilin-1, a-synuclein,
TH and ubiquitin with the presence of MBs in brains of Parkinsons
disease patients and individuals without any neurodegenerative diseases.
Background: Synphilin-1 an amino acid protein associates with
a-synuclein and promotes the formation of cytoplasmic inclusions
recognized as the pathological hallmarks of neurodegenerative diseases. Recent studies have suggested that intranuclear inclusions Marinesco bodies (MBs) described in the substantia nigra and locus
coeruleus of elderly brains, dementia with Lewy bodies, trinucleotide
repeat neurodegenerative disease are involved in degradation of proteins by the ubiquitin-proteasome system. Mbs frequency increases
with aging and correlates signicantly, in inverse fashion, with striatal
concentration of the dopamine transporter and tyrosine hydroxylase
(TH).
Methods: The studies were performed on two parkinsonian brains
and the brains of 22 individuals who died of various causes but without
the clinical signs and symptoms of neurodegenerative diseases. Immunocytochemical examinations were performed on the substantia nigra
and different structures of the brain using monoclonal antibodies
against a-synuclein, tau-protein, ubiquitin and beta amyloid. Levels of
synphilin-1, ubiquitin, a-synuclein and TH were estimated by Western
blotting in the substantia nigra and striatum.
Results: MBs were found in substantia nigra in parkinsonian brains
and 80% of non-neurological brains. The level of synphilin-1 in parkinsonian brains was increased in the substantia nigra and caudate
nucleus, whereas the level of TH was decreased in the substantia nigra,
caudate nucleus and putamen. In the non- neurological brains with MB
the level of synphilin-1 was increased, and the level of TH was
reduced. No change in a-synuclein level was revealed in parkinsonian
as well as non-neurological brains. However, -synuclein dimer/monomer ratio was dramatically increased in the substantia nigra of parkinsonian brains. Moreover, there were positive correlations between
synphilin-1 level and MB frequency as well as aging.
Conclusions: Our results suggest that increased expresion of synphillin-1 parallels MBs formation and aging and may contribute to the
loss of dopaminergic neurons and the decrease in TH in the nigrostriatal system.
734
Exercise-induced alterations in striatal glutamate in an animal
model of Parkinsons disease
C.K. Meshul, J.K. Wiedemann, C. Moore, R.J. Koch, R.H. Walker
(Bronx, New York, USA)
Objective: To determine the effects of treadmill exercise on striatal
glutamate synaptic function and dopamine levels following a unilateral
lesion of the nigrostriatal pathway in rats.
Background: Exercise prior to a lesion of nigrostriatal dopamine
(DA) pathway is neuroprotective for DA cells of the substantia nigra in
animal models of Parkinsons disease (PD). There have been very few
studies in which exercise is initiated following damage to DA neurons.
Methods: The nigrostriatal pathway was unilaterally lesioned with
6-hydroxydopamine (6-OHDA). Four weeks later, rats were exercised
on a treadmill for one hour/day, ve days/week for four weeks (6-OH/
EXER, 6-OHDA only, EXER only, Control). In vivo microdialysis of
the dorsolateral striatum was carried out for glutamate and DA analysis.
The density of striatal nerve terminal glutamate immunolabeling was
determined. Behavioral tests included apomorphine-induced rotations,
cylinder test, and forelimb reaching.
Results: A 26% decrease in striatal extracellular glutamate was seen
with 6-OHDA lesions alone. Rats which exercised (EXER) showed a
30% decrease in striatal glutamate. A 74% decrease in glutamate was
seen in the 6-OHDA/EXER compared to controls. There was a 2-fold

S225

increase in extracellular striatal DA in the EXER group compared to

controls. In 6-OHDA and 6-OHDA/EXER groups, extracellular DA
levels were reduced to 25% of the controls. There was a 28-53%
increase in the density of nerve terminal glutamate immuno-gold labeling in each of the treatment groups compared to controls, inversely
correlating with the decrease in extracellular glutamate. In the
6-OHDA/EXER group, there was a 50% decrease in the number of
apomorphine-induced rotations, a 50% increase in the time animals
spent running on the treadmill, no change in the use of the affected
forepaw in the cylinder test, and a 5-fold increase in the ability of the
affected forepaw to reach compared to the 6-OHDA group.
Conclusions: The data suggest that exercise reduces extracellular
levels of striatal glutamate by increasing synaptic release, increases
levels of DA in the non-lesioned group, and results in an improvement
in some motor behaviors but not others. Further studies at the neurochemical level of the role of exercise in PD are warranted.
735
A computerized survey of pain in Parkinsons disease patients: A
pilot feasibility study
D.B. Page, F. Weaver, D.J. Wilkie, T. Simuni (Chicago, Illinois,
USA)
Objective: To assess the feasibility of using a computerized, stylustouch based questionnaire to retrieve real-time data on pain in Parkinsons disease (PD) patients in a clinical setting. We also describe
sensory pain data.
Background: The cornerstone of PD is motor dysfunction; however,
approximately 40% of PD patients exhibit bothersome pain symptoms
that go unrecognized and/or untreated.
Methods: Fourteen patients with PD, treated at a university PD
center, were selected to complete a computerized version of the McGill
Pain Questionnaire (PAINReportIt). These data were supplemented
with written questionnaires measuring demographics, medical history,
depression (BDI-II), impairment of activities of daily living (ADL),
and severity of PD (PDQ-39, UPDRS Part II-III). We also asked
patients to comment on using PAINReportIt.
Results: 93% percent of subjects completed at least 75% of the
questionnaire (mean97.6%), in an average of 19.9 minutes. 5 of 14
subjects (36%) required physical assistance from the research assistant
to complete PAINReportIt; subjects requiring assistance scored signicantly worse on several PD scales (UPDRS ADL and Motor and
PDQ-39 scales), suggesting that PD-specic symptoms may contribute
to the need for assistance. In exit interviews, 90% of subjects supported
use of the computer for data collection. Subjects reported a mean
24-hour maximum pain intensity of 4.5 on a 0-10 scale (range 0-9; SD
2.9). Over half (57%) reported continuous pain. 79% of subjects
described pain using neuropathic pain descriptors and 36% using
nociceptive pain descriptors. The most frequently reported sites of pain
were back (71%), shoulders (43%), neck (29%), and legs (29%). The
top ve descriptors for pain were: tiring (50%), hurting (50%), sharp
(50%), sore (43%), and penetrating (43%).
Conclusions: PAINReportIt is an effective tool to collect pain data
in real-time from PD patients. Results can be printed in a one-page
summary, which may be used to assist with pain diagnosis and management. This computerized survey will need modication to meet the
needs of PD-related motor disabilities. Preliminary analysis of pain
data reveals a heterogeneous pain pattern. In the future, this tool can be
used to characterize pain in a larger cohort of PD patients and could be
linked to decision support for pain treatment and patient education.
736
Clinical correlates of camptocormia in Parkinsons disease
D. Ottaviani, D. Tiple, C. Colosimo, G. Fabbrini, G. Defazio,
A. Berardelli (Rome, Italy)
Objective: To evaluate the clinical features of patients with Parkinsons disease (PD) and camptocormia, and to compare them with those
patients without camptocormia.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S226 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: Camptocormia (Greek: karmos bent kormos
trunk of a tree) is a rare condition, characterized by an abnormal exion
of the trunk that appears when standing or walking and disappears in
the supine position. It can be associated with a wide spectrum of
musculoskeletal and neurological conditions, including PD but also
muscular dystrophies, inammatory myopathies, segmental dystonia,
amyotrophic lateral sclerosis, or present in idiopathic form. Only a few
studies have dealt with the issue of camptocormia and PD. The nosological position and the origin of this unusual symptom associated with
PD remain unclear.
Methods: Clinical data including age, sex, disease duration, presence
of motor and cognitive complications, treatment regimen, were collected in consecutive PD outpatients. Parkinsonian features were assessed using the motor (part III) Unied Parkinsons disease Rating
Scale (UPDRS) and the severity of the disease was scored by the
Hoehn and Yahr (HY) staging. Presence of camptocormia, dened as
an anterior exion of the thoracolumbar spine of at least 45 degrees
appearing in orthostatism or after gait and disappearing in the recumbent position, was evaluated.
Results: In our population of 274 PD patients, 27 (9.8 %) presented
camptocormia. According to previous reports, we found a positive
correlation between the severity and the duration of the disease and the
presence of camptocormia (P 0.0001), Patients with camptocormia
were also characterized by a signicantly higher mean age than patients
without it. Four of them had received a vertebral surgery, in a percentage signicantly higher (P 0.03) than those patients without camptocormia. Hallucinations and cognitive disturbances were also signicantly more common in the group with camptocormia.
Conclusions: The frequency of camptocormia in our population is
considerably higher than that reported from others authors. In addition,
in our study camptocormia is also associated with the advanced PD
Previous vertebral surgery might be considered a risk factor for the
development of camptocormia.

738
Prevelance of dyskinesia switching from pulsatile to continuous
rotigotine administration in MPTP-treated marmosets
K. Stockwell, D.K.A. Scheller, S. Rose, M. Jackson, P. Jenner
(Monheim, Germany)
Objective: The present study investigated the prevalence of dyskinesia following the switch from pulsatile rotigotine to continuous
administration of rotigotine in the MPTP-treated marmoset model of
Parkinsons disease (PD).
Background: Rotigotine is a non-ergolinic dopamine agonist developed for the treatment of idiopathic Parkinsons disease (PD). Clinically, it is administered transdermally to provide stable plasma levels.
Methods: Stable motor decits in common marmosets (Callithrix
jacchus; 325-389g; 5-8years, n6) were induced by treatment with
MPTP (2.0mg/kg sc for 5 days). Animals were treated with pulsatile
rotigotine (0.15 mg/kg, sc, BID, 3.5 h apart) for 28 days followed by 28
day continuous infusion of rotigotine (0.18 mg/kg/day, sc) via osmotic
minipump. Behavioral assessments (locomotor activity, motor disability and dyskinesia) were made weekly by trained observers. Experiments were carried out in accordance with UK legal requirements.
Results: Pulsatile rotigotine treatment transiently improved locomotor activity and motor disability however mild dyskinesia was initially
observed that gradually increased in severity over the 28 days to a
marked/moderate rating. Switching from pulsatile rotigotine treatment
to continuous rotigotine administration tended to reduce dyskinesia
duration and severity but did not inhibit its expression.
Conclusions: Switching from pulsatile to continuous rotigotine treatment tended to reduce dyskinesia expression whilst sustaining the
improvement in motor disability. Thus, pre-existing dyskinesia induced
by pulsatile administration of dopamine agonists in monkeys can be
improved by a switch to continuous rotigotine administration.
739

Reaching out to rst degree relatives

J. Posen, Z. Heiblum, N. Giladi (Tel Aviv, Israel)

The effectiveness of pramipexole, cabergoline and pergolide in

early and advanced Parkinsons disease and comparision of the
results with each other
O. Yilmaz, B. Yucel-Altan, S. Oruc, S. Gok, N.S. Oztekin
(Afyonkarahisar, Turkey)

Objective: Provide rst-degree relatives with an opportunity to receive information, raise concerns, and assess needs for appropriate
future activities.
Background: Parkinsons disease (PD) as a chronic degenerative
disease with inherent uncertainties, impacts not just on patients but also
on their families well-being and social functioning. Additional stressors for rst-degree relatives (children and siblings) may be the genetic
component for PD. There is, however, a lack of information regarding
this populations needs. As a rst step in reaching out to rst-degree
relatives, the Movement Disorder Unit (MDU) planned a seminar
specically for children and siblings of Parkinsons patients.
Methods: Personal letters were sent between October 2006 and January
2007 to patients treated by physicians in the MDU. The letters explained
the projects rationale and requested patients agreement to our contact
with their siblings and children as well as postal details. Letters were then
sent to relatives residing in Israel, inviting them to a seminar to be held in
the center of the country. The Tel Aviv Medical Center ethics committee
authorized this contact with rst-degree relatives.
Results: Letters were sent to 496 patients, 259 responded. Names of
563 rst-degree relatives were received including 455 children averaging three children per family and 86 siblings: 220 sons, 234 daughters, 36 brothers, and 50 sisters. Reported ages ranged between 11 and
70. Details were given of ve children under 18. 539 letters were sent
to relatives inviting them to the seminar. The extent of response will be
reported at the conference following the seminar that will be held on
the 23 rd of February 2007.
Conclusions: The method of approaching children of patients via the
patients is an efcient one and allows them to choose whether or not to
be involved.

Objective: To evaluate efcacy, tolerability and side effects of

pramipexole, cabergoline, and pergolide in the early and advanced
stages of Parkinsons disease (PD) and to compare the results with each
other.
Background: Though too many studies have been reported, there is
no universally accepted rst choice of dopamine agonist, and prescribing decisions are usually made on the basis of personal experience,
experience with a particular agent, or desire (by the clinician or patient)
to try something new.
Methods: 67 patients were enrolled to the study according to Hoehn &
Yahr (H&Y) clinical stage and were divided into three groups who were
evaluated by a blind physician every week for the rst month, and monthly
for six months. The rst group consisted of 33 patients Hoehn & Yahr
stage I-II, 15 of them using pramipexole, 10 pergolide, and eight cabergoline as monotherapy. The second group consisted of 34 patients, in
H&Y stage III-IV, nine pramipexole, 17 using cabergoline and eight using
pergolide as an adjunct to levodopa. The patients were evaluated according
to UPDRS, global clinical impression scale (GCIS).
Results: In monotherapy, differences among UPDRS were statistically signicant in the pramipexole, pergolide and cabergoline groups
(p0,01). In pramipexole group the changes in UPDRS were signicant in tremor, rigidity and body bradykinesia, in cabergoline group in
rigidity and body bradykinesia and in pergolide group, in rest and
action tremor. In advanced PD the differences among UPDRS, were
signicant in all three groups (p0,01). In the pramipexole and the
cabergoline groups, GCIS were signicant both in the early and advanced stages (p0,01). However, in the pergolide group, related to the
side effects, both in the early and advanced stages, especially because
of nausea and hallucinations, GCIS were insignicant (p0,408 and
p0.096 respectively).

737

Movement Disorders, Vol. 22, Suppl. 16, 2007

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Conclusions: The results of this double blind study revealed that both
in early and advanced PD, pramipexole and cabergoline improves
impairment and disability due to PD and allowing a signicant difference in GCIS compared to pergolide.
740
Striatal dopamine transporter imaging correlates with depressive
symptoms and Tower of London task performance in Parkinsons
disease
I. Rektorova, H. Srovnalova, R. Kubikova, J. Prasek (Brno, Czech
Republic)
Objective: Investigate whether the 123I-FP-CIT (DaTSCAN) uptake
in striatum correlates with depressive symptoms and cognitive performance in Parkinsons disease (PD) patients.
Background: Imaging with DaTSCAN provides a marker for presynaptic neuronal degeneration. Little is known about association between 123I-FP-CIT striatal uptake and non-motor symptoms of PD.
Methods: 20 patients with PD (mean age 61.7 12.7 years; disease
duration 5.6 3.5 years) underwent the 123I-FP-CIT single photon
emission computed tomography. Following scales used to evaluate
depressive symptoms and cognitive performance: Montgomery and
Asberg Depression Rating Scale (MADRS), Mini-mental State Examination (MMSE), verbal uency test (VFT), Stroop test, Tower of
London task (TOL), Clock test. The ratios of striatal to occipital
binding for the entire striatum, putamina, and putamen to caudate index
were calculated in both the basal ganglia (QuantiSPECT, Mirada Solutions, 2004). The association between neuropsychiatric measures and
DAT availability for each ROI was calculated by use of Pearson
correlation; where applicable, multiple regression analysis was used to
control for age and disease duration.
Results: We found signicant correlations between MADRS, TOL,
clock test scores and 123I-FP-CIT uptake in various striatal ROIs.
Multiple regression analysis conrmed the signicant relationship between TOL performance and putamen to caudate ratio and between
MADRS and both the striatal and putaminal DAT availability.
Conclusions: Our results support the hypothesis that depressive
symptoms in PD patients are related to the dopaminergic hypofunction
in both the striata and putamina, while cognitive performance on the
TOL task is rather related to the dopaminergic decit in the caudate
nuclei.
741
Nociceptin/orphanin FQ receptor antagonists reverse parkinsonism in MPTP-treated mice and non-human primates
M. Morari, R. Viaro, M. Marti, R. Sanchez-Pernaute, O. Isacson
(Ferrara, Italy)
Objective: To evaluate the ability of the nociceptin/orphanin FQ
(N/OFQ) receptor (NOP receptor) antagonist J-113397 to reverse parkinsonian-like symptoms in mice and non human primates treated with
MPTP.
Background: N/OFQ has been shown to inhibit nigrostriatal dopaminergic transmission and motor function (Marti et al., 2004, J Neurosci 24, 6659-6666) and to inuence symptoms and neurodegeneration
associated with experimental parkinsonism (Marti et al., 2005, J Neurosci 95, 9591-9601). In particular, pharmacological blockade of hyperactive N/OFQergic transmission in the substantia nigra by NOP
receptor antagonists improved motor function in rats made akinetic by
haloperidol or 6-OHDA lesioning.
Methods: C57BL/6J mice were treated with MPTP (420 mg/kg
i.p., 90 minutes apart) and motor activity evaluated by means of the bar,
drag and rotarod tests (Marti et al., 2005). Seven days after MPTP,
J-113397 was administered (0.01 mg/kg i.p.) and behaviour monitored
at 10 and 60 minutes post-injection. Macaca fascicularis primates
developed stable moderate-to-severe parkinsonism four months after
i.v. administration of MPTP (6-12 mg; Parkinson Rating Scale 18-22).
The effect of J-113397 (0.01 mg/kg i.m.) was evaluated by means of a
PRS scale 30 minutes after drug administration. L-DOPA was given

S227

both in mice (10 mg/kg, i.p.) and macaques (30 mg/kg, i.m.) as a
control.
Results: MPTP-treated mice displayed increased time on bar, reduced number of steps and impaired performance on the rotarod
compared to vehicle-injected mice. L-DOPA reversed motor decits in
all behavioural tests, its action being still evident after 60 minutes.
J-113397 reproduced L-DOPA effects, although its action was less
pronounced and short-lasting. L-DOPA also reversed parkinsonism in
MPTP-treated macaques (45% improvement compared to baseline).
Again, J-113397 mimicked L-DOPA effects, although less effectively
(20%). J-113397 was most effective against hypokinesia.
Conclusions: Our data suggest that endogenous N/OFQ actions
contribute to motor decits associated with MPTP administration in
both mice and non human primates, and strengthen the view that NOP
receptor antagonists may represent a novel therapeutic approach to
Parkinsons disease. Supported by the Michael J Fox Foundation for
Parkinsons Research.
742
Clinical characteristics of Parkinsons disease among Jewish ethnic
groups in Israel
R. Djaldetti, S. Hassin-Baer, M.J. Farrer, T.A. Treves, Y. Barhum,
M.M. Hulihan, S. Yust-Katz, E. Melamed (Petah Tiqva, Israel)
Objective: To compare the course of PD among Ashkenazi and
Yemenite Jews in Israel and to investigate the prevalence of the
LRRK2 G2019S mutation in the Yemenite patients.
Background: Differences in prevalence rates and clinical characteristics of Parkinsons disease (PD) were reported among different ethnic
groups. The Yemenite Jews are a distinctive ancient ethnic group that
was separated from the Ashkenazi Jews. The LRRK2 G2019S mutation
was reported to cause parkinsonism in 18% in Ashkenazi Jews in New
York (NEJM 2006;354:424-5).
Methods: All Yemenite (n61) and Ashkenazi-originating countries
patients (n51) were included. Their medical records were screened
for age of disease onset and duration, rst clinical signs, the response
to levodopa therapy and presence of response uctuations, dyskinesias,
depression and cognitive impairment. Continuous variables were compared using ANOVA. Progression of the disease was compared using
linear regression, measured by the association between disease duration
and the UPDRS motor scores.
Results: Age of disease onset was lower in the Yemenite compared
to the Ashkenazi Jews (54.613.2 vs 62.19.5 years; p0.001), but
there was no difference in family history among groups. There was no
difference in the clinical presentation, disease duration and the prevalence of uctuations, dyskinesias, depression and psychiatric symptoms
between groups. However, UPDRS motor scores were higher in the
Yemenite compared to Ashkenazi Jews (3418.8 vs 2418; p0.01)
and disease progression was signicantly faster (r0.48, p0.05).
Albeit a small sample, the LRRK2 mutation was negative in all
Yemenite patients.
Conclusions: There are no differences in the clinical characteristics
of PD between Yemenite and Ashkenazi Jews, but the rate of disease
progression is more rapid in the Yemenite group. These ethnic differences might be attributed to genetic and environmental factors. Surprisingly, no Yemenite patients were identied with the Lrrk2 G2019S
mutation. Its absence may be explained by the fact that the Yemenite
ethnic division was completely separated from the Ashkenazi Jews.
743
Falls in Parkinsons disease: Analysis of the DoPaMiP study, a
cross-sectional survey in South-West of France
O.O.O. Rascol, L.L. Negre-Pages, DoPaMiP D. Study Group
(Toulouse, France)
Objective: To describe the prevalence of falls and the clinical features of Parkinsons disease (PD) patients with falls.
Background: Falls are frequent in patients with PD but poorly
studied.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S228 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Methods: Cross-sectional survey conducted in 450 outpatients with
idiopathic PD (UKPDSBB), 18 years, MMSE24, no deep brain
stimulation, no serious disease that may affect the vital forecast, accepting to participate to the survey and randomly selected by 28
neurologists; Data collection: (1) clinical examination (demographics,
UPDRS and Hoehn & Yahr (HY), treatments) (2) a self-administered
patient questionnaire assessing anxiety and depression symptoms
(HADS scale), sleep quality (Pittsburg scale), quality of life (PDQ 39).
Descriptive analyses on SAS software were performed to compare
patients with and without falls. Patients with falls were dened those
with a score 1 on UPDRS item 13 in the ON condition.
Results: A preliminary descriptive analysis was performed on 419
valuable patients (mean age 69 9 years, mean disease duration
6 4 years, HY median score 2). 20% (87) of our sample reported
falls in the ON condition. Patients with falls were more frequently
female, older (729 vs 689yrs), had more frequently home care
assistance, longer disease duration (85 vs. 44yrs, p10-4), more
frequent motor complications (55% vs. 32%, p10-4), more orthostatic
hypotension (24% vs. 9%, p10-4), more severe HY stage (median 3
vs. 2, p10-4), more severe total UPDRS (4819 vs. 2914, p10-4).
Most individuals items of UPDRS were more severe in patients with
falls except tremor (item 20) (1.51.8 vs 1.42, p0.6). Patients with
falls had more depressive symptoms (56% vs. 32%, p10-4) and higher
quality of life score (37 14 vs. 2512, p 10-4) while there were no
difference for anxiety symptoms and sleep quality. Patients with falls
had longer treatment duration (75 vs. 44, p10-4), received higher
LDopa equivalent daily dose (1152 942 vs. 899750, p10-4).
Conclusions: Twenty percent of DOPAMIP population of patients
with PD had falls in the ON condition. Such patients had older age,
were more frequently female, had longer disease duration, more severe
PD symptoms except tremor, longer treatment duration with higher
L-dopa daily dose, more motor complications, more orthostatic hypotension, more depressive symptoms and lower score of QoL.
744
L-Dopa induced dyskinesia in Parkinsons disease: Analysis of the
DoPaMiP study, a cross-sectional survey in South-West of France
L.L. Negre-Pages, O.O. Rascol, DOPAMIP D. Study Group
(Toulouse, France)
Objective: To describe the prevalence and clinical features of patients with L-dopa-induced dyskinesia (LDID) in a large series of
ambulatory patients with Parkinsons disease (PD) recruited in non
specialized neurological out-patient clinics.
Background: Prevalence, clinical features and quality of life of
patients with L-Dopa induced dyskinesia are poorly known.
Methods: Cross-sectional survey conducted in 450 outpatients with
idiopathic PD (UKPDSBB), 18 years, MMSE24, no deep brain
stimulation, no serious disease that may affect the vital forecast, accepting to participate to the survey and randomly selected by 28
neurologists. Data collection: (1) clinical examination (demographics,
UPDRS and Hoehn & Yahr (HY), treatments) (2) a self-administered
patient questionnaire assessing anxiety and depression symptoms
(HADS scale), sleep quality (Pittsburg scale), quality of life (PDQ
39).Descriptive analysis were performed to compare patients with and
without LDID. LDID patients were dened as those with a UPDRS
item 32 1 in the ON condition.
Results: A preliminary descriptive analysis was performed in 419
analysable patients (mean age 69 9 yrs, mean disease duration
6 4 yrs, HY median score 2). There were 24% (102) LDID
patients. LDID patients had higher level of education and higher
working position. LDID patients had longer disease duration (85 vs
44 years, p10-4), younger age at PD onset (5811 vs 6610,
p10-4), more motor uctuations (68% vs 17%, p10-4), more orthostatic hypotension (24% vs 9%, p10-4), more severe HY (2.5 vs
2, p10-4), more severe total UPDRS (3919 vs 3116, p10-4).
LDID patients had more depressive and anxious symptoms (HADSD7: 56% vs 32%, p10-4; HADS-A7: 60% vs 44%, p10-4) and
poorer QoL (35 13 vs 2413, p 10-4). LDID patients had longer

Movement Disorders, Vol. 22, Suppl. 16, 2007

treatment duration (85 vs 43, p10-4), received higher L-Dopa

equivalent daily dose (1424 959 vs 793669, p10-4).
Conclusions: 1/4 of DOPAMIP patients with PD had LDID. Such
patients had younger age at onset, longer disease duration, more severe
PD symptoms, more motor uctuations, more frequent orthostatic
hypotension, more depressive and anxious symptoms, lower QoL while
no difference were observed for hallucinations and sleep disorders.
Patients had longer treatment duration with higher L-dopa equivalent
daily dose.
745
The natural history of treated Parkinsons disease
J.J. Duarte, L.M. Garca Olmos, L.E. Clavera (Segovia, Spain)
Objective: Determined the predictable variables associated with the
progression and duration of illness of patients with Parkinsons disease.
Background: There have been many studies of clinical features that
related to progression and mortality of Parkinsons disease. A more
rapid progression in Parkinsons disease has been reported in patients
with rigidity-hypokinesia as the predominant symptom, a higher age at
onset, and delay in starting levodopa treatment.
Methods: On september 30, 2005, the les of 274 patients with
Parkinsons disease who had visited the outpatient clinic of General
Hospital in Segovia since January 1, 1980 were traced. A total of 273
patients were further analyzed. Age at reaching Hoehn-Yahr stage III,
in combination with age at onset, can be used to measure the rate of
progression of the disease. Survival analysis was used to study the
relation-ship of sex, presenting symptom and levodopa, and bromocriptine treatment with clinical course (progression and death).
Results: We demostrate that females, living in urban areas, with
an age of onset, belowed responding to a combination of L-Dopa
and bromocriptine, take a longer period of time to reach stage III.
Predictable variables of progression (Cox regression) in our cohort
were age at onset, the younger the patient the longer it takes to reach
stage III (x3 the mean). The lower the H-Y staging at diagnosis the
longer it takes to reach stage III (x2 the mean). Patients treated with
bromocriptine, initially or subsequently in combination with other
drugs, have a slower progression to stage III (x1 the mean). On the
other hand, patients with a predominant hypokinetic-rigid form of
PD at onset have a 30% chance of a more rapid progression.
Conclusions: Patients with a predominant hypokinetic-rigid form of
PD at onset have a 30% chance of a more rapid progression.
746
Full-length expression of Park2 gene in human leukocytes
M. Kasap, G. Akpinar, E. Ergul, H.A. Idrisoglu, A. Sazci (Kocaeli,
Turkey)
Objective: Our aim was to demonstrate the presence of full-length
Park2 transcript in human leukocytes.
Background: Research on Parkinsons disease fails to pinpoint a
single gene or a gene product as the causative agent. However, it is
widely accepted that an early appearing form of the disease (early onset
Parkinsonism) is mainly caused by the mutations in Park2 gene, coding
for E3 ubiquitin ligase. Hitherto, studies related to the biochemistry or
other aspects of the Park2 gene product used cDNA generated from
substantia nigra of the mid-brain. This is mainly because the presence
of full length cDNA in human leukocytes is, so far, not demonstrated
although the presence of splice variants of Park2 gene were conrmed
(Sunada et al., 1998; Tan et al., 2005).
Methods: Total RNA was isolated from human leukocytes. After
conrming RNA integrity, using gene specic primers and two step
reverse transcriptase PCR, a 1.4-kb Park2 cDNA was synthesized and
cloned into an expression vector. The identity of the gene product was
then conrmed by DNA sequencing.
Results: A full length Park2 cDNA was synthesized from human
leukocytes using two gene specic primers. The 1.4-kb product was
cloned and sequenced to reveal its identity and the corresponding
sequence was deposited into NCBI databank under the accession
number of EF375726. Conditions used to amplify the full-length

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Park2 cDNA from a 35 year-old Parkinson patient suffering from
the disease since the age of 30 and an healthy-looking 33 year-old
individual failed to amplify a full-length product from six other
healthy-looking individuals and two late-onset parkinson patients,
although spliced gene products can easily be amplied from the
same RNA preps.
Conclusions: The results of this still progressing study raised the
question of whether the presence of full-length Park2 mRNA in
human leukocytes is of any importance to the development of the
disease.
747
Results of the PRAMI study: Description of the therapeutic management of patients with idiopathic Parkinsons disease (PD)
M. Dujardin, P.L. Lleu (Paris, France, Metropolitan)
Objective: To describe the therapeutic management of patient with
idiopathic Parkinsons disease by neurologists.
Background: Therapeutic management of Parkinsons disease in
France.
Methods: Pharmaco-epidemiological study conducted on 363 patients with PD included by a representative sample of 81 French
neurologists. Demographic and disease characteristics, neuropsychiatric symptoms, treatment procedures were collected. Logistic regression
was performed to identify the determinants of the therapeutic management of PD.
Results: Mean age was 69 years old, 61% were male. Hoehn and
Yahr stage was 2 for 63% and 2.5 for 37% of patients. According to neurologists 65% of the patients were considered on initial
stage of PD (no uctuation), 35% on advanced stage (with
complications). Mean UPDRS III score was 24 (SD14) (19 for
initial stage group, 32 for advanced stage group). A depression was
previously diagnosed for 30% of the patients. Most of the patients
(97%) had a medical treatment for their PD: 85% were treated by
levodopa, 57% by dopamine agonist (38% had levodopa alone, 11%
had dopamine agonist alone, 46% had combination of a dopamine
agonist with levodopa). Patients were treated with antidepressants in
24% of the cases.The results of the logistic regression show a
markedly increased probability of having levodopa alone for 70
years old patients (OR3). The probability to be treated with a
dopamine agonist was increased for 70 years old patients (OR4),
with initial stage PD (OR25). Patients were twice as likely to be
treated by levodopa associated with a dopamine agonist as PD
duration was 7 years (OR2) or as patients had motor complications (OR2).
Conclusions: This study provides a representative baseline on the
therapeutic management of PD in France. Following the actual recommendations of the French consensus conference (2000), the age, the
diseases duration and the motor complications remain the main factors
inuencing the therapeutic strategy.
748
Chronic Captopril treatment accelerates injury in an early stage
rat model of Parkinsons disease
E. Thornton, R. Vink (Adelaide, SA, Australia)
Objective: To characterize the effects of chronic Captopril treatment
on motor function in an early stage rat model of Parkinsons disease
(PD).
Background: Angiotensin-converting enzyme (ACE) inhibitors like
Captopril are commonly used for the treatment of hypertension and
chronic heart failure. Previous studies in PD have shown that chronic
treatment with ACE inhibitors increases both dopamine content and
release, resulting in a reduction of motor uctuations and dyskinesia in
moderately severe PD. Additionally, in experimental models of PD
where near total destruction (95%) of dopaminergic neurones occurs,
ACE inhibitors are neuroprotective by limiting oxidative stress. However, ACE is responsible for the breakdown of neuropeptides such as
substance P (SP), high levels of which have been previously shown to

S229

result in neuronal cell death. Thus, inhibiting SP degradation in early

stage PD may have detrimental effects on dopaminergic neurones.
Methods: Animals were treated with either Captopril (5mg/kg) or
equal volume vehicle for 7 days prior, and for 21 days after intrastriatal
injections of 6-hydroxydopamine (6-OHDA; 22L of 5g/L). Intrastriatal 6-OHDA is known to create a loss of dopaminergic function
similar to the early stages of human PD. Motor function of all animals
was then assessed using the rotarod test of motor decits on days 3, 7,
10, 14, 17 and 21 post-lesion. Histology for SP and tyrosine hydroxylase was performed on the days of maximum decit to conrm the
functional outcome results.
Results: 6-OHDA lesioned animals treated with Captopril demonstrated a signicantly greater motor decit on day 3 than vehicle treated
controls (p0.01). By day 7 Captopril treated animals were no longer
signicantly different to vehicle treated controls, although they never
displayed the level of motor performance as observed for the vehicle
treated controls throughout the entire 21-day assessment period.
Conclusions: Although previous data have shown a benecial effect
of Captopril in the latter stages of PD, our data suggests that chronic
treatment in the early stages of PD accelerates onset of functional
decits. Thus, the interpretation of Captoprils effects in PD may be
critically dependent on the levels of SP, and the role that it potentially
plays during the progression of the disease.

749
Prevalence of non-motor symptoms in Parkinsons disease patients
L. Vela, K.F. Lyons, J.A. Pareja, J.L. Dobato, F.J. Barriga,
C. Sanchez, M. Baron, A.P. Polo, L. Borrega (Alcorcon, Madrid,
Spain)
Objective: The aim of this study was to determine the type and
frequency of nonmotor symptoms (NMS) in a population of Parkinsons disease (PD) patients and the relationship between NMS and the
severity of PD.
Background: Although motor symptoms are the cardinal features of
PD, NMS are also very frequent. They can present before diagnosis and
almost inevitably emerge with disease progression. Usually NMS are
under-recognized, under-treated and a major cause of disability for PD
patients.
Methods: One hundred consecutive PD patients without cognitive
decline were studied. Patients were interviewed with a structured
questionnaire about the presence of NMS. Questions were grouped into
four domains: autonomic, psychiatric, cognitive and sensory symptoms. Patients had to answer yes, no or dont know to each item.
Basic demographic information, Hoehn and Yahr stage, levodopa complications and drug history were recorded. Descriptive statistics were
used to examine the sample and comparisons between groups were
made with either the Students t-test or one way analysis of variance
(ANOVA). Statistical signicance was set at p 0.05.
Results: The questionnaire was completed by 45 men and 55 women
(mean SD; age 71.62 10.5 years; mean disease duration 7.35
6.4 years). 45% had motor uctuations and 43% had dyskinesia. 86%
were on levodopa preparations while 62% were taking dopamine
agonists. The mean number of NMS was 8.0 5.8 per person. Eight
patients did not present any NMS. The most frequent NMS were dry
mouth (52%), sadness (54%), anxiety (44%) and the need to move
(41%). There was a signicant association of total NMS score with HY
stage (p 0.001). Disease duration correlated with the presence of
more motor symptoms (p0.05). NMS also uctuated in most of the
patients with motor uctuations.
Conclusions: NMS are very frequent in PD patients and correlate
with disease progression and severity. On average most PD patients
have at least 8 different NMS; however, they are often overlooked by
physicians. Therefore, it is important to identify and treat NMS as part
of routine care for PD patients.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S230 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

750
Identifying predictors of somnolence and edema in patients with
early Parkinsons disease treated with pramipexole: A secondary
analysis of the CALM-PD study
K.M. Biglan, A. Brocht, M.P. McDermott, K. Kieburtz, Parkinson
Study Group CALM-PD Investigators (Rochester, New York, USA)
Objective: To identify baseline characteristics associated with the
development of somnolence and edema in patients with early Parkinsons disease treated with pramipexole.
Background: The CALM-PD trial evaluated the development of
motor complications in 301 subjects with early Parkinsons disease
(PD) randomized to initial treatment with either pramipexole or levodopa. Edema and somnolence were reported more commonly in subjects treated with pramipexole.
Methods: All analyses were performed only in the 151 subjects
randomized to initial pramipexole treatment. The association of baseline demographic and disease specic factors associated with the rst
report of somnolence and edema in those subjects randomized to initial
pramipexole were evaluated using the Cox Proportional Hazard. Secondary analyses evaluated the development of moderate and severe
somnolence and edema in the same way.
Results: Forty-nine subjects experienced 62 events of somnolence
with 18 subjects experiencing 21 events rated as moderate or severe.
None of the baseline characteristics were associated with the development of any severity somnolence. Less severe disease as measured by
UPDRS (H.R. 0.96, 95% C.I. 0.93-1.00, p0.05) and greater comorbid
illness burden (H.R. 1.32, 95% C.I. 1.02-1.70, p0.04) were associated
with a higher risk of non-mild somnolence. Fifty subjects experienced
60 events of edema with 18 subjects experiencing 20 events rated as
moderate or severe. Again none of the baseline characteristics were
associated with the development of any severity edema. Later Hoehn
and Yahr stage (H.R. 1.12, 95% C.I. 0.98-1.28, p0.10) and greater
comorbid illness burden (H.R. 1.44, 95% C.I. 1.06-1.94, p0.02) were
associated with a greater risk of non-mild edema.
Conclusions: Somnolence and edema are common complications
following initial treatment with pramipexole. More severe somnolence
and edema may be related to Parkinsons disease severity and comorbid
illness burden.
751
Non-motor dysfunction contributes to swallowing dysfunction in
PD and could be a target for future therapy
N. Sengupta, H.N. Jones, J.C. Rosenbek, M.S. Okun, R.L. Rodriguez,
F.M. Skidmore, C. Swartz, H.H. Fernandez (Gainesville, Florida,
USA)
Objective: Because executive dysfunction in Parkinsons disease
(PD) may diminish dual-task performance, we hypothesize that nonmotor features, specically uctuations in alertness and/or excessive
daytime sleepiness, contribute to swallowing difculties in PD.
Background: Aspiration pneumonia is the most common cause of
mortality in PD, with dysphagia reported in over 70% of patients. Thus
far, treatments for dysphagia have been mostly mechanical. Nonmotor contributors have not been a primary target of treatment.
Methods: We recruited consecutive patients at the UF Movement
Disorders Center who scored a 2 (occasional choking) on swallowing
performance in Part II of the Unied Parkinsons Disease Rating Scale
(UPDRS), along with age-matched controls. Dysphagic patients underwent a clinical swallowing evaluation and a beroptic endoscopic
evaluation of swallowing. Both cases and controls completed the
Epsworth Sleepiness Survey (ESS), Clinical Assessment of Fluctuation
(CAF), and One Day Fluctuation Assessment (ODFA). The MannWhitney Rank Sum Test was used to compare performance between
dysphagic patients and controls.
Results: Eight PD patients with dysphagia, as determined by UPDRS
scores and swallowing evaluation, had a mean age of 69.4 years (range:
57-86); and PD duration of 7.8 years (SD 4.0). The ve non-dysphagic
PD controls had a mean age of 71.2 years (range 65-80); and PD
duration of 5.8 years (SD 5.7). The mean ESS score for dysphagic

Movement Disorders, Vol. 22, Suppl. 16, 2007

patients was 12.5 (range: 1-22), with 12 dened as abnormal;

whereas controls had a mean of 6.2 (range 3-10) (p0.03). The mean
CAF score for dysphagic patients was 6.25 (range 0-12), whereas the
mean for controls was 0.4 (range 0-2) (p0.01). Finally, the mean
ODFA score for dysphagic patients was 8.38 (range 5-15), whereas the
mean for controls was 2.8 (range 0-9)(p0.03).
Conclusions: Dysphagic patients exhibit higher levels of excessive
daytime sleepiness and uctuating alertness/confusion, as compared to
non-dysphagic patients. These non-motor dysfunctions may inuence
swallowing dysfunction in PD, and could be a target of future therapy.
752
The impact of the use of Access Therapy Controller on the postoperative outcome
N. Kovacs, I. Balas, L. Kellenyi, E. Pal, F. Nagy (Pecs, Hungary)
Objective: To determine the impact of Access Therapy Controller
use on the postoperative outcome in case of bilateral subthalamic deep
brain stimulation.
Background: In case of Kinetra implantable pulse generator, the
patient might be enabled to make minor adjustments of the stimulation
settings. Some centers regularly train their patients to recognize clinical
features, understand of the stimulation manipulation and the use of
controller. However, the potentially achievable therapeutic effect of the
Access Therapy Controller has not been determined yet.
Methods: In our study 30 consecutive idiopathic Parkinsons disease
patients, who previously underwent bilateral subthalamic deep brain stimulation, have been enrolled. All of them were trained for the use of Access
Therapy Controller, however, 14 of them did not dare use it during the one
year of follow up (non-user group). The preoperative and the postoperative
UPDRS and Schwab and England Activity of Daily Living (ADL) scores
had been compared between the controller user and non-user groups.
Because the acquired data did not follow the normal distribution, MannWhitney test was performed to measure signicance.
Results: Preoperatively all the demographic and clinical attributes of
both groups were similar. The immediate postoperative scores did not
differ either. However, one year later the Access Therapy Controller
user group had slightly higher reduction in UPDRS part II subscores
(37.8% vs. 32.79%, p0.05) and ADL scores (31.2% vs. 25.8%).
Nevertheless, the long-term outcome measured by the motor subscores
of the UPDRS and the occurrence of stimulation-related adverse reactions were similar in both groups.
Conclusions: Our small study demonstrates that in selective patient
group the use of Access Therapy Controller might produce additional
improvement in the quality of life to the effect of bilateral subthalamic
deep brain stimulation. Further multi-center studies are required to
prove this advantage and perform cost effectiveness.
753
Identifying predictors of response to pramipexole treatment in
early Parkinsons disease: A secondary analysis of the CALM-PD
study
K.M. Biglan, A. Brocht, M.P. McDermott, K. Kieburtz, Parkinson
Study Group CALM-PD Investigators (Rochester, New York, USA)
Objective: To determine baseline characteristics in patients with
early Parkinsons disease associated with a good response to initial
pramipexole treatment.
Background: The CALM-PD trial evaluated the development of
motor complications in 301 subjects with early Parkinsons disease
(PD) randomized to initial pramipexole or levodopa treatment and
titrated to one of three dosage levels over a period of 10 weeks
depending on need. Open-label levodopa could be prescribed at any
time, if needed, to treat emerging symptoms.
Methods: All analyses were performed only in the 151 subjects
randomized to initial pramipexole treatment. The primary analysis
evaluated the association of baseline demographic and disease-specic
factors associated with the ability to complete two years on
pramipexole without supplemental levodopa using logistic regression.
Secondary analyses evaluated baseline factors associated with a ten-

POSTER SESSION III, THURSDAY, JUNE 7, 2007

week improvement in the total UPDRS score 10 points (the median)
using logistic regression.
Results: Of the 151 subjects initially randomized to pramipexole,
132 completed two years of follow-up or required open-label levodopa
prior to two years and were included in the primary analysis. Fifty-eight
(44%) completed two years without requiring supplemental levodopa.
Worse total UPDRS score (OR 0.97, 95% CI 0.94-1.00, p0.03) and
worse Hoehn and Yahr (HY) stage (OR 0.47, 95% CI 0.23-0.98,
p0.05) at baseline were associated with a reduced odds of completing
two years on pramipexole monotherapy. Of the 142 subjects that
completed the 10-week pramipexole titration phase, 66 (46%) had an
improvement of 10 points on the total UPDRS at 10 weeks. Worse
total UPDRS score and worse HY stage at baseline were associated
with greater odds of having a 10-week improvement 10 points.
Conclusions: Less severe disease, and not initial dosage level of
pramipexole, is associated with a lower risk of requiring supplemental
levodopa within two years; however, more severe disease is associated
with a greater short-term improvement in UPDRS.
754
Prevalence and characteristics of punding among Parkinson patients in North-Central Florida
F.N. Nguyen, Y.-L. Chang, M.A. Shapiro, C.E. Jacobson,
C.L. Swartz, M.S. Okun, H.H. Fernandez (Gainesville, Florida, USA)
Objective: In this study we determine the prevalence and associated
features of punding in PD residents of North-Central Florida.
Background: Punding is a term used to characterize complex,
purposeless stereotyped motor behaviors. First described in cocaine and
amphetamine abusers, punding has only recently been reported in
Parkinsons disease (PD). To date, there are limited data on the prevalence of punding in PD.
Methods: A mail-in survey was conducted on patients diagnosed
with probable PD from the University of Florida Movements Disorders Center. Patient survey responses were rst screened to determine
those who met probable punder criteria. They were then interviewed
and classied as having no, mild, moderate or severe punding. Punding
was dened as a stereotyped behavior that was excessive, disruptive
and interruptive.
Results: 122 of 464 mailed surveys were completed. 29 out of 122
met our initial criteria for probable punding. Subsequent follow-up
interview revealed the prevalence of punding was 8.20 % in our cohort
(ve mild and ve moderate punders). Punding behavior included
excessive gardening, collecting and crafting. The punder and nonpunder group did not differ signicantly in age composition (72 vs. 67
years), education level (16 vs 15 years), illness duration (8 vs 8 years)
or calculated levodopa equivalent dosage (LED) (692 vs 663). The
punder group endorsed more sleep problems (e.g., missing sleep,
p.002). In addition, more people from the punder group were taking
dopamine agonists (90% versus 54%; p.027) but not other types of
medications compared to the control group.
Conclusions: The repetitive and purposeless behaviors with excessive and disruptive qualities found in these 10 punders were consistent
with previous reports of punding in PD. The punding prevalence is
lower than reported by Evans et. al. (8.20 % vs 14%). Moreover, in this
cohort punding behaviors may be associated with dopamine agonist
usage and sleep problems.
755
The scores of UPDRS correlate with objectively measured motor
performance characteristics in Parkinsons disease
K. Noorvee, D. Uueni, M. Paasuke, P. Taba (Tartu, Estonia)
Objective: The aim of the study was to measure the relationship
between the motor performance characteristics and Unied Parkinsons
Disease Rating Scale (UPDRS) total and motor scores in parkinsonian
patients.
Background: In Parkinsons disease (PD), the motor performance
characteristics are affected by clinical signs including hypokinesia,
rigidity and difculty in initiating of movements. UPDRS covers dif-

S231

ferent aspects of PD and enables to follow the longitudinal course of

PD, and has found a wide range of users. The Part III of UPDRS Motor
Examination provides a detailed assessment of PD motor impairment
based on physical examination of the patient.
Methods: Twelve patients with PD (ve women and seven men, with
mean SE age 67.41.2 years) with disease severity of Hoehn-Yahr
II-III stage were tested. The mean duration of the disease was 8.13.4
years. Postural sway was registered during 30 seconds standing with
eyes opened and closed on two force platforms. Isometric maximal
voluntary contraction force and rate of force development of knee
extensor muscles were measured using isometric dynamometer. Gait
characteristics (cadence, stride length, mean velocity) were tested using
optoelectronic motion analysis system.
Results: Spearman correlation analysis demonstrated positive correlations between UPDRS total score, age of subject, and postural sway
during standing with open eyes and eyes closed, and mean velocity of
gait. Strong statistically signicant negative correlations (p0.01) were
found between UPDRS total score, maximal voluntary contraction
force and rate of force development of knee extensors, and stride
length. UPDRS motor score demonstrated correlations with age of
subject and postural instability standing eyes closed, and was negatively correlated with maximal voluntary contraction force, rate of
force development of the knee extensor muscles, with mean velocity of
gait, and stride length during gait.
Conclusions: There is a strong concordance between UPDRS scores
and objectively measured motor performance characteristics.
756
Task-specic limb dystonia with slow onset: Initial symptom of
Parkinsons disease or association of two diseases?
I. Nestrasil, P. Kanovsky (Olomouc, Czech Republic)
Objective: Dystonia can be found in the early stage of Parkinsons
disease (PD) only very rarely. It is uncommon in untreated patients or
preceding parkinsonian symptoms. Sudden onset of dystonic spasms and
slowness of movement was formerly described for autosomal dominant
rapid-onset dystonia-parkinsonism (DYT 12). We describe 2 cases of
sporadic occurrence of task-specic limb dystonia (writers cramp) with
slow onset and with subsequent development of parkinsonian symptoms.
Methods: Two men suffered from task-specic dystonia and subsequently mild parkinsonian symptoms (Hoehn&Yahr 1.0) developed. A
46-year-old man (patient A) was referred for a graphospasm, which
occurred 3 years before. He presented with difculty during writing.
After 2 years of disease course, a positionally-sensitive tremor occcured and some months later he started to complain of right hand
stiffness. A 59-year-old man (patient B) complained of twisting of his
right hand during writing typical for writers cramp. It appeared 6 years
ago and did not progress. Later he noticed a hypokinesia and resting
tremor of his right hand.
Results: Neurological examination showed a dystonic pattern typical
for graphospasm in both subjects during writing with the right hand. In
patient A there was also a positionally-sensitive tremor, which is a
tremor occurring when holding a pen and continuing during writing. In
both subjects there were hypomimia (poker face), mild hypokinesia
and rigidity, and decreased arm swinging of the right upper limb. In
patient B there was also resting tremor of the right hand. UPDRS III
scores were 7 for patient A and 6 for patient B. L-dopa alleviated
parkinsonian symptoms. Polymyography showed a dystonic pattern
with muscle hyperactivity of wrist and nger exors.
Conclusions: Task-specic limb dystonia preceding a development of
PD is a very uncommon condition. It remains unknown, whether writers
cramp can precede the development of PD or whether these cases present
an association of two different disorders. Abnormal functioning of the
basal ganglia is believed to be the underlying pathology of the both
writers cramp and PD. The present work shows that a pathophysiology of
the both writers cramp and PD can be related.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S232 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

757
Retention rate and tolerability of rotigotine transdermal skin patch
for a real life population of Parkinsons disease patients in the
UK
P. Reddy, S. Muzerengi, A. Forbes, R. Weeks, K. Ray Chaudhuri
(London, United Kingdom)
Objective: To address the tolerability and retention rate of rotigotine
transdermal patch in a real life clinic population of Parkinsons disease
(PD) patients across all disease stages and severity.
Background: Rotigotine is the rst transdermal patch, which contains rotigotine, a non ergot selective D1/D2/D3 receptor active dopamine agonist for treatment of patients with early and advanced stages
of PD. There are few reports of efcacy and tolerability of rotigotine in
clinical practice since license was granted in 2006.
Methods: A systematic clinical and demographic analysis of clinic
database held at Kings and Lewisham hospitals with analysis of all PD
cases started on rotigotine skin patch.
Results: 36 PD patients (mean age 67.1 yrs, range (45-84), mean
duration of disease 9.1 yrs (range 1 26yrs), mean Hoehn and Yahr score
(HY) 2.5 (1-4)) were on rotigotine (mean dose 5.72 mg (2-8 mgs), mean
duration 5.7mnths (1-8) and had full records for analysis since 2006
August. Indications for rotigotine included motor sleep problems (44.4%),
dyskinesias (22.2%), switch from ergot or other agonists (36.1%) with
successful outcome at follow up with reduction of dyskinesias and improvement in night time symptoms particularly restless legs and periodic
limb movements. Only two have discontinued due to skin rash. Two
patients have elected to remain on rotigotine skin patch after being intolerant of apomorphine infusion because of skin nodules.
Conclusions: Although small in number this ongoing clinical audit
based study shows that rotigotine is well tolerated across all stages and
duration of PD and is particularly useful for dyskinesias and nocturnal
problems of PD where continuous dopaminergic stimulation is an
attractive option.
758
Comparison of proton-MR-spectroscopy of the substantia nigra in
patients with Parkinsons disease, relatives and controls
A. Di Santo, U. Klose, K. Schweitzer, J. Godau, D. Berg (Tuebingen,
Baden-Wurttembe, Germany)
Objective: To establish Proton-MR-spectroscopy at the SN and see
whether changes in the ultrasound signal correspond to patterns of
cerebral metabolite ratios validating ultrasound as a diagnostic marker
for Parkinsons disease (PD). As some healthy relatives of PD patients
show similar SN hyperechogenity compared to their disease affected
siblings we were especially interested whether metabolic changes may
also be detected in this healthy group.
Background: There has been evidence that in selected brain areas the
ratios of certain cerebral metabolites (e.g. NAA/Cr or NAA/Cho) differ
signicantly between patients with PD and healty control groups. Up to
now, the SN which we have shown to be hyperechogenic on transcranial ultrasound in PD patients due to iron deposit, has not yet been
subjected to detailed Proton-MR-spectroscopy-analysis.
Methods: Up to now, we analyzed the Proton-MR spectra of the SN
on a 1.5 T whole-body system (Avanto, Siemens Erlangen, Germany)
with a CSI-Sequence and short echo TE of 30 ms. 22 PD-patients (12
idiopathic, 10 monogenetic patients), 10 relatives with hyperechogenity
of SN and 20 controls (10 were relatives without hyperechogenity). To
evaluate the spectra of the main cerebral metabolites (N-acetylaspartate
(NAA), choline (Cho), creatine (Cr), glutamate (Glu), myo-Inositolphosphate(mI) we used the program CULICH.
Results: First analysis show validity and reproducibility of results.
Up to now, no signicant group differences could be detected for Cr,
GPC, mI and NAA and the respective ratios.
Conclusions: Groups will be enlarged and the analysis will be
completed. Up to know, we can conclude that it is possible to perform
Proton-MR-spectroscopy at the SN in a valid and reproducible way.
Lacking group differences may be explained by methodological limitations, differences in underlying pathomechanisms or effects of ther-

Movement Disorders, Vol. 22, Suppl. 16, 2007

apeutic interventions. To test the latter hypothesis Proton-MR spectroscopy of drug-nave patients is currently being performed.
759
Additional value of SPECT imaging in comparison with clinical
diagnosis in 248 patients with parkinsonism
A.M.M. Vlaar, M.J.P.G. Kroonenburgh Van, T. Nijs De, W.H. Mess,
S.C. Tromp, A. Winogrodzka, W.E.J. Weber (Maastricht, Netherlands)
Objective: Investigate the diagnostic accuracy of SPECT in patients
with unclear parkinsonism.
Background: Single Photon Emission Computer Tomography
(SPECT) visualizing the dopaminergic pathways by using post- or
presynaptic radiotracers is one of the most employed techniques in the
differential diagnostic of idiopathic Parkinsons disease (IPD). However, despite its widespread use, the exact diagnostic accuracy of this
technique in parkinsonian syndromes remains controversial.
Methods: Clinical records of 248 patients with parkinsonism who
underwent SPECT imaging in the period 2001-2006 were checked to
derive the clinical diagnosis after follow-up. The clinical diagnosis in
different parkinsonian syndromes were established according to general accepted clinical criteria. If the diagnosis could not be derived the
patient was invited for re-examination.
Results: In 221 patients the clinical diagnosis was available: PD 127,
essential tremor (ET) 22, vascular parkinsonism (VP)16, drug-induced
parkinsonism (DIP) 5, atypical parkinsonian syndromes (APS) like
Multiple System Atrophy and Progressive Supranuclear Paralysis 27
and the restgroup (disorders without dopaminergic involvement) 24.
The diagnostic power of SPECT was calculated for different clinical
problems. See table for the results.
Conclusions: 1) FP-CIT SPECT provides relatively accurate differentiation between IPD and ET, VP and DIP, and between IPD or APS
and patients with one of the other disorders. 2) IBZM SPECT has only
small additive value to differentiate IPD from VP and DIP. 3) The
accuracy of SPECT imaging with both radiotracers FP-CIT and IBZM
to differentiate between IPD and APS is relatively low.

IPD
IPD
IPD
IPD
IPD

vs ET
vs VP
vs DIP
vs APS
& APS vs other*

presynaptic (FP-CIT) SPECT

postsynaptic (IBZM) SPECT

82 (11-674)
61 (8-490)
33 (2-697)
1 (0-4)
44 (16-120)

1 (0-4)
8 (2-30)
2 (0-24)
7 (2-17)
2 (1-3)

* ET, VP, DIP and restgroup

760
Anti-psychotic treatment discontinuation and drug-induced psychosis in Parkinsons disease
F. Morgante, A. Epifanio, M. Zappia, R. Marconi, G. Paolo,
A. Quartarone, A. Quattrone, L. Morgante (Messina, Italy)
Objective: To test whether antipsychotic drugs can be withdrawn in
patients with Parkinsons disease (PD) with an history of drug-induced
psychosis successfully treated.
Background: Few data are available in literature when and if it is
possible to withdrawn antipsychotic drugs in PD patients who had a
remission of hallucinations.
Methods: We enrolled two groups of PD patients without dementia
on antipsychotic drugs (clozapine: n 10; quetiapine: n 11) who
were free of any on-going hallucination evaluated by the hallucination
item of the Brief Psychiatric Rating Scale (BPRS). The present study is
the second phase of a prospective open-label trial where we had
demonstrated the equivalence in terms of efcacy and tolerability of
quetiapine compared to clozapine for psychosis in PD. After monitoring motor and psychiatric symptoms for 6 months, we slowly and
gradually withdrew clozapine (6.25 mg every two weeks) and quetiapine (25 mg every two weeks) up to complete withdrawal within 1-3
months. Patients were assessed up to 3 months after complete discontinuation.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Results: After removal of the antipsychotic drugs, hallucinations
occurred in 10 out 21 patients (at 3 months follow up) without differences between the two groups (clozapine: n 5; quetiapine: N 5).
Predictive factor for the re-emergence of the hallucinations after discontinuation was the hallucination score of the BPRS at baseline before
starting the antipsychotic treatment (p 0.0001): rebound psychosis
occurred in PD patients with higher BPRS scores. Age, gender, disease
severity and duration, and levodopa equivalent daily doses do not
correlate with the reoccurrence of the hallucinations.
Conclusions: Our data demonstrate that rebound psychosis occurs
after discontinuation of clozapine and quetiapine in PD. However the
withdrawal do not determine re-emergence of psychosis in the subgroup of patients with milder hallucinations at baseline. Antipsychotic
treatment should not be suspended in PD, especially in those patients
with more severe psychosis.
761
PD patients with STN-DNS gain weight in relation to motor improvement: A prospective study
A. Foubert, E. Krim, C. Perlemoine, P. Burbaud, E. Cuny,
S. Maurice-Tison, V. Rigalleau, F. Tison (Pessac, France)
Objective: To determine the weight gain and its causes in a prospective study of STN-DBS operated Parkinsons (PD) patients.
Background: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has proved to be effective in the management of late
complications of levodopa treatment in PD. Several complications of
STN-DBS may occur: recently non-motor adverse events emerged, one
of which is weight gain (WG). We previously demonstrated that
STN-DBS operated patients had a signicant WG compared to controls
(mean 9.7, SD7 kg) during the rst year post-op, mainly due to fat
mass (FM) increase (Mov Disord. 2004;19:206-12). There was a signicant correlation between WG and pre-op levodopa-equivalent daily
dose (LEDD), and a reduction in resting energy expenditure (rEE)
without daily energy intake (DEI) adjustment. Still, there are however
no prospective pre-op/post-op data.
Methods: Nine PD patients were enrolled in a prospective study with
indirect calorimetry assessment, before (3 weeks) and after (3 months)
STN-DBS.
Results: We conrmed prospectively a signicant WG (mean 2.77
SD2.99 kg, p0.005) and increased BMI (mean 1.01 SD0.9
kg/m2, p 0.05) as early as 3 months post-op. There was a nonsignicant tendency to FM increase, DEI was not modied but rEE
tended to decrease. WG was correlated with an improvement of UPDRS III off motor score (r2 0.746; p 0.0219), Schwab and
England functional scale on (r2 0.84, p 0.02 and decrease in the
Goetz dyskinetic score (r2 0.66; p 0.049).
Conclusions: Although this prospective study enrolled a small number of patients, we conrmed that STN-DBS patients gain weight as
early as 3 months post-op in relation with their motor improvement
(parkinsonism and dyskinesia).
762
Towards adaptive deep brain stimulation: Recording local eld
potentials during stimulation
L. Rossi, G. Foffani, S. Marceglia, A. Priori (Milan, Italy)
Objective: The aim of this work is to propose a novel solution for
recording local eld potentials (LFPs) from the stimulating electrode
during deep brain stimulation (DBS).
Background: DBS is an effective treatment for advanced idiopathic
Parkinsons disease (PD). The main limitation of DBS is that PD motor
uctuations are not fully controlled by the constant-parameters stimulation, thereby affecting the patients quality of life. In PD, motor
uctuations are related to neuronal rhythms in the target nuclei, which
could be effectively measured with LFPs recorded through DBS electrodes. We developed the idea that measuring LFPs during DBS from
the same stimulating electrode could provide the information necessary
to adapt stimulation parameters to the patients clinical needs. However, a technical drawback that prevents LFPs from being recorded

S233

from the DBS target nuclei during stimulation is the stimulus artefact.
To solve this problem, we designed FilterDBS.
Methods: We designed an electronic amplication system for artifact-free LFP recordings (in the frequency range 2-40 Hz) during DBS.
After dening the estimated system requirements, we tested the FilterDBS system by conducting experiments in vitro and in vivo in patients
with advanced PD undergoing DBS of the subthalamic nucleus.
Results: The FilterDBS system completely suppressed the DBS
artefact without inducing signicant spectral distortion.
Conclusions: Our device pioneers the development of an adaptive
DBS system controlled by LFPs and can be used in novel closed-loop
brain-machine interface applications in patients with neurological disorders.
763
A comparison of cerebral glucose metabolism in Parkinsons disease, Parkinsons disease dementia, and dementia with Lewy bodies
S.W. Yong, P.H. Lee, Y.J. Kim (Suwon, Kyunggi-do, Korea)
Objective: To clarify the difference of metabolic patterns amongst
patients with Parkinsons disease, Parkinsons disease dementia (PDD),
and dementia with Lewy bodies (DLB) using 18F-FDG positron emission tomography.
Background: The current diagnostic consensus for differentiating
dementia with Lewy bodies (DLB) from Parkinsons disease dementia
(PDD) relies heavily on an arbitrary criterion, the so-called 1-year rule.
However, DLB and PDD share many clinical and neurobiological
similarities, including similar motor effects, neuropsychological proles, and neuropathological ndings. A direct comparitive study of
metabolic patterns in these two conditions using PET images is required.
Methods: We consecutively enrolled 12 patients with PDD, 7 patients with DLB, and 16 patients with PD without dementia. 18F-FDG
PET scan was performed on all patients. PET images were reconstructed by iterative reconstruction using the CT images, and were
normalized to a standard template provided by SPM2 on MATLAB
version 7.1. Statistical comparisons between groups were performed on
a voxel-by-voxel basis using t statistics (two-sample t-test). The uncorrected height threshold (P) less than 0.001 and extended threshold
(Ke) more than 100 was considered to be signicant.
Results: Compared to the patients with PD, the PDD patients showed
patterns of decreased metabolism in the right parietal lobe, inferior
frontal and medial frontal lobe (P 0.001). In the patients with DLB,
the metabolic pattern was similar to that in the PDD patients except that
the metabolic decits in the fronto-parietal area were greater and
decits were also observed in the right temporal cortex. In a direct
comparison between DLB and PDD, signicant metabolic decrease
(p 0.005) was noted in anterior cingulate in patients with DLB.
Conclusions: In comparison with the PD patients, the DLB and PDD
patients exhibited grossly similar metabolic patterns, i.e. showed
greater metabolic decits in the parietal and frontal regions, although it
was more pronounced in patients with DLB. Our study supports the
concept that PD, PDD, and DLB has similar underlying neurobiological characteristics, and they can be regarded as a spectrum of Lewy
body disorders.
764
Extraction of typical features from surface EMG signals in Parkinsons disease
S.M. Rissanen, M. Kankaanpaa, M.P. Tarvainen, J. Nuutinen,
I.M. Tarkka, A. Meigal, O. Airaksinen, P.A. Karjalainen (Kuopio,
Finland)
Objective: The aim of this study was to develop and test different
mathematical methods for extraction of typical features from surface
electromyographic (EMG) signals in Parkinsons disease (PD).
Background: Surface EMG measurements and analysis are mainly
used to examine level of muscle activation and fatigue. Conventional
methods of surface EMG analysis are amplitude and spectral analysis.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S234 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

However, in the case of Parkinsons disease, more novel methods are
needed to analyze the detailed structure of EMG signal. The main
changes in the EMG signal caused by PD are increased tonic background activity and an alternating pattern of EMG bursts. The increased
background activity can be seen as increased amplitude of the signal
but the characterization of the burst-like activity of EMG requires
methods that are sensitive in detecting 1) the distribution of the signal
values and 2) the recurrences of the signal values in time domain.
Methods: The methods of EMG analysis used in this study were
specically chosen and developed based on their potential suitability
for characterizing Parkinsonian EMG signals. The used methods involved novel structural (histogram and crossing rate distribution based)
methods and nonlinear (entropies, fractal analysis, recurrence quantication analysis) methods. All algorithms for the data analysis were
programmed by MatlabTM. For the extraction of typical features from
Parkinsonian EMGs, principal component analysis was used. The
methods were tested with EMG data measured from patients with PD
and healthy controls.
Results: Results of the analysis showed that the structural and
nonlinear methods were suitable methods for characterizing Parkinsonian EMG signals. By using structural methods the burst-like activity
of EMG could be seen as a change in the shape of the histogram and
crossing rate distribution. Reduced complexity and increased recurrence of Parkinsonian EMG, instead, were revealed by nonlinear methods of EMG analysis.
Conclusions: Structural and nonlinear methods of EMG analysis are
potential methods for extracting typical PD related bursting features
from EMG signals. These methods form a promising approach for the
surface EMG analysis in Parkinsons disease. Further studies regarding
their diagnostic value are suggested.
765
Low dose methylphenidate improves freezing in advanced Parkinsons disease during off-state
L. Pollak, E. Dobronevsky, T. Prokhorov, S. Bahunker, J.M. Rabey
(Zerin, Israel)
Objective: In the present study we examined the effect of MPD alone
[without levodopa (LD)] on gait characteristics of advanced Parkinsons disease (PD) patients OFF medication.
Background: Freezing gait (FG) is one of the common and incapacitating motor features of PD. It can appear spontaneously during
walking or in certain situations like at gait initiation, on turning, etc. FG
is not associated with LD plasma levels and occurs mainly during on
but also during off medication. It is a common cause of falls in PD
patients with advanced disease. Methylphenidate (MPD) has been
shown lately in several studies to improve motor functions in PD when
given in addition to levodopa. However, the mechanism of action is not
completely clear. The effect of MPD is explained by its capacity to
increase extracellular dopamine levels by blocking the dopamine transporter (DAT) system in dopaminergic terminals. In addition to the
effect of MPD on DAT some experimental reports have shown that
MPD also inhibits the norepinephrine transporter. As a consequence, in
advanced PD MPD may also increase norepinephrine levels in the
striatum and the brainstem locomotor centers. This could also contribute to its plausible therapeutic effect on gait disturbances. However,
these studies are controversial.
Methods: Eight subjects with advanced PD were examined to assess
the effect of low dose MPD on gait. The patients were tested during
off state before and two hours after the intake of 10mg MPD while
walking in the outpatients clinic, a track which we call an 8 trajectory. The total walking time, total freezing time, number of freezing
episodes and non-freezing walking time were assessed. The obtained
data were analyzed by the Wilcoxon Signed Rank test with a type I
error 0.05.
Results: Patients showed a statistically signicant improvement in all
gait parameters after MPD intake. Moreover, a good correlation in the
grade of improvement for each individual gait characteristic was found.
Conclusions: This study demonstrates that low doses of MPD may
improve gait and freezing, in patients with severe PD, without the need

Movement Disorders, Vol. 22, Suppl. 16, 2007

for exogenous LD. A double blind study is now required to conrm

results.
766
Cerebrovascular risk factors and procedural learning in idiopathic
Parkinsons disease
E. Pourcher, H. Cohen (Quebec, Quebec, Canada)
Objective: To determine the impact of cerebro vascular risk factors
in the proceduralization of a new skill in Parkinsons disease (IPD).
Background: Firmly ascertained by epidemiological prospective
studies in the process of Alzheimerisation, the weight of comorbid
vascular risk factors in the worsening of cognitive prognosis of Parkinsons disease has not been formally assessed.
Methods: Two groups of 12 IPD patients, one with (IPDV) and the
other without (IPD-V) vascular risk factors, matched for age, disease
duration and Hoehn & Yahr staging were compared on a non motor
procedural reading task involving semantically related and unrelated
inverted word pairs.
Results: IPDV patients were found to be signicantly worse than
IPD-V in all aspects of performance : acquisition of procedural skills,
semantic processing and task repetition.
Conclusions: Comorbid vascular risk factors in the absence of overt
cerebro vascular disease seem to worsen performances linked to cortico
striatal loops efciency. Longitudinal studies of IPD-V and IPDV
patients are warranted to establish if distinct proles and curves of
cognitive decline separate the two groups.
767
Starting therapy in Parkinsons disease with L-dopa or agonists
and the occurrence of late L-dopa motor problems in daily practice
M.W.I.M. Horstink, C.A. Haaxma, G.F. Borm, B.R. Bloem
(Nijmegen, Netherlands)
Objective: Is starting with monotherapy agonists (StMnAg) effective
in daily practice?
Background: StMnAg reduces the occurrence of late motor problems
(LMP) compared to L-dopa therapy. However, if patients cannot be
satisfactory treated with agonists, L-dopa is added at the risk of LMP.
Secondly, LMP can be treated by adequate adjustments of daily L-dopa
intake. Differences over time between these strategies in patients starting with monotherapy levodopa (StMnLd) or agonist (StMnAg) could
suggest advantage of one of these strategies over the other.
Methods: Follow-up of cohort of 148 PD with StMnLd (90) or
StMnAg (58). Patients were included only when they were free of LMP
at rst visit. All patients were treated by the same neurologist according
to generally accepted uniform strategies. Outcome measures: latency
time (Tlat) to occurrence of LMP (TlatLMP) or follow up time if
remaining free of LMP (TlatFree). L-dopa intake is analyzed between
8.00 AM until 12 PM.
Results: 126 PD (85%) got LMP, 79 with StMnLd and 47 with
StMnAg. TlatLMP in StMnLd: 7.3 3.5 yrs; in StMnAg 7.8 3.2 yrs
(T-test p .46). TlatLMP did neither depend on StMnLd or StMnAg
(regression analysis p .47) nor on age starting PD (p .99). In
Parkinsons disease (PD) with LMP the cumulative percentage of PDs
with frequencies of daily l-dopa intake up to 8 times is 98.3%; maximal
frequency: 15 (5 patients). Frequency of l-dopa intake in PD with LMP
depended on the duration of PD (regression analysis p 0.000) rather
than on StMnLd or StMnAg (p 0.60). 22 PDs (15%) remained free
of LMP, 11 with StMnLd and 11 with StMnAg. TlatFree: StMnLd:
6.0 2.4 yrs; StMnAg: 7.6 2.9 (T-test p .17). If we exclude PDs
who could have been treated to short to develop LMP, i.e., shorter than
mean TlatLMP in PD with LMP, in the remaining 11 PDs (7%)
TlatFree in 5 with StMnLd is 8.3 0.9 yrs; and in 6 with StMnAg
9.6 1.8 yrs. The difference (1.34 yrs) is not signicant (p .16) but
note that the group is very small.
Conclusions: In routine practice the vast majority of patients did not
benet from StMnAg. Mainly because it was not possible to maintain
agonist monotherapy over longer periods. StMnAg possibly postponed
LMP about 1.34 years in a small minority of patients.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

768
Correlation between postural changes and cognitive impairment in
Parkinsons disease
G. Marco, P. Lucia, P. Susy, F. Sandro, S. Maura (Ancona, Italy)
Objective: Our aim was to investigate the correlations between
postural changes and cognitive functions in 30 non-demented PD
patients.
Background: Parkinsons disease (PD) is complicated especially in
the advanced stages by postural changes (balance and gait disorders)
that can be monitored by a computerized postural examination. The
disease may also be associated to a cognitive impairment involving
especially the frontal lobe functions that can be observed even in the
early stage.
Methods: The patients underwent, besides a clinical examination, a
static posturography before and after levodopa administration in both
condition of open eyes (OE) and closed eyes (CE); they were also
evaluated by a neuropsychological battery exploring all cognitive domains, mood and quality of life.
Results: Most of the posturographic parameters, before levodopa
assumption, (Mean distance AP, RMS Dist-AP, Sway area, Power
AP. . .) were inversely correlated with the Frontal Assessment Battery
(FAB). This correlation is lost in after levodopa condition. We suggest
that levodopa can interfere with the motor aspect of the test, but does
not inuence the cognitive one. The Mean distance ML, that is the
medial-lateral oscillation, is inversely correlated with FAB in both
conditions. This result brings to the hypothesis that levodopa improves
more the anterior-posterior parameters than the medial-lateral ones.
The test exploring the quality of life (PDQ 39) showed a direct
correlation with the postural parameters only after levodopa administration, probably because of a better insight of the patient in the
condition on, and with Beck depression scale, as expected. Postural
reexes detected by the clinical scale UPDRS were directly correlated
with the years of the disease, the freezing, the falls and the time of
therapy with levodopa.
Conclusions: The alterations of some postural parameters and cognitive functions may appear early in the course of the disease. In
addition, our data conrm the high correlation between cognitive
functions, especially the executive performances, and the postural
control, probably because of an underlying common neural network.
769
Parkinsons disease, malignant melanoma and body mass index
E.C. Lai, S. Moore (Houston, Texas, USA)
Objective: To use an electronic medical record (EMR) to identify
and characterize a group of patients seen at a large Department of
Veterans Affairs Medical Center who were diagnosed with both Parkinsons disease/Parkinsonism (PD) and malignant melanoma (MM),
and to gain information about their weight and body mass index (BMI)
at the time of their illnesses.
Background: Several studies have reported an increased risk of MM
in PD patients. In separate studies, obesity has been linked to MM and
to PD. Diabetes and MM have been found to be increased in certain
occupational cohorts. Smoking is generally regarded as inversely related to PD and a recent study has found smoking to be inversely
related to MM in one cohort. Healthy weight is classied as BMI
(18.5-24.9), overweight BMI (25.0-29.9), and obesity BMI 30. The
Department of Veterans Affairs has an national electronic medical
record system. The current study was done to pilot data acquisition at
the local level.
Methods: A patient database was queried to nd patients with
ICD-9CM codes for both MM and PD who were seen at the Michael E.
DeBakey VA Medical Center and its satellite clinics during FY19982006. A chart review was then done rst to verify the diagnosis and
then to collect information including weight changes, presence of
diabetes, and smoking history. Medication, serial BMIs, and tumor
staging information are part of the EMR.
Results: Fifteen patients were found with both conditions, 12 diagnosed with MM at the VA and 3 by an outside facility. All 15 patients

S235

were white and all but one male. Of the 12 with VA pathology reports,
5 had malignant melanoma in situ, 2 supercial spreading type, and 5
malignant melanoma not otherwise specied. Eight had a history of
non-melanoma skin cancer and 4 had no previous history of skin
cancer. Of the 12 MM diagnosed at the VA, 10 had PD rst and 2 MM
rst. Using the closest available BMI at diagnosis the average BMI was
29.1 (range 23-34) for PD and 29.8 (range 20-42) for MM. When each
patients highest BMI was used, only one was never overweight; the
average highest BMI was 33.8 (range 24-51).
Conclusions: VA patients seen in our facility who have both conditions, PD and MM tend to be overweight or obese. Further work is
planned to continue to describe this patient group and to expand the
study to include patients from other regions.
770
Cognitive effects of sanamide in early Parkinsons disease (PD)
patients
T. Sharma, R. Anand, F. Stocchi, R. Borgohain, S. Rossetti, 015
Study Group (Newark, Delaware, USA)
Objective: This study evaluated the cognitive effects of 100 and 200
mg/day doses of sanamide, a new anti-PD agent that combines selective, reversible MAO-B and glutamate release inhibition, compared to
placebo as an add-on therapy in non-uctuating, early idiopathic PD
patients receiving a stable dose of a single DA-agonist.
Background: PD affects several cognitive domains, even in patients
with early disease. The most severe areas of impairment are reaction
time, working memory and executive function.
Methods: A subset of 151 PD patients performed the Cogtest Battery
as a part of a phase III 24-week randomised placebo controlled trial.
The test included Auditory Number Sequencing (ANS), Spatial Working Memory (SWM), Strategic Target Detection (STDT), Tapping
Speed, Simple Reaction Time and Choice Reaction Time.
Results: Data were converted to z-scores based on healthy control
data from the Cogtest database. Changes from baseline to endpoint
were assessed with repeated measures analysis of variance. Cogtest
found impairments across several cognitive domains and in executive
function in these patients. At baseline, no patients were cognitively
intact, while 50% were impaired in 1 of the domains. Using LOCF
method, statistically signicant effects of sanamide were found (vs
placebo) for executive function as measured by the STDT (p0.037)
and working memory indexed by ANS (p0.035). A trend level
difference was found in SWM (p0.079). Also, cognitive effects were
seen as early as 12 weeks after starting sanamide.
Conclusions: Signicant decits in multiple cognitive measures,
most notably in executive function, were found in patients with early
idiopathic PD on DA-agonists. Improvements in executive function and
working memory were observed with sanamide, with a trend for
improvements in spatial working memory. These data suggest cognitive decits are prevalent even in treated PD patients. Cognitive impairments are a clinically relevant, yet understudied aspect of PD,
which improved with addition of sanamide to a DA-agonist, suggesting sanamide possesses actions beyond DA enhancement. New trials
will investigate sanamide cognitive effects.
771
The effectiveness of pramipexole and levodopa as an initial treatment for Parkinsons disease
ztekin, M.F. O
ztekin, R.S. Polat, B. Renkliyildiz (Ankara,
N.S. O
Turkey)
Objective: The aim of the study is to compare the development of
dopaminergic motor complications after initial treatment of early PD
with pramipexole vs levodopa.
Background: Pramipexole and levodopa both ameliorate the motor
symptoms of early Parkinson disease (PD), and several controlled studies
which have compared long-term outcomes after initiating dopaminergic
therapy with pramipexole vs levodopa resulted in favour of levodopa.
Methods: Thirty patients with early PD who required dopaminergic
therapy to treat emerging disability, enrolled to the study and were

Movement Disorders, Vol. 22, Suppl. 16, 2007

S236 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

randomly assigned to receive pramipexole, 0.5 mg 3 times per
day(n15); or carbidopa/levodopa, 25/100 mg 3 times per day (n
15). For patients with residual disability, the dosage was escalated
during the rst 10 weeks. From week 11 to month 24, investigators
were permitted to add open-label levodopa to treat continuing or
emerging disability. MAIN OUTCOME MEASURES: Time to the rst
occurrence of any of 3 dopaminergic complications: wearing off,
dyskinesias, or on-off motor uctuations; changes in scores on the
Unied Parkinsons Disease Rating Scale (UPDRS), assessed at baseline and follow-up evaluations.
Results: Initial pramipexole treatment resulted in signicantly less
development of wearing off, dyskinesias, or on-off motor uctuations
(28%) compared with levodopa (51%) (P.001). The mean improvement in total UPDRS score from baseline to 24 months was greater in
the levodopa group than in the pramipexole group (9.4 vs 4.7 points;
P.001). Somnolence was more common in pramipexole-treated patients than in levodopa-treated patients (33% vs 18%; P .003), and the
difference was seen during the escalation phase of treatment.
Conclusions: Fewer patients receiving initial treatment for PD with
pramipexole developed dopaminergic motor complications than with
levodopa therapy. Despite supplementation with open-label levodopa
in both groups, the levodopa-treated group had a greater improvement
in total UPDRS compared with the pramipexole.
772
The data base of Quality Development in Neurology and Psychiatry (QUANUP)-group results of the Parkinsons disease pilot
project
M. Muengersdorf, P. Scherer, A. Simonow, P. Reuther, R. Ehret
(Berlin, Germany)
Objective: This pilot project tested the feasibility of the data base.
The data analyses describe the population characteristics and the relationship between quality of life (QOL) and clinical disease related and
sociodemographic predictors.
Background: The Quality development in Neurology and Psychiatry
(QUANUP) developed a Parkinsons disease (PD) data base. The goal
was to establish standardized procedures for disease management and
patient-care by means of a patient-centered approach.
Methods: In 2005, 16 centres entered clinical and paraclinical data
for 322 outpatients with Parkinsons disease (PD) or PD-like syndromes into the computerized QUANUP data base. All of the centres
were neurological practices in Germany.
Results: In this population, PD (idiopathic) was the most frequent
diagnosis (94.0%). The mean age was 70.4 years (SD 8.5), the disease
duration was 7.6 years (SD 5.6). On the Hoehn&Yahr scale 9.7%
scored 1 to 1.5, 35.3% scored 2 to 2.5, 43.7% scored 3 to 3.5, 11.0%
scored 4, and 0.3% scored 5. On the Unied Parkinsons Disease
Rating Scale (UPDRS) the patients had a mean score of 37.6 (SD 16.7).
On the Mini Mental State Examination (MMSE) there were 11.5%
suspicious of dementia with a score of 24 or less. The ve symptoms
that have been mentioned most often were bradykinesia (83.5%), rigor
(78.8%), resting tremor (65.5%), dysarthrophonia (61.1%), and posture
tremor (60.1%). Using regression models, worse QOL as measured by
COOP scales was associated with higher age, longer disease duration,
other subtypes of PD than the tremordominant PD. Risk items as
depression, dementia, dyskinesia, motor uctuations, gait disturbances,
psychosis, tremor, bladder dysfunction, and sexual dysfunction were
associated with a decrease of QOL.
Conclusions: The data base was accepted by the neurologists who
participated in the pilot project. The population consisted of patients with
typical sociodemographic and disease related characteristics, most of them
being diagnosed as (idiopathic) PD patients. Aspects of QOL were associated with risk items [Spieker S. et al. 2003] and with age, disease
duration, and with the non-tremordominant subtype of the disease. These
results are consistent with data from the literature.

Movement Disorders, Vol. 22, Suppl. 16, 2007

773
Levetiracetam administration for the management of levodopa-induced dyskinesias in Parkinsons disease: An ongoing, multicenter,
double-blind, placebo-controlled, parallel, crossover trial (the VALID-PD study) study design and baseline patient characteristics
P. Stathis, S. Konitsiotis, G. Tagaris, V. Kyriakakis,
G. Hadjigeorgiou, The VALID-PD Study Group (Athens, Greece)
Objective: To evaluate efcacy, tolerance, and safety of Levetiracetam (LEV) vs placebo on levodopa-indused dyskinesias (LID), in
patients with Parkinsons disease (PD).
Background: LID are associated with irregular oscillatory discharge
characteristics of basal ganglia. LEV inhibits hyper-synchronization of
abnormal neuronal ring in experimental models of epilepsy. LEV also
reduces LID in MPTP-lessioned macaques and modulates priming
phenomenon which associated with long-term changes in synaptic
function that can lead to dyskinesias in PD.
Methods: PD patients, H&Y IIb, persistent LID, severity 2 on
item 32 and duration 2 on item 33 of the UPDRS. Two periods (each
consists of 500mg and 1000mg daily dose regimen periods) of drug or
placebo administration, everyone of 4 weeks duration separated (crossover) by 2 weeks washout. Patients assessed from their self-evaluation
diaries for the last 2 days before each visit. Goetz dyskinesia scale in
the morning after the rst levodopa (challenge) dose, the UPDRS,
Scwhab & England scale, and safety measures were assessed as well.
Primary efcacy variable: % change of the on with LID time from
patient diaries. A sample size of 50 patients (25 active drug vs 25
placebo, 80% power, 5% level of signicance) will be sufcient to
detect signicant reduction of 1.6 hour/day of the on with LID time.
Results: Up to today (n23, 16 males 69.6%). Age 65.09.7.
Disease duration 13.36.2 years. Levodopa dose 767.9370.1 mg/d.
H&Y for the on period was 2.60.7 and for off 3.10.4. Patients
assessment for both on and off periods in S& E was 62.419.4%
and for the investigators 61.116.4 %. The UPDRS (meanSD,
during on and off) total (items 1-31): 3215.9 and 66.015.8, part
IV: 10.52.3. The mean timeSD (hrs) of each period from the patient
diaries 7.52.5 hours for on with dyskinesias, 4.11.4 for on
without dyskinesias and 4.22.2 for off and total score in Goetz
scale (challenge) was 14.26.1.
Conclusions: Clinical and demographic characteristics of the PD patients enrolled in the study look to be in agreement with other similar
reports.
774
Factors that inuenced in quality of life in parkinsonian patient
according SF-36 (short form 36)
A. Machin, M. Hamdan, Y. Saelan (Surabaya, East Java, Indonesia)
Objective: To determine which factors among disease severity,
length of illness, Parkinsons medicine, depression and dementia most
inuence quality of life Parkinsons patient.
Background: The concept of quality of life is a sense of wellbeing, which is inuenced by many factors.Therefore its important to
know what factors contribute, to increase of quality of life.
Methods: Fifty patients who attended outpatient clinic neurologic
department of geriatric department of January to March 2001 fulll
inclusion criteria were interviewed and scored for disease severity
(using Hoehn -Yahr), depression (using Hamilton depression ratting
scale), dementia (using mental impairment score). Quality of life was
accessed using SF-36 questionnaire.
Results: The results multivariate regression analysis are, disease
severity inuence PF, SF, EV, GHP and CH of SF-36, signicantly.
Whereas depression inuence MH, EV and GHP signicantly. Length
of illness inuence CH but not signicantly (p0.066). dementia and
Parkinsons medicine dont make any contribution in SF-36 score.
Conclusions: Two of ve independent variables that inuence quality of
life signicantly are disease severity and depression. Neither Parkinsons
medicine nor dementia inuence QoL. Its because kinds of medicine used
are too variable. Dementia isnt widely distributed in our sample. Length
of illness inuence CH but not signicantly (p0.066).

POSTER SESSION III, THURSDAY, JUNE 7, 2007

S237

775

777

Freezing of gait severity and executive dysfunction in patients with

Parkinsons disease
M. Amboni, A. Cozzolino, K. Longo, M. Picillo, P. Barone (Naples, Italy)

Dementia and depression in Parkinsons disease

A. Akyol, A. Ozkul, E. Turgut, E. Yilmaz, U. Akyildiz, S. Memis
(Aydin, Turkey)

Objective: The purpose of this study is to correlate frontal functions,

by means of a battery of neuropsychological tests, with FOG severity,
quantied by FOG-Questionnaire, in parkinsonian patients in early
stage of disease with and without freezing at on state.
Background: Freezing of gait (FOG) is a common and debilitating
disturbance in patients with Parkinsons disease (PD). FOG refers to
paroxysmal events, usually lasting seconds, in which a subject is unable
either to initiate locomotion (start hesitation) and to turn (turning
hesitation) or to walk through a doorway. FOG response to dopaminergic drug is poor. A frontal lobe dysfunction has been proposed to
explain the occurrence of FOG.
Methods: 13 PD patients in early stage of disease (H&Y2.5) with
freezing during on (FOG ) and 15 age-,H&Y score- and disease
duration-matched PD patients without freezing (FOG-) were investigated; all patients were neither demented nor depressed. The assessment included UPDRS I-IV, FOG-Questionnaire, Stroop test, Frontal
Assessment Battery (FAB), phonological verbal uency, Ten-point
clock test (TPCT).
Results: There were no signicant differences in UPDRS I, III, IV
and MMSE scores between the two groups. Correlation analysis, performed by means of Pearsons correlation coefcient, showed signicant negative correlation between FOG Questionnaire score and
TPCT score (p0,05), phonological verbal uency (p0,05), Stroop
part II score (p0,01), FAB total score (p0,01).
Conclusions: FOG severity seems to correlate with lower scores at
frontal tests in patients with PD in early stage of disease (H&Y2.5).

Objective: Depression and dementia has been recognized as a common problem in Parkinsons disease (PD).
Background: The aim of our study was to assess the frequency, risk
factors, and interrelations between depression and dementia in PD.
Methods: We evaluated 240 patients who fullled the diagnostic
criteria of PD, had normal CT scans and responded well to L-dopa
treatment retrospectively. Besides neurological examination in all patients, severity of disease was evaluated using the unied scale for
evaluation of parkinsonism (UPDRS), Hoehn Yahr (HY) and Webster
scale (WS). Moreover, a sociodemographic questionnaire, mini mental
state examination (MMSE), and Hamilton depression test (HD) were
used.
Results: Our patients (105 female, 13 male) were between 30-88
years of age (mean 68.56 10.3). Depression was revealed in 43.6%,
and dementia was in 33.3%. Patients with cognitive impairment were
older and have higher WS and UPDRS scores (p0.05). In demented
patients depression was more frequent. Depressive patients had higher
HY and WS scores (p0.05). Disease duration had positive correlations with WS (r0.223, p0.006), UPDRS (r0.278, p0.002),and
HY (r0.198, p0.035). WS correlated with HY (r0.633, p0.000)
and UPDRS (r0.854, p0.000) positively. The other correlations
were between HY and UPDRS (r0.692, p0.000), HY and HD
(r0.339, p0.046);and HD and UPDRS (r0.331, p0.049). Neither
depression nor dementia had association with hemisphere dominancy.
Conclusions: Depression and cognitive impairment was correlated
with severity of motor disability. Therefore regular mood and cognitive
state screening and appropriate treatment should be considered in
especially older PD patients.

776
Comparative scintigraphic analysis of the parotid glands in healthy
volunteers and in patients with sialorrhea and Parkinsons disease
(PD)
D.H. Nicaretta, A.L.Z. Rosso, C. Maliska, J.P. de Mattos,
S.A.P. Novis, M.M.B. Costa (Rio de Janeiro, RJ, Brazil)
Objective: The objective is to study the parotid glands function in PD
patients with sialorrhea comparing with controls subjects without PD.
Background: In spite of the association between sialorrhea and PD,
since its original description, little is known about its pathophysiology.
Methods: We analyzed the parotid gland activity of 14 PD patients,
who presented with sialorrhea, and compared it with a control group of
eight healthy volunteers. The study consisted in the analysis of uptake,
distribution and elimination of marked saliva with sodium pertechnetate (Tc-99m). This dynamic study of the parotid glands was accomplished by a gamma camera of wide eld Diacam-Siemens with matrix
128 128, and 40 sequential frames, with 60 seconds each.
Results: From the 134 PD patients evaluated by UPDRS, 14 patients
presented with sialorrhea (10,45%). Of these 11 were male and three
female, with ages ranging between 42 and 76 years (means SD
61.64 9,06 y), and a history of PD symptoms ranging beween one to
16 years. The control group was composed by ve females and three
males, with ages ranging from 39 to 77 years (means SD 61.62
14,36 y). Two patients were in the HY- I stage, six in the HY- II and
six in the HY- III. According to the UPDRS sialorrhea quantication,
nine patients were classied at grade 1, four at grade 2 and one at grade
4. PD patients sialorrhea could not be related with age, time or severity
of the disease. We noticed no difference between the results of the
uptake of pertechnetate by the parotid gland in the two groups, but the
excretion speed of the parotid gland in PD patients was greater than that
observed in healthy individuals that has statistical signicance p0,05.
Conclusions: We concluded that sialorrhea in PD is not of the
productive type. The increase of excretion speed is an inuential factor
in the retention sialorrhea and this might explain its higher prevalence in PD.

778
Clinical characteristics and prevalence of Parkinsons disease in
orhangazi district of Bursa, Turkey (a population-based door to
door study) (Bursa, Turkey)
M. Zarifoglu, S. Erer, N. Karli, M. Boz, A. Bican (Bursa, Turkey)
Objective: To investigate the clinical ndings and prevalence of
Parkinsons disease (PD) among 40 years and older population in
orhangazi district of Bursa, Turkey.
Background: There are few studies of the prevalence rate of PD and
age differences have been indicated in the reported prevalence data.
Methods: This population based study was planned in 3 phases. In phase
1; door to door home interviews were performed by residents using a short
questionnaire to screen for parkinsonism (questions about tremor, bradymimia, bradykinesia). In phase 2; suspected subjects in phase 1 were
examined and videotaped. Cases were evaluated according to the scales for
PD by movement disorders experts and a diagnosis has been made. In
phase 3; video records of all diagnosed cases were reviewed and nal
diagnosis were made by agreement of all three experts.
Results: This study has been performed between May 2004 and
August 2006 in orhangazi district of Bursa, Turkey. 1256 of the target
population has been screened according to the study questionnaire.
Cases with suspectful symptoms of Parkinsonism have been detected in
phase 1. In these subjects, tremor in 247, bradykinesia in 32, bradymimia in 17, nally rigidity in 28. A certain diagnosis of PD has been
made in 13 cases in phase 2 and 3.
Conclusions: The prevalence of PD is 1,393 % among 40 age and
older population. In our study the prevalence rates of PD substantially
high when comparing with the other studies.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S238 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

779
Midbrain iron measured with MRI in early Parkinsons disease
W. Martin, M. Wieler, M. Gee (Edmonton, Alberta, Canada)
Objective: To evaluate midbrain iron content with MRI in patients
with early Parkinsons disease (PD) not yet receiving symptomatic
medical treatment.
Background: Basal ganglia structures, including the substantia nigra
(SN), are prominent on MRI, in part because of their high iron content.
Conventional MRI techniques, however, have not shown denitive abnormalities in the midbrain in patients with PD. Biochemical studies have
reported increased iron content in the SN in PD, with changes most marked
in severe disease. This suggests that measurement of regional iron content
in the SN may provide an indication of the pathological severity of the
disease. Since the major neuropathological changes in PD occur in the
lateral SN compacta (SNc), we hypothesized that this region would have
the most prominent change in iron content and that this abnormality could
be quantied by novel MRI sequences.
Methods: Twenty-two subjects (ages 61.9 9.0 yr) with early,
untreated PD were evaluated as part of a larger prospective, longitudinal study. They underwent neurological examination, functional,
cognitive and MRI assessment. MRI was done at 3.0 tesla, with
measures of effective transverse relaxation rate (R2*) made in the
medial and lateral SNc, the medial and lateral SN reticulata (SNr). MRI
data were compared to data from 11 healthy control subjects (ages
55.9 7.3 yr).
Results: In control subjects, we observed a signicant correlation
(r2 0.86, p 0.007) between R2* and previously published regional
iron concentrations. The lateral SNc opposite the most severely affected limbs showed the greatest increase in measured R2* in PD
patients compared to controls (PD: 27.4 3.5 s-1; control: 21.6 3.6
s-1; p0.0002). No signicant changes in R2* were evident in the SNr;
there were no signicant correlations between R2* and either UPDRS
motor score or years since disease onset in any of the ROIs considered.
Conclusions: Our data provide evidence that lateral SNc iron content
is elevated in early PD and that this change can be imaged quantitatively with appropriate MRI sequences. A longitudinal study is currently underway to determine whether this measurement changes with
disease progression.
780
COMT val158met genotype inuences attentional control in Parkinsons disease
C.H. Williams-Gray, A. Hampshire, A.M. Owen, R.A. Barker
(Cambridge, United Kingdom)
Objective: To explore the relationship between the COMT val158met
polymorphism and attentional control in Parkinsons disease (PD), and
its neural basis.
Background: A key cognitive decit in PD relates to inability to shift
attention from one stimulus to another. This decit, however, is not
universal and its neurochemical and anatomical basis is uncertain. Our
previous work suggests that a common functional polymorphism
(val158met) within the gene encoding the dopamine regulating enzyme,
COMT, may mediate some of the cognitive heterogeneity observed in
PD (Foltynie T, Goldberg TE, Lewis SG, et al. Planning ability in
Parkinsons disease is inuenced by the COMT val158met polymorphism. Mov Disord 2004;19(8):885-891).
Methods: We scanned 29 PD patients homozygous for either val
(n16) or met (n13) at the COMT val158met polymorphism using fMRI
during performance of an attentional shifting task. Subjects were presented
with compound stimuli comprising superimposed faces and buildings and
were instructed to work out the correct target through a process of trial and
error (Hampshire A, Owen AM. Fractionating attentional control using
event-related fMRI. Cerebral Cortex 2006;16(12):1679-1689). ROIs were
dened at co-ordinates of peak activation during task performance in a
whole group analysis, and subsequently used in a cross-group comparison
between val and met homozygotes.
Results: Val homozygotes adopted a typical strategy for solving the
task similar to that used by controls (Hampshire et al, 2006), favouring

Movement Disorders, Vol. 22, Suppl. 16, 2007

shifts within rather than between stimulus dimensions. In contrast, met

homozygotes adopted an abnormal strategy, suggesting impaired ability to form an attentional set. The principal brain areas activated
during the task were the dorsolateral PFC, ventrolateral PFC and
posterior parietal cortex. Cross-group comparisons revealed a signicant underactivation across these ROIs in met versus val homozygotes
and an interaction between genotype and levodopa dose.
Conclusions: COMT genotype inuences attentional control in PD
through a direct impact on frontoparietal activation, an effect presumably mediated through altered regulation of cortical dopamine levels.
This work adds to our understanding of the complex effects of the
COMT polymorphism on cognitive ability, and may have implications
for optimising dopaminergic therapy as a function of genotype in PD.
781
Long-term efcacy and safety of zonisamide in advanced Parkinsons disease
M. Murata, K. Hasegawa, I. Kanazawa (Tokyo, Japan)
Objective: To evaluate the long-term efcacy and safety of zonisamide in advanced Parkinsons disease (PD).
Background: Previously we reported zonisamide, anti-epileptic
agent, improves motor function in advanced patients with PD. A
12-week double-blind study revealed that zonisamide is safe and effective on PD at 25-100 mg/day as an adjunctive treatment.
Methods: We conducted an open administration of zonisamide (25100 mg, once a day) to 92 patients with PD as adjunctive therapy.
Changes of motor symptoms were evaluated up to 56 weeks according
to the UPDRS and Hohen-Yahr staging. Laboratory tests were carried
at before and 4, 16, 28, 40, 52-56 weeks. All adverse experiences,
reported spontaneously on general questioning or observed directly by
investigator, were recorded.
Results: The mean age was 64.310.3 years, mean disease duration
was 8.64.5 years, and mean Yahr staging was 2.4/3.3 (on/off).
L-dopa was given for all the patients, dopamine agonists were used by
91.1%, and selegiline was used by 46.7% of the patients. Of the 92
patients entered the study, 62 patients were completed 52 weeks study.
Mean zonisamide dose was 48.4 mg/day. UPDRS II (on), II (off) and
III were signicantly improved at 4 weeks (p0.001) and were more
improved at 52 weeks. UPDRS IV was signicantly improved at 28
weeks and more at 52 weeks. Main adverse effects were somnolence
(10.9%), apathy (10.9%), appetite loss (9.8%), depressive state (8.7%),
dyskinesia (7.6%) and hallucination (7.6%). The rate of adverse events
did not increase with time.
Conclusions: Zonisamide is safe, effective and well tolerated at 25100mg for 1 year as adjunctive treatment in advanced patients with PD.
782
An overview of specialist multidisciplinary services for Parkinsons
disease patients at Llandough Day Hospital
M.M. Oliver, P.C. Sewter, E. Morgan, J. Pinkerton, B. Clarke
(Penarth, Vale of Glamorgan, United Kingdom)
Objective: To describe the benets of multidisciplinary team support
in managing Parkinsons disease patients in an intermediate care setting.
Background: Parkinsons disease patients are best cared for by a
collaborative multi professional approach. The team illustrated consists
of: Doctor, Nurse, Occupational Therapist, Parkinsons disease Nurse
Specialist, Physiotherapist, Podiatrist, and Speech and Language Therapist. This team is based in a Day Hospital setting as part of an Acute
NHS Trust. For the last 10 years we have been running a specialist
monthly Parkinsons Day in conjunction with day to day proactive
management of Parkinsons disease patients.
Results: This qualitative review described key roles within the team
and illustrates the importance of mutual respect and understanding of
roles within these disciplines, without replication of function. Falls are
not a presenting feature of Idiopathic Parkinsons disease, however, are
recurrent in up to 40% of patients, and up to 90% of patients will

POSTER SESSION III, THURSDAY, JUNE 7, 2007

eventually become fallers, this justies the specic falls prevention
programme within this setting.
Conclusions: NICE guidelines support the implications of managing
patients with Parkinsons disease with a strong multidisciplinary approach. We are already working/implementing this approach and look
forward to providing increased quality care which will improve patients quality of life in their disease management.
783
Neuropsychological effects of bilateral STN DBS in advanced Parkinsons disease
R. Borgohain, R. Reddy, M.K. Panigrahi, S. Shanmukhi,
T. Suryaprabha, A. Jabeen (Hyderabad, Andhra Pradesh, India)
Objective: To assess neuro-psychological effects of bilateral subthalamic nucleii (STN) high frequency deep brain stimulation (DBS) in
advanced Parkinsons disease (PD).
Background: Deep brain stimulation of the STN has been an important
step forward in treatment of patients with advanced PD and medically
intractable levodopa induced motor uctuations and dyskinesia. Very little
has been studied as regards neuropsychological effects of this therapy.
Methods: All patients underwent neuropsychological and UPDRS III
examination before and after bilateral STN DBS. The neuropsychological battery included mini-mental status examination (MMSE), frontal
lobe executive tests (trial making test & Stroop tests), verbal uency
tests, verbal memory [Rey auditory verbal learning tests (RAVLT)] and
visual memory [Benton visual retention (BVRT) & visual N back
tests] and mood and behavioral (UPDRS I) tests.
Results: Thirty-three patients who underwent bilateral DBS of STN
at our center since 2001 were grouped in less than 1 year (n 10) and
more than 1 year (n 23) post DBS groups. The overall mean age and
disease duration were 56.9(10.9) years and 11.1(4.9) years respectively. There was signicant mean reduction in UPDRS motor scores
from 57.8 (14.5) to 26.2 (12.3) in off medication status. Levodopa
requirement postoperatively was decreased signicantly from a levodopa equivalent dose of 976 (362) to 414 (228). Statistically
signicant improvement in cognitive variables were seen in response
inhibition (Stroop test) in the less than 1 year group & attention (digit
forward) in the more than 1 year group. Deterioration was noted in the
more than 1 year group of executive function parameters (digit backward, phonemic uency & Stroop test) and visual & verbal memory
functions (BVRT, RAVLT). These were statistically not signicant in
the less than 1 year group. A similar signicant but mild deterioration
in MMSE from baseline was noted in post DBS more than 1year group.
Conclusions: Presence of signicant but mild cognitive impairment
after DBS further supports use of this surgical approach for treating the
motor symptoms of advanced PD.
784
Postural verticality problems in parkinson patients
B.R. Bloem, K.A.B. Ravenshorst, I. Benatru, A.S. Gissot,
D. Perennou (Nijmegen, Netherlands)
Objective: To examine whether patients with Parkinsons disease
(PD) have an impaired vertical perception, possibly in a backward
direction, and if this is related to their postural instability.
Background: Postural instability is a major source of handicap for
patients with PD but the pathophysiology still remains largely unclear.
When tested with dynamic posturography on a tilting platform and using
multidirectional perturbations, PD patients are predominantly unstable
when they are being perturbed backward. The nature of this backward
disequilibrium (BD) has to be claried. Recently, it has been shown that
in elderly subjects, spontaneous BD was related to a backward tilt in
vertical perception (Mankoundia et al. 2007, in press), suggesting that
these subjects aligned their erect posture on this erroneous referential of
verticality. Here, we examined whether PD patients also have an impaired
vertical perception. This assumption is supported by mounting evidence
for higher-order somatosensory integration decits in PD patients.
Methods: Seventeen patients with idiopathic PD (mean age 71.29 5.3
years; mean UPDRS motor score 25.3 13.1; mean disease duration

S239

6.9 4.3 years) and 32 matched healthy controls (mean age 72.8 13.4
years) were included. Patients were tested during a subjective optimal ON
phase. Subjective Postural Vertical (SPV) was measured in the sagittal
plane using a custom-made tilting frame (gure). Blindfolded subjects
were passively tilted either forward or backward, and had to realign
themselves with respect to gravity. We measured the mean position of the
SPV (SPV orientation) and the SPV SD (SPV uncertainty). The number of
falls, the retropulsion test, the Backward Disequilibrium Score (BDS) and
the gait disability were also evaluated.
Results: The mean position of the SPV in PD patients was placed much
further backward (-4.75 1.89) compared with control subjects (-1.10
1.46) (signicant difference between PD patients and controls; p
0.001). We also found a signicant correlation between this posterior shift
in SPV orientation and both the retropulsion test and the BDS.
Conclusions: PD patients seem to have a posterior displacement of
their SPV, suggesting that their body scheme is impaired. This may
contribute to the backward directed instability and falls that are commonly seen in Parkinson patients.

FIG. 1 (784).
785
Pathological gambling in Parkinsons disease
M. Yamamoto, Y. Kageyama (Takamatsu, Japan)
Objective: Surveying for a pathological gambling (PG) in Parkinsons disease (PD). We evealed prevalence of PG in PD with dopaminergic treatment by hospiat-based survay. We investigated background
of patients with PG.
Background: There are several reports about drug-induced PG in PD.
Methods: We evaluate 250 consecutive patients with PD who were
treated with levodopa and/or dopamine agonists (DA) with DSM-IV-TR.
Results: We diagnosed pathological gambling in 3 patients. Prevalence
of PG is 1.2%. All were male and had young onset (42, 46 and 45 y-o) of
PD. These three patients were being treated with levodopa in combination
with DA (pergolide2, pramipexole1). All had a long history of PD.
Two of 3 patients had disabling dyskinesia and remarkable motor uctuations, but one did not have any motor complications. The 2 patients also
had other behavioral problems, punding, hypersexuality and aggression.
The 1 patient had no behavioral problem and successfully underwent
psychotherapy without a change of drugs.
Conclusions: PG was rare in PD in this survey. Treatment of PG
patients who had other behavioral problems was very difcult, but one
patient without other behavioral problems was successfully treated by

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S240 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

psychotherapy. The complications of various behavioral problems were
key factors affecting the successful treatment of PG.
786
Dopamine dysregulation syndrome is similarly common among
Parkinsons disease patients treated with rotigotine transdermal
patch as with ropinirole
N. Giladi, H. Shabtai, A. Levi, T. Gurevich, Y. Balash, I. Girshovich,
C. Peretz (Tel Aviv, Israel)
Objective: To compare the occurrence of DDS among Parkinsons
disease (PD) patients treated with two different DA: ropinirole (given
3 times a day as tablets) or rotigotine (once-daily transdermal patch
which gives true continuous dopaminergic stimulation).
Background: Dopamine Dysregulation Syndrome (DDS) presented
as impulse control disturbances and obsession has been associated with
dopamine agonists (DA) therapy in patients with Parkinsons disease
(PD). The prevalence of this syndrome and its relation to the mode of
administration of the DA is still not clear.
Methods: All patients were recruited from Tel Aviv Medical Center; 68
subjects were treated with ropinirole (ropinirole-group) and were part of a
cross-sectional study about DDS in PD and 63 subjects were treated with
rotigotine (rotigotin-group) as part of 3 multi-center, prospective studies.
DDS was diagnosed by direct pre-formulated questions to the patient/
spouse/care giver looking for new and uncontrolled obsessions to gamble,
spend money, eat or be involved in sexual activity.
Results: The ropinirole-group compared to the rotigotine-group was
younger in age of PD onset (54.210.8 vs 58.61.3 years), with more
men (63% vs 56%), longer disease duration (10.46.4 vs. 5.60.5
years) and longer duration of treatment with DA and levodopa
(3.62.5 vs. 1.81.1; 9.25.0 vs. 4.34.2 years, respectively). 20%
of the ropinirole-group and 17% of the rotigotine-group developed
DDS with similar frequencies of obsession to gamble (6.2% vs. 4.8%),
spending money (none vs. 6.3%), over-eating (4.6% vs. 11.1%), and
being involved in sexual activities (13.9% vs. 17.5%).
Conclusions: DDS in the form of impulse control disturbances and
signicant behavioral disturbances are frequent among ropinirole as
well as rotigotine-treated PD patients. Further prospective studies are
needed to better understand the dose response effect as well as the role
of the mode of administration and the different receptor prole of the
DAs on the risk to develop DDS.
787
Myocardial [(123)I] metaiodobenzylguanidine is preserved uptake
in hereditary Parkinsons disease with LRRK2 I2020T mutation
(HPD Sagamihara family)
Y. Ogino, M. Ogino, S. Ujiie, F. Sakai (Sagamihara, Kanagawa,
Japan)
Objective: To evaluate changes in myocardial uptake of (123) Imetaiodobenzylguanidine (MIBG) in hereditary Parkinsons disease
with LRRK2 I2020T mutation (HPD Sagamihara family).
Background: Decrease in myocardial uptake of (123)I-MIBG has
been reported in PD and Diffuse Lewy body disease (DLBD) both of
which have Lewy bodies (LBs) as a pathological hallmark. It is
assumed that the decrease in myocardial uptake of (123) I-MIBG and
the presence of LBs pathology are closely related since LBs were not
found in most cases of HPD Sagamihara family, we assumed that
myocardial uptake of (123) I-MIBG was preserved. We performed this
study to evaluate the hypothesis that LBs pathology and decrease in
myocardial (123) I-MIBG uptake of (123) I-MIBG are closely related.
Methods: [(123)I] MIBG cardiac Scintigram was performed and
H/M ratio was calculated in eight hereditary Parkinsons disease with
LRRK2 I2020T mutation (HPD Sagamihara family) patients and thirteen sporadic Parkinsons disease patients.
Results: H/M ratio in early phase in Sagamihara family is 1.43-2.08
(average 1.83, SD 0.22), in PD 1.17-1.48 (average 1.26, SD 0.10).
Myocardial uptake of (123)I-MIBG in HPD Sgamihara family was well
preserved until late stage of the disease.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Conclusions: Our data showed that myocardial uptake of (123)

I-MIBG is preserved in HPD Sagamihara family as we assumed. It has
been reported that Myocardial uptake of (123) I-MIBG is also preserved in PARK2 (lacks LBs pathology) same as we found in HPD
Sagamihara family. The decrease in myocardial uptake of (123) IMIBG seems to be limited to diseases with LBs pathology. We assume
that the mechanism responsible for Lewy body formation is also
responsible for decrease of (123)I-MIBG uptake. Our results suggest
that PARK8 has different disease mechanism from PD.
788
Nighttime sleep problems and daytime sleepiness in Parkinsons
disease
D. Verbaan, S.M. van Rooden, M. Visser, J. Marinus, J.J. van Hilten
(Leiden, Netherlands)
Objective: To evaluate nighttime sleep problems (NSP) and daytime
sleepiness (DS) in patients with Parkinsons disease (PD) and control
subjects, and to assess the relation with demographic, disease-related
and clinical characteristics in patients.
Background: In PD, NSP and DS are regarded as important features
of the non-motor symptom complex.
Methods: A cohort of 420 patients with PD was evaluated for the
occurrence of NSP and DS, motor and non-motor symptoms, as well as
for demographic and disease-related characteristics. A reliable and
valid instrument, the SCOPA-SLEEP, was used to study NSP and DS.
Results were compared with those of 150 control subjects. In patients,
associations between NSP and DS and demographic and clinical characteristics were also studied.
Results: Compared to control subjects, a signicantly greater proportion of patients had excessive DS (43% vs. 10%), were poor
nighttime sleepers (27% vs. 9%), or used sleep medication (17% vs.
12%). In both patients and control subjects, women experienced more
NSP compared to men. Difculties with falling asleep were similar in
patients and control subjects. In patients, depressive symptoms accounted for 21% of the variance in NSP and was the major contributor
to the total explained variance of 30%. When analysing the depressed
and non-depressed patients separately, depressive symptoms had also
the largest contribution. Furthermore, NSP were related to daily dopamine-agonist and levodopa dose, whereas DS was related to age,
daily dopamine-agonist dose and disease severity. Patients without
NSP and DS had the least motor, cognitive, and autonomic problems,
and the least psychiatric complications. Patients with excessive DS had
more motor and cognitive problems than poor sleepers, while poor
sleepers experienced more depressive symptoms and motor uctuations
compared to patients with excessive DS.
Conclusions: NSP and DS occur frequently in PD, with excessive DS
being reported more common. The strong relation between NSP and
depression in PD is of major importance for patient management.
789
Comparison of sympathetic skin response and urodynamic study in
Parkinsons disease
M. Tavsan, O. Mertoglu, U. Sener, Y. Zorlu, F. Zorlu (Izmir, Turkey)
Objective: We aimed to investigate the relationship between sympathetic skin response(SSR) and urodynamic parameters to evaluate
urinary dysfunction in Parkinsons disease (PD).
Background: Dysfunction of autonomic nervous system is frequent
in Parkinsons disease. Symptoms of dysautonomia include cardiovascular, gastrointestinal, urogenital, sudomotor and thermoregulatory
dysfunction. SSR is a noninvasive procedure and correlation beetween
urinary dysfunction is obscure.
Methods: 35 patients (18 men and 17 women) with idiopathic PD
were included in the study. The clinical disability of the patients was
graded using the Unied Parkinsons Disease Rating Scale. SSR signals were recorded on both hands and the widest amplitude response
and its corresponding latency were evaluated. Urodynamic study including lling, pressure ow phases and sphincter EMG was performed
to all patients. During the study amplitude of detrusor overactivity,

POSTER SESSION III, THURSDAY, JUNE 7, 2007

bladder capacity, incontinance status and existance of sphincter
pseudodyssynergia were also evaluated.
Results: Duration of disease (cut off level was taken as 10 years) had
no effect on UPDRS, SSR and urodynamic parameters in terms of
detrusor overactivity, incontinence, bladder capacity and pseudodysinergia (p0 05). Negative and positive SSR ndings and severity of
UPDRS scale also had no signicant relation to urodynamic parameters
(p0,05).There was no statistically signicant association between
urodynamics and disease duration, UPDRS, SSR.
Conclusions: The most frequent urinary symptoms in PD are difculty in voiding, nocturnal urinary frequency, sensation of urgency,
urge incontinence, diurnal urinary frequncy, enuresis and urinary retention. Although both SSR and detrusor overactivity, incontinance,
sphincter dyssynergia show dysautonomia in PD, in our study none of
the urodynamic parameters corralated with SSR.
790
Inuence of intestinal levodopa on non-motor symptoms of Parkinsons disease a case report
M. Koegl-Wallner, R. Saurugg, P. Schwingenschuh, P. Katschnig,
K. Wenzel, M. Maric, T. Hinterleitner, E. Ott (Graz, Styria, Austria)
Objective: We report one case of improved quality of life due to a
positive effect of Duodopa on a sensory non-motor symptom.
Background: Patients with advanced Parkinsons disease (PD) suffer
from well known motor but also from a wide range of non-motor
symptoms. Studies have shown that intestinal levodopa therapy (Duodopa) improves motor function and reduces motor uctuations in PD
patients. So far, reports on the effect of Duodopa on oftentimes very
disabling non-motor symptoms have been rare.
Methods: A 70 year old male patients with advanced PD diagnosed
in 1991 (Hoehn und Yahr stage III) suffered from severe motor uctuations not treatable despite of an optimised oral levodopa therapy
(levodopa/carbidopa/entacapone dopamine agonist). The patient reported sudden onsets of OFFs, and severe peak dose dyskinesias. In
addition to these disabling motor features, the patient was affected by
sensory non-motor symptoms such as heat, pain, and a feeling of
collapsing, all these long term symptoms frightened the patient. Because of severe motor uctuations (as mentioned above), we start a
treatment with intestinal levodopa therapy (Duodopa).
Results: In our patient motor function improved under continuous
intraduodenal levodopa therapy, as shown in studies before. Remarkable was, that the sensory non-motor symptom, our patient suffered
from during a long time, disappeared under Duodopa-treatment. This
led to an signicant improvement in Quality of life not only for the
patient but also for his caregivers.
Conclusions: Our case report demonstrates an efcacy of intestinal
levodopa treatment in both motor and non-motor symptoms of advanced PD. Because non-motor symptoms are often more disabling for
PD patients than motor features, more attention should be paid to this
positive treatment effect of Duodopa.
791
Use of Parkinsons disease sleep scale (PDSS) and polysomnography in sleepy Parkinsons disease (PD) patients
S. Muzerengi, A. Williams, V. Dhawan, D. Whitehead,
P. Martinez-Martin, K. Ray Chaudhuri (London, United Kingdom)
Objective: To assess the polysomnographic correlation and diagnosis
with the use of Parkinsons disease (PD) sleep scale (PDSS) in a subset
of PD patients presenting with severe excessive daytime sleepiness
(EDS).
Background: Sleep problems are a major non-motor symptom of PD
and occur in over 80% cases.1 Many have severe EDS which may be
multifactorial and may indicate poor sleep at night due to sleep disordered breathing or a narcolepsy type phenotype 2.
Methods: Patients are normally screened in the clinic using the PDSS
and the Epworth Sleepiness Scale (ESS). Additionally they underwent
overnight polysomnography under standard conditions.

S241

Results: 34 treated PD patients with severe daytime sleepiness, mean

age 66.27 9.84, 23 male (67.6%), mean duration of disease 6.80
4.10, mean Hoehn and Yahr (HY) score 2.70 0.77, mean Epworth
sleepiness score (ESS) 12.36 4.98, have been studied. PDSS revealed impaired scores on overall quality of night sleep, daytime
dozing, and sleep refreshment with low total score. Polysomnography
(PSG) has been performed on 12 (mean age 66.5814, ESS 12) so
far showing impaired sleep efciency and sleep time, mean sleep
efciency 61.84 13.10, mean total sleep time 267.04 66.63; 50%
had periodic limb movements (all showed item 3 score on PDSS 5).
Conclusions: Abnormalities on the Parkinsons disease sleep scale
predict abnormalities of sleep pattern on polysomnography conrming
the important clinical role of using PDSS as a screening instrument.
References:
Dhawan V, Healy DG, Pal S, Chaudhuri KR, Sleep-related problems
of Parkinsons disease Age and Ageing 2006; 35:220 228.
Arnulf I, Konofal E, Merino-Andreu M et al. Parkinsons disease and
sleepiness An integral part of PD. Neurology 2002; 58:1019-1024.

792
Results of the PRAMI study: Prevalence of neuropsychiatric symptoms in patients with idiopathic Parkinsons disease (PD)
M. Dujardin, P.L. Lleu (Paris, France)
Objective: To estimate the prevalence of neuropsychiatric symptoms
in French patients with PD and to compare the depression prevalence
in a PD population (cases) with other neurological affections (non PD
control).
Background: Prevalence of depression in PD patients compared to
other neurological affections is nowadays not well known.
Methods: Case control pharmaco-epidemiological study conducted
with a representative sample of 81 French neurologists between September 2005 and August 2006. The study population consisted of 363
patients with PD (PD population). Among them, 252 patients (PD
cases) were matched on age and gender with 252 patients with another
neurological affection (non PD control). Demographic and disease
characteristics, neuropsychiatric symptoms, treatment procedures were
collected.
Results: Among the 363 patients (PD population) the mean age was
69 years old, gender ratio M/F was 1.6. For 32% of patients PD were
diagnosed since more than seven years. Hoehn and Yahr stages was
2 for 63% of patients and at least 2.5 for 37%. According to neurologists, 65% of the PD patients were considered on initial stage (no
uctuation), 35% on advanced stage (with complications). Mean
UPDRS III score was 24 (SD14) for the whole PD population (19 for
initial stage group, 32 for advanced stage group). Psychiatrics symptoms were reported by neurologist as followed: symptoms of anxiety
(55% of patients), signs of apathy (29%), diagnosis of dementia (3%)
and diagnostic of depression (30 %). Mini international neuropsychiatric interview (MINI) evaluated 34% (N123) of patients with depression. Among them 57 were new patients diagnosed with depression
(16% of PD population).Case-control analysis allowed to compare 252
cases patients with 252 control patients essentially suffering from:
neurovascular (22%), neurodegenerative (22%), peripheral nerves
(21%) disorders. Depression diagnosed by MINI showed a signicant
higher prevalence of depression patients with PD than with other
neurological diseases, all affections combined (35% vs 28%, p0.05).
Conclusions: This study conrmed PD is associated with high prevalence of neuropsychiatric symptoms such as depression, anxiety and
apathy. However depression remains underdiagnosed. It is also interesting to note that depression is observed with a signicant higher
prevalence in PD than other neurological diseases.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S242 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

793
Community based prevalence study of Parkinsons disease describing age at onset distribution in Cardiff, UK
M.M. Wickremaratchi, E. Morgan, C. OLoghlen, D. Sastry,
N.P. Robertson, Y. Ben-Shlomo, H.R. Morris (Cardiff, United
Kingdom)
Objective: To measure the prevalence of Parkinsons disease (PD)
and Young Onset PD in the City of Cardiff, UK.
Background: PD is the second most common neurodegenerative
disorder in the world. Prevalence studies help to estimate disease
burden and thus help inform health service provision. There has been
a considerable recent interest in Younger Onset PD (YOPD) following
the identication of genetic causes in some patients. The underlying
prevalence of YOPD and autosomal recessive PD is unclear. Although
a number of prevalence studies have been carried out, none have
focussed on community based ascertainment of YOPD.
Methods: Primary care records of over 35 practices serving a population of over 250,000 were actively searched to identify any possible
patient with Parkinsonism and Parkinsons disease using standardized
search strategies for diagnostic codes and drug prescription. Relevant
patient records were scrutinized and those individuals with records
consistent with PD were issued postal questionnaires in order to obtain
additional clinical and demographic information. All patients were then
invited to attend for a formal clinical assessment.
Results: We identied 289 patients with PD in a population of
253,826 providing crude and age-standardized prevalence of 114 and
88 per 100,000 respectively. Four percent of prevalent cases reported
onset before the age of 45 years, 10% before 55 years and 31% before
65 years.
Conclusions: We believe that this large scale primary care based
study of PD provides an accurate and representative epidemiological
information on the PD population in South Wales. Onset of PD before
the age of 65 occurred in 44% of the population although YOPD (onset
before 40 or 45 years) remains relatively rare but represents an important resource for the investigation of the genetic aetiology of PD and in
documenting phenotypic variation.
794
Placebo inuences on dyskinesia in Parkinsons disease
C.G. Goetz, E. Laska, C. Hicking, P. Damier, T. Muller, J. Nutt,
C.W. Olanow, O.O. Rascol, H. Russ (Chicago, Illinois, USA)
Objective: Determine clinical features that are prognostic indicators
of placebo response among dyskinetic Parkinsons disease (PD) patients.
Background: Placebo-associated improvements occur in parkinsonism, but responses in dyskinesia have not been studied.
Methods: Placebo data from two multicenter studies with identical
design comparing sarizotan to placebo (Paddy-1 and Paddy-2) for
treating dyskinesia were accessed. Sarizotan (2 mg/day) failed to improve dyskinesia compared to placebo, but both treatments signicantly improved dyskinesia compared to baseline. Step-wise regression
was used to identify baseline characteristics that inuenced dyskinesia
response to placebo, and these factors were entered into a logistic
regression model to quantify their respective inuence on placeborelated dyskinesia improvements and worsening. Because placeboassociated improvements in parkinsonism have been attributed to dopaminergic augmentation, we also examined the association between
changes in parkinsonism and changes in dyskinesia.
Results: 484 subjects were treated with placebo in the two studies.178 met criteria for positive (dyskinesia improvement) and 37 for
negative (dyskinesia worsening) placebo responses. Older age, lower
baseline UPDRS score and lower total daily levodopa doses were
associated with positive placebo response, whereas lower baseline
dyskinesia score was associated with negative placebo response. Placebo-associated dyskinesia changes were independent of changes in
UPDRS score, and all effects seen in the sarizotan group were statistically explained by the placebo-effect regression model.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Conclusions: Dyskinesias are affected by placebo-treatment. The

independence of placebo induced changes in dyskinesia and parkinsonism argues against a dopaminergic augmentation mechanism to
explain placebo-associated improvements in parkinsonism. The magnitude and variance of placebo-related changes and the factors that
inuence them could be helpful in the design of future clinical trials of
antidyskinetic agents and the calculation of suitable sample sizes to
detect differences from placebo.
795
New formulation of carbidopa/levodopa (IPX054 (VADOVA
IRER) vs. standard carbidopa/levodopa in stable PD patients
C.G. Goetz, S. Leurgans, V.K. Hinson, A. Hsu, W. Fan, S. Wang,
T. Nguyen (Chicago, Illinois, USA)
Objective: IPX054 is a bilayer tablet of immediate release and
controlled release carbidopa/levodopa (CD/LD). We compared IPX054
given BID vs standard CD/LD QID in patients with stable Parkinsons
PD.
Background: At the same total 24-hour mg dosing, formulations with
short and long-acting effects may provide benet and minimize dosing
frequency.
Methods: 12 PD patients were on CD/LD 25/100 QID but no MAO
or COMT inhibitors and were clinically stable with only mild uctuations and no troublesome dyskinesia. They were randomized to a
double-blind crossover study comparing IPX054 (50/200) BID (8
a.m./4 p.m. placebo given noon/8 p.m.) and standard CD/LD 25/100
QID. At the end of each 2 week treatment, patients self-recorded an
at-home videotape and performed a modied UPDRS motor examination and Rush Dyskinesia Rating Scale (RDRS) at 30 minute intervals
over 8 hours (8 a.m.-4 p.m. inclusive), covering one drug cycle of
IPX054 or two drug cycles of standard CD/LD). The primary outcome
was the number of the 17 videotape epochs in which the patients were
rated as ON without dyskinesia or ON without troublesome dyskinesia
by a blinded observer based on the videos (Wilcoxon Signed Rank
Tests).
Results: The 9 men and 3 women had a mean age of 69 years (SD
11.0) and mean PD duration of 6 years (SD 3.4). All participants
completed the videotapes and only 1 time point was missing from 1
patient (99.5% taping compliance). Of the 17 videotape epochs (taken
every 30 minutes), IPX054 treatment and CD/LD treatment showed no
signicant differences (p0.14). However, the mean number of ON
without dyskinesia or without troublesome dyskinesia scores was modestly higher on IPX054 vs CD/LD (11.0 SD 3.8 vs 9 SD 3.8). No
patient developed troublesome dyskinesias during the videotape assessments. The mean modied UPDRS scores favored IPX054 treatment as
well (14.4 SD 4.8 vs 16.9 SD 6.1), with a statistical trend p0.052).
Mean RDRS scores were modestly better on IPX054, (0.45 SD 0.61 vs
0.69 SD 0.91), but this difference was not signicant.
Conclusions: In stable PD patients without troublesome dyskinesia,
BID IMX054 provided comparable clinical efcacy to CD/LD QID
during objectively observed assessments. In this population, substitution with IPX054 maintains CD/LD efcacy and provides ease of
administration which may offer improved treatment compliance.
796
Tolcapone (TASMAR) in the treatment of advanced Parkinsons
disease: Results of a postmarketing surveillance study
G. Ebersbach (Paracelsusring, Germany)
Objective: To assess efcacy, tolerability, and safety of adjunctive
tolcapone therapy in patients with advanced Parkinsons disease (PD)
who were intolerant of or did not respond to other catechol-O-methyltransferase (COMT) inhibitors.
Background: Tolcapone (TASMAR), a potent inhibitor of COMT,
is an effective adjunct to levodopa/carbidopa therapy in patients with
uctuating advanced PD.
Methods: This observational study of 237 men and women including
high percentage of elderly patients (median age 69.9 yrs) with advanced PD was conducted in 97 centers. Subjects received adjunctive

POSTER SESSION III, THURSDAY, JUNE 7, 2007

tolcapone therapy according to medical need at a dose and duration
determined by the treating physician. The recommended starting dose
of tolcapone was 100 mg TID. Treatment efcacy was assessed by
patients and physicians at study end based on a clinical global assessment of PD symptoms. The observation period was 6 months and
included 13 laboratory measurements of liver enzymes. Adverse events
were evaluated by spontaneous reports and observation of patients at
assessment visits.
Results: Tolcapone was administered for the rst time during this
study in 92% of patients, and tolcapone replaced entacapone in 75% of
patients. The initial median daily dose of tolcapone was 300 mg in 85%
of patients; 14% of patients required a dose adjustment. Improvement
of patient symptoms based on a global efcacy assessment was reported by 84% of physicians and 80% of patients. Liver transaminase
levels were monitored in 78% of patients. Elevations in liver enzymes
above the upper limit of normal were found in 8%, 7%, and 3% of
patients for AST, ALT, and both enzymes, respectively. However,
these elevations were mostly mild (2- to 3-fold increase) and not
clinically signicant. The most frequently reported adverse event was
diarrhea (8 patients). None of the 15 reported adverse events related to
tolcapone was serious or life-threatening.
Conclusions: This study conrms that tolcapone is efcacious, well
tolerated, and safe in the adjunctive treatment of advanced PD. Tolcapone was even well tolerated in elderly patients 70 yrs who usually
tend to complications during drug therapy. Elevations in transaminase
level were mostly mild, and no instance of serious hepatotoxicity was
observed.

S243

798
Automated gait detection algorithm from three dimensional acceleration signals of ankles in patients with Parkinsons disease
J.-Y. Kim, J.-Y. Lee, K.S. Park, B.S. Jeon (Seoul, Jongno-Gu, Korea)

Sanamide potentiates the effects of DA-agonists in early stage

Parkinsons disease (PD) patients
R. Anand, M. Onofrj, A.H. Schapira, S.M. Rossetti (Pescara, Italy)

Objective: Accurate evaluation of motor uctuation in advanced

Parkinsons disease (PD) is a difcult and laborious task, but is essential to adjust antiparkinsonian medications. It requires prolonged observation for accurate assessment. As prolonged observation or visual
monitoring is impractical if not impossible, we at the Biomedical
Signal and Information Laboratory and Neurology Department of
Seoul National University Hospital are developing an unrestrained
monitoring system which will allow long-term analysis of movement.
We are currently focusing on gait, which is an important feature of
advanced PD.
Methods: We developed 1) wearable activity monitoring system
(W-AMS), which allows continuous measurement of 3-dimensional
acceleration at the ankles, 2) information transfer and storage in a ash
memory, and 3) off-line gait detection algorithm from 3-dimensional
acceleration information. The algorithm consists of movement detection, swing phase extraction and swing peak detection. The foot pressure and movement images were recorded simultaneously as the reference signal. The detected swing peaks were compared with foot
pressure signal and movement images.
Conclusions: W-AMS can be attached easily and doesnt interfere
with daily activity. It will promise for long-term gait monitoring and
quatitative assessment. Kinetic data extraction (cadence, gait speed
estimation, balance, etc.) is possible from gait detection result. Also
freezing of gait detection algorithm will be developed by frequency
analysis of each extracted gait with this synchronized gait monitoring
system.

Objective: To evaluate the efcacy and safety of sanamide as

add-on to a single DA-agonist, compared with a DA-agonist alone, in
early stage PD patients.
Background: The benets of DA-agonist monotherapy in early PD
patients are reduced over 2-3 years. Addition of L-dopa, a common
practice, leads to motor uctuations and potentially, dyskinesias. Sanamide, a new anti-PD agent that combines selective and reversible
MAO-B and glutamate release inhibition, has shown potential efcacy
in potentiating the effects of DA-agonists.
Methods: In this double-blind, placebo-controlled, multinational,
24-week, Phase III study, 270 non-uctuating PD patients (5yr duration) on a stable dose of a single DA-agonist, were randomly assigned
to receive sanamide (Low Dose: 50-100mg/day, or High Dose: 150200mg/day) or placebo. Efcacy and safety evaluations were performed regularly.
Results: The proportion of patients receiving each DA-agonist was:
ropinirole 45%, pramipexole 20%, carbergoline 7%, other 28%. In the
ITT analysis, the mean changes from baseline to endpoint indicated
DA-agonists alone were less effective (p0.05) than the DA-agonist
and sanamide Low Dose group; UPDRS-III: -6.07.2 vs. 3.67.1
and UPDRS-II: -2.23.8 vs. -1.23.5. The proportion of responders
at endpoint was also higher in both sanamide groups, whether based
on a 30% improvement in UPDRS-III (High Dose: 54.3%; Low
Dose: 50.6%; Placebo: 36.4%) or a score of 1, 2 or 3 on CGI-Change
(High Dose: 65.8%; Low Dose: 67.9%; Placebo: 49.4%). Treatment
with sanamide also improved cognitive function, as assessed by the
Cogtest battery in a subset of patients. A trend for improvement in
quality of life was also noted. In general, sanamide was well tolerated
with the incidence of most common adverse events being comparable
to that of placebo, and no increase in adverse events, abnormal vital
signs, laboratory tests or ECG parameters, compared to DA-agonists
alone, despite no restriction on dietary tyramine intake.
Conclusions: Sanamide potentiated the effect of a single DAagonist in early stage PD patients, improving both motor symptoms and
ADL. Benecial effects of sanamide on cognition were also noted and
are being investigated in new trials.

FIG. 1 (798).

797

Movement Disorders, Vol. 22, Suppl. 16, 2007

S244 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Gait detection algorithm accuracy
mean No. of gait
average of detected gaits
detection rate
false positive

Normal : 5 persons

PD patients : 5 persons

50 gaits
47 gaits
47/5094%
2.4/50 gait

125 gaits
116 gaits
116/12593%
2.2/125 gait

799
Dementia in Parkinsons disease (PD): A 20-year prospective Sydney Multicentre study
W.G.J. Reid, M.M.A. Hely, J.G.J.L. Morris, C.T. Loy, G.M. Halliday
(Sydney, NSW, Australia)
Objective: To study the changes in cognitive functioning and dementia in a group of community-living denovo patients with PD over
a 20-year period.
Background: To date there have been few if any very long-term
prospective clinical and neuropsychological studies of PD that have
documented changes in motor and cognitive functioning in the same
patients using the same tests over twenty years of treatment.
Methods: 111 community living denovo patients with PD were
studied over twenty years using a detailed clinical and neuropsychological test battery. The tests were administered at baseline prior to
starting drug treatment, 3, 5, 10, 15 and 20 years. Neuropsychological
and dementia status was assessed at each time interval and was correlated with variables such as age, symptoms of PD and treatment
response.
Results: At the 20-year follow-up, 80.0% of the remaining 15 patients were found to be demented. This is consistent with our previous
observation that dementia prevalence rises sharply in PD after ten
years. Age of onset also had a signicant impact on dementia-free
survival. A younger age of onset was associated with a longer dementia-free survival (Hazard Ratio1.08, p0.001). Subjects with disease
onset at 75 years were 4.82 times more likely to develop dementia at
any stage of PD compared to those with disease onset at the age of 55
years.
Conclusions: Our results show dementia is common in PD even in
the early untreated stage of disease and that the majority of patients
with PD if followed up over a long time will progress to dementia and
that older age of onset is a strong predictor of dementia at any stage in
the course of the disease.
800
Treatment of levodopa induced dyskinesias with Levetiracetam
D. Richardson, M. Eisa, A. Toenjes, R. Bajwa, D. Miller, B. Jabbari
(New Haven, Connecticut, USA)
Objective: To determine the effect of escalating doses of Levetiracetam (UCB, Pharma) on levodopa-induced dyskinesias (LID) in Parkinsons disease.
Background: Altered ring and hypersynchronization of globus pallidus
interna (Gpi) neurons has been implicated in the pathophysiology of
levodopa induced dyskinesia (Hill et al. Mov. Disord. 2003). Levetiracetam desynchronizes neural cell ring, as seen in human models of
epilepsy (Gorgi, et al Epilepsia 2000). The use of Levetiracetam may
therefore reduce the dyskinesias by altering the ring pattern in the GPi.
Methods: Ten subjects with PD and LID were studied in an open
label protocol. Each subject had a neurological, Mini Mental Status
Examination (MMSE) and Brief Psychiatric Rating Scale (BPRS) at
baseline and at the conclusion of the study. The Unied Parkinsons
Rating Scale (UPDRS), Abnormal Involuntary Movement Scale
(AIMS), Lang-Fahn Dyskinesia/ADL Scale (LFDS) and Dyskinesia
Rating Scale (DRS, Goetz 1964) were performed at baseline, on follow
up (weeks 6, 10 and 14) and at the conclusion visit (week 16).
Levetiracetam was initiated at 250mg (week 2). Subsequent increases
were 500mg (week 4), 1000mg (week 6) and 3000mg (week 10). A two
week taper followed by a two week wash-out period (weeks 14-16)
occurred prior to the conclusion visit. Baseline anti-parkinson medications were not changed during the study.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Results: Eight men and two women (mean age 61.5) were enrolled.
One subject dropped out after enrollment. Three subjects could not
tolerate Levetiracetam doses higher than 500-1000mg/day. The AIMS
score improved in all six remaining subjects who reached 3000mg/day
dosage (37%-90%: mean 50%). Four of these six patients demonstrated
a 1-3 grade improvement on the DRS. Four of the six also showed
improvement on the BPRS (10.2-30.7, mean 25). No signicant improvements were noted on the UPDRS, MMSE or LFDS.
Conclusions: The results of this study suggest that Levetiracetam
(500mg -3000mg) can improve LID in Parkinsons disease. Verication with a larger blinded study is recommended.
801
Anxiety, depression and swallowing disorders in patients with
Parkinsons disease
Y. Manor, B. Meirav, N. Giladi, R. Mootanah, J.T. Cohen (Tel-Aviv,
Israel)
Objective: To characterize the relationships between level of anxiety,
depression and the patients subjective report as well as objective
assessment of swallowing disturbances (SD) in patients with Parkinsons disease (PD).
Background: Swallowing disturbances are associated with affective
responses such as anxiety and depression. SD, anxiety and depression
are common in patients with PD. The relationships between these
affective symptoms and SD in patients with PD are unclear.
Methods: Sixty-six PD patients, non-demented by MMSE, answered
if experiences any SD, completed SD questionnaire, and underwent
evaluation for anxiety and depression. All patients underwent detailed
clinical swallowing evaluations and those diagnosed with SD by the
speech language pathologist (SLP) also had beroptic endoscopic
evaluation of swallowing to determine aspiration.
Results: Thirty-seven PD patients (24 males) with SD, diagnosed by
the SLP (mean age 69.1 10.1 years, disease duration 8.5 6.1 years
and H&Y stage 2.3 1.2) and 29 PD patients (24 males) without SD
(mean age 64.5 13.2 years, disease duration 7.5 6.1 years and
H&Y stage 1.8 1.0) participated in the study. The SD patients group
reported signicantly higher anxiety and depression than patients without SD (t(66) 3.116, p0.003, and t(67)2.056, p0.044, respectively). The clinical characteristics of the two groups were similar.
Among the patients with SD, 50% exhibited aspiration. No signicant
difference was found in anxiety and depression level between patients
with and without aspiration.
Conclusions: Patients with PD with SD exhibit more depression and
anxiety than PD patients without SD. The contributing role of anxiety
or depression to the development or worsening of SD and as a result,
their place in the treatment strategy needs further investigation.
802
Healthcare educational needs and experiences of people living with
Parkinsons disease (PD): An exploratory study
S. Giles, J.M. Miyasaki (Toronto, Ontario, Canada)
Objective: To understand the educational needs and experiences of
patients with advanced PD and their family.
Background: Educational and information needs of patients and
family members in medical settings are well documented. However, the
subjective experiences of PD patients and their family members educational needs has not been reported.
Methods: Phenomenological framework (van Manen, 1997). Sample
of 5 caregivers and 4 late stage PD patients. In-depth, semi-structured
interviews yeilded data for interpretive phenomenological analysis.
NVIVO 7 qualitative research software progam was used for data
management/analysis.
Results: Themes: We really dont know what to ask for: participants
f did not know what questions to ask in order to obtain assitance.
Maybe its better not know: while participants stated they wanted all
prognostic health outcome information, when pressed they thought it
might be better not to know. Just take the pills: participants reported
that they were under pressure to not ask questions and comply. It was

POSTER SESSION III, THURSDAY, JUNE 7, 2007

really shocking: participants used the internet without direction from
healthcare workers and were overwhelmed by the information they
found. They learned to advocate for themselves including nding a
neurologist. They felt their family physician could not provide this
resource. Theres really not much there: participants reported that there
was not enough support from health care team. Youre not out there
trying to gure it out on your own: participants reported being overwhelmed and feeling helpless and requested health care team support
so that they could navigate the complex healthcare system. Those with
elevated socioeconomic status had challenges acquiring private care
services.
Conclusions: Frustration in obtaining healthcare resources, from
referral to a neurologist to in-home assistance was pervasive. Providing
a single referral source for allied health care would decrease caregiver
strain and avoid reinventing the wheel. Within the Ontario healthcare
system, Community Care Access is determined locally. Eligibility for
nursing, personal care or respite depends on the patients location and
the priorities set by the Local Integrated Health Network. Coordinating
in-home care across the province would diminish family frustration and
allow central referral to inform caregivers what they can expect, and
inequities in the system would be minimized.

S245

count the number of remaining teeth. We counted out the tooth with
dental caries at C4 stage whose dentin hardly remains.
Results: The average numbers of remaining teeth in the disease
control group, the CVA group, and PD patients were 19.2, 19.4, and
15.6 respectively; the decrease in PD patients was signicant (Fig. 1).
The case rates of periodontal disease in the disease control and the
CVA groups were 35.2% and 35.6% respectively. By contrast, the case
rate in the PD patients was 83.3% and was signicantly high (Fig. 2).
Conclusions: PD patients show signicantly smaller number of remaining teeth and higher case rate of periodontal disease than disease
control group. The enhanced incidence of tooth loss and periodontal
disease were apparent even compared with the CVD group, which
reportedly is known to show frequent oral problems. The brushing is
crucial for the prevention of the progression of periodontal disease.
Motor and cognitive impairments of PD patients may be cardinal
causes of the impaired oral hygiene. Education of dental hygiene may
be necessary from the early stage of PD to increase remaining teeth and
to maintain good QOL.

803
Unilateral haemorrhage in Gpe (globus pallidum externum) improving the Parkinsonian and psychotic symptomatology. A case
report
M. Balaz, I. Rektorova, I. Rektor (Brno, Czech Republic)
Objective: We report the case of 75 years old patient with unilateral
basal ganglia lesion caused by the hemorrhage located in the external
globus pallidum.
Background: The lesions on various lacations within basal ganglia
affect the parkinsonian and other movement disorders symptomatology.
Methods: A case report of 75 year old woman with Parkinson
disease.
Results: After the onset of hemorrhage the dyskinesia, hallucinations
and delusions occured. The lesional effect of the bleeding led to the
bilateral improvement of parkinsonian symptomatology with only minimal neurological sequalae (minimal hemiparesis). Lesion mimicking
the DBS STN surgery with lasting lesional effect (stable improvement
in UPDRS III at 3 and 6 months after the haemorrhage) allowed for the
substantial decrease of dopaminergic medication. This resulted in the
slow decrease and eventually cessation of both dyskinesias and hallucinations.
Conclusions: We suppose that the onset of choreatic dyskinesias
observed after the brain haemorrhage in patient with stable dose of
dopaminergic treatment suggested the benecial effect of the lesion on
the parkinsonian and psychotic symptomatology.
804
Increased periodontal disease and tooth loss in Parkinsons disease
A. Hanaoka, K. Kashihara (Okayama, Japan)
Objective: To evaluate if the incidence of periodontal disease and
tooth loss is high in patients with Parkinsons disease (PD).
Background: Impairment of oral hygiene may lead to periodontal
disease, tooth loss, and consequently may compromise the quality of
life (QOL). In patients with Parkinsons disease, both motor and
cognitive decline may disturb oral hygiene.
Methods: We investigated the case rate of periodontal disease and
the number of remaining teeth in 54 consecutive patients with PD of 60
to 75 years old. We provided two age-matched control groups; One is
comprised of 45 patients with acute cerebral vascular accident who
showed no motor nor cognitive impairment before the attack (CVA
group). Another is comprised of the other 54 neurological patients
without cognitive or motor impairment (disease control group). We
determined 3 mm or more of periodontal pockets as periodontal disease. We also observed the whole buccal cavity with a dental mirror to

FIG. 1 (804).
805
Punding in Parkinsons disease
A. Bora Tokcaer, O. Kapucu, N. Erdogmus Ince, U.O. Akdemir
(Ankara, Turkey)
Objective: To evalute the cerebral metabolism in Parkinsons disease
(PD) patients with punding behaviors.
Background: Punding is a complex stereotyped behaviour characterized by an intense fascination with repetitive manipulations of different
kinds of objects. Punding behaviour is believed to be secondary to
dopaminergic excess. In some cases it is associated with dopamine
dysregulation syndrome. Punding behaviours are usually correlated
with persons occupation and hobby experiences. However the behaviours of obsessive-compulsive disorders (OCD) are related to social
and territorial concerns. The pathophysiology of punding has still to be
unknown.
Methods: We presented 5 PD patients who had punding behaviours.
18F-FDG brain positron emission tomography (PET) were done in
three patients.
Results: All of ve patients were male, their ages were between
42-59. Three of them were engineers, 1 patient was a teacher and the
other one was working as a secretary. Two of them had 2 different
punding behaviours, other patients had only one stereotyped behaviour.
Playing saz (a local stringed instrument), repetitive writing, dismantles
radios, computer games were punding behaviours of these patients. The
medications were levodopa, pergolide, pramipexole and cabergoline.
Three patients were taking additional doses of LD or dopamine agonists to be able to move during the night for their hobbies. PET studies
demonstrated prefrontal cortical hypometabolism in two patients and
bilateral occipitotemporal hypometabolism in one patient.
Conclusions: This is a small study to evaluate cerebral metabolism in
PD patients with punding behaviours. Our results are conict. Further
case control studies should be planned to search the pathophysiology of
this abnormal behavior.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S246 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

806
Long-term pre-administration of dopamine agonists alters L-dopa
induced circling behavior in the 6-OHDA lesioned rat
E.L. Lane, S.B. Dunnett (Cardiff, Wales, United Kingdom)
Objective: This experiment was designed to determine the effect of
long-term exposure to dopamine agonists on subsequent L-dopa treatment.
Background: L-dopa is the most effective drug treatment for Parkinsons disease (PD) currently available. However, with long-term treatment, most patients develop choreic and dystonic movements, termed
dyskinesia. Dopaminergic agonists have less propensity to induce dyskinesia and are often administered in the early stages of PD. It is
unclear whether this early treatment phase impacts on the generation of
dyskinesia once L-dopa therapy is initiated.
Methods: Unilaterally 6-OHDA lesioned rats were injected with
dopamine agonists (piribedil, 1mg/kg; ropinirole, 1mg/kg; bromocriptine, 1mg/kg) or saline (0.9%) daily for 21 days. All rats were then
administered with L-dopa (10 mg/kg with benserazide 15 mg/kg in
saline i.p.) daily for a further 21 days. Rotational behavior was recorded
in automated rotameters at regular intervals and abnormal involuntary
movements (AIMs) were scored simultaneously.
Results: Over the 21 days, piribedil induced a constant level of
contralateral rotation, whilst circling in response to both ropinirole and
bromocriptine increased progressively. None of the dopamine agonists
evoked signicant levels of AIMs. The rst administration of L-dopa
evoked contralateral rotations in all rats. However, rotations in animals
exposed to piribedil and ropinirole peaked earlier and at higher rate
than in animals pre-treated with saline and bromocriptine. Furthermore,
the duration of rotational behavior was longer in animals pre-treated
with ropinirole or piribedil. With daily L-dopa administration, rotational behavior in the saline-treated group increased to the levels seen
in the piribedil and ropinirole treated groups. However, the lower
rotation rate in the bromocriptine pre-treatment group was sustained
throughout L-dopa administration. There was no signicant difference
in the severity of AIMs between any of the pre-treated groups and the
saline control group.
Conclusions: While locomotor sensitivity is altered by the pretreatment with dopamine agonist, the induction and development of
AIMs are unaffected. This suggests that, in PD patients, early stage
treatment with dopamine agonists is unlikely to predispose the patients
to earlier development of dyskinesia.
807
Increased resting-state functional connectivity in de novo, untreated
Parkinsons disease
D. Stoffers, J.L.W. Bosboom, J.-B. Deijen, E.C. Wolters, C.J. Stam,
H.W. Berendse (Amsterdam, Noord-Holland, Netherlands)
Objective: To determine whether changes in resting-state functional
connectivity can be found in the earliest, untreated stages of PD.
Additionally, we will try to nd associations between the level of
synchronisation and measures of motor and cognitive performance.
Background: Mounting evidence suggests that advanced Parkinsons
disease (PD) is associated with pathologically increased synchronisation of oscillatory brain activity. In the present study we used synchronisation likelihood (SL), a non-linear measure of functional connectivity, to determine whether changes in resting-state cortico-cortical
coupling can be found in the earliest, untreated stages of PD and to nd
associations between functional connectivity and clinical measures.
Methods: Whole-head 151-channel MEG recordings were obtained
in an eyes-closed resting-state condition in eighteen de novo, untreated
PD patients and twenty-one age-matched controls. In addition, subjects
underwent extensive motor and neuropsychological testing. Data analysis involved calculation of short as well as long distance SL values in
six frequency domains in the 0.5 to 48Hz range.
Results: In our de novo PD patients, SL was increased posteriorly in
the theta band and more widespread in the low alpha band relative to
controls. Importantly, we found no changes in SL in any of the other
frequency bands. Neuropsychological assessment revealed abnormal

Movement Disorders, Vol. 22, Suppl. 16, 2007

perseveration in de novo patients, which was associated with higher

low alpha interhemispheric SL values between central and parietal
areas. No asscociation of motor performance with SL was found.
Conclusions: Increased cortico-cortical coupling in the lower frequency range (between four to ten Hertz) is an early stage feature of
drug-naive PD patients that may reect disturbed thalamocortical interplay as a direct result of striatal dopaminergic depletion. Some
features of PD-related cognitive dysfunction are associated with higher
interhemispheric connectivity in the low alpha band.
808
Interleukin-10 gene transfection of C17.2 cells improves behavior
in rat model of Parkinsons disease through inhibition of microglia
activation
X.J. Wang, W.G. Liu, Y.H. Zhang, G.Q. Lu, S.D. Chen (Shanghai,
China)
Objective: Currently, to seek effective drugs to inhibit immune
reaction after grafting and improve the therapeutic effect of transplantation has become one of the hot topics in Parkinsons disease (PD)
research. In the present study, we transplanted c17.2 neural stem cells
transfected with Interleukin-10 gene (IL-10-c17.2 NSCs) into the
brains of 6-OHDA-lesioned PD model rats. From 10 to 60 days after
grafting, IL-10 expression was detected in IL-10-c17.2 NSCs in vivo.
Behavioral testing indicated that IL-10 gene transfection improved the
rotational behavior of the rats. Immunohistochemistry analyses revealed that IL-10 gene transfection of C17.2 cells inhibited intracerebral cellular (ED1 and CD8) and humoral (C3 and IgM) immune
responses in the rats. Correlation analyses indicated that the effect of
IL-10 gene transfection of c17.2 cells on rat behavior was signicantly
correlated with its effect on ED1 staining in rat brain, but not with its
effect on CD8, C3 and IgM staining. Furthermore, IL-10 was shown to
inhibit the production of TNF-alpha, NO and superoxide in microgliaenriched cultures. Our results demonstrate the potential application
value of IL-10 in the transplantation treatment of PD and its inhibition
of microglia activation may be a mechanism for IL-10 neuroprotection.
809
Shoulder pain in Parkinsons disease
W.P. Stamey, J. Jankovic (Houston, Texas, USA)
Objective: To assess the frequency of shoulder pain in patients
presenting to a tertiary clinic with Parkinsons disease (PD) or parkinsonism.
Background: Although an under-recognized early symptom, shoulder pain is common in patients with PD and may precede classic motor
symptoms, leading to diagnostic confusion. After extensive evaluation,
invasive procedures, and treatments, many patients referred to our
clinic have related that shoulder discomfort resolved only with pharmacological treatment of PD, suggesting that shoulder pain may be a
PD-related symptom.
Methods: We searched our database of newly encountered patients
with PD during the prior 12-month period for any complaint of shoulder discomfort, dysfunction, or injury, and analyzed their demographic
and clinical data.
Results: PD or parkinsonism was diagnosed in 479 patients, mean
age 65 years (range 41-83, SD 10.2). Though not systematically
queried, 48 patients (10%) (44% women) volunteered shoulder pain as
an important symptom, and 7 (15%) related this was the presenting
complaint of their parkinsonism. Specic diagnoses among the 48
patients were: PD 38 (79%), undetermined 4 (8.3%), dementia
with Lewy bodies 2 (4%), vascular 2 (4%), progressive supranuclear palsy 1 (2%), drug-induced 1 (2%). Side of shoulder pain
and initial motor symptoms agreed in 29 patients (60%) and side of
maximum severity of parkinsonism was highly correlated with side of
pain at 0.48 (p0.0007). An additional 10 patients (21%) reported
bilateral pain. Side of shoulder pain was not specied in 2 patients
(4%). Only 5 individuals (10%) experienced pain in the shoulder
opposite to the side of maximum parkinsonian symptoms.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Conclusions: In our retrospective analysis shoulder pain was present in
10% of patients with parkinsonism. This is most likely an underestimate,
as the complaint of shoulder pain was not specically sought by protocol
at examination and often only included in a case history if volunteered.
Despite these limitations, we believe shoulder pain is a common and
frequently under-recognized or misdiagnosed symptom of early PD. Better
understanding of this important symptom may not only lead to early
diagnosis and prevention of unnecessary interventions, but also provide
insights into the prodromal period of PD. A prospective study in our clinic
is under way to study the true frequency of this condition, and the response
of these patients to anti-parkinsonian medications.
810
Apolipoprotein E polymorphism and Parkinsons disease
G. Akpinar, E. Ergul, I. Kara, H.A. Idrisoglu, K. Bayulkem, A. Sazci
(Kocaeli, Turkey)
Objective: To investigate whether APOE polymorphisms were associated with Parkinsons disease.
Background: Almost a dozen of gene have been identied in connection with Parkinsons disease. Parkinsons disease is one of the
common neurodegenerative disease worldwide. However, idiopathic
Parkinsons disease can not be explained by the genes discovered thus
far. Therefore we wanted to see whether APOE polymorphisms were in
any way associated with Parkinsons disease.
Methods: The study included 233 patients with Parkinsons disease
and 300 healthy controls. The three allelic APOE polymorphism was
assessed with single stage PCR-based method and digested with restriction enzyme HhaI, followed by gel electrophoresis, and silver
staining of polyacrylamide gels. All statistics were performed with the
SPSS v12.0 package. A value of P 0.05 was taken as signicant.
Results: We report that APOE gene polymorphisms were associated
with Parkinsons disease (Chi215.912; df5; P0.007). The frequency of the 2, 3, and 4 alleles was 10.08%, 84.12%, and 5.59%
in cases, and 9%, 79.33%, and 11.67% in controls respectively. The
E2/4 genotype had protection towards Parkinsons disease
(Chi26.244; df1; P0.012; OR0.232; 95%CI0.067-0.804).
Likewise the E3/4 genotype also had protection towards Parkinsons
disease(Chi25.859; df1; P0.016; OR0.521; 95% CI0.3060.889). However the E2/2, E2/3, E3/3, E4/4 genotypes were not associated with Parkinsons disease.
Conclusions: APOE polymorphisms were associated with Parkinsons disease.
811
Prevalence of sporadic Parkinsons disease in Arabic villages in
Israel: A door-to-door study
R. Inzelberg, M. Masarwa, R. Strugatsky, C. Baldwin, L. Farrer,
R. Friedland (Kfar Saba, Israel)
Objective: Estimate the prevalence of Parkinsons disease (PD) in
Arabic villages of Wadi Ara in the population aged 65 years.
Background: The prevalence of PD differs according to population
and methodology. There is limited information about its prevalence in
Arab populations. The G2019S LRRK2 (PARK8) mutation is common
in incident Arabic PD patients in North Africa.
Methods: This door-to-door survey was carried out in the Arabic
villages of Wadi Ara (Northern Israel). Consecutively ascertained
residents aged 65 years, who agreed to participate in the study
underwent neurological examination, motor UPDRS and Minimental
State Examination (MMSE). DNA was extracted from blood leukocytes. The diagnosis of PD was based on standard criteria and that of
dementia on DSM-IV criteria. All subjects were examined by a neurologist. A senior movement disorders specialist reviewed all records
and examined all patients who agreed to come to the Movement
Disorders Clinic. Medical and family history, medications and response
to medications were recorded. All patient DNAs were screened for the
G21019S LRRK2 mutation.
Results: Of the 811 subjects that were approached, 776 accepted to
participate in the study (refusal rate 4.3 %). PD was diagnosed in 11

S247

patients [5 males, mean age (SD): 75 (5)]. Mean disease duration was
7 (5) years. Two patients were newly diagnosed by the study team. Two
patients suffered from PD dementia. One patient reported additional PD
patients in her family [family members suffered from young onset PD
(age at onset 19 37) and carried a 202 A in exon 2 deletion in parkin
gene (PARK2)]. Screening for the G2019S mutation of LRRK2 was
negative for all PD patients. PD prevalence was calculated as 1.60 %
(95 % CI 1.57-1.65).
Conclusions: The prevalence of PD in Arabic villages of Wadi Ara
is similar to that observed in the elderly population in Europe and
Northern America. The G21019S mutation of the LRRK2 gene was not
found in PD patients from this population.
812
Parkinsons disease: A dual hit hypothesis
C.H. Hawkes, K. Del Tredici, H. Braak (Romford, Essex, United
Kingdom)
Objective: To provide a unifying explanation for the earliest pathological and clinical signs of classical Parkinsons disease (PD).
Background: Lewy body disease pathology (Lewy neurites / bodies)
in classical PD probably commences simultaneously in the medullary
vagal motor nucleus and olfactory bulb and then advances rostrally
through the brainstem. We suggest a common pathological mechanism
to explain how such anatomically separate structures become involved
early in PD.
Methods: Our own data and the literature was reviewed for data
suggesting premotor involvement of the olfactory and vagal motor
system.
Results: Olfactory disorder. There is extensive evidence for olfactory
involvement whether measured by psychophysical, neurophysiological
or pathoanatomical methods (Braak et al 2003). Prospective studies
from Hawaii and Holland imply that olfaction is damaged well before
motor symptom onset. Vagal disorder. The vagus is signicantly impaired, as shown by heart rate variability, dysphagia or gastric dysfunction and medullary pathology. Prospective assessment of bowel
habit indicates that constipation is a premotor sign. Lewy body pathology occurs early in the gastric enteric plexus (Braak et al 2006) where
vagal motor bres terminate.
Conclusions: We propose that a pathogen, possibly viral, damages the
nasal olfactory neuroepithelium, causing smell impairment, and is then
swallowed in secretions. After entering the gastric enteric plexus, it travels
retrogradely along vagal motor bres to the medulla. Subsequent rostral
progression to the substantia nigra results in classical PD features.
813
Increase in vitamins A,C and E in Parkinsons disease following
pramipexole treatment
Y. Iwasaki, K. Ikeda, O. Kano (Tokyo, Japan)
Objective: To determine the level of vitamins A,C and E both before
and after treatment with pramipexole.
Background: Oxidative stress plays an important role of cell death in
Parkinsons disease(PD). Vitamins A,C,and E are an important antioxidants and deciency of these agents has been implicated in the
mechanism of cell death. Pramipexole has been shown to reduce
oxidative stress and be neuroprotective in cell and animal models of
neurodegeneration.
Methods: We studied 30 PD patients to determine the levels of
vitamins A,C,and E both before and after treatment of pramipexole.
We found pramipexole treatment up to 1mg/day was well tolerated.
Results: Vitamins A,C,and E were found increased following treatment with pramipexole. Correlation coefcients in PD group showed
no signicant relationship among variables age,sex,duration and severity of PD.
Conclusions: PD patients show increases in oxdative stress,were
found to be increases in PD patients by pramipexole treatment, suggesting that pramipexole may interrupt oxidative stress in PD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S248 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

814
Speech dysfunction in drug-naive patients with early Parkinsons
disease and its response to dopaminergic therapy
H.-W. Shin, H. Kim, S.Y. Kang, Y.H. Sohn (Seoul, Korea)
Objective: To investigate speech dysfunction in drug-naive patients
with early Parkinsons disease (PD), and its response to dopaminergic
therapy.
Background: Speech dysfunction is an important disabling symptom
of PD, which includes dysphonia with hoarseness, reduced loudness,
monotonous voice, and articulatory disturbance with stuttering or slow
speech. Although these disturbances are frequently observed in PD
patients (70-89%), the origin of each disturbance, i. e., either from
disease itself or from the effect of antiparkinson medications, is not
clearly understood yet. Thus we evaluated speech dysfunction selectively in drug-naive patients with PD, and its response to dopamine
replacement therapy.
Methods: Subjects were drug-naive patients with PD and agematched healthy controls. Their speech function was assessed using a
prolonged vowel phonation /a/, reading a standardized passage in a
natural manner, and a repetition of the syllable /p/, /t/, /k/, and
/ptk/ as rapidly and regularly as possible in a single breath. In
patients, these assessments were repeated after 3-months levodopa
therapy.
Results: PD patients showed shorter maximum phonation time and
more limited intensity range than normal controls. Speech intelligibility
and the rate of repetition with the syllables were signicantly reduced
in PD patients, compared with controls. Maximum phonation time was
signicantly improved after levodopa treatment, but other dysfunctions
were unchanged.
Conclusions: Various measures of speech function are impaired in
drug-naive PD. However, most of these disturbances except for maximum
phonation time may be unrelated to dopaminergic deciency in PD.
815
Hyponatremia and rhabdomyolysis induced by pramipexole during the treatment of Parkinsons disease
M.F. Oztekin, N.S. Oztekin, B. Acar, S. Gencler (Ankara, Turkey)
Objective: The aim of the study is to emphasize the importance of
monitoring serum sodium and ADH levels especially during dose
adjustment periods in patients treated with pramipexole.
Background: Pramipexole is intruduced as a non-ergot dopamine
D2/D3 receptor agonist for treating Parkinsons disease as an add on
drug in advanced parkinsonian patients with motor uctuations.
Methods: The rst patient, a 76 year old woman with Parkinsons
disease, who was using newly administered pramipexole 3 mg/day
along with levodopa was admitted with somnolance and excessive
tremor. Laboratory tests showed severe hyponatoremia Blood concentration of ADH measured at the onset was higher(38.5 pg/ml)
than normal (0.3-3.5 pg/ml under normal osmolarity.Under the
suspicion of SIADH due to pramipexole, the drug was discontinued
and as a result, her consciousness level improved rapidly together
with a prompt reduction in ADH level (9.2 pg/ml) The second
patient, a 63 year old woman with Parkinsons disease was admitted
to the emergency ward with delirium, high fever. She was using
levodopa and pramipexole 4.5 mg/ day.After the dose escaleted to
4.5 mg/d she she had been found lying on the oor, in a state of
mental confusion,She developed rhabdomyolysis (CPK 3206 U/L
with MB isoenzyme 3.5 ng/ml, LDH 610 U/L, GOT 78 U/L),
accompanied by mild leukocytosis (WBC 13000/mm3) and hyponatremia(Na:107(N:135-145 mmol/L). Blood concentration of
ADH measured at the onset was higher(32.5 pg/ml) than After the
cessation of pramipexole her consciousness level improved rapidly
together with a reduction in ADH level (7.1 pg/ml).
Results: First patient developed hyponatremia due to inapproprate
secretion of antidiuretic hormone (ADH)syndrome in the dose escalating phase of pramipexole treatment.The second patient also
developed hyponatremia and profound rhabdomyolysis following
dose increase.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Conclusions: In conclusion, these two cases, along with others

previously reported, show the development of acute episodes of
hyponatremia and rhabdomyolysis in Parkinsons disease may
somehow related to the treatment of the basic disease with dopaminergic drugs. Since pramipexole is classied as a dopaminergic
receptor agonist, these cases may provide new insight into a link
between ADH and the dopaminergic receptor in the central nervous
system.
816
Pathological gambling secondary to dopaminergic therapy in Parkinsons disease
P. Katschnig, P. Schwingenschuh, R. Saurugg, K. Wenzel,
K. Petrovic, E. Ott (Graz, Austria)
Objective: To report two cases of pathological gambling secondary
to dopaminergic therapy in Parkinsons disease.
Background: A relationship between impulse control disorders,
especially pathological gambling, and dopaminergic treatment in
Parkinsons disease (PD) has been reported in several recent
studies.
Methods: We present two patients treated in our movement disorders clinic who developed pathological gambling under a dopamine agonist monotherapy and a combination treatment with both a
dopamine agonist and levodopa. The rst patient is a 65-year-old
man with a 4-year history of PD. When he rst presented to our
outpatient clinic, he received levodopa 400 mg daily. After a worsening of motor symptoms, we initiated an add-on therapy with
ropinirole which was well tolerated up to 18 mg per day. While
further increasing the ropinirole dose to 24 mg per day, pathological
slot machine gambling developed leading to a massive nancial
burden for the patient. Before taking 24 mg of ropinirole per day,
the patient had played cards weekly but never used to play slot
machines. After stopping ropinirole, the pathological gambling
disappeared. The second patient is a 47-year-old man who had
suffered from PD for 3 years before coming to our clinic. The initial
treatment consisted of 3 mg pramipexole per day, later levodopa
was added. While initiating dopaminergic treatment with
pramipexole, the patient, who had previously only played slot
machines approximately twice a year, developed pathological gambling. Because of his young age, we tried an alternative dopamine
agonist and switched from pramipexole to ropinirole which unfortunately did not change the patients urge to play. Recently, we
stopped ropinirole and increased levodopa to prevent a worsening of
motor symptoms. Following this change in treatment, the patients
urge to play vanished.
Results: Our experience underlines the observation that dopaminergic treatment with dopamine agonists or a combined therapy of a
dopamine agonist with levodopa may cause pathological gambling or
may enhance the probability of suffering from pathological gambling in
predisposed individuals.
Conclusions: Therefore, it seems important to have impulse control
disorders in mind when taking the patients history and initiating
dopaminergic treatment.
817
Experiences using Japanese translation of wearing-off questionnaire (19 symptoms)
Y. Kajimoto, I. Nakanishi, T. Kondo (Wakayama City, Japan)
Objective: In this study, we compare the sensitivity of information
obtained using a patient questionnaire with that of information gathered
by clinicians at a routine clinical visit in recognizing the wearing-off
(WO) phenomenon in Parkinsons disease (PD).
Background: Patients with PD often show WO phenomenon within
2 years of treatment with antiparkinsonian medication. Recently, a
19-item WO questionnaire (WOQ-19) that is highly sensitive for identifying patients with WO phenomenon for routine clinical use has been
reported (Stacy, 2006). In this study, we used a Japanese translation of
WOQ-19 (JWOQ-19) for assessing PD patients in our hospital.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Methods: The subjects were idiopathic PD patients who were over
30 years of age with illness durations of less than 5 years or more than
5 years. The patients were asked to complete JWOQ-19, Parkinsons
Disease Questionnaire-39 (PDQ-39), and the Self-rating Depression
Scale (SDS), after a routine clinical examination by a neurologist.
Clinical ndings were assessed along with disease stage according to
the Hoehn-Yahr staging, and the Unied Parkinsons Disease Rating
Scale (UPDRS).
Results: The evaluation of WO phenomenon using JWOQ-19 was
more sensitive than those based on a clinicians impression and
using UPDRS Question 36 in both the 5 yr and 5 yr patient
groups. The most common motor WO symptom was tremor, and
it was not related to disease duration. The frequently detectable
nonmotor symptoms were numbness, anxiety, mood change
and cloudy mind/dullness thinking even in the early stage of the
illness. In the evaluation of ADL using PDQ-39, regarding body
discomfort, the score of patients with WO phenomenon was signicantly higher than that without WO phenomenon in the 5 yr
and 5 yr patient groups (p 0.05), and regarding social support,
the score of patients with WO phenomenon was signicantly higher
than that without WO phenomenon in only the 5 yr patient group
(p 0.05). There was no signicant difference in SDS score between the patients with and without WO phenomenon determined
using JWOQ-19.
Conclusions: JWOQ-19 is a useful screening tool for WO phenomenon in PD patients, by determining either motor or nonmotor symptoms.
818
N-acetyltransferase 2 polymorphism and risk factors in early onset
Parkinsons disease
G.A. Klodowska-Duda, J. Samelska, B. Jasinska-Myga, M. Bialecka,
K. Safranow, U. Mazurek, G. Opala (Katowice, Poland)
Objective: The aim of the study was the evaluation of the relationship between NAT2 polymorphism and Parkinsons disease onset,
exposure to environmental risk factors and clinical evaluation.
Background: The etiology of Parkinsons disease remains unknown.
N-acetyltransferase 2 (NAT2) is an enzyme which metabolizes substances suspected to be related to Parkinsons disease. The role of
NAT2 in pathogenesis of Parkinson disease is controversial, because
results of the studies are inconsistent.
Methods: 202 subjects were examined 102 patients (62 men) with
idiopathic Parkinsons disease (PD) group 36-82 years old and 100
subjects (60 men) of the control group (K) 36-83 years old matched to
Parkinson s disease patients for age, sex and exposure to specic risk
factors (pesticides, hydrocarbons, heavy metals, smoking, drinking
well-water, toxic effects related to hydrocarbons exposure). Parkinsons disease patients were divided into two subgroups: with early
onset disease before 50 PD1 (n52) and after 50 PD2 (n50). The
control group was divided respectively into patients for two subgroups:
K1 and K2 with 50 subjects in each.Genetic research was conducted
based on the PCR/RFLP method.
Results: Slow acetylation genotype (SA) was observed in 59,9% of
the PD patients and 64% of the control group. Frequency of fast
acetylator genotype (FA) was statistically higher in the PD1 subgroup
compared to the K1 subgroup (50% vs. 24%, p0,008). The FA
genotype was the independent risk factor of early onset of Parkinsons
disease (OR 3,76, 95%CI 1.47-9.65; p0,0052). There was no interaction between acetylation status and Parkinsons disease risk factors
in the examined groups (PD & K, PD1 & K1, PD2 & K2) and the
relationship within clinical evaluation.
Conclusions: Fast acetylator genotype may be a risk factor of early
onset Parkinsons disease. There is no connection between acetylation
genotype, examined exposures and onset of disease and the relationship
between acetylation genotype and clinical evaluation.

S249

819
Better sexual function in women with Parkinsons disease: A case
control study
C. Akbostanci, B.S. Arica, N. Eryigit, Z. Ayturk (Ankara, Turkey)
Objective: The purpose of this study was to investigate the frequency
of increased libido probably induced by antiparkinson medications, and
to investigate the frequency of sexual dysfunction in Parkinsons disease (PD).
Background: There are several case reports and a few case series
about hypersexuality in PD, but the subject has never been investigated
in a case control manner.
Methods: There were 51 (25 women) cases with PD, and 50 (25
women) control cases. Control cases did not have any disease nor on
any drugs that could interfere with sexual dysfunction, and were similar
to PD group in terms of age, gender, and partnership. We investigated
sexual dysfunction using the related items from the questionnaire
SCOPA-AUT (items 22 (erection), 23 (ejaculation), 23a(drugs used for
impotence) for men; 24(vaginal lubrication) and 25 (ability to reach
orgasm for women). We added two more questions about libido: Do
you experience increased sexual desire after you begin taking antiparkinson medication? and Does your sexual desire decreased after the
diagnosis of Parkinsons disease?. For the control group the added
questions were: Did you experience increased sexual desire in the last
two years? and Did your sexual desire decrease in the last two
years?. Hoehn Yahr stages, types and doses of antiparkinsonian medication were also included in the analyses.
Results: There was no difference between PD and control cases in
terms of increased libido, decreased libido, sexual functions of erection,
ejaculation, and vaginal lubrication. Doses and types of antiparkinsonian drugs were similar when patients with and without increased
libido were compared. The only difference we could nd in this study
is that the women with PD had much less difculty reaching an orgasm.
While 90% of women from the control group had difculty reaching an
orgasm, the ratio was 37.5% among women with PD (p0,043).
Conclusions: To the best of authors knowledge this is the rst study
to show better sexual function in women with PD, when compared with
age-matched controls.
820
The neuronal activity of putamen in patients with Parkinsons
disease before treatment
K. Isonishi, F. Moriwaka, S. Kaneko, T. Kashiwaba (Sapporo,
Japan)
Objective: To clarify the neuronal activity of posterior half of putamen in patients with Parkinsons disease(PD) before medication.
Methods: From January 1999 to December 2006, 42 patients with
Parkinsons disease (PD) (25 women and 17 men, mean age 69 year,
Hoehn-Yahr(H-Y) stage 12.5, duration from onset to 1H-MRS examination 1.48 year) were investigated. All patients were rst visitors
looking for diagnosis and no patients had medication before. Based on
clinical manifestations more affected and less affected putamen was
postulated. Proton magnetic resonance spectroscopy(1H-MRS) was
performed at 1.5T and spectrum was collected from an 8 cm3 voxel in
posterior half of putamen. Assigned and quantied spectra were calculated to the ratio NAA/Cr. The ratio obtained from both more
affected side and less affected side was compared. The difference
between both sides was analyzed by Students t-test.
Results: NAA/Cr ratio in both side tend to decrease with progression
of H-Y stage. Patients can be classied into 3 groups, that is, NAA/Cr
ratio in more affected side is smaller than that of less affected side(group A, n17), the ratio in more affected side is larger than that of
less affected side(group B, n17) and the ratio is same between both
sides(group C, n8). In group A, more affected side NAA/Cr was
1.72}0.27 and less affected side was 1.91}0.36. In group B, more
affected side NAA/Cr was 2.11}0.34 and less affected side NAA/Cr
was 1.68}0.28. In group C, more affected side NAA/Cr was
1.77}0.24 and less affected side NAA/Cr was 1.74}0.24. Not only
more affected side NAA/Cr between group A and B but also less

Movement Disorders, Vol. 22, Suppl. 16, 2007

S250 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

affected side NAA/Cr between both group were signicantly different.
And more affected side NAA/Cr in group B was signicantly larger
than that of group C.
Conclusions: Results were summarized as follows, (1) there were
three patterns of more affected putamen in PD based on NAA/Cr
ratio, (2) there was signicant increase of NAA/Cr in less affected
putamen(groupA) and in more affected putamen(groupB). NAA/Cr
is not necessarily specic for dopaminergic neurons, however, the
presence of increased NAA/Cr is suggestive that neurons in posterior half of putamen including dopaminergic neurons are still active.
Therefore it may be expected a neuroprotection in early stage of PD
patients.
821
Pain patients in Parkinsons disease
S.O. Machnev, O.S. Levin (Moscow, Russian Federation)
Objective: Pain dysfunction is the important part of Parkinsons
diseases (PD) clinical presentation.
Background: Pain is estimated to occur in approximately 50% of
patients. In some patients, it can be more debilitating than the motor
decits. However, the mechanisms underlying such painful symptoms,
structure of pain syndrome and treatment approaches are badly investigated.
Methods: A total of 68 subjects with PD participated in this study,
(mean age 58,5/- 7,3 years, mean duration of disease 4,3/- 2,6
years, stage of disease according Hoehn -Yahr 2 and 3). To estimate
symptoms of Parkinsonism, we used Unied Parkinsons Disease
Rating Scale (UPDRS, Fahn, 1987), to estimate painful sensation visual analogue scale. Also we used McGills pain questioner and
specially developed scale to estimate pain in patients with PD. The
degree of depressive syndrome was estimated using Becks scale
(Beck, 1996).
Results: Forty two (61%) had pain dysfunction. Most of patients
suffered from low back pain and shoulder girdles pain. Less frequently
patients experienced hip, buttock, shin, foot and neck pain. 70% of
patients had vertebral pain, 18, 2% of patients had radicular pain. 30%
of patients with PD have ill-dened pain sensation in limbs, which they
described as general sense of tension and discomfort. The intensity of
pain ranged during the day and depended on phases of levodopa action.
64% of patients had more signicant pain dysfunction in limbs on the
side the onset of PD. The study revealed reduction of pain threshold in
patients with PD mainly of the more affected side. The result of this
study showed correlation between the degree of pain dysfunction and
depressive syndrome(p0.05). Signicant hypokinesia, limb rigidity,
motor uctuation, affective disorders and degenerative changes in
spinal cord are the important factors which inuence on the presence of
pain dysfunction.
Conclusions: Pain dysfunction in PD is the composite problem and
depends on many factors. High frequency of vertebral pain syndrome
can be explained by the presence of postural disorders, from the other
hand, the development of pain dysfunction can be related with depression and nociceptive and antinococeptive systems dysfunction, in consequence of dopaminergic decit.
822
Smell identication in Parkinson and Alzheimer patients: Are
Tabert et als* odours appropriate for PD?
H. Thagesen, L. Korbo, M. Baunsgaard, P. Moeller (Roskilde,
Denmark)
Objective: To develop a new and cheap smell identication test for
broad categories of neurodegenerative diseases. We tested a set of
universally well-known odours to allow comparison between results
obtained in hospitals located in different cultures. We also wanted to
measure if patients make more identication errors if they have to
choose between names belonging to the same odour category rather
than names belonging to different categories, and if it is harder to
decide that the name of a perceived odour is not represented among the
names provided. Finally, we investigated if the 10 odours used by

Movement Disorders, Vol. 22, Suppl. 16, 2007

Tabert et al* are optimal also for use in other neurodegenerative

diseases than Alzheimers disease (AD).
Methods: Thirty patients with mild AD (18 women), 35 PD patients
(19 women) and an age-matched control group of 42 subjects (23
women) participated. Each subject was required to perform 24 odour
identications and for each odour had to choose between 5 answers:
one of 4 given names or the reply, none of the names provided. Four
of the odours were not represented by their names on the 24 answering
sheets. For the remaining 20 odours, 10 answering sheets consisted of
4 odour names (plus the none of the names provided answer) referring to 4 different odour categories (f.ex, vanilla, petrol, onion, grape
fruit) and the other 10 sheets had names drawn from 2 different
categories, with 2 names from each (f.ex, lemon and orange from one
category and vanilla and cinnamon from the other). Ten of our odours
and the associated names were identical to those used by Tabert et al.
The other 10 were well-known odours.
Results: PD patients scored fewer correct answers than the control
group (t(53.2)6.829, p0.000). In the control group, women scored
higher than men (t(24.4)3.044, p0.006) whereas this was not the
case in the PD patient group. For none of the groups did we nd any
signicant difference between behaviour observed with the odours used
by Tabert et al, and the other 10 odours we tested. More detailed
analyses and results from the AD group will be presented at the
meeting. Ref: * Tabert M.H. et al:A 10-item smell identication scale
related to risk for Alzheimers disease. Annals of Neurology vol. 58,
155-160 July 2005.
823
Dietary factors in Korean patients with Parkinsons disease
T.-B. Ahn, J.Y. Lee, B.S. Jeon, J.-W. Cho, K.H. Seo, M.R. Cho,
R.W. Choue (Seoul, Republic of Korea)
Objective: To investigate the diet factors associated with Parkinsons
disease.
Background: Both genetic and environmental factors play important
roles in the pathogenesis of Parkinsons disease (PD). Although smoking signicantly lowers the risk of PD, the contribution of many
environmental factors remains unproven. In this study, we investigate
the risk associated with diet, one of the commonest environmental
factors, in a case-control study.
Methods: We recruited PD patients who were diagnosed by UK
Brain Bank criteria. Age- and sex-matched controls were those who
admitted to the department of orthopedic surgery and had no history of
neurologic illness. Data were gathered with a structured interview and
a self-administered food-frequency questionnaire (FFQ).
Results: Ninety seven PD patients (Male23, Female74) and 66
controls (Male14, Female52) were recruited. The mean age of PD
patients were patients who completed the FFQ was 64.58.7 years, and
of controls, 64.49.6 years. Mean disease duration was 2.31.6 years.
Food group analysis showed that PD patients less frequently ate pulse,
vegetables, fruits, oil than controls. More specically, PD patients
consumed less boiled rice with cereals, soybean, shellsh, plant oil,
yogurt, and all kinds of fruits and vegetables. Further analysis shows no
difference in the risk factors such as smoking habit, rural living, and
well water drinking.
Conclusions: Our study suggests the potential role of fruits, vegetable protein and vegetable oil in lowering the risk of PD, which was
inconspicuous in the western studies. As Korean traditional menu
mainly consists of foods derived from plant, our results are greatly
different from those of the western studies.
824
Alpha-synuclein-overexpressing neurosphere as an in vitro model
of alpha-synucleinopathies
M. Fukuda-Tani, T. Yasuda, H. Mochizuki, Y. Mizuno (Tokyo,
Japan)
Objective: The purpose of this paper is the elucidation of the function of accumulated alpha-synuclein in the pathogenesis of alphasynucleinopathies.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Background: An abnormal accumulation of alpha-synuclein in neurons and glial cells has been noted in the brains of patients with
alpha-synucleinopathy, including Parkinsons disease, dementia with
lewy body, and multiple systemic atrophy. Besides these pathological
ndings, a little is known how these abnormally accumulated alphasynuclein is involved the molecular pathogenesis of these disorders.
Methods: We isolated the neurosphere-forming cells (NSFCs) from
the subventricular zone (SVZ) of adult mice. Then we established the
alpha-synuclein-overexpressing NSFCs by using the high-titer recombinant retroviral vector encapsulated with the vesicular somatitis virus
G protein (VSV-G).
Results: We could successfully isolate the NSFCs from adult mouse
SVZ, which had abilities for self-renewal and differentiation into
various neuronal and glial cell populations. Additionally we established
the NSFCs which overexpress alpha-synuclein (wild-type or A53Tsubstituted) by using the pseudotyped retroviral vector.
Conclusions: The NSFCs overexpressing alpha-synuclein can be a
valuable in vitro tool for the examination of functions of alphasynuclein in neurons, astrocytes, and oligodendrocytes.
825
Direct and indirect costs in Parkinsons disease: A patient survey
C. Peralta, A. Calle, S. Garcia, G. Gomez-Arevalo, G. Mizraji,
O.S. Gershanik (Buenos Aires, Argentina)
Objective: 1) To assess the basic health care system coverage of
Parkinsons Disease (PD) patients in Argentina. 2) To address the
impact of direct and indirect costs of PD into the monthly patients
household economy.
Background: Parkinsons disease is a chronic condition which poses
a high amount of direct and indirect costs to the health care system and
to the society. However, few data regarding the impact of such costs
into the patients household economy is available.
Methods: Consecutive PD patients attending the Movement Disorders outpatient clinic were asked to answer a questionnaire designed to
that effect.
Results: We present the results of the rst 20 patients that completed
the questionnaire in an ongoing study. There were 4 women and 16
men, mean disease duration 8 years. All patients had social security, 5
(25%) had obtained a disability certicate and only 4 (20%) had taken
advantage of the 70% drug discount sanctioned by the Argentinean law
for chronic diseases. 3 patients (15%) continued working after 8 years
PD duration, while after 6 years they rst modied their usual working
routine and their income was reduced by 17% on average. Most of the
patients modied or lost their jobs due to PD motor difculties (8
patients, 40%), and only 2 (10%) due to non-motor features such as
depression or attentional difculties. The majority of the monthly
household income was destined to cover drug-related costs and payment of private or public social security (29% and 20%). Lesser
amounts were assigned to cover transportation services (7%), rehabilitation (2.3%), or payment to neurology consultants (0.7%). There were
no costs related to hospital or emergency room attendance as well as to
ambulatory or admitted procedures.
Conclusions: In our survey, PD patients rst had to modify their
working routine after 6 years of disease duration. Direct costs seemed
to be the most relevant burden to patients household income representing almost a 60% of their monthly budget.
826
The familial Parkinsonism gene LRRK2 regulates neurite process
morphology
D. MacLeod, K. Inoue, A. Abeliovich (New York, New York, USA)
Objective: Mutations in LRRK2 underlie an autosomal-dominant,
inherited form of Parkinsons disease (PD) that mimics the clinical
features of the common sporadic form of PD. The LRRK2 protein
includes putative GTPase, protein kinase, WD40 repeat, and leucinerich repeat (LRR) domains of unknown function. Here we show that
PD-associated LRRK2 mutations display disinhibited kinase activity

S251

and induce a progressive reduction in neurite length and branching both

in primary neuronal cultures and in the intact rodent CNS.
Background: Recently, autosomal-dominant mutations in leucinerich repeat kinase-2 (LRRK2; also PARK8, dardarin, OMIM 609007)
were described in a familial Parkinsonism syndrome that mimics the
clinical and pathological features of the common, sporadic form of PD.
Methods: Primary cortical cultures were transfected with plasmid
vectors encoding wild-type or mutant forms of V5 epitope-tagged
LRRK2 (or vector control), along with enhanced green uorescent
protein (eGFP) sequences to allow for the identication of transfected
cells (5% of neurons; data not shown).
Results: Overexpression of either of two clinically associated missense mutant forms of LRRK2 within the kinase domain, G2019S and
I2020T, led to a dramatic reduction in neurite length and branching
evident with respect to both the longest neuronal processes, corresponding to axons, as well as total neurite outgrowth. This was conrmed by antibody staining for axonal and dendritic markers. Neuronal
polarity, as quantied by the ratio of axons to dendrites, appeared
unaltered.
Conclusions: Our data suggest a role for LRRK2 in the maintenance
of neuronal process length and complexity in the mammalian brain.
Autosomal-dominant mutations in LRRK2 lead to disinhibited kinase
activity, a reduction in neuron process length and complexity, and the
accumulation of tau-positive inclusions with lysosomal characteristics.
Ultimately, neurons that overexpress mutant alleles of LRRK2 undergo
apoptosis. Suppression of LRRK2 with shRNAs or a dominant inhibitory allele leads to an opposite phenotype of increased neurite process
length and complexity.
827
Urinary dysfunction in Parkinsons disease
H. Blackett, R. Walker, B. Wood (Ashington, Northumberland,
United Kingdom)
Objective: To evaluate the prevalence of urinary symptoms in patients with idiopathic Parkinsons disease (PD) attending PD Northumbria and to evaluate whether urinary dysfunction is related to disease
stage or duration.
Background: Urinary dysfunction is common in PD and has been
identied as one of the key non-motor features that may adversely
affect quality of life. Previous studies, published prior to the development of rigorous diagnostic criteria for PD, may have overestimated its
prevalence through the inclusion of patients with other forms of parkinsonism. There is conicting evidence for the association of urinary
dysfunction with disease stage or duration.
Methods: Consecutive review patients attending our PD clinics between September 2006 and February 2007 with a diagnosis of idiopathic PD according to the UKPDS Brain Bank Criteria were invited to
participate. Thirty-two consenting subjects completed the International
Prostate Symptom Score (IPSS). Although originally an index of prostate disease, the IPSS has been used extensively to quantify urinary
dysfunction in both men and women. Disease severity was measured by
the Modied Hoehn and Yahr stage.
Results: There were 22 male and 10 female subjects. The mean age
was 69.6 years (SD 8.3) and the median disease duration 3 years (0-13
years). Median Modied Hoehn and Yahr was 2.0 (1.0-5.0). 19
(59.4%) patients had IPSS scores consistent with moderate or severe
bladder symptoms. The most common urinary symptoms reported were
nocturia (87.5%), urgency (71.9%) and urinary frequency (71.9%).
There was a weak correlation between Modied Hoehn and Yahr and
IPSS, but no correlation between IPSS and disease duration.
Conclusions: Urinary symptoms are common in a representative
population of individuals with PD. While there is some association with
disease severity, as measured by the Modied Hoehn and Yahr stage,
there is no association between bladder symptoms and duration of PD.
Further research is required to quantify these results and investigate
associations of urinary dysfunction in PD.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S252 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

828
Results of rotigotine transdermal patch in advanced Parkinsons
patients with motor uctuations and in combination with levodopa:
Results of the CLEOPATRA-PD trial
W. Poewe, W.H. Oertel, E. Martignoni, E. Tolosa, N.P. Quinn,
B. Boroojerdi, M. Rupp (Innsbruck, Austria)
Objective: The aim of this CLEOPATRA-PD trial was to investigate
the efcacy and safety of this compound in combination with levodopa
in patients with advanced PD compared to placebo and an active
comparator (pramipexole).
Background: Neupro (Rotigotine transdermal patch) is a broadspectrum D3/D2/D1 dopamine receptor agonist, which is approved in
the EU for treatment of idiopathic Parkinsons disease (PD). It is
applied once-daily and delivers stable plasma levels over 24 hours.
Methods: PD patients inadequately controlled with levodopa were
randomized to placebo, pramipexole (up to 4.5 mg/day), or rotigotine (up to 16mg/24 h). This trial had a 4 weeks pre-treatment phase,
an up to 7 week titration phase and a 16 weeks maintenance phase.
Primary endpoint was the reduction in OFF time (hours) based on
the patient diaries. Secondary parameters included UPDRS, status
after wake up and the Quality-of-Life questionnaire PDQ-39.
Results: Of the 506 subjects randomized (101 placebo, 204
rotigotine, 201 pramipexole) 85% (430) completed the trial. Compared to baseline, mean daily decreases in off time were 2.46
hours (rotigotine), 2.81 hours (pramipexole) and 0.88 hours (placebo, p0.0001 for the rotigotine group compared to placebo,
ANCOVA). The difference between the active treatment groups was
not signicant (p0.19). The following parameters improved signicantly compared to placebo in both treatment groups: UPDRS II
and III scores, OFF status at wake up, ON without troublesome
dyskinesia status as well as the total score for the PDQ-39. There
were no signicant differences between the pramipexole group and
the rotigotine group. Application site reactions, nausea, and vomiting were the most common adverse events. The incidence of psychiatric adverse events was similar between rotigotine and placebo
and higher with pramipexole. The frequency of adverse events
leading to trial discontinuation was similar between all groups
(5.4%-7%).
Conclusions: The data from this comparative trial of transdermal
rotigotine up to a dose of 16mg/24 h and pramipexole up to 4.5mg/day
demonstrate a similar efcacy and safety prole.
829
Screening for Parkinsons disease in underserved communities in
Alachua County, Florida
R.L. Stephen, M.S. Okun, C.W. Garvan, C.E. Jacobson,
R.L. Rodriguez, H.H. Fernandez (Ganesville, Florida, USA)
Objective: To institute a program for educating and screening for
Parkinson disease (PD) in underserved communities.
Background: It is unknown how many people living in rural and
underserved areas may have PD. Through a community outreach grant
from the National Parkinson Foundation, this program was designed to
educate and screen for PD in underserved communities.
Methods: A standardized oral presentation on PD was presented
to multiple and diverse underserved community groups in Alachua
County, Florida. The presentation described the signs and symptoms
of PD, explained the impact on patients and families, and outlined
currently available treatment, community resources, and other opportunities for care. A PD screening questionnaire (Kam 2000) was
distributed to all attendees and completed (as a group) in a standardized manner.
Results: 199 participants over 16 sites were surveyed. Subject characteristics included: 47.2% Caucasian; 38.2% African American,
11.1% Asian, .05% Hawaiian. One-hundred and thirteen (56.8%) were
identied by the screening questionnaire as possible PD patients;
53(26.6%) as probably not PD, 10(5%) as probably PD, 10(5%) were
scored as unclear and 13(6.5%) of surveys were incomplete (survey
questions unanswered). Results were further quantied based on in-

Movement Disorders, Vol. 22, Suppl. 16, 2007

come level and revealed the largest group of possible/probable PD had

an income level of less than $25,000/year (26.1% of respondents). This
was compared to 8.5% of the participants in the income range of
$40,000-75,000/year. African-Americans screened similar to Caucasians, potentially revealing a similar prevalence of PD, although the
INFORM PD database reveals that the University of Florida Movement
Disorders Centers PD clinic population to be 92.6% Caucasians and
2.5% African-American.
Conclusions: This program is a potential model for education and
screening of PD populations in underserved communities. More research will be required to determine the impact of this program, and to
follow-up in care delivery for possible/probable PD cases. We suspect
that there are large numbers of both Caucasians and African Americans
in Alachua County with undiagnosed PD, and that many are in low
income groups.

PARKINSONISM-OTHER
830
In vivo magnetic resonance imaging, sensorimotor behavioral and
pathological phenotyping of aged PLP-SYN transgenic mouse
model of multiple system atrophy
P.-O. Fernagut, M. Biran, A. Vital, G. Raffard, J.-M. Franconi,
K.G. Petry, P.J. Kahle, F. Tison (Bordeaux, France)
Objective: To determine if oligodendroglial alpha-synuclein expression can lead to age-dependent motor disorder and neuropathology.
Background: Multiple system atrophy (MSA) is a sporadic neurodegenerative disorder mainly occurring after 60 years and characterized by a combination of parkinsonian, cerebellar, and autonomic symptoms due to a degenerative process affecting the
striatonigral, olivo-ponto-cerebellar and autonomic systems. Widespread accumulation of glial cytoplasmic inclusions (GCIs) containing alpha-synuclein represents the cytopathological hallmark of
MSA and is recapitulated in mice expressing human wild-type
alpha-synuclein in oligodendrocytes under the control of the proteolipid promoter (PLP-SYN, Kahle et al., 2002, EMBO;3(6):583-88).
To date, the phenotype of these mice was mainly characterized in
mice aged 12 months.
Methods: Sensorimotor behavioral assessment, 9.4 T magnetic
resonance imaging (MRI) and spectroscopy were used to evaluate
PLP-SYN mice and controls aged 12, 18, and 24 months.
Results: Although PLP-SYN mice displayed normal locomotor, exploratory behavior and rotarod performances, they were impaired when
challenged to complete a more difcult task (traversing a narrow beam)
and displayed a signicantly increased number of errors compared to
control mice (3.7 0.4 vs 1.2 0.2, p0.001). MRI revealed that total
brain volume was signicantly smaller in PLP-SYN mice (448.9
7.48 mm3 vs 507.0 3.9 mm3, -11.45%, p0.0001) while ventricular
volume was not different from controls, suggesting mild brain atrophy.
After correction for brain volume differences between groups, there
was a tendency toward increased ventricular volume and decreased
cerebellar volume, however no specic pattern of atrophy emerged.
Histopathologically, alpha-synuclein positive oligodendroglial inclusions had an uneven distribution in the brain with the following pattern:
cortexcerebellumponshippocampus. Sparse inclusions were
found in the striatonigral system.
Conclusions: Taken together, these data suggest that PLP-SYN
transgenic mice partly recapitulate the disease and its progression.
831
The risus sardonicus: A warning sign of multiple system atrophy
Y. Pamblanco-Bataller, E. Lopez-Valdes, S. Fanjul, C. Cemillan,
J. Domingo (Leganes, Madrid, Spain)
Objective: Reporting a case of orofacial dystonia remembering a
risus sardonicus in a levodopa naive patient, at an early stage of a
probable multiple system atrophy.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Methods: A 60-year-old man presented with severe dystonia, triggered by speaking of orofacial, platisma and pectoral musculature,
resulting in a facial distortion mimicking the risus sardonicus of cephalic tetanus. He also suffered dystonic dysarthria and dyspnea without laringeal dystonia (video). At this time there were no other abnormalities in the neurological examination. There was no history of drugs
intake. Six months later appeared an asymmetrical brady-kinetic rigid
syndrome and one year later a gait ataxia. He also reported urinary
frequency, urgency and nocturnal incontinence. Levodopa was started,
but as the dystonia worsened it was stopped.
Results: Laboratory investigation excluded an alternative cause (hemogram, biochemistry, antinuclear antibodies, anti-DNA, lues, borrelia, serology, copper, ceruloplasmin, ferritin, thyroid hormone, vitamin
B12 and folic levels were normal). Brain CT and MRI showed cerebral
atrophy. Brain SPECT-IBZM did not disclosed pathological alterations.
Conclusions: According to the proposed diagnostic criteria (Gilman
et al) our patient suffers a probable multiple system atrophy-parkinsonian type. The initial symptom was a facial dystonia mimicking a risus
sardonicus in absence of levodopa treatment. Our case supports that
this form of dystonia, should be considered an early warning sign to
differentiate the parkinsonian type MSA (multiple system atrophy)
from Parkinsons disease.
832
Intracranial dural arteriovenous stula presenting with Parkinsonism
H.J. Park, S.K. Song, H.W. Shin, Y.H. Sohn (Seoul, Korea)
Objective: To describe a patient with parkinsonism and cognitive
dysfunction due to intracranial dural arteriovenous stula (DAVF).
Background: DAVF is a relatively rare disorder that consists of
abnormal arteriovenous shunts within the dura mater. The clinical
manifestations of DAVF are generally benign, which include headache,
pulsatile tinnitus and papilloedema. Parkinsonism or dementia as the
presenting symptom of DAVF is extremely rare. We present a patient
with DAVF who showed progressive parkinsonism and cognitive impairment.
Methods: Case description.
Results: A 54-year-old man presented with progressive gait disturbance for 1 year and cognitive decline for 2 months. He had no history
of head trauma, central nervous system infection, or skull surgery. On
neurological examination, he showed symmetric bradykinesia in the
bilateral arms and legs. His gait was slow and short stride, and arm
swing was reduced. He had tendency of falling while walking on the
narrow space. When stretching out his arms, he showed moderate
degree of postural tremor. He demonstrated defects of word uency and
calculation function on neuropsychological test. T2-weighted brain
MRI showed high signal intensity in the subcortical white matter.
Gadolium enhanced T1-weighted brain MRI revealed dilated and tortuous vessels in the pons, cerebellum and temporo-parieto-occipital
region. Cerebral angiography showed DAVF in the left sigmoid sinus,
fed by the left occipital artery, left vertebral artery and left meningohypophysial artery. Selective embolization of feeding vessels improved
his cognitive function completely, while his parkinsonism persisted
partially.
Conclusions: The parkinsonism and cognitive impairment in this
patient may be associated with the venous ischemia of the cerebral
white matter caused by DAVF. DAVF should be considered for the
differential diagnosis in patient with progressive dementia or parkinsonism, because it can be reversible by selective embolization.
833
Applause Sign secondary to inltrative cerebral lymphoma
D.A. Gallagher, J.M. Schott, A. Childerhouse, T. Wilhelm, A. Gale,
A. Schrag (London, United Kingdom)
Objective: To report the case of a man who had a strongly positive
applause sign in the context of a histologically conrmed cerebral
lymphoma.

S253

Background: The Applause sign, in which a patient asked to

imitate three claps shows a tendency to perseverate with continuous
applause, is commonly seen in Progressive Supranuclear Palsy (PSP).
To our knowledge, the applause sign has not previously been reported
in the context of non-neurodegenerative disease.
Methods: A 64 year old man with an eight year history of depression
was referred to psychiatry with worsening mood and inappropriate
behaviour. Venlafaxine and risperidone were started. He was referred
to neurology eight months later having developed transient episodes of
confusion, which had increased in frequency and severity. On examination he was disorientated to time and place. There were bilateral
grasp reexes, and evidence of marked perseveration with a positive
applause sign. There were extrapyramidal features including hypomimia, bradykinesia, general paucity of spontaneous movements and
slow gait. There was a mild right facial droop without objective facial
weakness. Neurological and general examination was otherwise normal. Neuropsychometric assessment showed generalized cognitive impairment. There was impairment on test of recognition memory for
words (10-25th percentile) and faces (5th percentile); naming (5th
percentile); and recognition of famous faces (5th percentile). There
was evidence of dyspraxia, and he performed poorly on tests sensitive
to frontal lobe dysfunction: verbal uency, Luria sequencing task and
proverb interpretation.
Results: MRI revealed diffuse white matter lesions, including right
anterior striatal and left supralenticular involvement(A) and extensive
frontoparietal subcortical involvement(B). Diagnosis of diffuse T-cell
rich large B cell lymphoma was made on obturator lymph node biopsy.
Conclusions: Whilst the clinical phenotype in this case was not in
keeping with PSP, the presence of the applause sign provided evidence
for organic brain disease affecting frontal-basal ganglia circuitry. This
case demonstrates that the applause sign may be seen in inltrative as
well as neurodegenerative conditions, and may be a useful bedside test
of frontal-subcortical involvement in a range of clinical contexts.

FIG. 1 (833).

834
The specicity and sensitivity of applause sign in differentiating
PSP and other parkinsonian syndromes
J. Wu, O. Sitburana, J. Jankovic (Houston, Texas, USA)
Objective: We tested the applause sign in different groups of subcortical dementia patients in order to determine its sensitivity and
specicity in differentiating progressive supranuclear palsy (PSP) from
other disorders.
Background: PSP is a progressive neurodegenerative disease characterized by parkinsonism, falls, supranuclear gaze palsy and subcortical dementia. Parkinsons disease (PD), PSP, multiple system atrophy
(MSA) and corticobasal degeneration (CBD) all share features of a
frontal lobe-like syndrome characterized by memory impairment, impaired ability to manipulate acquired knowledge and slowed thought

Movement Disorders, Vol. 22, Suppl. 16, 2007

S254 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

processes. Several neuropsychological assessments had been developed in an attempt to distinguish these disorders. The applause sign
was recently reported as a specic characteristic of PSP when compared to PD and frontotemporal dementia (FTD). However, the sensitivity and specicity of applause sign in differentiating PSP from
other subcortical dementias are still unclear.
Methods: 25 subjects with idiopathic PD, 16 subjects with PSP, 12
patients with MSA , 8 patients with CBD, and 25 subjects as normal
control were included in this study. Subjects were asked to clap three
times after demonstration by the examiner. The performance was
scored as following: score3 if the subject claps only three times;
score2 if the subject claps four times; score1 if the subject claps
ve to ten times; score0 if the subject can not stop.
Results: The mean clap score in PD, PSP, MSA and CBD was
2.810.64, 2.370.99, 2.01.16 and 11.31 respectively. The most
striking nding is that in CBD group, 6 out of 8 patients showed
applause sign. Four of the eight patients scored 0, two other patients
scored 1. The three clap test score can not discriminated patients with
PSP from those with PD (P0.068) or with MSA (P0.188). However, the three clap test score can discriminate patients with CBD with
PD (P0.05) and PSP (P 0.05). The clap score can also discriminate
patients with MSA with PD (P0.05).
Conclusions: Applause sign is not a specic nding only detected in
PSP patients. Patients with other subcortical dementia such as PD,
MSA or CBD can also showed applause sign. It also failed to discriminate patients with PSP from PD or MSA. The three clap test scores
signicantly differed patients of CBD from PD, MSA and PSP.
835
A new American kindred with hereditary diffuse leukoencephalopathy with spheroids (HDLS)
L.A. Brown, J.A. VanGerpen, C.W. Wider, R.J. Uitti, D.W. Dickson,
Z.K. Wszolek (Jacksonville, Florida, USA)
Objective: To report the clinical and pathological features of a new
kindred with HDLS.
Background: HDLS is an autosomal dominant disorder characterized
by cerebral white matter degeneration with axonal spheroids leading to
progressive cognitive and motor dysfunction. At present seven families
from Sweden, Japan, The Netherlands, Australia, and the United States
have been described. Clinical phenotype includes cognitive, behavioral, and psychiatric dysfunction, Parkinsonism, ataxia, and epilepsy.
Methods: We currently follow a new kindred with HDLS comprised
of 7 affected individuals (5 deceased). We examined 25 members of
this family and reviewed available genalogical and clinical records.
Neuropathological studies were performed.
Results: Mean age of symptomatic disease onset was 35 years (range
20-57), and mean disease duration was 16 years (range 3-46). Initial
symptoms included memory distubance and behavioral change in 5
affected individuals. Irritability, depression, anxiety, and cortical signs
were noted at disease onset in 4 family members. Disorientation, severe
gait disturbance, and postural instability were observed in 5 individuals
within 2 years of disease onset. Ataxia was noted in one affected
individual, and epilepsy was noted in another. Dysarthria and double
incontinence were present in 3 deceased individuals prior to death. MRI
of an affected individual revealed frontal atrophy with conuent, increased T2 signal changes, cerebellar atrophy, and thinning of the
corpus callosum. Histopathologic studies of one member of this kindred revealed cerebral white matter degeneration, axonal spheroids and
ballooned neurons in the cortex, and myelinated ber loss.
Conclusions: We describe here a new kindred with HDLS with
phenotype dominated at onset by cognitive, behavioral, and psychiatric
dysfunction followed by eventual loss of motor function. Parkinsonism
was not observed in this kindred. While HDLS neuropathology is
consistent across all known kindreds there is signicant intra- and
inter-variability of clinical phenotype. Further research is needed to
better clincally and pathologically characterize this disorder and locate
genetic abnormalities.

Movement Disorders, Vol. 22, Suppl. 16, 2007

836
Epidemiological data of nervous diseases in Ukraine
Y.V. Lekomtseva (Kharkiv, Ukraine)
Objective: Aim of this study was to evaluate current epidemiological
characteristic of nervous diseases (ND) which takes the second place in
the structure of all diseases in Ukraine.
Background: Successful approach to neurological issues depends on
epidemiological data of ND.
Methods: Administrative database and statistical methods were applied. Figures were calculated on one hundred thousand of population
[per 100,000 of a year for Ukrainian residents].
Results: The prevalence of ND was registered 4358.2 and morbidity
1488.2 per 100 000 for the residents in 2005 in Ukraine. The greatest
part took CVD (cerebrovascular diseases) their overall prevalence
was 8082.4 per 100 000 inhabitants of 2005 year, morbidity 1457.0,
mortality 231.4. In the structure of CVD great part took dyscirculation encephalopathies (64.3%) and vascular dementias (21.51%). In
2005 126 new cases of stroke have been registered with a median age
of 57 years, approximately 78% patients who presented with ischemic
strokes, 19.4% hemorrhages (age rates were 64.311.97 and
55.18.44 y. respectively). Prevalence of strokes was 308.9 of 2005
year; morbidity 236.2, mortality 72.4 [1]. The prevalence of
Parkinsons disease was 51.5/100 000 and morbidity 7.0/100 000.
Mean Parkinsons disease duration was 26.28.6 years; the disease
most often occurred at the age of 73.5619.21 years (median age of
onset - m.a.); correlation between clinical forms: rigidity (12%), tremor
(35%) and rigidity-tremor (53%). The prevalence of epilepsy was 61.7,
morbidity 6.9. In the structure of epilepsy partial seizures took a place
in 56.5% clinical cases with mean diseases duration of 14.20.8 y.,
m.a. was 3214.91 y. [1]. The prevalence of MS (multiply sclerosis)
was 48.4 and morbidity 3.2. The male to female ratio was 1:2.
According to the clinical course the types of MS were found to be as
follows: relapsing-remitting (48.6%), secondary-progressive (28.9%),
relapsing-progressive (12.4%) and progressive (10.1%). Migraines
data: prevalence 69.4, morbidity 8.3. Clinical types: migraine
without aura (68%), migraine with aura (19%), opthalmoplegic migraine (4%), others (9%). The female/male ratio was 1.8:1.2 with mean
diseases duration of 6.24.6 and m.a. 19.82.8 y. 1. Voloshin P.V.,
Mishchenko T.S., Lekomtseva Y.V. Analysis of prevalence and morbidity of nervous diseases in Ukraine // International neurological
Journal, 2006, No. 3(7).- P.9-13.
837
Atypical PSP: A radiological diagnosis
V.K. Gontu, D.P. Auer, N.B. Bajaj (Derby, United Kingdom)
Objective: There has been a call recently to re-classify progressive
supranuclear gaze (PSP) palsy into two distinct phenotypes: Richardsons syndrome and PSP-P.
Background: Richardsons syndrome is the more typical clinical
presentation with vertical supranuclear gaze palsy, postural instability and falls presenting within the rst two years of disease onset.
PSP-P has a less classical presentation with asymmetrical parkinsonism and some levodopa response making the differentiation from
Parkinsons disease (PD) more difcult. In PSP-P cases, vertical
supranuclear gaze palsy can eventually develop although in our own
experience (see other poster) this can take up to 6 years. A variety
of MRI imaging techniques have been proposed as having clinical
utility in the radiological diagnosis of PSP. These techniques are a
radiological correlate of the consistent neuropathological feature of
mid-brain atrophy characteristic of PSP. These techniques can be
especially valuable in differentiating PSP-P patients from PD where
supranuclear gaze palsy remains absent.
Methods: We present the case of a 53 year old man presenting to the
hand surgeons in 2004 with a years history of stiffness and clumsiness
of the right upper limb. He was noted to have some parkinsonian
features and sinemet was started with some initial symptomatic response. Early postural instability and falls within the rst two years
were noted. MMSE remained normal throughout 3 years into the

POSTER SESSION III, THURSDAY, JUNE 7, 2007

illness. The patient was falling weekly and was tted with a helmet. He
complained of dysphagia and a quiet voice. He maintains a full range
of eye movements 3 years into the illness.
Results: T1, axial T2, T2* and diffusion weighted MRI imaging at
3T showed marked focal brain atrophy, predominately affecting the
upper brain stem with an AP diameter of 1.3cm. Additional marked
fronto-temporal atrophy was seen. The patient was given a nal diagnosis of PSP-P on clinical and radiological criteria.
References:
1. Revesz T, Lees AJ et al Characteristics of two distinct clinical
phenotypes in pathologically proven progressive supranuclear
palsy: Richardsons syndrome and PSP-parkinsonism. Brain.
2005 Jun;128(Pt 6):1247-58
2. Csoti I, Solymosi L et al. Measurement of the midbrain diameter
on routine magnetic resonance imaging: a simple and accurate
method of differentiating between Parkinson disease and progressive supranuclear palsy. Arch Neurol. 2001 Jul;58(7):1076-9.
838
A combined case of tauopathy and Alpha-synucleinopathy
A. Marcos Gonzalez, L. Silveira Moriyama, D.R. Williams,
S. OSullivan, L.A. Massey, R. Da Silva, J.L. Holton, A.J. Lees
(Sevilla, Spain)
Objective: To report the case of a patient presenting with parkinsonism and clinical features consistent with progressive supranuclear palsy
(PSP), whose post mortem neuropathological examination revealed the
coexistence of PSP and multiple system atrophy (MSA).
Background: PSP is a tauopathy characterized by progressive parkinsonism, pseudobulbar symptoms, gaze palsy and frequent falls. MSA is an
alpha-synucleinopathy characterized also by progressive parkinsonism
combined with pyramidal, cerebellar and autonomic dysfunction. Dual
pathology with PSP and MSA in the same patient is rare and so far only
two cases have been reported (Takanashi 2001 and Uchikado 2006).
Methods: We describe the case of a 62-year-old woman who presented
with rigidity, tremor, reduced vertical upgaze, and several falls, with a poor
response to L-Dopa. No urinary problems, constipation or sweating abnormalities had been mentioned. Neuropathological examination of the
brain was performed. Histological examination of 10m thick sections
from formalin xed, wax-embedded brain tissue utilised routine stains and
immunohistochemical staining for alpha-synuclein and tau protein.
Results: Tau immunohistochemistry showed coiled bodies, neurobrillary tangles and neuropil threads in the basal ganglia, substantia
nigra, pons and dentate nucleus. In addition small numbers of tufted
astrocytes were present in the putamen. Alpha-synuclein immunoreactive glial cytolasmic inclusions were widespread and there were also
alpha-synuclein positive neuronal nuclear and neuronal cytoplasmic
inclusions in the frontal cortex, basal ganglia, substantia nigra and
pons. There was no A deposition. These pathological ndings are
compatible with PSP and MSA.
Conclusions: Our case shows a patient with clinical features of PSP
without symptoms of MSA, whose histological examination was consistent with dual pathology. As the prevalence of PSP is 5,3-6,4 per
100.000 and of MSA is 3,0-4,4 per 100.000, the coexistence of both in
a single person is an unlikely but possible event.
839
Normal pressure hydrocephalus and vascular parkinsonism: Discrete or overlapping clinical entities?
A.J. Espay, R.K. Narayan, A.P. Duker, E.T. Barrett,
G. de Courten-Myers (Cincinnati, Ohio, USA)
Objective: To report a case meeting criteria for vascular parkinsonism (VaP) but whose response to cerebrospinal uid diversion and
neuropathological ndings were consistent with normal pressure hydrocephalus (NPH).
Background: None of the currently available diagnostic guidelines
for VaP and NPH have been supported on neuropathological correlations for validation.

S255

Methods: Review of clinical, imaging, gait analysis, neuropsychological and neuropathological data.
Results: This 80-year-old smoker, hypertensive, and hypercholesterolemic man had a staggering progression of shufing gait, urinary incontinence, short-term memory loss, and multiple falls for 2.5 years. He
required two hospitalizations for sudden-onset freezing of gait. Levodopa
600 mg/day failed to provide any benets. Over two years, his overall
index of mental functioning dropped from the average (65th percentile) to
the extremely low range (1 percentile), and all executive functions
(including behavioral initiation, planning, and problem solving) dropped
from high-average to 1 percentile. Brain MRI showed conuent subcortical and periventricular white matter punctate areas of increased T2 signal
consistent with chronic small vessel ischemic disease. There was mild to
moderate ventricular dilatation. A 3-day external lumbar drainage (ELD)
hospitalization was undertaken to determine eligibility for ventriculoperitoneal shunt (VPS) placement. Gait analysis after ELD showed signicant
improvement ( 40%) in gait velocity and stride length with modest
increase (20%) in cadence. Cognitive parameters improved but remained
within the abnormal range. Upon a revised diagnosis of NPH, he consented
to VPS placement, unfortunately succumbing to procedure-related complications. Autopsy revealed communicating hydrocephalus with leptomeningeal brosis and supercial gliosis of cerebral cortex. There were no
macroinfarcts or lacunes, Lewy bodies, or tau pathology. Microvascular
pathology in basal ganglia and frontal white matter associated with focal
reduction in oligodendrocytes were present to an extent consistent with
advanced age.
Conclusions: VaP and NPH appear to be overlapping entities within
a neuropathological continuum. ELD responsiveness may be more
informative and relevant than the specic clinical type of lower-body
parkinsonism.
840
Manganese encephalopathy due to methcathinone abuse
O.S. Levin, N.A. Amosova, N.V. Fedorova (Moscow, Russian
Federation)
Objective: The aim of the study was to investigate clinical and MRI
features of toxic encephalopathy associated with methcathinone
(ephedrone) abuse.
Background: Methcathinone (ephedrone) is amphetamine-related
substance prepared from ephedrine or phenylpropanolamine with addition of potassium permanganate and acetic acid and used intravenously. Methcathinone was extensively used as home drug for obtaining of narcotic effect during past decade in Russia.
Methods: Forty two patients (mean age 21.65.8 yrs) with encephalopathy occured after regular methcathinone using were studied. We used
Methcathinone Encephalopathy Rating Scale (MERS) for comprehensive
quantitative assessment of different neurologucal or neuropsychological
manifestations of encephalopathy. We also used MR scan (0.5 T).
Results: The rst signs of cerebral dysfunction had appeared after
3-14 months (mean 6.84.9) of drug abuse. The main clinical manifestation of encephalopathy were moderate bilateral hypokinesia and
axial more than limb rigidity with poor response to levodopa (89%),
moderate to severe multifocal dystonia with involvement of limbs and
axial muscle (86%), moderate to severe postural instability with frequent backwards falls (95%), pseudobulbar syndrome with moderate to
severe dysartria (100%), corticospinal signs (14%), action myoclonus
(14%). Neuropsychological investigation didnt reveal global intellectual decline but patients were compromised in performing executive
tasks. All patients also developed marked personality changes suggesting frontal lobe dysfunction (abulia, apathy, disinhibition) and hypersomnia. MRI revealed abnormal high-signal-intensity area in the medial segment of global pallidus and in midbrain (in projection of
substantia nigra pars reticulate) at T1-weighted MR images in all
patients who had investigated during rst two years after neurologic
signs had appeared. But this sign has tended to disappeare later due to
manganese elimination. Follow-up revealed delayed progression in
33% patients during 1 to 4 years after stopping of abuse.
Conclusions: Similarity of neurological decit and MRI data to
features of chronic manganese intoxication allowed to conclude that

Movement Disorders, Vol. 22, Suppl. 16, 2007

S256 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

damage of central nervous system in patients with methcathinont abuse
is associated with accumulation of manganese in the brain.
841
A focus on head drop and camptocormia
N. Yardimci (Ankara, Turkey)
Objective: In camptocormia, there is typically marked anterior curvature
of the thoracolumbar spine, arising as a result of weakness or abnormality
in the tonus of the paraspinal muscles. Head ptosis is characterized by
marked anterior curvature or angulation of the cervical spine resulting
from weakness of the neck extensor, or increased tone of the exor
muscles. The deformity in head ptosis and camptocormia is not xed and
is corrected by passive extension or lying in supine position. It is not
possible to straighten the neck or back voluntarily.Both of them occur after
age 60 either subacutely or chronically progressive in years.Histological
changes in the paraspinal muscles of patients with idiopathic head ptosis
were also seen in patients with camptocormia with an obvious extrapyramidal cause.The evaluation of these disorders can indeed be challenging
and often no denite diagnosis is made.
Background: Are camptocormia and head drop distinct entities?
Methods: Table 1 Table 2.
Results: Neuromuscular disorders conned to the paraspinal muscles
are a frequent cause.However, also central disorders may lead to a
sagging of the head or trunk or of both due to a lessened tone of the
head and trunk extansors.Reclination of the trunk or head might be
explained as a pathologically increased innervation of the ventral
muscles as in dystonic movement disorders. Both may occur occasionally, as a consequence of musculotendinous contractures secondary to
certain neuromuscular disorders.
Conclusions: In most instances head drop and camptocormia are not
related.It is rare to nd a patient with both disorders except in extrapyramidal disorders such as MSA and postencephalitic parkinsonism.
However, the similarities between them suggest that both head ptosis
and camptocormia may occur as a result of similar pathophysiological
processes affecting paraspinal muscles of different parts of the spine.

Table 1. Causes of camptocormia

Neuromuscular

ALS, IBM, nemaline myopathy,

fascioscapulohumeral dystrophy
Idiopathic, postencephalitic, MSA,
Villiusk encephalomyelitis,
valproate toxicity

Parkinsonism
Idiopathic

Table 2. Causes of head ptosis

Motor neuron
Peripheric nerve
Neuromuscular junction
Muscle : inammatory;
congenital;
metabolic

Movement Disorders, Vol. 22, Suppl. 16, 2007

ALS, postpolio syndrome

Chronic inammatuar polyneuropathy
Myasthenia gravis
Polymyositis; IBM, nemaline,
mitochondrial myopathy ; Cushing,
hypothyroid, hypokalaemic myopathy

842
Acquired hepatocerebral degeneration and dopamine transporter
imaging using [123I]-FP-CIT SPECT
J.-M. Kim, Y.K. Kim, S.E. Kim, B.S. Jeon (Seongnam-si, Korea)
Objective: To study the nature of parkinsonism in acquired hepatocerebral degeneration (AHD), the integrity of nigrostriatal dopaminergic system was examined.
Background: The clinical and neuroradiological characteristics of
AHD have not yet been fully understood. AHD may involve basal
ganglia dysfunction and induce parkinsonism.
Methods: During a 2-year period, consecutive patients with liver
cirrhosis were screened for parkinsonism. MRI and dopamine transporter (DAT) SPECT using [123I]-FP-CIT were performed.
Results: We found seven liver cirrhosis patients with parkinsonism.
In all patients, MRI showed hyperintensities on T1-weighted images,
affecting bilateral basal ganglia. DAT density was normal in six patients. In one patient, striatal [123I]-FP-CIT uptake was reduced similar
to the pattern of Parkinson disease (PD). This patient was responsive to
levodopa therapy.
Conclusions: These results suggest that presynaptic dopaminergic
system may be intact in most cases of parkinsonism in AHD. One
patient showed levodopa-responsiveness and reduced DAT density of a
PD pattern. Further studies are required to solve the issue of direct
damage to nigral dopaminergic neurons in AHD.
843
Possibility of development of forms of pathological dependence on
levodopa preparations and its prevention
I.V. Bogdanova (Kharkiv, Ukraine)
Objective: To evaluate possibility of development of dependence on
levodopa(L) preparations in patients(PTS) with Parkinsonism(P), using
primitive manganese containing psychostimulators.
Background: L therapy of manganese P is inefcient. During the use
of primitive home-made manganese containing psychostimulators P is
forming.
Methods: We analyzed anamnesis, results of clinical neurological
examinations of PTS aged 17-36 years with dependence on narcotic
substances and psychostimulators, with history of disease 6-36 months;
and patients with the L dependent form of Parkinsons disease (PD),
staged 2,5-4 by Hoehn and Jahr scale, with history of disease not less
than 5 years.
Results: Use of home-made manganese containing psychostimulators formed P. Efciency of anti-parkinsonic therapy was low.
Authentic differences have not been detected between PTS received
complex therapy incl. L/carbidopa preparations(L/C-p), and PTS
received complex therapy without L/C-p. Catamnestic observation
discovered that 20%PTS of psychostimulators dependent category
periodically continued to take L/C-p without medical indications in
order to provoke the change of condition of consciousness, hallucinosis. In PD group 10%PTS receiving L therapy more than 5
years, tendency to uncontrollable increasing of doses of L/C-p
without objective indications was formed. Relative compensation of
PD specic disturbances was reached by L/C-p dosing 500-750
mg/d at 75%PTS and 1000 mg/d at 25%. Despite of compensation
specied part of PTS increased by themselves dosage up to 25003000 mg/d. Treatment with the L optimizing preparation (L/C/
entacapone), allowed to reduce dyskinesia, uctuations of main
symptoms and to reduce L dosage without deterioration of motor
functions. Despite of objective improvement of neurological status
and 15-30% UPDRS decrease, PTS did not follow recommended
doses and started to take additional L/C-p. Considering dissonance
between objective improvement of neurological status and psychological need of PTS for higher doses, formation dependence from L
as from psychoactive substance is assumed.
Conclusions: Optimizing levodopa therapy prevent dependence from
high L dozes before development of levodopa related mental complications. Optimal is use of L/C-p with high bioavailability (L/C/enta-

POSTER SESSION III, THURSDAY, JUNE 7, 2007

capone) to achieve clinical improvement with lower L dozes and to
keep positive answer to L for a long time.
844
Possibility of development of secondary Parkinsonism caused by
parasitary diseases
I.V. Bogdanova (Kharkov, Ukraine)
Objective: To evaluate case of patient (50 yrs, veterinary) with
secondary Parkinsonism (P), caused by chronic acquired toxoplasmosis, toxocarosis
Background: In scientic literature and reviews combination of P
with parasitary diseases are not presented, however it is possible to
assume, considering prevalence of parasitic diseases and frequency of
levodopa-negative forms of P, that such cases are not rare.
Methods: We analyzed anamnesis, results of clinical neurological
examinations. MRI, immune-enzyme and polymerase chain reaction
analysis were performed.
Results: The patient practiced veterinary, suffered from bronchial
asthma, and from 39 yrs began to complain on rigidity, slowness of
movements, restriction of self-service, change of a bearing, and gait, a
silent mufed voice, fatigue, depression, weight loss. The neurologic
status was: bradykynesia, muscular hypertonus of plastic type, infringements of a stereotype of walking (shufing, propulsory), loss of skills
of self-service, trembling hyperkynesis. Parkinson disease was diagnosed and different antiparkinsonic treatments were given, however,
with no improvement. Levodopa-test was negative, levodopa caused
intolerance and worsening of patients condition. Short and unstable
improvements were after plasmapheresis, detoxication preparations.
Disease progressed, patient was stabilised on dopamine agonists. After
verication of toxoplasmosis, toxocarosis by immune-enzyme and
polymerase chain reaction analysis adequate ethiopathogenic treatment
was given. Patients condition improved but didnt restored completely.
At MRI of brain multiple periventriculary nidi of lesions of substantia
alba were diagnosed.
Conclusions: Features of the given clinical case are: - Formation of a
clinical picture of P after toxoplasmosis, toxocarosis; - levodopa-negative
variant of P; - short-duration efciency of methods of desintoxication and
plasmapheresis, antiparasitic preparations; - Steady character of neurologic
infringements, progredientness of the process. It is possible to assume, that
P development was provoked by long-term contact to certain antigenes and
formation of inadequate immune answer. Inefciency of levodopatherapy
and adverse reactions even to small dozes of levodopa were caused by by
young age and probably safe endogenic dopamine synthesis. P symptoms
could be caused by damage at receptors level. Better efcacy of DA
conrms this assumption.
845
Olfaction in dardarin/LRRK2 associated Parkinsonism
L. Silveira-Moriyama, L.C. Guedes, A. Kingsbury, J.J. Ferreira,
C. Sampaio, E.R. Barbosa, V. Bonifati, N.P. Quinn, A.J. Lees
(London, United Kingdom)
Objective: To evaluate the smell sense in patients with familial or
sporadic Parkinsonism related to mutations in LRRK2/Dardarin gene
(Park8).
Background: Mutations in the Park8 locus are likely to be the commonest genotype for autosomal dominant Parkinsons disease. The disease
course resembles that of idiopathic Parkinsons disease (IPD) with onset in
late life and responsiveness to levodopa is common. Despite a report of
Lewy body pathology in the olfactory bulb1 and a few reports of clinical
smell test in Park8 patients2,3 there is no consensus in the literature about
the presence of smell decit in Park8 patients.
Methods: Patients with Parkinsonism without dementia and conrmed Park8 mutations were recruited in London (n5) and Lisbon
(n16) and tested with the 40-item University of Pennsylvania smell
test (UPSIT). We also tested 100 controls and 115 IPD patients in
London and 18 controls and 14 IPD patients in Lisbon. Results were
analyzed by Mann-Whitney U tests.

S257

Results: There was no signicant difference between smoking status or

gender distribution between the IPD, control and Park8 groups. Mean age
for the subjects in Lisbon: IPD72.9(6.39), Park868.87(8.99), controls54.77(14.18); in London: IPD66.48(11.57), Park866.51(6.79),
controls66.38(8.19). There was a signicant difference between the
average UPSIT score in the Park8 and control groups in both centers
(London: exact p.001; Lisbon: exact p.0005) but no difference between the Park8 and IPD groups in either center (London: exact p.943;
Lisbon: exact p.530).
Conclusions: Our study suggests that the sense of smell is affected in
patients with Parkinsonism associated with Dardarin/LRRK2 mutations. This is supportive of the phenotypic similarity between this
genetic disorder and patients with IPD. Smell loss is present in 70100% of IPD patients and the olfactory bulb and tract are affected in
virtually all cases of IPD. The nding of similar clinical smell decit in
Park8 cases raises issues concerning the probable underlying pathological process in the two conditions.
846
Annonacin, a natural mitochondrial complex I inhibitor, causes tau
pathology in cultured neurons
M. Escobar Khondiker, M. Hollerhage, P.P. Michel, M.-P. Muriel,
P. Champy, T. Yagi, A. Lannuzel, E.C. Hirsch, W.H. Oertel,
R. Jacob, M. Ruberg, G.U. Hoglinger (Marburg, Germany)
Objective: To test the hypothesis that annonacin, a prototypical
anonaceous acetogenin, contributes to the etiology of the human disease, we investigated whether annonacin affects the cellular distribution of phosphorylated tau protein.
Background: A neurodegenerative tauopathy endemic to the Caribbean Island of Guadeloupe has been associated with the consumption
of anonaceous plants that contain acetogenins, potent lipophilic inhibitors of complex I of the mitochondrial respiratory chain.
Methods: We treated primary cultures of rat striatal neurons for 48
hours with annonacin.
Results: There was a concentration-dependent redistribution of
tau phosphorylated on several epitopes from the axons to the cell
body. Annonacin caused an immediate retrograde transport of mitochondria with phospho-tau attached to their outer membrane.
Taxol, a drug that displaces tau from microtubules, prevented the
retrograde movement of mitochondria and the somatic redistribution
of phospho-tau. Inhibition of annonacin-induced reactive oxygen
species by antioxidants did not prevent the redistribution of phospho-tau. In contrast, annonacin-induced redistribution of phosphotau was prevented by expression of the NDI1 NADH-quinoneoxidoreductase of Saccharomyces cerevisiae, which can restore
NADH-oxidation in complex I-decient mammalian cells, and by
stimulation of energy production via anaerobic glycolysis. Consistently, other ATP-depleting neurotoxins (MPP, 3-NP, CCCP)
reproduced the somatic redistribution of phospho-tau, whereas toxins not leading to ATP depletion did not cause phospho-tau redistribution.
Conclusions: Together, these data suggest that annonacin leads via
ATP depletion to retrograde transport of mitochondria to the soma and,
in parallel, induces changes in the intracellular distribution of phosphorylated tau in a way that shares characteristics with human diseases.
847
Unusual presentation of progressive supranuclear palsy with palatal and diaphragmal myoclonus: A case report
N.M. Browner, S. Fahn (New York, New York, USA)
Objective: We describe a 61 year old woman with eight year
history of coughing spells and six year history of generalized
slowness, gait freezing and falls. The coughing spell consisted of
brief rhythmical exhalations, a reminiscence of coughing, that continued for 15 20 minutes throughout the day. During the spell
patient could not hum and her speech sounded spasmodic; one could
observe rhythmical movements of the soft palate, abdominal wall
and neck muscles. Patient did not report an ear click. Exam revealed

Movement Disorders, Vol. 22, Suppl. 16, 2007

S258 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

hypomimia, tachiphemia, diminished vertical gaze, prominent body
bradykinesia, stooped posture, and positive pull test. Neurophysiology testing showed intermittent regular 1.7 1.8 Hz tremor, originating from brainstem, with 75 200 msec EMG discharges in
cranial nerve VII to XI muscles, palate and both hemidiaphragms.
Fluorodeoxyglucose positron emission tomography (FDG PET)
scan showed markedly reduced FDG uptake in anterior frontal and
temporal areas, and to a lesser degree in the basal ganglia and
brainstem. Clinical presentation, neurophysiology and neuroimaging suggested the diagnosis of progressive supranuclear palsy with
palatal and diaphragmal myoclonus. Trials of Sinemet 300 mg daily,
Requip 15 mg daily, Keppra 2000 mg daily and Clonazepam 2 mg
daily were not benecial and were poorly tolerated due to drowsiness.
848
Treatment of camptocormia by ultrasound-guided deep ventral
injection of botulinum toxin to the iliopsoas muscle
R. von Coelln, A. Raible, F. Asmus (Tuebingen, Germany)
Objective: To evaluate feasibility and effectiveness of ultrasoundguided ventral injection of botulinum toxin to deep portions of the
iliopsoas muscle in patients suffering from camptocormia.
Background: Camptocormia is characterized by an abnormal posture
of the trunk with pronounced exion of the thoraco-lumbar spine,
which increases during walking and abates in the recumbent position,
consistent with a primary action dystonia of the spine. Therapeutic
efforts to alleviate this debilitating, mostly Parkinsons disease (PD)associated symptom, have been mostly futile. Injection of botulinum
toxin A (BtA) into the rectus abdominis muscle has been benecial
only in a small subgroup of patients with palpable dystonic contractions
of the abdominal wall.
Methods: We treated four patients with PD-associated camptocormia
with ultrasound-guided ventral injections of BtA (Dysport) into deep
portions of the iliopsoas muscle. Two patients with pure forward
exion of the trunk received bilateral injections, and two patients who
presented with marked trunk deviation to one side received unilateral
(ipsilateral) injections. Starting from 500 U of BtA per side, the dose
was increased by 500 U in 3- to 4-month intervals up to 1,500 U per
side, or until side effects occurred. Outcome was evaluated at 2, 4 and
12-16 weeks after each treatment by detailed interview, physical examination, and by measuring the height for quantitative assessment of
posture.
Results: Treatment was generally well tolerated. At the initial dose,
two patients reported a transient and moderate improvement of posture
within a few days after injection. At the highest dose, two out of four
patients complained of weakness of hip exion (e.g. when climbing
stairs or tying shoes), and one experienced signicant worsening of her
general condition including her posture, albeit closely associated with
the passing away of a close relative. Clinical examination detected mild
to moderate hip exor paresis. Objective assessment of posture failed
to show a signicant benet at follow-up visits.
Conclusions: Injection of BtA to the iliopsoas is not in itself a
promising approach for the treatment of camptocormia. An appropriate
target structure for BtA treatment of this severe postural disorder
remains to be identied.
849
Anti-beta2-glycoprotein I antibody and vascular Parkinsonian
Y.-Y. Chang, M.-Y. Lan, Y.-L. Tseng, C.-S. Su, Y.-F. Kao, H.-S. Wu,
J.-S. Liu (Kaohsiung, Taiwan)
Objective: To investigate the prevalence of antibodies to phospholipid-binding proteins (APLA) and to study the association of these
antibodies with clinical features, risk factors, white matter hyperintensities (WMH) lesion in patients with vascular parkinsonism (VP).
Background: VP is a distinct and heterogeneous clinical entity.
Neuroimaging studies show multiple infarcts or ischemic lesions in the
basal ganglia or subcortical white matter. Moreover, anti-beta2-GPI

Movement Disorders, Vol. 22, Suppl. 16, 2007

antibodies (AB2GPI) are a subset of the APLA family and have been
identied to be closely associated with ischemic stroke.
Methods: 44 patients with clinical diagnosis of VP were enrolled for
the study. Enzyme-linked immunosorbent assays (ELISA) were used
for AB2GPI determination. Risk factors for stroke such as hypertension
(H/T), DM, hyperlipidemia, smoking, body mass index (BMI), presence of heart disease and the past history of stroke were documented.
Unied Parkinsons Disease Rating Scale (UPDRS), Hoehn and Yahr
score (HYS), Minimal-Mental Status Examination (MMSE) and Hospital Anxiety and Depression Scale (HADS) had been used for the
clinical evaluation. The semiquantitative visual rating scale was used
for the evaluation of WMH in T2-weighted MR images.
Results: AB2GPI have been obtained in nine (20.5%) of these
patients. There was no signicant difference in age between AB2GPI
IgG (70.6 7.4 yr) and AB2GPI IgG- (69.7 9.3 yr) group.
AB2GPI appeared more frequent in women (30.8%) than men (16.1%).
No signicant differences in the risk factors (H/T, DM, hyperlipidemia,
cigarette smoking, BMI, heart disease or past stroke history) were
evident between AB2GPI and AB2GPI- groups. Compared with
AB2GPI- group, the AB2GPI group had signicantly higher UPDRS
and HADS scores, and lower MMSE score. Otherwise, there was no
apparent difference in UPDRS motor score between AB2GPI and
AB2GPI- groups. The MRI study revealed higher level of WMH in the
deep white matter and periventricular areas in the AB2GPI group.
Conclusions: This study suggests that APLA or AB2GPI dependent
variety, is an important predictor of poor clinical outcome in VP. Apart
from the traditional therapy with anti-platelet agents, further treatment
modalities such as immuomodulatory treatment or anticoagulation therapy might be considered in the VP patients with positive AB2GPI.

850
Comparison of brain MRI and 18F-FDG PET in the differential
diagnosis of multiple system atrophy from Parkinsons disease
S.J. Chung, K.Y. Kwon, C.G. Choi, J.S. Kim, S.K. Lee, C.S. Lee,
M.C. Lee (Seoul, Republic of Korea)
Objective: We aimed to investigate the diagnostic value of brain
MRI and 18F-FDG PET in the differentiation of MSA from PD. Brain
imaging ndings were also analyzed according to the clinical phenotypes of multiple system atrophy-parkinsonian (MSA-P) and multiple
system atrophy-cerebellar (MSA-C).
Background: The differential diagnosis of multiple system atrophy
(MSA) from Parkinsons disease (PD) is often difcult and carries a
high rate of misdiagnosis or delayed accurate diagnosis.
Methods: We reviewed retrospectively the results of brain MRI and
18
F-FDG PET in the patients with MSA-P, MSA-C, and PD. One
experienced neurologist, one neuroradiologist, and one nuclear medicine physician were blinded to the clinical diagnosis. 18F-FDG PET
images were evaluated using visual analysis and statistical parametric
mapping (SPM) analysis.
Results: Thirty-ve patients with MSA (23 MSA-P, 12 MSA-C) and
17 patients with PD were included. The overall concordance rates
between clinical diagnosis and imaging diagnosis among MSA-P,
MSA-C and PD were 80% by visual MRI analysis, 88.5% by visual
18
F-FDG PET analysis, and 84.3% by SPM analysis of 18F-FDG PET.
Sensitivity of brain MRI versus SPM analysis of 18F-FDG PET for the
differentiation of MSA from PD was 72.8% versus 95.5% (p 0.063),
specicity was equally 100%, positive predictive value was equally
100%, and negative predictive value was 60% versus 90%.
Conclusions: Our results suggest that brain MRI and 18F-FDG PET
have the diagnostic usefulness in the differentiation of MSA (MSA-P
and MSA-C) from PD. 18F-FDG PET showed the tendency for higher
sensitivity in the differential diagnosis of MSA from PD than brain
MRI, suggesting the need for additional well controlled prospective
studies to answer this question.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

851
The cognitive prole versus motor severity and clinical course of
patients with Parkinsonism associated to vascular disease (vascular
Parkinsonism) and Parkinsons disease
C. Panea, H. Nicolae, I. Codita, G. Vulpe (Bucharest, Romania)
Objective: 1)To characterize the cognitive prole of patients with
Vascular Parkinsonism (VP) versus Parkinsons disease (PD). 2) To
analyze the correlation between motor severity, clinical course and
cognitive prole in both diseases.
Background: It is well known that up to 78% of PD patients will
develop dementia at some point, with onset typically occurring a few
years after the appearance of motor symptoms. The correlation between
incidence of dementia and clinical course is not so well established in
parkinsonism concomitant with vascular disease (VP) an insufcient
dened entity. An early diagnosis of cognitive impairment and its
predictable onset is important to design appropriate medical and social
interventions.
Methods: We studied 14 patients, 7 with PD and 5 with VP, referred
for treatment reconsideration. All subjects were assessed using a standard protocol. Diagnosis of PD was based on established criteria, while
VP was considered in those with bilateral, symmetric lower limb
predominant parkinsonism, with brain imaging showing diffuse subcortical ischemic lesions. Both groups were assessed using patient
diary, UPDRS, MMSE, DSM-IV, Hoehn & Yahr(H&Y), Schwab &
England(S&E) scales, Digit Span Test. Data obtained for each group
were statistically compared.
Results: Three (60%) of 5 VP patients were male, with mean age
72 6.5 years, while in PD group 5 (73%) were male with mean age
74 5,3 years. Mean age of onset was signicantly lower in PD group
(65 1,2 y) in comparison with VP group (68 1,9y). Disease duration
was shorter for VP versus PD despite the fact that H&Y (3) and S&E
(70%) were identically for both groups. The motor disabilities (UPDRS) were similar in both groups, but the cognitive prole was
different: dominant memory impairment, disorientation and a MMSE
score lower in VP group and disexecutive functions and sleep impairment more preeminent in PD group.
Conclusions: The cognitive prole is different and decline is more
rapid in VP than in PD, probably because of vascular component,
despite similar motor signs at one moment. Because the levo-dopa
treatment response is worse or absent in VP and cognitive impairment,
maybe an earlier different/ combined approach would be more successful.
852
Severity of tau deposition in progressive supranuclear palsy is
associated with clinical phenotype
D.R. Williams, J.L. Holton, A.J. Lees, T. Revesz (Melbourne, VIC,
Australia)
Objective: To investigate whether regional variations in the types of
tau lesions and differences in overall tau load explain the clinical
differences between clinical syndromes associated with PSP-tau pathology.
Background: Although pathological heterogeneity of PSP has also
been established, attempts to correlate this with clinical ndings have
only rarely provided conclusive results. Clinical syndromes associated
with PSP-tau pathology now include Richardsons syndrome (RS),
PSP-Parkinsonism (PSP-P) and pure akinesia with gait freezing
(PAGF).
Methods: Quantitative tau pathology assessment was performed in
17 brain regions in 42 cases of pathologically diagnosed PSP (22 RS,
14 PSP-P and 6 PAGF). Neurobrillary tangles, tufted astrocytes,
coiled bodies and thread pathology (CBTh) were quantitated and a
grading system was developed separately for each region. Using these
grades the overall tau load was calculated in each case. To establish a
simplied system for grading the severity of tau pathology, all data
were explored to identify the minimum number of regions that satisfactorily summarised the overall tau severity.

S259

Results: The mean severity in all regions of the RS group was higher
than in PSP-P and PAGF, and the overall tau load was signicantly
higher in RS than in PSP-P (p0.002). Using only the grade of CBTh
lesions in the SN, caudate and dentate nucleus, a reliable and repeatable
12 tiered grading system was established (PSP-tau score: 0, mild tau
pathology, restricted distribution; 8, severe, widespread tau pathology). PSP-tau score was negatively correlated with disease duration
(Spearmans rho -0.36, p0.028) and time from disease onset to rst
fall (Spearmans rho, -0.49, p0.003). The PSP-tau score in PSP-P
(median 3, range 0-5) was signicantly lower than in RS (5, 2-10,
p0.001). The two cases carrying the tau-H2 protective allele had the
two lowest PSP-tau scores.
Conclusions: We have identied signicant pathological differences
between the major clinical syndromes associated with PSP-tau pathology and the restricted, mild tau pathology in PSP-P supports its clinical
distinction from RS. The grading system we have developed provides
an easy-to-use and sensitive tool for the morphological assessment of
PSP-tau pathology.

FIG. 1 (852).
853
A patient with Gauchers disease successfully treated with pallidotomy, a 3-year follow up
M.R. Sobstyl, M. Zabek, H.M. Koziara, Z.K. Wszolek, J.E. Young
(Warsaw, Poland)
Objective: To report the long-term results of successful surgical
treatment of parkinsonism due to the Gaucher disease.
Background: Gauchers disease is the most common hereditary
lysosomal storage disorder and presents with wide phenotypic variations. The clinical spectrum of this illness also includes parkinsonism.
However, not much is known about possible surgical treatment of
end-stage parkinsonism in Gauchers disease.
Methods: We present the patient diagnosed with Parkinsons disease
at age 38 years. He exhibited the left leg rigidity and bradykinesia.
Treatment with levodopa produced only partial benet. He became
wheelchair bound and developed a hepatosplenomegaly 9 years later. A
diagnosis of Gaucher s disease was made. The clinical genetic testing
performed at the NIH revealed the presence of N37OS/IVS21 mutation further substantiated the diagnosis of type I Gauchers disease.
The therapy with imiglucerase was introduced. Two years later, despite
the optimal treatment of his Gauchers disease, the parkinsonian symptoms worsened. His H&Y scale was 5/5. He was referred for consideration for the surgical treatment of his severe parkinsonism. At age 54
years, he underwent the right-sided posteroventrolateral pallidotomy.
Results: The surgery was uneventful and he immediately improved.
He was able to walk independently and take care of himself. The
levodpa therapy was continued at the same dose. His H&Y scale was
2/5 in on state. On the last examination performed 3 years later, he
had mild parkinsonism and was able to perform all daily living functions independently. His H&Y scale was unchanged.
Conclusions: This case demonstrates a benet of surgical treatment
for genetically proven Gauchers disease type 1. However, further
studies on larger population of patients with this illness are needed to
know if surgery is indeed a treatment option for end-stage parkinsonism due to Gauchers disease. It may well be that the DBS procedure
may be even a better surgical therapy for these patients.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S260 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

854
CBD correlation of FDG PET, MRI and cognitive features
R. Borgohain, T. Suryaprabha, R.M. Kandadai, M.K. Panigrahi,
S. Shanmukhi, S.A. Jabeen (Hyderabad, Andhra Pradesh, India)
Objective: To study cerebral glucose metabolism, cognitive and
magnetic resonance imaging features in correlation with clinical features of corticobasal degeneration (CBD) patients.
Background: Corticobasal degeneration is characterized by progressive asymmetric rigidity & localized cortical signs ie, apraxia, cortical
sensory loss, focal dystonia & is refractory to levodopa. Asymmetry in
earlier stages is mistaken for Parkinsons disease as dementia is a late
feature.
Methods: Ten consecutive CBD patients who fullled the criteria of
Lang et al (94) were studied. A detailed neurological assessment was
done, including tests for cortical functions and UPDRS motor scores.
They underwent neuropsychological assessment for executive functions including verbal uency (phonemic & categorical), verbal working memory (N Back 1&2 tests), planning (Tower of London), attention and response inhibition (Stroop test). Verbal memory (Reys
auditory verbal learning test) and visual memory and visuospatial
functions (complex gure test) were also assessed. These were also
studied in age and sex matched controls. All patients underwent MRI
followed by FDG PET CT brain scan.
Results: Out of 10 patients, 7 were men and 3 women. All were right
handed and 6 were college educated (5 males, 1 female), 3 school
educated (1male, 2 female) , one was illiterate. Mean age of presentation was 62.2 5.32yrs. Mean duration of onset of disease was
4.22.39 yrs. Mean Hoehn &Yahr score was 3.90.99. Mean UPDRS
motor off score was 46.621.1 & on score was 40.021.74. CBD
patients when compared to controls showed statistically signicant
difference in certain tests of executive dysfunction (phonemic uency
& Tower of London), and verbal and visual memory (RAVLT and
complex gure test). All showed impaired right parietal functions
(complex gure test). MRI brain showed asymmetrical left temperoparietal atrophy in 8 patients & right in two. This correlated with FDG
PET brain scan ndings which had showed asymmetric focal hypometabolism in basal ganglia, and inferior parietal, temporal, frontal lobes
& the clinical ndings of asymmetric rigidity, dystonia & apraxia.
Conclusions: FDG PET CT Scan brain in CBD correlated with
clinical ndings and MRI Brain radiologic ndings but not with some
neuropsychological tests.
855
Parkinson plus syndromes in a primary peripheral neurological
center
F.R. Rodolico (Giarre, Sicily - CT, Italy)
Objective: I work as a neurologist in the province of Catania, in
Giarre where live about 40.000 people. Since the beginning of the
ninety years I started to take care of patients with Parkinsons disease
(PD) and Movement Disorders. In the last years our attention was
attracted by those patients who did not have a good prognosis such as
Parkinson patients.
Background: Parkinson - Plus Syndromes (PPS) are several primary
degenerative disorders with Parkinsonian features, such as bradykinesia, rigidity, tremor, and gait disturbances in common. They are associated with complex clinical presentation that reect degeneration of
various neuronal systems. However, because of the common Parkinsonian features these disorders have been collectively named Parkinson-Plus Syndromes.
Methods: We have examined our cases in the last 15 years and
among the 161 we have examined for primary Parkinsonism we had: 67
female and 94 male; 123 affected with PD, 9 with PSP, 5 with CBGD
and 21 with MSA. Therefore we had 35 persons who had a worse
prognosis than those with Parkinsons disease, because of complication
and unresponsiveness to l-dopa.
Results: We observe the problems related to our activity in the care
of patients with PPS in the territory and at the patients home.- The
main result of our study is to show how it is important to follow up

Movement Disorders, Vol. 22, Suppl. 16, 2007

patients with PPS in collaboration with other specialists, caregivers and

house physicians in order to avoid several complication such as aspiration, falls, hypotension, urinary infections and bedsores and situations
specic for MSA, CGBD and PSP.
Conclusions: We consider that the care of the patients with PPS is
very important not only to avoid complications, but also to relive
patient and families of a desperate feeling of abandon.
856
High dose levodopa therapy is not toxic in multiple system atrophy:
Experimental evidence
N. Stefanova, M. Kollensperger, M. Hainzer, A. Cenci, W. Poewe,
G.K. Wenning (Innsbruck, Austria)
Objective: The objective of the present study was to analyze
whether high dose levodopa delivery in a transgenic mouse model of
multiple system atrophy (MSA) is associated with neurotoxicity
exacerbated by the presence of oligodendroglial -synuclein inclusion pathology.
Background: Levodopa is generally regarded the rst choice therapy
for Parkinsonism associated with MSA. However, MSA patients often
show a poor or unsustained levodopa response which inicts high dose
therapy. This is generally attributed to progressive striatal degeneration
with loss of dopamine receptors. Experimental evidence suggests that
dopaminergic stimulation may accelerate the striatal disease process in
MSA, possibly by pro-oxidative mechanisms. Intact nigrostriatal dopamine release augments striatal lesion size in the unilateral nigral and
striatal double lesion rat model of MSA-P. Further, neuronal vulnerability to exogenous oxidative stress is increased in a transgenic MSA
mouse model with oligodendroglial -synuclein inclusions.
Methods: Control and transgenic MSA mice underwent pulsatile
treatment with either vehicle, low or high dose levodopa for a period of
one month. Locomotor activity in the experimental groups was
screened by open eld activity system. Further, immunohiostochemistry for tyrosine hydroxylase, DARPP-32 as well as classical cresylviolet staining were performed on serial sections to determine neuronal
numbers in substantia nigra pars compacta, striatum and cerebellar
cortex. Morphometry was performed using the optical fractionator
stereology method.
Results: Behavioural and neuropathological indices failed to show
evidence for neurotoxic effects of levodopa in this -synuclein transgenic MSA model.
Conclusions: In conclusion, we provide the rst pre-clinical evidence
that high dose levodopa therapy is not neurotoxic in the presence of
oligodendroglial MSA-like -synuclein pathology. The present results
support the safety of levodopa therapy in MSA.
857
PSP/CBD overlap a case series
S. Singhal, V.K. Gontu, D.P. Auer, N.B. Bajaj (Nottingham, United
Kingdom)
Objective: To describe three patients presenting with mixed features
of progressive supranuclear palsy (PSP) and corticobasal degeneration
(CBD).
Background: Patients with PSP typically present with early falls,
bulbar symptoms and a supranuclear gaze palsy (SNGP). Radiological
features include symmetrical midbrain atrophy. Patients with CBD
present with a jerky, rigid, apraxic limb and parkinsonism. Motor and
sensory cortical signs may be present with asymmetrical parietal,
frontal and paracentral cortical atrophy on imaging. There is controversy over whether these two 4 repeat tauopathies should be considered
distinct processes or part of a spectrum. Cases showing characteristic
features of each condition are a useful starting point for taking this
debate further.
Methods: Three patients who presented to our service between 2003
and 2004 were retrospectively identied. Clinical case records and MRI
scans were reviewed.
Results: Patients presented at ages 57, 59 and 60. All had early falls
and developed SNGP within a year of presentation. Over 3 years of

POSTER SESSION III, THURSDAY, JUNE 7, 2007

follow-up, all displayed markedly asymmetrical upper limb rigidity
sufcient to severely affect function, ipsilateral ideomotor and mimicry
apraxia, nger myoclonus, primitive reexes and pseudobulbar dysarthria. MMSE was 25, 28 and 28 at presentation with visuospatial and
executive function most severely impaired in all. Two had dystonic
posturing of an arm, and two had mild dysphasia with an extensor
plantar. MRI in the rst year of presentation showed markedly asymmetrical midbrain atrophy without focal cortical atrophy or ischaemia
in 2 of the 3. The other showed only minor ischaemic changes.
Conclusions: We present a case series of patients with clinical
features of CBD and PSP. We propose the term CBD-PSP overlap to
describe such cases, and suggest they represent an intermediate state in
a tauopathy disease spectrum. The nding of asymmetric midbrain
atrophy in two patients is atypical for either pure CBD or PSP and
would be worthy of further study in a larger case series.
References:
1. Nat Clin Pract Neurol 2006 Dec;2(12):658-65. Are frontotemporal lobar degeneration, progressive supranuclear palsy and corticobasal degeneration distinct diseases? Sha S, Hou C, Viskontas
IV, Miller BL.
2. Mov Disord 2005 Aug;20 Suppl 12:S21-8 PSP and corticobasal
degeneration: lumping versus splitting Scaravilli T, Tolosa E,
Ferrer. I
858
Anal sphincter EMG in the diagnosis of atypical Parkinsonian
syndromes
K. Winge, P. Jennum, A. Lokkegaard, L. Werdelin (Copenhagen,
Denmark)
Objective: To evaluate the value of anal sphincter EMG in the
diagnosis of atypical Parkinsonian syndromes, and to assess the patophysiological background of the changes.
Background: Diagnosis of atypical Parkinsonian syndromes (APS) is
primary based on clinical diagnostic criteria. However the clinical
pattern of Parkinsons disease (PD) and APS patients is variable and it
is often difcult to differentiate between these patients. There is therefore a need for diagnostic procedures which can differentiate between
the different PD-syndromes in early stages. Patients with APS, especially Multiple System Atrophy (MSA), may present neurogenic affection of the autonomic system, including the anal sphincter in contrast to
PD patients. The patophysiological background is probably related to a
dual innervation of the Onufs nucleus by somatic and autonomic
innervations thus expressing specic lesions of these systems.
Methods: Patients were included in a prospective manner on the
basis on fullment of criteria for probable disease according to
published diagnostic criteria for MSA, Progressive Supranuclear Palsy
(Steele-Richardson-Olszewskis syndrome, PSP) and Parkinsons disease (PD). 14 patients with MSA, 8 patients with PSP and 6 patients
with PD were included and investigated using anal sphincter electromyography (asEMG), EMG from m. gastrocnemicus (to exclude other
types of motor neuron lesions) and motor evoked potentials (MEP)
evoked from cortical and sacral stimulation to the anal sphincter.
Results: Data from all examinations are not available at this stage,
but table 1 outline preliminary data from asEMG) from 12 with MSA,
5 with PSP and 6 with PD. Remaining data will be available at the
presentation.
Conclusions: AsEMG may support the clinical diagnosis in the
differentiation between PD and APS, though it may not separate MSA
from PSP. Once data from the EMG of MEP examinations are available, conclusions regarding pathophysiological mechanisms may be
reached.

Duration (msec)
Amplitude (V)
% polyphasic
No normal (%)

PD

MSA

PSP

7.3*# (4.9-9.6)
295 (93-437)
12.8 (3-32)
6$ (100)

16.3* (7.5-24.8)
199 (36-468)
18.2 (0-48)
1 (8)

15.9# (12.5-21.5)
304 (101-788)
28.4 (0-59)
0$ (0)

(*p0.002, #p0.006, $p0.01, p0.05)

S261

859
Sleep disorders in Parkinsons disease: A correlation with clinical
characteristics
S.-M. Cheon, H.Y. Cho, M.J. Park, H.W. Lee, J.W. Kim (Busan,
Republic of Korea)
Objective: The authors conducted this study to clarify the correlation
of sleep disorders and the clinical characteristics of PD.
Background: Sleep disturbances are very common in the patient with
Parkinsons disease (PD) and several sleep disorders are known to have
pathological association with PD, such as REM sleep behavior disorder
(RBD), Restless legs syndrome (RLS) and periodic limb movement
disorders (PLMD).
Methods: Total 87 patients with PD (41 males, age 60.18.0 years,
Hoehn and Yahr stage 1-4) were recruited. We interviewed bed partners of parkinsonian patients for sleep disorders with semi-structured
questionnaire and compared interview results with the clinical characteristics of PD, including duration of symptoms and treatment, equivalent L-dopa dose, predominant motor symptoms, and the results of
cognitive function tests.
Results: RBD (26.4%), RLS (24.1%), PLMD (18.4%) and apnea
(17.2%) were frequently reported by bed partners of patients. PLMD
and apnea were more common in male patients and RLS in women.
Dose of L-dopa, predominant motor symptom, duration of symptom
and treatment were not related with each of these sleep disorders and
daytime sleepiness (Epworth sleepiness scale). The cognitive proles
of the patients also didnt show any association with sleep disorders but
verbal learning was negatively correlated with daytime sleepiness.
Conclusions: The pattern of sleep disorders was different depending
on the gender of patients with PD. There was no signicant association
between clinical characteristics of PD and specic sleep disorder but
daytime sleepiness was correlated with impaired memory function.
860
Parkinsonism as late-onset side effect of cerebral radiotherapy. A
case report
F. Zanini, M. Untereiner, W. Pilloy, M. Kruger, N.J. Diederich
(Luxembourg, Luxembourg)
Background: Parkinsonism has only rarely been described as sequela
of cerebral radiotherapy.
Methods: In 1990 a 31-year-old man underwent extensive neurosurgery for giant prolactinoma that had inltrated the chiasma, sphenoid
bone, carotid arteries and left temporal lobe. Because the tumor ablation was incomplete, focalized radiotherapy was performed, with an
isodose of 59 Gy calculated to cover the target tumor. 5 years later the
patient developed progressive Parkinsonism characterized by tremor at
rest, postural tremor and rigidity of the left arm, gait ignition failure,
freezing and absence of postural reexes. He also had psychomotor
retardation and questionable focal epileptic seizures. While the seizures
temporarily improved with valproic acid and levetiracetam, Parkinsonism was refractory to levodopa and dopamine agonists. In 2006 the
patient was admitted to our institution for further investigations.
Results: The apomorphine and levodopa challeging tests were negative. MRI excluded tumor relapse. Brain imaging by SPECT visualized involvement of pre-and postsynaptic dopaminergic pathways,
while cardiac MIBG-SPECT was normal. Retrospective radiotherapy
dose calculations applied to MRI slices performed in 2005 showed that
the treatment isodose had also covered the lower frontal border of the
striatum(see below). The patients condition remained essentially unchanged after progressive withdrawel of all dopaminergic medication,
replacement of valproic acid by lamotrigine and optimization of hormonal substitution.
Conclusions: Parkinsonism refractory to dopaminergic medication
could be a rare late-onset side effect of cerebral radiotherapy, performed before 3D treatment planning was available and applied in the
context of extended surgical procedure. Videoclips accompany this
presentation.Red line marks isodense zone of 58,9 Gy, including frontal
striatal parts.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S262 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

862
Red ags for multiple system atrophy
M. Kollensperger, K. Seppi, F. Geser, M. Sawires, N.P. Quinn,
V. Koukouni, P. Barone, M.T. Pellecchia, W.H. Oertel, N. Schimke,
E. Dupont, G. Deuschl, C. Daniels, N. Giladi, T. Gurevich,
C. Sampaio, M. Coelho, O. Lindvall, C. Nillson, A. Albanese,
F. Del Sorbo, E. Tolosa, A. Cardozo, T. Klockgether, M. Abele,
R. Djaldetti, G. Meco, C. Colosimo, W. Poewe, G.K. Wenning
(Innsbruck, Austria)

FIG. 1 (860).

861
Acute akinetic mutism due to subdural tension pneumocephalus
G. Luetjens, H.-H. Capelle, J.K. Krauss (Hannover, Germany)
Objective: To report a case with subdural tension pneumocephalus as
a cause of akinetic mutism.
Background: Parkinsonism with akinetic mutism is known to be
secondary to various causes such as normal pressure hydrocephalus,
supratentorial tumors, bilateral infarction of arteria choroidea anterior
and subarachnoid hemorrhage. Akinetic mutism caused by subdural
tension pneumocephalus associated with decompression of a chronic
subdural hygroma has not been described thus far.
Methods: A 56-year-old underwent subtotal resection of a right
parietooccipital glioblastoma. Six months later, he presented with
headache due to a right frontoparietal hygroma. A burr hole was
made and the subdural hygroma was drained. Twenty-four hours
later his clinical picture deteriorated and the patient presented with
severe parkinsonism in an akinetic mutistic state. The parkinsonism
features were rated according to the Unied Parkinsons Disease
Rating Score (UPDRS).
Results: An extensive subdural tension pneumocephalus due to a
presumed valve mechanism around the drainage was demonstrated
in CT imaging. On the UPDRS III motor subscore, the patient had
a score of 78. After removal of the drainage and implantation of
subdural-peritoneal shunt with programmable valve (ProGAV Aesculap) and replacing the air by Ringer solution the patients clinical
state improved within three days reected by a UPDRS III score of
11. The improvement in the UPDRS subscore and the clinical
presentation was mirrored by the decrease of intracranial air in the
postoperative CT scans.
Conclusions: While the development of various movement disorders is known to be associated with chronic subdural hematoma,
the occurrence of severe parkinsonism with akinetic mutism
caused by subdural tension pneumocephalus has not received
attention yet. Surgical revision rapidly may reverse this unusual
condition.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To dene a set of clinical warning signs i.e. Red Flags

suggestive for MSA.
Background: The clinical diagnosis of MSA is fraught with difculty
and there are no pathognomonic features to discriminate the common
parkinsonian variant (MSA-P) from Parkinsons disease. Existing published diagnostic criteria have adequate specicity but sensitivity is
suboptimal. Besides the poor response to L-dopa, and the additional
presence of pyramidal or cerebellar signs (ataxia) or autonomic failure
as major diagnostic clues, certain other features (red ags), may raise
the clinical suspicion of MSA.
Methods: A standardized red ag check list was administered within
the European MSA Study Group (EMSA-SG) to 58 patients with
probable MSA-P and 116 patients with probable PD matched for age
and disease duration. Red ags with specicity over 95% were selected
for further analysis. The resulting set was then applied to 17 patients
with possible MSA-P that on follow-up fullled criteria of probable
MSA-P.
Results: There was no signicant difference of age at examination
(p 0.241) and disease duration (p 1.000) between groups. Selected
red ags were grouped by factorial analysis into related categories.
With 2 or more of 6 red ags present specicity was 98.3% and
sensitivity was 84.5 % in our cohort. When applying these criteria to
patients with possible MSA-P, 76.5% of them would have been correctly diagnosed as probable MSA-P up to two years earlier than with
the consensus criteria alone.

EMSA Red Flags

Early instability
Rapid progression
Pisa syndrome or disproportionate
antecollis
Stridor or sighs
Severe dysphonia, dysarthria or dysphagia
Emotional incontinence

within 3 years of disease onset

wheelchair dependent 10
years from disease onset
prolonged episodes of lateral
trunk exion or severe neck
exion
Diurnal or nocturnal stridor
or involuntary deep
inspiratory sighs
inappropriately without
sadness or laughing without
mirth

Conclusions: We propose a combination of 2 out of 6 red ags as

additional diagnostic criteria for probable MSA-P.
863
Creutzfeldt-Jacob disease mimicking progressive supranuclear
palsy
F.M. Huber, V. Sazdovitch, U. Heinemann, S. Hak, J.J. Hauw,
F. Zanini, D.W. Droste, N.J. Diederich (Luxembourg-City,
Luxembourg)
Background: Sporadic Creutzfeldt-Jakob disease (sCJD) does not
always present with typical clinical signs. However, unlike other dementia syndromes it progresses rapidly and death ensues within a few
months. We present a case with particularly slow deterioration and
initial mimicking of Progressive Supranuclear Palsy (PSP).
Methods: Case observation.
Results: 67-year-old Italian woman with unremarkable family history has left-sided resting and postural tremor, unresponsive to dopaminergic medication and she experiences several falls. The neurological exam six months later reveals vertical gaze paresis, slowing of
horizontal saccades, bilateral bradykinesia and rigidity, and ideomotor

POSTER SESSION III, THURSDAY, JUNE 7, 2007

apraxia. The diagnosis of possible PSP is formulated, the differential
diagnosis being corticobasal degeneration (CBD). Neuropsychological
testing a month later also demonstrates marked cognitive decline
(MMSE score 13/30), associated with executive dysfunction (Frontal
Assessment Battery score 8/18). The encoding capacities are preserved.
The paraclinical tests are not contributory at that moment, with normal
EEG, marked, but unspecic atrophy at the MRI, and patchy zones of
hypometabolism on the HMPAO-SPECT. Temporary stabilization is
seen under treatment with donepezil. The patient is readmitted for
severe dysphagia 17 months after disease onset. She now discloses
intermittent, bilateral myoclonus, and almost complete mutism. EEG
reveals slow, rhythmic activity with sharp, sometimes bi- and triphasic
waves.There are increased levels of protein 14-3-3 and S100b in the
CSF. MRI visualizes hyperintensities of the cortical ribbon, the nucleus
caudatus and the putamen. The patient dies 19 months after disease
onset. At autopsy, the diagnosis of Creutzfeldt-Jakob study is established by neuropathological study, including positive PrP immunohistochemistry and Western Blot (showing Parchiis type I PrP). Other
pathologies that can be discarded, include PSP, CBD, Parkinsons
disease, Picks disease, argyrophilic grain disease and Alzheimers
disease.
Conclusions: The present case adds to the scare literature of slowly
evolving CJD mimicking Parkinsonism related to tauopathies. Critical
retrospective review of the initial clinical signs only identies the
horizontal saccade abnormalities and early apraxia as possible hints
orienting to sCJD.
864
Neurological features of Wilsons disease precipitated by liver
transplantation
K.E. Kotschet, A.J. Hughes (Fitzroy, VIC, Australia)
Objective: Case Report The debate concerning the pathogenesis of
neurological features in Wilsons disease continues. We present a case
which confronts some accepted concepts, and raises other issues regarding the risks of liver transplantation for patients with Wilsons
disease. We review the case of a 50 year old gentleman, newly
diagnosed with chronic liver disease due to Wilsons disease and with
minimal neurological involvement. The diagnosis was based on the
combination of low serum caeruloplasmin, elevated 24-hour urinary
copper excretion, presence of Kayser-Fleischer rings, and compatible
liver histology. Penicillamine chelation was tried for less than two
weeks, but was poorly tolerated and was discontinued. An orthotopic
liver was subsequently transplanted and excellent graft function was
attained. However, in the weeks to months following transplantation,
the patient developed extrapyramidal and frontal lobe disconnection
signs characteristic of neurological Wilsons disease. This deterioration
occurred despite evidence of biochemically normal copper metabolism.
Treatment with dimercaprol (BAL, British Anti-Lewisite) and zinc
sulfate has yielded no improvement at two years post transplant, and
there was no symptomatic gain from levodopa for Parkinsonism. It is
usual to see stabilisation or improvement of neurological symptoms
following liver transplantation for Wilsons disease. The occurrence of
profound neurological deterioration, as seen in this patient, has not
previously been reported. The mechanism underlying this is uncertain,
although we postulate unrestrained chelation therapy provided by the
transplanted liver, in an older patient relatively nave to decoppering
therapies. This case example supports the theory that symptomatic
neurological Wilsons disease is due to the toxicity of free copper,
rather than copper accumulation.
865
Pattern of cerebrospinal uid tau forms is altered in progressive
supranuclear palsy
A. Padovani, B. Borroni, F. Gardoni, L. Parnetti, L. Magno,
M. Malinverno, E. Saggese, P. Calabresi, M.G. Spillantini,
M. Di Luca (Brescia, Italy)
Objective: To characterise and measure Tau forms in order to verify
the differential patterns among neurodegenerative disorders.

S263

Background: Cerebrospinal uid (CSF) total Tau levels vary widely

in neurodegenerative disorders, thus being not useful in their discrimination over Alzheimer Disease. No CSF marker for Progressive Supranuclear Palsy (PSP) is currently available.
Methods: Seventy-eight patients with neurodegenerative disorders and 26 controls were included in the study. Each patient
underwent a standardized clinical and neuropsychological evaluation, MRI, and CSF total-Tau and phospho-Tau dosage. In CSF and
cerebral cortex, a quantitative immunoprecipitation was developed.
An extended (55kDa), and a truncated (33kDa) forms of Tau were
recognised. CSF samples were assayed, the optical density of the
two Tau forms was measured, and the ratio calculated (Tau ratio,
33kDa/55kDa forms).
Results: Tau ratio 33kDa/55kDa was signicantly decreased in patients with PSP (0.460.16) when compared to controls, including
healthy subjects (1.160.46, P0.002) and Alzheimer Disease
(1.380.68, P0.001), and when compared to Frontotemporal Dementia (0.980.30, P0.008) or Corticobasal Degeneration Syndrome
(0.980.48, P0.02). Moreover, in PSP patients Tau form ratio was
lower than in other neurodegenerative extrapyramidal disorders, such
as Parkinson Disease (1.160.26, P0.002) and Dementia with Lewy
Bodies (1.440.48, P0.001). Tau ratio 33kDa/55kDa did not correlate either with demographic characteristics, cognitive performances or
with motor impairment severity.
Conclusions: Truncated Tau production shows a different pattern in
PSP compared to other neurodegenerative disorders, supporting the
view of disease-specic pathological pathways. These ndings are
promising in suggesting the identication of a marker for PSP diagnosis in clinical practice.
866
Clinical characterization of a Chilean family with Kufor Rakeb
disease and mutations in ATP13A2, a lisosomal ATPase
M.I. Behrens, P. Chana, T. Parrao, P. Venegas, M. Miranda,
C.V. Rojas, A. Ramirez (Santiago, Chile)
Objective: To present the clinical description of 5 members of a
Chilean family with Kufor Rakeb disease and mutations in ATP13A2.
Background: Kufor Rakeb is a rare autosomal recessive disease of
juvenile onset characterized by slowly progressive parkinsonism, pyramidalism, and cognitive deterioration. Herein, we describe a family
affected with this disorder in which we have found two loss-of-function
mutations in ATP13A2, a neuronal P-type ATPase gene (Ramirez et al,
Nat Genet 2006;38:1184).
Methods: Data correspond to a prospective clinical examination of
affected and non affected members of a Chilean family over a period of
10 years. Medical records, neuropsychological testing and CT scans
were performed with previous informed consent and approval of the
ethics committee of the Hospital Sotero del Rio.
Results: The family is composed of non consanguineous parents
and 17 children from an area near Santiago, Chile. The last ve
siblings had normal development until ages around 12-18, when
insidiously started to show slowness of movements and decay in
school performance, with rigidity and difculty walking. When rst
evaluated, 2-5 years after disease onset, there was bradykinesia,
subtle tremor, cogwheel rigidity, stooped gait and spasticity. Some
showed visual hallucinations and upward gaze palsy. There was
little response to levodopa, although it was tried late in the disease
course. Disease evolution was slow, with progressive deterioration
of motor and mental activity until wheelbound and anarthric 15-20
years after. In advanced stages they showed facial-faucial-nger
mini myoclonus, insomnia and epilepsy. Three have died from
pneumonia after 20-27 years of disease. Some of the unaffected
members show subtle ocular dysmetria and ne tremor.
Conclusions: The affected family members showed a homogeneous
pattern of juvenile onset parkinsonism, piramidal signs, dementia,
supranuclear vertical gaze palsy, insomnia and facial-faucial-nger
mioclonus, very similar to the description of the Jordanian family
recently reported (Williams et al, Mov Disord 2005;20:1264). Interestingly, the presence of subtle ocular dismetria and ne action tremor in

Movement Disorders, Vol. 22, Suppl. 16, 2007

S264 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

some unaffected carriers suggests that haploinsufciency may cause
neurological symptoms.

FIG. 1 (866).

867
Atypical PSP: A representative case series
V.K. Gontu, D.P. Auer, N.B. Bajaj (Derby, United Kingdom)
Objective: To illustrate the clinical heterogeneity and difculty in
diagnosis of PSP.
Background: In recent years, the phenotype of PSP has been extended to include classical (Richardsons syndrome) and atypical cases
(PSP-P). Richardsons syndrome presents with vertical Supranuclear
Gaze Palsy, postural instability, falls, dysarthria, dementia, and
pseudobulbar palsy within the rst two years of onset. PSP-P can
present with parkinsonism (asymmetric bradykinesia, tremor and some
response to levodopa) but a vertical SGP can develop later in the course
of the disease. Advances in the MRI imaging diagnosis of PSP has also
extended the clinical phenotype with patients showing brain-stem features of PSP who have not yet developed vertical gaze palsy (see other
poster).
Methods: We present a case series of atypical PSP. None of these
patients had a supranuclear gaze palsy at presentation although one was
noted to have slowed vertical saccades.
Results: In 2 patients, the diagnosis became obvious clinically with
the subsequent development of a SGP after 3 years in patient 1 and 6
years in patient 2. In a further patient, the diagnosis has been suggested
on MRI imaging criteria although this patient has not yet developed a
SGP. Patient 1 presented to the psychiatrists with a dementia; patient 2
to the care of the elderly medical team with poorly responsive parkinsonism and patient 3 to the neurologist with dopa responsive Parkinsons disease.
Conclusions: This case series illustrates the heterogeneity in PSP
presentation and the difculties in diagnosing patients presenting late
with vertical SGP.
References
1. I Litvan, Y Agid, D Calne et al Clinical research criteria for the
diagnosis of progressive supranuclear palsy: report of the
NINDS-SPSP international workshop Neurology, Jul 1996; 47: 1
9
2. Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC,
Kilford L, Holton JL, Revesz T, Lees AJ. Characteristics of
two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardsons syndrome and PSPparkinsonism. Brain. 2005 Jun;128(Pt 6):1247-58. Epub 2005
Mar 23.
868
Isolated gait ignition failure: Abnormalities in pre- and post-synaptic dopamine receptor imaging
M.C. Kraus, U. Haberkorn, H.-M. Meinck (Heidelberg, Germany)
Objective: In the present study we investigated presynaptic dopamine reuptake and postsynaptic dopamine receptor density in the basal
ganglia of IGIF patients using DaT- and IBZM-SPECT scans.
Background: Isolated gait ignition failure (IGIF) is a rare disorder of
gait initiation with inability to start showing a short, broad-based
tripping instead. It is often provoked in particular situations, especially
when passing through narrow doors. There are no other features of e.g.
Parkinsons disease, normal pressure hydrocephalus or subcortical arteriosclerotic encephalopathy, and a lack of response to dopaminergic
medication is found.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Methods: We examined four patients, one woman and three men

(63-79 years), suffering from impairment of gait initiation with
sudden tripping when walking through narrow doorways or when
turning. Once started, their gait was uent and fast with a preserved
bilateral arm swing and normal length and width of stride. One
79-year old male patient suffered from dementia, urinal incontinence and mild rigity of his right limbs; another suffered from
dysarthria and hypophonia. CT and MRI scans did not show any
abnormalities. Imaging of 123I-DaTSCAN started 4 h after intravenous injection of 150-185 MBq; imaging of 123I-IBZM SPECT
began 1.5 to 2 h p.i. of 156-215 MBq 123IBZM. Ninety-six projections were obtained over 360 using a 3-headed gamma camera
(Multispect 3, Siemens, Germany) equipped with a medium energy
collimator. Data was registered for 60 s in each projection and
recorded in a 128 128 matrix. Images were reconstructed iteratively using the ordered subsets expectation maximization (OSEM)
algorithm. For semi-quantitative analysis regions of interest were
dened in 3 consecutive slices, and ratios of basal ganglia to a
reference region (frontal cortex) were calculated.
Results: In all patients a reduced uptake was seen for both tracers
which was most pronounced in the putamen.

Table 1: Uptake ratios of radiotracer in the basal ganglia

versus frontal cortex for DaT- and IBZM-SPECT (mean
values calculated from three slices).
Scan
normal
Patient
1
2
3
4

DaT-SPECT
2.5

IBZM-SPECT
1.5

Age*

Duration*

Right**

Left**

Right**

Left**

77
63
79
75

2
3
2
2

2.00
1.54
1.56
1.36

2.00
1.36
1.51
1.28

1.04
1.54
1.39
1.21

0.99
1.42
1.18
1.12

* years ** cerebral hemisphere

Conclusions: Our study shows that IGIF is associated with reduced
pre- and postsynaptic radiotracer uptake in the basal ganglia. This
nicely ts in with the Parkinsonian type of gait disturbance. It suggests
that IGIF nosologically is related to, but neither identical with Parkinsons disease nor with multiple system atrophy. Loss of postsynaptic
dopamine receptors could explain why these patients do not respond to
dopaminergic medication.
869
Clinical phenptypes of Parkinsons disease: How does psychopathology cluster with motor symptoms?
A.F.G. Leentjens, J. Reijnders, U. Ehrt, R. Lousberg, D. Aarsland
(Maastricht, Netherlands)
Objective: To identify subgroups of patients with Parkinsons disease (PD) characterized by specic constellations of psychopathological and motor symptoms.
Background: Based on the predominant motor symptoms, two clinical presentations of PD are distinguished: a hypokinetic-rigid and a
tremor-dominant form. It has been suggested that these clinical presentations a are also characterized by different prevalence of non-motor
symptoms, such as depression, apathy, and cognitive symptoms. Starkstein (1998) reported a higher prevalence of depressive symptoms in
the hypokinetic-rigid form of PD. No studies have attempted to replicate this nding.
Methods: A cluster analysis of motor and mental symptoms was
performed in 350 patients with PD as dened by the UK-PDS-BB
criteria. Patients were randomized over two samples: one used for
an exploratory cluster analysis, and one used for a conrmatory
analysis. Instruments used to assess patients included the UPDRS
and the Montgomery Asberg Depression Rating Scale (MADRS).
Individual items from these scales were used as indicators of different symptoms. The cluster variate is given in table 1.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Results: 175 patients participated in the exploratory analysis: 97
men and 78 women with a mean age of 70 (SD 9.9) years. The
average disease duration was 8.7 (SD 6.0) years, and the median
H&Y score was 2.5. The clinically most relevant solution was a six
cluster solution that identied the following clusters: one characterized by hypokinesia and rigidity without psychopathology (1),
one characterized by tremor without psychopathology (2), one characterized by depression and apathy (3), one characterized by apathy
without depression (4), one characterized by cognitive symptoms
(5), and an advanced PD group with severe motor and non-motor
symptoms (6) (table 2). Apathy, depression and cognitive symptoms
all clustered predominantly with hypokinesia and rigidity, and not
with tremor.
Conclusions: Apathy, depressed mood, and cognitive symptoms all
occur preferentially in patients with hypokinesia and rigidity, and not in
those with tremor. The preferred six cluster solution also conrms that
apathy may be present as an independent clinical syndrome in the
absence of depression. A conrmatory analysis will be conducted to
conrm the present solution.

the cluster variate

Motor variables

hypokinesia
rigidity
rest tremor
action tremor
postural instability
lack of facial expression

Psychiatric variables

UPDRS
UPDRS
UPDRS
UPDRS
UPDRS
UPDRS

depressed mood
suicidality
anhedonia
lack of initiative
cognition

part
part
part
part
part
part

III
III
III
III
III
III

item
item
item
item
item
item

22
31
20
21
30
19

MADRS item 2
MADRS item 10
MADRS item 8
UPDRS part I item 4
MADRS item 6

clusters and symptom loadings in the preferred six cluster

solution
cluster no.

1
2
3
4
5
6
apathy
uncomplicated
uncomplicated depression without cognitive advanced
symptom/cluster hypokinetic/rigid tremor dominant and apathy depression symptoms disease
rigidity
hypokinesia
rest tremor
action tremor
postural
instability
lack of facial
expression
depression
suicidality
lack of initiative
anhedonia
concentration
problems

S265

Methods: Each affected participant has had detailed clinical assessment of parkinsonian features including use of UPDRS and Hoehn &
Yahr scales and Video recordings (AJL). Each affected individual has
also had Dopamine Transporter (DAT) scans performed. Furthermore
6 members of the family (5 affected and one unaffected) have been
screened for mutations in PD genes: Parkin, DJ-1, PINK1 and LRRK2
and for mutations in GTP cyclohyrolase 1 gene (GCH1), the most
common cause of DRD.
Results: After extensive clinical assessment (AJL) 3 individuals
from the kindred have been diagnosed as having benign doparesponsive parkinsonism and 2 with DRD. The median age of
disease onset is 50 with a median duration of 20 years. DAT scan
images are available on 4 affected individuals and 1 unaffected
sibling. Clinical details and DAT scan results are summarised in the
table below. All 51 exons of LRRK2 have been sequenced and no
mutations found, results from screening of other genes are awaited.
Conclusions: Accurate clinical diagnosis has been challenging in
this family, indeed patient 5:5 was treated for almost 20 years for
PD, the diagnosis only recently being revised to DRD. DAT imaging has supported the clinical diagnoses in all but one case, patient
5:4. This patient has had slowly progressive dopa-responsive parkinsonism for 18 years but has a normal DAT scan. This result
suggests that this individual may fall into the category of patients
who have parkinsonism with a normal DAT scan or that there may
be overlap between Parkinsonism and DRD in this kindred. We
await the outstanding molecular genetic screening results to help
further clarify the diagnoses in this family.

Table 1: Patient age, gender, diagnosis, age of disease onset,

disease duration and DAT scan result
Patient
5:1
5:2
5:3
5:4
5:5
5:6

Age

Gender

Clinical
diagnosis

Age of
disease
onset

Disease
duration
(years)

DAT scan

82
80
78
68
66
26

Male
Male
Male
Male
Male
Female

Parkinsonism
Normal
Parkinsonism
Parkinsonism
DRD
DRD

58
N/A
75
50
44
6

24
N/A
3
18
22
20

Abnormal
Normal
Abnormal
Normal
Normal
Not done

871

1.24
1.12
0.76
0.22

0.96
1.00
2.63
1.41

1.74
1.57
0.96
0.39

1.49
2.09
0.66
0.17

1.05
0.85
0.65
0.60

2.25
3.17
1.42
0.50

Reversible parkinsonism in a patient with Whipples disease

E. Gasparoli, P. Zamboni, M. Siviero, R. Manara, R. Marcolongo,
N. Bonetto, C. Briani (Padova, Italy)

0.69

1.04

0.96

2.03

0.75

3.00

1.31
0.34
0.10
0.28
0.16

1.22
0.37
0.04
0.96
0.22

1.87
2.61
1.00
1.52
2.52

1.94
0.91
0.09
1.49
.43

1.00
0.90
0.50
0.95
0.50

2.58
2.00
0.25
2.50
1.08

0.28

0.96

1.61

1.91

2.65

3.08

Objective: We describe a patient with a long history of arthralgias

and fever who was diagnosed with Whipples Disease (WD) several
years after the onset of the symptoms. A Parkinsonian syndrome was
present which dramatically improved after antibiotic therapy.
Background: WD is a rare multisystem disorder caused by Tropheryma
Whippleii (TW), characterized by gastrointestinal symptoms, arthralgias
and fever. Neurological complications are dementia, seizures, ataxia, hypothalamic dysfunctions and movements disorders.Among the latter supranuclear gaze palsy and myoclonus are the most frequent.Oculomasticatory, oculofacioskeletal, myorhytmia are patognomonic of WD.
Methods: A 53 yr-old man with a 17 yr history of relapsing polyarthralgias, skin alterations, weight loss, was admitted because of remittent fever.
Lymphoadenopathy was present. The neurological examination revealed
resting tremor and rigidity in both arms. No known causes of parkinsonism
were identied. Brain CT and MRI were unremarkable.
Results: Histological examination of a lymphonode showed PASpositive macrophages, consistent with WD. Polymerase chain reaction
conrmed TW infection. The patient underwent a 12 month therapy
with trimethoprim-sulfamethoxazole with remission of systemic and
extrapyramidal symptoms. We did not perform brain biopsy or cerebrospinal uid since lymphonode biopsy was already informative.
Conclusions: The dramatic improvement of neurological symptoms
after antibiotic therapy together with the remission of systemic symptoms
without other known causes of Parkinsonism strongly support a causal
relationship between WD and the Parkinsonian syndrome. This is, to our
knowledge, the second case of Parkinsonism in WD reported. In the other

870
Clinical and imaging characteristics of a dominant kindred with
benign Parkinsonism and dopa-responsive dystonia
A.J. Lewthwaite, T.D. Lambert, D.J. Nicholl, V. Bonifati,
K.E. Morrison (Birmingham, West Midlands, United Kingdom)
Objective: To describe clinical and imaging characteristics of a
dominant kindred with benign Parkinsonism and Dopa-Responsive
Dystonia.
Background: During our study of familial Parkinsons disease (PD)
we identied a family in which there are currently 5 affected members
in two generations. Four of the affected individuals are male and one
female. The affected individuals have been examined (AJL) and show
a phenotype ranging from slowly progressive dopa responsive parkinsonism to dopa-responsive dystonia (DRD). From family reports there
were also two members of the previous generation who may have had
parkinsonism.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S266 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

case bradykinesia was prominent, whereas tremor was the main symptom
in our patient. In either cases the neurological manifestations were reversible after therapy.Trimethoprim-sulfamethoxazole crosses the blood-brainbarrier, probably prompting the recovery of neurological symptoms. Parkinsonism may be a rare neurological manifestation of WD. Therefore, in
presence of parkinsonism associated with otherwise unexplained systemic
the possibility of WD should be considered.

mance on the frontal neuropsychological assessment battery (Dementia

Rating Scale - initiation sub-score) at follow-up. Depression, anxiety
and fear of falling were not correlated with the presence of FOG at
baseline or follow-up.
Conclusions: These results indicate that FOG is common in HLGD,
and that it is associated with signicant functional disability and
specic frontal cognitive disturbance of initiation.

872

874

Progressive multifocal leucoencephalopathy (PML) presenting as

Parkinsonism in an HIV positive man
K.L. Poston, D.L. Raszl (New York, New York, USA)

Parkinsonism in antiphospholipid syndrome a case report and

literature review
Y.-R. Wu, Y.-C. Huang, R.-K. Lyu (Taipei, Taiwan)

Objective: To report a case of Parkinsonism as the presenting symptom in PML.

Background: PML is a demyelinating disease of the central nervous
system caused by JC virus infection. The JC virus commonly infects
children and persists into adulthood in the latent form with reactivation
almost exclusively in the immunocompromised host. It is estimated
that 5% of AIDS patients with develop PML. Although neurologic
complications of opportunistic infections are common in AIDS, movement disorders are exceptionally rare as the presenting and dominate
nding in PML. There are case reports which describe chorea, dystonia
and progressive myoclonic ataxia as the presenting symptom in PML.
Methods: We describe the clinical, radiological and laboratory features of a man with HIV in whom PML presented as Parkinsonism.
Results: A 52 year old man presented with mild right hand rest
tremor and mircrographia. He was diagnosed with HIV fteen years
prior and had been on HAART for eight years. His CD 4 count was 259
one month after onset. Four months later he had severe bilateral rest
tremor with illegible handwriting. MRI scan showed mild diffuse
atrophy but was otherwise normal. He had no improvement after
starting amantadine 400mg per day and carbidopa/levodopa 25/100mg
four times a day. Seven months later his postural stability was severely
impaired leading to frequent falls. Neurological examination showed
normal cognition with moderate asymmetric upper extremity rigidity
and generalized bradykinesia. He had left foot dystonia and poor heel
tap. His bilateral rest tremor persisted despite increasing carbidopa/
levodopa to 25/250mg four times a day. He also developed a partial left
homonomous hemianopsia. Two months later he had severe rigidity,
falls and could only walk with a walker. Confusion, xed delusions and
personality changes lead to weaning of amatadine and carbidopa/
levodopa without worsening in his motor exam. Repeat MRI showed
multifocal abnormalities within the right occipital lobe, basal ganglia
and midbrain which were patchy and diffuse. There was no mass effect
and only mild enhancement. Lumbar puncture had a normal cell count,
slightly elevated protein and positive JC virus PCR.
Conclusions: In patients with HIV and new onset Parkinsonism,
lumbar puncture with JC virus PCR should be considered for the
diagnosis of PML despite an initial normal MRI scan.

Objective: To report an extremely rare case of antiphospholipid syndrome (APS) presented with parkinsonism and provide a literature review
to address the clinical proles and possible pathophysiology of such
patients.
Background: The antiphospholipid syndrome (APS) is dened as vascular thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies (aPLs), including lupus anticoagulant (LA), anticardiolipin antibody (aCL), or anti2-glycoprotein-I antibody (anti-2 GPI).
The neurological presentations include cerebral ischemia, seizures, multiple sclerosis-like syndrome, myelopathy, dementia, migraine, GuillainBarre syndrome, movement disorders, and psychiatric disorders. The
exact pathogenesis of cerebral involvement in APS is still not completely
understood. Among the APS related movement disorders, chorea is the
most frequently described and the others include dystonia, ballism, parkinsonism and paroxysmal dyskinesias. However, there were few reports
of parkinsonism in association with APS.
Methods: A case report and a literature review.
Results: A 51 year-old woman developed right hand weakness,
followed by progressive memory impairment, dysarthria, and parkinsonism. She had no history of previous stroke, hypertension and
diabetes mellitus. On neurological examination, she had right hemiparesis, right central facial palsy, mild dysarthria, and generalized
hyperreexia; in addition, rigidity and bradykinesia were predominant on the right side, associated with stooped posture, short steps
and postural instabilities. The laboratory examination showed presence of anticardiolipin IgG/IgM antibodies, lupus anticoagulant, and
elevated anti-nuclear antibody titer (1:1280). The results tted the
diagnosis criteria of antiphospholipid syndrome. Magnetic resonance imaging (MRI) of the brain revealed hyperintensity lesions on
T2-weighted image and hypointensity on T1-weighted image at both
parieto-occipital regions and the periventricular areas, which were
compatible with old infactions. The 99mTc-TRODAT-1 SPECT of
the brain demonstrated symmetric and slightly decreased uptake in
bilateral striatum, as observed in vascular parkinsonism. Levodopa
and Warfarin were not effective for relieving her symptoms.
Conclusions: Parkinsonism may be one of the systemic manifestations of APS, but these symptoms are not responded to the levodopa
and anticoagulant treatment.

873
Freezing of gait in older adults with high level gait disorders:
Association with impaired executive function
N. Giladi, V. Huber-Mahlin, T. Herman, J.M. Hausdorff (Tel Aviv,
Israel)
Objective: To study the relationship between episodic gait disturbances such as freezing of gait, disease progression and cognitive
function in patients with High Level Gait Disorders.
Background: Freezing of gait (FOG) is frequently observed in High
Level Gait Disorders (HLGD), but its relationship to disease progression and cognitive function is unknown.
Methods: Episodic gait disturbances, affective state and cognitive
function were assessed in twenty-ve patients with HLGD (mean age:
78.25.0 yrs). After a mean of 32.24.2 months, twenty-two patients
were reassessed.
Results: FOG was observed in 20% of the patients at baseline and in
40% at follow-up. The presence of FOG was associated with signicant
mobility disturbances, functional deterioration as well as poor perfor-

Movement Disorders, Vol. 22, Suppl. 16, 2007

875
CSF hypocretin-1 levels are normal in multiple system atrophy
W.F. Abdo, B.R. Bloem, B. Kremer, G.-J. Lammers, M.M. Verbeek,
S. Overeem (Nijmegen, Netherlands)
Objective: CSF hypocretin levels analysis in MSA.
Background: Sleep disturbances occur in 70% of patients with
multiple system atrophy (MSA). Disturbances of the hypothalamic
hypocretin neurotransmission (secondary to loss of hypocretin producing neurons) have been suggested as a possible cause, but CSF
hypocretin-1 levels have thus far not been measured in MSA.
Methods: We included 6 patients with the Parkinsonian variant of
MSA (disease duration of 3.1 yrs) and 6 with the cerebellar variant
of MSA (disease duration of 4.4 yrs) and analyzed the CSF hypocretin-1 levels. Results were compared to those of a large control
group (matched for age and gender) tested previously in our laboratory.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Results: At the group level, mean CSF hypocretin-1 levels were
normal for both MSA-C patients (358 pg/ml) and MSA-P patients (329
pg/ml). Moreover, hypocretin-1 levels of each individual MSA patient
(range 241-425 pg/ml) were completely within the range of normal
control subjects (range 220-660 pg/ml).
Conclusions: Our results show that CSF hypocretin-1 levels are
probably not primarily correlated with sleep disturbances in MSA. Our
ndings also suggest that measuring CSF hypocretin-1 levels has no
value as a biomarker for sleep disorders in MSA.
876
Brain energy metabolism and effects of coenyzme Q10 (CoQ10) in
progressive supranuclear palsy (PSP)
M. Stamelou, U. Pilatus, K.M. Eggert, W.H. Oertel, G.U. Hoeglinger
(Marburg, Germany)
Objective: (1) To examine whether there is a decit in cerebral
energy metabolism in PSP patients compared to healthy controls; (2)
to evaluate whether oral treatment with CoQ10 (a) is safe and
tolerable, (b) improves cerebral energy metabolism and (c) ameliorates clinical symptoms in PSP.
Background: Experimental evidence suggests a mitochondrial
dysfunction in PSP. Particularly, an inhibition of mitochondrial
complex I has been implicated in the etiology of PSP. CoQ10 has
been shown to reduce the toxicity of complex I inhibitors in vitro
and in vivo. Consistently, rst evidence suggesting a slowing of the
disease progression in Parkinsons disease has been obtained in a
recent clinical trial.
Methods: We used phosphorus and proton magnetic resonance
spectroscopy (31P-MRS and 1H-MRS) to quantify adenosinetriphosphate (ATP), adenosine-diphosphate (ADP), phosphocreatinine (pCre), total creatinine (tCre), inorganic phosphat (Pi) and
lactate (Lac) in the frontal cortex and putamen of 20 PSP patients
and 10 age- and sex matched controls. The PSP patients were then
randomized to receive either CoQ10 (Sanomit, 5mg/kg/day) or
corresponding placebo over a six-week period in a prospective,
double-blind, randomized, placebo-controlled, clinical phase IIa
trial. Prior to and after the treatment period, these patients were
assessed with regard to (1) serum levels of reduced and oxidized
CoQ10, (2) cerebral energy metabolites measured by 31P-MRS and
1H-MRS, (3) motor dysfunction (UPDRS III, PSP rating scale and
staging system, Hoehn and Yahr stage), (4) neuropsychological
dysfunction (FAB, MMSE, MADRS) and (5) activities of daily
living. Clinical parameters such as vital signs and laboratory tests
were conducted. All adverse events were recorded.
Results: The mean age of the PSP patients was 65,86,2 and the
mean disease duration 3,41,6 years. No side effects related to the
medication were reported. No pathological alterations of blood
parameters were observed. Data acquisition has been completed.
Data analysis is in progress.
Conclusions: CoQ10 was safe and well tolerated in PSP. Data
analysis will be completed for the Movement Disorders Congress 2007
and will be presented there for the rst time to the public.
877
Levodopa response in parkinsonism with multiple mitochondrial
DNA deletions
R.A. Wilcox, A. Churchyard, H. Dahl, W. Hutchinson, D. Kirby,
D. Thyagarajan (Brisbane, QLD, Australia)
Objective: We have followed a patient with known mitochondrial
disease who later developed parkinsonism and have sought to assess
her response to levodopa (L-dopa).
Background: There have been several reports of parkinsonian
features responding to L-dopa in mitochondrial disease. Also there
is accumulating evidence that mitochondrial respiratory chain dysfunction may have a role in the development of idiopathic Parkinsons disease.

S267

Methods: Standard techniques for assessing mitochondrial enzyme

deciency and mitochondrial DNA techniques were used (further details will be provided in the poster presentation).
Results: Our patient presented with an autosomal dominant
chronic progressive external ophthalmoplegia phenotype associated
with multiple mitochondrial DNA deletions in muscle. She is from
a family with many affected members in which testing via linkage
analysis has excluded mutations at common sites including DNA
polymerase gamma (POLG), adenine nucleotide translocase
(ANT-1) or C10ORF2 (Twinkle).
Conclusions: The patient presents prominent parkinsonism characterized by prolonged benet from levodopa (L-dopa) and later the
development of L-dopa induced dyskinesias and motor uctuations.
Thus L-dopa responsiveness and L-dopa induced dyskinesias and
motor uctuations may also occur in atypical parkinsonism of
mitochondrial disease just as they may in Parkinsons disease plus
syndromes such as multiple system atrophy.

RESTLESS LEGS SYNDROME

878
A patient survey assessing symptomology and treatment trends of
Restless legs syndrome in the UK
S. Tluk, A. Bharkhada, E. Gill, K. Ray Chaudhuri (London, England,
United Kingdom)
Objective: To analyse a large database of members of the UK Ekbom
support group (ESG) which documents aspects of effects of RLS
including (1) timings and locations of RLS attacks (2) coping strategies
(3) visits to general practitioner (GP) (4) patterns of treatment initiated.
Background: Restless legs syndrome (RLS) is characterized by an
irresistible urge to move the legs usually with unpleasant sensations
and pain manifesting at night with symptomatic relief on movement.
RLS has been poorly recognised in the UK with delays to referral for
treatment exceeding 10 years.1
Methods: Out of a ESG database of 500 patients 332 were conrmed
to have RLS based on the IRLSSG criteria. Data from 292 patients have
been analyzed using the details as above.
Results: Of, the 292 patients (mean age 61.413.7 (SD) years;
M:F 80:210), 76.7% had nighttime symptoms while 5.1% had
dominant daytime RLS. Attacks included both legs in 63.4%; one leg
only 4.5%; legs and arms 15.1%; legs and whole body 3.8%. Specic
exercise was helpful in 21% while showers/ hot baths helped 28.4%.
200 had, at time of enrolment to ESG, visited their GP regarding
symptoms; 63 were given benzodiazepines, 36 quinine, 25 levodopa,
14 dopamine agonists and 62 other treatment including anti-depressants
and analgesics. Complimentary therapy were reported with 21 on
herbal treatment, 11 on homeopathy and 10 on acupuncture.
Conclusions: In the UK, many RLS patients remain undiagnosed and
up to 32% in this cohort had not seen a GP. Attacks have considerable
phenotypic heterogeneity including involvement of whole body and
many have been given prescriptions of sedatives, quinine and antidepressants and only 7% were prescribed dopamine agonists.
References:
1. Holmes R, Tluk S, Metta V et al. Nature and variants of idiopathic restless legs syndrome: observations from 152 patients
referred for secondary care in the UK. J Neural Transmission. In
Press.
879
Lack of drug drug interactions between transdermal rotigotine and
oral contraceptives
M. Braun, J.-P. Elshoff, J.-O. Andreas, B. Strauss, R. Horstmann
(Monheim am Rhein, Germany)
Objective: This study investigated the effects of transdermal rotigotine comedication on the pharmacodynamics and pharmacokinetics of
oral contraceptives.
Background: Rotigotine is a D3/D2/D1 dopamine agonist which is in
clinical development as a once-daily transdermal system for the treatment

Movement Disorders, Vol. 22, Suppl. 16, 2007

S268 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

of Restless legs syndrome (up to 3mg/24h). This disease is prominent in
female subjects involving women of childbearing potential.
Methods: The inuence of transdermal rotigotine (3mg/24h) oncedaily on the suppression of ovulation by oral hormonal contraception
(0.03mg ethinylestradiol, 0.15mg levonorgestrel) was investigated in a
randomized, double-blind, placebo-controlled, crossover, multiple dose
trial in 43 healthy female subjects. Primary variables were progesterone
serum levels on days 19-21 of the respective menstrual cycle. Additional pharmacodynamic variables were: estradiol (days 10, 13, 14,
19-21) FSH and LH (days 10, 13, 14) serum levels. Pharmacokinetic
parameters for the oral contraceptive were Cmax,ss, tmax,ss, and
AUC(0-24)ss values for ethinylestradiol and levonorgestrel.
Results: The oral contraceptive led to suppression of progesterone
serum levels 2ng/mL under concomitant treatment with rotigotine as
well as with placebo, indicating suppression of ovulation. Accordingly,
FSH, LH and estradiol values were within the reference range for
non-ovulatory female subjects under treatment with oral contraceptive
and concomitant treatment with rotigotine or placebo. Plasma concentrations-time proles for ethinylestradiol and levonorgestrel were similar under concomitant treatment with rotigotine and placebo. The
statistical analysis showed ratios of geometric means near 1 for the
pharmacokinetic parameters Cmax,ss and AUC(0-24),ss of both, ethinylestradiol and levonorgestrel. The respective 90% condence intervals were within the acceptance range for bioequivalence (0.8;1.25).
Conclusions: These results demonstrate that transdermal rotigotine
(3mg/24h) does not interfere with pharmacodynamics and pharmacokinetics of oral contraceptives.
880
Clinical characteristics and prevalence of Restless legs syndrome in
orhangazi district of Bursa, Turkey (a population-based door to
door study) (Bursa, Turkey)
S. Erer, M. Zarifoglu, N. Karli, S. Akgoz, C. Cavdar (Bursa, Turkey)
Objective: To determine the clinical ndings and prevalence of
Restless legs syndrome (RLS) among 40 years and older population in
orhangazi district of Bursa, Turkey.
Background: RLS is relatively common, occuring 5-15% of the total
population.
Methods: This population based study was planned in 2 phases. In
phase 1; door to door home interviews were performed by residents
using a short questionnaire that included questions about symptoms
specically related to RLS. In phase 2; suspected participants in the
rst phase were evaluated according to standardized diagnostic criteria
and the use of scales for RLS by movement disorders experts and a
diagnosis has been made.
Results: This study has been performed between May 2004 and
August 2006 in orhangazi district of Bursa, Turkey. 1256 of the target
population has been screened according to the study questionnaire. 162
cases with suspectful symptoms of RLS have been detected in phase 1.
49 of suspected cases could not be evaluated in phase 2. A diagnosis of
RLS also has been made in 60 cases in phase 2. The prevalence of RLS
did not differ by age and gender. Results show that RLS is signicantly
related to choronic heart disease and asthma.
Conclusions: We dened that prevalence of RLS is 6,428 % among
40 age and older population. We suggest that our ndings of prevalence
rate also correlate with reported data.
881
Restless legs syndrome in Parkinsons disease own experience
A. Budzianowska, M. Golab-Janowska, K. Honczarenko (Szczecin,
Poland)
Objective: The aim of the study was to assess the frequency of
Restless legs syndrome (RLS) in Parkinsons disease (PD) and its
inuence on daily living patients.
Background: Restless legs syndrome (RLS) is a sleep and movement
disorder, manifested in an irresistible urge to move the legs relived by
motor activity and worsening during the rest and in the evening.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Methods: The study was performed in 96 PD patients, 49 men and 47

women of a mean age 67.9 years (42-83). They were subjected to
neurological examination and were asked to ll an original questionnaire based upon diagnostic criteria of the RLS created by International
Restless Legs Syndrome Study Group.
Results: Nine PD patients fullled criteria for RLS (9.47%), 7
women and 2 men. In our study the frequency of RLS in PD is
comparable to other studies. RLS in patients with PD is more frequent
among women (p0.06), patients with longer L-Dopa treatment
(p0.04) and in patients with domination of rigidity (p0.007). Symptoms of RLS in PD are: moderate, observed only in lower limbs, take
an average 2,6 hours per day and in average 3.25 day per week. RLS
in patients with PD cause increased frequency of sleep disturbances,
incuding problems with initiation of sleep (p0.0006), waking up
several times per night (p0.01), what can lead to excessive fatigue
during a day (p0.07), disturbances of mood (p0.004), avoiding
contacts with people (p0.004), increased frequency of accidents in
daily life (p0.001) and headache (p0.008).
Conclusions: Coexistence of RLS with PD has signicant inuence
on daily life of patients. It causes sleep disorders, disturbances of mood,
headache and increased frequency of accidents in daily life.

882
Restless legs syndrome (RLS): A community-based study from
Argentina
G. Persi, A. Ayarza, J.L. Etcheverry, V. Parisi, G. Pariso,
E.M. Gatto (Buenos Aires, Argentina)
Objective: To estimate the prevalence of RLS in a community-based
study in Buenos Aires, Argentina.
Background: RLS prevalence estimates range from 2.5% to 10% in
the North American and Northern European population, with lower
prevalence rates in Asian populations (0.1-0.7%). Only two studies
were performed in South America.
Methods: We conducted a community-based study to estimate the
prevalence of RLS in a cohort of patients from the city of Buenos Aires
and from two rural areas of the Province of Buenos Aires with 30,000
inhabitants. A self-assessment questionnaire, including the IRLSSG
diagnostic criteria, was used to determine RLS symptoms. Two questions were added to assess RLS knowledge and symptoms frequency in
our sample. We used the t-test and the chi-square test to compare
RLS-positive and RLS-negative subjects. All p-values 0.05 were
considered statistically signicant. Values were expressed as mean
SD or percentage.
Results: We analyzed 433 questionnaires (mean age: 42.5717.66
years) completed by 251 women (mean age 43.2217.13) and 182 men
(mean age 41.6618.38). The 4 essential RLS diagnostic criteria were
present in 20.79% (90/433 individuals, 67 women and 23 men, with a
mean age of 47.4719.46 years, range 16-83) and 15.01% reported
symptoms occurring at least 2 days a month. Patients with RLS were
signicantly older than those without RLS (47.4719.46 vs.
41.2816.96 years; p0.003). RLS symptoms were reported signicantly more often by women than men (26.69% vs 12.64; p0.0004).
RLS was more frequently reported by elderly patients (32.94% in 60
years-old vs 16.88% in 40 years-old). Of the total sample, 55 subjects
were physicians; 83.64% of them knew about RLS. Regarding the rural
population, we included 32 individuals (mean age: 45.3720.42 yearsold), 16 women; with a RLS prevalence of 18.75%.
Conclusions: In the present study, RLS has been shown to be a
common disorder. In accordance with other studies, we found a female/
male ratio of 2.9:1 being women younger than men. No relevant
differences were found between the rural and urban population. Interestingly, RLS prevalence was very similar to that reported by Nichols
et al (2003) in a primary care setting in the USA (15.30%) and different
from that reported in native South Americans.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

883
Botulinum toxin a treatment can improve symptoms of Restless
legs syndrome
D. Richardson, R. Bajwa, M. Eisa, D. Miller, V. Mohsenin,
B. Jabbari (New Haven, Connecticut, USA)
Objective: Restless legs syndrome (RLS) is associated with uncomfortable sensory symptoms. In many, these symptoms are most prominent in the lower limbs, in the region of the tibialis anterior muscle.
We hypothesize that an alteration in the sensory system involving the
tibialis anterior muscles also contributes to the increased motor activity
seen in patients with RLS. We propose that treatment of these muscles
with botulinum toxin A, which affects both the sensory and motor
systems, can improve lower leg symptoms.
Background: RLS is characterized by sensory complaints that
worsen in the evening and are associated with an urge to move the legs.
There has been some success in treatment of RLS with dopaminergic
agonists, but treatment failures are not uncommon. Two previous
observations (published abstracts) report improvement in RLS symptoms following injections of botulinum toxin A into the tibialis anterior
muscles (Kudelko et al. Mov. Disord. 2002: one patient; Rotenberg et
al. J.Clin. Sleep Med. 2006: three patients).
Methods: Twelve patients with RLS were enrolled in an open labeled
protocol. Ten of the twelve had predominantly lower limb sensory
symptoms. Polysomnography was performed at baseline and at 4-6
weeks after treatment. A neurological examination and the RLS rating
scale was performed at baseline and at 1, 2, 3 and 4 months after
treatment. Each tibialis anterior muscle was injected with forty to fty
units of botulinum toxin A (BOTOX, Allergan-Inc.).
Results: There were 3 males and 9 females with a mean age of 60.5
(/- 10.4 SD) enrolled. Botox injections resulted in a statistically
signicant decrease in symptoms of RLS in the patients with predominantly lower limb symptoms (see gure 1). There was no affect on the
frequency of periodic leg movements or the arousal index. No side
effects were reported.
Conclusions: RLS patients with prominent lower leg sensory symptoms may benet from botulinum toxin A treatment of tibialis anterior
muscles. Verication by randomized, blinded studies is recommended.

FIG. 1 (883).
884
Restless legs syndrome and chiari type 1 malformation
Y. Kaplan (Tokat, Turkey)
Objective: In this presentation, we will discuss 5 cases of Restless
legs syndrome (RLS), who had early onset and bad response to therapies and who were also considered to be possibly related to Chiari
type 1 Malformation (CM-1).

S269

Background: The Chiari Malformation is a developmental anomaly

at the base of the brain which results in the downward displacement of
some of the brains structures into the spinal canal. These malformations have been subdivided to reect the degree of displacement as well
as the varying etiology of the malformations. Type 1 malformations
consist of a downward displacement of the cerebellar tonsils out the
inferior opening of the skull into the spinal canal. There are studies in
the literature drawing attention to the possible role of upper brainstem,
diencephalon, red nucleus and cerebellum in the pathogenesis of RLS.
Methods: We currently follow up 5 RLS cases who were also
diagnosed as CM-1 at our neurology polyclinic. The patients fullled
the diagnostic criteria for RLS according to the International Restless
Legs Syndrome Study Group. None of the patients had familial RLS
history or other conditions known to be related to RLS.
Results: Four of these patients were female and 1 of them was male.
The age range was between 20 and 32. The duration of the disease was
between 2 to 12 years. Four of the patents indicated occasional pain in
occipital-suboccipital area upon questioning CM-1 related symptoms.
One case was totally asymptomatic. The neurological examination of
patients was normal. The standardized laboratory tests and electroneuromyographies were normal. All cases had used at least 2 different
drugs known to be effective in RLS treatment (such as L-dopa, dopamine agonists, antiepileptic drugs). The Johns Hopkins Restless Legs
Severity Scale values were 2 for 2 cases and 3 for 3 cases. There was
no accompanying intracranial, parenchymal or cervical lesion other
than CM-1 in brain and cervical MRIs of patients.
Conclusions: CM-1 related involvement of cerebellum and/or connections of cerebellum with basal ganglia may have contributed to
development of RLS in these cases.
885
Disruption of working life among persons with moderate to severe
Restless legs syndrome
E. Lainey, S. Albrecht, J. Koester (Ridgeeld, Connecticut, USA)
Objective: In the course of enlisting restless legs syndrome (RLS)
patients for a clinical trial, we obtained data permitting quantitative
assessment of the syndromes impact on patients working lives.
Background: Because (RLS) begins or worsens during a patients
attempts to rest, it typically disrupts sleep, causing detriments that may
extend into daytime.
Methods: For a 12-week randomized, double-blind trial of the dopamine agonist pramipexole, adults with RLS were recruited to receive
either the active drug or placebo. At baseline, all subjects met diagnostic criteria of the International RLS Study Group and had a score
15 on the Groups RLS severity scale (IRLS). To characterize the
syndromes baseline impact on quality of life, subjects completed the
Johns Hopkins Restless Legs Syndrome Quality of Life (RLSQOL)
questionnaire, an RLS-specic tool whose questions include several
exploring whether the patient works full- or part-time and encounters
difculties in doing so.
Results: Among 344 recruited patients (62.5% women), the mean
age was 51.4 years (/13.0) and the mean RLS duration was 3.1 years
(/ 6.5). The mean IRLS score was 23.5 (/5.1), signifying moderate to severe RLS. Among the 337 patients with analyzable RLSQoL
data, 74.5% (251) were working (Question 14), with 77.3% of them
(194 of 251) reporting at least a 7-hour work day (Question 17). But
among workers, 36.3% (91 of 251) had found it difcult to work a full
day at least a few times during the preceding month (Question 15), and
32.0% (79 of 247) sometimes worked fewer hours than they would like
(Question 18). Eight patients ascribed their absence of employment to
RLS.
Conclusions: Although most RLS patients were working and most of
the workers reported a full-length workday, substantial proportions also
reported RLS interference with working life, as indicated by increased
difculties and decreased work-time.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S270 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

886

888

Clinical characterization of familial and sporadic Restless legs

syndrome
L.A. Brown, S.-C. Lin, J.E. Young, R.J. Uitti, Z.K. Wszolek
(Jacksonville, Florida, USA)

Pramipexole is effective treatment for RLS patients suffering from

afternoon or early evening RLS symptoms
A.S. Walters, E. Lainey, J. Koester (Edison, New Jersey, USA)

Objective: To investigate the clinical differences between familial

and sporadic Restless Legs Syndrome (RLS).
Background: RLS is a neurological condition characterized by a
distressing need or urge to move the legs, usually accompanied by
uncomfortable sensation in the legs described as a crawling, muscle
ache or tension. While it has been documented that severity of RLS
increases with age, very little is known about the severity of RLS
based on heritability. Several studies have demonstrated the prevalence of RLS in women is nearly twice that of men.
Methods: Medical and genealogical records of patients with RLS
were analyzed. The International Restless Legs Study Group (IRLSSG)
criteria were used to assess severity of RLS. Familial RLS was dened
as having two or more rst degree relatives with a physician conrmed
diagnosis of RLS.
Results: Mean age at onset (AAO) of familial samples (n64) was
41.8 years while sporadic was 53.29 years (p0.035). All patients with
mild RLS reported having a positive family history. Patients with
moderate, severe, and very severe RLS reported a positive family
history 70%, 53%, and 73% of the time, respectively. Male to female
ratio of RLS affected patients who reported only mild or moderate RLS
was 1:2, while those reporting severe or very severe RLS had a male to
female ratio of 1:1.
Conclusions: These statistics demonstrate that AAO of familial RLS
is signicantly younger than sporadic RLS, although case ascertainment bias may confound this observation. Patients with mild to moderate RLS were more likely to report a positive family history and were
more commonly female. Patients with severe to very severe RLS were
less likely to report a family history and gender distribution of affected
individuals was equal. This data suggests that familial RLS may be less
severe than sporadic RLS. This interpretation is preliminary because of
small sample size and potential case ascertainment bias. Further clinical
studies of RLS are required.

887
Pregabalin in Restless legs syndrome with and without neuropathic
pain
M. Sommer, C.G. Bachmann, K.M. Liebetanz, J. Schindehutte,
T. Tings, W. Paulus (Goettingen, Germany)
Objective: To explore the effectiveness of pregabalin in Restless legs
syndrome (RLS) in a pragmatic clinical setting.
Background: RLS is a common neurological disorder complicated in
many patients by augmentation to dopaminergic therapy or comorbidities such as neuropathic pain.
Methods: After observing improvement of restless legs symptoms in
seven patients treated with pregabalin for neuropathic pain, we extended the clinical observation to a total of sixteen patients with
secondary RLS, in most of them due to neuropathy, and to three
patients with idiopathic RLS.
Results: Three patients discontinued pregabalin because of side
effects (rash, fatigue, loss of efcacy). The other sixteen patients
self-rated a satisfactory or good alleviation of RLS symptoms and
maintained pregabablin, ve with add-on medication, on a mean daily
dose of 305 mg (standard deviation, 185 mg), and with a mean duration
of 217 (standard deviation, 183) days.
Conclusions: This data proposes pregabalin as a new option in the
treatment of secondary RLS for patients with neuropathic pain, which
should be further investigated with randomised, placebo-controlled
trials.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: Because pramipexole is dosed once daily (2-3 hours

before bedtime) for treatment of restless legs syndrome (RLS), it is
important to determine its effectiveness in patients who experience an
onset of RLS symptoms in the afternoon or early evening.
Background: Symptoms of RLS display a circadian pattern with
worsening in the evening and night that is hypothesized to correlate
with oscillations in central dopaminergic function. Although most
patients experience onset of symptoms late in the evening, a smaller
subset report symptom onset earlier in the day. Pramipexole is a
D3/2-selective dopamine agonist with a half-life of 8-12 hours.
Methods: The primary efcacy endpoint (International RLS Study
Group Rating Scale [IRLS] total score) from a double-blind, multicenter trial was analyzed in the subset of patients with early-onset
symptoms (designated as those occurring from 3 -9 pm at baseline) and
in the total population. Fixed doses of pramipexole (0.25-0.75 mg QD)
or placebo were administered daily 2-3 hours before bedtime for 12
weeks. 12 weeks.
Results: Table 1 displays IRLS results for the early-onset and
total populations. Pramipexole was statistically signicantly superior to placebo in patients with early-onset symptoms. This result
was similar to the difference between treatments in the total population.
Conclusions: Pramipexole is effective in patients with RLS symptom
onset in the afternoon and early evening. A probable explanation for
these results is the long half life of pramipexole with up to 1/2 of the
drug being present after 12 hours and up to 1/4 of the drug being
present after 24 hours. Additional research may further explain this
effect.

Change From Baseline to Final Visit in IRLS Total Score

Total Population (N 339)

Baseline (SD)
Adjusted* mean
change
Adjusted* difference
(95% condence
interval)
P-value

Early-Onset Population
(N 159 [47%])

Pramipexole
(n 254
[75%])

Placebo (n 85
[25%])

Pramipexole
(n 118
[75%])

Placebo (n 41
[25%])

23.4 (5.1)

23.5 (5.2)

23.6 (5.2)

23.7 (5.4)

-13.5

-9.3

-13.9

-8.7

-4.3 (-6.4, -2.1)

0.0001

-5.1 (-8.5, -1.8)

0.0031

*ANCOVA adjusted for baseline and age.

889
Burden of illness associated with Restless legs syndrome: Findings
from patients visiting primary care settings in the US
R.P. Allen, W.J. Kwong, M.O. Calloway, L. Palmer (Baltimore,
Maryland, USA)
Objective: To assess the perceived burden of illness associated with
Restless legs syndrome (RLS), using patient-reported outcome measures in US primary-care settings.
Background: RLS is associated with signicant sleep disturbance
and may have a negative impact on patients health and well-being.
Methods: A major objective of this multicenter, 12-week, longitudinal study (protocol RRL104025) was to assess the prevalence and
burden of disease associated with RLS in patients presenting at US
primary-care settings. 139 primary-care physicians screened 25,397
patients consecutively visiting their practices for RLS using IRLSSG
diagnostic criteria. Up to 10 individuals with RLS per investigator were
then recruited to the 12-week longitudinal phase. Patients were given
the Johns Hopkins RLS Quality of Life questionnaire (RLSQoL), the
Hospital Anxiety and Depression Scale (HADS), and the Medical
Outcomes Study (MOS) Sleep Scale.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Results: Of 25,397 individuals screened, 3124 (12.3%) had a conrmed diagnosis of RLS. 1,027 individuals with RLS (91% not treated
with a dopamine agonist for RLS) were enrolled in the 12-week study,
and of these, 923 (89.9%) completed. At baseline, RLSQoL mean
overall life impact score was 65.5 (range 0 100; 65 indicates moderate
impact and 92 indicates no signicant impact based on validation
studies). At baseline, 45% and 30% of patients had HADS scores
denoting at least moderate anxiety and depression, respectively (score
10 on anxiety/depression subscales). As reported by the MOS Sleep
Scale at baseline, 44% of patients reported getting enough sleep little
or none of the time, 47% reported having trouble falling asleep all or
most of time, 40% reported that they awaken and have trouble going
back to sleep all or most of time, and only 13% reported getting the
amount of sleep needed.
Conclusions: Based on a large-scale survey of US primary-care
settings, the burden of disease in patients with RLS is substantial in
areas of mental health, sleep, and quality of life.
890
Restless legs syndrome and menopause
Y. Kaplan, H. Aytan, F. Demirturk, A.C. Caliskan (Tokat, Turkey)
Objective: The aim of our study was to evaluate the prevalence of
Restless legs syndrome (RLS) in a large sample of postmenopausal
women. We also investigated relation between RLS and clinical features of menopause.
Background: Some animal and human studies indicate that estrogen
can modulate dopaminergic activity. Both central and peripheral portions of nervous system contain vast estrogen receptors. Therefore,
hypoestrogenemic status of the menopause - which is characterized by
decreased ovarian follicular response to gonadotropins and estrogen
can be expected to to have affect on the nervous system at a multitude
of anatomical or functionel sites.
Methods: 523 women with menopause were enrolled in this prospective study. Patients with gynecologic and non-gynecologic malignancy, patients taking chemotherapy and radiotherapy treatment, patients with peripheral vascular disease, and cases who were still taking
or had taken hormone replacement therapy for more than six months in
the past were excluded. 334 (63.86%) did not meet the inclusion
criteria used in the current study and were subsequently excluded. 189
postmenopausal patients were evaluated. 73 of them had all RLS
criteria. 47 of them had familial history or at least one factor related
with RLS. Because of this, these patients also were excluded. 26 of the
rest 142 patients (18.3%) had no factors related with RLS. These
patients were classied as group 1 and a control group comprised of 24
postmenopausal women that were free of RLS.
Results: Onset age of menopause was earlier and surgical menopause
was more frequent in group 1. The Pearson correlation between onset
ages menopause and RLS is strong and signicant.
Conclusions: Estrogen can modulate dopaminergic activity in the
nigrostriatal system. The data concerning neurobiological interactions
of estrogen with nigrostriatal dopaminergic system point out to the
possibility that hypoestrogenemia in menopausal women may be related to some kind of menopausal RLS.
891
Pramipexole for Restless legs syndrome (RLS) in patients with
comorbid cardiovascular (CV) disease
J.W. Winkelman, E. Lainey, J. Koester (Brighton, Massachusetts,
USA)
Objective: Our objective was to determine pramipexoles efcacy in
RLS patients with comorbid CV disease or risk factors.
Background: The prevalence of CV disease is elevated among patients with daily RLS symptoms and has potentially serious consequences. Therapies used to treat RLS in patients with CV disease need
to be effective while not interacting with concomitant CV drugs or
increasing adverse event (AE) risk. Pramipexole is a non-ergoline,
D3/2-selective dopamine agonist, which is renally eliminated and has no
predicted CYP-450 drug-drug interactions.

S271

Methods: Data from 3 double-blind RLS trials (duration: 3-12

weeks) were pooled. Pramipexole was dosed from 0.125 to 0.75 mg
QD. Change from baseline to nal visit was analyzed via ANCOVA
(adjusted for baseline and age) for the primary endpoint (International
RLS Study Group Rating Scale [IRLS]) in the subset of patients with
comorbid CV disease or risk factorsincluding arrhythmia, coronary
artery disease, heart failure, diabetes, hyperlipidemia, hypertension,
and myocardial infarctionand in the subset without them. AE rates
were also calculated by subset.
Results: Over a third of patients (34.7% or 272/784) had CV comorbidities. The placebo-adjusted difference from baseline for IRLS total
score was -5.4 1.3 (P 0.0001) in the CV group and -6.5 0.9
(P 0.0001) in the non-CV group. In the CV group, the AE rate was
74.9% for pramipexole and 71.4% for placebo; in the non-CV group it
was 72.4% for pramipexole and 59.4% for placebo. AE-associated
withdrawals occurred in 7.2% on pramipexole and 5.2% on placebo in
the CV group versus 6.5% on pramipexole and 4.2% on placebo in the
non-CV group.
Conclusions: Pramipexole was effective for RLS in patients with CV
comorbidities and was well tolerated. Few patients discontinued for
AEs and AE rates were similar in patients with and without CV disease.

892
Restless arms syndrome heralding MGUS-related anti-MAG polyneuropathy
J. Horvath, T. Landis, P.R. Burkhard (Geneva, Switzerland)
Objective: To describe a case of Restless arms syndrome lasting for
over ten years until adequate therapy was started. The patient was
eventually diagnosed with polyneuropathy associated with monoclonal
gammopathy of undetermined signicance (MGUS) and anti-myelinassociated glycoprotein (MAG) antibodies.
Background: Arm restlessness may be associated with typical Restless legs syndrome (RLS) in 22-50% of patients, especially with severe
RLS. Occasionally, RLS may even start in the upper limbs before
invading the legs. Periodic arm movements have also been reported in
RLS patients. To the best of our knowledge, however, isolated restless
arms syndrome has not been reported before.
Methods: The 59-year-old patients symptoms started insidiously
more than 10 years ago and progressively worsened over the past 5
years leading to severe insomnia. 5 to 15 minutes after going to bed, he
started feeling a creepy sensation in both arms that he described as
itching and burning with an urge to move. Stretching and scratching his
arms or putting them under running cold water offered some temporary
relief, but needed to be repeated several times a night. His wife noticed
repetitive stretching of his arms while he was asleep and periodic leg
movements for the last year as well. The patient was symptom-free
during the day. The condition was misdiagnosed as a chronic allergic
disorder and ineffectively treated for many years. Two years ago he
started taking pramipexole. A single daily dose of 0.5 mg at night made
the symptoms completely disappear and it has remained efcient. 8-9
months ago, while on pramipexole, the patient developed a progressive
sensorimotor polyneuropathy with the characteristics of an MGUS
anti-MAG polyneuropathy.
Results: This patient presented with symptoms located in his arms
which, were they to have occurred in the legs, would have met the
clinical diagnostic criteria of RLS, including a remarkable benet from
low doses of a dopamine agonist. Because symptoms were restricted to
the upper limbs for many years, we propose to label it restless arms
syndrome. Our case can be referred to as a symptomatic Restless arms
syndrome as it preceded a polyneuropathy. We have no explanation as
to why legs were unaffected.
Conclusions: We believe that similar cases may have remained
undiagnosed, warranting further research to assess the true prevalence
of the condition.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S272 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

893

894

MRI determined brain iron deciency in Restless legs syndrome

(RLS)
J. Godau, K.J. Schweitzer, A. Di Santo, T. Gasser, D. Berg,
U. Klose (Tuebingen, Germany)

Prevalence of Restless legs syndrome in a primary-care population

R.P. Allen, M.O. Calloway, W.J. Kwong, L. Palmer (Baltimore,
Maryland, USA)

Objective: To assess alterations of the brain iron status in RLS

patients using MR relaxometry.
Background: Impairment of brain iron homeostasis is regarded as
one major pathophysiologic factor for RLS. Histopathological and MRI
studies demonstrated decreased iron content in the substantia nigra
(SN) of RLS patients. Accordingly, most RLS patients demonstrate SN
hypoechogenicity on transcranial B-mode sonography. Electrophysiologic and fMRI studies suggest a pathophysiologic process that also
involves areas outside the nigrostriatal system.
Methods: 6 patients with idiopathic RLS with songraphic hypoechogenicity of the SN and 19 healthy controls with normal SN echogenicity underwent MRI using a 1.5 Tesla scanner and a three-echo
two-dimensional Turbo spin-echo sequence (TE: 14 ms, 85 ms, 156ms,
Slice Thickness 3.5 mm, 30 slices with the SN in the middle slice, FOV
100, 256256 matrix). Circular ROIs were placed bilaterally in 10
areas (1 pallidum, 2 red nucleus, 3 dentate nucleus, 4 putamen, 6
temporal white matter, 5,7,8 dorsolateral, medial and ventral thalamus,
9 head of the caudate nucleus, 10 hippocampus) on the rst-echo
images. T2 values were calculated from the signal intensities of rst
two echos and mean T2 values were compared between groups for each
ROI.
Results: In all assessed ROIs average T2 values were increased in
RLS patients (red) compared to controls (blue), suggesting decreased iron levels. Please compare Fig. 1. Differences were significant for the thalamus, red nucleus and caudate nucleus (all
p0.005) and less signicant for putamen and temporal white
matter (all p0.05).
Conclusions: Increased T2 values in all assessed brain regions
suggest a multiregional decrease of tissue iron content in idiopathic
RLS patients. This MRI approach underlines the relevance of brain
iron metabolism for the development of idiopathic RLS and is the
rst proof of a pathophysiological condition which likely affects the
whole brain of RLS patients. Further histopathologic and MRI
studies in larger patient cohorts are needed to substantiate these
ndings.

Objective: To estimate the prevalence of Restless legs syndrome

(RLS) in US primary-care settings.
Background: RLS is a neurological disorder with a prevalence of
510% in the general population (any frequency/severity). RLS is
underdiagnosed and undertreated.
Methods: This multicenter, 12-week longitudinal study (protocol
RRL104025) assessed the effect of RLS education on the awareness of
RLS in the primary-care setting. Secondary objectives included assessing the prevalence of RLS in primary care. A total of 139 physicians,
nurse practitioners, or physician assistants enrolled study subjects.
Each administered an RLS screening questionnaire to 200 consecutive patients presenting to clinic. Patients reporting 2 RLS-like symptoms on screening were evaluated by the study investigator to determine if they met NIH IRLSSG diagnostic criteria for RLS. Up to 10
individuals with a conrmed RLS diagnosis per investigator were
recruited to participate in the 12-week longitudinal phase of the study.
The RLS treatments received by these subjects before and at the end of
the study were examined.
Results: A total of 25,397 individuals were screened. An RLS
diagnosis was conrmed in 12.3% (3124/25,397) of those screened,
which is similar to a prior study using the full NIH IRLSSG diagnostic
criteria that reported a prevalence of 13.3% in a US primary-care
population (Hening et al, Sleep Med 2004; 5: 237 46). In the present
study, only 2.5% (638/25397) of those screened had been previously
diagnosed with RLS. A total of 1027 subjects with RLS participated in
the 12-week study, and 89.9% (923/1027) completed the study. Of the
subjects with RLS who entered the 12-week study, only 8.6% (88/
1027) had received a dopamine agonist for their symptoms prior to the
study.
Conclusions: This study corroborates previous studies that RLS is
prevalent in primary-care settings, but often goes underdiagnosed and
undertreated. These ndings suggest that RLS education may improve
disease awareness and diagnosis of RLS in primary care, so that RLS
patients are appropriately treated.

895
Cognitive functions in patients with Restless legs syndrome
S. Fulda, J. Winkelmann, T.C. Wetter (Munich, Germany)

FIG. 1 (893).

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To assess cognitive functions in patients with Restless

legs syndrome (RLS) and healthy control subjects.
Background: RLS is a burdening disorder with sleep disturbances
and impaired quality of life. Comparatively less is known about cognitive functioning in patients with RLS.
Methods: 25 drug-free patients with RLS and 20 healthy control
subjects matched individually for age (/- 3 years), gender, and education level participated in this ongoing study. Neuropsychological
testing in the morning (2 hours) assessed short-term attention and
concentration, working memory, short- and long-term mermory, verbal
uency, and concept formation.
Results: In the subsample of 20 RLS patients and 20 matched
controls, RLS subjects showed decreased performance in short-term
attention. In addition, a trend for worse performance in the verbal
uency task and spatial working memory was observed.
Conclusions: Our preliminary results suggest that RLS patients experience decits in the domain of attention and possibly executive
function. Whether these are caused by the sleep disturbances accompanying RLS and are reversed by adequate treatment has to be determinded in independent studies.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

896
Pramipexole improves daytime symptoms among patients with
Restless legs syndrome (RLS) with impaired daytime function
C.A. Kushida, S. Albrecht, J. Koester (Stanford, California, USA)
Objective: Our aim was to analyze the specic effects of
pramipexole on daytime RLS symptoms.
Background: RLS is a sensorimotor disorder characterized by a
broad range of symptoms, affecting patients during the evening and
nighttime. However, RLS symptomsand associated poor sleepmay
also impair daytime function. Pramipexole, a non-ergoline, D3/2-selective dopamine agonist, has shown efcacy across the spectrum of RLS
symptoms.
Methods: Data from double-blind trials were pooled for endpoints
that reect daytime RLS symptoms. Three trials included International
RLS Study Group Rating Scale (IRLS) question 5 (daytime tiredness/
sleepiness) and question 9 (impact on ability to conduct daily affairs)
and Epworth Sleepiness Scale (ESS). In addition, 2 of these trials used
visual analog scales (VAS) to assess RLS severity during the day. In
this analysis, patients with baseline ESS scores 10 were dened as
daytime impaired. The changes from baseline in VAS and ESS scores
were analyzed by ANCOVA, while the IRLS items were analyzed with
Wilcoxon Mann-Whitney tests.
Results: Overall, 31% of patients were considered daytime impaired.
Pramipexole signicantly improved most daytime-associated endpoints
in both populations (Table 1). ESS reductions were not signicant in
the total population and IRLS daily affairs scores were reduced but did
not attain signicance in the daytime-impaired subset.
Conclusions: Pramipexole was generally effective for daytime RLS
symptoms within this large database. ESS scores were signicantly
improved in patients who were the most impaired based on ESS
baseline assessment.

Pramipexole Difference From Placebo in Mean Change From

Baseline
Total Population
Endpoint (Total N/Subset n)
IRLS daytime sleepiness
IRLS daily affairs
VAS RLS severity During the Day
ESS daytime sleepiness

N
784
784
671
782

(adj.)# mean
-0.46
-0.31
-8.7
-0.4

Daytime Impaired
N
244
244
219
242

(adj.)# mean
-0.37*
-0.31
-10.8
-1.5*

*P 0.05; P 0.01; P 0.001. # VAS and ESS are displayed

with adjusted means (ANCOVA)
897
Pain in the Restless legs syndrome is more common in patients with
frequent RLS
W.A. Hening, R.P. Allen, C.J. Earley, C. Allen, C. Hening (New
York, New York, USA)
Objective: To determine whether patients are more likely to report
that Restless legs syndrome (RLS) symptoms are painful if they have
a more severe case of RLS.
Background: In most cases, patients with RLS do not complain about
pain as one of the primary symptoms. However, there has been a
clinical link between painful RLS and peripheral neuropathy, as well
as several studies that suggest that as many as half of RLS patients
(Bassetti et al., 2001) or those with RLS symptoms (Hening et al.,
2004; Allen et al., 2005) are bothered by pain. In diagnosing family
members of RLS and control probands in a case-control family study,
we have been impressed that those with more severe RLS are more
likely to report pain with their symptoms. To conrm this impression,
we have now examined the responses of family members with RLS to
a question about whether their symptoms were painful and stratied
them by frequency of symptoms, one measure of severity.
Methods: Family members of 133 RLS and 56 non-RLS probands
were blindly interviewed and diagnosed with RLS using a validated
telephone diagnostic interview. 289 of the family members were diag-

S273

nosed with denite RLS; all answered a question about whether their
RLS feelings were painful. Severity of RLS was graded by frequency
of symptoms.
Results: Overall, just 21% of these largely non-patient persons with
RLS endorsed their RLS symptoms as painful. However, this varied
signicantly by severity of RLS: while 17 of 42 (41.5%) with daily
symptoms reported pain, only 28 of 122 (23.0%) with weekly, but not
daily symptoms, and only 17 of 126 (13.5%) of those with less than
weekly symptoms reported pain with their symptoms (2 3 chi square,
2df, chi14.0, p.001).
Conclusions: More severely affected RLS patients are more likely to
report that their RLS symptoms are painful. This may be due to either
a change in the quality of symptoms or to a subjective judgment that
more intense and bothersome symptoms of the same quality deserve to
be called painful. Pain, as well as sensory discomfort in general, may
form an important part of the morbidity of RLS and should be a specic
target of treatment.
898
Circadian time course of laser evoked potentials (LEP) and laser
induced pain thresholds in patients with idiopathic RLS
C.G. Bachmann, C. Harder, T. Tings, C. Baier, W. Paulus, S. Happe
(Goettingen, Germany)
Objective: We evaluated circadian a-ber function by studying late
laser evoked potential (LEP) components N2 and P2 and pain thresholds after laser stimuli from 8 a.m. to 10 p.m. in 15 patients with
idiopathic Restless legs syndrome (RLS) and 15 age matched controls.
Background: The common condition of RLS is a circadian occurence
of uncomfortable sensory symptoms usually accompanied by an urgent
desire to move the legs. Neurophysiological, neuroanatomical, and
neuropharmacological studies suggest a complex interaction between
peripheral and central mechanisms. However, the pathophysiology of
RLS is still incompletely understood. Several studies assessing the
functioning of small bers (A-, C-bers) in RLS patients showed
differing results.
Methods: Heat stimuli were generated by an infrared Tm-YAG laser
(wavelength 2,01 m, pulse duration 1 ms, spot diameter 7 mm) and
were delivered on the dorsum of the right and left foot of healthy
controls and patients. After each stimulus patients and controls were
asked to classify their sensation with a numeric rating scale. In addition, we determined the effect of L-DOPA (100/25 mg levodopa/
benserazide) on pain thresholds after laser stimuli and on late LEP
components in idiopathic RLS patients 1 h after oral administration at
8 p.m. Initially ultralate LEP components, corresponding to C-ber
nociceptor activation, were uncovered by ischemic nerve blockade.
Results: Circadian latencies and amplitudes of laser evoked late
potentials N2 and P2 do not vary signicantly between healthy controls
and RLS patients. However, RLS patients showed signicantly increased heat pain thresholds 1 h after L-DOPA administration, suggesting a L-DOPA-mediated relief of pain perception after laser stimuli. In contrast, L-DOPA had no effect on latencies and amplitudes of
late LEP. In accordance with previous studies, ultralate LEP components were present only in a minority of the control group. Therefore,
we decided not to use them to assess the functioning of C-bers.
Conclusions: Overall, we did not nd any alterations pointing to
impaired function of A bers in idiopathic RLS patients. Our ndings
indicate that L-DOPA may ameliorate pain thresholds by a spinal or
central mechanism not assessable by recording of A-ber pathways.
899
Where dopamine meets opioids: A meta-analysis of the placebo
effect in RLS treatment studies
S. Fulda, T.C. Wetter (Munich, Germany)
Objective: To quantify the placebo response in RLS treatment studies by means of meta-analysis.
Background: The Restless legs syndrome (RLS) is a common sensory-motor disorder of sleep/wake motor regulation. The unique re-

Movement Disorders, Vol. 22, Suppl. 16, 2007

S274 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

sponsiveness of RLS to both dopaminergic agents and opioids places it
at the cross-road of the two systems implicated in the placebo response.
Methods: We searched the electronic databases PubMed and the
Cochrane Clinical Trials Registry (from 1980 to October 2006),
checked reference lists of retrieved articles, hand-searched abstract
books of sleep, neurology and movement disorder congresses and
visited clinical trial register web sites. All randomized, double-blind,
placebo-controlled studies exploring a pharmacological treatment in
subjects with RLS were considered. Outcome measures from ve
domains were extracted: RLS severity, subjective sleep parameters,
sleep parameters derived from nocturnal polysomnography, periodic
leg movements during sleep (PLMS) and daytime functioning.
Results: 35 trials were eligible for the meta-analysis. In 23 trials the
pooled placebo response rate was 37.80% (95% CI: 27.66 - 47.33). The
placebo effect was large for the primary outcome measure in most
studies, which is the International Restless Legs Severity Scale (1.34,
CI: -1.72 - -0.95), notably smaller for other RLS severity scales,
moderate for daytime functioning, small to moderate for subjective and
objective sleep parameters, and absent for PLMS and sleep efciency.
Conclusions: Placebo treatment has a strong impact on primary
outcome measures in RLS treatment studies. We propose that
RLS is the model disease to study the mechanisms of the placebo
effect.

900
Dose-response relationships for pramipexole in Restless legs syndrome
K.D. Sethi, E. Lainey, J. Koester (Augusta, Georgia, USA)
Objective: Although dosages of dopamine agonists for Restless legs
syndrome (RLS) are lower than for Parkinsons disease, the practical
issue remains of optimizing patients regimens. In 3 pramipexole trials,
investigators sought useful dosing patterns.
Background: Dopamine agonists have become the preferred drugs in
RLS treatment.
Methods: A 3-week European trial studied xed pramipexole doses
of 0.125, 0.25, 0.50, or 0.75 mg/day. A 12-week US trial studied xed
doses of 0.25, 0.50, or 0.75 mg/day. A 6-week European trial studied
exibly titrated doses (75 mg/day) starting with 0.125 mg/day for 1
week. Trials were double-blind and placebo-controlled; patients met
International RLS Study Group criteria, including baseline score 15
on the Groups scale (IRLS).
Results: Among xed-dose trials, the 3-week trial had 86
pramipexole and 21 placebo recipients. Pramipexole reduced mean
IRLS more (adjusted for age and baseline score) than placebo: 6.2 for
0.125 mg/day (P0.0055), 9.2 for 0.25 mg/day (P0.0001), 11.2 for
0.50 mg/day (P0.0001), and 9.9 for 0.75 mg/day (P0.0001). The
12-week trial had 254 pramipexole and 85 placebo recipients. Again
pramipexole was superior to placebo: 3.6 for 0.25 mg/day (P0.0086),
4.6 for 0.50 mg/day (P0.0011), and 4.7 for 0.75 mg/day (P0.0005).
The exible-dose trial had 224 pramipexole and 114 placebo recipients.
After 6 weeks, 52.2% of pramipexole recipients had 50% reduction
in IRLS score, compared with 28.9% for placebo. Among these
pramipexole responders, 19.7% required 0.125 mg/day, and 87.2%
required 0.50 mg/day. After 1 week at 0.125 mg/day, 30.6% of the
pramipexole group rated themselves as much or very much better
(on the Patient Global Impression scale), compared with 7.0% for
placebo.
Conclusions: At xed dosage, pramipexole regimens from 0.125 to
0.75 mg/day were effective against RLS. At exibly titrated dosage
(simulating real-world practice), a week of 0.125 mg/day was often
effective, but higher dosages were often necessary to achieve optimal
benet.

Movement Disorders, Vol. 22, Suppl. 16, 2007

901
Rotigotine transdermal patch provides high responder rates in
patients with Restless legs syndrome 24 month results from a
multi-national, multi-centre, open-label, follow-up trial
C. Trenkwalder, K. Stiasny-Kolster, D. Garcia-Borreguero,
B. Hoegl, J. Keffel, E. Schollmayer, W.H. Oertel (Kassel, Germany)
Objective: This trial is an open-label extension of a six-arm, doubleblind, prospective, placebo-controlled, 7 week, dose-nding study and
ongoing in 33 centers in Europe. The aim of this trial is to determine
efcacy, tolerability and safety of long-term application in patients with
moderate to severe idiopathic RLS.
Background: Rotigotine, a non-ergolinic D3/D2/D1 dopamine agonist formulated as a once-daily transdermal delivery system, is licensed
in Europe for the treatment of Parkinsons disease and in development
for RLS. The patch technology releases the drug continuously, and
provides stable plasma levels over 24 hours.
Methods: Once the individually adjusted optimal dose is reached in
the titration period, rotigotine was administered with dose-adjustments
allowed at anytime to maintain optimal treatment. Data are presented
assessing the 24 months follow-up after the titration period. Efcacy
variables included IRLS score, CGI, QoL-RLS score, RLS-6 scales,
and responder/ remitter analysis. The safety was evaluated by monitoring AEs, Labs and ECGs.
Results: A total of 295 patients entered the open-label extension trial
and 191 completed 2 years of maintenance. The baseline CGI item I
score was 5.1 0.9 (5markedly ill). Optimal dose treatment resulted
in a reduction of 2.8 1.2 points (as observed) to a mean score of
2.2 1.0 (2 borderline ill). Responder rates were 75% and 95% for
CGI item I (severity of illness) and CGI item II (global rating of change
of condition), respectively. A total of 30% of patients were symptom
free at month 24 (CGI Item I: normal, not at all ill). The most common
adverse events were application site reactions (50%), nasopharyngitis
(12%), nausea (11%), erythema (8%), back pain (11%), and fatigue
(8%). No clinical signs of augmentation were reported.
Conclusions: Treatment with the rotigotine transdermal patch was
well-tolerated and showed clinically relevant improvement in the CGI
scores. A total of 30% of patients were symptom free. All improvements were apparent in the titration period and were sustained during
24 months follow-up.
902
The prevalence of Restless legs syndrome and its association with
peripheral neuropathy in dialysis patients
A. Bogucki, A. Pozdzik-Koseda, J. Wyroslak (Zgierz, Poland)
Objective: To evaluate (1) the prevalence of Restless legs syndrome (RLS) and (2) the relationship between RLS and clinical and
neurophysiological signs of peripheral neuropathy in renal dialysis
patients.
Background: RLS occurs as a primary, idiopathic disease or as the
secondary disorder. Chronic renal failure is one of the most common
causes of the secondary RLS. RLS is associated with the increased
mortality in dialysis patients, but the quick remission is usually observed after successful renal transplantation. The pathogenesis of RLS
in patients with uremia remains unknown. Attempts were made to
correlate the presence of RLS with several clinical and biochemical
parameters, but no clear association was found. Peripheral neuropathy
is also a common consequence of uremia. Both, RLS and uremic
neuropathy manifest with dysesthesia.
Methods: We studied 85 hemodialysis and 15 peritoneal dialysis
patients (age 33-86, mean 10.8 years). The duration of dialysis ranged
from 1 month to 26 years, mean 4.6 years. The RLS Study Group
diagnostic criteria were used to diagnose RLS. The severity of symptoms was evaluated with IRLSSGS Scale. In all cases the neurological
examination as well as motor and sensory nerve conduction studies
were performed.
Results: RLS was diagnosed in 23 (23%) of all studied patients. The
duration of RLS symptoms was from 1 month to 26 (mean 4.8) years.
RLS developed before (up to 10 years) the start of dialysis in 4 patients

POSTER SESSION III, THURSDAY, JUNE 7, 2007

and at the time of dialysis initiation in 8. In 11 patients the rst
symptoms occurred after 6 months to 26 (mean 10) years of dialysis.
Clinical and electroneurophysiological signs of peripheral neuropathy
were found in 21.4% of patients with RLS and in 41.6% of patients
without RLS. No correlation was observed between the severity of RLS
and the presence of neuropathy.
Conclusions: Our studies revealed (1) similar to reported by others
- prevalence of RLS in dialysis patients and (2) the more common
occurence of peripheral neuropathy in patients without RLS.
903
Restless legs syndrome a clinical, etiological and electrophysiological study
S. Vanchilingam, M. Umaiorubahan (Chennai, Tamilnadu, India)
Objective: To nd out theclinical, etiological and electrophysiologocal charectristics of Restless legs syndrome (RLS) in patient population.
Background: RLS is a sensorimotor neurological condition that
affects sleep and day time functioning. Various studies have estimated
the incidence of RLS to be between 5% and 13% in the general
population. Two subsets Primary and Secondary RLS have been
recognized which differ in their clinical, etiological and electrophysiological presentations. The aim of the study is to evaluate the clinical,
etiological and electrophysiological characteristics of patients with
RLS.
Methods: Patients coming to the neurology outpatient department
with symptoms suggestive of RLS were screened using the IRLSSG
questionnaire for the epidemiological study of RLS. Those diagnosed to have RLS were included in the study. A detailed clinical
examination and biochemistry work up was done, a routine nerve
conduction study was done on all patients and the data was analyzed. All the patients were started on Tab. ropinirole.
Results: A total of 50 patients were enrolled into the study. Of the 50
patients, 5 patients were diagnosed to have primary RLS with normal
clinical and electrophysiological examination. These 5 patients were
noted to have an earlier age of onset, had on association with PLMS &
PLMW and had other family members with similar complaints. The
remaining 45 patients had other medical conditions associated, the most
common being Diabetic Mellitus, Chronic Renal Failure, Parkinsons
disease, Peripheral Vascular Disease. Most of these patients had abnormal electrophysiology.
Conclusions: On studying 50 patients with RLS, two subsets of
patients population were found. Primary RLS with younger age of
onset, normal excellent response to ropinirole. The Seconday RLS with
later age of onset commonly associated with peripheral neuropathy due
to Diabetes Mellitus, Chronic Renal Failure and an average response to
T. ropinirole.
904
Sleep problems in patients with RLS have a negative impact on
quality of life and increase the RLS health burden
R.P. Allen, P. Stillman, A.J. Myers (Baltimore, Maryland, USA)
Objective: To investigate the link between sleep disturbance and
quality of life in patients with Restless legs syndrome (RLS).
Background: RLS is characterized by sensory and motor symptoms that adversely affect sleep as a result of motor restlessness,
delayed onset of sleep and multiple awakenings during the night.
Such sleep disturbance may lead to reduced daytime functioning and
quality of life, and increase the overall health burden of the disorder.
Methods: 10,564 patients attending 62 primary care practices in 6
European countries were screened for RLS based on the 4 internationally recognized IRLSSG/NIH diagnostic criteria (Allen R et al. Sleep
Med 2003;4:101-19). Patients satisfying these essential diagnostic criteria, with frequent (2 days/week) and moderately/extremely distressing RLS symptoms, were invited for a diagnostic interview with a
trained primary care physician. Diagnosed patients completed both the
Medical Outcomes Study (MOS) Sleep and Short-Form 36 (SF-36)
Quality of Life Questionnaires.

S275

Results: Of 10,564 patients screened, 3.5% (365/10,564) were

diagnosed with RLS. Within this diagnosed RLS population, signicant correlations between MOS and SF-36 Questionnaire scores
indicated that measures of quality of life decrease as sleep problems
increase. An analysis of variance (ANOVA) showed that overall
sleep problems (represented by the MOS Sleep Problems Index II)
have a signicant negative impact on all SF-36 Quality of Life
domains (p0.001): the strongest relationships were seen in the
Vitality (F79.7), Bodily Pain (F44.9) and Social Functioning
(F39.4) domains.
Conclusions: The degree of sleep problems and sleep loss reported by RLS patients would be expected, based on results from
other studies, to adversely affect the health of any person. The
results from this study conrm that the severe degree of sleep
problems produced by RLS has a signicant negative impact on a
patients overall quality of life, and may be one of the critical
factors driving the health burden of RLS. Diagnosis and effective
treatment of RLS become important in order to reduce or prevent
development of these severe complications of the disorder.

SPASTICITY
905
Autosomal dominant spastic paraplegia (SPG36) with sensory deficits and muscle wasting maps to chromosome 12q23-24
K. Karle, M. Bonin, A. Dorr, S. Forlani, J. Kassubeck, S. Klimpe,
A. Seibel, B.P.C. Van de Warrenburg, P. Bauer, L. Scholls
(Tubingen, Germany)
Objective: To link autosomal dominant hereditary spastic paraplegia
(HSP) in a large German family.
Background: Hereditary spastic paraplegias (HSP) are characterized
by lower limb spasticity and weakness due to degeneration of the
corticospinal tract. To date 30 genetic loci for HSP have been described
(SPG1-33), among them 13 loci for autosomal dominant disease.
Fourteen HSP genes have been mapped, eight of them causing autosomal dominant disease.
Methods: Linkage to known loci for autosomal dominant HSP was
excluded in this family by haplotype analysis. A whole genome screen
was performed by a 10K SNP-based microarray approach.
Results: The genome scan gave evidence for a novel disease locus on
chromosome 12q. This novel locus was conrmed by a signicant
multi-point LOD score of 3.2 by ne-mapping with microsatellite
markers. A haplotype between markers D12S318 and D12S1023 segregated with the disease. The locus was termed SPG36 according to the
HUGO Gene Nomenclature. Linkage to SPG36 was excluded in 20
additional autosomal dominant HSP families. Candidate genes were
selected and sequenced. No disease-causing mutations were identied
in (DYNLL1, CDAP4, IQCD, HSPB8 and ATXN2). Presence of
sensory decits and muscular atrophy represented electrophysiologically by a predominantly demyelinating peripheral neuropathy in addition to spastic paraplegia constitute a complicated phenotype in our
SPG36 family.
Conclusions: SPG36 maps to chromosome 12q23-24 and comprises
the fourth autosomal dominant subtype of complicated HSP.
906
SPG10 is responsible for about 3% of autosomal dominant spastic
paraplegia in Germany
K. Karle, R. Schule, J. Kassubeck, S. Klimpe, T. Klompstock,
S. Otto, L. Schols (Tubingen, Germany)
Objective: To determine occurrence and frequency of SPG10 in
German autosomal dominant spastic paraplegia families and to decipher phenotypic characteristics.
Background: SPG10 is an autosomal dominant form of hereditary
spastic paraplegia (HSP) that is caused by mutations in the KIF5A gene
coding for the heavy chain of kinesin, the neuronal motor of fast
anterograde axonal transport. Only four mutations have been identied
so far, all of them affecting the motor domain of kinesin and thus most

Movement Disorders, Vol. 22, Suppl. 16, 2007

S276 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

likely disabling axonal transport. No KIF5A mutations have been
described in German HSP families and the frequency of SPG10 in
Germany is unknown.
Methods: In 85 index patients from families with autosomal dominant HSP SPG10 has been investigated by direct sequencing of the
motor domain of KIF5A.
Results: Three novel KIF5A mutations were detected including
c.759G/T, c.768del3 and c.217G/A. The novel mutations affect highly
conserved amino acid residues within the kinesin heavy chain motor
domain. The c.759G/T mutation was found in a large German family
and cosegregated with the disease. The mutation leads to replacement
of a highly conserved lysine residue by asparagine (K253N) in the
switch II loop region of the KIF5A motor domain. Onset of gait
disturbance varied from infancy to 30 years of age. All patients presented clinically with pure HSP, but moderate subclinical peripheral
neuropathy of sensory-motor type with axonal and demyelinating characteristics was detected by neurophysiology.
Conclusions: SPG10 is responsible for 3% - 4% of autosomal dominant HSP families in Germany and is characterozed electrophysiologically by accompanying peripheral neuropathy.
907
Retrospective cross-over evaluation of two botulinum toxin type A
preparations (Botox and Dysport) in the treatment of upper
limb spasticity
Y. Parman, H. Hanagasi, B. Topcular (Istanbul, Turkey)
Objective: To evaluate the dose regimens used, the efcacy and the
safety of two botulinum toxin type A preparations in upper limb
spasticity.
Background: Botulinum toxin type A is a safe and effective therapy
for a variety of disorders of excess muscle activity including upper limb
spasticity. Two serotype A toxins BOTOX and Dysport are widely
used. In this study, the dose ratio (Dysport:BOTOX) effect on spasticity, duration of effect and safety of the two serotypes were compared.
Methods: The study was a non-interventional, retrospective crossover evaluation of patient records conducted in one site. Patients of
either sex older than 18 years of age who had received at least two
treatment cycles each of both BOTOX and Dysport for the treatment of
upper limb spasticity were eligible for inclusion.
Results: Assesments of evaluations from a total of four patients (two
male, two female) were included in the study. The patients mean age
was 43 years (range 28-59). The number of evaluable injections sites
per patient ranged from two to four with a total of 12 evaluable sites.
The dose of BOTOX administered ranged from 25 to 50 units while the
dose of Dysport ranged from 125 to 232.5 units. The mean Dysport:
BOTOX dose ratio was 5.29 (range 3.65-7.50). The mean improvements in the Modied Ashworth Scores for each treatment group were
0.81 for BOTOX and 0.95 for Dysport. The mean improvements in the
Spasm Frequency Scores for each treatment group were 0.40 for
BOTOX and 0.25 for Dysport. Overall improvement in disease severity
after each injection was rated as 4.50 for BOTOX and 4.71 for Dysport.
The mean duration of effect was 88 days for BOTOX and 90 days for
Dysport.
Conclusions: The study conrms that botulinum toxin type A has a
useful role in the treatment of upper limb spasticity. The results show
a trend towards slightly greater efcacy for Dysport compared to
BOTOX. A single dose conversion ratio between the two products is
not appropriate. Comparative studies in larger groups should be considered to further delineate the proles of these drugs.
908
Botulinum toxin to treat spasticity secondary to ipsilateral cerebellopontine oligodendroglioma
A.M.L. Quek, R.C.S. Seet, E.C.H. Lim (, Singapore)
Objective: To describe ipsilateral spasticity from an infratentorial
lesion, and successful treatment with botulinum toxin.
Background: A man presented with progressive right-sided hearing
loss, ipsilateral facial weakness and numbness, unsteady gait and

Movement Disorders, Vol. 22, Suppl. 16, 2007

diplopia on looking to the right. Examination revealed right LMN facial

palsy, abducens palsy with multidirectional nystagmus, hearing loss
and facial sensory loss. He had mild right hemiparesis and an extensor
plantar response. MRI brain and subsequent excision biopsy revealed a
55x4 right cerebellar oligodendroglioma, which crossed the midline.
He underwent chemotherapy and cranial irradiation. He remained well
over the next 5 years, although he noted mild right-sided upper limb
stiffness, which increased markedly over the next 2 years, to the extent
that he had difculty writing legibly. His lower limbs likewise worsened, and he noted mild gait difculties. He was referred to our
Movement Disorders service. When he walked, he demonstrated toe
clawing, hip circumduction and inversion of the ankle. His handwriting
posture was signicant for ulnar-exion of the wrist, with excessive
exion of the ring and little ngers, indicating overactivity of the exor
carpi ulnaris (FCU), exor digitorum profundus and supercialis (FDP
and FDS).
Methods: He received botulinum toxin-A to his upper limb (40 units
to FCU 25 units each to FDP and FDS) under electromyographic
guidance with electrical stimulation. He opted to forego treatment of
his lower limb spasticity.
Results: He was assessed 3 weeks later and reported 70% functional
improvement in terms of discomfort and stiffness, as well as improvement in handwriting. His abnormal posturing, rated using the Dystonia
Evaluation Scale (DES), improved from a score of 0 to 2 ((0, dystonia
as bad as at its worst; 1, slightly improved; 2, moderately improved; 3,
almost normal; and 4, normal).
Conclusions: Supratentorial lesions causing ipsilateral hemiparesis is
called the Kernohans notch syndrome, where the lesion causes displacement of the crus cerebri, which is compressed by the tentorium
cerebelli. In our patient, a similar mechanism (displacement and compression by the tentorium) can explain the ipsilateral weakness and
subsequent spasticity, except that the lesion, in this case, is infratentorial.
909
Quality of life following botulinum toxin (Dysport) in upper limb
spasticity following stroke
A. Hughes, I. Baguley, L. Davies, S. de Graaff, P. Katrak,
P. McCrory, J. Sandanam (Melbourne, VIC, Australia)
Objective: To evaluate the role of botulinum toxin (Dysport) in the
treatment of upper limb spasticity following stroke using a range of
measures and to determine the association between improvement in
spasticity and change in quality of life.
Background: Botulinum toxin is well established to improve spasticity following stroke. Whether this leads to an improvement in quality
of life in this patient group is less well established.
Methods: One hundred and two subjects with post-stroke upper limb
spasticity were enrolled in a prospective, multicentre, placebo-controlled, double blind study using two cycles of treatment with botulinum toxin or placebo at 12 week intervals. Subjects were assessed at
eight time points across the 28 week window. Assessments included
measures of quality of life (primary end point), spasticity, functional
status, goal attainment, depression, pain, patient disability, carer burden
and global benet.
Results: Ninety-six patients were available for intention to treat
analysis. Botulinum toxin lead to a signicant reduction in spasticity
(Modied Ashworth Scale) compared to placebo across all three joints
studied (ngers, wrist and elbow) at all time points. Signicant improvement compared to placebo was also seen in the patient rated
functional outcome measure, the goal attainment scale, global benet
and pain (only after the initial injection). Despite these changes no
signicant improvement was seen in quality of life (Assessment of
Quality of Life measure - AQoL), depression, patient disability, or
carer burden.
Conclusions: Despite signicant improvements in spasticity, patient
rated functional outcomes and goal attainment scales there was no
improvement in quality of life, patient disability, or carer burden. This
inconsistency will be discussed.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

S277

910

912

Clinical and electrophysiological evaluation of post stroke spasticity an attempt to correlate

R.R. Garlapati, M. Umaiorubahan (Chennai, Tamilnadu, India)

A N1 randomized placebo-controlled multiple cross-over pilot

study of FP0011, a novel antiglutamate agent, in advanced PD
O.O. Rascol, L. Lacomblez, J. Ferreira, L. Negre-Pages,
J.-C. Lemarie, L. Bossi (Toulouse, France)

Objective: To evaluate patients with post-stroke spasticity clinically

and electrophysiologically and attempt to correlate clinical severity of
spasticity with electrophysiological studies.
Background: Subjective tests to grade spasticity have high inter- and
intra-rater variabilities, while objective tests are more precise. Hence, if
Modied Ashworth Scale can be shown to correlate closely with
objective neurophysiological tests, then it would be a valuable tool for
grading spasticity.
Methods: 60 Patients coming to the neurology department of Sri
Ramachandra Medical College were grouped into six different groups
based on the grade of spasticity on Modied Ashworth Scale and Nerve
Conduction Studies were done on them. The studies done were minimum F-Wave latency, F/M ratio, F-wave persistence and H/M ratio. F
wave latency was studied on ulnar,median,tibial and peroneal nerves
while H/M ratio was studied on exor carpi radialis and soleus muscles.
Results: F-Wave latency, F- Wave Persistance, F/M ratio and H/M
ratio were signicantly increased depending on the grade of spasticity,
while in modied Ashworth scale 1 and 1 the values were not
statistically signicant.
Conclusions: Electrophysiological evaluation in spasticity correlated
well with Modied Ashworth scale grading for spasticity and hence can
be used as an objective method for the grading of post-stroke spasticity.
911
Spasticity treatment with BTX-A improves the functional hand
development in patients with cervical spinal cord injury
E. Gasparoli, F. Piccione, A. Merico, M. Cavinato (Venezia Lido,
VE, Italy)
Objective: The aim of the present study is to verify the improvement
of specic daily activities after BTX-A type A (BTX-A) injections in
spastic upper limb muscles of patients with C5-C6 spinal cord injury
(SCI). This was performed to realise an active and passive functional
hand.
Background: Appropriate spasticity management of upper limbs is
essential to prevent contractures, deformities and stiffness in SCI
patients. The use of BTX-A reduces spasticity and prevent permanent
shortening of muscles and tendons.No study showed improvement of
functional abilities after BTX-A injection in SCI patients.
Methods: A group of 10 SCI patients with spasticity and/or muscular
stiffness at upper limb was enrolled (duration of disease 42 weeks).BTX-A was used with doses of 50-100 U for limb at least three
injections into the muscles Flexor Carpi Radialis, Brachial Biceps,
Pectoralis Major, Infraspinatus and Extensor Digitorum Communis. All
patients were assessed before the treatment and 4 weeks after injection,
using Range of Motion (ROM), Modied Ashworth score (ASH), the
personal hygiene and the global scores of FIM (Functional Independence Measure) and NHPT score (Nine Hole Peg Test).
Results: 7 patients with C5 level, treated predominantly at Brachial
Biceps, presented a signicant increase of elbow ROM (24 3.2).
The other 3 subjects with C6 level, treated mainly at Flexor Carpi
Radialis, had a signicant enhancement of wrist ROM (33 2.7).
The ASH decreased more in the 3 C6 level patients (-0.40.5) than in
the 7 C5 level patients (-0.9 0.3). Only 3 with C5 level could not
accomplish the NHPT task and did not reach the functional hand aim.
The functional achievement in the other 7 patients determined significant increments of FIM scores both in patients with active hand and in
passive hand (personal care sub-score: 7.8 0.4).
Conclusions: The use of botulinum toxin reduces the exor spasticity,the shortening of muscles and tendons, maintaining the physiological
movements of the shoulder, elbow, wrist and hand joints. The achieved
target was the improvement of the elbow and wrist extension decit to
obtain a passive functional hand in patients with C5 level and an active
functional hand in patients with C6 level.

Objective: To assess the efcacy and safety of FP0011, a novel

antiglutamate agent, in PD.
Methods: Proof-of-concept (N-of-1), randomized, double-blind, placebo-controlled, multiple-crossover study in 8 PD patients with motor
complications (age: 686 years), PD duration: 136 years, UPDRS III
ON: 146, time spent OFF: 4.41.0 hrs/d). Each single patient (Nof-1) trial included a 1-month open run-in period for FP0011 titration
(60 mg/d to a maximum of 120 mg/d), followed by 4 crossover phases,
each consisting of two 10-day treatment periods (FP0011 or placebo)
separated a 4-day placebo washout. Efcacy was assessed using exploratory endpoints including UPDRS (parts II, III and L-dopa-responsive and -non-responsive subscores), diaries, 7-point rating scales and
CGI. For each patient, within crossover differences between FP0011
and placebo were described individually and by their mean across
crossovers. A patient was considered to do better on FP0011 over
placebo when his/her mean difference was in favour of FP0011. The
statistical signicance of the treatment effect was determined by analysing the original values (one value per subject per visit) within a
crossover mixed model (patient being taken as random effect) taking
into account the intra-subject variability and the period and treatment
effects.
Results: Six patients completed the 4 crossovers. One dropped out
during run-in (fatigue and dyskinesia) and 1 during the 3rd crossover
(increased alkaline phosphatase). 7/7 patients did better on FP0011
versus placebo regarding UPDRS III (-2.10 points, i.e. -16%, p0.08).
The same trend was observed for UPDRS IIIII. 5/7 patients did better
on FP0011 versus placebo on the UPDRS levodopa-responsive score
(-18 %, p0.12) and 6/7 patients did so on the non-responsive score
(-14 %, p0.06) Diaries analysis showed that 6/7 patients had an
increased ON time without troublesome dyskinesia on FP0011 (0.58
hours vs placebo). No trend was observed with other endpoints Drugrelated AEs were mild to moderate(somnolence, insomnia, dyskinesia,
nausea, fatigue, severe seborrhoea, headache, worsening of OFF, increased alkaline phosphatase).
Conclusions: These results suggests that FP0011 may improve axial
and non axial parkinsonian symptoms and good ON time.
913
Comprehensive spasticity treatment for institutionalized adults
with mental retardation
C.E. Gill, C.R. Blair, H.M. Taylor, C. Nixon, P.D. Charles
(Nashville, Tennessee, USA)
Objective: To determine if comprehensive treatment improves spasticity measures and the time to perform daily care tasks for individuals
with mental retardation (MR) living in a public developmental center
(DC).
Background: Individuals with MR frequently have disabling comorbidities, such as spasticity, which is found in an estimated 35% of DC
residents.
Methods: We conducted a prospective, open-label, single-center
outcomes evaluation of comprehensive spasticity treatment for people
identied as having spasticity which interfered with care delivery. A
multidisciplinary team familiar with each subject identied between
one and three care tasks that could benet from spasticity treatment.
Each subject underwent a baseline evaluation consisting of six timed
care tasks, a Modied Ashworth Scale (MAS) rating, and three range
of motion (ROM) measurements across the relevant joints. The appropriate treatment(s), including botulinum toxin type A (BTX-A) injections or intrathecal baclofen (ITB) therapy were prescribed and optimized to provide symptom control. All patients received physical and
occupational therapy prior to and throughout the program. One year
after treatment initiation, a follow-up evaluation identical to baseline
was conducted. The centers ethical committee approved this protocol

Movement Disorders, Vol. 22, Suppl. 16, 2007

S278 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

and no study-related procedures were conducted until informed consent
was granted.
Results: 20 subjects (7 male, 13 female; Age Range: 22-71) were
treated with ITB (7), BTX-A (11) or both (2). The average length of
time to complete a care task decreased after treatment by
16.7660.21% (p0.0204, Wilcoxon signed rank test). ROM increased by an average of 13.8822.21 degrees (p0.0001). The MAS
decreased by 0.750.74 points (p0.0001).
Conclusions: Comprehensive treatment for people with MR and long
standing spasticity reduced muscle tone, increased ROM and successfully facilitated care delivery, as objectively measured by the time
required to complete a task.
914
Analysis of surgical intrathecal [i.t.] baclofen [ITB] implant results
emphasizing revision surgery in a mixed pediatric/adult population
Y.M. Awaad, N. Roosen, K. McIntosh, M. Waines (Bloomeld Hills,
Michigan, USA)
Objective: Increasingly, spasticity is managed with ITB using surgically
implanted programmable pumps [Medtronic, Inc.]. ITB revision surgery,
unrelated to programmable pump end-of-life is not uncommon, requiring
special attention during pre-, intra-, and post-operative management.
Background: We retrospectively reviewed our recent revision surgery experience based upon 1) primary implants at our own institution
[primary-implant-patients], and 2) operative revisions for cases originally implanted outside our institution [revision-only-patients].
Methods: Since 2002, we treated 41 patients [21F/20M; 22 children
(14F/8M) vs. 19 adults (7F/12M); 30 primary-implant-patients: 16
children (10F/6M) vs. 14 adults (5F/9M); 11 revision-only-patients:
6 children (4F/2M) vs. 5 adults (2F/3M)]. Clinical charts, operative and
imaging reports were reviewed to evaluate reasons for revision surgery
and diagnostic work-up requirements.
Results: Eight of 30 primary-implant-patients required 14 revisions
and 7 of 11 revision-only-patients needed 13 procedures. Seven patients
with slowly increasing baclofen-resistant spasticity had either 1) unsuspected pump-catheter connector defects [N4] with a subcutaneously
dislocated i.t. catheter [N1], 2) an X-ray-documented pump-catheter
connector defect [N1] or 3) an X-ray-demonstrated fractured catheter
with i.t. fragment [N2] requiring laminectomy [N1]. Injection studies
revealed i.t. peri-catheter arachnoiditis [N1; managed without laminectomy], and connector-related dye leakage [N3]. Implant infections occurred in 4 cases [3 were multiply pre-operated]. Scintigraphy revealed
occult CSF leakage [N1]. Intrinsic pump failure was rare [N1].
Conclusions: ITB, although very gratifying, has a high, predominantly technique-related complication incidence during implant life.
Meticulous surgical technique, high clinical suspicion, appropriate
work-up, and timely surgical management are emphasized to reduce
surgical ITB complications.
915
Functional assessment following intrathecal baclofen therapy in
children with spastic cerebral palsy
Y.M. Awaad (Bloomeld Hills, Michigan, USA)
Objective: The purpose of this paper is to describe outcomes of
intrathecal baclofen therapy (ITB) for 29 patients with cerebral palsy
focusing on impairments, functional limitations and disability. Patients
received individualized rehabilitation and were followed up to 24
months.
Background: The primary outcome measures were the Ashworth
Scale and the functional skills and caregiver assistance scales of the
Pediatric Evaluation of Disability Inventory (PEDI).
Methods: Ashworth Scale scores were signicantly reduced. All
areas of functional skills and caregiver assistance improved.
Results: Comparing groups of adults and patients less than 18 years,
there were no signicant differences, but there was a relationship
between age and dose. Comparing groups of patients in high and low
levels of independent functional mobility, no signicant differences
were found.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Conclusions: These results provide suggestive evidence that ITB and

rehabilitation have positive effects across the dimensions of disablement.
This study serves as a basis for high-level scientic studies of these.

Spearman rank correlations between variables at baseline and

follow-up visits.
Months Post
Implant

Variables

Spearman rank
correlations

1
1
6
6
6
12
12
12
18
18
18

32
32
30
30
30
25
25
25
22
22
22

Dose & Ashworth

Dose & Age
Dose & Age
Ashworth & Mobility skills
Dose & Social function skills
Dose & Age
Dose & Self-care skills
Dose & Self-care assistance
Dose & Age
Dose & Self-care skills
Ashworth & Self-care skills

.540
.444
.411
.462
.395
.460
-.474
-.428
.636
.538
.470

.005
.011
.024
.047
.046
.021
.022
.042
.001
.021
.001.021.042

Table 1
916
Retrospective cross-over evaluation of two botulinum toxin type A
preparations (Botox and Dysport) in the treatment of lower
limb spasticity
Y. Parman, H. Hanagasi, B. Topcular (Istanbul, Turkey)
Objective: To evaluate the dose regimens used, the efcacy and the
safety of two botulinum toxin type A preparations in lower limb
spasticity.
Background: Botulinum toxin type A is a safe and effective therapy
for a variety of disorders of excess muscle activity including lower limb
spasticity. Two serotype A toxins BOTOX and Dysport are widely
used. In this study the dose ratio (Dysport:BOTOX), effect on spasticity, duration of effect and safety of the two serotypes were compared.
Methods: The study was a non-interventional, retrospective crossover evaluation of patient records conducted in one site. Patients of
either sex older than 18 years of age who had received at least two
treatment cycles each of both BOTOX and Dysport for the treatment of
lower limb spasticity were eligible for inclusion.
Results: Assesments of evaluations from a total of six patients (three
male, three female) were included in the study. The patients mean age
was 50.5 years (range 35-64). The number of evaluable injections sites
per patient ranged from one to six with a total of 18 evaluable sites. The
mean Dysport:BOTOX dose ratio was 5.45 (range 3.21 9.0). The
mean improvements in the Modied Ashworth Scores for each treatment group were 0.83 for BOTOX and 0.58 for Dysport. The mean
improvements in the Spasm Frequency Scores for each treatment group
were 0.91 for BOTOX and 0.55 for Dysport. The mean improvement
in walking speed was 0.103 m/sec in the BOTOX group and 0.071
m/sec in the Dysport group. Overall improvement in disease severity
was rated as 4.55 for BOTOX and 4.18 for Dysport.
Conclusions: The study conrms that botulinum toxin type A has a
useful role in the treatment of lower limb spasticity. The results show
a trend towards greater efcacy for BOTOX compared to Dysport. A
single dose conversion ratio between the two products is not appropriate. Comparative studies in larger groups should be considered to
further delineate the proles of these drugs.
917
Botulinum toxin treatment for hip exor spasticity in older children and adults a report on 22 patients
A. Stenner, G. Reichel, W. Hermann (Zwickau, Germany)
Objective: Spastic hip exion handicaps gait and posture, bedding,
hygiene of personal areas and sexual activities. Patients who are able to
walk will experience hip exion accompanied with forward-bent position of the upper body and shortening of contralateral movements. In
botulinum toxin (Btx) treatment of younger children, ventral approach
usually under general anesthesia or algesio-sedation is preferred,
whereas dorsal approach seems to be safer for older children and adults.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

Background: In spite of descriptions of the dorsal technique dating
from 1999, no reports about its effectiveness and long-term treatment
of adults are available as of now. Effort and success of this type of
treatment are still unclear.
Methods: 22 patients with hip exor spasticity were treated with Btx
over a period of nine months up to eight years. Injections were applied
after identication of the muscle belly of m. psoas major via imaging
procedures and measuring of the distance between skin and middle of
the muscle (gure 1 A-B), and of the distance between spinous process
and middle of the muscle being projected on the skin (gure 1 B-P).
After local anesthesia, a self-developed coated needle marked with an
adhesive strip was injected at the level of the spinous process and
pushed into the middle of the muscle belly (gure 2). Slight active or
passive hip exion was then used to back-up the position in the muscle
electromyographically.
Results: Our youngest patient was eight, our oldest one 67 years old.
Doses applied were 1 ml in children and 2 ml in adults (1 ml is
equivalent to 200 MU Dysport or 50 MU Botox). In four patients
with ICP, we were able to extend the rst interval of treatment because
of its constant efciency, and then it was stopped completely. In
younger patients, the optimum level for injections was L3, in older
patients it was L4. We did not have any discontinuations of treatment
because of insufcient results or side effects.
Conclusions: Dorsal injection of Btx into the m. psoas major, supported by imaging procedures and electromyography, proved a success
even in long-term treatment of hip exor spasticity. It is quite practical,
not troublesome for patients, and very effective. There are no side
effects.

S279

TICS
918
Different perception of tourette syndrome among patients, relatives and physicians
E. Cubo, J. Rivera, J. Almazan (Burgos, Spain)
Objective: To analyze the perception of physicians, patients with
Tourette Syndrome (TS) and their relatives regarding the diagnosis of
TS.
Background: Clinical, psychological, social and economical features
in TS have been studied; nevertheless, no research has been made about
the difference between the perception of TS and its consequences
among patients, relatives and physicians.
Methods: Five focus groups (FG) were created: one of physicians,
two of TS patients and two of TS patients relatives. Physicians were
recruited from personal specialized in movement disorders and, TS
patients and relatives were recruited from voluntary TS organizations.
Results: Physicians thought TS diagnosis were difcult to make due
to the complexity of the symptoms. For patients, the difculty to
diagnose is related to the non-knowledge of the disease for physicians.
Relatives said that the difculty in making a diagnosis by physicians is
that symptoms were hidden due to the guilt complex of the family.
According to doctors and TS patients the family often denied the
connection between the symptoms and TS. Clinical concepts were
badly understood by TS patients relatives.
Conclusions: Qualitative methods and concretely created focus
groups have shown to be useful in order to study different perceptions
of a single disease and its consequences. The way of communicating
diagnosis of TS to patients and relatives should be improved for a better
understanding. Educational programs should be implemented to avoid
stigmatization of TS within patients and their relatives.
919
Adult onset simple phonic tic after caudate stroke
G. Meritxell, P.-S. Claustre, P. Victor, V. Rosa, O. Carlos, R. Jaume
(Barcelona, Spain)

FIG. 1 (917).

Objective: Our aim was describe a case with adult onset simple
phonic tic after caudate stroke.
Background: Tic disorders presenting during adulthood have infrequently been described in the literature. Phonic and motor tics are
most frequently due to Tourette syndrome, but there are many other
causes of tics. Stroke is a rare cause of secondary tics. The relationship between stroke and tourettism has been described, but cases
of simple phonic tic as a consequence of stroke have never been
reported.
Methods: A 71-year-old man with hypertension as a vascular risk
factor, and without familial medical history of tics, suffered a subcortical stroke located in left caudate nucleus. In the acute phase of stroke
he presented a mild right dysmetria and the patient recovered completely one week after the onset. Two weeks later, he developed a
simple phonic tic consisting of onomatopoeic sound, persisting one
year after stroke onset.
Results: Except for the tic, the results of the patient physical and
neurological examinations were normal. We performed the neuropsychological tests that were also normal. The magnetic resonance imaging (MRI) showed ischaemic lesion involving the left caudate nucleus
and some old lacunar strokes. Functional imaging using single photon
emission computerized tomography (SPECT) has highlighted abnormal
patterns of blood ow and metabolism in the left basal ganglia, and left
frontal cortical areas of the brain. The phonic tic only responded
partially to pimozide treatment, but the patient stopped it due to severe
somnolence.
Conclusions: It seems to be both functional and structural abnormalities in the brain of people with tic disorders. While the exact
neurochemical cause is unknown, it is believed that abnormal neurotransmitters contribute to the disorders. It has also found changes
within the brain itself, specically in the basal ganglia and the
anterior cingulate cortex. Studies demonstrated in Tourette syn-

Movement Disorders, Vol. 22, Suppl. 16, 2007

S280 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

drome a reduced volume of the caudate nucleus, compared with
healthy subjects. Reduced excitability of cortical inhibition is one
factor that contributes to the difculty that patients have in suppressing involuntary tics. We have shown that simple phonic tic
could be a consequence of subcortical stroke involving the basal
ganglia, specically caudate nucleus.
920
Deep brain stimulation for tourette syndrome: Hope or hype?
Personal remarks
D. Servello, M. Sassi, S. Defendi, A. Brambilla, M. Porta (Milan,
Italy)
Objective: To assess the efcacy of Deep Brain Stimulation for the
treatment of Tourette Syndrome refractory to conservative treatment.
To determine the correct indications to DBS in a complex therapeutic
environment for this syndrome.
Background: During past years, deep brain stimulation in intractable
Tourette Syndrome (TS) has risen a growing interest: a few patients
treated with DBS have been reported in literature with encouraging
results.
Methods: More than 500 patients are routinely followed in a multidisciplinary environment by the AIST (Italian Tourette Syndrome
Association): among these, 22 have been treated with DBS starting
from November 2004. All of the patients demonstrated resistance to
conservative treatments such as typical and atypical neuroleptics and
psychic approaches such as the Habit Reversal Training. Morevoer, a
marked social impairment was reported by the parents, often with a
forced scholarity interruption.
Results: Centro-medianus, Ventralis Oralis and Para-fascicularis nuclei of the thalami were targeted bilaterally in 21 patients: among these,
one patient was also targeted at the Nucleus Accumbens due to intense
obsessive-compulsive behaviours. One patient was targeted at the Globus Pallidus Internus posteriorly and at the Nucleus Accumbens. Long
term follow-up results conrm the efcacy and safety of the DBS
treatment.
Conclusions: Questions are raised regarding the efcacy of the
different targets for DBS, but also proper indication to surgery and
post-surgical management of these patients.

FIG. 1 (920).

921
Fragile X syndrome associated with tic disorders
S.A. Schneider, M.M. Robertson, R. Rizzo, J. Turk, K.P. Bhatia,
M. Orth (London, United Kingdom)
Objective: To describe patients with Fragile X syndrome associated
with tic disorders.
Background: Fragile X syndrome is the most common identiable
cause of inherited mental retardation. Movement disorders other than

Movement Disorders, Vol. 22, Suppl. 16, 2007

late onset tremor-ataxia in association with fragile X syndrome seem to

be rare.
Methods: Here we describe ve male patients from three unrelated
families with fragile X syndrome that presented with motor and phonic
tics.
Results: Clinically, four patients fullled diagnostic criteria for
Gilles de la Tourette syndrome (GTS) while one patient would have
been diagnosed with an adult onset tic disorder. However, in all
patients onset of tics was considerably later than in typical GTS. Three
patients had atypical tics and only one patient reported waxing and
waning of tic intensity over time. Four of the ve patients showed
clinical signs typical of fragile X syndrome, in particular dysmorphic
features, learning difculties and speech and language problems that
required special treatment. All patients had co-morbidities common to
both GTS and fragile X syndrome.
Conclusions: We suggest considering fragile X syndrome in GTS
complicated by co-morbidity with late onset of atypical tics, in particular when learning disability and dysmorphic features are present.
922
Determinants of quality of life in Gilles de la Tourette syndrome
S.S. Al Faqih (Ramadi, Iraq)
Objective: To assess the association between tic severity, attention
decit disorder, obsessive compulsive behavior, and quality of life in
patients with Gilles de la Tourette syndrome.
Background: Gilles de la Tourette syndrome is a multidimensional
disorder with disturbances in motor function and behavior. However,
little is known about what variables are associated with quality of life
in these patients.
Methods: We evaluated 40 outpatients with a diagnosis of Gilles de
la Tourette syndrome. The mean age was 10 (range 5-17 years). Tics
were assessed with the Yale Global Tic Severity Scale. Behavioral
scales included the Leyton Obsessional Inventory, Yale-Brown Obsessive Compulsive Scale, and ADHD Rating Scale. The patients parent
also completed the TNO-AZL Childrens Quality of Life scale (TACQOL). The TACQOL subscales include Physical Complaints, Motor
Functioning, Autonomy, Cognitive Functioning, Social Functioning,
Positive Emotions, Negative Emotions. We assessed the correlation
between tic ratings, obsessive compulsive behavior, ADHD and quality
of life scales using Spearmans Rank correlation.
Results: There were 37 boys and 3 girls who completed the study.
Tic severity was minimally related to quality of life in patients with
Gilles de la Tourette syndrome. Of the correlations based on subjective
and objective tic ratings, no QOL measure was associated with objective tic ratings and subjective tic ratings correlated with only one of the
QOL subscales (Autonomy rs -0.35, p0.029). In contrast, there was
a consistent relationship between the three obsessive compulsive inventories and the Physical Complaints QOL subscale (Leyton rs
-0.60, p0.001; YBOCS Total Score rs -0.57, p0.001; YBOCS
Interference rs -0.33, p0.04). Similarly, ADHD was signicantly
related to ve of the QOL subscales (Physical Complaints rs -0.32,
p0.05; Motor Functioning rs -0.42,p0.01; Autonomy rs -0.36,
p0.02; Cognitive Functioning rs -0.33, p0.04; Social Functioning
rs -0.40, p0.01).
Conclusions: Quality of life in children with Gilles de la Tourette
syndrome is more closely associated with co-morbidities of obsessive
compulsive behavior and ADHD than with tic severity. Though neurologists may be most comfortable in treating tics, to promote quality
of life, neurological treatment must incorporate and even prioritize
treatment strategies to abate co-morbidities.
923
Motor tics in a patient with Joubert syndrome
A.A. Contreras, J.J. Guzman de Villoria, A.A. Traba, F.F. Grandas
(Madrid, Spain)
Objective: Joubert syndrome is an autosomal recessive disorder
characterized by cerebellar hypoplasia, hypotonia, development delay
and eye movement and respiratory abnormalitie. Magnetic resonance

POSTER SESSION III, THURSDAY, JUNE 7, 2007

imaging typically shows partial or total vermian aplasia, elongated
superior cerebellar peduncles and a deep interpeduncular fossa, resulting in the characteristic molar tooth pattern. Clinical features present
in the neonatal period and early childhood. Movement Disorders, are
rare in adulthood. We report on a patient with Joubert syndrome and
repetitive cervical involuntary movements resembling motor tics. A
38-year-old man was taken by his parents to the clinic for the appearance of brisk involuntary repetitive movement of the neck and elevation of eyebrows over the last year. The cervical movements could be
partially controlled by will and improved when wearing a tightly tted
cap. He was the rst son of a consanguineous marriage and was born
after a prolonged labor. The patient experienced cognitive and motor
delay (gait and speech were achieved at the age of 5 years) and a
diagnosis of cerebral palsy was made. Divergent strabismus was observed.There was no exposure to neuroleptic drugs. On examination it
was found mental retardation (IQ 57) , macroglossia, dysarthria, bilateral divergent strabismus, short stature, scoliosis and a wide-based gait.
Frequent repetitive brisk movements of neck rotation to the right and
brief elevations of the eyebrows were observed. No hypertrophy of the
cervical muscles was detected. The remainder of the neurological
examination was unremarkable. EMG recordings of the cervical muscles showed bursts of EMG activity ( 100 ms) in sternocleidomastoid
and trapezius of the right side. A brain MRI revealed atrophy of the
cerebellar vermis, elongated superior peduncles and a pronounced
interpeduncular fossa with the aspect of molar tooth. This case,
which fullled the diagnostic criteria for Joubert syndrome, showed
craniofacial abnormal involuntary movements consistent with motor
tics or dystonic tics (improvement with a sensorial trick) starting in the
adulthood. It enlarges the range of motor abnormalities that may appear
in this developmental disorder.
924
Quality of life of patients with Gilles de la Tourettes syndrome:
Results of the pilot study
F. Galland, L. Malet, Y. Worbe, A. Hartman, L. Mallet, P.P. Derost,
D. Morand, I. de Chazeron, P.-M. Llorca, Y. Agid, F. Durif,
I. Jalenques (Clermont-Ferrand, France)
Objective: The aim of our study is to investigate QOL of patients
with GTS age of 16 years or older. Since the QOL assessment included
self-rating questionnaires, a pilot study was necessary in order to
appraise the feasibility of the study.
Background: Only one paper about quality of life (QOL) of patients
with Gilles de la Tourettes syndrome (GTS) has been published, using
a generic self-rating questionnaire SF 36, and a generic hetero questionnaire QOLAS. Authors concluded that QOL is impaired in patients
with GTS. However methodological limits were quite important : rstly
QOLAS was developed initially to assess patients suffering from
epilepsy or dementia and secondly social dimensions of SF-36 are
limited.
Methods: In the nal study, a total of 372 members of the French
Association of GTS will receive 4 questionnaires: the GTS-related
symptoms questionnaire, the WHOQOL-26 (World Health Organization Quality Of Life), the FSQ (Functional Status Questionnaire) and
the SCL-90 (self-rating questionnaire about psychiatric symptoms). All
are validated in French language. Socio-demographic data will also be
assessed.
Results: The feasibility study included 17 patients who fullled
DSM-IV criteria for GTS, referred to a specialized unit in a university
hospital.The mean time to complete them was: 31.5 10.2 minutes.
Fifteen of 17 patients had completed and returned the questionnaires:
10 were perfectly completed, one was unusable due to a lot of invalid
answers and 4 had only one answer missing which didnt invalidate the
assessment. The questionnaires didnt seem lengthy or cumbersome.
Thus questionnaires were easily completed by subjects, we can conclude that acceptability was excellent.
Conclusions: Some adjustments were realized to make questionnaires more readable. The nal study will start in March 2007.

S281

925
Ziprasidone in treatment of tics in Tourette syndrome
M. Blazquez-Estrada, M.T. Calatayud-Noguera, B. Blazquez-Menes
(Oviedo, Asturias, Spain)
Objective: To evaluate the efcacy of Ziprasidone on motor and
vocal tics in patients with Tourette syndrome (TS).
Background: TS is a neurological disorder characterized by motor
and vocal tics. Although the genetic factors play a fundamental role in
its appearance, the etiology still continues unclear. TS may be associated to obsessive-compulsive disorder (OCD) and attention-decit hyperactivity disorder (ADHD). Tics only require treatment when they
interfere with the functioning of the patient or associates conductual
disorders. It is preferable the monotherapy to low doses. The most
frequently used drugs for tics are antipsychotics.
Methods: We included 14 patients with TS according to DSM-IV
criteria. Eleven of the patients had been treated with many typical and
atypical neuroleptics without success, and which had also given unacceptable side effects. Informed consent was obtained. We used Yale
Global Tic Severity Rating Scale and Goetz Scale to assess the outcome.
Results: All patients who were treated with Ziprasidone showed
clinical improvement in their vocal and motor tics although to differing
degrees with a signicant p value in the scales used. The successful
Ziprasidone doses were between 20-120 mg daily. In patients who
associated behavioural disorders a moderate improvement was observed. In one patient it was necessary to suspend the treatment for
pregnancy. Side effects were mild and transient, Ziprasidone was well
tolerated with infrequent and transient drowsiness.
Conclusions: Ziprasidone is useful and safe in the treatment of tics
and has low side effect prole. Further double-blinded studies should
be undertaken.
926
A case of a patient with coexistent Tourette syndrome and benign
hyperbilirubinemia
S.G. Khachatryan, Z.D. Tavadyan, G.R. Melikyan (Yerevan,
Armenia)
Objective: To report a case of a patient with Tourette syndrome (TS)
and coexistent benign hyperbilirubinemia (BH) admitted Department
of Neurology, Erebouni Medical Center.
Background: BH (e.g. Gilbert syndrome (GS)) had never been reported before in coexistence with tic disorders. Anyway, GS is known
to be associated with chromosome 2 mutation which is also reported in
association with TS in some populations.
Methods: The patient was diagnosed TS according to DSM-IV
criteria at admission. Syndrome of BH (possible GS) was diagnosed on
the clinical grounds and based on laboratory and ultrasound data
(genetic testing is not available in Armenia).
Results: A 16 year-old male patient had onset of attention decit and
hyperactivity disorder (ADHD) in early childhood, multiple motor tics
from age of 3-4 year (head-shaking, repetitive blinking, sudden retroexions of the head, sudden exions of the legs, body exions),
phonic tics from age of 9 (bird sounds, yelling, snorting) with typical
waxing and waning course, and obsessive-compulsive disorder with
echolalia and echopraxia during the last year. TS was refractory to
conventional treatment. From age of 9 mild lymphocytosis (41-46) and
mild hyperbilirubinemia without transaminase (ALT/AST) elevation
was found and persisted without any signicant change until now (total
21.3-34.7 mkmol/L, direct 0-6.2 mkmol/L, indirect 19-34.7 mkmol/L).
No relation between severity of TS and levels of bilirubin was found.
Viral hepatites were ruled out. Neurological examination was normal.
Medical history of the patient included jaundice of the newborn, rubella
and tonsillitis at age of 6. Repeated abdominal ultrasonography showed
no abnormality.
Conclusions: Although never described in TS patients, this nding
could contribute to understanding of genetic basis of TS and GS.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S282 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

927

929

Two cases of coexistent Tourette syndrome and temporal lobe

epilepsy
Z.D. Tavadyan, S.G. Khachatryan, G.R. Melikyan (Yerevan,
Armenia)

Excitability of cortico-spinal system at rest is associated with tic

severity in Gilles de la Tourette syndrome
M. Orth, A. Munchau, J.C. Rothwell (London, United Kingdom)

Objective: To report a comorbidity of Tourette syndrome (TS) and

temporal lobe epilepsy (TLE) in two patients admitted Armenian
Republican Epilepsy Center Erebouni.
Background: Rare comorbidity of TS and partial epilepsy is described in literature. Same environmental factors could inuence development of these disorders.
Methods: A report of two cases of comorbid TS and TLE. Patients
were diagnosed TS and TLE according to DSM-IV criteria and EEG
and MRI ndings at admission.
Results: Patient 1: A 21-year-old male had onset of simple partial
seizures (SPS) (rising epigastric discomfort, vertigo, and palpitation),
complex partial seizures (CPS) (staring, oral automatisms) and generalized tonic-clonic seizures (GTCS) at age of 6. One year later tic
disorder appeared with multiple motor tics (shrugging, hand-rubbing,
repetitive eye blinking, head and limb jerking, nose twitching, ear
moving), phonic tics (snifng, throat clearing, grunting, squeaking),
compulsive behavior (repetitive nger scratching with self-injury) and
typical waxing and waning during the course. EEG: right frontotemporal spike and wave activity. MRI: right hippocampal sclerosis.
Patient 2: A 19-year-old male patient at age of 15 had onset of tic
disorder with waxing and waning course of multiple motor tics (shrugging, head-rotation, grimacing, excessive blinking) and vocal tics
(coughing, throat cleaning, snorting). At age of 18 partial epilepsy with
SPS, CPS and GTCS developed (patterns of seizures are almost similar
for both patients). EEG: left fronto-temporal spike and wave activity.
No MRI performed. Low birth weight and probable perinatal injury had
been reported by parents of both patients.
Conclusions: We speculate that TLE and TS could coexist because
of common environmental factors. Presumably in the mentioned cases
birth injury and low birth weight could inuence development of both
disorders.
928
Biofeedback assisted relaxation training for children and adolescents with tics and associated disorders
S. Natriashvili, U. Haller, S. Ohmann, C. Popow (Vienna, Austria)
Objective: To evaluate the efcacy of biofeedback assisted relaxation training in children and adolescents with chronic tic disorders.
Background: Biofeedback and training of self-regulation skills in
children and adolescents with neuropsychiatric disorders is increasingly considered important. There are however only a few studies
evaluating effects and side effects and no study evaluating the duration
of effects following biofeedback training for tic disorders.
Methods: We prospectively investigated 13 children with chronic tic
disorders (ICD 10 diagnoses F 95.1 and 95.2) aged 6-18 years. Pretreatment assessment included disease and treatment history, evaluation of comorbidic conditions and standard psychological assessment.
Tic frequency and severity was estimated by Yale Global Tic Severity
Scale (YGTS) before and after biofeedback training. All patients were
subjected to 10-15 sessions of biofeedback training using the Soft
Biofeedback hardware and Softmed 6.2 software. Awareness training mainly concentrated on observing and attaining regular abdominal
breathing and on attaining a relaxed state. In addition, patients were
asked to perform daily awareness training at home.
Results: After ve to six biofeedback sessions, frequency and severity of tics and vocalizations assessed by YGTS improved in 11 patients.
In 4 (31%) patients tics and vocalizations completely disappeared, in 7
(54%) the symptoms decreased by about 50%, and 2 (15%) patients had
only minimal tic reduction. In all children we observed a signicant
reduction of comorbidic hyperactivity
Conclusions: Our data suggest that biofeedback produces signicant
improvement tic severity and associated problems. The data are very
encouraging, long term outcome studies are, however, needed.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: 1) To determine in patients with Gilles de la Tourette

syndrome (GTS) whether reduced intracortical inhibition is restricted
to inhibitory inter-neurones or represents one feature of a more widespread reduction of motor cortex excitability. 2) To evaluate the signicance of any of these ndings for the clinical phenotype, i.e. motor
and phonic tics.
Background: Alterations of motor cortex excitability as measured with
transcranial magnetic stimulation (TMS), in particular reduced short interval cortical inhibition (SICI), reduced short interval afferent inhibition
(SAI) and shortened cortical silent periods have been shown in GTS. At
higher intensities, SICI is recruited more gradually than normal.
Methods: We measured SICI, SAI using standard protocols. In addition
we obtained input-output curves both at rest and during contraction of the
target muscle with single pulse TMS in 20 untreated GTS patients (12
uncomplicated, 4 with co-morbid attention decit hyperactivity disorder
(ADHD), 4 with co-morbid obsessive-compulsive disorder (OCD)) and in
24 healthy subjects. SICI, SAI, and recruitment of cortico-spinal output
and silent period duration elicited by TMS were correlated with tic severity
and distribution rated with both standard clinical scales as well as detailed
video analysis.
Results: Motor thresholds were the same in GTS and healthy
subjects; SICI and SAI were reduced in patients. In the resting state,
recruitment of MEPs above threshold was more gradual in patients
than controls. In contrast, recruitment of MEPs during pre-activation was the same in both groups, as was the duration of the SP.
Correlation analysis showed that reduced levels of excitability at
rest (input-output curves but not SICI or SAI) relate, in uncomplicated GTS patients, to video ratings of complex tics, and hand and
nger tics, with less excitability predicting fewer tics. The correlations disappear for measures made during voluntary activation.
Conclusions: Our results indicate that the distribution of excitability
in the corticospinal system in patients at rest is different to that in
healthy individuals. We suggest that this is an adaptive response to
abnormal basal ganglia-motor cortex inputs in an effort to reduce
unwanted movements.
930
Behavioral decits in rats selectively bred for decient prepulse
inhibition of the startle response
K. Schwabe, M. Dieckmann, J.K. Krauss, M. Koch (Hannover, Germany)
Objective: Selectively breeding rats for high and low prepulse inhibition (PPI) of startle leads to groups with different PPI already in the
rst two generations. We here tested whether this difference is stable
during subsequent breeding and whether the low PPI is accompanied
by other behavioral decits.
Background: PPI of startle is a measure of sensorimotor gating that
is decient in some neuropsychiatric disorders, such as schizophrenia
and Tourettes syndrome. Experimentally induced PPI decits in rats
are used as endophenotype to study the biological mechanisms and
therapeutic strategies of these disorders.
Methods: Rats with the highest and lowest PPI, respectively, were
chosen for subsequent selective breeding. Different spatial and operant
paradigms were used to assess their learning and memory abilities as
well as their behavioral exibility. Moreover, the two groups were
tested for social and motivational behavior.
Results: Selective breeding lead to further segregation of PPI
during the next six generations. In the delayed alternation T-maze
task the two groups did not differ in learning and memory, but rats
with low PPI showed enhanced preservation during switching between an egocentric and allocentric radial maze task. Enhanced
preservation was also found in an operant behavioral task, where
different demands, i.e., a different number of presses for a pelletreward, were assigned to and switched between two levers of a
Skinner box. Rats with low PPI stayed longer at the ineffective lever

POSTER SESSION III, THURSDAY, JUNE 7, 2007

before switching, thus being less able to adjust their behavior to
changing reward values. When rats had the choice between leverpressing for reward pellets or freely available standard rat chow, rats
with low PPI chose the standard chow, suggesting anhedonia. Additionally, rats with low PPI showed decits in social behavior
during interaction with a juvenile rat.
Conclusions: Rats selectively bred for low PPI may provide a nonpharmacological model that can be used to study the pathophysiology
of disorders with known or proposed abnormalities in neuronal basal
ganglia circuits shown by decits in sensorimotor gating. They may be
used to evaluate new therapeutic strategies ranging from pharmacological treatment to deep brain stimulation.
931
Motor and behavioral outcomes after bilateral GPi deep brain
stimulation for severe Tourette syndrome
J. Shahed, J. Poysky, C. Kenney, R. Simpson, J. Jankovic (Houston,
Texas, USA)
Objective: To describe the safety and early efcacy of bilateral deep
brain stimulation (DBS) in the globus pallidus interna (GPi) in 3
patients with severe, medication-refractory Tourette syndrome (TS).
Background: DBS is an emerging therapy for disabling, medically
refractory TS. Debate persists as to optimal patient selection and target
choice. We have previously shown in one patient that GPi DBS can
improve tics and psychiatric co-morbidities (Shahed et al, Neurology
2007.68:159-60).
Methods: Patients underwent pre-operative neuropsychological evaluation to determine suitability for the procedure. They underwent
bilateral staged procedures for DBS placement. DBS parameters were
adjusted according to each individuals symptoms. Tic, cognitive and
behavioral assessments were compared to baseline evaluations to objectively measure progress.
Results: Patient 1: 16 year-old male. At 1 year, the Tic Symptom
Self Report (TSSR) improved by 90%, the Yale Global Tic Severity
Scale (YGTSS) by 76%, and the Rush Video Rating Scale (VRS) by
50%, but tics/minute increased by 63% (Shahed et al, 2007). The
Yale-Brown Obsessive-Compulsive Score (Y-BOCS) improved by
69%. Attention improved. He returned to school full-time in regular
classes. Patient 2: 35 year-old male. At 3 months, TSSR improved
by 16%, YGTSS by 35%, VRS by 17%, tics/minute by 37%, and
Y-BOCS by 24%. He was subsequently able to hold and play with
his newborn daughter. Patient 3: 16 year-old male. At 3 months,
TSSR improved by 48%, YGTSS by 38%, VRS by 19%, tics/minute
by 69%, and Y-BOCS by 22%. He returned to school full-time with
improved attention and academic performance. No surgical adverse
events occurred. Patient 1 pushed repetitively on the IPG site. High
stimulator settings (up to 5V, 210sec, 185Hz) were used to optimize benet.
Conclusions: In 3 patients with severe TS, GPi DBS appears safe and
early results demonstrate effectiveness. Patients who were otherwise
disabled can achieve improved psychosocial functioning. Systematic
assessments of tics and co-morbidities are required when managing
such cases. The use of several measures provides a more complete
impression of symptom severity and impairment. Presence of signicant co-morbidities should not preclude treatment with DBS, as they
may in fact improve with this treatment.

S283

centrotemporal epilepsy (tics did not worsened with carbamazepine),

and another one diagnosed with myoclonic epilepsy who developed a
typical Tourettes syndrome after starting a therapy with valproic acid.
Methods: Case 1. A 9 years old male was evaluated in 1999 because
of one year history of simple motor tics consisting of facial grimacing
and shoulders shrugging, and simple phonic tics consisting of snifng,
without obsessive-compulsive or attention decit/hyperactivity disorders. Two months later, he had two oculofacial clonic movements. An
EEG showed right centrotemporal epileptiform activity consisting on
spikes. Brain MRI was normal. Tics were not treated because they had
mild intensity. Seizures were controlled with carbamazepine 300 mg/
day. Two years later the patient had neither seizures or tics and
carbamazepine was withdrawn. The patient remains asymptomatic
from both disorders during the last ve years.
Results: Case 2. A 14 years old male, following tetanus vaccination,
began with episodes of myoclonic twitches in the four limbs. An EEG
showed generalized polyspike-wave activity. After a treatment with
valproic acid 500 mg/8 hours and clonazepam 0.5 mg/12 hours myoclonic twitches disappeared and EEG became normal. Then, the patient
developed motor tics consisting of right shoulder shrugging and neck
rotation to the right, together with phonic tics consisting of throat
clearing. Tics were controlled with ziprasidone 20 mg/12 hours.
Conclusions: We report two unusual cases of tics disorders associated, respectively, with epilepsy with centrotemporal spikes and with
generalized myoclonic epilepsy. It can not be excluded that this association occurred by chance. In case 1, tics were not worsened by
carbamazepine. In case 2 the onset of tics occurred after starting
treatment with valproic acid; therefore it could be considered the
possibility that tics could be related with this drug (tics have not been
described as side effect of valproate).
933
Early-onset Tourette syndrome
F. Richer, P. Lesperance, S. Chouinard, G. Rouleau (Montreal,
Quebec, Canada)
Objective: This study examined whether early-onset Gilles-de-laTourette syndrome (TS) is associated with a specic symptom prole.
Background: TS involves a variety of phenotypes in which tics,
compulsions and other symptoms can show wide variations in severity.
Information on the sources of this phenotypic variability should facilitate progress on the etiology and pathophysiology of this neurodevelopmental disorder. The onset of clinically signicant tics in TS is often
between 5 and 8 years. However, tics can appear much earlier in life
and few studies have examined the characteristics of early-onset TS to
determine if and how they differ from phenotypes with a later onset of
tics.
Methods: We analyzed data from 184 individuals with TS aged 9-18
to delineate some of the variables associated with early-onset TS.
Subjects underwent evaluations by trained professionals using standard
scales assessing tics, obsessive-compulsive symptoms, ADHD, and
other behavioral symptoms as part of a larger investigation.
Results: Early onset of tics (0-4 years) was associated with higher
severity of vocal but not motor tics, as well as with the presence of
echolalia and coprolalia. These associations were not linked to the
patients age at evaluation, gender, or associated co-morbidities.
Conclusions: Early-onset TS appears to be linked to the severity of
vocal symptoms.

932
Tic disorders associated to epilepsy: 2 cases
H. Alonso-Navarro, T. Adeva-Bartolome, F.J. Jimenez-Jimenez
(Salamanca, Spain)
Objective: To report 2 patients with tics associated to epileptic
syndromes.
Background: The association between Tourettes Syndrome and
epilepsy has been infrequently reported in the literature. Some antiepileptic drugs, such as phenytoin, carbamazepine, and lamotrigine, can
induce tics in epileptic patients, simulating Tourettes syndrome. We
report one patient diagnosed with Tourettes syndrome who developed

TREMOR
934
A new familial disorder: Saccadic oscillations of the eyes
A.G. Shaikh, K. Miura, L.M. Optican, S. Ramat, R.J. Leigh, D.S. Zee
(Baltimore, Maryland, USA)
Objective: We discovered a novel familial disorder saccadic oscillations of the eyes. Using combined clinical, experimental and,
computational approach we identify possible underlying molecular
decits.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S284 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: A fundamental principle of motor control is reciprocal
innervation: when an agonist muscle contracts, its agonist muscle is
inhibited. This behavior makes movements fast and accurate and is
assured by mutual inhibition within the circuits of premotor neurons
that drive the motor neurons that innervate pairs of agonist and antagonist muscles (Fig 1A). Such mutual inhibition, however, can predispose a neural circuit to instability and oscillations (Fig 1B). Appropriately tuned properties of neural membranes and superimposed
inhibition are natures solution to unwanted oscillatory behavior. We
propose that certain familial movement disorders are due to instabilities in the central circuits of mutually inhibitory premotor neurons. We
hypothesize these instabilities reect alterations in intrinsic membrane
properties of premotor neurons and inadequacy in external inhibition,
which, in turn, are due to inherited dysfunction of ion channels, i.e., a
channelopathy.

Methods: We used quantitative eye-tracking techniques to study the

behavior of our patients, and developed a conductance-based neuromimetic model of the premotor neural network within the brain stem that
generates saccades to develop a specic hypothesis for the mechanisms
underlying their saccadic oscillations (Fig 2).
Results: Mother and daughter who had intermittent saccadic oscillations, and consequent brief periods of impaired vision. Unlike the
healthy subject the patient had nearly continuous, small-amplitude, 20
Hz saccadic oscillations around all three axes of eye rotation (horizontal, vertical, and torsional; Fig.1C). The computational neuromimetic
model suggests that saccadic oscillations can be caused by a channelopathy causing reduced external inhibition of reciprocally innervating neural circuit.
Conclusions: This combined clinical, experimental and computational approach has implications for understanding the pathophysiology
and for developing rational therapies for abnormal oscillations in a
variety of motor systems including those that lead to common neurological disorders such as familial essential tremor of the head and arms.

935
Novel molecular mechanism of essential tremor a computational
approach
A.G. Shaikh, S. Ramat, L.M. Optican, K. Miura, D.S. Zee
(Baltimore, Maryland, USA)

FIG. 1 (934).

FIG. 2 (934).

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: Predicting possible neurological correlates of essential

tremor (ET) by simulating its neuromimetic computational model.
Background: ET is a common neurological disorder but its source is
still uncertain. ET has been attributed to oscillations in olivocerebellar
or in thalamo cortical circuits, but no anatomical abnormalities have yet
been described. Here we propose that specic abnormalities in membrane properties underlie ET, and that they are determined by specic
genetic mechanisms. The key features of this hypothesis are 1) there is
an inappropriately large strength of the post-inhibitory rebound (PIR,
which is the rebound burst of discharge that occurs when inhibition is
removed from a bursting type of neuron) and 2) instability arises in a
neural network formed by agonist and antagonist excitatory and inhibitory burst neurons that are interconnected by relatively low latency
local feedback loops. The putative circuit of bursting could encompass thalamic cortical relay neurons (TC) and neurons within the
thalamic reticular nucleus (TR). These neurons exhibit hyperpolarization activated cation currents (Ih) and low threshold calcium currents
(IT), which play a fundamental role in generating and controlling the
strength of PIR.
Methods: We created a biologically plausible (neuromimetic) model
of a cerebello-thalamocortical neural network with TC and TR neurons
(Figure 1).
Results: Altering the membrane properties of TC and TR neurons
consistent with increased expression or activation of Ih or IT, we
increased PIR which produced an oscillation that resembles that of
essential tremor (Figure 2). Physiologically realistic membrane properties of model TC and TR neurons results in the range of frequencies
(4-6 Hz) of tremor that is observed clinically. Clinical recordings of ET
show modulation of the amplitude and frequency of the primary tremor
waveform. If tremor amplitude and frequency uctuate and are not
independent, the cause could be combined signals from independent
oscillators. Simulating ET with multiple oscillators, with minor (yet
physiologically realistic) differences in neuronal membrane properties,
mimics additional clinical features of ET.
Conclusions: Genetically determined increase in the expression or
activation of ion channels carrying Ih and IT is a plausible explanation
for ET being a familial trait.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

S285

electrotonic coupling. Animal models of IO hypertrophy show progressively developing high-conductance through soma-somatic connexin gap junctions resulting in synchronized discharge of the IO
neurons at approximately 2 Hz. Therefore we hypothesize increased
electrotonic coupling through connexin in the hypertrophied IO
might be a neurophysiological correlate of ocular and/or palatal
tremor (Figure 2). Pharmacotherapy with a selective connexin
blocker - quinine may offer an attractive option to control possible
inter-neuronal coupling and thus oscillations.
Methods: One patient with ocular oscillations resulting from a pontine stroke was treated with quinine sulfate (325 mg four times a day).
Results: The treatment signicantly reduced the amplitude of the
vertical but not the horizontal component of the ocular oscillations in a
dose-dependent manner. Quinine therapy signicantly reduced median
vertical eye velocity by 52%.
Conclusions: We propose oscillations in OPT could be due to increased coupling through the connexin mediated gap junctions in
hypertrophied IO and selective blocker of connexin such as quinine
could extend a therapeutic benet.

FIG. 1 (935).
936
Connexin gap junctions neurophysiological correlate and therapeutic target for oculopalatal tremor
A.G. Shaikh, S. Hong, D. Solomon, K. Liao, L.M. Optican,
R.J. Leigh, D.S. Zee (Baltimore, Maryland, USA)
Objective: Oculopalatal tremor (OPT) could be due to increased
coupling through the connexin mediated gap junctions in hypertrophied
IO. We used a selective blocker of connexin - quinine to test this
hypothesis and investigate its possible therapeutic benet.
Background: OPT, often recognized as oculopalatal myoclonus is
associated with inferior olive (IO) hypertrophy commonly a late
sequel to brainstem or cerebellar stroke. The clinical phenotype
includes ocular oscillations and a palatal tremor at 2-3 Hz frequency. The IO hypertrophy has been attributed to denervation from
interruption in projection from the cerebellar nuclei to the contralateral olivary nucleus via the central tegmental tract (Figure 1). A
group of transmembrane proteins - connexins form the gap junctions
and allow ion conduction between neighboring neurons to facilitate

FIG. 1 (936).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S286 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

937

939

The effect of muscle loading on tremor dynamical characteristics in

the essential tremor patients
S. Blesic, J. Maric, N. Dragasevic, S. Milanovic, V.S. Kostic,
M.R. Ljubisavljevic (Al Ain, United Arab Emirates)

An open label study of pramipexole for the treatment of essential

tremor
L. Lay-Son, D. Saez, O. Trujillo (Santiago, Chile)

Objective: The aim of the present study was to further investigate

scaling properties of the postural hand tremor in subjects with ET, and
the effect of peripheral muscle loading on the scaling properties.
Background: It has recently been shown that frequency harmonics
found in power spectra of pathological tremors contain information on
scaling properties in the period dynamics of multimodal oscillation
series, with each nite frequency band (mode) corresponding to uctuation period of single independent oscillator. In our previous studies,
it was shown that, in some pathological tremors, different modes of
oscillations display different scaling patterns. This property was not
clearly visible in the case of the essential tremor (ET) in postural
conditions.
Methods: The time series of nger tremor movements were recorded
in 20 ET patients, three times in a single session, from both hands, with
and without 500-g wrist cuff loading. The time series were analyzed by
detrended uctuation analysis and dynamic calculated for the rst to
spectra harmonics.
Results: Our results show that the addition of weight signicantly
changes the dynamics of the rst two frequency modes of tremors.
Namely, the dynamical characteristics of the main two frequency
components of the recorded tremors change in value after the addition
of load. In addition, the main two components, otherwise indistinguishable without load, become signicantly different after weight loading.
The effect was more pronounced on the tremor dominant side, and
shows the dependence on the handedness of ET patients.
Conclusions: The results indicate that weight loading can help to
distinguish between the peripheral and central components of ET and
may suggest the presence of a single oscillator in ET.

Objective: To evaluate efcacy of pramipexole in a short-term

open-label study in order to determine whether this dopamine agonist warrants further investigation as a treatment of Essential
Tremor (ET).
Background: Essential Tremor (ET) is one of the most common
movement disorder specially in aging. Treatment with propanolol,
primidone, clonazepam, gabapentin or topiramato have demonstrated limited effectiveness and the presence of intolerable side
effects. The effect of pramipexole in ET has not been reported in the
literature.
Methods: The authors conducted a 8-week, open-label trial of
pramipexole as monotherapy in four patients with ET. Patients were
assessed with the complete Tremor Rating Scale (TRS) and the Clinical
Global Impression (CGI) at baseline, and after 8-weeks of treatment
with a standard-dose of 0.25 mg bid. All four subjects were female,
mean age was 69.5 /- 4.7 years, range disease duration was 2-50
years, and only 1 patient had a family history of ET.
Results: All patients showed an improvement in the overall score
of the TRS. The percentage range were from 35 to 74% of improvement compared with baseline. The most signicant improvement
was in the subscale related with the tremor. In the TRS writing
section, water pouring section, and activities of daily living section
the reduction of the scores was lesser that in the tremor subscale.
Related to the CGI-improvement, subjects rated themselves as much
improved (n1) and moderately improved (n3). Pramipexole was
well tolerated, the adverse events were nausea in one patient, and
somnolence in another one for a few days at the beginning of the
trial.
Conclusions: According to our preliminary data pramipexole can be
effective and safe as therapeutic agent in ET. A double-blind, placebocontrolled trial of pramipexole is highly warranted.
940
Disappearance of essential tremor after capsular infarction
N.S. Oztekin, M.F. Oztekin (Ankara, Turkey)

938
Effectiveness of piracetam in action tremor/myoclonus of patients
with Parkinsons disease
R. Neshige (Kurume City, Fukuoka, Japan)
Objective: To study the effect of piracetam on action tremor/myoclonus in Parkinsons disease (PD).
Background: From our observation, approximately more than twothirds of PD has had action tremor/myoclonus in their courses of the
disease. Typical resting tremor has hardly any effect on the execution
of voluntary movement and shows relatively good response to levodopa. On the other hand, action tremor/myoclonus, which shows poor
response to levodopa, may interfere with dextrous movements and can
therefore be source of signicant disability.
Methods: Ten patients disturbed severely by their action tremor/
myoclonus were studied. Five patients entered the open-label study and
the other 5 patients entered the crossover, double-blind, placebo-controlled study. Another 2 patients with mainly typical resting tremor also
entered the open-label study. In the crossover, double-blind study
he/she received 12 g of piracetam or placebo daily for 2 weeks,
followed by identical placebo or piracetam.
Results: After the administration of piracetam, there was subjectively
signicant improvement in all 10 patients with mainly action tremor/
myoclonus although there were no improvements in 2 patients with
mainly typical resting tremor.
Conclusions: Piracetam was an effective treatment of patients with
PD who were disturbed severely by action tremor/myoclonus.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: Essential tremor(ET)is the most common adult-onset

movement disorder characterized by a 4-12 Hz postural and kinetic
tremor. Although the precise localization of the primary pathology
of essential tremor is unknown, the inferior olive and cerebellerthalamic-cortical loops have been implicated in the pathogenesis of
this disorder. The aim of the study is to emphasize the role of these
structures reporting a patient whose ET disappeared after capsular
infarction.
Background: The main pathophsyological hypotheses is the disturbance of olivoserebeller rhythmicity transmitted via the cerebellothalamocortical pathway. In the stroke literature, most reports
have proposed that ET disappears after lesions of the part of
cerebello-thalamo-cortical loop such as cerebellum, pons or thalamus.
Methods: We present a patient with essential tremor who spontaneously improved after a sensorimotor stroke related to a right
posterior internal capsule infarction extending to the lentiform nucleus. This nding supports the presence of cortical or transcortical
motor loops that are likely involved in essential tremor and suggests
a possible link with the cerebellar-thalamic-cortical pathway.
Results: Our patient a 70 year-old man admitted with left hemiparesis. He reported that he had tremor in both upper extremities.He
reported that his left arm tremor had disappeared simultaneously with
his left hemiparesis. The cranial MRI revealed a decreased attenuation
in the right posterior internal capsul that extended in to lentiform
nucleus. The EMG study revealed tremor frequency in the 5 Hz. Range
in the right arm extensor muscles. His left ET had simultaneously
disappeared with left hemiparesis. At the 3rd month follow up examination; although there was no tremor on his left arm, there was tremor

POSTER SESSION III, THURSDAY, JUNE 7, 2007

on his right arm. Neurological examination revealed that strength in his
left arm returned to normal.
Conclusions: Cerebello-thalamo- cortical pathway involvement is
thought to be responsible for pathogenesis of ET in our patient. This is
consistent with the literature. In addition, although left hemiparesis has
improved completely during follow up period, ET has not reappeared.
941
Impaired motor speech and balance control in essential tremor
M. Kronenbuerger, P. Buderath, B. Frank, C. Fromm, V.A. Coenen,
V.M. Tronnier, K.L. Kiening, W. Ziegler, D. Timmann (Aachen, Germany)
Objective: To assess different areas of motor control, including
speech, posture, and tandem gait as evidence of cerebellar compromise
in essential tremor (ET).
Background: It is believed that ET is a mono-symptomatic tremor
disorder with clinical signs of cerebellar dysfunction present in some
ET subjects in the advanced stage. If tremor in ET is related to a
disturbance of olivocerebellar pathways leading to cerebellar dysfunction, one may expect dysarthria, postural instability, and disturbance of
tandem gait in ET.
Methods: Dysarthria by the use of a syllable repetition task (Modias),
balance control by the use of dynamic posturography (EquiTest), and
the number of missteps on tandem gait over 2 minutes were assessed in
25 ET subjects (mean age 47 23 years) in different stages of ET
(mean disease duration 17 16 years, range 2 to 58 years), and in 25
healthy controls (mean age 47 21 years) on two consecutive days.
Additionally, 12 ET subjects (mean age 64 11 years) in the advanced
stage of ET requiring Deep Brain Stimulation (DBS) of the ventrolateral thalamus were studied with DBS turned off and on in a random
sequence. None of the participants took medication known to affect
central nervous system functioning.
Results: ET subjects had a reduced maximum syllable repetition rate in
the speech test, swayed more as well as had more falls in the dynamic
posturography, and made more missteps on tandem gait than did controls
(p 0.05 ANOVA). DBS tended to reduce the number of missteps on
tandem gait and falls on the dynamic posturography, but worsened sway
(effects of DBS were not signicant). Please see the table attached.
Conclusions: (i) Reduced maximum syllable repetition rate, increased postural instability, and tandem gait disturbance are additional
evidence for motor impairment in ET which may be related to cerebellar dysfunction. (ii) Results point to motor disorders consistent with
cerebellar disease in early stages of ET. (iii) Cerebellar motor functions
are not necessarily worsened with DBS activation.

Table
Controls

ET-Subjects

Day 1 / Day 2

Day 1 / Day 2

ET-DBS-Subjects
DBS-OFF / DBSON*

syllable duration
176.6 26.3 /
192.2 27.7 /
190.9 19.1 /
[ms]
173.3 31.5
199.7 45.9
193.7 17.4
sway area (i.e. in
condition 5)
31.4 24.7 /
34.8 26.9 /
[cm2]
15.8 14.2 / 11.4 9.0
28.1 27.3
39.8 39.9
falls per completed
EquiTest
0.24 0.6 / 0.0 0.0 1.4 1.9 / 0.9 1.9 1.4 1.3 / 0.7 0.8
missteps over 2
min tandem
gait
1.0 2.5 / 1.4 3.4
3.4 3.5 / 2.8 3.1 2.8 4.5 / 1.2 1.1

values refer to means standard deviation; *tested in randomised order

942
Essential tremor easy to see, difcult to describe and control
N. Yardimci, S. Benli (Ankara, Turkey)
Objective: The traditional view that essential tremor (ET) is a familial monosymptomatic disorder with a benign prognosis has recently
been challenged, as it is now known to be a progressive and clinically
heterogeneous condition with sporadic and familial forms.In classic ET
upper limbs(95% of patients) and less commonly the head(34%),
lower limbs(30%), tongue(7%), face(5%) and trunk(5%) exhibit a postural or kinetic tremor. The pathogenesis of ET is not fully

S287

understood, though electrophysiologic studies are consistent with a

central source of tremorogenic oscillation with possible modulation by
muscle adrenoceptors.A disturbance of olivocerebellar rhythmicity is at
present the most probable hypothesis for the etiology of ET.
Background: Presently there is no cure for essential tremor nor are
there any medications that can slow the progression of tremor.Propranolol and primidone are commonly used to treat ET, although
propranolol is the only medication that is approved by the Food and
Drug Administration (FDA) for this purpose.It is estimated that at least
30% of patients with ET will not respond to propranolol or primidone
so additional therapies are required.
Methods: We performed a literature review using Medline, Science
Citation Index to identify clinical trials in patients with ET published
between 1966 and 2006 regarding relative benets and risks of pharmacological and surgical therapies.
Results: Propranolol and primidone reduce limb tremor.Alprazolam,
atenolol, gabapentin (monotherapy), sotalol, and topiramate are probably effective in reducing limb tremor. Propranolol reduces head
tremor. Clonazepam, clozapine, nadolol, and nimodipine possibly reduce limb tremor. Botulinum toxin A may reduce hand tremor but is
associated with dose-dependent hand weakness. Botulinum toxin A
may reduce head tremor and voice tremor, but breathiness, hoarseness,
and swallowing difculties may occur in the treatment of voice tremor.
Chronic deep brain stimulation (DBS) and thalamotomy are highly
efcacious in reducing tremor but each procedure carries a small risk of
major complications.
Conclusions: Pathological and genetic studies will yield new insights
into disease pathogenesis and mechanisms, which may result in the
development of more effective symptomatic therapies including neuroprotective strategies.
943
Health-related quality of life in essential tremor patients undergoing deep brain stimulation
C. Kenney, A. Diamond, A. Davidson, L. Shinawi, J. Jankovic
(Houston, Texas, USA)
Objective: To determine the effect of ventral intermedius (VIM)
deep brain stimulation (DBS) on health-related quality of life
(HRQoL) in essential tremor (ET) patients using disease-specic
instruments.
Background: Several studies have concluded that DBS improves
motor function in medically-refractory ET patients; less emphasis has
been placed on HRQoL measures. Generic HRQoL scales are multidimensional questionnaires that cover a wide variety of areas and can
be applied to many diseases, but may lack sensitivity in areas important
to ET, such as tremor or social embarrassment.
Methods: ET patients who underwent VIM-DBS were assessed
prospectively using several clinical scales at baseline and 6 months
after implantation: Tremor Rating Scale (TRS), Quality of Life in
Essential Tremor Questionnaire (QUEST), Questions on Life Satisfaction Module (QLSm), Mini-Mental Status Examination (MMSE), and
Geriatric Depression Scale (GDS).
Results: At total of 7 patients (4 male), age 67.9 13.9 years,
consented to be enrolled in this study. The TRS improved by 66.6%
(p0.001) from baseline to 6 months (Table 1). Three portions of
the QLSm improved signicantly including QoL in relationship to
leisure activities/hobbies, controllability/uidity of movement, and
hand dexterity. The total QUEST score improved from 39.3 6.2
to 13.0 6.4 (p0.004). On average, patients were moderately to
very satised with several variables related to the neurostimulator:
reliability, inconspicuousness, manipulation, and absence of false
bodily sensations. Depression improved signicantly while MMSE
scores did not changed appreciably.
Conclusions: Improvements in motor function for ET patients undergoing VIM-DBS translate into improved QoL using disease-specic
clinical scales.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S288 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Tremor rating scale

Geriatric Depression Scale
Mini Mental Status Examination

Baseline

6-month follow-up

Statistical signicance

56.9 3.6
3.6 1.7
29.5 0.3

19.0 3.2
2.1 1.7
29.3 0.5

p0.001
p0.04
p0.61

944
The spectrum of orolingual tremor a proposed classication
system
M.A. Silverdale, S.A. Schneider, K.P. Bhatia, A.E. Lang
(Manchester, United Kingdom)
Objective: To develop a classication scheme for orolingual tremor.
Background: Orolingual tremors include tremors of the jaw, tongue,
pharynx and face. There is currently no accepted classication scheme
for such tremors. A proper classication scheme for orolingual tremors
should aid in providing more accurate diagnosis and permit future
research studies.
Methods: We performed a literature search on orolingual tremor
(OLT), and reviewed cases and videos of OLT presenting to the
Toronto Western Hospital Movement Disorders Center and the Institute of Neurology, Queen Square.
Results: A classication scheme for OLT was developed, based
on the consensus statement of the Movement Disorders Society on
tremor (1). We included eight of our own cases in this classication
system, providing examples of several types of OLT which have not
previously been reported. The proposed classication includes physiological OLT, enhanced physiological OLT, classical essential
OLT, indeterminate OLT syndrome, task- and position-specic
OLT, orthostatic OLT, dystonic OLT, OLT associated with dystonia, parkinsonian OLT, palatal tremor with OLT, drug-induced OLT
and psychogenic OLT.
Conclusions: The spectrum of orolingual tremor covers a wide
range of movement disorders. As the eld of orolingual tremors is
continuously developing, they are likely to remain orolingual tremors which do not easily t into the proposed preliminary classication system. We encourage others to publish cases of unusual
orolingual tremor, so that the classication of the condition can be
rened and improved.
945
Evaluation of postoperative outcome on tremor due to posterior
fossa tumors
T.M. Kinfe, H.-H. Capelle, J.K. Krauss (Hannover, Germany)
Objective: To investigate the postoperative course of patients with
posterior fossa tumors suffering from tremor.
Background: Tumors lesions of the posterior fossa affecting the
dentatothalamic pathways have been shown to be associated with
tremor in some instances. Thus far, little is known about the impact of
tumor removal in these patients.
Methods: This series included a retrospective evaluation of six
patients with posterior fossa tumors. Patients with a family history of
movement disorders or neuroleptic medication were excluded. All
patients had postural or kinetic tremors. Tremor was mainly unilateral.
There were ve women and one man. Mean age at surgery was 59 years
(range 45-80). Five patients underwent total or subtotal resection, one
patient had a stereotactic biopsy. The histological diagnosis was epidermoid in two patients, metastasis in two other patients and vestibular
schwannoma and low-grade glioma in one, respectively.
Results: Two patients had no improvement of tremor, postoperatively. In both patients the tumor involved the dentate nucleus directly
(one patient with low-grade glioma, and one patient with a metastasis).
In the other patients in whom a compressive effect on the dentate
nucleus or the dentatothalamic pathways was present without invading
the cerebellar structures, gradual postoperative amelioration of the
tremor was observed.
Conclusions: The postoperative course of tremor due to posterior
fossa tumors depends mainly on the involvement of the dentate nucleus
or the dentatothalamic pathways. While in patients with primary in-

Movement Disorders, Vol. 22, Suppl. 16, 2007

volvement of these structures tremor does not show an amelioration

after surgery, tremor in those patients with compression of tremorgenerating structures responds favorably.
946
Unilateral tremor associated with autosomal dominant essential
tremor
P. Hedera, F. Phibbs, J.Y. Fang, P.D. Charles, M.K. Cooper,
T.L. Davis (Nashville, Tennessee, USA)
Objective: To analyze the prevalence of unilateral tremor in a cohort
of families with autosomal dominant (AD) essential tremor (ET).
Background: Diagnosis of ET is based on clinical criteria only. The
presence of bilateral, predominantly symmetrical postural and/or kinetic tremor involving mostly upper extremities and the absence of
additional neurologic signs, such as dystonia or parkinsonism support
the diagnosis of ET. Unilateral upper extremity tremor is not typical for
ET and accepted diagnostic criteria classify unilateral arm tremor as
possible ET if additional neurologic signs are absent.
Methods: We have analyzed the presence of unilateral arm tremor in
familial ET cohort consisting of 133 kindreds with 412 affected individuals. Inclusion criteria in patients with unilateral arm postural and/or
kinetic tremor required the duration of tremor for at least ve years,
without hypokinetic-rigid syndrome, dystonic posturing, or history of
sudden onset of tremor. Patients who had also resting tremor had to fail
dopaminergic challenge with at least two different medications. We did
not include patients with a task-specic tremor. Furthermore, we analyzed only subjects with at least one living rst degree relative (parent,
sibling, offspring) who met diagnostic criteria for denite ET based on
the presence of bilateral tremor, conrmed by a member of our research
group. Individuals with unilateral tremor without a known family
history or with only historical evidence of additional relatives with
otherwise typical ET were excluded.
Results: Twenty-one subjects from nineteen families met the inclusion criteria. The majority of them (16/21) had strictly unilateral tremor
and ve had a minimal, ne postural tremor affecting the contralateral
extremity. We have also identied an additional 23 individuals from
our cohort who had denite ET but reported the previous history of
unilateral arm tremor ranging from 11 to 33 years before the onset of
a contralateral tremor.
Conclusions: Our data shows that individuals with unilateral tremor
can be identied in 14% (19/133) of familial AD ET kindreds, suggesting that unilateral tremor in ET may be more common than previously recognized.
947
Internal Family Systems psychotherapy successfully applied in two
cases of psychogenic tremor
F.P. Le Doze, L. Carluer, G.L. Defer, R.C. Schwartz (Caen Cedex,
France)
Objective: To assess the impact of Internal Family Systems (IFS)
psychotherapy in two cases of psychogenic tremor.
Background: Psychogenic movement disorders are therapeutically
challenging although cognitive behavioral, psychodynamic therapies
and hypnosis along with pharmacological treatments have shown to be
effective (V.K. Hinson, 2006).The IFS model of psychotherapy was
developed by R. Schwartz (1995) who discovered ways to resolve inner
polarizations and to unload extreme beliefs and emotions that maintain
many emotional and some somatic symptoms. The treatment involves
helping clients focus internally on the extreme beliefs and emotions
(carried by sub-personalities called parts) that surround their symptoms and create new inner dialogues among the parts and between the
parts and the Self, considered as the core of the psyche.
Methods: Case report 1 : 44 y.o. woman affected by parkinsonianlike right arm tremor, worsening over one year and signicantly interfering with daily life. Case report 2 : 51 y.o. man affected by disabling
trunk shaking enhanced by sitting and standing up positions. In both
cases, no neurological disease or pharmacological etiology could be
disclosed; the criteria of variability and distractibility were fullled. 4

POSTER SESSION III, THURSDAY, JUNE 7, 2007

IFS therapy sessions in 4 to 6 months were needed. (Videos before and
after treatment available).
Results: CR 1 : progressive improvement since the rst session
resulting in a complete and sustained (over an 18 month follow-up
period) disappearance of the symptom. CR 2 : partial but signicant
improvement with loss of disability.
Conclusions: On the basis of these cases although a larger experience
is needed, IFS psychotherapy is effective in the management of some
psychogenic movement disorders and allows an explanatory mapping
of the psychological conicts and psychodynamic basis related to the
symptom. Its acceptance as a psychotherapy by medical patients is
high. Self-leadership used as a therapeutic tool makes this approach
particularly innovative and promising in the medical eld.
948
Dopa- responsive pseudo-orthostatic tremor in parkinsonism
A. Thomas, L. Bonanni, S. Varanese, F. Anzellotti, K. Armellino,
A. DAndreagiovanni, D. Monaco, M. Onofrj (Pescara, Italy)
Objective: To describe peculiar form of tremor appearing (or severely increasing) while standing, inabilitating standing posture, markedly responding to L-Dopa administration refractory to gabapentin.
Background: A number of studies reported OTs preceding or following PD, but L-Dopa did not reduce the fast OT of these patients
and standing inability. In 5 of 8 cases of Primay OT , with the 14-16HZ
frequency, L-Dopa induced a reduction of standing tremor, as stated on
visual analogue scales9 in one of these cases clear PD symptoms were
evidenced.
Methods: The tremor was assessed with UPDRS subscale III The
severity of the possible presence of a postural standing tremor was
measured with the clinical rating scale for tremor. Tremor was also
measured with surface EMG of weight bearing muscles. In patients 3
and 4 the standing tremor was EMG recorded before and after the
occurrence of PD symptoms. In patients 1 and 2 EMG recordings were
only performed when PD symptoms had already occurred.
Results: In all patients EMG recording of the standing tremor
showed maximal amplitude at the frequency of 6.0-7.0 Hz with sporadically overlapping irregular faster activities, with the classic reciprocal alternating pattern of tremor. FA analysis showed dominant
frequency at 6.2-71 and sporadic subharminics (1/10 of the dominant
frequency amplitude) at 13.0-14.2 Hz. Dopaminergic treatment induced
the subjective disappearance of tremors during standing in all patients
in on condition. TR ratings and UPDRS scores showed that L-Dopa
administration reduced tremor in all patients.
Conclusions: We found evidence of a peculiar form of standing
tremor dramatically responding to L-Dopa accompanied by denite
parkinsonian symptoms. Genetic mutations were Park 2, Park 6 and a
mtDNA deletion, thus evidencing that this form of standing tremor is
not specically linked to a genetic aetiology and appears symptomatically before the diagnosis of parkinsonism could be dened. We
propose therefore that in the 4 described cases a denition as Pseudo
OT could be appropriate because of the dramatic L-Dopa response and
of the frequency of tremor. Pseudo OT could be used in different
assessments of tremors separating Primary OT and further variants like
Fast OT and OT-Plus.
949
Lack of association between catecholamine-O-methyl transferase
Val158Met polymorphism and essential tremor
E. Ergul, A. Sazci, K. Bayulkem (Kocaeli, Turkey)
Objective: To investigate the role of catecholamine-O- methyl transferase Val158Met polymorphism in essential tremor.
Background: Essential tremor (ET) is the most common human
movement disorder of unknown etiology.
Methods: We analyzed the alleles and genotypes of COMT
Val158Met polymorphism in a total of 167 unrelated essential tremor
patients and compared them with those of 171 unrelated healthy control
subjects, using a polymerase chain reaction restriction fragment length
polymorphism method.

S289

Results: The allele frequency of COMT L was 43.11% in the

essential tremor patients, and 42.69% in controls.The expected allele
frequency of COMT-L was 42.90% in essential tremor patients and
42.89% in controls. The frequencies of COMT-HH, HL, and LL
genotypes were 26.3%, 61.1%, and 12,6% in the essential tremor
patients, and 25.1%, 64.3%, and 10.5% in controls.
Conclusions: COMT Val158Met polymorphism is not associated
with essential tremor (Chi20.497;df2;P0.780).
950
Adult-onset Alexander disease with palatal tremor and intraventricular tumor
Y. Okuma, T. Hirayama, J. Fukae, K. Noda, K. Fujishima,
N. Hattori (Izunokuni, Shizuoka Prefecture, Japan)
Objective: To describe a patient with adult-onset Alexander disease
(AD) who showed palatal tremor and intraventricular tremor.
Background: AD is a rare leukoencephalopathy caused by dominant
mutations in the GFAP gene. Adult-onset AD is the most variable and
least common form. Ventricular tumor has never been reported.
Methods: Case report.
Results: A 33-year-old woman noted unsteady gait and speech
disturbance at age 28. She was admitted to our hospital at age 29. On
admission, mental status was normal except for occasional emotional
incontinence. She had palatal tremor and her voice was tremulous. Her
gait was slightly ataxic and spastic. Deep tendon reexes were increased and Babinskis sign was present bilaterally. MRI showed
hyperintensities on T2 WI in the periventricular white matter and the
brainstem. Intraventricular tumor was observed in the lateral ventricle
around foramen of Monro. Her son had a febrile seizure at age 4, and
MRI showed T2 hyperintensities in the frontal white matter. A novel
GFAP mutation (R376W) was identied in both the patient and her son.
The patient became quadriparetic, and underwent PEG for dysphagia.
Conclusions: Adult-onset Alexander disease should be considered in
patients who show progressive ataxia, spasticity, and palatal tremor.
Association of AD and ventricular tumor may be conrmed by future
studies.
951
Conrmation that dystonic tremor with features of parkinsonism is
a cause of scans without evidence of dopaminergic decit
(SWEDDs)
D.J. Hensman, J.W. Frank, P.G. Bain (London, United Kingdom)
Objective: To further investigate the possible causes of normal DAT
scans, namely scans without evidence of dopaminergic decit
(SWEDDs), in the context of parkinsonism.
Background: SPECT imaging of the dopamine transporter, using
123
I-FP-CIT (DaTSCANTM), reveals depletion of the nigrostriatal dopaminergic pathway in Parkinsons disease (PD). However, about 10%
of patients entering clinical trials of early PD have normal presynaptic
dopamine imaging (SWEDDs). We present data supporting a previous
publication that some of the SWEDDs patients may have dystonic
tremor with features of parkinsonism (Bhatia et al. Mov Disord 2006;
21 (sup15): S707-8).
Methods: 5 female RH patients (mean age: 63yrs; range 49-77) with
dystonic tremor and features of parkinsonism underwent 123I-FP-CIT
imaging. The scans were visually reported and compared to patients
data over a mean follow-up period of 1.4 years (range 0.5-4 years).
Results: DAT imaging was normal in every case. Two patients had
a history of tremor in a rst degree relative. At onset tremor affected the
right hand (n2), both hands (n1)and the head (n2). Subsequently,
at the time of scanning, all 5 patients had bilateral action tremor of the
arms, with tremulous spirals, and head tremors (3 complex, 1 yes-yes
and 1 no-no). Three patients had a rest tremor, one a jaw tremor and
one a 4Hz leg tremor on standing. Subtle signs of dystonia were present
in all cases (vocal (n2), cervical (n2) and focal hand (n2)). Mild
features of parkinsonism included: facial impassivity (n2), bradykinesa (non-fatiguing: n2), rigidity (n1), reduced arm swing (n2),
reduced passive shoulder shrug (n1) and gait abnormalities (n5).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S290 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Levodopa markedly worsened tremor in 1 case who was misdiagnosed
as having PD, and had no effect in another; whilst alcohol improved
tremor in 1 and had no effect on 2 (the others did not drink).
Conclusions: Dopamine transporter imaging is normal in patients
with dystonic tremor or dystonia associated tremor. This data is helpful
in distinguishing these patients from those with idiopathic PD, particularly as some of the dystonic tremor patients may have mild signs of
parkinsonism, including rest tremor, which has and could lead to a
misdiagnosis of PD (Hensman and Bain. Mov Disord 2006; 21: 177880) and thus be a cause of SWEDDs.
952
Quantitative tremor analysis in 300 consecutive tremor patients
M. Lohle, T. Glockler, I. Hoffmann, K. Kreutzmann, S. Junghanns,
M. Wolz, H. Reichmann, A. Storch (Dresden, Saxony, Germany)
Objective: To evaluate the epidemiological, clinical and neurophysiological characteristics of tremor syndromes and dene the diagnostic
value of quantitative tremor analysis.
Background: There are only a few studies examining tremor syndromes with quantitative tremor analysis including EMG and accelerometric data. Thus, the diagnostic value of this method for tremor
differentiation is yet to be determined.
Methods: We studied 300 consecutive patients referred to our
university movement disorder outpatient clinic suffering from
tremor syndromes. Syndromic diagnosis of tremor was made according to the MDS clinical criteria and with the aid of functional
imaging (29.3% of patients). All patients underwent quantitative
neurophysiological tremor assessment (EMG and accelerometry) of
both hands.
Results: Most frequent diagnoses were essential tremor (ET;
n99, 33.0%) and Parkinsons disease (PD; n98, 32.7%). Other
tremor syndromes were orthostatic tremor (n5, 1.7%), exaggerated
physiological tremor (n24, 8.0%), dystonic tremor (n12, 4.0%),
cerebellar tremor (n8, 2.7%) and miscellaneous tremor syndromes
(n19, 6.3%). 17.3 % of tremor patients (n52) could not be
classied. We did not nd relevant differences in the neurophysiological features of ET and PD patients, but differences between
exaggerated physiological tremor and centrally generated tremor
syndromes. We found harmonic oscillations in postural tremor in
45.5% of ET and 64.2% of PD patients, but not in patients with
exaggerated physiological tremor.
Conclusions: Quantitative tremor analysis is not able to distinguish
between central tremor syndromes, but only between exaggerated physiological tremor and pathological tremor syndromes.
953
The effect of oxcarbazepine on essential tremor
N. Yardimci, S. Benli (Ankara, Turkey)
Objective: Essential tremor (ET) is one of the most common tremor
disorders in adults with prevalence estimates from population studies
ranging from 0,4% to 5%. ET is characterized by the presence of
postural and kinetic tremor. In general clinical practice, although primidone and propranolol have been regarded as the mainstays of pharmacologic therapy for ET, it is observed that at least 30% of patients will
not respond to either of them.
Background: Antiepileptic drugs, besides their anticonvulsant
activity, have a broad spectrum of action which may be utilized in
other pathological conditions. For example in essential tremor besides primidone, other antiepileptics such as gabapentin, topiramate
and levetiracetam have shown potential to reduce tremor. We report
a novel nding of signicant improvement in essential tremor with
oxcarbazepine.
Methods: Case summary: A 67-year old woman suffering from
severe neuropathic pain attended to our outpatient clinic. Her medical
history included type II diabetes mellitus for 2 years and moderately
severe essential tremor for more than 30 years. For essential tremor she
used to take propranolol 120mg/day with a modest control previously
but because of interfering with her diabetes therapy her endocrinologist

Movement Disorders, Vol. 22, Suppl. 16, 2007

discontinued propranolol. With primidone 125 mg/day she had experienced unbearable somnolence had to discontinue. As a consequence
she was ignoring her tremor which had been existing with her for more
than 30 years when she attended to the neurologist for neuropathic pain.
Her neurologic examination and electrophysiologic recordings revealed
diabetic neuropathy. She was treated with oxcarbazepine and the dose
was gradually increased to 900 mg twice daily.
Results: Amazingly, we have observed that our patient experienced
a signicant improvement in her tremor following the initiation of
oxcarbazepine and the dose increment provided better control both in
neuropathy and tremor.
Conclusions: Essential tremor is a common neurologic disorder with
uncertain pathophysiology. Our case suggests that while the exact
therapeutic action remains unclear, oxcarbazepine may be a new theureupetic choice for essential tremor. To our knowledge, this is the
second case report and further investigations of oxcarbazepine, searching its effect on essential tremor by means of controlled clinical trials
are warranted.
954
Bilateral effects of unilateral deep brain stimulation
N. Kovacs, I. Balas, L. Kellenyi, E. Pal, F. Nagy (Pecs, Hungary)
Objective: To evaluate the possibility of active tremor reduction on
the non-target side during unilateral thalamic deep brain stimulation.
Background: It is unanswered whether unilateral deep brain stimulator (DBS) has only contralateral or bilateral effects on tremor. Previous studies demonstrated a clinically irrelevant improvement on the
non-target side after thalamic DBS implantation, which was considered
as the result of mechanical effects alone.
Methods: Fourteen consecutive patients underwent unilateral Vim
DBS implantation because of drug-resistant tremor were included in
the study. (Eight tremor dominant Parkinsons disease, seven essential tremor, one tremor of other origin mitochondrial encephalomyopathy). All of them previously underwent contralateral
thalamotomy. Simultaneous bilateral triaxial accelerometry, surface
electromyography (sEMG) and video recordings were performed.
After at least 12 hour-long drug withdrawal, the rest and postural
tremor had been recorded in both stimulation on and stimulation off
states for at least 1 hour-long interval. Besides, Fahn-Tolosa-Marin
tremor rating scale was applied in both conditions.
Results: Having turned off the stimulation, postural-kinetic tremor
developed on both the target and the non-target side in only one case.
The co-recording of accelerometry and sEMG proved tremor related
muscle activation behind this phenomenon on both sides. After turning
on the stimulation, the postural-kinetic tremor of both sides disappeared
suggesting bilateral effect of the unilateral Vim DBS.
Conclusions: In a relatively small group of patients, we were able to
nd a case where the unilateral DBS probably had bilateral tremor
reductive effect. However, in the majority of patients only contralateral
effect could be demonstrated.
955
The differences of characteristics in physiologic tremor between
dominant and non-dominant hand in normal population
I.U. Song, J.S. Kim, D.S. Jeong, K.S. Lee (Seoul, Republic of Korea)
Objective: This studys purpose was to investigate electrophysiological differences in physiologic tremor between both hands in healthy
population.
Background: Recently there has been a lot of research about the
characteristics in physiological tremor in healthy individuals. But it has
been still unclear whether handedness affects the physiologic tremor or
not.
Methods: 37 neurologically intact volunteers (27 males and 10
female) were selected to participate in present study, based on the
following criteria: 1) absence of any known neurological disease or
medical condition associated with tremor; 2) absence of any tremorogenic medications; 3) absence of orthopedic or arthritic condition
involving the upper limbs that could inuence the recordings. Using a

POSTER SESSION III, THURSDAY, JUNE 7, 2007

tremor analysis system based on a solid state gyroscopic sensor sensitive to angular rate, the following parameters were determined: angle
rate, amplitude peak frequency and regularity. We analyzed all physiologic resting and postural tremor of both dominant and non-dominant
hands during 20 seconds.
Results: Physiologic resting tremor disclosed no difference between
dominant and non-dominant hands in all above-mentioned ve parameters. However, on postural state, dominant hands appeared lower score
in parameters of angular rate (degree/second), peak frequency (Hz) and
Q coefcient of constancy than non-dominant hands tremor, signicantly. On the dominant hand, there were signicantly lower scores of
angular rate, angular displacement and peak frequency at resting state
than postural state, whereas there was no difference in other parameters. On the non-dominant hand, there were lower scores of all parameters at resting state than postural state, signicantly.
Conclusions: The analysis of physiologic tremor showed signicant
differences between both hands during postural state, while no difference at resting state. Thus, these results were demonstrated that dominant hands could sustain more stability of muscle tone than nondominant hands. We thought that it might be the reason of results in the
present study that common unimanual tasks and ne movements are
mainly performed by dominant hands. In the present study, we could
nd out that asymmetry of handedness disclosed asymmetry of the
physiological tremor as well as preferential use of one limb.
956
Blood harmane concentration is correlated with cerebellar metabolism in essential tremor
E.D. Louis, W. Zheng, X. Mao, D.C. Shungu (New York, New York,
USA)
Objective: To assess in essential tremor (ET) patients whether blood
harmane concentration is correlated with cerebellar N-acetyl aspartate/
total creatine (NAA/tCr), a neuroimaging measure of neuronal dysfunction or degeneration.
Background: On proton magnetic resonance spectroscopic imaging
(1H MRSI), there is a decrease in cerebellar NAA/tCr in ET, signifying
cerebellar neuronal dysfunction or degeneration. Harmane, which is
present in the human diet, is a potent tremor-producing neurotoxin.
Blood harmane concentrations seem to be elevated in ET.
Methods: Twelve ET patients underwent 1H MRSI. The major
neuroanatomical structure of interest was the cerebellar cortex. Secondary regions of interest (ROIs) were the central cerebellar white
matter, cerebellar vermis, thalamus, and basal ganglia. Blood concentrations of harmane and another neurotoxin, lead, were also assessed.
Results: Mean SD cerebellar NAA/tCR was 1.52 0.41. The
mean SD log blood harmane concentration was 0.70 0.52
g-10/mL (range 0.13 1.72 g-10/mL). Cerebellar NAA/tCR was
strongly associated with age in years (r -0.70, p 0.01) and with
gender (p 0.01). In a linear regression model that adjusted for age
and gender, log blood harmane concentration was a predictor of cerebellar NAA/tCR (beta -0.41, p 0.009); every 1 g-10/mL unit
increase in log blood harmane concentration was associated with a 0.41
unit decrease in cerebellar NAA/tCR. The association between blood
harmane concentration and brain NAA/tCR only occurred in the cerebellar cortex; it was not observed in secondary brain regions of interest.
Furthermore, the association was specic to harmane and not another
neurotoxin, lead.
Conclusions: Higher blood harmane concentration was associated
with lower cerebellar NAA/tCR in ET. This association between blood
harmane concentration and brain NAA/tCR was particular to the primary ROI, namely, the cerebellar cortex; it was not present in the other
brain ROIs we examined. Furthermore, the association was specic to
harmane and was not observed as a feature of another neurotoxin, lead,
which we also studied. This study provides additional support for the
emerging link between harmane, a neurotoxin, and ET. Further studies
are warranted to address whether cerebellar harmane concentrations are
associated with cerebellar pathology in postmortem studies of the ET
brain.

S291

957
Does Parkinsonian tremor inuence patients quality of life
(QOL)?
T. Kondo, Y. Kajimoto, I. Nakanishi (Wakayama, Japan)
Objective: In this study, aiming to clarify the effects of tremor on
patients QOL, we correlated tremor severity with each domain of
Parkinsons disease Questionnaire 39 (PDQ-39).
Background: According to the guidelines for the therapy of Parkinsons disease (PD), decreasing the incidence of tremor among the
cardinal symptoms of PD is not the primary target of PD therapy.
Because, tremor disappears during motion, it does not disturb patients
daily lives. However, depending on the patients social situation,
tremor may become a factor that degrades their quality of life (QOL).
Methods: The tremor severity and QOL of 97 patients were assessed
using items 16, 21 and 22 of the Unied Parkinsons Disease Rating
Scale (UPDRS) and PDQ-39, respectively.
Results: No patients showed correlations between tremor severity
and the subdomains of PDQ-39, such as stigma. When comparing
patients whose duration of visiting our clinic was less than one year
with those whose duration of visiting our clinic was more than one
year, mobility and communication in PDQ-39 showed weak negative
correlations.
Conclusions: This study indicated that tremor has no effect on the
QOL of patients with PD. The negative correlations of tremor and
mobility/communication in the patients whose duration of visiting our
clinic was more than one year may suggest that the patients adapt to
tremor during the course of the disease.

958
Validity of family history in essential tremor
P.K. Manharlal, S. Fook-Choong, Y. Yuen, T.E. King (Singapore,
Singapore)
Objective: To investigate the sensitivity and specicity of family
history data given by essential tremor (ET) patients about relatives
affected by ET.
Background: Current evidence indicates that genetic factors are
important causes of ET. Family history information from ET probands
about affected relatives may not be accurate and its validity may be
compromised.
Methods: Consecutive ET patients were interviewed to obtain family
history information. Subsequently the relatives of these ET probands
were interviewed and examined with prior informed consent to ascertain presence of ET based on internationally accepted clinical criteria.
Probands reports of tremor in relatives served as the screening and the
relatives diagnosis of ET based on clinical examination served as
reference standard. The sensitivity and specicity of family history
information were calculated.
Results: Thirty-three patients and 32 relatives participated in this
study. Fifteen relatives (46.8%) were diagnosed as having probable or
denite ET. They came from 12 families. The median age of the
affected relatives was 42 years (range 17 to 64) and 53.3% were males.
Only 5 out of 15 relatives with ET were identied by probands. This
result makes the overall sensitivity of probands report 33.3%. Of the
remaining 17 (53.2%) relatives who did not have ET, the probands
reported only one having tremor, making the overall specicity of
probands report 94.1%.
Conclusions: Family history information reported by ET patients are
inaccurate and poorly validated. Clinical examination of all at-risk
relatives remains the most valid means of assessing the familial occurrence of ET.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S292 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

959
Clinical characteristics and prevalence of essential tremor in
orhangazi district of Bursa, Turkey (a population-based door to
door study) (Bursa, Turkey)
S. Erer, M. Zarifoglu, N. Karli, A. Semra, Y. Demet (Bursa, Turkey)
Objective: To determine the clinical ndings and prevalence of
essential tremor (ET) among 40 years and older population in
orhangazi district of Bursa, Turkey.
Background: The prevalence ET is higher than the other movement
disorders however prevalence rate may differ according to study population and methodology.
Methods: This population based study was planned in 3 phases. In
phase 1; door-to-door home interviews were performed by residents
using a short questionnaire consisting screening questions for tremor.
In phase 2; suspected participants in the rst phase were examined and
videotaped. In this phase cases were evaluated according to the scales
for tremor by movement disorders experts and a diagnosis has been
made. In the third phase video records of all diagnosed cases were
reviewed and nal diagnosis and the type of tremor were made by
agreement of all three experts.
Results: This study has been performed between May 2004 and
August 2006 in orhangazi district of Bursa, Turkey. 1256 of the target
population has been screened according to the study questionnaire. In
phase 1; 274 cases with complaining of trembling after the short
evaluation 247 subjects with tremor symptoms were detected. 56 out of
247 suspected cases could not be evaluated in phase 2. In phase 3; a
diagnosis of ET has been made in 21 cases, physiologic tremor in 26,
psychogenic tremor in 6, secondary to hyperthroidy tremor in 2 and
poststroke tremor in 1. We found that bilateral postural and kinetic
tremor of the hands has been signicantly presented.
Conclusions: We found that prevalence of ET is 2,25 % in our study.
Our data suggests that the prevalence rate of physiologic tremor is
higher than ET and other types of tremor.
960
Harmonic frequencies in tremor
P.H. Kraus, A. Hoffmann, G. Ellrichmann (Bochum, Germany)
Objective: To improve quantication and differential diagnosis of
tremor we evaluated accelerometry signals by time series analysis.
Background: For quantication and differential diagnosis of tremor,
electromyography (EMG) and accelerometry or mechanical devices
can be used as approaches supplementary to clinical assessment. Accelerometry essentially provides information regarding tremor amplitude and frequency. As there is a signicant overlap in the frequencies
of different tremor species, this parameter, however, is of limited value
for specic diagnosis. EMG provides information about agonistic and
antagonistic muscle activity, but the specicity of this procedure is
insufcient for individual diagnosis. For these reasons, new approaches
focus on more sophisticated analyses of tremor recordings. The results
of time series analysis reported here are particularly promising.
Methods: Accelerometry signals in parkinsonian tremor (PT, n32)
and essential tremor (ET, n15) were compared. Magneto-resistive
accelerometers were employed to measure resting tremor and postural
hand tremor for 32 second epochs while the patient was sitting in a
chair with armrest, and the results for the tremor-dominant side assessed by means of spectral analysis (Hamming window).
Results: PT exhibited a higher number of harmonics in fast Fourier
transformation (FFT) spectra than did ET. Spectral analysis improved
the selectivity of this parameter for tremor type (p 0.05). This
difference in number of harmonic peaks between PT and ET is due to
a striking deviation of acceleration signals from the sinusoidal in PT
that is not evident in the displacement time series. Multivariate analysis
(discriminant analysis) with frequency and harmonic number as factors
allowed an almost complete separation of ET and PT. These results are
in accordance with the hypothesis of a more complex central oscillator
in at least a subgroup of PT patients. Our ndings are consistent with
those of Deuschl et al. (1995), who, employing a different approach,

Movement Disorders, Vol. 22, Suppl. 16, 2007

also identied features of PT time series not consistent with a simple

sinusoidal pattern.
Conclusions: The number of harmonics proved to be a more selective parameter for tremor type than frequency or amplitude. Nevertheless, more and rarer tremor types should be included in further studies
to allow a practical contribution of complex parameter analysis to
problems of clinical differential diagnosis.
961
Essential tremor characteristics during different arm posture positions and mechanical load
N. Dragasevic, S. Radovanovic, J. Maric, A. Tomic, N. Kresojevic,
I. Petrovic, M. Svetel, V.S. Kostic (Belgrade, Serbia)
Objective: The aim was to investigate frequency and amplitude of
the essential tremor (ET) movements in different mechanical conditions (extended arms, with load on the forearm and with forearms
placed on the surface).
Background: It is proposed that ET is caused by a central oscillator
and includes the pathways encompassing the cerebellum, thalamus and
brainstem. Peripheral mechanisms have also been suggested. It has
been shown that frequency of ET is stable and independent of mechanical properties of the limb or body posture. However, some studies
claim that might be two subdivisions of ET, one of them characterized
by alterations induced by changes in mechanical properties of the limb
or posture.
Methods: Experiment was performed on 27 patients, and both tremor
dominant and non-dominant arm were recorded. Tremor was recorded
in 3 sessions. First session was with arms extended and pronated in the
horizontal plane. Second session was with the load of 500 g attached on
each extended and pronated arm in the region of hand ankle. Third
session was with arms relaxed and placed on the surface. Two-axial
accelerometer was used.
Results: Findings based on power spectrum analysis speak toward
subtle changes in amplitude and frequency of tremor movements in the
condition of supported relaxed arm. Load on the forearm showed that
neither amplitude nor frequency of the tremor movement did not
changed. However, patients could be divided into two groups on the
basis of tremor frequency and amplitude. Again, changes in the tremor
frequency and amplitude did not reach statistical signicance after
posture and/or load changes.
Conclusions: Heterogeneity between patients in age, age of onset and
duration of the disease might speak for various underlying mechanisms.
Changes in posture position and load did not change tremor characteristics. That further support claims that tremor is of central origin and it
is not inuenced by peripheral mechanical input. However, clear frequency and amplitude distinctions might speak for different physiological characteristics, even though posture and load dependence of the
tremor can not be conrmed.
962
Unique software algorithms for tremor analysis comparing the
novel approach and standard techniques
A.G. Shaikh, T.O. Crawford, R.M. Tripp, D.S. Zee (Baltimore,
Maryland, USA)
Objective: Here we present a novel tremor analysis technique.
Background: Accelerometry and spectral analysis are commonly
used to quantify tremors. Tremor is often intermittent or mixed with
non-tremor involuntary movements making its spectral analysis imprecise. Even when tremors are constantly present, spectral analysis neither directly calculates the variation in frequency nor allows an approximation of the percent time when tremor was present.
Methods: We report a novel software algorithm allowing 1) separation between tremor and non-tremor movements, 2) calculation of
percent time when tremor is present, and 3) quantitative characterization of segregated tremor signals to yield both the amplitude of the
tremor and an amplitude weighted mean frequency (WMF). A three
axis linear accelerometer was used to quantify tremors in 328 patients
[essential (n36); lithium induced (n51); Parkinsons disease

POSTER SESSION III, THURSDAY, JUNE 7, 2007

(n64); neuroleptic induced (n97); and ataxia-telangiectasia
(n80)]. Activity was recorded as patients performed postural, action,
and resting tasks. The WMF, and total tremor amplitude were computed using a composite vector reecting activity recorded around all
three axes. Furthermore a weighting procedure incorporating the amplitude was used to compute mean frequency such that stronger accelerations contribute to the frequency calculation proportionately more
then weaker ones. The amplitude was calculated cycle-by-cycle for
each axis independently and then combined vectorially. Further algorithms were used to calculate the cycle-to-cycle frequency difference
and thereby separate tremor from non-tremor movements.
Results: WMF was not signicantly different from the dominant
frequency of the waveform computed with the spectral analysis. Correlation between tremor amplitude computed with the composite analysis and peak energy of the power specturm was stronger only when the
percent tremor time was larger.
Conclusions: This nding suggests that reported software algorithms
offer more appropriate measure of tremor that is mixed with nontremor involuntary movements. This technique further yields the standard deviation of the tremor frequency a measure of tremor variability in a given patient.
963
Holmes tremor due to midbrain hematoma
F.M. Oztekin, N.S. Oztekin (Ankara, Turkey)
Objective: Holmes tremor is a rare clinical condition usually due to
the lesions of the midbrain. Holmes tremor has a characteristic rest,
intention, and postural component.
Background: Holmes is an irregular tremor and sometimes with
dystonic features. The lesions effect different parts of the central
nervous system mainly brainstem,cerebellum and thalamus. The syndrome arises as a consequence of a lesion in the upper brainstem and
cerebral peduncles, which, it is postulated, interrupts the cerebellorubrothalamic pathway.
Methods: We present a case of Holmes tremor secondary to a
traumatic midbrain hematoma.Holmes tremor have developed in a
middle age male patient three months after he had suffered a left
sided stroke due to a traumatic midbrain hematoma following a car
accident. An irregular left sided tremor was present at rest, persisted
during several postures and intentional tremor was also present.
Results: MRI revealed the site of the lesion starting from right
talamus to mesencephalon and extending to upper pons. His tremor did
not respond to any medication.The presented case of Holmes tremor
which is well documented supports the view that the tremor develops
following combined lesions of cerebellothalamic and nigrostriatal pathways.
Conclusions: To conclude modern neuroimaging techniques in this
case conrm that a combination of damage to the cerebello-rubrothalamic pathway and the nigrostriatal pathway is required for the full
Holmes tremor syndrome to occur.
964
Reduced purkinje cell number in essential tremor: A postmortem
study
E.D. Louis, J.E. Axelrad, L.S. Honig, I. Flores, G.W. Ross,
R. Pahwa, K.E. Lyons, P.L. Faust, J.P.G. Vonsattel (New York, New
York, USA)
Objective: To quantitatively assess Purkinje cell numbers in brains
of essential tremor (ET) patients and similarly-aged controls.
Background: Clinical and functional imaging evidence suggests that
cerebellar dysfunction occurs in ET. In recent postmortem studies, we
documented increased numbers of torpedoes (Purkinje cell axonal
swellings) in ET patients without Lewy bodies. Purkinje cell loss,
however, has never been rigorously assessed.
Methods: Postmortem cerebellar tissue was available on 14 ET cases
(six with Lewy bodies and eight without Lewy bodies) and 11 controls.
Calbindin immunohistochemistry was performed on parafn sections
of cerebellum. Images were digitally recorded and blinded measure-

S293

ments made of Purkinje cells per millimeter of Purkinje cell layer

(linear density).
Results: The 14 ET cases were similar to the 11 controls in terms of
age, gender, race, postmortem interval (PMI), and brain weight. Purkinje cell linear density was inversely correlated with age (r -0.53,
p 0.006) and number of torpedoes (r -0.42, p 0.04). Purkinje
cell linear density differed by diagnosis: controls (1.36 0.50 cells/
mm), ET cases with Lewy bodies (1.31 0.42 cells/mm), and ET cases
without Lewy bodies (0.84 0.32 cells/mm), p 0.04, with the most
signicant difference being between ET cases without Lewy bodies and
controls, where the reduction was 38.2% (p 0.04). In an adjusted
linear regression analysis that compared ET cases without Lewy bodies
vs. controls, decreased linear density (outcome variable) was associated
with ET (beta 0.22, p 0.03). We further stratied ET cases without
Lewy bodies into those with 10 or more torpedoes (N 3 cases) vs.
those with fewer than 10 torpedoes (N 5 cases). Purkinje cell linear
density was 0.56 0.16 cells/mm in ET cases with ten or more
torpedoes. This was 58.8% lower than that of controls.
Conclusions: We demonstrated a reduction in Purkinje cell number
in the brains of ET patients that do not have Lewy bodies. These data
further support the view that the cerebellum is anatomically, as well as
functionally, abnormal in these ET cases.
965
Tremor associated to chronic inammatory demyelinating peripheral neuropathy (CIDP): Treatment with Pregabalin
H. Alonso-Navarro, A. Fernandez-Daz, M. Martn-Prieto,
J.J. Ruiz-Ezquerro, T. Lopez-Alburquerque, F.J. Jimenez-Jimenez
(Salamanca, Spain)
Objective: To report a patient with tremor associated with CIDP
which improved after treatment with Pregabalin.
Background: The association of tremor to peripheral neuropathy is
infrequently reported. Busby & Donaghy (J Neurol 2003; 250: 714724) reported presence of tremor in 3 out of 45 patients with chronic
motor-sensory demyelinating neuropathy and in 1 out of 26 patients
with subacute motor-sensory demyelinating neuropathy. The treatment
of tremor associated with CIDP is usually unsuccessful.
Methods: A 68 year-old male was diagnosed with a CIDP at age 63,
developed a progressive postural and kinetic asymmetric tremor in both
hands at age 64. CIDP was treated with Prednisone associated with
other immunosuppressant drugs such as Azathioprine or Cyclophosphamide, and periodical administration of intravenous immunoglobulin.
Results: Drugs used attempting to improve tremor, such as Propranolol, Primidone, Phenobarbital, Clonazepam, Alprazolam, Gabapentin,
and Topiramate, were unsuccessful. In July, 2006, a treatment with
Pregabalin at increasing doses up to 150 mg twice daily was started.
With this treatment, tremor (evaluated with the Fahn-Tolosa-Marn
scale) improved markedly. Tremor improvement is maintained after 6
months of follow-up.
Conclusions: Tremor associated to peripheral neuropathies, and specically, to CIDP is infrequent, and the treatments are usually unsuccessful. Tremor of our patient improved markedly with Pregabalin,
while other drugs used in the treatment of postural tremors were not
useful. Recently, Zesiewicz et al. (Mov Disord 2007; 22: 139-141)
reported two patients with a marked improvement of essential tremor
with Pregabalin 200 mg/day. Pregabalin could be useful in the treatment of postural tremor resistant to other therapies.
966
Isolated tongue tremor after removal of cerebellar pilocytic astrocytoma: Functional analysis with Subtracted ictal SPECT coregistered to MRI study
S.J. Kim, W.Y. Lee, J.Y. Kim, B.J. Kim, D.W. Seo (Seoul, Republic of
Korea)
Objective: To report a unique case of isolated tongue tremor after
removal of cerebellar pilocytic astrocytoma, and to localize the activated structures corresponding to isolated tongue tremor.

Movement Disorders, Vol. 22, Suppl. 16, 2007

S294 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Background: Tongue tremor is a rare form of focal tremor. Even
though the dysfunction of dentato-rubro-olivary circular pathway has
been proposed as its mechanism, the origin of rhythmic activities
remains unknown.
Methods: A 26 year-old woman was hospitalized with complaints of
isolated tongue tremor 3 months after removal of cerebellar pilocytic
astrocytoma. We performed brain magnetic resonance imaging and
single photon emission computed tomography, and analyzed Subtracted ictal SPECT coregistered to MRI (SISCOM).
Results: We observed the destruction of cerebellar dentate nucleus
and bilateral asymmetric inferior olivary hypertrophy (Figure 1). The
SISCOM showed hyperperfusion in the right inferior olivary nucleus,
right lateral pons, bilateral red nuclei, left thalamus, right mesial
temporal and frontal areas (Figure 2).
Conclusions: We describe a patient with isolated tongue tremor after
removal of cerebellar pilocytic astrocytoma. By using SISCOM, we
found that isolated tongue tremor was closely related with abnormal
activation of olivary nucleus and red nucleus in Guillain-Mollaret
triangle.

Methods: We examined the effect of counting backwards from

100, a stroop test and voluntary foot tapping on classical rest tremor
of the hands in 13 patients with denite Parkinsons disease and a
visible Parkinsonian rest tremor. It was clinically assessed by UPDRS item 20 and measured by accelerometry (ACC) of the resting
hands. Three baseline assessments of tremor were followed by 6
assessements during 6 minutes under one of the provocation methods and this procedure was repeated 3 times for all three modalities
in a randomized order. Each provocation procedure was followed by
at least 3 min. rest.
Results: Each of the provocation procedures lead to a signicant
increase in tremor amplitude as measured by the ACC total power and
UPDRS tremor scores (p0.001). The ACC total power increased by
1-2 orders of magnitude, the tremor score by 1-2 points on average.
This effect did not show signicant differences between the different
provocation procedures. In the ON-state the baseline as well as the
provoked tremor was decreased signicantly, whereas the relative
effect of the provocation was similar to the OFF state.
Conclusions: As the provocation methods can mimic what patients
may face in stressful moments during everyday life when their tremor
becomes most disturbing, each clinical and electro-physiological assessment of rest tremor should include a provocation. Our data show
that the type of provocation does not seem to be important.

968
Clinical features of Parkinsonian tremor
F. Papengut, J. Raethjen, G. Deuschl (Kiel, Germany)

FIG. 1 (966).

967
Provoking Parkinsonian tremor
J. Raethjen, K. Austermann, F. Papengut, G. Deuschl (Kiel,
Germany)
Objective: Different ways to provoke Parkinsonian tremor in a
clinical setting are assessed.
Background: Classical Parkinsonian rest tremor can vary greatly
over time and can typically be provoked by mental or motor tasks of the
non-affected extremities.

Movement Disorders, Vol. 22, Suppl. 16, 2007

Objective: To evaluate if tremor suppression on movement initiation

is a characteristic feature of Parkinsons disease and whether this
feature can be utilised as a simple clinical diagnostic test.
Background: Parkinsonian Tremor (PT) is often misdiagnosed
due to its variability of the clinical expression. Only rest tremor is
by itself a positive criterion for Parkinsons Disease (PD) because it
is rarely found in other conditions. It has been proposed that the
tremor amplitude is suppressed when the hands are lifted from rest
position.
Methods: Videotapes were taken of patients suffering from PD
(n50) while the control group (n48) consisted of other tremor
syndromes like Essential Tremor, cerebellar tremor, psychogenic
tremor, Holmes tremor and neuropathic tremor. PD was diagnosed
according to the Brain Bank Criteria and/ or the results of a -CITSPECT. The following sequences were recorded: Patient sitting on a
chair, the forearm resting completely relaxed, counting backwards until
a stable tremor did occur (or not). The patients subsequently lifted their
hands (test 1). In a second task the patients moved towards a target with
the index nger (test 2). The videos were assessed by reviewers blinded
for the clinical diagnosis. They had to estimate reduction or increase of
the tremor amplitude when the patient prepared or started to move
using a newly developed tremor suppression scale. They were asked for
their clinical diagnosis (PT vs. not PT) based on the amount of tremor
reduction.
Results: Validating test 1 with 98 patients by 3 raters of different
neurological experience (1 movement disorder expert, 2 neurologists)
the raters found tremor suppression in 86 %, 84 %, 86 % of the PD
patients, respectively. The test showed a good sensitivity (0.81) and
specicity (0.81) discriminating PT from other tremor syndromes. The
interrater reliability was 0.73. A reevaluation of 92 patients (PD n47,
controls n47) with the same raters in a slightly modied test (test 2)
do not lead to signicantly different results.
Conclusions: Assessing tremor suppression on movement initiation
is a simple and reliable tool to separate PT from other tremor syndromes.

POSTER SESSION III, THURSDAY, JUNE 7, 2007

S295

969

971

Thalamic stimulation induced gustatory dysfunction in a patient

with essential tremor
J. Roggendorf, J. Vent, M. Maarouf, C. Haense, A. Thiel, G.R. Fink,
R. Hilker (Koeln, Germany)

Long duration accelometry to assess efcacy of oral 1-octanol in

patients with essential tremor
F.B. Nahab, S. Baines, D. Ippolito, M. Hallett (Bethesda, Maryland,
USA)

Objective: To investigate gustatory function in a patient with deep

brain stimulation (DBS) of the thalamic ventral intermediate nucleus
(VIM) for essential tremor (ET).
Background: Deep brain stimulation of the VIM nucleus is an
effective therapy for medically intractable essential tremor. Characteristic side effects include paresthesia, dysarthria and gait ataxia. This is
the rst report of DBS-induced hypogeusia in a patient with effective
VIM stimulation for ET.
Methods: Unilateral gustatory testing with taste strips in four concentrations per quality was performed with the DBS device switched on
and off. Furthermore, cerebral metabolic rates of glucose (rCMRGlc)
were measured with 18-Fluorodeoxyglucose-PET in stimulator off and
on conditions.
Results: Unilateral gustatory testing conrmed normogeusia with the
DBS device switched off whereas an isolated hypogeusia was presented under active VIM stimulation. Under VIM stimulation, 18-FDG
PET revealed a bilateral metabolic activation in the orbitofrontal cortex
and in the inferior temporal lobe, whereas a decreased rCMRGlc was
observed in the right insular cortex and the right basal ganglia.
Conclusions: We suggest that hypogeusia in our patient results from
a DBS-induced perturbation of the taste pathway connecting the brainstem with the parvicellular region of the ventral posteromedial nucleus
and gustatory projection sites. PET demonstrated a stimulation-induced
metabolic deactivation in the right insular cortex. Therefore, the change
in palatability might result from a modied hedonic representation of
the stimulus in the gustatory insular cortex due to DBS interference
with thalamocortical bres. This case demonstrates that gustatory dysfunction can be a side effect of thalamic VIM-DBS. Our ndings
provide direct evidence that the thalamus acts as an important relay
station in the gustatory pathway aswell.

Objective: To assess the utility of long-duration accelerometry in

quantifying the efcacy of oral 1-octanol for the treatment essential
tremor (ET).
Background: Numerous measures of tremor severity have been developed to quantify ET. All are however temporally biased since they
reect only a brief time interval. Recent work by Van Someren, et al.
(2006) showed the utility of long-term tremor recordings in PD patients
which correlated with UPDRS score. We hypothesized that portable
uni-axial accelerometers could be used to measure the efcacy of oral
1-octanol over hours to days in patients with ET.
Methods: SUBJECTS: This study was conducted using 5 patients
who participated in a pharmacokinetic study of oral 1-octanol. Subjects
wore an Actiwatch on the most affected wrist during an entire 9-day
admission. TREMOR ASSESSMENT: We used the Actiwatch to
record tremor and activity for the study duration using the algorithm
developed by Van Someren & Tremor Analysis software (v. 1.08).
DATA ANALYSIS: Actiwatch data was saved and exported to a
spreadsheet. We utilized the Tremor Score (T) and Activity Score
(A) variables from the Van Someren algorithm in our analysis. Since
ET primarily occurs during action & posture, periods of inactivity
could be construed as periods of improved tremor. To avoid this bias,
each data epoch (1min) required a non-zero value of tremor and activity
for it to be included in the analysis. We calculated this Tremor
Severity (S) as S T/(AT) To calculate overall S for a given time
period, we divide S for all time points by the number of time points.

970
Reconstruction of the petrosal bone for treatment of kinetic tremor
due to cerebellar herniation and torsion of cerebellar outow
pathways
T. Kinfe, O. Sedlaczek, W. Bergler, C. Blahak, M. Hennerici,
J.K. Krauss (Hannover, Germany)
Objective: We report causal treatment of kinetic tremor in a patient
with cerebellar herniation after petrosectomy taking into account preoperative functional imaging.
Background: The dentatothalamic pathways are known to play a
crucial role in the development of kinetic tremor. Kinetic tremor due to
lesions of the cerebellum and the cerebellothalamic pathways are a
therapeutic challenge. Usually there are no treatment options to repair
the primary structural decit.
Methods: Videographic and both structural and functional imaging
studies were performed in a 42-year-old man who was referred with
right-sided kinetic tremor. Six months earlier, a glomus jugulare tumor
has been removed via a right petrosectomy. At the time of the referral,
the patient suffered also from gait ataxia, dysarthria, nystagmus and a
peripheral facial palsy.
Results: MRI studies revealed a herniation of the right cerebellum
into the bony decit after the petrosectomy, accompanied by torsion of
the cerebellar outow pathways. Functional MRI demonstrated the
herniated cerebellar tissue to be functionally intact. Transfemoral angiography showed no compromise of blood ow in the superior cerebellar artery. The patient underwent a lateral suboccipital craniotomy
with reposition of the herniation and plastic reconstruction of the
petrosal bone. Apart from facial palsy, the decits improved within
days, and the tremor disappeared almost completely.
Conclusions: Functional imaging is useful in decision-making to
plan surgical repair after petrosectomy. In the rare case, kinetic tremor
can be cured by reconstruction of the posterior fossa.

FIG. 1 (971).

Movement Disorders, Vol. 22, Suppl. 16, 2007

S296 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

1-OCTANOL: Subjects received single-doses of 1-octanol at 6 am
based on the following schedule: Day 1-1mg/kg, Day 2-8mg/kg, Day
3-16mg/kg, Day 4-32mg/kg, Day 5-Washout, Day 6-64mg/kg (formulation A), Day 8-64mg/kg (formulation B), Day 9-discharge. Additional ratings & measures were also collected.
Results: Mean daily Tremor Severity scores (S.D.) from 6-9 am for
all subjects were inversely correlated with drug dose [Fig1]. Tremor
Severity scores on drug days were then compared with mean tremor
levels on non-drug days & also showed a dose-dependent improvement
from baseline [Fig2].
Conclusions: Our ndings have major implications for the standardization of inter- and intrasubject tremor ratings & may more closely
reect overall disability since it reects a more natural time course.
972
Palatal tremor and ataxia associated with sporadic adult-onset
Alexanders disease
N. Jodoin, C. Vandendries, D. Grabli, G. Bruneteau, D. Rodriguez
(Paris, France)
Objective: To report the case of a young woman with palatal tremor
and ataxia, leading to a diagnosis of Alexanders disease.
Background: Palatal tremor has been subdivided into essential and
symptomatic palatal tremor. The symptomatic form is characterized by
the presence of other brainstem symptoms, in addition to the palatal
tremor. Recently, the combination of progressive ataxia and palatal
tremor, as well as positive family history and specic radiological
abnormalities was associated to the presence at autopsy of Rosenthal
bers or to the detection of a mutation in the GFAP gene. Here, we
report a sporadic case of progressive ataxia and palatal tremor in a
young woman with adult-onset Alexanders disease.
Methods: Case report with video.
Results: A 22 year-old woman presented with a one year history of
tremulous voice, ear click, and oro-pharyngo-laryngeal myoclonus. She
reported unsteadiness since the age of 16. There was no family history
of neurological disease. Examination showed gaze-evoked nystagmus,
dysarthria, dysphagia, palatal tremor, and rythmic synchronous jerks of
tongue, larynx, and platysma. She also had a static and kinetic cerebellar syndrome, and a pyramidal syndrome with bilateral Babinski
sign. Brain MRI showed symmetrical, periventricular white matter
abnormalities with frontal preponderance with some discreet lesions in
the medulla and thalamus after contrast enhancement. Atrophy involving corpus callosum, cerebellar vermis, and medulla was obvious.
Molecular analysis revealed the R416W mutation of the GFAP gene,
conrming the diagnosis of Alexanders disease.
Conclusions: Palatal tremor can be the presenting symptom of adultonset Alexanders disease. Confronted with a palatal tremor, its asso-

Movement Disorders, Vol. 22, Suppl. 16, 2007

ciation with cerebellar and pyramidal syndromes, and marked brainstem and cervical cord atrophy on MRI are suggestive of Alexanders
disease, even in absence of family history.
973
Experience in therapy of essential tremor (ET) combined with
arterial hypertension (AH) by prolonged beta-adrenoblockators
(Dilatrend, Carvedilol): The pilot study
D.V. Pokhabov, V.G. Abramov (Krasnoyarsk, Russian Federation)
Objective: Investigate the AH spread among ET patients in our
region (Krasnoyarsk, Siberia, Russia), evaluate the possibility to treatment both diseases with prolonged beta-adrenoblockators.
Background: Many authors admit, that ET therapy by beta-adrenoblockator Propranolol has shown its advantage. But in clinical practice, ET and AH are often combined, and Propranolol therapy does not
fully compensate AH. Modern beta-blockators are able to compensate
AH safely and successfully. Arterial hypertension is most common
among people over 50 years old - 61.8% of population have it, according to the ofcial Russian statistics.
Methods: Weve researched the presence of AH among ET patients, who referred to our department during the year. When ET and
AH were combined, weve prescribed 25 mg of Dilatrend or carvedilol per day. The complete (almost complete) reduction of tremor
was considered a very good result, reduction by 75% - good result,
by 50% satisfactory result, by 25 % - poor result, by less then that
insignicant.
Results: During the year, we have diagnosed 172 patients with ET.
41 of them were younger then 50 years, 131 - older. AH was present for
16 patients (39%) in rst group, and for 103 patients (79%) in second
group. Wider spread of AH among ET patients, then in general population (61,8%) is noticeable. We can not reliably judge reasons for
connection of these conditions, but it can be estimated, that: 1. Affective disorders (anxiety, depression) of ET provoke the AH; 2. AH
damages the brain leading to vascular ET; 3. The changes (hypersensitivity) of adrenoreceprors play their own part in pathogenesis of
both diseases. Indications for use of beta-blockators for AH therapy
were present in 32 patients. For 27 patients prescription of carvedilol
allowed to compensate AH. For three patients (11.1%), there was no
effect upon tremor; For four patients (14.8%), effect was poor; For 14
patients (51.8%) satisfactory; For ve (18.5%) good; For one
patient (3.7%) very good.
Conclusions: The therapy of prolonged beta-adrenoblockators can be
an advantageous way to treat the combination of ET and AH diseases,
which requires additional large placebo-controlled research. Research
to investigate the reasons of common AH and ET combination is
needed.

Movement Disorders
Vol. 22, Suppl. 16, 2007, pp. S296-S307
2007 Movement Disorder Society

Movement Disorders, Volume 22, Supplement 16, 2007

Index of Key Words

123I -FP-CIT SPECT, 608, 842

123-I-FP-CIT, 480
18F-FDG PET, 385, 850
3 Tesla functional imaging, 461
3T magnetic resonance imaging, 483
4 leads, 219
4-aminopyridine, 49
5-ht3 receptor antagonist, 50
6-OHDA rotation test, 404
99mTc-ethyl cisteinate dimer, 368
-synuclein, 276
-synuclein pathology, 20

Animal model of Parkinsons disease, 73

Animal model of sleep disorders, 73
Antagoniste, 120
Anterocollis, 700, 728
Anti-beta2-glycoprotein I antibody, 849
Antibiotics, 724
Antibodies, 670
Antidepressant drugs, 355
Antidepressants, 340
Antiglutamate, 912
Antiparkinson therapy, 336
Antiparkinsonian therapy, 578
Antiphospholipid antibodies, 874
Antiphospholipid syndrome, 874
Anxiety, 332, 504
Apathy, 205, 229, 272, 522, 535, 608
APOE polymorphism, 810
Apomorphine, 315, 613, 622
Apoptosis, 63, 82, 563
Applause sign, 833, 834
Apraxia, 694
Apraxia of eyelid opening, 169
Aripiprazole, 90
Arm abduction, 154
Arm movements, 311
ARSACS, 48
Arterious venous malformation, 365
ARX gene, 444
Ashkenazi Jews, 438, 742
Assessment, 602, 681
Association, 247, 269, 435, 810
Associative plasticity, 142
Asterixis, 665
Astrocytes, 598
Asymmetry, 955
Asymptomatic mutation carriers, 559
Ataxia, 4, 34, 36, 41, 42, 45, 49, 51, 52, 334, 417, 463, 972
Ataxia with oculomotor apraxia, 4
Ataxin-2, 37
Athetosis, 107
ATP13A2 mutation, 866
Atrophy, 299
Attention, 661, 780
Atypical, 145
Atypical clinical presentation, 556
Atypical Movement Disorders, 681
Atypical onset movement disorders, 89
Atypical parkinsonism, 432, 857
Atypical phenotype, 393
Atypical PSP, 837, 867
Auditory startle reaction, 110
Automated gait detection algorithm, 798
Automatic auditory attention, 520
Autonomic, 827
Autonomic symptoms, 645
Autosomal dominant, 946
Autosomal recessive spastic ataxia of Charlevoix-Saguenay, 48
Autosomal recessive spastic paraplegia, 412

AADC, 405
Abuse, 533
Accelerometry, 41, 50, 961, 962
ACE I/D polymorphism, 269
Aceruloplasminemia, 450
Acquired hepatocerebral degeneration, 842
Action tremor, 938
Acute dystonia, 360
Add-on to DA-agonist, 797
Addiction, 786
Adenosine A2A receptors, 502
Adherence, 336
Adjunct therapy, 83
Adult Neurogenesis, 81
Adult-onset case, 387
Adult-onset primary torsion dystonia, 147
Adult-onset Alexander disease, 950
Adult-onset dystonia, 370
Advanced glycosylation endproducts, 652
Advanced Parkinsons disease, 274, 544
Age at onset, 886
Aggregation, 76, 79
Aggresomes, 55
Aging, 282
Akinetic mutism, 861
AKT signaling pathway, 61
Alcohol, 971
Aldehyde metabolism, 652
Alexanders disease, 349, 972
Alien hand, 674
Alien hand syndrome, 708
Alpha synuclein, 596
Alpha-foeto-protein, 4
Alpha-synuclein, 22, 62, 74, 76, 824, 830
Alpha-synucleinopathy, 824, 838
Alzheimer pathology, 549
Alzheimers disease, 612, 623, 678, 682, 822
Amantadine, 495, 649
Amyloid deposition, 457
Analgesic drugs, 536
Analysis, 962
Analytic framework, 313
Anatomy, 657
Anhedonia, 522
Animal model, 637, 930

S296

INDEX OF KEY WORDS

Awake bruxsim, 678
Awareness, 606
Axial, 156
Background contraction, 111, 123
Baclofen, 914
Balance, 546, 631, 784
Basal ganglia, 150, 177, 284, 468
Bcl-2, 614
Bedtime entacapone and levodopa, 514
Behavior, 515, 540
Behavioural symptoms, 511
Benign hereditary chorea, 92
Benign hyperbilirubinemia, 926
Best medical treatment, 278
Bifocal deep brain stimulation, 191, 218
Bilateral coordination, 254
Bilateral GPi stimulation, 219
Bilateral striatopallidodental calcinosis, 673
Biofeedback assisted relaxation training, 928
Biomarkers, 74, 596
Blepharospasm, 127, 139, 146, 158, 366, 384, 591, 687
Blepharospasmus, 386
Blinding, 228
Blink reex, 110, 118, 126, 144
Body mass index, 562, 769
Body weight, 577
Bone marrow stromal cells, 407
Botox (R), 56
Botulinic toxin, 402
Botulinum toxin, 8, 60, 84, 117, 122, 132, 133, 135, 146, 165, 263,
271, 363, 366, 367, 388, 389, 396, 399, 491, 532, 666, 690, 728,
848, 909, 911, 913, 917
Botulinum toxin A, 383, 883
Botulinum toxin type A, 56, 700, 907, 916
Botulinum toxin type B, 401
Bowel, 531
Boxing, 560
BRAAK regions, 440
Bradykinesia, 95
Brain iron content, 779
Brain perfusion, 454
Brain-derived neurotrophic factor, 103
Bromocriptine, 714
Bulging eyes, 40
Burden, 633
Burden of illness, 889
B-vitamins, 259
C17.2 neural stem cells, 808
Cabergoline, 611, 739
CAG repeat length, 696
CAG repeats, 89
CAMCOG, 541
Camptocormia, 391, 467, 736, 841, 848
Capecitabine, 361
Capsular infarction, 940
Captopril, 748
Carbamazepine, 702
Carbidopa, 618
Cardiac 123I-metaiodobenzylguanidine scintigraphy, 488
Cardiac dysautonomia, 506
Cardiac failure, 267
Cardiac MIBG-Scitigraphy, 787
Cardiac sympathetic nerve, 11
Cardiopulmonary capacity, 565
Cardiovascular, 891
Caregiver strain, 288
Caregivers, 633, 723, 730
Caregivers burden, 656

S297

Carers, 265
Case sampling in movement disorders, 691
Case-control study, 268, 650
Casein kinase, 294
Caspase, 63
Catecholamine, 68
Catechol-o-methyltransferase gene, 780
Caudate stroke, 919
Cav 1.3 alpha 1 Calcium - Channels, 604
Cavernoma, 196
CBD/ PSP Overlap, 857
Celiac disease, 143, 391
Cell death, 68
Cell model, 70
Cell replacement therapy, 406
Cell transplantation, 404
Central effect, 122
Cerebellar ataxia, 125
Cerebellar multiple system atrophy, 33
Cerebellar tremor, 50
Cerebello pontine angle tumor, 164
Cerebellum, 140, 150, 884, 941, 956, 964
Cerebral glucose metabolism, 763
Cerebral palsy, 915
Cerebral radiotherapy, 860
Cerebro vascular risk factors, 766
Cerebrospinal uid, 88, 865
Cerebrospinal uid markers, 256
Cerebrospinal uid total tau-protein levels to phosphorylated tau protein levels, 707
Ceruloplasmin ferroxidase activity, 640
Cervical dystonia, 367
Cervical, 390
Cervical cord, 154
Cervical dystonia, 110, 131, 153, 158, 164, 383, 388, 401
Characteristic, 373
Chemical shift imaging, 758
Chiari malformation, 884
Chocolate consumption, 605
Chorea, 85, 87, 91, 93, 96, 99, 105, 106
Choreoathetosis, 97, 98, 107
Chromosome 18p, 426
Chromosome 8q12, 412
Chronic low back pain, 363
Circadian Pattern, 888
Circadian Rhythms, 503, 518
CISI-PD, 258
Classication, 29, 944
Clinical assessment, 628
Clinical autonomic testing, 308
Clinical care, 632
Clinical characteristics, 341, 880
Clinical course, 851
Clinical evaluation, 409
Clinical ndings, 778, 959
Clinical impression of severity, 258
Clinical manifestation, 392, 673
Clinical marker, 706
Clinical models of depression, 513
Clinical practice, 281
Clinical rating scales, 234
Clinical scale, 694
Clinical tests, 566, 968
Clinical trial, 228, 695, 795
Clinico-electrophysiological correlation, 910
Clinico-pathological study, 504
Clonidine growth hormone testing, 308
Clozapine, 113, 498, 569, 760
Cluster analysis, 869

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S298 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Coefcient of friction, 685
Coenzyme Q10, 876
Coffee drinking, 139
Cognition, 323, 721, 770, 873
Cognition disorders, 200
Cognitive decline, 424
Cognitive dysfunction, 505, 832
Cognitive function, 509, 530, 594, 675, 768, 895
Cognitive impairment, 777
Cognitive prole, 851
Cognitive-emotional disturbances, 717
Coherence analysis, 395
Cohort studies, 645
Combined parkinsonism, 838
Communication, 251
Community, 626
Community based, 328, 793
Comorbidity, 529
Compliance, 331, 336, 713
Complications, 345, 914
Compound heterozygous mutation, 48
Compulsions, 2, 96
Computational model, 935
Computer program, 668
Computer simulation, 235
Computerized cognitive assessment, 317
(COMT) inhibitors, 796
COMT val158met polymorphism, 247, 949
Concomitant antidepressant therapy, 337
Constipation, 531
Continuos subcutaneous, 613
Continuous dopaminergic stimulation, 651, 738
Continuous levodopa duodenal infusion, 544
Control case study, 792
Conversion disorder, 466
Copper, 469, 704, 864
Copper deciency, 697
Cortical dopamine, 478
Cortical excitability, 46
Cortical morphometry, 299
Cortical motor areas, 482
Cortical myoclonus, 27
Corticobasal degeneration, 854, 863
C-reactive protein, 653
Creutzfeldt-Jakob disease, 382, 863
CYP1A2, 443
DAT scans, 868, 870
Data base, 772
Data integrity, 313
Data storage, 668DaTSCAN740
Daytime, 896
DBS, 17, 162, 193, 278, 344, 517, 610
DBS Targeting, 461
Deep brain stimulation, 14, 31, 107, 121, 130, 170, 171, 173, 174,
175, 179, 183, 185, 187, 189, 190, 192, 197, 201, 203, 207, 209,
211, 212, 213, 217, 220, 221, 223, 242, 279, 280, 293, 318, 359,
380, 385, 425, 534, 584, 658, 686, 688, 732, 752, 762, 920, 931,
941, 943, 954, 969
Deep brain stimulation of the subthalamic nucleus, 23
Deep brain surgery, 177
Deep hypothermia and circulatory arrest, 105
Deep-brain stimulation of the subthalamic nucleus, 194
Degradation, 80
Deletion, 426, 442
Dementia, 7, 22, 94, 292, 507, 523, 609, 620, 799
Dementia associated with Parkinsons disease, 520
Dementia with Lewy bodies, 464, 520, 763
Dependence on levodopa, 843

Movement Disorders, Vol. 22, Suppl. 16, 2007

Depression, 272, 295, 376, 522, 582, 592, 593, 594, 605,
636, 688, 727, 777
Depression and anxiety, 801
Depressive, cognitive, 740
Detailed clinical study, 427
Detrended uctuation analysis, 937
Developing countries, 185
Diagnosis, 711, 731, 878, 918
Diagnostic, 564, 759, 858
Diagnostic criteria, 862
Dialysis patients, 902
Diaphragmal myoclonus, 847
Diet, 823
Differential diagnosis, 299
Differential diagnosis of Creutzfeltd-Jacob disease, 707
Differential diagnosis of Parkinsons disease, 330
Diffusion tensor image, 462
Diffusion tensor imaging, 129, 448, 487
Diffusion tensor imaging (DTI), 450, 451
Diffusion tensor imaging with ber tracking, 447
Diffusion tensor MRI, 460
Diffusion weighted MRI, 382
Diffusion-weighted imaging, 28
Dilution, 366
Diplopia, 590
Direct costs, 825
Disease duration, 9
Disease progression, 552
Disease severity, 301, 488
DJ-1, 61, 415, 716
Dopa responsive dystonia, 141
Dopamin agonists, 739
Dopamine, 273, 285, 637
Dopamine agonist, 494, 595, 659, 753, 754, 785, 786, 806,
816, 878, 879
Dopamine agonist therapy, 767
Dopamine dysregulation, 275, 534, 720
Dopamine dysregulation syndrome, 261, 587, 719, 730
Dopamine responsive, 392
Dopamine toxicity, 75
Dopamine transporter, 75, 472, 842
Dopamine transporter imaging, 465, 581, 951
Dopamine unresponsive parkinsonism, 872
Dopaminergic agonists, 2, 719
Dopaminergic cell death, 69
Dopaminergic symptoms, 300
Dopaminergic therapy, 814
Dopaminergic treatment, 261
Dopa-responsive dystonia, 870
Dose, 900
Dose-escalation, 401
Double transgenic mice, 262
Driving, 566, 602, 721
Drug interaction, 879
Drug induced parkinsonism, 585
Drug interferences, 724
Drug-induced dystonia, 360
Drug-induced psychosis, 760
DTBZ, 326
Dual task, 530
Dull look, 403
Duodopa, 712
Dural arteriovenous stula, 832
Dysexecutive syndrome, 106, 526
Dyskinesia, 19, 35, 84, 145, 249, 306, 307, 310, 501, 600, 644, 794,
803, 806
Dyskinesias, 495, 773
Dysonia, 840
Dysphagia, 751

INDEX OF KEY WORDS

Dysphagia and deglutition, 109
Dysthymia, 508
Dystonia, 14, 91, 120, 125, 127, 128, 129, 133, 135, 137, 138, 140,
142, 150, 155, 156, 157, 159, 162, 184, 192, 198, 346, 351, 354,
361, 365, 369, 371, 372, 373, 374, 376, 378, 380, 381, 382, 386,
389, 390, 391, 392, 397, 398, 418, 426, 444, 453, 690, 708
Dystonia parkinsonism syndrome, 680
Dystonic tremor, 951
Dystono-dyskinetic syndrome in mitochondrial disease, 385
DYT 5, 375
DYT1, 13, 168, 418
DYT1 dystonia, 364, 370, 393
Early diagnosis, 472
Early diagnosis of Parkinsons disease, 573
Early onset chorea, 101
Early onset Parkinsons disease, 441, 508, 529, 543, 582
Early onset primary dystonia, 168
Early Parkinsons disease, 324
Early stage, 756
Early stage Parkinsons disease, 31, 599
Early symptoms, 281
Eating dystonia, 351
Echogenicity of substantia nigra and basal ganglia, 330
Echophenomena, 933
EDS, 788
Efciency, 240, 629
Electroconvulsive therapy, 236, 688, 699
Electrode, 178
Electroencephalogram (EEG), 709
Electroencephalography, 26
Electrogastrography, 516
Electromyography, 396, 558, 764
Electrophysiological methods, 109
Electrophysiology, 16
Embryonic stem cells, 406
Emergency room, 671
Emergency service, 646
EMG, 111, 952
EMG-fMRI analysis, 27
Emotion, 208, 273
Emotional behavior, 517
Emotional expressivity, 229
Employment, 607
Encephalitis, 99
Endocarditis, 674
Endophenotype, 147, 364, 930
Endoplasmic reticulum (ER), 37
Endothelin, 431
Energy expenditure, 318
Entacapone, 225, 304, 331, 644
Environmental exposure, 289, 818
Envrionment factors, 823
Ephedrone, 348
Epidemilogy, 745
Epidemiological study, 743, 744
Epidemiology, 437, 555, 650, 793, 882, 903
Epilepsia partialis continua, 666
Epilepsy, 932
Episodic ataxia, 49
Epsilon sarcoglycan, 143, 442
Epsilon sarcoglycan gene, 16
ER stress, 76
ERD/S, 114
Erythropoietin, 47, 635
Essential blepharospasm, 144
Essential tremor, 344, 493, 935, 937, 939, 941, 942, 943, 946, 949,
953, 956, 958, 959, 960, 961, 964, 969, 971
Essential tremor combined with arterial hypertension, 973

S299

Estrogen, 302
Ethnicity, race, 239, 248, 397
Etinal degeneration, 44
Etiology, 671
Evaluation, 199
Excessive daytime sleepiness, 791
Executive functions, 114, 543
Exercise, 132, 734
Expression, 62
External lumbar drainage, 839
Extracellular microrecordings, 116
Eye, 638
Eye of the tiger sign, 683
Facial emotion recognition, 172, 204, 205
Facial hemispasm, 146
Facial Movement Disorders, 396
Fahr disease, 673
Falls, 260, 297, 546, 661, 743, 873
Familial Parkinsons disease, 333
Family history, 958
Fatigue, 510, 601
FDG PET, 9, 42, 222, 523, 854
Fear of falling, 677
Feasibility, 475
Feature analysis, 764
Fibroblast growth factor 20, 435
Fibroblasts, 404
Finding PD resources, 322
Finger movements, 119
Finger tapping, 526
First degree relatives, 737
FLAIR, 455
Fluctuations, 280, 630
Fluctuations and/or dyskinesias, 266
Fluoxetine, 133
fMRI, 15, 148, 452, 453, 456, 466, 479
Focal and generalized dystonia, 368
Focal distonic hyperkinesis, 402
Focal dystonia, 132, 139, 152, 161, 166
Focal hand dystonia, 134, 148, 362, 394
Focal task-specic dystonia, 377
Focus group, 918
Follow-up study, 347, 353
Food, 290
Foot movement, 452
Foot scan system, 655
FP0011, 912
Fragile X syndrome, 921
Frataxin, 47
Freezing, 775
Freezing of gait, 173, 207, 255, 321, 615, 641, 765
Friedreich ataxia, 38, 47
Frontal assessment battery, 543
Frontal dysfunction, 775
Frontal subcortical pathway, 462
Frontotemporal dementia, 525, 535, 678, 703
Full-length expression, 746
Functional assessment, 915
Functional connectivity, 152, 807
Functional hand, 911
Functional imaging, 970
Functional magnetic resonance imaging, 306
Functional MRI, 482
Functional MRI - EMG, 27
Fundamental frequency variation, 253
FXTAS, 7, 34
G2019S LRRK2 PD, 559
Gabapentine, 445

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S300 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

GAG deletion, 418
Gait, 254, 509, 545, 548, 677, 873
Gait decits, 610
Gait disorders, 6, 358, 663
Gait disturbances, 556
Gait evaluation, 655
Gait freezing, 207, 765
Gait ignition failure, 676
Gait variability, 530, 675
Gastrointestinal, 59
Gauchers disease, 410, 430, 853
GBA, 413
Gender, 886
Gene expression, 66
Gene expression proling, 440
Gene mapping, 905
Gene networks, 74
Gene therapy, 405
Genetic, 250, 379, 811
Genetic counselling, 437
Genetic Parkinsons disease, 409
Genetic studies, 410
Genetic testing, 101
Genetics, 1, 5, 94, 333, 377, 414, 422, 424, 428, 431, 434, 906
Genotype, 443
Geste, 120
Gilbert syndrome, 926
Gilles de la Tourettes syndrome, 922, 924, 929
Glial cells, 662
Globus pallidus externus, 171
Globus pallidus interna, 931
Glucocerebrocidase, 430
Glucocerebrosidase, 70, 410, 420
Glucose metabolism, 457
Glucose-6-phosphate dehydrogenase, 647
Glutamate, 734
Glutathione, 561
GPe lesion, 803
GPi, 162
Greece, 429
Grip force, 685
GTP cyclohydrolase, 375
Guadeloupe, 846
Guillain-Mollaret triangle, 966
Gut, 59
Gynecomastia, 585
Gyroscope, 115
Hair analysis, 352
Hallervorden-Spatz, 408
Hallucinations, 246, 503, 538, 803
Hand tapping, 706
Handedness, 955
Handwriting, 634
Haplotype and disease association, 722
Harmonics in tremor spectra, 960
Hashimoto, 386
HDRS, 774
Head drop, 841
Health related quality of life instrument, 226
Heart rate variability, 506
Hematoma, 963
Hemiatrophy, 140
Hemichorea, hemiballism, 218
Hemifacial spasm, 389, 399, 527, 687, 690
Hemochromatosis, 419
Hemorrhage, 87
Hengli and Dysport, 701
Hereditary, 45

Movement Disorders, Vol. 22, Suppl. 16, 2007

Hereditary diffuse leukoencephalopathy with spheroids, 835

Hereditary spastic paraplegia (HSP), 5, 439
Hereditary spastic paraplegia with thin corpus callosum
(HSP-TCC), 451
High eld MRI, 484
Hipersialorrhea, 263
HIV, 872
H-MR-Spectroscopy, 758
Hoehn-Yahrn, 774
Holmes tremor, 196, 963
Home care, 855
Homeostatic mechanisms, 25
Homocysteine, 108, 259, 561, 580
Homocystinuria, 374
Hospitalization, 550
Human bone marrow, 598
Human leukocyte, 746
Human substantia nigra, 65
Huntingtons disease, 6, 29, 88, 89, 90, 100, 101, 102, 103, 171,
193, 518, 519, 693, 696, 706
Huntingtons disease-like 2, 85
Hyperglycemia, 87, 93
Hyperhomocystinemia, 339
Hyperkinesias, 99
Hypermanganesemia, 474
Hypermocystinemia, 609
Hyperphosphorylation, 294
Hypersexuality, 151, 275, 595, 819
Hypocretin, 875
Hypoestrogenemia, 890
Hypogeusia, 969
Hypogonadism, 36
Hypokinetic disorder, 199
Hypokinetic dysarthria, 586
Hyponatremia induced malignant syndrome, 725
Hypoperfusion, 485
Hypothalamic disturbance, 667
Hypotonia, 97
Hypotonic cerebral palsy, 141
Iba1, 78
IBZM-SPECT, 868
Idebenone, 38
Idiopathic extensor truncal dystonia, 363
Idiopathic Parkinsons disease, 321, 539, 580, 594
IFS psychotherapy, 947
Imaging, 398
Immediate- and extended release levodopa, 726
Immobilisation, 394
Immunohistochemistry, 237
Implicit learning, 621
Impulse control disorder, 309, 534
In vitro pharmacological prole, 502
Inappropriate ADH, 815
Incidence, 312, 329, 347
Incidental Lewy body disease, 298
Independent instrument, 226
India, 43, 44, 903
Indirect costs, 825
Infarct, 616
Infection, 667
Inammation, 77, 653
Information processing, 628
Information sharing, 251
Informational and practical needs of PD patients, 322
Informations call center, 646
Infratentorial, 908
Inhibition, 152
Initial symptom, 334

INDEX OF KEY WORDS

Interhemispheric inhibition, 684
Interleukin-10, 277, 808
Internal globus pallidus, 220
International cooperative ataxia rating scale, 52
Interstitial cells of Cajal, 710
Intestinal levodopa, 790
Intimate partner, 533
Intra cortical inhibition, 128
Intraduodenal levodopa infusion, 274
Intra-operative monitoring, 187
Intrathecal baclofen therapy, 915
Iodine-123 uoropropyl (FP)-CIT, 458
Ion channels, 935
Ipsilateral motor cortex, 119
Ipsilateral spasticity, 908
Iron, 3, 893
Iron chelator, 654
Isolated gait ignition failure, 868
Isolated tongue tremor, 966
Istradefylline, 502, 618
IVIG, 680
J-113397, 741
Jaw movement, 64
Jaw tremor, 381
Joint torque asymmetry, 599
Joubert, 923
Jules Froment, 617
Juncthophilin-3 gene, 85
KF RING, 704
Khat chewing, 352
Kinematic, 126
Kleine-Levin syndrome (KLS), 667
Klipell-Feil sydrome and pheochromocytoma, 700
Knowledge, 527
Korean patients, 473
Kufor Rakeb disease, 866
Lack of association, 949
Lactacystin, 264
Lariam, 350
Laser evoked potentials (LEP), 898
Laser light, 615
Late motor problems, 767
Late-onset Huntingtons disease, 94
Late-onset primary dystonia, 161, 166
L-dopa, 501
L-dopa induced dyskinesia, 744
L-dopa responsive dystonia, 705
Lethal catatonia, 699
Levetiracetam, 773, 800
Levitating hand, 674
Levodopa, 109, 170, 225, 255, 283, 290, 327, 490, 547, 577, 591,
600, 611, 618, 634, 644, 771, 795, 844, 856, 877
Levodopa induced dyskinesia (LID), 356
Levodopa infusion, 494
Levodopa pharmacodynamics, 726
Levodopa pharmacokinetics, 726
Levodopa plasma levels, 516
Levodopa/carbidopa/entacapone, 843
Levodopa-induced dyskinesia, 202, 800
Levodopatherapy, 641
Lewy bodies dementia, 682
Lewy body, 22, 294, 470
Lewy body dementia, 420
Lewy body pathology, 549
Life quality, 376
Limbic, 479
Lipid rafts, 54, 71

S301

Load, 961
Local eld potentials, 335, 762
Locomotor activity, 492
Locus of control, 607
Longitudinal study, 305, 329
Long-term, 901
Long-term efcacy, 781
Long-term follow-up, 213, 920
Low frequency drive, 395
Lower limb, 916
Lower urinary truct dysdunction, 622
LRRK2, 24, 54, 55, 71, 82, 237, 270, 333, 411, 414, 422, 423, 428,
603, 729, 742, 787, 811, 826, 845
LRRK2, 319
LRRK2 G2019S, 291, 438
LRRK2 gene, 436
LRRK2 mutations, 429, 433
Lymphocytes, 104, 563
Lymphoma, 833
Lysosome, 80
Machado-Joseph, 40
Macular atrophy, 51
Magnetic resonance, 480
Magnetic resonance imaging, 355
Magnetic resonance spectroscopy, 33
Major depression, 454, 508
Malignant melanoma, 769
Management, 747
Manganese, 348, 358
Manganic encephalopathy, 354
MAPT, 416
Mariana dementia, 525
Marinesco bodies, 733
Masked facies, 229
Mastication, 64
Maternal imprinting, 136
McGill pain questionnaire, 735
MCI, 303
MCP sign, 34
Mechanical vibrations, 548
Mechanism, 672
Medication review, 626
Medium spiny neurons/spine-retraction, 604
Meoquine, 350
MEG, 148
Meige syndrome, 138
Melanized nigral neurons, 65
Melanoma, 547
Melatonin receptors, 66
Membrane trafcking system, 54
Memory, 859
Memory disturbance, 835
Memory impairment, 317
Menopause, 890
Mental fatigue, 521
Mental retardation, 444, 913
Mental rotation, 364
Mentation, 545
Mercury, 669
Mesenchymal stem cell, 77
Meta-analysis, 304, 899
Metabolic syndrome, 90
Metabolomics and biomarkers, 603
Methcathinone, 840
Methylphenidate, 675, 765
MGUS anti-MAG polyneuropathy, 892
MIBG, 11
MIBG scintigraphy, 470

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S302 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Microarrays, 62
Microeletrode Recording, 658
Microglia, 808
Microglial activation, 78
Microlesion effect, 214
Microrecording, 177
Micturition, 622
Midbrain, 196, 484, 963
Midbrain hyperechogenicity and hyposmia, 30
Midbrain sonography, 664
Midodrine, 360, 497
Miraxion (ultra-pure ethyl-EPA), 693
Mirror movements, 672
Mitochondrial, 877
Mitochondrial DNA polymorphisms, 722
Mitochondrial membrane potential, 67
Mixed connective tissue disease, 705
MoCA, 620
Model, 157
Modied Ashworth Scale, 910
Modifying factors in drug therapy, 314
Molar tooth, 923
Molecular genetics, 161, 166, 168
Molecular pathogenesis, 325
Molecular screening, 624
Monocytes, 104
Mood in Parkinsons disease, 513
Morbidity, 836
Morphometry, 468
Mortality, 551
Motoneuron, 697
Motor and imagery task, 709
Motor control, 121
Motor cortex function, 372
Motor excitability, 112
Motor uctuation tractament, 274
Motor uctuations, 57, 231, 315, 495, 496, 516, 544
Motor function, 693, 748
Motor inhibitory effect, 496
Motor learning, 137, 519
Motor neuron disease, 106
Motor performance characteristics, 755
Motor signs, 691
Motor symptom progression over time, 201
Motor symptoms, 12, 588
Motor tics, 923
Motor training, 394
Mouse model, 830
Movement, 466
Movement analysis, 232
Movement disability, 717
Movement disorders, 198, 374, 445, 533, 671, 686, 692, 695
Movement disorders units, 681, 691
MPTP, 19, 57, 83, 310, 570, 741
MR Spectroscopy, 876
MRI, 131, 455, 463, 467, 663, 779, 849, 893
MRI ndings, 477
MSA, 471, 511, 875
Multidisciplinary, 597, 782
Multipe system atrophy, 497
Multiple sclerosis, 209, 384
Multiple system atrophy, 9, 10, 11, 39, 477, 483, 512, 698, 830,
831, 850, 856, 862
Muscle hypertrophy, 131
Muscles, 64
Musicians dystonia, 377
Mutation, 413, 423
Myasthenia gravis, 96
Myoclonus, 113, 445, 446, 449, 453, 666, 936, 938

Movement Disorders, Vol. 22, Suppl. 16, 2007

Myoclonus dystonia, 16, 92, 136, 143, 160, 379, 395, 442
Myoclonus dystonia syndrome, 220
N-Acetyltransferase 2, 818
NARP-MILS syndrome, 35
Natural course, 159
Near-inared spectrography, 467
Needs assessment, 632
Negative 14-3-3 protein, 707
Nervous diseases, 836
Neupro, 224, 338, 572, 828
Neural plasticity, 320
Neural stem cells, 81
Neurite maintenance, 826
Neurodegeneration, 576
Neurodegeneration with brain iron acumulation, 408
Neurolaments, 88
Neurogenetics, 437
Neurogenic bladder, 230
Neuroimaging, 129, 464
Neuroleptic malignant syndrome, 490, 699
Neuroleptic-induced parkinsonism, 357
Neuromuscular jitter, 117
Neuromyotony, 670
Neuron rescuing, 654
Neuronal activity, 31, 642, 820
Neuropathology, 369
Neurophysiology, 113, 124, 447, 858
Neurophysiology of speech, 174
Neuroprotection, 58, 67, 233, 614
Neuropsychiatric disturbances, 194
Neuropsychiatry, 792
Neuropsychological assessment, 541, 612
Neuropsychological evaluation, 854
Neuropsychological functions, 783
Neuropsychological test, 505
Neuropsychology, 222, 292, 538
Neurorestoration, 58
Neurosphere, 824
Neurospychological sequelae, 23
Neurotoxicology, 669
Neurotransplantation, 277
Neurturin, 407
New locus, 1
New mutation, 729
NICE guidelines, 782
Nigral iron, 235
Nociceptin, 741
Nocturnal disturbances, 636
Nocturnal sleep fragmentation, 514
Non motor symptoms, 510, 568, 590, 790
Non-human primate, 83
Non-linear analysis, 558
Non-motor, 606
Non-motor dysfunction, 751
Non-motor aspects of movement disorders, 18
Non-motor complications, 750
Non-motor problems, 312
Non-motor symptoms, 225, 288, 506, 524, 528, 559, 588,
593, 749, 809
Nonpharmacologic managements, 243
Non-pharmacological measures, 556
Non-target side, 954
Noradrenaline, 727
Nordic walking, 679
Normal pressure hydrocephalus, 839
Novel mutation, 660
Novelty seeking, 252
Nutrition, 562

INDEX OF KEY WORDS

Objective tremor/bradykinesia registration, 187
Obstructive sleep apnea syndrome, 698
Occupation, 291
OCD, 517
Octanol, 971
Oculopalatal tremor, 936
Odour identication test, 822
Olfaction, 298, 524, 581, 643, 812, 845
Olfactory dysfunction, 289
Olfactory function, 664
Olfactory hallucination, 236
Oligodendroglioma, 908
ON and OFF time, 300
Onuf, 230
Ophthalmolgical feauters, 638
Opsoclonus, 934
Optic neuritis, 384
Orally disintegrating tablet (ODT), 337
Orgasm, 819
Orofacial dystonia, 165, 831
Orolingual, 944
Oromandibular, 361
Oromandibulary dyskinesia, 352
Orthostatic hypotension, 509
Orthostatic tremor, 189, 948
Osmotic infusion pump, 635
Other Babinski sign, 687
Other clinical, 18
Outcome predictors, 130
Overdose, 296
Oxcarbazepine, 953
Oxidative stress, 100, 415, 561, 662
P300, 589
Pain, 489, 491, 536, 735, 821, 897
Pain thresholds, 542, 821
Painful hands moving ngers, 689
Painful legs moving toes, 689
Paired associative stimulation, 25
Pakinsonian ndings, 95
Palatal myoclonus, 847
Palatal tremor, 950, 972
Pallidal stimulation, 372
Pallidal T1 hyperintensity, 474
Pallidotomy, 184, 186, 853
Pallidum and STN, 191
PAM reex, 144
Pank2, 408, 683
Pantothenate Kinase Associated Neurodegeneration (PKAN), 683
Park 2, 287
Park 2 gene, 660, 746
Park 8, 237, 845
Park 9, 434
Parkin, 63, 421, 425, 427
Parkin gene, 436
Parkin null mice, 662
Parkin protein, 262, 282
Parkin regulation, 325
Parkinson, 59, 179, 195, 231, 239, 319, 411, 428, 433, 501, 563,
637, 638, 659, 749, 758, 767, 777, 786, 800, 822, 912
Parkinson clinic, 314
Parkinson plus syndrome, 855
Parkinson therapy, 651, 738
Parkinson, perception, 248
Parkinsons disease, 12, 17, 18, 20, 21, 23, 24, 26, 30, 32, 55, 57,
58, 69, 72, 75, 77, 82, 108, 112, 115, 116, 123, 126, 169, 170,
172, 173, 174, 175, 176, 178, 180, 181, 182, 183, 185, 186, 190,
194, 197, 200, 201, 202, 204, 206, 207, 208, 210, 212, 216, 217,
221, 223, 227, 230, 232, 233, 234, 235, 236, 238, 240, 241, 242,

S303

243, 244, 246, 247, 249, 251, 252, 255, 257, 258, 259, 260, 261,
263, 264, 265, 266, 267, 268, 269, 270, 271, 272, 273, 275, 276,
277, 278, 279, 280, 281, 284, 286, 289, 291, 292, 293, 295, 296,
297, 298, 301, 302, 305, 306, 307, 308, 309, 311, 312, 315, 316,
317, 318, 320, 323, 326, 327, 328, 329, 332, 337, 339, 340, 342,
343, 344, 345, 405, 407, 414, 416, 422, 423, 424, 425, 427, 429,
430, 434, 435, 436, 438, 440, 441, 443, 452, 454, 456, 458, 461,
465, 470, 473, 475, 478, 481, 487, 488, 489, 494, 496, 500, 503,
504, 505, 507, 514, 515, 521, 523, 524, 526, 528, 531, 532, 536,
538, 540, 541, 542, 546, 547, 548, 549, 550, 552, 553, 554, 555,
557, 558, 560, 562, 564, 565, 566, 567, 568, 569, 570, 571, 572,
574, 575, 576, 577, 578, 582, 583, 584, 585, 586, 587, 589, 590,
591, 592, 593, 595, 596, 597, 598, 600, 601, 602, 603, 605, 606,
608, 609, 611, 613, 615, 617, 620, 621, 623, 624, 625, 626, 628,
629, 630, 631, 632, 633, 634, 635, 636, 639, 640, 642, 643, 645,
646, 647, 648, 649, 650, 653, 655, 656, 657, 658, 660, 661, 679,
692, 710, 711, 712, 713, 714, 715, 716, 717, 719, 720, 721, 722,
723, 724, 725, 727, 728, 729, 730, 731, 732, 733, 735, 736, 737,
740, 743, 744, 745, 747, 748, 750, 752, 753, 754, 755, 756, 757,
759, 760, 761, 762, 764, 765, 766, 768, 769, 770, 771, 772, 773,
775, 776, 778, 779, 780, 781, 782, 783, 784, 785, 787, 788, 789,
790, 791, 792, 793, 795, 796, 797, 798, 799, 801, 802, 804, 805,
806, 807, 808, 809, 810, 811, 812, 813, 814, 816, 817, 818, 819,
820, 821, 823, 825, 827, 850, 859, 869, 881, 957, 960, 967
Parkinsons disease/AIMs/dyskinesias, 604
Parkinsons disease dementia, 457, 462, 763
Parkinsons disease management, 314
Parkinsons disease sleep scale, 571
Parkinsons disease with dementia, 256, 464, 682
Parkinsons disease without dementia, 612
Parkinsons disease, essential tremor, stroke, polymicrogyria, 672
Parkinsons disease, vascular parkinsonism, 245
Parkinsons plus syndromes, 432
Parkinsons psychosis, 498
Parkinsonian disorders, 460
Parkinsonian dyskinesias, 335
Parkinsonian mice models, 81
Parkinsonian speech, 253
Parkinsonian syndromes, 459, 627, 858
Parkinsonism, 12, 287, 348, 349, 350, 413, 419, 420, 421, 474, 480,
484, 486, 616, 665, 676, 711, 832, 835, 840, 853, 864, 866, 870,
871, 874, 877, 948
Parkinsonism and tremor, 669
Parkinsonism as late-onset side effect, 860
Parkinsonism dementia complex of Guam, 525
Paroxysmal, 145
Paroxysmal dystonia, 393
Paroxysmal kinesigenic dyskinesia, 86, 702
Paroxysmal nonkinesigenic dyskinesia, 155
Partial epilepsy, 927
Participation, clinical trials, 695
Pathoanatomy, 20
Pathologic hypersexuality, 309
Pathological gambling, 241, 720, 785, 816
Pathology, 24, 370, 964
Pathophysiology, 387
Patient educational needs, 802
Patients, 553
Pb toxicity, 492
PBMCs, 287
PD, 254, 303, 476, 829
PDQ-8 and PDQ-39, 226
Pediatric, 708
Pediatric Movement Disorders, 155
Pedunculopontine nucleus, 17
Percutaneous endoscopic gastrostomy, 39
Performance, 895
Perfusion MRI, 485
Perfusion SPECT imaging, 241

Movement Disorders, Vol. 22, Suppl. 16, 2007

S304 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Periodic leg movements, 357
Periodontal disease, 804
Perioperative course in Parkinsons disease, 212
Perioral, 84
Peripheral neuropathy, 108, 491, 902, 965
Peripheral territory, 855
Perseveration, 176
Persistent developmental stuttering, 684
Personality, 216
PET, 326, 489
PET cerebral glucose metabolism, 180
Petrosectomy; kinetic tremor, 970
PFS-16, 601
Pharmacokinetics, 283, 649
Pharmacological and surgical therapies, 942
Phasic and tonic contraction, 400
Phasic/tonic, 367
Phenotype, 51, 319
Phenotypic-genotypic correlations, 441
PHONIC TIC, 919
Phosphotau, 256
Physiologic tremor, 955
Physiopathology, 841, 942
Physiotherapy, 238, 328, 629, 679
Physiotherapy stretching, 446
PINK1, 421
PINK1 mutation, 574
Piraceta, 698
Piracetam, 938
Pituitary adenylate cyclase activating polypeptide, 570, 579
PKAN, 167, 378
Placebo, 794
Placebo effect, 899
Plasticity, 128, 158
Pleiotrophin, 72
POLG, 417
Poliomyelitis, 583
Polymerase, 417
Polyphasic MEP, 160
Polysomnography, 537, 791
Population based, 353
Population based case control study, 557
Population based study, 30
Portugal, 411
Positron emission tomography, 805
Posterior fossa tumors, 945
Post-marketing drug-surveillance, 500
Postpump, 105
Post-stroke spasticity, 909, 910
Posttraumatic parkinsonism, 560
Postural asymmetry, 731
Postural control, 227
Postural hypotension, 290
Postural impairment, 316
Postural instability, 631
Postural vertical, 784
Posture, 768
Posturography, 316
Potency, 56
PPN, 610
Pramipexole, 465, 739, 771, 813, 815, 885, 888, 891, 896, 900, 939
Pramipexole therapy, 750
Pre-clinical diagnosis, 232
Preclinical phase, 573
Prefrontal, 286
Pregabalin, 887, 965
Pregnancy, 302
Premotor cortex, 32
Premotor Parkinsons disease, 581

Movement Disorders, Vol. 22, Suppl. 16, 2007

Premotor stage, 537

Premutation, 7
Presymptomatic and symptomatic, 6
Prevalence, 270, 341, 433, 555, 836, 882, 894
Prevalence of non motor symptoms, 539
Prevalence rates, 692, 880, 959
Primary care, 889, 894
Primary dystonia, 130, 213, 468
Primary generalized dystonia, 219
Primary hemifacial spasm, 701
Primary immunodeciency, 680
Primary motor cortex, 25, 28
Primary progressive freezing gait, 676
Pro-apoptotic protein expression, 614
Procedural learning, 766
Progression, 745
Progressive multifocal leucoencephalopathy, 872
Progressive supranuclear palsy, 10, 477, 512, 535, 833, 834, 846,
847, 863, 865
Proinammatory cytokines, 589
Prolactinoma, 860
Prolonged beta-adrenoblockators (Dilatrend, Carvedilol), 973
Propriospinal, 449
Propriospinal myoclonus, 447, 448
Prosody, 253
Prospective evaluation, 211
Prospective study of depression in Parkinsons disease, 513
Proteasome inhibition, 654
Protein tau, 262, 282
Proteomics in human substantia nigra, 652
Protoon-magnetic resonance spectroscopy (1H-MRS), 820
Provocation, 967
Pseudobulbar palsy, 97
Psoas, 917
PSP, 511, 852, 876
Psychiatric disorders, 529
Psychoactive substances, 354
Psychogenic, 449
Psychogenic movement disorder, 138, 479
Psychogenic tremor, 947
Psychometric properties, 625
Psychomotor dysfunction, 512
Psychopathology, 869
Psychosis, 569
Psychostimulators induced parkinsonism, 843
Psychotherapy, 947
Psycosis, 223
PTEN, 61
Pulmunary function, 231
Pulse generator, 686
Punding, 754
Punding behaviour, 805
Pure akinesia with gait freezing, 852
Pure parkinsonian tremor, 257
Putamen, 365, 471
Putamen topography, 93
Putaminal hemorrhage, 665
Qualitative study, 802
Quality of care, 597, 648
Quality of life, 14, 192, 206, 234, 242, 265, 279, 288, 305, 321, 342,
383, 539, 592, 712, 732, 752, 772, 817, 904, 909, 922, 924, 943,
957
Quantied motor tests, 573, 575
Quantitative sensory testing, 542
Quantitative tremor analysis, 952
Questionnaire, 527
Quetiapine, 498
Quick Questionnaire, 718

INDEX OF KEY WORDS

Radiological predictive factors, 210
Ragged-red bers, 35
Randomised trial, 10
Randomized controlled trial, 701
Rapid onset dystonia parkinsonism, 191
Rasagiline, 267, 300, 340
Rasagiline monotherapy, 324
Rate of prevalence, 778
RBD, 118, 284
Reaching out, 737
Reach-to-grasp-movement, 195
Recombinant adenoviral vector, 72
Recurrent falls, 486
Recurrent inhibition, 122
Red ags, 862
Rehabilitation, 238, 297, 320
Relatives, 342
Relaxometry, 476
Reliability, 694
Religon, 239, 248, 397
REM sleep behavior disorders, 528, 664
Repetitive transcranial magnetic stimulation, 28, 119, 356, 554
Respiratory complications, 203
Rest tremor, 968
Resting-state, 807
Restless arms, 892
Restless legs, 882, 897
Restless legs syndrome, 2, 3, 878, 879, 880, 881, 883, 884, 885,
886, 887, 888, 889, 890, 891, 894, 895, 896, 898, 899, 900, 903,
904
Restless upper limbs, 892
Restoration of gait, 245
Retinopathy, 36
Retrocollis, 153, 725
Reversible, 871
Reversible posterior leukoencephalopathy syndrome, 497
Reward, 252
Rhabdomyolysis, 815
Richardsons syndrome, 837, 852, 867
Rigidity, 617
Risk factors, 268, 647
Risus sardonicus, 831
RLS, 1, 893, 901, 902
Ropinirole, 296, 713
Ropinirole 24-hour, 295, 301, 307
Rotenone, 579
Rotigotine, 224, 659, 828, 901
Rotigotine transdermal patch, 338, 572, 757
Rotigotine transdermal system, 639
rTMS, 371, 378
Rural, 829
Saccades, 311
Sadness, 749
Safety margin, 685
Sanamide, 323, 770, 797
Salsolinol, 710
Sarizotan, 794
SCA, 42, 45, 46
SCA 12, 43
SCA 7, 44
SCA2, SCA3, 432
Scale for the assessment and rating of ataxia, 52
Scintigraphic analysis, 776
SCOPA-motor scale, 625
Screening, 829
Secondary dystonia, 164
Secondary parkinsonism, 844
SEEG, 114

S305

Segawa, 375
Segawa disease, 387
Segmental, 156
Segmental dystonia, 211
Self-rating questionnaire, 924
Sensitivity and specicity, 483
Sensorimotor integration, 112, 134, 250
Sensory integration, 227
Sensory symptoms, 897
Sepiapterin reductase deciency, 141
Serum, 103
SF-36, 774
Sham surgery, 228
Sharp syndrome, 705
Shoulder pain, 809
Sialorrhea, 271, 532, 776
Side of onset, 551
Side-effects, 346
Silver syndrome, 5
Simulation, 934
Single ber EMG, 117
Single photon emission computed tomography, 368, 627
Single photon emission computer tomography, 564, 715, 759
SISCOM, 966
Site and voltage of subthalamic nucleus deep brain stimulation, 182
Sjogrens syndrome, 127
Sleep, 357, 510, 537, 621, 639, 788
Sleep apnea, 431
Sleep disorders, 859, 881
Sleep disorders in Parkinsons disease, 73
Sleep disturbances, 518, 553, 571, 578, 875, 904
Small bers, 898
Smallest allele, 43
Smell test, 303
Snapin, 13
SNCA duplication, 409
Social cognition, 172
Social impact, 723
Somatization, 588
Somatosensory cortex, 371
Sonographic area of substantia nigra, 640
Spasmodic dysphonia, 167
SPAST, 439
Spastic paraplegia, 905, 906
Spasticity, 8, 907, 911, 913, 914, 916, 917
SPECT, 458, 471, 472, 486
Speech and motor performance, 182
Speech dysfunction, 814
Speech intelligibility, 343
Speech pause ratio, 244
Speech rate, 244
Speech treatment, 343, 586
SPG10, 906
SPG36, 905
SPG4, 439
SPG5, 412
Spinal cord insults, 446
Spinal surgery, 345
Spine exion, 736
Spinocerebellar ataxia, 33, 40
Spiral analysis, 121
Sporadic cases, 624
Sporadic Parkinsons disease, 325
Spreading, 159
Stability, 60
Stair climbing, 677
Stalevo, 283, 331
Standard of care, 648
Standing tremor, 948

Movement Disorders, Vol. 22, Suppl. 16, 2007

S306 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Statins, 557
Statistical methods, 313
Stereology and image analysis, 65
Stereotactic neurosurgery, 116
Steroid responsive myelopathy, 448
Stimulation, 178
Stimulation parameters, 215
STN, 203
STN deep brain stimulation, 587
STN-DBS, 21, 180, 200, 204, 205, 210, 222, 657, 761
Storage, 60
Stretch reex, 123
Striatum, 507, 734
Striosomes, 53
Stroke, 550, 702, 936, 940
Subcellular distribution, 37
Subclinical FXTAS, 463
Subclinical hypothyroidism, 98
Subcortical dementia, 834
Subdural pneumocephalus, 861
Subdural-peritoneal shunt, 861
Sub-phenotype, 933
Sub-Saharan Africa, 568
Substantia nigra, 15, 66, 459, 476, 481
Sub-syndromal depression, 341
Subthalamic deep brain stimulation, 169, 176, 181, 202, 208,
246, 783
Subthalamic high frequency of stimulation (STN-HFS), 709
Subthalamic nucleus, 175, 181, 190, 195, 217, 221, 293, 584, 642
Subthalamic nucleus deep brain stimulation, 215, 216
Subthalamic nucleus local eld potentials, 215
Subthalamic nucleus stimulation, 186, 641
Subthalamic stimulation, 515, 540
Supplementary motor area (SMA), 356, 478
Supranuclear gaze palsy (SGP), 867
Surgery, 198, 199
Surgical therapy, 193, 206, 218
Surgical treatment, 945, 970
Surround inhibition, 86, 400
Survival, 696
Susceptibility-weighted imaging, 481
Swallowing, 240
Swallowing disurbances, 801
Swallowing dysfunction, 751
SWEDDs, 951
Switch from oral dopamine agonists, 338
Sydenhams chorea, 95, 104
Sympatehetic skin response, 789
Symptom of onset, 551
Synaptic terminals, 71
Synchronization, 335
Synphilin-1, 79, 733
Synuclein, 80
Synucleinopathy, 70, 118
Syringomyelia, 91
Systematic review, 102
T1 hyperintensity, 358
Tai chi, 260
Tardive akathisia, 359
Tardive dyskinesia, 53, 347, 351, 355, 359
Tardive dystonia, 353
Task specicity, 362
Task-specic, 165
Task-specic limb dystonia, 756
Taste, 643
Tau, 416, 826
Tau forms, 865
Tauopathy, 838, 846

Movement Disorders, Vol. 22, Suppl. 16, 2007

Taupathy, 857
Tau-protein, 576
Telephone counseling, 243
Temporal lobe epilepsy, 927
Tempo-rhythmic correction method, 245
Tendon Reex, 111
The subthalamic nucleus, 642
Therapeutic, 747
Therapeutic choice, 953
Therapeutic response, 753
Therapeutics, 102
Therapy, 493, 973
Theta burst stimulation, 554
Thorburn, 154
Thymectomy, 670
Thymidylate synthase, 580
Thyroid function tests, 98
Thyrosine hydroxylase deciency, 380
Tibialis anterior muscle, 883
Tic disorders, 928
Tics, 921, 922, 925, 929, 932, 933
Timing, 26
Titf-1, 92
TMS, 86, 142
TMS and imagery paradigm, 521
Tolcapone, 304, 327, 500, 796
Tolerability, 757
Tonic vibration reex, 8
Tooth loss, 804
Topiramate, 493
Torsin A, 13
Torticollis, 369
Tourette syndrome, 379, 918, 920, 921, 925, 926, 927, 930, 931, 932
Tourettism, 919
Toxicity, 469
Toxin, 956
Toxoplasmosis, 844
Tracheotomy, 39
Trajectory control, 519
Transcranial duplex scanning, 715
Transcranial magnetic stimulation, 32, 46, 125, 134, 160, 250, 400,
684, 929
Transcranial sonography, 3, 459, 473, 475, 574, 627
Transcranial ultrasound, 330
Transdermal delivery device patch, 738
Transdermal drug delivery device patch, 651
Transdermal patch, 224
Transdermal system, 828
Transgenic mouse, 856
Trauma, 390
Treadmill walking, 565
Treatment, 153, 399, 402, 490, 887
Tremor, 41, 157, 209, 214, 257, 332, 334, 381, 419, 482, 567, 934,
939, 940, 944, 945, 952, 954, 957, 962, 965, 967
Tremor suppression, 968
Trial, 38
Tripchlorolide, 233
Trunk deviation, 848
Tumorigenesis, 406
Twenty year prospective study, 799
TWSTRS, 388
Tyrosinase, 68
Tyrososine hydroxylase (TH), 716
Ubiquitin, 79, 703
Ubiquitin-proteasome pathway, 264
UCH-L1, 276
Ultrasonic vocalization, 285
Uncoupling protein, 67

INDEX OF KEY WORDS

Understanding PD treatment options, 322
Unied Huntingtons disease rating scale (UHDRS), 100
Unilateral, 946
UPDRS, 545, 575, 755
UPDRS scores, 324
Upper limb, 907
Urinary bladder, 827
Urinary dysfunction, 789
Vacuous chewing movements, 53
Vaginismus, 124
Vagus, 812
Validity, 718, 958
Valproate, 349
Valvular heart disease, 714
Vascular, 616
Vascular events, 339
Vascular parkinsonism, 485, 839, 849, 851
VCP, 703
Venezuela, 373
Ventricular tumor, 950
Ventrointermedial nucleus of the thalamus, 189
Veralipride, 346
Vertical supranuclear gaze palsy, 837
Vesicular dysfunction, 69
Vesicular monoamine transporter-2, 415
Video recording, 668
VIM-DBS, 214
Visual hallucinations, 197, 456
Vitamins, 492, 813
Voice/speech, 285
Voltage-gated potassium channels, 579
Voxel-based analysis (VBA), 450, 451
Voxel-based morphometry, 147, 567

S307

Vulvar vestibulitis syndrome, 124

Walking speed, 599
Wearable activity monitoring system, 798
Wearing off, 19, 310, 718, 817
Wearing off phenomenon, 630
Wearing off questionnaire, 266
Weight, 761
Weight change, 623
Weight gain, 21, 183
Weight load, 937
Weight loss, 249, 552
Whipples disease, 871
White matter, 29, 487
White matter disorder, 663
White matter tracts, 460
Wilsons disease, 184, 455, 469, 697, 704, 864
Wilsons disease facies, 403
Wilsons disease psychosis, 151
Wilsons disease scale, 151
Wilsons scale, 403
Wireless, 115
Work, 885
Working memory, 286
Writers cramp, 15, 135, 136, 137, 167, 362, 398
Yale global tic severity scale, 928
Yemenite Jews, 742
Young onset, 583
Young-onset Parkinsons disease, 607
Zarit burden interview, 656
Ziprasidone, 925
Zitter rat, 78
Zona incerta, 179

Movement Disorders, Vol. 22, Suppl. 16, 2007

Movement Disorders
Vol. 22, Suppl. 16, 2007, pp. S308-S324
2007 Movement Disorder Society

Movement Disorders, Volume 22, Supplement 16, 2007

Author Index

015 Study Group, 770

Aalto, S., 207
Aarsland, D., 424, 520, 528, 869
Aasly, J.O., 603
Abbruzzese, G., 8, 119, 139, 434, 565
Abdo, W.F., 875
Abe, K., 199
Abe, M., 28
Abele, M., 862
Abeliovich, A., 826
Abramov, V.G., 245, 402, 973
Abu-Isa, J., 380
Acar, B., 815
Achenbach, H., 304
Adamek, D., 733
Adams, B.E., 307
Adeli, G., 309
Adeva-Bartolome, T., 932
Adi, N., 62, 66, 429, 440
Adler, C.H., 689
Adwan, Z.M., 638
Agari, T., 178, 199
Agarwal, N., 704
Agarwal, P., 391, 583
Agarwal, V., 704
Aggarwal, A., 151, 403
Aggeliki, B., 667
Agid, Y., 10, 284, 427, 511, 924
Agnetti, V., 349, 363, 724
Agostino, R., 126
Aguilar, M., 559
Aharon-Peretz, J., 547
Ahlstrom, G., 217
Ahmad, N., 203
Ahmed, I., 296, 457
Ahn, T.-B., 823
Aiello, I., 363
Airaksinen, O., 558, 764
Ajebbar, K., 727
Ak, F., 101, 133
Akagi, N., 471
Akamanon, C., 631
Akan, O., 374
Akbostanci, C., 819
Akdemir, U.O., 805
Akgoz, S., 880
Akgun, Y., 198
Akhvlediani, K., 358
Akpinar, G., 247, 269, 660, 746, 810
Akyildiz, U., 777
Akyildiz, U.O., 616
Akyol, A., 616, 777
Al Faqih, S.S., 922
Albanese, A., 364, 862
Albani, F., 634
Albani, G., 516
Albert, T.Y., 361

Albrecht, S., 885, 896

Alburquerque, D., 656
Alexander, S., 605
Alexander, Z., 319
Ali, S.F., 325
Aljanati, R., 437, 556
Alladi, P.A., 65
Allcock, L.M., 661
Allen, C., 897
Allen, R.P., 889, 894, 897, 904
Allenby, K., 618
Almazan, J., 918
Alonso-Navarro, H., 932, 965
Alroy, I., 291
Altenmuller, E., 152, 377
Alterman, R., 197, 201, 213
Alterman, R.L., 130
Alvarez, M., 613
Alvarez, M.V., 689
Alvarez-Sabin, J., 480
Alves, G., 305, 312, 329
Alwarith, H., 352
Amboni, M., 775
Aminoff, M.J.405
Ammendola, S., 417
Amosova, N., 354, 512
Amosova, N.A., 840
An, J.-Y., 505
Anand, A., 287
Anand, R., 323, 770, 797
Anderson, G., 59
Anderson, K.E., 619
Anderson, S.W., 721
Anderson, T., 240
Anderson, T.J., 311
Andreas, J.-O., 879
Andres-Mateos, E., 325
Andrieux, J., 409
Anheim, M., 4
Anitha, M., 59
Antonini, A., 241, 336, 454, 544, 646
Anzellotti, F., 118, 948
Apartis, E., 16, 447, 448
Arabia, G., 650
Arai, A., 272
Arai, K., 39
Arbelo, J.M., 613
Arbus, C., 515
Ardouin, C., 515, 540
Argyriou, A.A., 429
Arica, B.S., 819
Ariga, H., 61, 716
Arkuszewski, M., 578
Armellino, K., 948
Arnaoutoglou, M., 386, 493
Arnaoutoglou, N., 386, 493
Arnulf, I., 284
Arpa, F.J., 263

S308

Arroyo, S., 258, 633

Arruda, W.O., 40
Aslan, D., 692
Asmus, F., 92, 848
Aso, T., 28
Assaf, Y., 663
Au, W.L., 226, 714
Auburger, G., 37
Auer, D.P., 837, 857, 867
Auff, E., 50, 542, 703
Auguet, M., 491, 501
Auquier, P., 515
Austermann, K., 967
Avanzino, L., 8, 119
Avdelidou, E., 386, 493
Avraham, E., 79
Awaad, Y.M., 914, 915
Axelrad, J.E.964
Ayalp, S., 672
Ayarza, A., 882
Aybek, S., 216, 246
Ayling, H., 298
Aytan, H., 890
Ayturk, Z., 819
Azman Ali, R., 690
Azulay, J.P., 587
Azzedine, H., 412
Baba, M., 272
Baba, Y., 552
Bachmann, C.G., 577, 887, 898
Bachoud-Levi, A.-C., 292
Badarny, S., 547
Badia, X., 718
Baez, S., 153
Baezner, H., 191, 192, 211
Baguley, I., 909
Bagyeva, G.K., 624
Bahunker, S., 765
Baier, C., 898
Baier, P.C., 406
Baik, J.S., 140, 145
Bain, P.G., 951
Baines, S., 971
Bajaj, N.B., 837, 857, 867
Bajica, T., 700
Bajwa, R., 800, 883
Bak, T.H., 511, 525, 535
Bal, J., 418, 444
Balas, I., 752, 954
Balash, Y., 663, 786
Balaz, M., 383, 803
Baldwin, C., 811
Baloyannis, S.I., 386
Baloyannis, S.J., 493
Balzamo, E., 73
Banach, T.A., 710
Banaszkiewicz, K., 546

AUTHOR INDEX
Bangert, M., 152
Bankiewicz, K., 405
Barbano, R.L., 359
Barbieri, S., 335
Barbosa, E., 434
Barbosa, E.R., 845
Bardien, S., 428
Bardinet, E., 171
Bares, M., 146, 383
Bares, R., 523
Bargallo, N., 456
Barhom, Y., 598
Barhum, Y., 742
Barichella, M., 646
Barillot, C., 299
Barker, G.J., 460
Barker, R.A., 518, 706, 780
Barnard, M.C., 494
Baron, M., 749
Barone, P., 82, 522, 593, 650, 775, 862
Barreto, J., 370
Barreto, L.B., 95
Barrett, E.T., 189, 839
Barriga, F.J., 749
Bar-Shira, A., 438
Bartova, P., 459
Baruzzi, A., 634
Bas, J., 563
Basak, A.N., 436
Basri, H., 690
Bassetti, C.L., 567
Bastan, B., 53
Bastiaanse, C.M., 316
Bateman, B.T., 550
Batocchi, A.P., 384
Battaglia, F., 519, 521
Batukaeva, L.A., 526
Bauer, P., 905
Baunsgaard, M., 822
Baumer, T., 250, 379
Baxter, L.R., 637
Bayes, A.R., 548
Baysal, A.I., 257
Bayulkem, K., 247, 269, 810, 949
Beal, F., 603
Beato, R.G., 95
Beaugendre, Y., 16, 447, 448
Beck, M., 158
Beck, S., 400
Becker, N., 85, 89, 582
Bedard, M., 328
Bednarik, J., 117
Behari, M., 43, 44, 469
Behrens, M.I., 866
Bek, S., 351
Belvisi, D., 126
Benabid, A.-L., 641
Benatru, I., 221, 784
Benavides, O., 656
Benbir, G., 144
Benecke, R., 14, 388
Benedetti, F., 709
Ben-Itzhak, R., 675
Benli, S., 942, 953
Benninger, D., 567
Ben-Shlomo, Y., 793
Bensimon, G., 10, 511
Benson, L., 514
Benti, R., 454

Bentivoglio, A.R., 193, 384

Berardelli, A., 8, 32, 126, 129, 139, 736
Bercovich, D., 438
Berendse, H.W., 524, 629, 807
Berg, D., 3, 232, 330, 476, 523, 758, 893
Bergamasco, B., 194
Bergler, W., 970
Bergmann, K.J., 555
Bergmann, S., 7
Bernardi, G., 5
Bernhard, D., 59
Berthet, A., 604
Bertolasi, L., 124
Bertoni, J.M., 324
Bertucci Filho, D.C., 582
Beskardes, F., 719, 730
Besnard, T., 168
Bespalova, E.V., 624
Beukers, R.J., 453
Bezard, E., 604
Bharkhada, A., 878
Bhatia, K., 392
Bhatia, K.P., 125, 142, 160, 370, 372, 381,
392, 468, 921, 944
Bhatia, M., 287
Bhatia, R., 44
Bhatt, M., 151, 403
Bhidayasiri, R., 184, 560
Bialecka, M., 818
Bican, A., 778
Bick-Sander, A., 673
Bie, R.M., 186
Biglan, K.M., 750, 753
Biguzzi, S., 162
Bilen, S., 101, 133
Bindu, P.S., 591
Biolsi, B., 219, 220, 385, 393
Bioulac, B., 73
Biran, M., 830
Biseul, I., 172, 204, 208, 273
Bishopric, N., 62, 429
Bisiacchi, P., 45
Blackett, H., 827
Blackstone, C., 680
Blahak, C., 191, 192, 211, 970
Blain, C.R.V., 460
Blair, C.R., 913
Blanchard, S., 21
Blanchet, P.J., 355
Blau, N., 141
Blazquez-Estrada, M., 267, 925
Blazquez-Menes, B., 925
Blehm, R.G., 297
Blesic, S., 937
Bloch, B., 604
Bloem, B.R., 316, 573, 575, 597, 629, 731,
767, 784, 875
Blyth, S., 537
Bocian, E., 426
Bockova, M., 114
Bocquillon, P., 727
Boczarska-Jedynak, M., 578
Boesch, S., 47, 596
Boesveldt, S., 524
Boetzel, K., 23
Boeve, B.B., 528
Bogdanov, M., 603
Bogdanova, I.V., 843, 844
Bogucki, A., 902

S309
Bohlhalter, S., 286, 694
Boisseau, P., 7
Bologna, M., 32, 126
Bonanni, L., 118, 682, 948
Bonetto, N., 871
Bonifati, V., 55, 411, 433, 434, 845, 870
Bonin, M., 905
Bora Tokcaer, A., 589, 805
Bordelon, Y.M., 557
Bordet, R., 727
Borgohain, R., 770, 783, 854
Borm, G.F., 316, 573, 575, 629, 767
Boronat-De Ferrater, M., 480
Boroojerdi, B., 224, 639, 659, 828
Borreca, A., 5
Borrega, L., 749
Borrell, E., 182, 215
Borroni, B., 865
Bosboom, J.L.W., 807
Bosch, A.D., 186
Bossi, L., 310, 912
Bostantjopoulou, S., 608
Bouhouche, A., 412
Boulanger, Y., 355
Boules, M., 637
Bounolleau, P., 447
Bour, L.J., 160, 177, 395, 453
Bourdain, F., 447
Bourguignon, A., 205
Bouslam, N., 412
Bove, M., 8, 119, 565
Bowen, J.D., 22
Bowers, D., 229
Boz, M., 778
Braak, H., 812
Brambilla, A., 920
Braun, M., 879
Brefel-Courbon, C., 489, 536
Brega, A.G., 34
Breslow, D., 288, 592
Bressman, S.B., 377
Briani, C., 871
Brice, A., 85, 168, 412, 425, 427, 436, 439
Bricka, B., 439
Bridges, S., 566, 602
Brocht, A., 750, 753
Brodsky, M.A., 494
Bronnick, K., 520
Bronstein, J.M., 557
Brooks, D.J., 457
Brotchie, J.M., 19, 58, 83, 310
Broussolle, E., 221, 427, 617
Brown, L.A., 227, 835, 886
Brown, P., 215
Brown, R., 510
Brown, R.G., 460, 511, 513
Browner, N.M., 847
Brozgol, M., 530, 677
Bruecke, T., 319
Bruggemann, N., 377, 574
Bruneteau, G., 972
Brunner, E., 577
Bruno, M., 479
Brunt, E., 42
Brussel, T., 476
Buck, D., 431
Buderath, P., 941
Budzianowska, A., 881
Bukowczan, S., 546

Movement Disorders, Vol. 22, Suppl. 16, 2007

S310 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Bulvik, S., 598
Burbaud, P., 761
Burguera, J.A., 613
Burgunder, J.-M., 450, 451
Burgut, T.F., 212
Burkhard, P., 246
Burkhard, P.R., 216, 628, 892
Burn, D., 513
Burn, D.J., 430, 661
Burne, J.A., 109, 123
Burrow, A., 705
Burton, N., 189
Butler, K., 134
Buzo, R., 437
Buzo, R.M., 556
Bye, L., 590
Byers, T., 334
Cable, J., 343
Cafferata, E., 72
Cagniard, B., 75
Cakmur, R., 131, 356, 399
Calabresi, P., 360, 458, 482, 865
Calatayud-Noguera, M.T., 925
Calender, A., 168
Calero, B., 625
Caliskan, A.C., 890
Caliskan, N., 692
Caliskan-Erle, E., 3
Calistri, V., 129
Calle, A., 825
Callizot, N., 58
Calloway, M.O., 889, 894
Calopa, M., 464, 559, 563, 613
Caltagirone, C., 610
Camacaro, M., 51, 373
Camargos, S.T., 408, 441, 508, 529, 543,
569, 630
Campdelacreu, J., 464
Campo, R., 274
Canesi, M., 241, 289, 336, 454, 544
Cannella, M., 103
Canovas, D., 532
Capano, A., 341, 551
Capelle, H.-H., 191, 192, 209, 211, 218,
861, 945
Capone, F., 17
Cappa, F., 45
Caputo, E.206, 335
Carballo, M., 414, 613, 647
Cardoso, F., 91, 95, 104, 408, 441, 508,
529, 543, 569, 630
Cardozo, A., 862
Caria, A., 223
Carlos, O., 919
Carlson, N.E., 57, 496
Carluer, L., 447, 947
Carod, F.J., 625
Carolyn, G., 148
Carr, J.A., 428
Carrabba, G., 116
Carri, M.T., 82
Carrillo, F., 414, 647
Carson, R., 478
Casarejos, M.J., 262, 282, 662
Castell-Conesa, J., 480
Castelli, L., 194
Castrioto, A., 360
Castro, A., 613

Movement Disorders, Vol. 22, Suppl. 16, 2007

Cattaldo, S., 516

Cavallo, M.A., 162
Cavdar, C., 880
Cavinato, M., 911
Caviness, J.N., 689
Cazeneuve, C., 85
Cemillan, C., 831
Cenci, A., 856
Ceppi, D., 180
Cesar, B.P., 630
Chabardes, S., 641
Chabrier, P.-E., 491, 501
Chacon, J., 455
Chacon, J.R., 613
Chaikin, P., 618
Champy, P., 846
Chana, P., 656, 866
Chandran, S., 164, 725
Chang, J.W., 207
Chang, V.C., 688, 699
Chang, Y.-L., 754
Chang, Y.-Y., 127, 849
Chapman, J., 653
Charles, P.D., 31, 913, 946
Charnet, C., 501
Charon, C., 412
Chartier-Harlin, M.-C., 409
Chase, B.A., 74
Chau, C., 197
Chau, W., 148
Chaudhuri, K.R., 639
Chelly, J., 444
Chen, C.C., 215
Chen, C.-M., 722
Chen, J.J., 340
Chen, L., 75
Chen, Q., 450, 451
Chen, R., 148
Chen, S., 233, 614
Chen, S.D., 407, 701
Chen, S.-D., 264, 277, 570, 579, 808
Chen, S.-Y., 293
Chen, X.-P., 450
Cheon, S.-M., 606, 859
Chereau, I., 515
Chertoff, M., 72
Chi, W., 75
Chiba, H., 332
Chien, H., 434
Childerhouse, A., 833
Chinnery, P.F., 92, 430
Chitnis, X.A., 460
Chladek, J., 114
Cho, H.Y., 859
Cho, J.-W., 483, 823
Cho, K., 676
Cho, M.R., 823
Choi, C.G., 850
Choi, J.Y., 671
Choi, S., 676
Choue, R.W., 823
Chouinard, S., 238, 266, 933
Chovancova, Z., 108, 477
Christe, B., 628
Christine, C.W., 405
Chroni, E., 429
Chu, C.Y., 67
Chua, E., 443
Chung, E.J., 35, 167, 473

Chung, E.-J., 36
Chung, S.J., 170, 243, 850
Churchyard, A., 877
Chwala, W., 546
Ciammola, A., 90, 103
Ciarmiello, A., 90
Cicale, D., 417
Cif, L., 168, 219, 220, 385, 393
Cilia, R., 241, 454
Cinquepalmi, A., 194, 709
Ciucci, M.R., 285
Clarimon, J., 294
Clark, G.R., 430
Clark, R.A., 325
Clarke, A., 693
Clarke, B., 782
Clarke, K., 56
Classen, J., 158, 627
Claustre, P.-S., 919
Claustres, M., 161, 166, 168
Clavera, L.E., 745
Clementi, M., 45
Clot, F., 16, 168
Cobaleda, S., 713
Coca, C., 455
Cocco, G.A., 349, 724
Cochen De Cock, V., 284
Cochen, V., 448
Coco, E., 363
Codita, I., 851
Coelho, M., 102, 411, 433, 862
Coenen, V.A., 657, 941
Cogiamanian, F., 116, 206, 335
Cohen, H., 2, 415, 766
Cohen, J.T., 801
Cohen, O.S., 653
Cohen, R., 172
Coll, D., 532
Collins, A.E., 99
Colloca, L., 709
Collod-Beroud, G., 161, 166, 168
Colosimo, C., 129, 539, 736, 862
Comella, C.L., 514
Comes, G., 366, 388
Comi, G., 180
Compta, Y., 256
Comte, F., 385
Constance, S., 637
Constantinescu, R., 88
Contarino, M.-F., 177
Contin, M., 634
Contreras, A.A., 923
Cook, M., 463
Cookson, M.R., 70, 294
Cooper, M.K., 946
Cooper, S.A., 227
Cordivari, C., 113, 120, 134
Cornet, S., 501
Corral, S., 669
Correia Guedes, L., 411, 433
Cosculluela, M.V., 548
Costa, M.M.B., 776
Costa, V., 386, 493
Cote, L., 121
Cottencin, O., 171, 727
Coubes, P., 219, 220, 385, 393
Cowey, M., 449
Cowling, J., 314
Cozzolino, A., 775

AUTHOR INDEX
Cozzolino, L., 650
Crawford, T.O., 41, 962
Criscuolo, C., 417
Crocker, S., 496
Crosio, C., 82
Crovato, D., 610
Crupi, D., 521
Cuadrado, E., 274
Cubo, E., 336, 918
Cucos, L., 185
Cucurella, G., 274
Cunin, P., 292
Cunningham, M.C., 441, 508, 529, 543,
569, 630
Cunninham, M., 408
Cuny, E., 761
Da Silva, R., 838
Dagli, S., 87
Dahan, V., 238
Dahl, H., 877
DallOra, E., 124
Dalla Libera, A., 434
Damier, P., 7, 16, 794
DAndreagiovanni, A., 948
Dang, M., 149
Daniel, G., 319
Daniels, C., 23, 862
Daniels, S.R., 702
Daras, M., 96
Dario, G., 522
Dat Vuong, K., 623
Date, I., 178, 199, 635
David, T., 653
Davidescu, I.E.-V., 342
Davidson, A., 279, 943
Davies, A.D.M., 503, 538
Davies, L., 909
Davin, C., 161
Davis, T.L., 31, 946
Dawson, J.D., 721
Dawson, T.M., 325
Dawson, V.L., 325
Day, B., 449
de Chazeron, I., 924
de Courten-Myers, G., 839
De Fabregues, O., 274, 532
De Gaspari, D., 241, 454
de Graaff, S., 909
de Jong, B.M., 466
De la Cerda, A., 94
De la Vega, V., 339, 609
De Lucrezia, D., 327
De Mari, M., 650
De Martino, M.E., 522
de Mattos, J.P., 776
de Medina, O., 437, 556
De Michele, G., 417
de Nijs, T., 715
de Pedro, J., 633
de Ruiter, M.B., 453
de Silva, R., 416
De Sousa, M., 309
De Souza, A., 591
de Souza, M., 595
De Weerdt, W., 694
Debie, R.M.A., 83, 105, 309, 620
Debilly, B., 179
Debu, B., 641

Dedic, V., 571

Deeb, J., 643
Defazio, G., 139, 364, 736
Defebvre, L., 409, 727
Defendi, S., 920
Defer, G.L., 947
Deijen, J.-B., 807
Dekomien, G., 424
Del Fante, M., 544
Del Sorbo, F., 862
Del Tredici, K., 812
Delaunoy, J.-P., 4
Delea, T.E., 331
Delgado, T., 274
dell Aquila, C., 259
Delliaux, M., 171
Delmaire, C., 171
Delphini, M., 587
Delval, A., 171
Demet, Y., 959
Demirturk, F., 890
Dengler, R., 209
Denis, E., 412, 439
Depienne, C., 412, 439
Derenne, J.P., 284
Derost, P.-P., 179, 924
Deshraj, A., 601
Destee, A., 409, 727
DeToledo-Morrell, L., 487
Deuschl, G., 14, 15, 23, 25, 176, 181, 195,
394, 621, 658, 862, 967, 968
Dev, A., 704
Devos, D., 171, 727
Devos, P., 727
Devoto, J., 189
Dhaenens, C.-M., 161, 166
Dhawan, V., 791
Di Fonzo, A., 411, 434
Di Iorio, W., 482
Di Lazzaro, V., 17
Di Luca, M., 865
Di Rocco, A., 519, 521
Di Santo, A., 3, 330, 758, 893
Di Virgilio, G., 292
Diamond, A., 279, 943
Diamond, A.L., 391, 583
Diaz, F., 414, 647
Diaz-Martin, J., 414, 647
Dickin, C., 227
Dickson, D.W., 835
Didone`, G., 124
Dieckmann, M., 930
Diederich, N.J., 506, 537, 860, 863
Diefenbach, K., 431
Dieguez, E.M., 437, 556
Diender, M.G., 731
Diez-Tejedor, E., 263
Dileone, M., 17
Dimitrios, K., 667
Dinapoli, L., 126
Ding, Y., 75
DiSanto, A., 476
Djaldetti, R., 486, 547, 742, 862
Djarmati, A., 1, 379, 442, 574
Djebli, N., 492
Doan, J.B., 227
Dobato, J.L., 749
Dobi, D., 553
Dobronevsky, E., 498, 765

S311
Dodel, R., 18
Dognitz, L., 352
Doi, Y., 471
Domingo, J., 831
Dominici, F., 122
Donmez Colakoglu, B., 399
DoPaMiP D. Study Group, 743 744
Dormas, L.O., 441
Dotchin, C.L., 568
Doudnikoff, E., 604
Downes, J.J., 538
Draganski, B., 29, 468
Dragasevic, N., 46, 159, 303, 376, 696,
937, 961
Drapier, D., 172, 204, 205, 208, 273
Drapier, S., 21, 172, 204, 205, 208, 210,
273, 712
Drillet, G., 21
Driver-Dunckley, E.D., 689
Droste, D.W., 863
Dschunja, D., 280
Duane, D.D., 367
Duarte, J.J., 745
Dubois, B., 18, 427, 511
Ducati, A., 709
Duchesne, S., 299
Ducreux, D., 447, 448
Duda, J., 549
Dufek, J., 108
Duff-Canning, S., 620
Duhem, S., 727
Dujardin, K., 171, 409, 515, 727
Dujardin, M., 747, 792
Duker, A.P., 189, 839
Dunnett, S.B., 806
Dupont, E., 862
Dupont, P., 447
Durif, F., 179, 515, 924
Durr, A., 16, 85, 168, 412, 425, 439, 905
Dutra, W.O., 104
Duysens, J.E.J., 316
Dyer, P., 314
Dzoljic, E.D., 268
Earl, N.L., 307
Earley, C.J., 897
The Early Symptoms Study Group, 281
Eberling, J.L., 405
Ebersbach, G., 796
Eblan, M.J., 410
Ebner, F., 452
Echebarria, S.G., 681, 691
Edison, P., 457
Eduard, T., 456
Edwards, M., 392
Ege, A.A., 101
Ege, F., 445
Eggert, K.M., 500, 876
Egidi, M., 116, 206
Ehret, R., 772
Ehrt, U., 869
Eibach, S., 673
Eidelberg, D., 519
Eisa, M., 800, 883
Eizenstein, O., 663
El Assawy, N., 516
Elbaz, A., 284
Eleopra, R., 162
The ELEP Group, 258, 633

Movement Disorders, Vol. 22, Suppl. 16, 2007

S312 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Elfertit, H., 385
Elibol, B., 53
Ellenbogen, A., 726
Elleuch, N., 412
Elliott, R.A., 322
Ellmen, J., 283
Ellmen, J., 644
Ellrichmann, G., 960
Elshoff, J.-P., 879
Emmanuelle, A., 284
Emptaz, A., 409
Enders, L., 377
Engelender, S., 79
Enzinger, C., 452
Epifanio, A., 650, 760
Erdogmus Ince, N., 805
Erer, S., 692, 778, 880, 959
Erginoz, E., 261
Ergul, E., 247, 269, 660, 746, 810, 949
Erickson-Davis, C., 607
Eriksson, B., 685
Ertan, S., 719, 730
Eryigit, N., 819
Escobar Khondiker, M., 846
Espay, A.J., 189, 839
Espinosa, J., 274
Esselink, R.A., 186
Etcheverry, J.L., 882
Evans, A.H., 534, 720
Evers, C.M., 322
Evidente, V.G., 689
Evoli, A., 384
Eyal, A., 79
Ezquerra, M., 256, 559
Fabbrini, G., 126, 129, 434, 736
Fabiani, F., 458
Fabiano, L., 113
Fadrna, T., 459
Faelli, E., 565
Fahn, S., 18, 847
Fainardi, E., 162
Falcon, C., 456
Fan, W., 514, 795
Fang, J.Y., 946
Fanjul, S., 831
Farley, B.G., 320
Farrell, K., 442
Farrer, L., 811
Farrer, M.J., 742
Fasano, A., 193
Faust, P.L., 964
Favre, C., 491
Fazekas, F., 452
Fazio, F., 180
Fedirko, E., 439
Fedorova, N.V., 354, 840
Fedoryshyn, L., 348
Fehlau, M., 621
Feigin, A., 519
Feld, Y., 404
Fenelon, G., 292
Fernandez, M., 455
Fernandez-Daz, A., 965
Fernagut, P.-O., 830
Fernandez, H., 229, 242, 732
Fernandez, H.H., 239, 248, 313, 397, 566,
602, 637, 648, 695, 751, 754, 829
Fernando, M., 643

Movement Disorders, Vol. 22, Suppl. 16, 2007

Ferraye, M., 641

Ferreira, J., 102, 434, 912
Ferreira, J.J., 411, 433, 845
Ferri, L., 231
Ffrench-Mullen, J., 62, 440
Fiaschi, A., 112, 364
Fietze, I., 431
Filla, A., 417
Finberg, J.P.M., 404
Fincati, E., 173, 434
Findik, A., 374
Findley, L.J., 643
Fink, G.R., 222, 969
Fiorio, M., 364
Fischer, S., 25
Fisher, B., 599
Fishman, P.S., 619
Fitzer-Attas, C., 340
Fitzer-Attas, C.J., 300
Fleury, M.C., 4
Flores, I., 964
Flores, J.M., 446
Floris, R., 5
Floyd, A.G., 121
Flutie, J.H., 367
Foffani, G., 335, 762
Fogel, W., 191
Foncke, E.M., 453
Foncke, E.M.J., 136, 160, 177, 395
Fook-Choong, S., 958
Foote, K., 242, 517, 732
Foote, K.D., 313, 648
Forbes, A., 757
Ford, B., 121, 688
Forjaz, M.J., 258, 625
Forlani, S., 412, 439, 905
Forman, M.S., 549
Forni, C., 73
Forsaa, E.B., 305
Foubert, A., 697, 761
Fournier, S., 172, 204, 208, 273
Fox, C., 586
Fox, C.M., 285, 320
Fox, S.H., 19, 83, 310, 620
Frackowiak, R.S.J., 29, 468
Fraddosio, A., 259
Frades, B., 633
Fraix, V., 425, 641
Franca, D., 91
Francavilla, G., 8
Franconi, J.-M., 830
Frank, B., 941
Frank, J.W., 951
Franques, J., 4
Franzin, A., 180
Frasson, E., 124
Frati, L., 103
Frauscher, B., 664
Frederic, M.Y., 161, 166, 168
Friedland, R., 811
Friedman, A., 235, 348, 418, 426
Friedman, J., 680
Fromm, C., 941
Frost, E., 182
Frucht, S., 96, 683
Fuchs, G.A., 280
Fujimoto, K., 332
Fujishima, K., 950
Fukae, J., 950

Fukuda-Tani, M., 824

Fukumoto, M., 471
Fukuyama, H., 28
Fulda, S., 357, 895, 899
Funayama, M., 421
Fung, V.S.C., 143
Funkiewiez, A., 515
Furukawa, K., 68
Gabriel, H., 375
Gabriella, S., 522
Gaenslen, A., 3, 330, 523
Gaig, C., 559
Gajdulewicz, M., 444
Galazka-Friedman, J., 235
Gale, A., 833
Galeano, G., 625
Galistu, P., 724
Gallagher, D.A., 275, 534, 720, 833
Galland, F., 924
Gallardo, M., 51, 373
Gallea, C., 479
Gallo, B.V., 187, 203, 341, 551
Gambarin, M., 364
Gamez, J., 532
Gandhi, S., 370
Ganesharajah, T., 370
Gao, L., 414, 647
Garbin, E., 124
Garca de Yebenes, J., 262, 282, 662
Garca Olmos, L.M., 745
Garcia Ruiz, P.J., 613
Garcia, S., 825
Garcia-Borreguero, D., 901
Gardoni, F., 865
Garg, J., 469
Garibotto, V., 180
Garlapati, R.R., 910
Garlepp, M., 270
Garraux, G., 478
Gartner, M., 189
Garvan, C., 242, 648, 732
Garvan, C.W., 313, 829
Garvin, C., 566, 602
Gasbarra, A., 610
Gascon, J., 464
Gasparoli, E., 871, 911
Gasser, T., 3, 92, 330, 523, 893
Gatto, E.M., 882
Gaudiello, F., 5
Gavarini, S., 219, 220, 385, 393
Gee, M., 779
Gehre, S., 81
Gencler, S., 815
Gentleman, S.M., 20, 504, 507
Gerasimou, G., 608
Gerhard, A., 392
Gerloff, C., 152, 250
German Parkinson Study Group,
Neurostimulation Section, 23
Gerrits, M.C.F., 136
Gershanik, O.S., 72, 825
Gerstner, A., 378
Gervais-Bernard, H., 221
Geser, F., 862
Geurts, A.C., 731
Ghilardi, F., 519, 521
Ghorayeb, I., 73
Giannetti, A.V., 91

Giannetti, J.G., 408

Gielen, F.I.H., 657
Giese, M.A., 232
Gieteling, E.W., 466
Gil Robles, S., 219, 220, 393
Gil, K.M., 710
Giladi, N., 224, 254, 291, 422, 438, 509,
530, 533, 547, 659, 663, 675, 677, 737,
786, 801, 862, 873
Gilead, L., 547
Giles, S., 632, 802
Gilio, F., 32
Gill, C.E., 31, 913
Gill, E., 878
Gill, S., 234, 265
Gillioz, A.-S., 21
Giorgi, L., 295, 301
Giraudo, S., 434
Girlanda, P., 139, 142
Girshovich, I., 786
Gissot, A.S., 784
Giuffrida, J., 115
Giunti, P., 38, 125
Gjerstad, M.D., 528
Glaser, C.A., 99
Glik, A., 674
Glisky, M.L., 321
Gluzman, Z., 404
Godau, J., 3, 330, 476, 523, 758, 893
Godge, Y.R., 164
Goetz, C.G., 18, 593, 617, 794, 795
Gok, S., 739
Glockler, T., 952
Goker-Alpan, O., 70, 410, 420
Golab-Janowska, M., 881
Goldenberg, H., 47
Goldman, J.G., 487
Goldwurm, S., 434
Gollob, K.J., 104
Gomez, A., 262, 282
Gomez, C.M., 33
Gomez-Arevalo, G., 825
Gomide, L., 569
Gong, Q.-Y., 450, 451
Gontu, V.K., 837, 857, 867
Goodman, A.O.G., 518, 706
Goodman, R.R., 121
Goodman, W.K., 517
Gopinathan, S., 495
Gorani, F., 452
Gordon, J., 599
Gosein, V., 239, 248, 397
Goyal, V., 469
Grabli, D., 972
Graeber, M.B., 20, 504, 507
Grafe, S., 60, 366, 388
Granata, A., 13
Grandas, F.F., 923
Granieri, E., 162
Grasso, L., 650
Gray, J., 38
Graziottin, A., 124
Greene, J.G., 59
Greene, P., 688
Gregori, B., 126
Gregory, A., 683
Grein, S., 60
Grid, D., 412
Grifth, A.F., 321, 413

AUTHOR INDEX

S313

Grigsby, J., 34
Grolleau, S., 536
Gronchi-Perrin, A., 216, 246
Grossbach, M., 152
Grosset, D., 336, 626
Grosset, K., 336, 626
Grozdev, I.S., 717
Gruber-Baldini, A.L., 619
Grundlinger, L., 509, 677
Grunewald, A., 143, 379, 442
Gryz-Kurek, E.A., 733
Gschliesser, V., 664
Guadagni, F.327
Guedes, L.C., 845
Gunduz, A., 110, 719, 730
Gunzler, S.A., 57, 496
Gurevich, T., 438, 509, 786, 862
Guthrie, B.L., 190
Guthrie, S., 190
Gutti, U., 410
Guyon-Marechal, I., 16
Guzman de Villoria, J.J., 923

Hasegawa, T., 68, 69, 76

Hasegawa, Y., 368
Hashimoto, K., 78
Haskin, J., 79
Hassin-Baer, S., 547, 653, 742
Hastings, T.G., 75
Hatano, T., 54, 71
Hattori, N., 54, 71, 421, 435, 479, 636, 950
Hattori, T., 622
Haubenberger, D., 542, 703
Hauert, C.-A., 628
Hausdorff, J.M., 254, 509, 530, 663, 675,
677, 873
Hauw, J.J., 863
Havrankova, P., 371, 398
Hawkes, C.H., 643, 812
Hayashi, T., 199
Hayick, S.J., 683
Hayran, M., 53
Hazan, J., 412
Hedera, P., 946
Hefter, H., 388
Heiblum, Z., 737
Heinemann, U., 863
Hellwig, D., 378
Hely, M.M.A., 799
Henchcliffe, C., 212, 550
Hengerer, B., 604
Hening, C., 897
Hening, W.A., 897
Hennerici, M., 970
Hensman, D.J., 951
Hering, S., 47
Herlofson, K., 305, 312, 329
Herman, T., 530, 677, 873
Hermann, A., 81
Hermann, W., 396, 917
Hernandez Vara, J., 613
Hernandez, B., 718
Hernandez, J., 559
Hernandez-Vara, J., 480
Herrojo Ruiz, M., 152
Hery, M., 712
Herzig, R., 459
Herzog, H., 42
Herzog, J., 176, 181, 195, 658
Hess, E.J., 150
Heyny-von Haussen, R., 652
Hicking, C., 794
Hiengkaew, V., 631
Higuera-Calleja, J., 640
Hilker, R., 23, 37, 222, 666, 969
Hillel, A., 291
Hindle, J., 513
Hinson, V.K., 555, 795
Hinterleitner, T., 790
Hirata, K., 78, 236, 636
Hirayama, T., 950
Hirsch, E.C., 846
Hjermind, L., 168
Hlustik, P., 477, 576
Ho, S.L., 67
Ho, W.L., 67
Hobson, P.J., 541
Hodges, J.R., 511, 525, 535
Hoegen-Esch, I., 312, 329
Hoegl, B., 901
Hoeglinger, G.U., 876
Hoek, H.W., 347

Haan, R.J., 186

Haarer, M., 222
Haaxma, C.A., 573, 575, 767
Haberkorn, U., 868
Hadjigeorgiou, G., 773
Haegele-Link, S., 705
Haegelen, C., 204, 208
Haense, C., 969
Hagenah, J., 377, 379
Hagenah, J.M., 574
Hagerman, P., 334
Hagerman, P.J., 34
Hagerman, R., 34
Hagiwara, M., 331
Hak, S., 863
Hainzer, M., 856
Halamek, J., 114
Hall, D., 34
Hall, D.A., 334, 419
Haller, U., 928
Hallett, M., 15, 122, 128, 132, 137, 362,
394, 400, 420, 478, 479, 642, 971
Halliday, G.M., 270, 729, 799
Halpern, A., 343, 586
Hama, K., 467
Hamdan, M., 774
Hameed, W.S.S., 98
Hampshire, A., 780
Han, S.W., 140, 145
Hanagasi, H., 135, 907, 916
Hanajima, R., 387
Hanaoka, A., 804
Hand, A., 251
Hanein, S., 412
Hanninen, J., 283
Happe, S., 898
Haq, I.U., 242, 517, 732
Harder, C., 898
Hardesty, D., 688, 699
Hardy, J., 408, 441
Hariz, M., 182, 215, 372
Harms, L., 352
Harrison, C.H., 31
Hartman, A., 924
Hartman, R., 323
Hasegawa, K., 24, 237, 368, 435, 781

Movement Disorders, Vol. 22, Suppl. 16, 2007

S314 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Hoffmann, A., 960
Hoffmann, G.F., 375
Hoffmann, I., 952
Hoffmann, K.-T., 673
Hoffmann, M., 703
Hogl, B., 664
Hoglinger, G.U., 846
Holler, I., 394
Hollerhage, M., 846
Holloway, R., 18
Holton, J.L., 230, 298, 370, 484, 838, 852
Honarmand, S., 99
Honczarenko, K., 881
Hong, S., 936
Hong, Z., 233
Honig, L.S., 964
Hoogduin, J.M., 27
Horiuchi, E., 368
Horne, M.K., 270
Horstink, M.W.I.M., 434, 573, 575, 767
Horstmann, R., 879
Hort, J., 707
Horvath, J., 892
Hoshiyama, E., 236
Hosseini, N., 685
Hou, J.-G.G., 545, 615
Houben, J., 593
Houeto, J.L., 515
Howard, E., 314
Howard, K., 334, 419
Hristova, D.R., 717
Hruska, K., 410
Hsu, A., 726, 795
Huang, X., 306
Huang, Y., 270, 729
Huang, Y.-C., 874
Huber, F.M., 863
Huber-Mahlin, V., 873
Huberman, M., 547
Huckabee, M.-L., 240
Huerta, C.339, 609
Hueto, J.-L., 425
Hughes, A., 909
Hughes, A.J., 864
Huizenga, H.M., 200
Hulihan, M.M., 742
Hummel, F., 152
Hundsberger, T., 705
Hunt, T., 56
Hunter, E.J., 684
Huntington French Speaking Group, 171
Hurt, C.S., 513
Hurtig, H., 549
Hutchinson, M., 147
Hutchinson, S., 147
Hutchinson, W., 877
Hutter, D., 33
Iaccarino, C., 82
Ibanez, R., 446
Ichimura, M., 502
Ichinose, H., 237
Idrisoglu, H.A., 247, 269, 660, 746, 810
Iguchi-Ariga, S.M.M., 716
Iguchi-ArigaSanae, S.M.M., 61
Iijima, M., 636
Ikeda, K., 813
Ilg, W., 232
Iliceto, G., 259, 434

Movement Disorders, Vol. 22, Suppl. 16, 2007

Illarioshkin, S.N., 624

Iltis, I., 33
Imam, S.Z., 325
Imamichi, Y., 421
Imamura, T., 470
Inbar-Borovsky, N., 530, 677
Inoue, H., 552
Inoue, K., 826
the INSERM National Dystonia Network
and GIS Maladies Rares, 166
Insola, A., 17
International Parkinsons Disease Non
Motor Group, 510
Inzelberg, R., 547, 674, 811
Ippolito, D., 971
Isacson, O., 741
Isaias, I.U., 130, 213, 241, 289, 454, 544
Ishii, F., 169, 202
Ishikawa, C., 39
Ishikawa, M., 584
Ishikawa, S., 716
Isik, U., 198
Ismailova, T., 354
Isonishi, K., 820
Itin, I., 514
Ito, H., 728
Ito, K., 39
Ito, T., 622
Itoh, H., 584
Itoyama, Y., 68, 69, 76
Ittrich, C., 604
Ivanov, C., 185
Ivanov, M., 185
Ivanova-Smolenskaya, I.A., 624
Ivanovic, N., 159, 376
Ives, N.J., 234, 265
Iwasaki, Y., 636, 813
Jabbari, B., 800, 883
Jabeen, A., 783
Jabeen, S.A., 854
Jabusch, H.-C., 152, 377
Jackson, M., 651, 738
Jackson, M.J., 499
Jacob, R., 846
Jacobson IV, C.E., 239, 248, 397, 695
Jacobson, C., 242, 517, 648, 732
Jacobson, C.E., 313, 754, 829
Jacquemont, S., 7
Jagid, J., 187, 203
Jagid, J.A., 551
Jagid, J.R., 341
Jaglin, J.A., 514
Jagust, W.J., 405
Jahanshahi, M., 372
Jalenques, I., 924
Janelidze, M., 358
Jankovic, J., 18, 279, 338, 359, 419, 639,
654, 687, 809, 834, 931, 943
Janssen, L., 316
Janssen, S., 637
Jaques, A., 216
Jardim, L., 434
Jarosz, J.M., 460
Jasinska-Myga, B., 578, 818
Jaume, R., 919
Jech, R., 183, 371, 398
Jenkinson, C., 234, 265
Jenner, P., 499, 651, 738

Jennings, D., 472

Jennings, D.L., 465, 581
Jennum, P., 858
Jeon, B.S., 483, 798, 823, 842
Jeon, S.H., 473
Jeong, D.-S., 955
Jeong, H.M., 606
Jeong, S.-Y., 97, 156
Jimenez, G., 339, 609
Jimenez, J.E., 228
Jimenez-Jimenez, F.J., 932, 965
Jinnah, H.A., 150, 157, 362, 369
Jodoin, N., 972
Jog, M., 328
Johansen, K.K., 603
Johansson, M., 668
John, B.A., 111
Johnels, B., 685
Johnson, J.M., 367
Johnson, M., 328
Johnston, L., 632
Johnston, T.H., 19, 58, 310
Jones, D.K., 460
Jones, H.N., 751
Joo, J.-H., 36
Jorgensen, H.U., 318
Jovic, J., 159
Ju, S.S., 361
Juergen, K., 593
Jun, S.M., 606
Juncadella, M., 464
Jung, G.Y., 462
Junghanns, S., 952
Jungnitsch, M., 81
Junque, C., 456, 464
Jurak, P., 114
Juren, S., 700
Juri, C., 656
Justiss, M.D., 602
Kabakci, K., 379
Kadota, T., 635
Kadowaki, T., 78, 236
Kadziolka, B., 418
Kaelin-Lang, A., 380, 461
Kafantari, A., 608
Kafri, M., 663
Kageyama, Y., 785
Kahle, P.J., 325, 830
Kailajarvi, M., 283
Kajimoto, Y., 817, 957
Kakita, A., 11
Kalaitzakis, M.E., 20, 504, 507
Kalbe, E., 23, 222
Kalhorn, C.G., 344
Kalliolia, E., 386, 493
Kamada, S., 48
Kamei, S., 636
Kaminski, A., 605
Kamolsawat, S., 631
Kanazawa, I., 781
Kanazawa, T., 11
Kanda, T., 502
Kandadai, R.M., 854
Kane, J.R., 285
Kaneko, S., 584, 820
Kang, G.A., 702
Kang, H.U., 462
Kang, J.-S., 666

AUTHOR INDEX
Kang, S.Y., 86, 137, 814
Kang, U.J., 75
Kanjanasut, N., 184
Kankaanpaa, M., 558, 764
Kannari, K., 272
Kano, O., 813
Kanovsky, P., 108, 383, 459, 477, 576, 756
Kao, C.C., 31
Kao, Y.-F., 849
Kaoroptham, S., 184
Kapisyzi, M., 553
Kaplan, Y., 884, 890
Kaplitt, M.G., 212
Kapsali, S.A., 386, 493
Kapucu, O., 805
Kara, I., 247, 269, 810
Karakas, E., 131
Karamanidis, C., 386
Karjalainen, P.A., 558, 764
Karle, K., 905, 906
Karli, N., 692, 778, 880, 959
Karpov, O., 598
Karsidag, S., 672
Kasadi, A., 697
Kasap, M., 660, 746
Kashihara, K., 470, 804
Kashiwaba, T., 820
Kassubeck, J., 905, 906
Katrak, P., 909
Katsarou, Z., 608
Katschnig, P., 452, 790, 816
Katzen, H.L., 341, 551
Kawada, S., 470
Kawarai, T., 5
Kawashima, N., 368
Kaya, D., 198
Keffel, J., 901
Keitz, M., 252
Kekelia, K., 192, 211
Kellenyi, L., 752, 954
Kellison, I.L., 229
Kenangil, G., 261
Kenney, C., 279, 359, 931, 943
Kenny, R.A., 661
Kessels, A.G.H., 564, 715
Keus, S.H.J., 629
Khachatryan, S.G., 926, 927
Khan, S.I., 111
Khatiashvili, I., 358
Khiat, A., 355
Khor, S., 726
Khullar, M., 287
Ki, C.S., 35, 167
Kieburtz, K., 88, 750, 753
Kiechl, S., 30, 475
Kiefer, C., 461
Kiening, K., 107
Kiening, K.L., 941
Kihira, T., 467
Kikuchi, A., 68, 76
Kim, B.J., 966
Kim, B.-J., 655
Kim, C.Y., 61
Kim, H., 814
Kim, H.-S., 580
Kim, H.-T., 93, 488, 497
Kim, J.E., 612
Kim, J.H., 140, 145, 612
Kim, J.K., 665

Kim, J.-M., 842

Kim, J.S., 850
Kim, J.-S., 86, 488, 497, 505, 955
Kim, J.W., 606, 859
Kim, J.Y., 35, 140, 145, 167, 473, 966
Kim, J.-Y., 483, 798
Kim, K.-K., 580
Kim, K.S., 665
Kim, M., 676
Kim, M.J., 170, 665
Kim, M.S., 243
Kim, O.-J., 580
Kim, S.E., 842
Kim, S.H., 485
Kim, S.J., 966
Kim, S.-J., 36
Kim, S.R., 170, 243
Kim, S.T., 473
Kim, W.-C., 580
Kim, W.-J., 678
Kim, Y.D., 612
Kim, Y.J., 763
Kim, Y.-J., 77
Kim, Y.K., 842
Kimura, K., 387
Kinfe, T., 218, 970
Kinfe, T.M., 945
King, M., 518
King, T.E., 98, 527, 958
Kingsbury, A., 298, 845
Kirby, D., 877
Kirchhoff, P., 496
Kishore, A., 495
Kistner, A., 540
Kitaura, H., 61
Kiziltan, G., 719, 730
Kiziltan, M.E., 110, 144
Klebe, S., 412
Kleensang, A., 1
Klein, C., 1, 143, 250, 377, 379, 442, 498,
574
Kleiner, G.I., 680
Klempir, J., 106
Klempirova, O., 106
Klepac, N., 100
Klepitskaya, O.S., 174
Klinger, H., 221, 540
Klimpe, S., 905, 906
Klockgether, T., 42, 862
Klodowska-Duda, G.A., 578, 818
Kloppel, S., 29
Klopstock, T., 906
Klose, U., 476, 758, 893
Kloss, M., 107
Klyushnikov, S.A., 624
Kmiec, T., 418
Knaani, J., 422
Knappmeyer, K., 684
Knol, D.L., 524
Knutzen, A., 15, 394
Ko, H.S., 325
Kobayashi, M., 69, 76
Kobayashi, T., 552
Kobori, S., 332
Kocak, Y., 445
Kocer, B., 101
Koch, M., 930
Koch, R.J., 734
Koegl-Wallner, M., 790

S315
Koelman, J.H.T.M., 27, 136, 395
Koelman, J.T.H.M., 160
Koenig, M., 4
Koerts, J., 252
Koester, J., 885, 888, 891, 896, 900
Kogler, M., 596
Koh, S.-B., 382
Kojima, S., 199
Kokona, B., 553
Kollensperger, M., 856, 862
Kollias, S.S., 567
Komnatska, I., 348
Kondo, A., 199, 635
Kondo, T., 467, 817, 957
Kondou, A., 178
Konig, I.R., 1
Konitsiotis, S., 773
Konrad, P.E., 31
Konstantinos, K., 667
Kontostavlaki, D., 63
Koo, Y.-H., 580
Korbo, L., 822
Korczyn, A., 659
Korczyn, A.D., 317
Korpela, K., 283
Kosaka, S., 584
Koshy, S., 290, 308
Kostic, S., 571
Kostic, V., 303
Kostic, V.S., 46, 159, 268, 376, 696, 937,
961
Kotschet, K.E., 270, 864
Koudelka, C., 496
Koukouni, V., 862
Kourtesi, G., 608
Koutsouraki, E., 386
Kovacs, N., 752, 954
Kowa, H., 24
Koziara, H.M., 853
Kraakevik, J.A., 297
Krack, P., 23, 425, 515, 540, 617
Krakow, K., 666
Kramer, P.L., 1, 377
Kraus, M.C., 107, 868
Kraus, P.H., 960
Krause, M., 23, 107
Krauss, J.K., 191, 192, 209, 211, 218, 861,
930, 945, 970
Kreisler, A., 161, 166
Kremer, B., 875
Kresojevic, N., 961
Kreutzmann, K., 952
Kricorian, G., 337
Krim, E., 761
Kroenke, K., 588
Krogstad, A.L., 685
Krolicki, L., 348
Kronenbuerger, M., 941
Kroonenburgh Van, M.J.P.G., 564, 715,
759
Kruger, M., 860
Kruja, J., 553
Krygowska-Wajs, A.T., 710, 733
Krystkowiak, P., 171, 515
Kuan, C.-C., 722
Kubikova, R., 740
Kubo, M., 649
Kubo, S.-I., 54, 71
Kuehnl, N., 280

Movement Disorders, Vol. 22, Suppl. 16, 2007

S316 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Kuhlmann, T., 406
Kukull, W.A., 22
Kulisevsky, J., 18
Kumazawa, R., 332
Kummer, A., 508, 529, 543, 569
Kunstmann, C., 656
Kunz, J., 733
Kuoppamaki, M., 225, 283, 644
Kupsch, A., 14, 23, 673
Kuramoto, S., 178, 199
Kurtis, M.M., 121
Kurz, M.W., 424
Kushida, C.A., 896
Kutalik, Z., 7
Kutukcu, Y., 351
Kuwahara, T., 471
Kuznetsov, Y., 355
Kwak, Y.T., 678
Kwakkel, G., 629
Kwok, H.H., 67
Kwok, J., 270
Kwon, K.Y., 850
Kwong, W.J., 889, 894
Kyratzi, E., 63
Kyriakakis, V., 773
Kyrylchuk, M., 348
Lacomblez, L., 511, 912
Lagueny, A., 697
Lai, E.C., 545, 615, 769
Lai, S.-L., 127
Laine, S., 425, 427
Lainey, E., 885, 888, 891, 900
Lallement, F., 712
Lalli, S., 180
Lam, C.-W., 421
Lam, G., 669
Lambert, T.D., 333, 870
Lamberti, P., 259, 650
Lambrecq, V., 73
Lammers, G.-J., 875
Lan, M.-Y., 127, 849
Landau, A., 634
Landau, S., 513
Landis, T., 892
Lane, E.L., 806
Lanford, D.N., 566, 602
Lang, A.E., 18, 309, 620, 708, 944
Lannuzel, A., 846
Lanotte, M., 173, 194, 709
Lapeyre-Mestre, M., 536
Larsen, J.P., 305, 312, 329, 424, 520, 528
Larson, E.B., 22
Larson, P., 405
Larsson, M., 668
Larvor, L., 409
Lasjaunias, P., 448
Laska, E., 794
Lau, P.N., 226
Lavallard-rousseau, M.-C., 447
Lawrence, A.D., 534
Lay-Son, L., 939
Le Doze, F.P., 947
Le Rhun, E., 409
Le, W., 614, 654
Leavitt, B.R., 693
Lebedova, Z., 106
Lederman, H.M., 41
Ledon, J.A., 341, 551

Movement Disorders, Vol. 22, Suppl. 16, 2007

Lee, C.-N., 655

Lee, C.S., 170, 243, 850
Lee, D.-H., 382, 655
Lee, H.W., 859
Lee, J.H., 9
Lee, J.Y., 823
Lee, J.-Y., 483, 798
Lee, K., 676
Lee, K.O., 207
Lee, K.-S., 488, 497, 505, 955
Lee, M.C., 170, 243, 850
Lee, M.S., 9, 207
Lee, M.-S., 580
Lee, P.H., 77, 671, 763
Lee, R.K.K., 714
Lee, S., 676
Lee, S.H., 485
Lee, S.-H., 382
Lee, S.K., 170, 850
Lee, T.Y., 170, 243
Lee, W.Y., 35, 167, 473, 966
Lee-Chen, G.-J., 722
Leehey, M.A., 34, 334
Leenders, K.L., 27, 42, 252, 466
Leentjens, A.F.G., 593, 869
Lees, A.J., 18, 134, 230, 275, 298, 304,
336, 348, 370, 408, 416, 441, 484, 534,
720, 838, 845, 852
Leigh, N., 460
Leigh, P.N., 10, 511
Leigh, R.J., 934, 936
Leinonen, M., 225
Leis, B.C., 413
Lekomtseva, Y.V., 836
Lemaire, J.-J., 179
Lemarie, J.-C., 912
Lenda, T., 733
Leone, A., 335
Leonhardt, A., 378
Leow, L., 240
Lepretre, F., 409
Leray, E., 210
Lerdlum, S., 184
Lergans, S., 514
Leroy, A., 292
Lesage, S., 436
Lesca, G., 168
Lesperance, P., 933
Lettieri, C., 162
Leu, S., 284
Leurgans, S., 18, 795
Lev, N., 415
Leventoglu, A., 257
Leverenz, J.B., 22, 413
Levi, A., 786
Levin, B.E., 341, 551
Levin, O.S., 512, 526, 821, 840
Levin, S., 422
Levy, Y., 598
Lew, M.F., 337
Lewis, M., 306
LeWitt, P.A., 18, 338, 572, 726
Lewthwaite, A.J., 333, 870
Lezcano, E., 613
Lhommee, E., 540
Li, J., 642
Li, S., 325
Li, S.-T., 196
Li, Y., 149, 642

Liang, Y., 637

Liani, E., 79
Liao, K., 936
Liddle, M., 626
Liebetanz, K.M., 887
Liepelt, I., 232, 330, 523
Lillo, P., 669
Lim, E.C.H., 165, 908
Lim, I.I.K., 629
Lim, T.S., 671
Limborska, S.A., 624
Limousin, P., 182, 215, 372, 534
Lin, P.T., 132
Lin, S.-C., 886
Lin, S.-H., 293
Lin, S.-Z., 293
Lindsey, C., 390
Lindvall, O., 862
Linnebank, M., 561
Litewka, L.D., 463
Liu, W.-G., 808
Liu, J.-S., 127, 849
Liu, W.-G., 277
Liu, Z., 622
Livrea, P., 259
Ljubisavljevic, M.R., 937
Lleu, P.L., 747, 792
Llorca, P.M., 515
Llorca, P.-M., 924
Lobjanidze, N., 358
Lobreau, J.P., 506
Lobsien, E., 673
Locatelli, M., 116, 206
Loekk, J., 723
Loennfors-Weitzel, T.M.-L., 461
Loesch, D.Z., 463
Lofrano, F., 565
Lohle, M., 49, 336, 952
Lohmann, E., 425, 427
Lohmann, K., 377, 574
Lohmann-Hedrich, K., 1, 379, 442
Lohnau, T.1
Loi, M., 364
Lokkegaard, A., 318, 858
Lolekha, P., 560
Lombardi, S., 45
Long, L.Y., 361
Longo, K., 775
Lopez, G., 420
Lopez-Alburquerque, T., 965
Lopez-Barneo, J., 414, 647
Lopez-Valdes, E., 831
Lopiano, L., 173, 194, 434, 544, 709
Lorberboym, M., 486
Lorenz, R., 627
Lorenzano, C., 32
Lorenzo-Bosquet, C., 480
Loria, G., 193, 384
Louis, E.D., 6, 956, 964
Lousberg, R., 869
Loy, C.T., 799
Lu, G., 74
Lu, G.-Q., 264, 277, 808
Lubarr, N., 683
Lucas, B., 727
Lucas, M., 414, 647
Lucia, P., 768
Ludolph, A., 10
Ludolph, A.C., 511

AUTHOR INDEX
Luetjens, G., 861
Luigi, T., 522
Luo, H., 390
Luo, N., 226
Lynda, W., 314
Lyons, K.E., 572, 964
Lyons, K.F., 749
Lyoo, C.H., 9, 207
Lyu, R.-K., 874
Ma, T.-S., 285
Maarouf, M., 969
MacAskill, M.M., 311
MacDougall, H.G., 255
Machin, A., 774
Machnev, S.O., 821
Machulla, H.-J., 523
MacLeod, A., 328
MacLeod, D., 826
MacMahon, D.G., 260, 338
Maddux, B., 115
Maeda, T., 481
Maeder, M.J., 594
Maetzler, W., 523
Magnano, I., 363
Magno, L., 865
Mah, R., 405
Mahadevan, A., 65
Mahant, N., 143
Mahawish, K.M., 531
Mahieux, F., 292
Maia, D.P., 95, 104, 408, 441, 508, 529,
543, 569, 630
Maier, H., 298
Mair, K., 345, 596
Maita, C., 716
Maita, H., 716
Majahi, L., 392
Maksimovic, J.M.268
Malet, L., 924
Malinverno, M., 865
Maliska, C., 776
Mall, G., 652
Mallet, L., 427, 924
Malone, K., 147
Malzahn, U., 431
Malzer, E., 416
Manara, R., 871
Mancini, F., 544
Mancini, P., 417
Mancione, L.331
Mander, G.J., 60
Mandir, A.S., 344
Mandybur, G.T., 189
Manfredi, L., 544
Manganotti, P., 112
Manharlal, P.K., 361, 958
Manku, M., 693
Mann, W.C., 566, 602
Manor, Y., 801
Mansouri, A., 406
Manus, N.D., 31
Manzoli, L., 118, 682
Mao, X., 956
Maraganore, D.M., 440
Marceglia, S., 116, 206, 335, 762
Marck, M.A. van der, 597
Marco, G., 768
Marco, M., 532

Marcolongo, R., 871

Marconi, R., 434, 650, 760
Marcos Gonzalez, A., 838
Marczewska, A., 646
Marder, K.S., 6
Marek, K., 465, 472, 581
Mares, J., 576
Maric, J., 46, 937, 961
Maric, M., 790
Marinelli, L., 565
Marinez, M., 161
Marini, P., 434
Marinkovic, J.M., 268
Marinus, J., 645, 788
Markopoulou, K., 74
Markova, E.D., 624
Marom, S., 404
Marotta, G., 241, 454
Marras, C., 620, 632
Marsili, G., 231
Marti, M., 741
Marti, M.J., 256
Marti, M.-J., 456
Martignoni, E., 434, 828
Martin, C.E., 197, 201
Martin, W., 326, 779
Martn-Prieto, M., 965
Martinelli, P., 634
Martinez, I., 182, 215
Martinez, J., 532
Martinez-Hernandez, R., 640
Martinez-Martin, P., 18, 258, 336, 510,
625, 633, 718, 791
Martino, D., 139
Martin-Palomeque, G., 446
Masarwa, M., 811
Mash, D.C., 62, 66, 440
Mason, S.L., 706
Massey, L.A., 230, 484, 838
Mastaglia, F.L., 270, 729
Mastronardi, R., 259
Mata, I.F., 413
Mather, E., 115
Mathias, C.J., 290, 308
Matouskova, A., 601
Matroos, G.E., 347, 353
Matson, W., 603
Matsui, T., 178, 199, 635
Matsumoto, S., 175, 584
Matsushima, M., 52
Matsuzaki-Kobayashi, M., 68
Matthias, L., 605
Mattos, J.P., 389
Matur, Z., 135
Matviyenko, Y., 348
Maura, S., 768
Maurice-Tison, S., 761
Maurits, N.M., 27
Mauro, A., 516
Mazurczak, T., 418, 444
Mazurek, U., 818
Mazzocchio, R., 122
Mazzoleni, R., 161, 166
Mazzone, P., 17, 193, 610
McCarthy, D.P., 566, 602
McClain, T., 228
McCormick, W.C., 22
McCorquodale, D., 440
McCreary, A.C., 83

S317
McCrory, P., 909
McDermott, M.P., 750, 753
McFarland, D.F., 320
McIntosh, K., 914
McKhann, G.M., 121
McMurray, B., 228
Meara, J.R., 541
Meco, G., 434, 862
Megrelishvili, M., 358
Mehdorn, H.M., 658
Meigal, A., 558, 764
Meinck, H.-M., 868
Meirav, B., 801
Mejias, R., 414, 647
Melamed, E., 415, 486, 598, 742
Melikyan, G.R., 926, 927
Mellick, G.D., 270
Memis, S., 777
Men, S., 131
Mena, M.A., 262, 282, 662
Mencacci, N., 90
Menendez, M., 339, 609
Menendez-Cuervo, J., 662
Menichetti, C., 458, 482
Merico, A., 911
Meritxell, G., 919
Mertens, P., 221
Mertoglu, O., 789
Meshul, C.K., 57, 734
Mesnage, V., 425
Mess, W.H., 715, 759
Mestre, M., 563
Mestre, T., 102
Metta, V.K., 601
Meunier, S., 128
Meyer, A.E., 41
Meza-Rojaz, G., 625
Micaleff, J., 536
Micankova, B., 117
Michel, P.P., 846
Micheli, F., 625
Michell, A., 706
Mihailescu, C., 490
Mihailescu, G., 342, 490
Mikos, A.E., 229
Milanese, F., 112
Milanovic, S., 937
Miller, D., 800, 883
Miller, D.W., 294
Millet, B., 205
Mima, T., 28
Miniovich, A., 498
Minks, E., 383
Minnerop, M., 42
Minten, A., 679
Miquel, F., 532, 559, 613
Miquel-Rodriguez, F., 480
Mir, P., 392, 414, 613, 647
Miranda, H.A., 189
Miranda, M., 484, 866
Miravite, J., 130
Miskevics, S., 592
Mistry, S., 234, 265
Mitchell-Hay, R., 601
Mitev, V.I., 150
Miura, K., 934, 935
Miwa, H., 467
Miyagi, T., 68
Miyamoto, M., 636

Movement Disorders, Vol. 22, Suppl. 16, 2007

S318 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Miyamoto, T., 236, 636
Miyasaki, J., 595, 802
Miyasaki, J.M., 309
Miyoshi, Y., 635
Mizraji, G., 825
Mizuguchi, M., 169, 202
Mizuno, Y., 54, 71, 276, 421, 824
Mizuta, I., 435
Mochizuki, H., 276, 824
Moeller, P., 822
Mohamad Ibrahim, N., 690
Mohsen, O., 404
Mohsenin, V., 883
Moisello, C., 519
Molaie, M., 474
Molaie, N., 474
Moller, B., 176
Molteni, S., 206
Momeni, P., 441
Monaco, D., 948
Montagna, P., 434
Montastruc, J.L., 536
Montes, S., 640
Montine, T.J., 22
Moore, C., 734
Moore, O., 422
Moore, S., 769
Moore, S.T., 255
Mootanah, R., 801
Moraga, D., 669
Morand, D., 924
Morari, M., 741
Moreau, C., 727
Moreira, M., 4
Moretto, G., 173, 364
Morgan, E., 782, 793
Morgante, F., 142, 760
Morgante, L., 650, 760
Mori, A., 618
Mori, C., 471
Mori, F., 11
Morita, Y., 471
Moriwaka, F., 820
Morris, C.M., 430
Morris, H.R., 793
Morris, J.G.J.L., 799
Morrison, K.E., 333, 870
Morsnowski, A., 181
Mortimer, A., 75
Morton, J., 518
Moschella, V., 5
Moskowitz, C.B., 6
Mouroux, V., 409
Mrakic-Sposta, S., 116, 335
Mueller, B., 312, 329
Mueller, J., 14
Muengersdorf, M., 772
Muhle, H., 1
Muigg, A., 345
Mukerji, M., 43, 44
Muller, T., 794
Mullins, J., 302
Munchau, A., 250, 377, 379, 929
Munger-Clary, H., 288
Munhoz, R.P., 12, 40, 85, 89, 562, 582,
585
Munneke, M., 597, 629
Munoz, E., 94
Munz, M., 92

Movement Disorders, Vol. 22, Suppl. 16, 2007

Murata, K.-Y., 467

Murata, M., 435, 781
Muratori, L.M., 6
Murck, H., 588
Murer, G.M., 72
Murgia, D., 113
Muriel, M.-P., 846
Murphy, C.M., 487
Musante, M.-L., 228
Mutez, E., 409
Muthane, U., 65, 290, 308
Muzerengi, S., 757, 791
Myall, D., 311
Myers, A.J., 904
Mylius, V., 378
Na, D.G., 483
Nader-Kawachi, J.A., 670
Nagai, M., 649
Nagata, K., 481
Nagral, A., 151, 403
Nagy, F., 752, 954
Nahab, F.B., 971
Naidu, Y., 510
Nakadate, K., 78
Nakamura, A., 11, 78
Nakamura, K., 702
Nakamura, Y., 698
Nakanishi, I., 817, 957
Nakashima, M., 470
Napoletani, M., 32
Narayan, R.K., 839
Nardocci, N., 364
Nasar, A., 314
Nation, D.A., 341, 551
Natriashvili, S., 928
Navi, B., 212
Nawara, M., 444
Nederveen, A.J., 453
Negre-Pages, L., 912
Negre-Pages, L.L., 743, 744
Negres-Pages, L., 336
Neimat, J.S., 31
Nelson, A.J., 148
Nerobeev, A.I., 188
Neshige, R., 938
Nestrasil, I., 108, 756
Neumann, A., 1
Neumayer, B., 596
Nevrly, M., 108, 576
Newman, E.J., 626
Neychev, V.K., 150
Ng, K.K.C., 714
Nguyen, F.N., 754
Nguyen, T., 726, 795
Nica, S., 490
Nica, S.M., 342
Nicaretta, D.H., 389, 776
Nicholl, D.J., 333, 870
Nicolae, H., 851
Nicoletti, A., 650
Nicoletti, G., 650
Nieddu, B., 363
Niethammer, M., 96, 550
Nijkrake, M.J., 629
Nijs De, T., 759
Nikolic, V., 571
Nikolov, B., 212
Nillson, C., 862

Nimwegen, M.L. van, 597

Nirenberg, M.J., 212
Nisenzon, A., 229
Nishida, M., 584
Nishikawa, N., 649
Nissinen, H., 225, 644
Nitzan, Z., 653
Nixon, C., 913
The NNIPPS Consortium, 10, 511
Noda, K., 950
Nomoto, M., 649
Nomura, Y., 387
Noorvee, K., 755
Nordby, H., 520
Nordera, G., 223
Nordh, E., 668
Noskowska, J., 426
Noth, J., 511
Novakova, L., 183
Novis, S.A.P., 389, 776
Nowock, J., 37
Noyce, A., 643
Nugent, C., 648
Nutt, J., 726, 794
Nutt, J.G., 57, 297, 494, 496
Nuutinen, J., 558, 764
Nyenhuis, D., 18
Oak, P.J., 164
Oakes, D., 88
Obeso, J., 613
Obi, T., 421
OConnor, S., 297
Odabasi, Z., 351
Odin, P., 510
ODwyer, J.P., 147
Oertel, W.H., 378, 500, 828, 846, 862, 876,
901
Offen, D., 415, 598
Ogawara, K., 39
Ogino, M., 24, 787
Ogino, Y., 24, 787
Ogliastro, C., 8, 119
Oh, K.-M., 655
Oh, S.H., 9, 207
OHanlon, S., 251
Ohmann, S., 928
Ohno, M., 470
Ojemann, S.G., 174
Okawa, S., 48
Okuma, Y., 636, 950
Okun, M.S., 229, 239, 242, 248, 313, 397,
517, 566, 602, 637, 648, 695, 732, 751,
754, 829
Olanow, C.W., 18, 499, 794
OLaoide, R., 147
Oliver, M.M., 782
Oliveras, C., 274
OLoghlen, C., 793
Ondo, W., 340
Ondo, W.G., 214, 255, 623
Onofrj, M., 118, 682, 797, 948
Oostra, B., 55
Oostra, B.A., 411, 434
Opala, G., 578, 818
Optican, L.M., 934, 935, 936
Orimo, S., 11
Orlacchio, A., 5, 5
Orlova, O.R., 188

AUTHOR INDEX
ORiordan, S., 147
Orozco, G., 51, 373
Orr-Urtreger, A., 291, 438
Orsi, A., 58
Orth, M., 379, 921, 929
Oruc, S., 739
Ory, F., 489
Os, J. van, 347
Osaki, Y., 471
Ossowska, K.I., 733
OSullivan, S.S., 230, 275, 534, 720, 838
Otruba, P., 108
Ott, E., 452, 790, 816
Otto, S., 906
Ottaviani, D., 736
Ouatik, N., 355
Ouchchane, L., 179
Overeem, S., 875
Owen, A.M., 780
Owen, R.L., 367
Oz, G., 33
Ozbabalik, D., 87
Ozbakir, S., 374, 445
Ozcakir, A., 692
Ozdoba, C., 461
Ozekmekci, S., 138, 261, 719, 730
Ozelius, L., 168
Ozgen, F., 351
Ozkan, S., 87
Ozkul, A., 777
Oztekin, F.M., 963
ztekin, M.F., 611, 771, 815, 940
O
ztekin, N.S., 101, 611, 739, 771, 815,
O
940, 963
Ozturk, S., 374, 374, 445
Paasuke, M., 755
Padma, M.V., 43, 44
Padovani, A., 865
Pagan, F.L., 344
Paganini, D., 45
Page, D.B., 735
Pagonabarrago, J.M., 487
Pahwa, R., 307, 324, 572, 964
Pal, E., 752, 954
Pal, P.Kr., 591
Palmer, L., 889, 894
Palomino, A., 414, 647
Pamblanco-Bataller, Y., 831
Pan, T., 654
Panagariya, A., 704
Pancetti, F., 669
Panea, C., 851
Panigrahi, M.K., 783, 854
Panisset, M., 238, 266, 271, 340
Pan-Montojo Puga, F., 81
Pantano, P., 129
Panzacchi, A., 180
Panzer, I., 343
Paolo, G., 760
Papapetropoulos, S., 62, 66, 153, 187, 203,
341, 429, 440, 551, 680
Papengut, F., 967, 968
Pappert, E.J., 401
Parashos, S.A., 607
Pardo, C.A., 369
Pareja, J.A., 749
Parisi, V., 882
Pariso, G., 882

Park, B.S., 494

Park, H.J., 832
Park, H.-J., 77
Park, H.Y., 671
Park, J.H., 140, 145
Park, K.S., 798
Park, K.-W., 382, 655
Park, M., 514, 676
Park, M.J., 859
Park, M.Y., 462
Parkes, H., 484
Parkinson Study Group CALM-PD
Investigators, 750, 753
Parman, Y., 135, 907, 916
Parnetti, L., 865
Parrao, T., 656, 866
Pascual, J., 346
Pastor, P., 559
Pastorello, E., 45
Patel, N.R., 294
Paternotte, C., 412
Patrono, C., 5
Patton, J.M., 338
Paulus, K.S., 349, 724
Paulus, W., 406, 554, 684, 887, 898
Pavel, M., 496
Pavlaki, M., 63
Payoux, P., 489
Pearce, R.K.B., 20, 504, 507
Pearson, D.W., 325
Pebet, M., 556
Peckham, E.L., 132
Pegna, A.J., 628
Peitsidou, E., 608
Pek, C.-H., 165
Pekemzovic, T., 159
Peker, S., 198
Pekmezovic, T., 696
Pekmezovic, T.D., 376
Pelissolo, A., 515
Pellecchia, M.T., 862
Peller, M., 15, 394
Pelosin, E., 565
Penzel, T., 431
Peppe, A., 610
Peragut, J.C., 587
Peraino, M., 231
Peralta, C., 825
Perani, D., 180
Perennou, D., 784
Peretz, C., 786
Perlemoine, C., 761
Peron, J., 21, 172, 204, 205, 208, 210, 273
Persi, G., 882
Pes, G.M., 349
Pesare, M., 194
Peskind, E.R., 22
Petrovic, I., 376, 961
Petrovic, I.N., 696
Petrovic, K., 816
Petrovic, M., 303
Petry, K.G., 830
Petzinger, G.M., 599
Pezzoli, G., 241, 289, 336, 454, 544, 646
Phanthumchinda, K., 560
Phibbs, F., 946
Philippot, P., 208, 273
Piano, C., 193
Piccione, F., 911

S319
Picillo, M., 775
Picozzi, P., 180
Pierantozzi, M., 610
Pierguidi, L., 360, 458, 482
Pieri, V., 537
Pietro, M., 699
Pietrzak, J., 426
Pilatus, U., 876
Pilch, J., 444
Pilleri, M., 223
Pilloy, W., 860
Pilosof, N., 415
Pin, C.S., 361
Pinkerton, J., 782
Pinsker, M., 195, 658
Pinsker, M.O., 181
Pintado, E., 414, 647
Piovesan, E.J., 594
Piovezan, M.R., 594
Pirio Richardson, S., 400
Pirkevi, C.S., 436
Pitossi, F., 72
Pittman, A.M., 416
Pituch, L., 322
Plagnol, S., 219, 393
Playfer, J., 513
Playfer, J.R., 503, 538
Pletnikova, O., 325
Plotnik, M., 254
Po, S.-W., 484
Poea-Guyon, S., 412
Poetter, M., 25, 195
Poewe, W., 14, 18, 30, 47, 298, 345, 475,
593, 596, 664, 828, 856, 862
Poirier, K., 444
Poirot, I., 727
Pokhabov, D.V., 245, 402, 973
Polat, R.S., 771
Poletti, B., 90, 103
Pollak, L., 765
Pollak, P., 425, 427, 515, 540, 617, 641
Pollo, C., 216, 246
Polo, A., 112
Polo, A.P., 749
Polo, G., 221
Pont, C., 274
Popa, T., 122
Pope, P., 26
Popow, C., 928
Porta, M., 920
Posen, J., 533, 737
Poston, K.L., 872
Potic, J., 571
Potter, M., 658
Poungvarin, N., 631
Pourcher, E., 2, 278, 766
Poysky, J., 931
Pozdzik-Koseda, A., 902
Praamstra, P., 26
Prabhakar, S., 287
Pragst, F., 352
Pramstaller, P.P., 250
Prasad, K., 43, 44
Prasek, J., 740
Prescher, A., 657
Presciutti, O., 482
PRIAMO Study Group, Italy, 539
Priori, A., 116, 206, 335, 762
Proce, P., 17

Movement Disorders, Vol. 22, Suppl. 16, 2007

S320 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Prokhorov, T., 498, 765
Protas, E.J., 615
Puente, V., 274
Pugliese, P., 650
Pullman, S.L., 121
Purchas, M.A., 260
Pusswald, G., 703
Quaranta, D., 193
Quartarone, A., 25, 142, 521, 760
Quatrale, R., 162
Quattrone, A., 650, 760
Quek, A.M.L., 908
Quinn, N.P., 125, 372, 457, 828, 845, 862
Rabey, J.M., 300, 498, 547, 765
Radovanovic, S., 46, 961
Raethjen, J., 967, 968
Raffard, G., 830
Raggio, V., 556
Raggio, V.E., 437
Ragothaman, M., 290, 308
Raible, A., 848
Raju, T.R., 65
Ramat, S., 157, 934, 935
Ramig, L., 343, 586
Ramig, L.O., 285, 320
Ramirez, A., 866
Ramirez, G., 373
Ramirez-Ruiz, B., 456, 464
Rammsayer, T., 554
Rampini, P., 116, 206
Ramsden, D.B., 67
Rao, A.K., 6
Rao, N., 618
Rascol, O.O., 18, 336, 489, 536, 593, 743,
744, 794, 912
Raskin, S., 40, 85, 89
Raszl, D.L., 872
Ratnagopal, P., 361, 432
Ravat, S.H., 164
Ravenshorst, K.A.B., 784
Ray Chaudhuri, K., 510, 590, 601, 757,
791, 878
Reches, A., 547
Reddy, P., 757
Reddy, R., 783
Redondo, L., 455
Rees, D., 58
Rehncrona, S., 217
Regis, J., 587
Reich, S.G., 619
Reichel, G., 396, 917
Reichmann, H., 49, 336, 593, 605, 952
Reid, W.G.J., 799
Reiff, J., 23
Reijmers, R., 679
Reijnders, J., 869
Reilly, R., 147
Reimold, M., 330, 523
Reina, G., 625
Reindl, M., 596
Reiners, K., 158, 627
Reinikka, K.J.E., 328
Reischel, G., 523
Rektor, I., 114, 146, 383, 803
Rektorova, I., 383, 740, 803
Relja, M., 100, 700
Remple, M.S., 31
Ren, R.-J., 570

Movement Disorders, Vol. 22, Suppl. 16, 2007

Rene, R., 464

Renken, R., 27
Renkliyildiz, B., 771
Renou, P., 7
Reshef, M., 404
Ressner, P., 459
Reuther, P., 772
Revesz, T., 230, 298, 370, 484, 852
Revilla, F.J., 189
Ribacoba, R., 339, 609
Ribas, C.B.562
Ribera, G., 274, 532
Richardson, D., 800, 883
Richardson, M.D., 174
Richelson, E., 637
Richer, F., 933
Rick, C.E., 234, 265
Rideout, H.J., 63
Riemer, R., 390
Rigalleau, V., 761
Rigon, P., 231
Riley, D., 115
Riley, J., 637
Ringel, S., 419
Rinne, J.O., 207
Rintala, D.H., 615
Rios, C., 640
Rissanen, S.M., 558, 764
Ritz, B., 557
Riva, R., 634
Rival, J.-M., 7
Rivas, M.T., 346
Rivera, J., 918
Rivier, I., 712
Rizzo, M., 721
Rizzo, R., 921
Rizzo, V., 142
Roberts, J.L., 325
Roberts, J.W., 413
Robertson, M.M., 921
Robertson, N.P., 793
Robinson, L., 251
Robottom, B., 302
Rodal, I., 262, 282
Rodgers, J.K., 555
Rodnitzky, R.L., 721
Rodol, G., 206
Rodolico, F.R., 855
Rodr`guez, M.S., 365, 670
Rodriguez, D., 972
Rodriguez, R., 242, 732
Rodriguez, R.A., 341, 551
Rodriguez, R.L., 239, 248, 313, 397, 566,
602, 648, 695, 751, 829
Rodriguez Oroz, M.C., 613
Rodriguez-Blazquez, C., 258
Rodriguez-Navarro, J.A., 262, 282, 662
Rodrguez-Ortiz, U., 365, 670
Rodriguez-Ribera, F.J., 263
Roggendorf, J., 969
Roggenkaemper, P., 366
Rojas, C.V., 866
Rojo, A., 559
Rolland, Y., 210, 299
Romer, M., 88
Romrell, J., 566, 602, 648
Roomi, J., 601
Roos, R.A.C., 629
Roosen, N., 914

Roots, I., 431

Ropele, S., 452
Ropipark Study Research Group, 713
Rosa, M.M., 102, 411, 433
Rosa, V., 919
Rose, S., 499, 651, 738
Rosenbek, J.C., 751
Rosenblatt, A., 693
Rosengren, L., 88
Rosenkranz, K., 134
Rosenow, F., 378
Rosner, J., 322
Rosner, S., 438
Ross, B., 148
Ross, G.W., 964
Rossetti, S., 323, 770
Rossetti, S.M., 797
Rossi, A., 122, 360, 458, 482
Rossi, L., 762
Rossi, V., 482
Rosso, A.L.Z., 389, 776
Rosso, M., 194
Rota Kops, E., 42
Roth, J.106, 183
Rothkegel, H., 554
Rothwell, J.C., 17, 125, 134, 142, 160,
372, 929
Rott, R., 79
Rouanet, M., 697
Rouaud, T., 21, 210
Roubertie, A., 168
Rouleau, G., 933
Rountree, S., 623
Rowan, E.N., 661
Rowe, D.B., 270, 729
Roze, E., 16, 284, 447, 448
Rozovski, U., 438
Ruberg, M., 846
Rubinstein, D., 34
Rubio, J.P., 270
Rudzinska, M., 546
Rufra, O., 537
Ruge, D., 372
Ruggeri, P., 565
Ruiz-Ezquerro, J.J., 965
Ruokoniemi, P., 283
Rupp, M., 828
Rusborg, S., 318
Russ, H., 794
Russaouen, O., 85
Russemann, A., 510
Russmann, H., 128
Ruzicka, E., 183, 371, 398
Ruzicka, F., 183
Sablonniere, B., 166, 168
Saelan, Y., 774
Saez, D., 669, 939
Safranow, K., 818
Saggese, E., 865
Sahathevan, R., 690
Sahin, G., 53
Sahin, R., 110
Sahin, S., 672
Saiki, H., 175, 584
Saka, E., 53
Sakai, F., 24, 787
Sakakibara, R., 622
Sakakibara, S.-I., 78

AUTHOR INDEX
Sakamoto, H., 698
Saki, M., 502
Salak, V.L., 555
Salem, G.J., 599
Samal, D., 50, 542
Samelska, J., 818
Samii, A., 413
Sampaio, C., 18, 102, 411, 433, 845, 862
Samuel, M., 513
Sanchez, C., 749
Sanchez, V., 455
Sanchez-Castaneda, C., 464
Sanchez-Pernaute, R., 741
Sandanam, J., 909
Sandro, F., 768
Sanna, G., 82
Sanotsky, Y., 348
Santucci, N., 327
Saquet, C., 168
Saracoglu, M., 350
Sarangmath, N., 290
Sasaki, H., 52
Sasaki, K., 502
Sassi, M., 920
Sasson, E., 663
Sassone, J., 90, 103
Sastry, D., 793
Satake, W., 435
Sau, G., 349, 363
Sauleau, P., 21, 204, 205, 208, 210, 712
Saunders-Pullman, R., 377
Saurugg, R., 790, 816
Saux, A., 220, 385
Savitt, J.M.325, 362
Savola, J.-M., 19
Sawamoto, N., 28
Sawires, M., 30, 475, 862
Sayin, S., 356
Sazci, A., 247, 269, 660, 746, 810, 949
Sazdovitch, V., 863
Scaglione, C., 634
Scanlon, B.K., 551
Scaramelli, A., 437, 556
Schallert, T., 285
Schapira, A.H., 593, 797
Scheiber-Mojdehkar, B., 47
Schellenberg, G.D., 413
Scheller, D.K.A., 651, 738
Scherer, P., 772
Schiavo, G., 13
Schimke, N., 862
Schindehuette, J., 406, 887
Schippling, S., 250
Schlegel, U., 244, 253
Schliesser, M., 627
Schmand, B., 200
Schmidauer, C., 475, 664
Schmidt, A., 377
Schmidt, M.A.M., 597
Schmidt, T., 657
Schneer, S.H., 413
Schneider, G.H., 23
Schneider, S.A., 125, 142, 370, 381, 468,
921, 944
Schnitzler, A., 23
Schollmayer, E., 901
Schols, L., 905, 906
Schott, J.M., 833
Schrader, C., 209

Schrag, A., 18, 275, 534, 720, 833

Schramm, A., 158
Schrempf, W., 49
Schuepbach, M., 380
Schule, R., 906
Schumacher, H.C., 550
Schumm, K., 107
Schupbach, M., 515
Schuster, S., 604
Schuurman, P.R., 200
Schuurman, R., 177
Schuurman, R.P., 186
Schwabe, K., 930
Schwaiger, J., 30, 475
Schwartz, R., 653
Schwartz, R.C., 947
Schwarz, J., 81, 336
Schweiger, D., 55, 411
Schweitzer, K., 330, 523, 758
Schweitzer, K.J., 476, 893
Schwid, S., 340
Schwingenschuh, P., 452, 790, 816
Sebastianelli, M., 458
Sechi, G., 349, 724
Sedlaczek, O., 970
Seet, R.C.S., 165, 908
Segawa, M., 387
Segro, V.R., 391, 583
Seibel, A., 905
Seibyl, J., 472
Seibyl, J.P., 465
Seidel, G., 574
Seidel, S., 50
Sela, B.-A., 653
Selikhova, M., 348
Semra, A., 692, 959
Sen, S., 306
Senard, A., 336
Sener, U., 789
Senghaas, P., 152
Sengun, C., 153, 187, 203, 680
Sengupta, N., 751
Senol, M.G., 350
Sensi, M., 162
Seo, D.W., 966
Seo, K.H., 823
Seo, M.-W., 84, 97, 156
Seoane, A., 274
Seppi, K., 30, 475, 596, 862
Serrano-Duenas, M., 625
Servello, D., 920
Servidei, S., 384
Sesar, A., 346, 613
Sethi, K.D., 301, 307, 337, 900
Sette, E., 162
Sevin, M., 7
Sewter, P.C., 782
Shabtai, H., 786
Shabtay, H., 317
Shadrina, M.I., 624
Shaftman, S., 18
Shah, M., 643
Shahed, J., 931
Shahul Hameed, W.S., 527
Shaikh, A.G., 41, 157, 934, 935, 936, 962
Shakil, S., 57
Shamim, E.A., 132, 137, 344, 362
Shamim-Uzzaman, Q.A., 344
Shang, H.-F., 450, 451

S321
Shankar, S.K., 65
Shanmukhi, S., 783, 854
Shapiro, M.A., 754
Sharma, B., 704
Sharma, T., 323, 770
Sharpley, A., 343
Shashidharan, P., 370
Shaw, K., 336
Shefeld, J.K., 359
Shehadeh, L., 62, 66, 429
Shifrin, C., 291, 422, 438
Shih, L.C., 155
Shihman, B., 509
Shils, J., 213
Shils, J.L., 130
Shin, D.-J., 93
Shin, H.-W., 86, 814, 832
Shin, H.-Y., 167
Shinawi, L., 279, 943
Shingo, T., 635
Shirakura, Y., 311
Shneerson, J., 518
Shoji, M., 272
Short, C., 314
Shrairman, R., 634
Shukla, G., 469
Shulman, L.M., 302, 619
Shungu, D.C., 956
Shunk, K.A., 367
Siderowf, A., 581
Sidransky, E., 70, 410, 413, 420
Siebner, H., 25
Siebner, H.R., 15, 250, 394
Sierra-Beltran, M.A., 365, 670
Sigaudo, M., 709
Silani, V., 90, 103
Silburn, P.A., 270
Silveira-Moriyama, L., 230, 298, 838, 845
Silverdale, M.A., 944
Silverstein, M.P., 239, 248, 397, 695
Simon, E.S., 675
Simonow, A., 772
Simonsen, L., 318
Simpson, R., 931
Simuni, T., 288, 592, 735
Singer, C., 66, 153, 187, 203, 429, 551,
680
Singh, M.B., 44
Singh, S., 469
Singhal, S., 857
Singleton, A., 408, 441
Sipetic, S.B., 268
Siri, C., 241, 454
Sitburana, O., 214, 623, 834
Siviero, M., 871
Skidmore, F.M., 751
Skoblar, B., 229
Skodda, S.K., 244, 253
Skogseid, I.-M., 14
Skoloudik, D., 459
Slominsky, P.A., 624
Smeding, H., 200
Smeding, H.M.M., 105
Smiljkovic, T., 571
Smolenteva, I.S., 526
Sobeih, M., 155
Sobstyl, M.R., 853
Socal, M., 434
Sohn, Y.H., 86, 814, 832

Movement Disorders, Vol. 22, Suppl. 16, 2007

S322 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Sohtaoglu, M., 261
Sokolova, M.O., 188
Solano, R.M., 262, 282, 662
Soleti, F., 384
Soliveri, P., 364
Solomon, D., 936
Soma, H., 52
Soman, T., 708
Sommer, M., 554, 684, 887
Son, E., 676
Song, I.-U., 488, 497, 505, 955
Song, J., 599
Song, S.-K., 832
Soto, A., 51, 373
Spagnolo, F., 180
Spanos, G., 386, 493
Sparks, J., 721
Speelman, H.D., 177
Speelman, J.D., 186, 200
Spevack, L., 271
Spidalieri, R., 122
Spielman, J., 343, 586
Spillantini, M.G., 865
Spinnewyn, B., 501
Spodar, K., 444
Sporkert, F.352
Sposta-Mrakic, S., 206
Sprenger, A., 574
Springer, U., 229
Squitieri, F., 90, 103, 434
Srikijvilaikul, T., 184
Srinivasan, S., 59
Sriram, S., 325
Srivastava, A.K., 43, 44
Srovnalova, H., 740
Stacy, M., 331, 588
Stam, C.J., 807
Stamelou, M., 876
Stamey, W.P., 687, 809
Stampfer-Kountchev, M., 596
Stangenberg, P., 658
Stanislaus, V., 123
Stanton, B.R., 511
Stanzani, C., 364
Stanzione, P., 610
Starr, P.A., 405
Stathis, P.281, 773
Stebbins, G.T., 18, 487
Steele, J.C., 525
Steeves, T., 620, 708
Stefan, K., 468
Stefani, A., 610
Stefanis, L., 63, 80
Stefanova, E., 303
Stefanova, N., 856
Steigerwald, F., 195, 658
Steinberg, L., 212
Steiner, H., 228
Steinling, M., 409
Steinmetz, H., 666
Stenner, A., 396, 917
Stephani, U., 1
Stephanie, B., 179
Stephen, R., 313, 648
Stephen, R.L., 829
Sterlacci, W., 298
Stern, M.B., 18, 581
Stevanin, G., 412
Stevens, J., 270

Movement Disorders, Vol. 22, Suppl. 16, 2007

Stiasny-Kolster, K., 901

Stibal, A., 380, 461
Stiggelbout, A.M., 645
Stillman, P., 904
Stocchi, F., 231, 295, 301, 327, 434, 770
Stockner, H., 30, 475, 596, 664
Stockwell, K., 651, 738
Stoessl, J., 326
Stoffers, D., 807
Stone, E., 711
Storch, A., 49, 81, 336, 375, 952
Storey, E., 463
Stover, N.P., 295
Stozek, J., 546
Strand, C., 370
Strauss, B., 879
Strecker, K., 336
Streitova, H., 146, 383
Stroomza, M.B., 598
Strothjohann, M.H., 280
Strugatsky, R., 811
Strybing, K., 212
Stubbleeld, B.K., 70
Sturm, B., 47
Su, C.-S., 127, 849
Subbakrishna, D.K., 308
Subhani, F.U.H., 600
Suchowersky, O., 227
Sue, C.M., 143, 270, 729
Suess, C., 81
Sugawara, M., 48
Sugeno, N., 68, 69, 76
Suh, Y.-L., 35
Suk, S.-H., 678
Sun, X.-K., 570
Sung, Y.-H., 93
Suppa, A., 32
Sureka, R.K., 704
Surovykh, S.V., 188
Surya, N., 315
Suryaprabha, T., 783, 854
Susy, P., 768
Suteerawattananon, M., 615, 631
Suzuki, K., 236, 636
Svetel, M., 46, 303, 376, 696, 961
Svetel, M.V., 159
Swartz, C., 751
Swartz, C.L., 754
Sweeney, M., 392
Sweeney, P.J., 154
Sycha, T., 542
Szargel, R., 79
Szczaluba, K., 418, 426, 444
Szczudlik, A., 546
Szlachta, K., 235
Szolna, A., 418
Taba, P., 755
Tabamo, R., 465
Tabrizi, S.J., 29
Tacchino, A., 119
Tagaris, G., 773
Tagliati, M., 130, 197, 201, 213
Taira, T., 716
Takagi, S., 332
Takahashi, H., 11, 332
Takahsima, H., 421
Takanashi, M., 686
Takanashi, Y., 728

Takeda, A., 68, 69, 76, 435

Talab, R., 707
Talagala, L., 478
Talelli, P., 125
Tambasco, N., 360, 458, 482
Tamburin, S., 112
Tamma, F., 206, 335
Tan, E.K., 423, 432, 443
Tan, L., 423, 443
Tan, L.C.S., 226, 714
Tan, N.C.K., 714
Tan, Y.-Y., 264, 570
Tang-Wai, D.F., 620
Tanji, H., 619
Taravini, I.R., 72
Tarkka, I., 558
Tarkka, I.M., 764
Tarsy, D., 155
Tarvainen, M.P., 558, 764
Tassone, F., 34, 463
Tassorelli, C., 434
Tatsumoto, M., 236
Tavadyan, Z.D., 926, 927
Tavsan, M., 789
Tawadros, P.B., 109
Tayarani-Binazir, K., 499
Taylor, G., 59
Taylor, H.M., 913
Teive, H.A., 12, 562, 585
Teive, H.A.G., 40, 85, 89, 582, 594
Teixeira, A.L., 104, 508, 529, 543, 569
Teixeira, Jr, A.L., 95, 408, 441, 630
Tekgol, G., 87
Telarovic, S., 700
Telstad, W., 312, 329
Tepper, C., 297
ter Bruggen, J.P., 679
Teresi, J., 18
Teri, L., 22
Terranova, C., 142
Tettenborn, B., 705
Texeira, A., 284
Tezenas, S., 427
Thagesen, H., 822
Thalamas, C., 489, 536
Thees, S., 567
Thiel, A., 969
Thielemans, B., 727
Thijssen, M.A.J., 160
Thobois, S., 16, 427, 540, 617
Thomas, A., 118, 682, 948
Thomas, K., 906
Thomas, P., 727
Thomas, S.K., 331
Thomas-Ollivier, V., 172
Thompsen, T., 595
Thomsen, T., 309, 620
Thomson, E., 626
Thony, B., 141
Thor, P.J., 710
Thorel, D., 168
Thornton, E., 748
Thyagarajan, D., 877
Tiberghien, F., 310
Tiete, J., 506
Tijssen, M.A., 453
Tijssen, M.A.J., 27, 105, 136, 395
Tilley, B.C., 18
Timmann, D., 941

AUTHOR INDEX
Tinazzi, M., 139, 364
Tings, T., 887, 898
Tiple, D., 736
Tisch, S., 17, 182, 215, 372, 534
Tison, F., 73, 515, 697, 761, 830
Titiz, A.P., 445
Tluk, S., 878
Toda, H., 175, 584
Toda, T., 435
Toenjes, A., 800
Toga, A.W., 299
Tola, M.R., 162
Tolosa, E., 94, 256, 559, 593, 718, 828,
862
Tomic, A., 961
Tomiyama, M., 272
Tommasi, G., 173
Toms, N.J., 113, 120
Topcular, B., 907, 916
Torchia, G., 650
Tornqvist, A.L., 217
Torres, K.C., 104
Tosun, D., 299
Totaro, P., 129
Toyoda, T., 169, 202
Toyoshima, H., 481
Toyoshima, I., 48, 237
Traba, A.A., 923
Tranchant, C., 4, 16
Trau, H., 547
Travers, D., 205
Trender-Gerhard, I., 392
Trenkwalder, C., 406, 554, 577, 639, 901
Treves, T.A., 742
Trevisan, C.P., 45
Trinkaus, D.B., 325
Tripathi, M., 44
Tripoliti, E., 182, 215
Tripp, R.M., 157, 962
Trivedi, M.A., 487
Trocello, J.-M., 448
Tromp, S.C., 564, 715, 759
Trompetto, C., 8, 119
Troncoso, J.C., 325
Tronnier, V.M., 941
Trottenberg, T., 14, 673
Trout, L.C., 115
Trujillo, O., 939
Tsagaraki, V., 281
Tsai, S.-T., 293
Tseng, Y.-L., 849
Tsipropoulou, V., 608
Tsoi, T.-H., 421
Tsuang, D.W., 22, 413
Tsuboi, Y., 552
Tuchinda, L., 184
Tuck, K., 449
Tuffery-Giraud, S., 161, 166, 168
Tugnoli, V., 162
Turan, S., 138
Turcotte, H., 266
Turgut, E., 777
Turk, J., 921
Turker, K.S., 64
Turnbull, C.J., 503, 538
Turner, K., 314
Turpin, D., 228
Twerdy, K., 345
Tychalas, A., 493

Tysnes, O.B., 312, 329

Uc, E.Y., 721
Uchihara, T., 11, 24
Uchimura, T., 618
Uchiyama, T., 622
Ueda, S., 78
Uitti, R.J., 835, 886
Ujihara, Y., 368
Ujiie, S., 24, 787
Ulla, M., 179
Uluduz, D., 138
Umaiorubahan, M., 903, 910
Umemura, A., 169, 202
Unger, M.M., 500
Unmuth, B., 330
Untereiner, M., 860
Urayama, S., 28
Urban, C., 232
Urban, D., 70
Urgosik, D., 183
Utsumi, H., 636
Uttner, I., 511
Uueni, D., 755
Uyama, E., 421
Uyanik, O., 110
Uzunel, F., 131, 356, 399
Vaamonde, J., 446, 613
Vacca, L., 327
Vahteristo, M., 644
Vaidyanathan, S., 648
Vaillant, M., 506, 537
Vainio, L., 288, 592
Vakil, E., 653
Valente, A.J., 325
Valente, E.M., 364
The VALID-PD Study Group, 773
Valis, M., 707
Valldeoriola, F., 456, 713
Van Beilen, M., 252
van Beilen, M., 466
van de Loo, S., 37
van de Warrenburg, B.P.C., 125, 905
Van de Winckel, A., 694
van der Brug, M., 294
van der Meer, J., 395
van der Meer, J.N., 453
van der Salm, S.M.A., 160
van der Wilt, G.J., 629
van Doeselaar, M., 55, 411
van Harten, P.N., 347, 353
Van Hemelrijk, B., 694
van Hilten, J.J., 18, 524, 645, 788
Van Laar, A.D., 75
van Os, J., 353
van Rooden, S.M., 645, 788
van Rootselaar, A.-F., 27, 160
Vanbellingen, T., 694
Vanchilingam, S., 903
Vandebona, H., 270
Vandendries, C., 972
Vandrovcova, J., 416
VanGerpen, J.A., 835
Varanese, S., 118, 948
Varma, T., 234, 265
Vasques, X., 219, 220, 385, 393
Vass, K., 50
Vavrek, D., 22
Vekrellis, K., 80

S323
Vela, L., 749
Velazquez, C., 625
Veltman, D.J., 453
Venegas, P., 866
Venn, A., 270
Vent, J., 969
Ventura, R., 437, 556
Verbaan, D., 524, 645, 788
Verbeek, M.M., 875
Vercelletto, M., 7
Verdure, L., 447
Vereijkeren, F., 679
Vergari, M., 116
Verin, M., 21, 172, 204, 205, 208, 210,
273, 299, 712
Viaro, R., 741
Vicentini, S., 124
Victor, P., 919
Vidailhet, M., 16, 284, 447, 448
Vighetti, S., 709
Viguera, M., 532
Vime, P.J., 414, 647
Vingerhoets, F., 216
Vingerhoets, F.J.G., 246
Vink, L., 373
Vink, R., 748
Virgili, J.R., 339, 609
Virues, J., 625
Visanji, N.P., 58, 83
Visentin, E., 643
Visser, J.E., 316
Visser, M., 645, 788
Visser, W., 244, 253
Vital, A., 830
Vitale, C., 82, 522
Vivancos-Matellano, F., 263
Vlaar, A.M.M., 564, 715, 759
Vlajinac, H.D., 268
Voermans, N., 731
Vogiatzi, T., 80
Vohanka, S., 117
Volkmann, J., 14, 176, 181, 195, 621, 658
Volonte, M.A., 180
von Coelln, R., 848
von Mering, M., 375
Vonsattel, J.P.G., 964
Voon, V., 309, 479, 595
Voss, T., 549
Vranova, H., 108, 576
Vuillaume, I., 161, 166, 168
Vulpe, G., 851
Vymazal, J., 371, 398
Wada, C., 48
Wada, K., 276
Wadia, P., 309, 620
Wahner, A.D., 557
Wailke, S., 621
Waines, M., 914
Wakabayashi, K., 11
Waldvogel, D., 567
Wali, G.M., 141
Walker, H.C., 190
Walker, N.D., 371, 398
Walker, R., 251, 711, 827
Walker, R.H., 734
Walker, R.W., 568
Walker, Z., 457
Walsh, R., 147

Movement Disorders, Vol. 22, Suppl. 16, 2007

S324 11TH INTERNATIONAL CONGRESS OF PARKINSONS DISEASE AND MOVEMENT DISORDERS

Walters, A.S., 888
Wan Yahya, N., 690
Wang, X.-J., 808
Wang, D., 190
Wang, G., 264, 570, 579
Wang, J.-G., 451
Wang, S., 726, 795
Wang, X.J., 407
Wang, X.-J., 277
Wang, Y.J., 67
Wang, Z.-Q., 264
Warner, T.T., 13
Watanabe, M., 435
Watts, R.L., 190, 307
Waucquier, N., 727
Weaver, F., 592, 735
Weber, T., 222
Weber, W.E.J., 564, 715, 759
Weder, B., 286
Weeks, R., 757
Weerdesteyn, V., 731
Weigel, R., 192, 211
Weiner, W.J., 619
Weise, D., 158, 627
Weiser, R., 373
Weiss, M., 15
Weisz, D., 130, 213
Welter, M.L., 425
Weniger, D., 286
Wenning, G.K., 596, 856, 862
Wentzel-Larsen, T., 305, 528
Wenzel, K., 790, 816
Wenzelburger, R., 195
Werdelin, L., 858
Werdelin, L.M., 318
Werneck, L.C., 12, 40, 85, 89, 582, 594
Werner, C.J., 652
Werner, P., 593
Wesnes, K., 661
Westaway, S.K., 683
Wetter, T.C., 357, 895, 899
Wevers, A.-K., 3
Wheatley, K., 234, 265
Wheelan, R., 147
Wheelhouse, L., 314
Wheelock, V., 390
Whishaw, I.Q., 227
White, K.D., 313
Whitehead, D., 791
Whitehead, D.L., 503, 538
Wickremaratchi, M.M., 793
Wider, C.W., 835
Widner, H., 217
Wiedemann, J.K., 734
Wieler, M., 326, 779
Wielinski, C.L., 607
Wiener, P., 322
Wilcox, R.A., 877
Wilensky, D., 551
Wilhelm, T., 833
Wilkie, D.J., 735
Willeit, J., 30, 475
Willer, J.C., 284
Williamon, A., 134
Williams, A., 234, 265, 791
Williams, D., 449
Williams, D.R., 230, 838, 852
Williams, K., 637
Williams, S.C.R., 460

Movement Disorders, Vol. 22, Suppl. 16, 2007

Williams-Gray, C.H., 780

Wilson, K., 513
Wilson, L., 228
Wineld, L., 121
Winge, K., 858
Winkelman, J.W., 891
Winkelmann, J., 895
Winkler, S., 379
Winogrodzka, A., 715, 759
Wiseman, M., 271
Witjas, T., 587
Witt, K., 23, 176
Wodarg, F., 181
Woehrle, J.C., 191, 192, 211
Wojtecki, L., 23
Wolf, E., 345, 596
Wolf, S., 652
Wolff von Gudenberg, A., 684
Wolters, E.C., 807
Wolz, M., 49, 605, 952
Wood, B., 249, 251, 711, 827
Wood, N., 392
Wood, N.W., 38, 125, 333, 370, 416
Wooff, E.L., 249
Worbe, Y., 924
Wszolek, Z.K., 835, 853, 886
Wu, H.-S., 127, 849
Wu, J., 834
Wu, Y.-R., 722, 874
Wu, Y.W., 701
Wullner, U., 42, 561
Wyroslak, J., 902
Xie, J., 540
Xie, W., 654
Xie-Brustolin, J., 221, 617, 641
Xiromerisiou, G., 386
Yabe, H., 649
Yabe, I., 52
Yagev-More, H., 438
Yaghisita, S., 237
Yagi, T., 846
Yagishita, S., 24
Yaka, E., 131, 356
Yamada, I., 698
Yamada, K., 169, 202
Yamada, T., 552
Yamamoto, A., 325
Yamamoto, K., 202
Yamamoto, M., 435, 785
Yamamoto, T., 622
Yamanaka, T., 202
Yardimci, N., 841, 942, 953
Yasha, T.C., 65
Yasuda, T., 276, 824
Yasuhara, T., 635
Ye, M., 407
Yener, G., 356
Yi, Z., 432
Yik, J.-H., 165
Yilmaz, E., 777
Yilmaz, O., 739
Yogev, G., 530, 677
Yokoi, F., 149
Yong, S.W., 77, 763
Yoo, B.G., 665
Yoon, J.H., 671
Yoon, W.T., 35, 167, 473

Yoshii, F., 332, 636

Yoshino, H., 421
Yoshiyama, Y., 39
Youdim, M., 654
Youn, J., 35
Young, J.E., 853, 886
Yousry, T., 484
Yu, Q., 121
Yuanzhe, L., 421
Yucel-Altan, B., 739
Yuen, Y., 958
Yust-Katz, S., 486, 742
Zabek, M., 853
Zabetian, C.P., 413
Zackrisson, T., 685
Zadikoff, C., 592, 620
Zagorovskaya, T.B., 624
Zamboni, P., 871
Zanette, G., 112
Zangerl, A., 596
Zanini, F., 860, 863
Zapf, A., 577
Zappia, M., 650, 760
Zarifoglu, M., 692, 778, 880, 959
Zarzana, A., 231
Zebatto, K.B., 367
Zecchinelli, A.L., 646
Zee, D.S., 41, 157, 934, 935, 936, 962
Zeilman, P., 648
Zeng, L.L., 701
Zerbe, G., 334
Zerr, P., 310
Zerrate, M.C., 369
Zeuner, K., 15
Zeuner, K.E., 394
Zezula, J., 50
Zhang, L., 34, 390
Zhang, X.-J., 614
Zhang, Y., 642
Zhang, Y.H., 407
Zhang, Y.-H., 277, 808
Zhao, Y., 423, 443
Zheng, H., 654
Zheng, W., 956
Zheng, Y.-L., 390
Zhong, J., 196
Zhou, D., 450, 451
Zhou, H.-Y., 264, 570, 579
Zhu, W., 654
Zhuang, P., 642
Zhuang, X., 75
Zibetti, M., 173, 194, 544, 709
Ziegler, A., 1
Ziegler, S.G., 410
Ziegler, W., 941
Zimprich, A., 92, 703
Zinzro, L., 182
Zitser, J., 153, 203, 551
Ziv, I.486
Zoccolella, S., 259
Zorlu, F., 789
Zorlu, Y., 789
Zrinzo, L., 215, 372
Zubal, G., 472
Zurowski, D., 710
Zurowski, M., 309, 595
Zylstra, A.A., 321