Clinical Correlation in Physical Therapy

GUILLAIN – BARRÉ SYNDROME

Umphred’s Neurological Rehab – Yellow  slightly higher in MEN > Women (often EQUAL)
Robbin’s Basic Pathology – Blue  WHITES > Blacks
Braddom’s Physical Medicine and Rehab – Green
Etiology and Risk factors
De Lisa Physical Medicine and Rehab – Orange
Goodman Pathology Implications – Violet  Bacterial (Campylobacter jejuni)

 Viral (Haemophilus influenza, Epstein-Barr Virus,

Guillain-Barre Syndrome (GBS) and Cytomegalovirus) infections

 A.K.A. acute inflammatory demyelinating  Molecular mimicry at the B cell level, an

polyneuropathy (AIDP) autoimmune theory, is the primary theory for the
cause of GBS because evidence exists for
 A rapidly progressive acute demyelinating disorder antibody-mediated demyelination.
affecting motor axons, resulting in ascending
weakness that can lead to death from failure of  Surgery
respiratory muscles within days of onset of
symptoms.  Vaccinations

 One of MC life-threatening diseases of the PNS.  90% has illness (e.g. respiratory or gastrointestinal)
during the preceding 30 days.
 Typically reach maximal weakness within 2 – 3
weeks. Spends weeks to months to recover.  Exogenous virus causing the immune system to
attack the peripheral nervous system.
Triggered by an infection or vaccination that breaks
down self – tolerance, thereby leading to an autoimmune  HIV is a major cause of inflammatory
response. demyelinating polyneuropathies.

Associated infectious agents include:

 Occurs early, often around the time of
 Campylobacter jejuni  Campy (Curved) and seroconversion (antibodies for the virus becomes
Jejuni (Jejunum [part of an intestine]), a bacteria present in the blood), and is clinically and
that can be obtained from raw meat, unpasteurized
milk, contaminated drinking water, or poultry. Effect histopathologically indistinguishable from the
is watery/bloody diarrhea. idiopathic form.

 Epstein – Barr virus

 Most typically a post infection demyelination of the
 Cytomegalovirus peripheral nerve with both perineurial and axonal
damage.
 Human immunodeficiency Virus (HIV)

 Zika Virus  Breakdown of the blood-nerve barrier and

segmental, macrophage-mediated damage to the
myelin sheath. Inflammation and demyelination
Injury is most extensive in NERVE ROOTS,
PERIPHERAL NERVES, and PROXIMAL NERVE result in varying degrees of axonal degeneration,
SEGMENTS and is associated with MONONUCLEAR and neurapraxia is prominent.
CELL INFILTRATES RICH IN MACROPHAGES

INCIDENCE The Miller Fisher syndrome is a relatively benign variant,

occurring in about 5% of AIDP cases. It is characterized
 Mostly seen in YOUNG ADULTS and in the 5th
through 8th decades. by clinical TRIAD ophthalmoplegia (paralysis of the
muscles surrounding eye), areflexia (a.k.a. hyporeflexia
 Clinical Correlation in Physical Therapy
GUILLAIN – BARRÉ SYNDROME

 absent reflex response), and ataxia (poor muscle

control, clumsy voluntary movements, cause difficulty
Clinical Manifestations (ASCENDING)
with walking and balance, hand coordination, speech
and swallowing, and eye movements). Antibodies to  Rapidly ascending symmetric motor weakness
that begin in the HANDS AND ARMS (instead of
GQ1b (Ganglioside Antibody) are common. (DE LISA, feet and legs) and DISTAL sensory impairments.
745)
 FIRST NEUROLOGIC SYMPTOM  Paresthesia
in the toes, followed within hours or days by
Acute motor axonal neuropathy (AMAN)  axonal
weakness distally in the legs.
variant that follows C. jejuni infection. Wallerian
degeneration (axon that is injured degrades and die)  Weakness spreads to involve arms, trunk, and
facial muscles.
occurs. The clinical presentation is similar to that of
AIDP but myelin is not affected. The antibody  Hypoventilation (Respiratory Acidosis)
mediators include GM1, GD1a, and GD16. (DE LISA,
 Axonal Degeneration
745)
 Demyelination

 Palatal and facial muscles become involved in

PATHOGENESIS about half of all cases; even the muscles of
mastication may be affected, but nerves to
 Lesions Occur throughout PNS from the SPINAL extraocular muscles typically are not involved.
NERVE ROOTS to the DISTAL TERMINATION of B
Motor and Sensory Fibers.  Flaccid Paralysis  Absence of DTRs
 C. jejuni is associated more commonly with the
axonal form  Areflexia  Absent response
 Greater sensory involvement is seen following
cytomegalovirus  Albuminocytologic Dissociation  High levels of
CSF protein w/o elevation in WBC
 Myelin of the Schwann cell is the primary target of
attack.  Sensory
 Lymphocytes (T cells) and macrophages are the
inflammatory cells present.  Detrusor hyperreflexia (inability to control micturition
 Demyelination, initiated at the node of Ranvier, reflex)
occurs because macrophages, responding to
inflammatory signals, strip myelin from the nerves.  Pre-ganglionic fibers of the ANS are myelinated,
they, too, may be subject to demyelination. If this
 Many believe that the axons are damaged during
the inflammatory process, according to what has occurs, tachycardia, abnormalities in cardiac
been called a “bystander effect.” rhythm, blood pressure changes, and vasomotor
symptoms occur.
 Presence of macrophages that invade periaxonal
spaces, causing the axon to degenerate within the  Toxins
ventral roots
o Lead (Axonal Degeneration, Motor, and
Sensory)

o Organophosphate (Axonal Degeneration,

Demyelination, Motor, and Sensory.
 Clinical Correlation in Physical Therapy
GUILLAIN – BARRÉ SYNDROME

Goodman PROGNOSIS

The primary methods of managing GBS have helped to

improve mortality rates, which can exceed 5%. Factors
that predict a poor outcome include onset at an older
age, a protracted time before recovery begins, and the
need for artificial respiration. A recent analysis of
prognostic predictors in GBS concluded a younger age,
absence of preceding diarrhea, lower levels of disability
and admission, longer interval between symptoms and
admission, and absence of ventilator support need all
being favorable factors. Although most persons recover,
up to 20% can have remaining neurologic deficits. After
1 year, 67% of clients have complete recovery, but 20%
remain with significant disability. Even after 2 years, 8%
have not recovered. Complications that can persist, even
when function is recovered, include neuropathic pain,
autonomic changes and distal weakness in the
extremities.

POLIOMYELITIS
Hysteria is the most common misdiagnosis. Because
of the speed of onset, a stroke involving the brainstem
will also be considered. Less-common causes of acute
neuropathies must also be considered, including tick
paralysis, and metabolic disorders such as porphyria.

Treatments

 The rehabilitation management of AIDP focuses on

the prevention of contractures, skin breakdown,
pneumonia, and depression. During the acute
phase, communication devices, a trapeze, pressure
relief bed surfaces, and bed rails are helpful.
Because AIDP presents with evolving weakness,
strengthening, bracing, adaptive equipment, and
vocational retraining are not appropriate until the
clinical findings have stabilized. Retraining for
activities of daily living (ADLs), wheelchair and
ambulation training, and bracing may be necessary
if there is significant residual impairment and
disability.
 Administration of high-dose immunoglobulins (Ig) (a
protein the immune system normally uses to attack
foreign organisms)
 Plasmapheresis or Plasma Exchange (removes
plasma from circulation and filters it to remove or
dilute circulating antibodies, significantly improves
the impairments in GBS)