Guillain Barre Syndrome

I. DEFINITION
GBS is an inflammatory disease resulting in axonal demyelination of
peripheral nerves. Characteristics of GBS include a quickly progressing,
symmetrical ascending paralysis starting with the feet; pain, starting with
the legs; absence of deep tendon reflexes; mild sensory loss in glove and
stocking distributions; cranial nerve dysfunction with possible facial palsy;
an autonomic nervous system response of postural hypertension and
tachycardia; and respiratory muscle paralysis.

II. CLINICAL MANIFESTATIONS

The disease is characterized by weakness which affects the lower limbs
first, and rapidly progresses in an ascending fashion. Patients generally
notice weakness in their legs, manifesting as "rubbery legs" or legs that
tend to buckle, with or without dysesthesias (numbness or tingling).
As the weakness progresses upward, usually over periods of hours to
days, the arms and facial muscles also become affected. Frequently, the
lower cranial nerves may be affected, leading to bulbar weakness,
(oropharyngeal dysphagia that is difficulty with swallowing, drooling,
and/or maintaining an open airway) and respiratory difficulties. Most
patients require hospitalization and about 30% require ventilatory
assistance.
Facial weakness is also commonly a feature, but eye movement
abnormalities are not commonly seen in ascending GBS, but are a
prominent feature in the Miller Fisher variant (see below.)
Sensory loss, if present, usually takes the form of loss of proprioception
(position sense) and areflexia (complete loss of deep tendon reflexes), an
important feature of GBS. Loss of pain and temperature sensation is
usually mild.
In fact, pain is a common symptom in GBS, presenting as deep aching
pain usually in the weakened muscles, which patients compare to the
pain from overexercising. These pains are self-limited and should be
treated with standard analgesics.
Bladder dysfunction may occur in severe cases but should be transient.
If severe, spinal cord disease should be suspected.
Fever should not be present, and if it is, another cause should be
suspected.
 In severe cases of GBS, loss of autonomic function is common,
manifesting as wide fluctuations in blood pressure, orthostatic
hypotension, and cardiac arrhythmias.
The symptoms are similar to those for progressive inflammatory
neuropathy.

III. EPIDEMIOLOGY
Severity of symptoms varies from so mild that medical attention is
unlikely to severe disease that may cause death in approximately 10% of
the cases.
. Its distribution is known to be worldwide, with an incidence of 1.3 to 2
per 100000. Men are more slightly commonly affected than women, and
GBS most oftenly occurs in young adults (20-24 yrs old) and the elderly
(70-74 yrs old)

IV. ETIOLOGY
The exact cause of GBS is not known. However, it is believed that the
disorder is an autoimmune disease, a condition in which the immune
system attacks its own tissues as though they were foreign substances.

Nerves are damaged by the immune system, usually in response to a

viral or bacterial infection or other illness. As the immune system
produces antibodies to fight the infection or illness, it may also produce
antibodies that attack the covering (myelin sheath) of the peripheral
nerves and sometimes the nerve fibers (axons). The resulting nerve
damage leads to tingling and numbing sensations, muscle weakness, and
paralysis.

Although it is not known exactly what triggers the body's response,

GBS most often develops after a respiratory or gastrointestinal infection.
A variety of infections may be associated with GBS. Those that have been
most commonly linked to the disease are Campylobacter jejuni (a
bacterial infection that affects the intestinal tract), mycoplasma (a type of
bacteria that can cause pneumonia), cytomegalovirus (CMV), Epstein-Barr
virus (EBV), and varicella-zoster virus, the virus that causes chickenpox
and shingles.
 The 1976 swine flu vaccine was linked to GBS. But since then, no clear
links have been found between any flu vaccine and GBS. Getting the flu is
much more of a health risk than is the chance that you could get GBS
after having the flu vaccine.

V. PATHOPHYSIOLOGY
All forms of Guillain-Barré syndrome are due to an immune response to
foreign antigens (such as infectious agents or vaccines) but mistargeted
to host nerve tissues instead (a form of antigenic mimicry). The targets of
such immune attack are thought to be gangliosides, which are complex
glycosphingolipids present in large quantities on human nerve tissues,
especially in the nodes of Ranvier.
The end result of such autoimmune attack on the peripheral nerves is
inflammation of myelin and conduction block, leading to a muscle
paralysis that may be accompanied by sensory or autonomic
disturbances.
However, in mild cases, axonal function remains intact and recovery
can be rapid if remyelination occurs. In severe cases, such as in the AMAN
or AMSAN variants, axonal degeneration occurs, and recovery depends on
axonal regeneration. Recovery becomes much slower, and there is a
greater degree of residual damage. Recent studies on the disease have
demonstrated that approximately 80% of the patients have myelin loss,
whereas, in the remaining 20%, the pathologic hallmark of the disease is
indeed axon loss.

VI. DIAGNOSIS
GBS may be difficult to diagnose in its early stages and requires a
detailed examination of the nervous system. Your doctor will ask about
your symptoms, including when they started and how they have changed
over time. A history of recent infection (especially respiratory or
gastrointestinal illness) may also be an important clue in the diagnosis.
Two important signs must be present for your doctor to diagnose GBS:
progressive weakness in both arms and both legs and the loss of reflexes.
Early on, your doctor may do a lumbar puncture to check your spinal
fluid for proteins and other markers that can help make the diagnosis.

VII. MEDICAL MANAGEMENT

 Treatment of GBS depends on how severe your symptoms are and
whether complications start. The main treatment for GBS is
immunotherapy given in the hospital. This treatment includes plasma
exchange or intravenous immune globulin (IVIG). In many people,
immunotherapy may speed recovery when used early in the course of the
disease. Treatment also involves preventing and managing complications
(such as breathing problems or infections) and providing supportive care
until symptoms begin to improve.

VIII. COURSE AND PROGNOSIS

Most of the time recovery starts after 4th week from the onset of the
disease. Approximately 80% of patients have a complete recovery within
a few months to a year, although minor findings may persist, such as
areflexia. About 5–10% recovers with severe disability, with most of such
cases involving severe proximal motor and sensory axonal damage with
inability of axonal regeneration. However, this is a grave disease and
despite all improvements in treatment and supportive care, the death
rate among patients with this disease is still about 2–3% even in the best
intensive care units. Worldwide, the death rate runs slightly higher (4%),
mostly from a lack of availability of life support equipment during the
lengthy plateau lasting 4 to 6 weeks, and in some cases up to 1 year,
when a ventilator is needed in the worst cases. About 5–10% of patients
have one or more late relapses, in which case they are then classified as
having chronic inflammatory demyelinating polyneuropathy (CIDP).

IX. OT INTERVENTION
Modifications during the plateau phase should be considered temporary.
Examples of interventions that may be required:
 Developing communication tools such as sign or picture board
 Ensuring access to the nurse call button
 Adapting TV and lights remote control
 Modifying the telephone for hands-free use
 Modifying positions for lying and sitting to those optimal for
function and comfort
 Positioning for head, trunk and upper extremity stability
 Teaching about GBS and recommending other support services
 Teaching strategies to reduce anxiety
Recovery phase interventions are oriented to the resumption of activities
and roles. Examples of interventions that may be required:
 Instructing in safe mobility as strength incrementally returns
 Demonstrating independent transfers
 Training in modified self-care techniques
 Providing temporary aids and equipment
 Adapting modes of communication according to person’s priorities
 Encouraging access to the community
 Modifying and encouraging routine activities as appropriate
 Adapting equipment and modifying behavior in home, leisure and work
activities
 Instructing in energy conservation
 Providing instruction on and modifying employment roles and tasks