A Family Doctor's Tale - GULLIAN-BARRE SYNDROME

DOC I HAVE GULLIAN-BARRE SYNDROME

GULLIAN-BARRE SYNDROME is a rare neurological disease which affects the nerves of the spine and brain causing progressive weakness of the muscles.

The causes may vary from reaction to an infection or extreme exposure to cold temperatures.

New treatments with immune globulins and removal of antibodies from the blood have improved the prognosis of the illness.

Most patients recover with gradual improvement in muscle strength .

Guillain-Barre Syndrome is a rare autoimmune progressive disease with relatively symmetrical weakness of 2 or more limbs due to neuropathy, areflexia and mild sensory symptoms of not more than 4 weeks duration.

The Guillain-Barre Syndrome is a condition also called acute inflammatory demyelinating polyneuropathy (AIDP).



In Guillain-Barre Syndrome, antibodies produced by the body's immune system attacks the gangliosides of cranial, spinal and peripheral nerve usually after:

1.a nonspecific respiratory or gastrointestinal viral illness

2.infectious mononucleosis

3.Campylobacter jejuni , cytomegalovirus infection

4.vaccination

5.extreme exposure to cold

The result of the antibodies attack on the peripheral nerves is inflammation of myelin and blockage of nerve conduction, resulting in muscle paralysis with sensory or autonomic deficits.



Guillain-Barre Syndrome occurs at any age usually at age 30-40.

It occurs more in men than in women.



The typical feature of Guillain-Barre Syndrome is

1.acute onset of symmetrical muscle weakness in both lower limbs with
ascending progression of the weakness upwards to the arms and face usually within hours or days.

2.the lower cranial nerves can also be affected leading to bulbar weakness, (dysphagia or difficulty with swallowing) and respiratory difficulties. If this happens , hospitalization is a must.

3.Sensory loss is usually minor with loss of position, numbness or tingling sensation.

4.areflexia or complete loss of deep tendon reflexes is always present.

5.Loss of pain and temperature sensation may be present but mild.

6.pain is a common symptom with deep aching pain usually in the weakened muscles.

7.Autonomic dysfunction such as tachycardia, diarrhea, bladder dysfunction may occur but are usually transient.

8.absence of fever is one of the sign of Guillian Barre Syndrome.
If there is fever, another cause should be suspected.

Recovery usually begins 2-4 weeks after progression of disease stops.



The diagnosis of Guillain-Barre Syndrome involve

1.History of a rapid onset and progression of ascending motor weakness, areflexia and the absence of fever.

2.physical and neurological examinations for peripheral neurological deficit.

3.cerebrospinal fluid examination-
typical CSF findings show albumino-cytological dissociation,

a. an elevated protein level (100 - 1000 mg/dL)
b. absent pleocytosis (increased cell count)unlike infectious causes.

4.Electromyographic studies and nerve conduction studies may show prolonged distal latencies, conduction slowing, nerve conduction blockage suggestive of demyelination.

5.Computed tomography or MRI may show actual demyelination of the nerve.

Complications for Guillain-Barre Syndrome are:


1.Progression of nerve involvement to whole body

2.Paralysis of respiratory muscles and swallowing can be life threatening



Treatment for Guillain-Barre Syndrome may be urgent especially in cases of respiratory distress:

1.Hospitalization and early intubation with a respirator on standby in case of difficulty in respiration

2.treatment of the underlying cause is started as soon as possible:

a. High dose intravenous immune globulin, which temporarily modifies the immune system and provides the body with normal antibodies from donated blood or

b.plasmapheresis in which abnormal antibodies are removed from the blood.

3.rehabilitation is started after the patient is stable and will focus on improving activities of daily living functions such as brushing teeth, washing and getting dressed.

4.physiotherapy to strengthen muscles

5.Speech therapy for speech and swallowing

6. Corticosteroids have no part to play in the treatment of Guillian Barre Syndrome.

Prognosis of Guillian-Barre Syndrome:


1.With all these new treatments the prognosis for most patients with Guillain-Barre Syndrome is good with 80% recovery within 1 year

2.Some of them may have persistent minor neurological deficits such as areflexia.

3.Some 5% recover but with severe disability involving severe proximal motor and sensory axonal damage with inability of axonal regeneration.

4.About 10% of patients have relapses and are as classified as having chronic inflammatory demyelinating polyneuropathy

5.Death is rare(5%) and may be due to severe paralysis, autonomic dysfunction and other severe pulmonary complications.