Guillain Barre syndrome (GBS) is an acute disorder of the peripheral nerves, and in particular the nerves which supply the muscles, leading to progressive weakness and paralysis including the muscles of the arms and legs, as well as sometimes the respiratory and swallowing muscles. It may be accompanied by some instability and "autonomic" control of things such as blood pressure and heart rate. Some unusual variants of the syndrome can also cause incoordination and double vision (Miller-Fisher variant, sensory and ataxic variants). The condition is monophasic, that is it occurs as an acute condition that worsens and then improves. Although serious and potentially fatal, with the availability of modern intensive care facilities, mortality rate has fallen and most patients make a complete functional recovery.

Current evidence suggests that GBS is an auto immune disease, caused by activation of the person's own immune system against their peripheral nerves. Two thirds of patients report a preceding infective illness such as a cold or diarrhea, usually occurring 2 or 3 weeks before onset of GBS. The symptoms of GBS usually come on gradually over the course of 2 to 3 weeks, plateau, and then begin to improve 2 to 4 weeks later with full recovery taking 6 to 12 months. About 1/10 of patients are left with some residual problem, usually weakness in a limb. Even with the best medical therapy, up to 6% of people die from GBS, usually related to the complications of immobility and weakness.

Patients who present with the typical form of GBS are usually easily diagnosed. Occasionally however, people with a spinal cord problems, Lyme disease and rabies can all be confused with GBS. Botulism can also be mistaken for GBS in infants and children.

WHAT CAN I DO ABOUT IT?

Early diagnosis is critical for GBS. All patients with GBS must be admitted to hospital for monitoring and for respiratory support with a ventilator if needed.

Diagnosis of the condition usually follows upon a neurological examination. A lumbar puncture may also be done to look for high protein levels in the cerebral spinal fluid which are usually associated with GBS, and to exclude other possible diagnoses. Electromyography and nerve conduction studies are also sometimes done to help confirm the diagnosis and later to help with prognosis.

Treatment centres around supportive care during the acute phase of the illness. Studies suggest that intravenous human immunoglobulin or plasmapharesis (replacement of the patient's own plasma with disease free plasma) will hasten recovery and attenuate the severity of the disease if given early. Corticosteroids appear ineffective.

Patients and families affected by this disease are often anxious and require emotional support especially during the acute phase before recovery begins. Once the acute phase is over, a realistic rehabilitation plan should be followed.

Dr. R. O'Brien

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