Guillain-Barré Syndrome
Guillan-Barré Syndrome (GBS)

GBS is a rare disorder in which the body's immune system attacks the nerves to the legs and arms. GBS occurs in about 1 or 2 people per 100,000. It usually presents as a weakness in the legs that spreads upwards to the arms, chest, and the face. Guillain-Barré is one of the leading causes of non-trauma-induced paralysis.

Symptoms

Weakness occurs, usually affecting the lower limbs first and rapidly progressing upwards, with or without numbness or tingling. The disorder can develop over the course of hours or days, or it may take up to 3 to 4 weeks. As the weakness progresses upward, the arms and facial muscles also become affected. In the very rare severe form, the patient may temporarily require a respirator to assist with breathing. Fever should not be present, and if it is, another cause should be suspected.

Cause

Symptoms usually begin a few days or weeks after a respiratory or gastrointestinal infection. Occasionally, symptoms are seen after surgery or vaccinations. The cause of the symptoms is the body's immune response to foreign proteins (such as infectious agents) that is misdirected against the patient's nerve tissues. This is known as an autoimmune disease.

Diagnostic Tests

The diagnosis of GBS is most often based upon the findings of muscle weakness beginning in the legs and progressing upwards. Cerebrospinal fluid analysis (through a spinal tap) and/or tests of nerves and muscles (such as nerve conduction studies) may be ordered to aid in the diagnosing of GBS.

Treatment

Supportive care with monitoring of all vital functions is important in the successful management of GBS. Either high-dose intravenous immunoglobulins (IVIg) or plasmapheresis is used in treatment. Therapy is not as effective if delayed beyond two weeks after the symptoms appear, so treatment should begin as soon as possible. Glucocorticoids have not been found to be effective in GBS. Following the acute phase, the patient may also need rehabilitation to regain lost functions.

Prognosis

With prompt treatment most will regain full or nearly full functional capacity. Recovery generally starts after the third or fourth week and can take a few weeks to a few years. Approximately 80% of patients have a complete recovery within a few months to a year, although minor defects may persist. About 5-10% of patients are left with a severe disability. Another 5-10% of patients have prolonged (or a relapse of) symptoms which characterizes another similar, but chronic condition called Chronic Inflammatory Demyelinating Polyneuropathy (CIDP).