Guillain-Barré Syndrome

What is Guillain-Barré Syndrome (GBS)?

Guillain-Barré (Ghee-yan Bah-ray) Syndrome, also called acute inflammatory demyelinating polyneuropathy and Landry's ascending paralysis, is an inflammatory disorder of the peripheral nerves (those outside the brain and spinal cord). It is characterized by the rapid onset of weakness and, often, paralysis of the legs, arms, breathing muscles and face. GBS is the most common cause of rapidly acquired paralysis in the United States today, affecting one to two people in every 100,000.

It typically begins with weakness and/or abnormal sensations of the legs and arms. It can also affect muscles of the chest, face and eyes. Although many cases are mild, some patients are virtually paralyzed. Breathing muscles may be so weakened that VENTILATOR is required to keep the patient alive. Many patients require an intensive care unit during the early course of their illness, especially if support of breathing with a VENTILATOR is required. Although most people recover, the length of the illness is unpredictable and often months of hospital care are required. The majority of patients eventually return to a normal or near normal lifestyle, but many endure a protracted recovery and some remain wheelchair-bound indefinitely.

What Causes GBS?

The cause is not known. Perhaps 50% of cases occur shortly after a microbial (viral or bacterial) infection such as a sore throat or diarrhea. Some theories suggest an autoimmune mechanism, in which the patient's defense system of antibodies and white blood cells are triggered into damaging the nerve covering or insulation, leading to weakness and abnormal sensation.

How is GBS Diagnosed?

Quite often, the patient's symptoms and physical exam are sufficient to indicate the diagnosis. The rapid onset of (ascending) weakness, frequently accompanied by abnormal sensations that affect both sides of the body similarly, is a common presenting picture. Loss of reflexes, such as the knee jerk, are usually found. To confirm the diagnosis, a lumbar puncture to find elevated fluid protein and electrical test of nerve and muscle function may be performed.

How is GBS Treated?

Because progression of the disease in its early stages is unpredictable, most newly diagnosed patients are hospitalized and usually placed in an intensive care unit to monitor breathing and other body functions.

Care involves use of general supportive measures for the paralyzed patient, and also methods specifically designed to speed recovery, especially for those patients with major problems, such as the inability to walk. Plasma exchange (a blood "cleansing" procedure) and high dose intravenous immune globulins are often helpful to shorten the course of GBS.

Most patients, after their early hospital stay and when medically stable, are candidates for a rehabilitation program to help learn optimal use of muscles as nerve supply returns.

What research is being done?

Scientists are concentrating on finding new treatments and refining existing ones. Scientists are also looking at the workings of the immune system to find which cells are responsible for beginning and carrying out the attack on the nervous system. The fact that so many cases of Guillain-Barré begin after a viral or bacterial infection suggests that certain characteristics of some viruses and bacteria may activate the immune system inappropriately. Investigators are searching for those characteristics. Neurological scientists, immunologists, virologists, and pharmacologists are all working collaboratively to learn how to prevent this disorder and to make better therapies available when it strikes.

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