Guillain-Barré syndrome

Guillain-Barré syndrome (GBS) causes progressive muscle weakness and paralysis (complete inability to use a particular muscle or muscle group), which develops over days or up to four weeks, and lasts several weeks or even months.

GBS typically occurs in a patient who has just recovered from a seemingly uncomplicated viral infection, most commonly cytomegalovirus, herpes, Epstein-Barr virus, or viral hepatitis. GBS may also follow a gastrointestinal infection with the bacteria Campylobacter jejuni, in which case the GBS is particularly severe. About 5% of GBS cases follow a surgical procedure. Individuals with lymphoma, systemic lupus erythematosus, or AIDS have a higher-than-normal risk of GBS. Other GBS patients may have recently received an immunization, while still others have no known preceding event. In 1976-77, there was a vastly increased number of GBS cases among people recently vaccinated against Swine flu. The reason for this has never been identified, and no other flu vaccine has caused such an increase in GBS cases.

The first symptoms of GBS start one to four weeks after the individual has recovered from the initial infection. They consist of muscle weakness (legs first, then arms, then face), accompanied by prickly, tingling sensations. Symptoms affect both sides of the body simultaneously, a fact that helps distinguish GBS from other causes of weakness and tingling. Normal reflexes are firstdiminished, then lost. The weakness eventually affects all the voluntary muscles, resulting in paralysis. When those muscles necessary for breathing become paralyzed, the patient must be placed on a mechanical ventilator which takes over the function of breathing. This occurs about 30% of the time. Severelyill GBS patients may also have problems with fluid balance, severely fluctuating blood pressure, and heart rhythm irregularities.

Diagnosis of GBS involves examining the fluid that bathes the brain and spinal canal through a spinal tap. This involves inserting a needle into the lowerback.

There is no direct treatment for GBS. Instead, treatments are aimed at the disabilities caused by the disease. The progress of paralysis must be carefullymonitored, in case mechanical breathing assistance becomes necessary. Careful attention must also be paid to the amount of fluid the patient is drinkingand eliminating. Blood pressure, heart rate, and heart rhythm also must be monitored.

A procedure called plasmapheresis, performed early in the course of GBS, has been shown to shorten the course and severity of the disease. Plasmapheresis consists of withdrawing the patient's blood, passing it through an instrument that separates the different types of blood cells, and returning allthe cellular components (red and white blood cells and platelets) along witheither donor plasma or a manufactured replacement solution. This is thought to rid the blood of substances that cause GBS symptoms.

It has also been shown that high doses of immunoglobulin, a substance naturally manufactured by the body's immune system in response to various threats, given intravenously (by drip through a needle in a vein), may be just as helpful as plasmapheresis.

About 85% of GBS patients make reasonably good recoveries. However, 30% of adult patients, and a greater percentage of children, never fully regain theirprevious level of muscle strength. Some of these patients suffer from ongoingweakness, others from permanent paralysis. About 10% of GBS patients begin to improve, then suffer a relapse. These patients suffer chronic GBS symptoms.About 5% of all GBS patients die, most from cardiac rhythm disturbances.

Patients with certain characteristics tend to have a worse outcome. These include people of older age, those who required breathing support with a mechanical ventilator, and those who had their worst symptoms within the first sevendays.

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