Rare Diseases: Guillain-Barré Syndrome

Monday, November 15, 2010

Guillain–Barré Syndrome is a rare autoimmune disorder in which the body's immune system attacks part of the peripheral nervous system. The syndrome was named after French physicians Georges Guillain, Jean Alexandre Barré and André Strohl. In 1916 they published a report in which they recorded and interpreted the tendon reflexes of their patients and recognized the peripheral nature of the illness. It is sometimes called Landry's paralysis after the French physician Jean Landry that in 1859 published a report on 10 patients with an ascending paralysis. The syndrome affects 1 or 2 people per 100.000. It can occur at any age, but is most common in people of both sexes between ages 30 and 50. GBS is a single disorder; however, it has several variant forms.The exact cause of Guillain-Barré Syndrome is unknown, but it often follows a minor infection, such as a lung infection, respiratory infection or gastrointestinal infection. It is frequently severe and the first symptoms include varying degrees of weakness or tingling sensations in the legs that can spread to the upper limbs and the face along with complete loss of deep tendon reflexes. The syndrome is one of the leading causes of acute non-trauma-induced paralysis in the world. Early symptoms of GBS can include fatigue, pain in the back of head, severe muscle pain, nausea, vomiting, loss of appetite, stomach pain and migraines. These symptoms can increase in intensity until certain muscles cannot be used and in severe cases lead to paralysis. The majority of patients require hospitalization and more than 30% require ventilatory assistance, as the lower cranial nerves can be affected, leading to bulbar weakness, difficulty swallowing and respiratory difficulties. Causes of death include respiratory distress syndrome, sepsis, pulmonary emboli and cardiac arrest.Recovery usually starts after the fourth week from the onset of the disorder. With prompt treatment by plasmapheresis or intravenous immunoglobulins and supportive care, up to 85% of patients with GBS achieve a full and functional recovery within 6-18 months, but 5-10% recover with severe disability. The syndrome can also occur with other medical conditions such as systemic lupus erythematosus, Hodgkin's disease and AIDS. It is believed Guillain-Barré Syndrome can be a rare side-effect of influenza vaccines, as there were reports of GBS affecting 10 per million who had received swine flu immunizations in the 1976 U.S. outbreak of swine flu.

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