Stiff man syndrome

Stiff-arming immunity's balancing act

Rare in number and dramatic in their symptoms, the adult patients who develop stiff-man syndrome suffer chronically rigid muscles, which make movement difficult and cause deformed joints. The powerful muscle spasms that later appear can rip muscles and break bones, and are usually treated with anxiety-reducing drugs. Although scientists know the syndrome is a central nervous system disease frequently associated with diabetes and epilepsy, they have failed to find autopsy evidence of nervous-system abnormalities. Now researchers from the Milan University Medical School in Italy report in the April 21 NEW ENGLAND JOURNAL OF MEDICINE that the disease may be caused by a derange dimmune system.

The group has found antibodies against the enzyme glutamic acid decarboxylase in the spinal fluid and blood of a woman with the disease. The enzyme is essential for proper functioning of a subset of nerve cells. The authors say antibodies against it may not themselves be the primary cause of stiff-man syndrome, but rather a result of earlier autoimmune attacks directed against other components of nerves. According to an accompanying editorial, if subsequent studies confirm these findings, stiff-man syndrome would be the first known example of a nervous-system disease related to a specific anti-enzyme autoantibody.

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