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Stiff-Person Syndrome

By Michael Rubin, MDCM, Professor of Clinical Neurology;Director, Neuromuscular Service and EMG Laboratory, Weill Cornell Medical College;New York Presbyterian Hospital-Cornell Medical Center

Stiff-person syndrome causes muscle stiffness that worsens gradually

This syndrome is more common among women and often occurs in people with type 1 diabetes, certain autoimmune disorders, or certain kinds of cancer, including Hodgkin lymphoma (Hodgkin disease).

The cause may be an autoimmune reaction—when the body produces antibodies that attack its own tissues. In stiff-person syndrome, these antibodies attack nerve cells in the spinal cord that control muscle movement. Antibodies that attack an enzyme called glutamic acid decarboxylase are present in 60% of people with stiff-person syndrome.

Muscles of the trunk and abdomen gradually become stiffer and enlarge. Muscles of the arms and legs are affected less. Usually, the disorder progresses, leading to disability and stiffness throughout the body.


  • Diazepam (a sedative) or another drug to relax the muscles

  • Immune globulin

  • Sometimes corticosteroids

  • Sometimes plasma exchange

The sedative diazepam can consistently relieve the muscle stiffness. If diazepam is ineffective, other drugs, such as baclofen (a muscle relaxant), may be tried.

Immune globulin (a solution containing many different antibodies collected from a group of donors), given intravenously, may help relieve symptoms for up to a year.

Corticosteroids can help but, if taken for a long time, have many side effects.

Plasma exchange, which involves filtering toxic substances (including the abnormal antibodies) from the blood, is sometimes tried but often without success.

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