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Stiff-Person Syndrome

By Michael Rubin, MDCM, Professor of Clinical Neurology;Attending Neurologist and 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.

  • Stiff-person syndrome often occurs in people with type 1 diabetes, certain autoimmune disorders, or certain kinds of cancer.

  • Muscles gradually become stiffer and enlarge, starting in the trunk and abdomen but eventually affecting muscles throughout the body.

  • Doctors suspect stiff-person syndrome based on symptoms but use electromyography and blood tests to help confirm the diagnosis.

  • Treatment focuses on relieving symptoms and can include diazepam (a sedative), baclofen (a muscle relaxant), corticosteroids, and plasma exchange.

Stiff-person syndrome is more common among women and often occurs in people with type 1 diabetes, certain autoimmune disorders, or certain kinds of cancer, including breast cancer, lung cancer, kidney cancer, thyroid cancer, colon cancer, and lymphomas.

The cause of stiff-person syndrome 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 about two thirds of people with stiff-person syndrome.

Sometimes the cause is unknown.

Symptoms

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.

Diagnosis

  • Electromyography

  • Blood tests

The diagnosis of stiff-person syndrome is suggested by symptoms. Tests are done to help confirm the diagnosis. They include electromyography and blood tests to detect the antibodies that are present in many people with stiff-person syndrome.

Treatment

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

  • Immune globulin

  • Sometimes corticosteroids

  • Sometimes plasma exchange

Treatment of stiff-person syndrome focuses on relieving symptoms. 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.