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What is Stiff-Person Syndrome?

Stiff-person syndrome is a rare progressive neurological disorder characterized by constant painful contractions and spasms of voluntary muscles, particularly the muscles of the back and upper legs. Symptoms may occur gradually, spreading from the back and legs to involve the arms and neck. Symptoms may worsen when the affected individual is anxious or exposed to sudden motion or noise. Affected muscles may become twisted and contracted, resulting in bone fractures in the most severe cases. Individuals with stiff-person syndrome may have difficulty making sudden movements and may have a stiff-legged, unsteady gait. Sleep usually suppresses frequency of contractions. Stiffness may increase and patients may develop a hunched posture (kyphosis) or a swayback (lordosis). Researchers theorize that stiff-person syndrome may be an autoimmune disorder. Other autoimmune disorders such as diabetes, pernicious anemia (a chronic, progressive blood disorder), and thyroiditis (inflammation of the thyroid gland) may occur more frequently in patients with stiff-person syndrome.

Is there any treatment for Stiff-Person Syndrome?

The drug diazepam, which relaxes the muscles, provides improvement in most cases. Baclofen, phenytoin, clonidine, or tizanidine may provide additional benefit. In some patients, immunomodulatory treatments such as intravenous immunoglobulin may be beneficial. Physical and rehabilitation therapy may also be needed.

What is the prognosis for Stiff-Person Syndrome?

There is no cure for stiff-person syndrome. The long-term prognosis for individuals with stiff-person syndrome is uncertain. Management of the disorder with drug therapy may provide significant improvements and relief of symptoms.

What research is being done concerning Stiff-Person Syndrome?

Research on stiff-person syndrome is aimed at enhancing scientific understanding of the disorder and evaluating new therapeutic interventions.

Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892

Updated 05-09-2002  

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