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  Stiff-Person Syndrome 
  
Reviewed  05-09-2002  

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What is Stiff-Person Syndrome?
Is there any treatment?
What is the prognosis?
What research is being done?

Organizations

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

Select this link to view a list of studies currently seeking patients.

 Organizations

National Rehabilitation Information Center (NARIC)
4200 Forbes Boulevard
Suite 202
Lanham, MD 20706-4829
naricinfo@heitechservices.com
http://www.naric.com
Tel: 301-562-2400 800-346-2742
Fax: 301-562-2401

National Organization for Rare Disorders (NORD)
P.O. Box 1968
(55 Kenosia Avenue)
Danbury, CT 06813-1968
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
National Institutes of Health
Bldg. 31, Rm. 4C05
Bethesda, MD 20892-2350
NIAMSInfo@mail.nih.gov
http://www.nih.gov/niams
Tel: 301-496-8188 877-22-NIAMS (226-4267)


NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.


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




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