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Articles » NINDS Sydenham Chorea Information Page: NINDS
 

NINDS Sydenham Chorea Information Page: NINDS

Article title: NINDS Sydenham Chorea Information Page: NINDS

Main condition: Sydenham chorea

Conditions: Sydenham chorea, rheumatic fever


What is Sydenham Chorea?
Sydenham chorea, also called St. Vitus dance, is a childhood movement disorder characterized by rapid, irregular, aimless, involuntary movements of the muscles of the limbs, face, and trunk. The disorder, which is considered a manifestation of rheumatic fever (streptococcal infection), typically has an onset between the ages of 5 and 15. Girls are affected more often than boys. The symptoms may appear gradually or suddenly, and may include muscle weakness, hypotonia (decreased muscle tone), and clumsiness. The symptoms vary in severity--from mild cases in which there is restlessness, facial grimacing, and a slight degree of incoordination of movements, to severe cases involving involuntary movements that incapacitate the child. The disorder may strike up to 6 months after the fever or infection has cleared. The chorea is believed to result from an autoimmune mechanism that occurs when the streptococcal infection causes the body to make antibodies to specific brain regions.

Is there any treatment?
There is no specific treatment for Sydenham chorea. Treatment is symptomatic and may include bed rest, sedatives, and the drug diazepam for controlling movements. Penicillin may also be prescribed for treatment of the fever or infection. Penicillin prophylaxis is often prescribed to avoid further infections with streptococcal bacteria.

What is the prognosis?
Generally the prognosis for patients with Sydenham chorea is good, and complete recovery often occurs. The duration of the disorder varies, with the average case lasting 3 to 6 weeks. Occasionally the course may be prolonged for several months.

What research is being done?
The NINDS supports research on movement disorders such as Sydenham chorea. The goals of this research are to increase understanding of these disorders and to find ways to prevent them.

Selected references

Swedo, S, et. al.
Sydenham's Chorea: Physical and Psychological Symptoms of St. Vitus Dance. Pediatrics, 91:4; 706-713 (April 1993).

Berkow, R (ed).
The Merck Manual of Diagnosis and Therapy. vol. II, 16th edition, Merck & Co., Inc., Rahway, NJ, pp. 678-679 (1992).

Thoene, J (ed).
Physicians' Guide to Rare Diseases. Dowden Publishing Co., Inc., Montvale, NJ, p. 443 (1992).

Bradley, W, et al (eds).
Neurology in Clinical Practice: Principles of Diagnosis and Management. vol. II, Butterworth-Heinemann, Boston, p. 1584 (1991).

Dajer, T.
Saint Vitus's Dance. Discover, pp. 86, 88, 90 (March 1990).

Joynt, R (ed).
Clinical Neurology. vol. 3, Chapter 38, J.B. Lippincott Co., Philadelphia, pp. 67-69 (1990).

Shannon, K, and Fenichel, G.
Pimozide Treatment of Sydenham's Chorea. Neurology, 50; 186 (1990).

 Organizations

National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield, CT 06812-8923
orphan@rarediseases.org
http://www.rarediseases.org/
Tel: 203-746-6518 800-999-NORD (6673)
Fax: 203-746-6481

This fact sheet is in the public domain. You may copy it.Provided by:
The National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892


 

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