Skip secondary menu

NINDS Sydenham Chorea Information Page

Synonym(s):   Saint Vitus Dance, Rheumatic Encephalitis

Table of Contents (click to jump to sections)


Listen to this page using ReadSpeaker


What is Sydenham Chorea?

Sydenham chorea (SD) is a neurological disorder of childhood resulting from infection via Group A beta-hemolytic streptococcus (GABHS), the bacterium that causes rheumatic fever.  SD is characterized by rapid, irregular, and aimless involuntary movements of the arms and legs, trunk, and facial muscles.  It affects girls more often than boys and typically occurs between 5 and 15 years of age. Some children will have a sore throat several weeks before the symptoms begin, but the disorder can also strike up to 6 months after the fever or infection has cleared.  Symptoms can appear gradually or all at once, and also may include uncoordinated movements, muscular weakness, stumbling and falling, slurred speech, difficulty concentrating and writing, and emotional instability.  The symptoms of SD can vary from a halting gait and slight grimacing to involuntary movements that are frequent and severe enough to be incapacitating.  The random, writhing movements of chorea are caused by an auto-immune reaction to the bacterium that interferes with the normal function of a part of the brain (the basal ganglia) that controls motor movements.  Due to better sanitary conditions and the use of antibiotics to treat streptococcal infections, rheumatic fever, and consequently SD, are rare in North America and Europe.  The disease can still be found in developing nations. 

Is there any treatment?

There is no specific treatment for SD.  For people with the mildest form, bed rest during the period of active movements is sufficient.  When the severity of movements interferes with rest, sedative drugs, such as barbiturates or benzodiazepines, may be needed.  Antiepileptic medications, such as valproic acid, are often prescribed.  Doctors also recommend that children who have had SD take penicillin over the course of the next 10 years to prevent additional manifestations of rheumatic fever. 

What is the prognosis?

Most children recover completely from SD, although a small number will continue to have disabling, persistent chorea despite treatment.   The duration of symptoms varies, generally from 3 to 6 weeks, but some children will have symptoms for several months.  Cardiac complications may occur in a small minority of children, usually in the form of endocarditis.  In a third of the children with the disease, SD will recur, typically 1 ½ to 2 ½ years after the initial attack.  Researchers have noted an association between recurrent SD and the later development of the abrupt onset forms of obsessive-compulsive disorder, attention deficit/hyperactivity disorder, tic disorders, and autism, which they call PANDAS, for Pediatric Autoimmune Neuropsychiatric Disorders Associated with Streptococcus infection.  Further studies are needed to determine the nature of the association and the biological pathways that connect streptococcal infection, autoimmune response, and the later development of these specific behavioral disorders.

What research is being done?

The National Institute of Neurological Disorders and Stroke (NINDS) and other institutes of the National Institutes of Health (NIH) conduct research related to SD in laboratories at the NIH, and support additional research through grants to major medical institutions across the country.  Currently, researchers are studying how the interplay of genetic, developmental, and environmental factors could determine a child’s vulnerability to SD after a GABHS infection.  Other researchers are exploring whether children whose symptoms either begin or get worse following a GABHS infection share a common set of abnormal biomolecular pathways responsible for their similar clinical symptoms. 

NIH Patient Recruitment for Sydenham Chorea Clinical Trials

Organizations

Column1 Column2
National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT   06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

 


Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



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.

Last updated February 14, 2007