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.
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.
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.
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.
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 |
WE MOVE (Worldwide Education & Awareness for Movement Disorders) 204 West 84th Street New York, NY 10024 wemove@wemove.org http://www.wemove.org Tel: 212-875-8312 Fax: 212-875-8389 |
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National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892
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Last updated February 14, 2007