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Description

Sydenham's chorea is a disorder affecting children and characterized by jerky, uncontrollable movements of the muscles of the face, the arms and legs and the trunk. Other symptoms of the disorder may include diminished muscle tone, muscle weakness, and emotional and behavioural disturbances, particularly obsessive-compulsive behaviours. The symptoms vary in severity, from mild cases in which there is facial grimacing, restlessness and slight incoordination to severe cases where involuntary movements make it impossible for the child to function normally. Choreic movements usually begin gradually, progressively worsening over a few weeks to a month.

It most often appears in children between the ages of 5 and 15, and is more common in girls than in boys, following an attack of rheumatic fever (a streptococcal infection) ? up to 6 months after the fever has cleared. It is thought to result from an autoimmune reaction to the streptococcal infection.

It is now a relatively rare disorder, probably due to the routine use of antibiotics to treat infections in children. It may occur in about 25% of children who have rheumatic fever.


Treatment

There is no specific treatment for Sydenham's chorea and symptoms usually resolve themselves in approximately 3 to 6 months. Bed rest, sedatives and medication to control movements may be prescribed. Penicillin prophylaxis may also be prescribed to avoid further streptococcal infection.

In some instances, there may be residual signs of chorea and behavioural abnormalities, which may wax and wane over a year or more.


Prognosis

Generally the prognosis for children with Sydenham's chorea is good. Complete recovery usually occurs, often within weeks of the onset.

In about 20 percent of patients, the disorder may recur, usually within 2 years of the initial occurrence. Recurrences have also been reported during pregnancy in women who had ARF during childhood.

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