Sydenham’s chorea, an autoimmune movement disorder caused by Streptococcus-induced antibodies that cross-react with basal ganglia antigens, is the most common cause of acute chorea in children worldwide. The clinical features include chorea and other motor findings as well as non-motor features such as obsessions, compulsions, and hyperactivity. The management is based on control of chorea and prophylaxis of new streptococcal infections. Although there are no evidence-based recommendations, valproic acid and other anticonvulsant agents are the first-line antichoreic agents. Risperidone and other neuroleptics are used for patients who fail to improve with anticonvulsant agents. If patients do not respond to the first- and second-line agents, they should be treated with corticosteroids. Prophylaxis with antibiotics is warranted to prevent recurrence of rheumatic fever.
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