Sydenham chorea

Sydenham chorea is a disabling pediatric hyperkinetic and neuropsychiatric disorder following streptococcal infection. Sydenham chorea occurs in approximately 10% of acute rheumatic fever and is one of its major manifestations. The disease may last for weeks or months, with a high risk of recurrence. The common features of rheumatic fever include sore throats due to streptococcal infections, rashes, pains in the limbs, and subcutaneous nodules, and this form of chorea typically presents, after varying intervals, with twitching of the mouth and limbs, shrugging of the shoulders, and clumsy gait. Those with this form of chorea are less likely to have severe cardiac or rheumatologic complications. The pathogenesis has been considered to be an autoantibody-mediated basal ganglia dysfunction. Anti-inflammatory treatments such as steroids, plasmapheresis and intravenous immunoglobulin treatment are effective.