Sydenham's chorea - analysis of 27 patients and a review of the literature

The first documented epidemic of Sydenham's chorea was in 1418. In 1686 Thomas Sydenham was the first to describe the condition, but it was Richard Bright who in 1831 first made the association between chorea and rheumatic fever. Chorea is one of the major diagnostic criteria for rheumatic fever. Chorea is often considered a benign self-limiting condition. Little is known about the pathophysiological process. A link between the group A β-haemolytic streptococcus through an antibody-mediated immune response targeting the basal ganglia has been described. Special investigations have been done to ascertain the pathophysiology, but none is of diagnostic value. The aims of this study were to analyse the clinical findings, the role of special investigations in the management and course of Sydenham's chorea and to review the literature. Data on 27 patients were analysed. Special investigations in these patients can be divided into those necessary to assist in diagnosing acute rheumatic fever and those to exclude other causes that mimic chorea. None of these tests was helpful in diagnosing chorea due to other causes; neither did they influence the management of these patients. In conclusion, Sydenham's chorea remains a clinical diagnosis and extensive and expensive special investigations are seldom warranted.