Treatment of Sydenham’s Chorea: A Review of the Current Evidence

BACKGROUND Sydenham's chorea (SC), the neurologic manifestation of rheumatic fever, remains the most prevalent form of chorea in children. Suggested treatments of chorea in SC include prophylactic penicillin, symptomatic (antipsychotic and anticonvulsant) medications, and immunomodulatory therapy (steroids, intravenous immunoglobulin (IVIG), and plasma exchange). In this manuscript, we undertook a systematic review of the published literature to examine the data supporting these therapeutic recommendations. METHODS A search of PubMed, Embase, Psychinfo, and clinicaltrials.gov was conducted for publications pertaining to the treatment of SC/rheumatic chorea from 1956 to 2016. RESULTS Penicillin prophylaxis appears to reduce the likelihood of further cardiac complications and the recurrence rate of chorea. Data on symptomatic therapy for chorea are limited to individual case reports or series and rare comparison studies. The efficacy of steroid use is supported by a single placebo-controlled study and several case series. Information on other immunomodulatory therapies such as IVIG and plasmapheresis are limited to a small number of reports and a single comparison study. DISCUSSION Treatment decisions in SC are currently based on the treating physician's clinical experience, the desire to avoid side effects, and the existence of only limited scientific evidence. Based on a review of the available literature, chorea often improves with symptomatic therapy and immunotherapy tends to be reserved for those who fail to respond. Steroids are beneficial; however, data using IVIG and plasmapheresis are very limited. Larger, well-controlled studies, using standardized assessment scales, are required if therapeutic decisions for SC are to be based on meaningful information.