Managing and treating Sydenham chorea: A systematic review

Brain Behav. 2023 Jun;13(6):e3035. doi: 10.1002/brb3.3035. Epub 2023 May 7.


Introduction: Sydenham's chorea (SC), prevalent in developing countries and occasionally affecting developed ones, poses a clinical challenge due to the lack of systematic guidelines for diagnosis and treatment. Resulting from Group A Beta-Hemolytic Streptococcus infection, SC presents various symptoms. This review aims to collect and evaluate available data on SC management to propose a cohesive treatment plan.

Methods: We searched PubMed, the Cochrane Library, Google Scholar, and for literature on SC management from inception until 24th July 2022. Studies were screened by titles and abstracts. Cochrane Collaboration's Risk of Bias tool (RoB-1) assessed Randomized Controlled Trials, while the Risk of Bias In Non-randomized Studies of Interventions (ROBINS-I) tool evaluated nonrandomized studies.

Results: The review includes 11 articles assessing 579 patients. Excluding one study with 229 patients, of the remaining 550 patients, 338 (61.5%) were females. Treatments used were dopamine antagonists in 118 patients, antiepileptics in 198, corticosteroids in 134, IVIG in 7, and PE in 8 patients. Dopamine antagonists, particularly haloperidol, were the primary treatment choice, while valproic acid (VPA) was favored among antiepileptics. Prednisolone, a corticosteroid, showed promising results with weight gain as the only side-effect. Our review emphasizes the importance of immunomodulators in SC, contrasting previous literature.

Conclusion: Despite limitations, dopamine antagonists can serve as first-line agents in SC management, followed by antiepileptics. The role of immunomodulators warrants further investigation for conclusive recommendations.

Keywords: Sydenham's chorea; acute rheumatic fever; gamma-aminobutyric acid; group a beta-hemolytic Streptococcus.

Publication types

  • Systematic Review
  • Review

MeSH terms

  • Anticonvulsants / therapeutic use
  • Chorea* / diagnosis
  • Chorea* / drug therapy
  • Dopamine Antagonists
  • Female
  • Haloperidol
  • Humans
  • Male
  • Valproic Acid / therapeutic use


  • Anticonvulsants
  • Valproic Acid
  • Haloperidol
  • Dopamine Antagonists