Evaluation, Diagnosis, and Treatment of Sydenham Chorea: Consensus Guidelines

Abstract

An international panel of 27 experts (pediatric and movement disorder neurologists, psychiatrists, and parent representatives) from all continents participated in a Delphi process to establish international consensus guidelines for the evaluation, diagnosis, and management of children with Sydenham chorea (SC) based on best evidence and expert opinion. In total, 88 recommendations reached consensus. Practitioners should identify key signs of SC (chorea and hypotonia), screen for behavioral, mobility, swallowing, speech, and cognitive impairments, and acute rheumatic fever (ARF) features including carditis. Etiological evaluation will differ according to population ARF risk. At all times, patients, families, and educators should receive support, information, and guidance to minimize the impact of SC on academic and social functioning. Antibiotic treatment is recommended at first presentation. Long-term secondary antibiotic prophylaxis should follow international or local guidelines, and measures to reduce pain and distress associated with intramuscular antibiotics will aid in adherence. Immunotherapy (corticosteroids) is recommended in moderate to severe SC. In those with inadequate recovery, intravenous immunoglobulin or plasma exchange should be given. In SC relapse, repeat clinical assessments, etiological investigation, and antibiotics plus corticosteroid therapy should be considered. This consensus guideline will standardize the evaluation and management of patients with SC and direct future research to improve the lived experience and outcomes of patients and families.