Sydenham’s chorea: an update on pathophysiology, clinical features and management

Abstract

ABSTRACT Introduction: Sydenham’s chorea is an autoimmune hyperkinetic movement disorder that emerges after a group A beta-hemolytic streptococcal (GABHS) pharyngitis. It is the neurological manifestation of acute rheumatic fever. Low quality studies are the current reference for the symptomatic and/or immunomodulatory treatment of Sydenham’s chorea. Areas covered: This review contemplates the clinical features and pathophysiological aspects of Sydenham’s chorea focusing on their implications for therapeutics. It also provides an updated perspective on treatment based on antipsychotics, corticosteroids and other immunomodulatory strategies. Expert opinion: Therapeutic options for Sydenham’s chorea are still largely empirical. Based on the available evidence on the natural history of Sydenham’s chorea and other immune-mediated movement disorders, an early anti-inflammatory or immune-based approach could prevent and/or attenuate chorea, cognitive and behavioral dysfunction. A treatment algorithm for Sydenham’s chorea is proposed.