Sydenham’s chorea: from pathophysiology to therapeutics

ABSTRACT

Sydenham’s chorea is an autoimmune chorea emerging after a group A beta-hemolytic streptococcal (GABHS) infection, i.e. a rheumatic chorea with or without the presence of carditis or arthritis. The disorder, defined by the presence of chorea, is also associated with cognitive and behavioral symptoms, including emotional lability, anxiety, depressive and obsessive-compulsive symptoms. The authors review the pathophysiology, clinical characteristics, and available evidence on therapeutic strategies, the latter including the secondary prevention of GABHS infections, reduction of chorea, and immune modulation. Sydenham’s chorea has been regarded as a model for pediatric autoimmune neuropsychiatric disorders, however, the field is marked by conflicting results and controversies. Regarding therapeutics, there are limited high-quality interventional studies and the selection of treatment strategy often relies on the clinician’s experience. A serial treatment algorithm is presented based upon the severity of clinical presentation and response to symptomatic pharmacotherapy.

KEYWORDS:

• Sydenham’s chorea
• rheumatic chorea
• rheumatic fever
• anti-basal ganglia antibodies
• pandas
• treatment
• penicillin
• dopamine antagonists
• corticosteroids

Article highlights

• Sydenham’s chorea is defined by the acute onset of an involuntary, random-appearing sequence of one or more discrete involuntary movements or movement fragments (i.e. chorea) as well as cognitive and behavioral symptoms.

• Recognizing its epidemiologic association with GABHS infections this disorder has often been cited as a model for immune-mediated neuropsychiatric disorders in childhood.

• Although classically seen as a self-limited condition, growing evidence has shown persistence and/or recurrence of motor and neuropsychiatric symptoms in adulthood.

• Dopamine antagonists and anti-epileptic drugs are the most commonly used symptomatic therapies in Sydenham’s chorea.

• Immunomodulatory strategies are reserved for selected patients with Sydenham’s chorea.

Declaration of interest

The Neuropsychiatry Program is funded by the UT Health Houston Department of Psychiatry and Behavioral Sciences. MCPN and ALT have Brazilian CNPq research scholarships. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or conflict with the subject matter or materials discussed in this manuscript apart from those disclosed.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.