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

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.

KEYWORDS:

• Sydenham chorea
• pathophysiology
• clinical features
• antibiotic therapy
• symptomatic therapy
• immunomodulatory therapy
• treatment update

Article highlights

• Sydenham’s chorea (SC) is classically recognized as a self-limited condition, but persistent and/or recurrent chorea are not uncommon outcomes.

• Besides chorea, behavioral and cognitive symptoms may be responsible for the negative impact of SC on social and occupational functioning. These symptoms may persist in adulthood even in the absence of chorea.

• Antibiotic prophylaxis aims to avoid GABHS reinfections and, therefore, cardiac complications and chorea recurrences.

• Neuroleptics and anti-epileptic drugs are the most prescribed symptomatic anti-chorea drugs in SC.

• Immunomodulatory therapies are usually reserved for patients whose symptomatic therapy was not effective. There is some evidence that their early use could prevent recurrences or persistence of symptoms.

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Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

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

Additional information

Funding

This study was partly supported by CNPq, Brazil. The Neuropsychiatry Program is funded by the UT Health Houston Department of Psychiatry and Behavioral Sciences.