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Contemporary approaches to Sydenham's chorea - where evidence meets reality
Andrea Santangelo1,2, Maria Cristina Maggio1, Alessandro Orsini3
1Department of Pediatrics, G. Di Cristina Children Hospital, Palermo, Italy.
Introduction:
Sydenham's chorea (SC) is the most common acquired chorea of childhood and a major neurological manifestation of acute rheumatic fever. Although often considered self-limiting, it can cause substantial motor, psychiatric, cognitive, educational, and social morbidity, with relapses in a relevant proportion of patients.
Areas Covered:
In this critical perspective, the authors critically examined current approaches to SC management, focusing on three complementary pillars: eradication of Group A β-hemolytic streptococcal infection and long-term secondary antibiotic prophylaxis; symptomatic control; and immunomodulation. Antibiotic prophylaxis is essential to prevent recurrent rheumatic fever, rheumatic heart disease progression, and neurological relapse, but does not treat the established autoimmune episode. Anti-seizure medicaments are generally preferred for symptomatic treatment because of their favorable efficacy-tolerability balance, while dopamine receptor antagonists should be reserved for severe hyperkinesia because of drug-induced movement disorders. Recent evidence supports earlier use of corticosteroids in moderate-to-severe or disabling SC, whereas intravenous immunoglobulin and plasma exchange remain escalation options for selected severe or steroid-resistant cases.
Expert Opinion:
Treatment response should be assessed by functional recovery, not motor improvement alone. Future progress will depend on prospective registries, standardized outcome measures, pragmatic multicenter trials, and biomarker studies to enable more individualized therapeutic decisions. Systematic neuropsychiatric assessment and multidisciplinary support are therefore integral to comprehensive care.
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