A Refractory Case of Sydenham Chorea Managed With Intravenous Pulse-Dose Methylprednisolone

TL;DR

A child with severe Sydenham's chorea improved after high-dose corticosteroid treatment when other therapies failed.

Contribution

Demonstrates the potential effectiveness of intravenous methylprednisolone in refractory Sydenham's chorea cases.

Findings

High-dose corticosteroids led to significant improvement in choreiform movements and emotional dysregulation.

Standard treatments like valproic acid and haloperidol were ineffective in managing the patient's symptoms.

Long-term penicillin prophylaxis was used to prevent recurrence of Sydenham's chorea.

Abstract

We describe the case of an eight-year-old female presenting with abrupt-onset involuntary movements, emotional lability, and gait disturbances, consistent with Sydenham's chorea (SC). Her condition deteriorated despite initial antibiotic treatment and symptomatic management. Notable findings included elevated antistreptolysin O titers and antideoxyribonuclease B antibodies, suggestive of recent Group A Streptococcus infection. Brain magnetic resonance imaging revealed punctate gliosis in the supratentorial white matter, and extensive workup excluded alternative diagnoses. Despite escalating therapy with valproic acid, clonidine, and haloperidol, the patient exhibited persistent choreiform movements and emotional dysregulation. High-dose corticosteroids (methylprednisolone) were initiated, resulting in significant symptomatic improvement and restoration of ambulatory function. Long-term prophylaxis with benzathine penicillin G was implemented to prevent recurrence. SC remains an under-researched complication of acute rheumatic fever, with treatment often extrapolated from limited case reports and expert consensus. This case underscores the potential role of corticosteroids in refractory SC. This case also highlights the complexity of managing prolonged SC and the importance of individualized, multifaceted treatment strategies.

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