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Corticosteroid treatment in Sydenham's chorea
1Department of Pediatrics, Child Neurology and Psychiatry Unit, Arcispedale Santa Maria Nuova, IRCCS, Viale Risorgimento 80, 42123 Reggio Emilia, Italy.
Summary
Corticosteroid therapy, including IV methyl-prednisolone and oral deflazacort, shows promise for treating Sydenham's chorea (SC), a movement disorder following streptococcal infections.
Area of Science:
- Pediatric Neurology
- Immunology
- Rheumatology
Background:
- Sydenham's chorea (SC) is an immune-mediated hyperkinetic movement disorder triggered by Group A Beta-hemolytic streptococcal (GABHS) infections.
- Conventional treatments for SC include symptomatic therapies like carbamazepine, valproate, and neuroleptics.
- Corticosteroid use is considered for severe or drug-resistant SC cases, or when first-line therapies cause disabling side effects.
Observation:
- A case series of 5 children with SC managed at a Child Neurology Unit.
- Diverse clinical presentations of SC were observed, including chorea paralytica, distal chorea, hemichorea, classic chorea, and associated mood disorders or dyspraxia.
- Patients received either intravenous methyl-prednisolone followed by oral deflazacort (severe cases) or oral deflazacort alone (mild to moderate cases).
Findings:
- Corticosteroid therapy demonstrated effectiveness in both short-term and long-term management of Sydenham's chorea.
- The proposed corticosteroid regimen was well-tolerated across various clinical SC presentations.
- No significant adverse side effects were recorded during the observation period.
Implications:
- Corticosteroid therapy, specifically IV methyl-prednisolone and oral deflazacort, presents a viable treatment option for children with Sydenham's chorea.
- This approach offers a well-tolerated and effective alternative for managing SC, particularly in severe or refractory cases.
- Further research into corticosteroid efficacy and safety in pediatric movement disorders is warranted.