Sydenham chorea
Sydenham chorea is an autoimmune movement and neuropsychiatric disorder that is triggered by Streptococcus pyogenes (group A streptococcus) infection. Only treat Sydenham chorea when it is sufficiently disabling to interfere with normal daily activities. Antiepileptic drugs are effective, but a response may not be seen for 1 to 2 weeks after starting therapy. Use:
1 carbamazepine (preferably modified-release) 5 to 10 mg/kg (adult and child) orally, twice daily (up to 1200 mg daily) until 2 to 4 weeks after the chorea subsides1 Sydenham chorea carbamazepine
OR
1 sodium valproate 7.5 to 12.5 mg/kg (adult and child) orally, twice daily (up to 2500 mg daily) until 2 to 4 weeks after the chorea subsides. Avoid in females of childbearing potential (see teratogenic and neurodevelopmental effects of antiepileptic drugs)2. Sydenham chorea sodium valproate
Limited evidence suggests that immunosuppression with a corticosteroid or intravenous immunoglobulin can improve the extent or speed of recovery. Consider this therapy in patients with moderate to severe chorea. Continue antiepileptic drugs until immunotherapy takes effect.
All patients with Sydenham chorea must have long-term antibiotic prophylaxis against Streptococcus pyogenes infection.
Children whose antipsychotic drugs are withdrawn suddenly can develop generalised choreiform movements that resemble Sydenham chorea. Treat this ‘withdrawal emergent syndrome’ by restarting the antipsychotic drug and slowly tapering the dosage.