Carbamazepine has been used successfully in the treatment of different move
ment disorders and was recently reported to be effective for nonhereditary
chorea, In view of the significant side effects associated with the drugs c
urrently used to treat chorea, we sought to further evaluate the efficacy o
f carbamazepine in children with rheumatic chorea. The study was prospectiv
e and included 10 children with chorea (eight females and two males; age ra
nge = 7-16 years) referred to our Pediatric Rheumatology Clinic between 199
5 and 1999, Nine had rheumatic fever and one had antiphospholipid antibody
syndrome that later evolved to systemic lupus erythematosus, All were treat
ed with carbamazepine. Improvement was evident within 2-14 days of initiati
on of low doses of carbamazepine (4-10 mg/kg daily). The plasma drug levels
were 2.8-8.2 mu g/mL (therapeutic antiepileptic range = 8-12 mu g/mL). The
chorea disappeared within 2-12 weeks. The duration of treatment was 1-15 m
onths. No side effects were observed. Recurrence was observed in three pati
ents who received a second trial of carbamazepine with a good response. We
suggest that carbamazepine may serve as a first-lint treatment for rheumati
c chorea. (C) 2000 by Elsevier Science Inc. All rights reserved.