Sydenham chorea (SC), the principal neurological manifestation of Rheumatic Fever (RF), is characterized by a state consisting of abrupt, involuntary not rhythmic or repetitive movements(1). A variety of drugs have been advocated for treatment of sc. We describe our experience with haloperidol and valproate in SC.

Subjects and Methods

We carried out a retrospective analysis of the medical records of 65 children suffering from RF with isolated or associated SC, from 1990 to 1995, followed-up at the Pediatric Rheumatology Outpatient Clinic. Nineteen of these patients came from the first care unit to our outpatient clinic and had been taking haloperidol for up to 3 days) The 46 remaining patients were included consecutively in the study. The diagnostic criterion of SC was clinical, after excluding other diseases. In addition to the choreic movements, uni or bilateral, we took into account the presence of emotional imbalance, motor problems and speech disturbance. The patients were clinically evaluated by both a pediatric neurologist and rheumatologist. Haloperidol at an initial dose of 1 or 2 mg/ day up to 5 mg/ day or sodium valproate at an initial dose of 20 mg/kg/ day up to 40 mg/kg/ day were used regardless of the severity of the disease. The patients were revaluated within a period of an average, 72 hours. In this revaluation, we maintained the dose in the cases that showed improvement, or we increased the dose gradually until a favorable clinical response was obtained. To monitor the hepatotoxicity due to the use of valproate, each. patient was periodically evaluated clinically and also by estimation of asparate transaminase (AST) and alanine transaminase (ALT). All patients were evaluated following the same protocol which covered the initial dose, dose modifications, and maximum dosage; lagtime for improvement of the symptoms; side- effects; lack of response to medication; and treatment duration. Significant improve- ment was defined by the disappearance of spontaneous movements, incoordination and weakness. All the children received secondary prophylaxis with benzathine penicillin every 21 days. The patients did not receive steroids during this study.

Results

Of the 65 children who took part in the study, 45 (69.3%) were females and 20 (30.7%) were males and 33 (50.7%) caucasoid and 32 (49.3%) non-caucasoid. The age at which symptoms began ranged from 5to 14 years (mean = 9.7 years). Ten children presented with recurrences, that took place at least 10 months after evolution without medication, making a total of 75 chorea episodes evaluated. Among the principal clinical manifestations we would draw attention to generalized chorea in the majority of cases (72%). Hemichorea was observed in 21 episodes (28%), 14 on the left and 7 on the right. Slurred speech (77.9%), muscular weakness (71.4%), gait disturbance (70.1%), and emotional imbalance (62.7%) were also found. Haloperidol was used in 47 episodes (62.7%) and in 8 (17%) of these a change to sodium valproate was necessary (5 because of side effects and 3 due to poor response! after three or four weeks of a daily dose of 5 mg of the medication). Valproate was used in 28 episodes (37.3%), in 3 of which (10.7%) it was necessary to change to haloperidol due to lack of response (Table 1). The evident control of choreic movements took place, on an average, 14 days after the introduction of haloperidol and 10 days after the introduction of valproate. The duration of treatment was from 1 to 20 months (mean = 3.5 months) for haloperidol and from 2 to 21 months (mean=3 months) for valproate. The follow-up time varied from 2 to 72 months (mean=24.7 months) for haloperidol and from 2 to 60 months (mean 18.6 months) for valproate (Table I). In 9 patients (8 with haloperidol and 1 with valproate), the reduction of dosage or withdrawal of the drug became necessary because of side effects (Table II).