RESUMO
BACKGROUND: Juvenile myoclonic epilepsy is a heterogeneous syndrome, both in genetic and clinical aspects. AIMS: This study was conducted to compare the efficacy of valproic acid in familial versus sporadic cases of this syndrome. SETTINGS AND DESIGN: Seventy patients with JME were identified; 24 patients (34.3%) had positive history of JME in their first degree relatives (group I) and 46 patients (65.7%) were sporadic (group II). MATERIALS AND METHODS: Valproic acid was started for the patients with upward titration. The cases were followed for one year after final titration of the drug with regular blood monitoring. Patients, who had no myoclonic, absence and grand mal seizures within one year, were considered excellent responders. STATISTICAL ANALYSIS: We used Student T-test and Fisher's exact test for quantitative and qualitative variables respectively. Logistic Regression test was used to evaluate the predictive factors for final treatment outcomes. RESULTS: Mean dosage of valproic acid was 800 mg/d in both groups (13 mg/kg and 12.4 mg/kg respectively). Mean therapeutic levels of the drug in group I and II were 74 microg/ml and 78.4 microg/ml respectively. Excellent responders' rate was 66.7% in group I and 76.1% in group II. History of absences and older age at the onset of grand mal seizures decreased excellent responders' rate in both groups. CONCLUSIONS: Considering response to valproic acid, there is no significant difference in familial versus sporadic cases of JME, whereas history of absences and older age at the onset of grandmal seizures, decrease the probability of being excellent responders in this syndrome.