Abstracts

Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epileptic syndrome with age-related onset. It is characterized by myoclonic jerks on awakening, generalized tonic-clonic seizures (GTCS) and absence seizures. Myoclonic jerks, the hallmark of the syndrome, are often omitted or forgotten by the patients who only seek for medical assistance after the first GTCS. The ideal treatment consists of valproate (VPA) associated with avoidance of precipitant factors (APF) which is considered effective in about 90% of the cases. We retrospectively assessed the response to VPA in 50 patients who had neither had correct syndromic diagnosis nor seizure control.
Fifty patients were included, 30 men, aged between 12 to 52 years, evaluated as outpatients between 1998 and 2003. We estimated the number of GTCS ([gt] or [lt] 20) during the disease. Monotherapy with VPA was tried in all patients. They were considered seizure-free if they hadn[rsquo]t had any seizure for at least one year.
These patients had been unsuccessfully treated with phenobarbital/primidone, carbamazepine/oxcarbazepine, phenytoin, clonazepam or other benzodiazepines, lamotrigine or acetazolamide in mono or polytherapy. The age of first seizure varied from 7 to 24 years (mean 13.8 [plusmn] 3.7) and the age at the diagnosis of JME after the onset the symptoms from 1 to 33 years (mean 10.3 [plusmn] 7.6). Institution of VPA+APF led to seizure control in 39 patients (78%). In these patients, the duration of the disease varied from 2 to 33 years (mean 8.9 [plusmn] 6.6) and the number of GTCS was [lt] 20 in 15 patients (38.4%) and [gt]20 in 24 (61.5%). Seizure control was reached within 1 month of therapy in 13 (33.3%), from 1- 6 months in 22 (56.4%) and above 6 months in 4. Of the 11 patients (22%) who had persisting seizures despite VPA+ APF the duration of the disease varied from 2 to 23 years (13.7 [plusmn] 8.3); 4 patients (36.3%) presented [lt] 20 and 7 (63.6%) [gt] 20 GTCS. Factors possibly related with persistence of seizures included: lack of compliance (8), abnormal lifestyle (10) insufficient treatment (1) and intolerable side effects (1).
Little is known about the factors involved in JME response to VPA+APF. In this series the duration of the disease and number of GTCS did not influence the response to treatment even when the diagnosis was established about one decade after seizure onset and use of other therapeutic schemes. Despite these factors, in 1/3 of the patients control of the seizures was reached in less than one month and in half of them within 6 months. About 20% of the patients in this series presented pseudoresistant and possibly truly resistant JME.
[Supported by: Funda[ccedil][atilde]o de Amparo [agrave] Pesquisa do Estado de S[atilde]o Paulo.]