Abstracts

Although considered a common epileptic syndrome, corresponding to 2.8-11.9% of all epilepsies and presenting distinct clinical characteristics, Juvenile Myoclonic Epilepsy (JME) is still not well diagnosed, a fact that may bring about important deleterious consequences. Valproate (VPA) is considered the drug of choice in control of the seizures in this syndrome. The aim of this study was to characterize the factors implied in the delay of diagnosis and the response after adequate therapeutic institution in JME patients. A series of 41 JME patients has been attended to by two MD in our outpatient clinic since October 2000 and continues to be so up to now. We analyzed the initial diagnosis, the delay and the factors implied in it as well as the prognosis after establishment of adequate treatment since most of the patients were receiving antiepileptic drugs (AED) other than VPA. At the time of admission only 8 out of the 41 patients (19.5%) had had syndromic diagnosis while 33 (80.5%) had not yet had the diagnosis of JME being more frequently labeled as indeterminate epilepsy. The diagnosis was established in a mean of 8.2 yr. (15 days to 34 yr.) after clinical onset. The electroclinical data of these patients agree with the classical description of this syndrome as follows: the age of onset of the epilepsy varied between 7 and 24 yr, presence of myoclonic seizures in all patients, generalized tonic-clonic seizures also present in 92.7% and absences in 43.9%. Only 4.9% had only myoclonic seizures. The main factors identified in the delay of diagnosis were: omission in 4 (9.7%) and asymmetry in 12 (29.3%) of the myoclonic jerks; normal first EEGs at the time of the institution of drug therapy (41%); some normal EEGs in the series of recordings in 73.2%; presence of focal abnormalities in the EEGs in 35.9% and asymmetry of the generalized paroxysms in 33%. The mean in years for the establishment of the diagnosis was 11.6 for the group with asymmetric compared to 8.5 in those with symmetric paroxysms. The institution of treatment with VPA associated with avoidance of precipitant factors (APF) led to seizure control in 92.5% of all patients in the first year. This rate dropped to 41.3% in the third year of follow-up. The main factor that implied in this drop was non-compliance. JME continues to be misdiagnosed and the response to VPA + APF in one year is excellent suggesting pharmacosensitivity although it has not continued to do so over the years. Despite all the instructions it is very difficult for the JME patients to rigorously follow them over the years. (Supported by FAPESP (Funda[ccedil][atilde]o de Amparo a Pesquisa do Estado de S[atilde]o Paulo), CAPES (Coordena[ccedil][atilde]o de Aperfei[ccedil]oamento de Pessoal de N[iacute]vel Superior))