[Juvenile myoclonic epilepsy]

In this paper we review the history of juvenile myoclonic epilepsy (JME) since the description by Herpin in 1852 and 1857 of the seizures of some epileptic patients which he called impulsions . Credit is due to Janz and Christian who in 1957 gave a detailed description of the condition which they called impulsive Petit mal and we know today as JME. Delgado-Escueta and his group in Los Angeles discovered the genetic basis of JME in some patients.

The homogeneity of the clinical and neurophysiological characteristics of series of patients with JME reported from many different parts of the work is striking. Diagnosis of JME is easy when the syndrome is known. However, in some patients a broad differential diagnosis should be considered as discussed in this article. The treatment of choice is still valproic acid as monotherapy. When this is not effective or side-effects occur, other useful drugs are available such as primadone. Of the other new antiepileptic drugs lamotrigine has been shown to be effective and good results have been obtained with topiramate, so both these drugs should be considered in some patients. Although response to treatment is excellent, a high incidence of relapse was seen in all studies when the patients stopped their treatment.