The history of juvenile myoclonic epilepsy

Abstract

Juvenile myoclonic epilepsy (JME) has been the subject of intensive research over the past 25years. It was discovered stepwise in Switzerland and France in the 19th century, adequately described in Germany and Uruguay in the 1950s, and rediscovered in North America in the early 1980s. Juvenile myoclonic epilepsy represents the most common idiopathic epilepsy syndrome. As a tribute to the primary author of the first extensive and detailed clinical description of JME, it is also called the Janz syndrome. Juvenile myoclonic epilepsy is an archetypical epileptic syndrome, with a fairly homogenous presentation and a still largely unknown etiology. Its clinical spectrum now includes cognitive and psychiatric symptoms as significant copathologies, and the elucidation of its probably multiple genetic mechanisms is an ongoing process. Juvenile myoclonic epilepsy may not qualify as a "benign" epilepsy, but seizures in most patients can be managed adequately and patients will not suffer severe limitations in their lifetime expectations.