DO PATIENTS WITH IDIOPATHIC GENERALIZED EPILEPSIES REQUIRE LIFELONG AED TREATMENT? – NO Martin Holtkamp Epilepsy-Center Berlin-Brandenburg, Department of Neurology, Charité – Universitätsmedizin Berlin, Germany
The International League Against Epilepsy (ILAE) recognizes different subtypes of idiopathic generalized epilepsy (IGE), four of which may persist in adulthood: childhood and juvenile absence epilepsy (CAE and JAE), juvenile myoclonic epilepsy (JME), and epilepsy with grand mal on awakening (EGMA). While CAE has been regarded as a rather benign condition that is self-limiting in adolescence in the majority of patients, the other three entities have been thought for decades to have high relapse rates after antiepileptic drug (AED) withdrawal and thus to require life-long AED treatment. The Berlin-based epileptologist Dieter Janz has characterized the aboved mentioned IGE subtypes in the 1950ies in detail, his early work contributed to the classification of epilepsies, and in particular to that of IGEs, by the ILAE in the late 1980ies. At first, Janz himself observed and published that AED withdrawal in JME and EGMA has very high relapsed rates and concluded that withdrawal should be avoided.
In the last 5 years or so, a couple of studies have focused on long-term seizure outcome in JME. In unison, these investigations found that after 20 years and more the majority of JME patients were seizure free for at least 5 years, some of them even without AEDs. Based on records of outpatients diagnosed and treated by Janz since the 1950ies, we identified cohorts of patients with CAE, JAE, JME and EGMA with follow-up periods of 40 to 45 years. In general, 50 to 60% of patients were in 5- year-terminal remission. The majority of these patients had seizure free periods of more than 20 years. In absence epilepsies and in EGMA, the age at investigation was an independent predictor for seizure freedom. The older the patients were, the more likely they were free of seizures. At least one trial of AED withdrawal was performed in every fourth to every other patient, relapse rates did not exceed 50%.
In summary, IGEs of adolescence with childhood and juvenile age of onset have a better prognosis than previously thought. With increasing age of affected patients, seizure outcome improves and AED withdrawal may be justified.