Premature death in juvenile myoclonic epilepsy

Objectives-To report three cases of premature death in juvenile myoclonic e pilepsy (JME), a benign form of idiopathic generalized epilepsy (IGE) in wh ich no case of epilepsy-related death has been reported. Material and metho ds-We retrospectively analyzed all medical records of JME patients first re ferred to two epilepsy centers (Marseilles, Nice) between 1981 and 1998. Re sults-Among 170 consecutive JME cases, 3 female patients died prematurely. No autopsy was performed. The first had a history of severe anorexia nervos a (DSM IV: 307.1). She died at age 34 and 2 days, from severe inhalation pn eumonia. The second is a woman with a history of infantile psychosis (DSM I V: 299.80) and with a case of IGE in her family. Her epilepsy was never con trolled. At age 16, she was found cyanotic and unconscious one morning in t he toilets. She died before resuscitation was undertaken. The third had a b orderline personality (DSM IV: 301.83) and a history of alcoholism and low compliance. Her epilepsy was never well controlled. She also received neuro leptics. At age 42, she was found dead in her home. Conclusion-In the first case, death was apparently unrelated to epilepsy. In the second, an awaken ing seizure seems to be responsible. In the third, death is also possibly s eizure-related. Cases two and three had persistent seizures and severe psyc hiatric disorders. Serious mental disorders seem to be risk factors for une xpected death. In JME, the overall death ratio was 1.4/1000 patient-years ( or 0.9 if we exclude case 1).