Premature death in juvenile myoclonic epilepsy

OBJECTIVES: To report three cases of premature death in juvenile myoclonic epilepsy (JME), a benign form of idiopathic generalized epilepsy (IGE) in which no case of epilepsy-related death has been reported. MATERIAL AND METHODS: We retrospectively analyzed all medical records of JME patients first referred to two epilepsy centers (Marseilles, Nice) between 1981 and 1998. RESULTS: Among 170 consecutive JME cases, 3 female patients died prematurely. No autopsy was performed. The first had a history of severe anorexia nervosa (DSM IV: 307.1). She died at age 34 and 2 days, from severe inhalation pneumonia. The second is a woman with a history of infantile psychosis (DSM IV: 299.80) and with a case of IGE in her family. Her epilepsy was never controlled. At age 16, she was found cyanotic and unconscious one morning in the toilets. She died before resuscitation was undertaken. The third had a borderline personality (DSM IV: 301.83) and a history of alcoholism and low compliance. Her epilepsy was never well controlled. She also received neuroleptics. 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 awakening seizure seems to be responsible. In the third, death is also possibly seizure-related. Cases two and three had persistent seizures and severe psychiatric disorders. Serious mental disorders seem to be risk factors for unexpected death. In JME, the overall death ratio was 1.4/1000 patient-years (or 0.9 if we exclude case 1).