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Premature death in juvenile myoclonic epilepsy
1Centre Saint Paul-H. Gastaut, 300 Bd de Sainte Marguerite, 13258 Marseille 09, France. piergen@aol.com
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).
Insights
Premature death is rare in juvenile myoclonic epilepsy (JME), but three cases were identified. Severe psychiatric disorders may increase the risk of unexpected death in JME patients with persistent seizures.
Area of Science:
- Neurology
- Epileptology
- Psychiatry
Background:
- Juvenile myoclonic epilepsy (JME) is typically considered a benign form of idiopathic generalized epilepsy (IGE).
- Historically, epilepsy-related deaths have not been reported in JME cases.
- This study investigates premature mortality within a JME cohort.
Observation:
- Three female patients out of 170 consecutive JME cases experienced premature death between 1981 and 1998.
- Case 1: Died at 34 from pneumonia, unrelated to epilepsy.
- Case 2: Died at 16, possibly from an awakening seizure, with uncontrolled epilepsy and infantile psychosis.
- Case 3: Died at 42, possibly seizure-related, with uncontrolled epilepsy, borderline personality, alcoholism, and neuroleptic use.
Findings:
- Two of the three deaths were potentially seizure-related.
- Patients with persistent seizures and severe psychiatric comorbidities experienced unexpected mortality.
- The overall death ratio in JME was 1.4/1000 patient-years (0.9 excluding the non-epilepsy death).
Implications:
- Serious mental disorders may represent significant risk factors for unexpected mortality in JME.
- This study highlights the importance of considering psychiatric comorbidities in the management of JME.
- Further research is warranted to understand the mechanisms and risk factors for premature death in JME.