Juvenile Myoclonic Epilepsy: Myoclonic Status Epilepticus without Coma - Report of Three Cases

Status epilepticus (SE) is rare in juvenile myoclonic epilepsy (JME). This report presents three patients with myoclonic status epilepticus (MSE). MSE is defined as prolonged period of myoclonic jerks that are correlated with epileptiform discharges on electroencephalogram. The precipitating factors among the three patients were: introduction of carbamazepine in case1, missing the dose in case2, and introduction of oxcarbazepine in case3. Of the three patients, one patient was a misdiagnosed case of JME. In him the diagnosis of JME was established after 35 years when he developed MSE with the addition of oxcarbazepine to the antiseizure medication (ASM) which he was taking. Detailed review of the history revealed that he used to get occasional myoclonic jerks with deprived sleep and stress. This patient illustrates that the diagnosis of JME can be missed or delayed if history of myoclonic jerks is not elicited, particularly in patents with pubertal onset epilepsy. The other lesson is that possibility of JME should be considered in patients with drug resistant epilepsy (pseudo-drug resistance).