Myoclonic status epilepticus in idiopathic generalized epilepsy

This 13-year-old girl with juvenile myoclonic epilepsy presented with frequent myoclonic jerks at 12 years of age. Her mother also had myoclonus and seizures. The girl was unable to tolerate valproic acid due to weight gain and transaminitis. On lamotrigine monotherapy, she experienced an increase in the frequency and severity of morning myoclonus, which kept her from getting out of bed in the morning. During elective video-EEG monitoring, she had a cluster of myoclonic jerks recurring every 10 to 30 seconds for 40 minutes. Sample EEG demonstrates a drowsy background with frequent 3 to 4 Hz bifrontally predominant generalized polyspike-wave discharges. Myoclonic jerks involving the trunk, arms, and legs occur in a sequence of three to four jerks with each polyspike-wave run lasting longer than 2 seconds. (Reproduced with kind permission of Springer Science+Business Media: Gerard E, Hirsch L. Generalised myoclonic status epilepticus. In: Panayiotopoulos CP, editor. The Atlas of Epilepsies. Springer, 2010.)