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Myoclonic status epilepticus in epilepsy with myoclonic-atonic seizures

This 65-year-old woman had mild mental retardation and epilepsy with myoclonic-atonic seizures since childhood. Seizures had been infrequent for many years. In the setting of withdrawing levetiracetam, she developed frequent myoclonic jerks characterized by sudden flexion of her trunk and neck with abduction of her shoulders and arms. The patient was able to talk and interact with examiners between jerks. EEG background mild diffuse slowing with 10 Hz alpha rhythm and frequent 4 to 5 Hz bifrontally predominant polyspike-wave discharges corresponding to the myoclonus. Myoclonic jerks continued frequently (10 to 25 per hour) for 24 hours. This improved dramatically with reinstitution of levetiracetam. (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.)