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Facial (eyelid, perioral) myoclonia with typical absence seizures (from video-EEG)

Basic sleep-awake video-EEG from girl at the age of 8 years old who started having episodes with eyelid blinking, no responsiveness, and de novo mouth movement. She was having 10 to 15 episodes daily. The video-EEG did not show abnormalities. During hyperventilation, the generalized spike-wave discharge evoked was associated with the following seizure: eyelid flutter, no response to verbal commands, and concomitant de novo mouth movements. Some high-amplitude, 3 Hz slow waves heralded or followed the generalized discharges. A few other episodes recorded lasted from 11 to 14 seconds with similar electroclinical expression. Her seizures were finally controlled on ethosuximide and lamotrigine. After 4 years of good electroclinical response, lamotrigine was phased-out. Eight months later, her EEG, during hyperventilation showed some irregular spike-wave discharges, and during intermittent photic stimulation at 10 flashes per second, a generalized spike-wave discharge was evoked, but there were no concomitant clinical events. About 6 months later, she started having similar absence seizures, and lamortigine was added to ethosuximide again. The patient history was noncontributory except for 10 febrile seizures when we was younger than 5 years old. Neurologic assessment was normal. Family history revealed her father had three febrile seizures in infancy. (Contributed by Dr. Athanasios Covanis.)

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Associated Disorders

  • Epilepsy with myoclonic absences
  • Facial (perioral and or eyebrow) myoclonia with absences
  • Perioral myoclonia with absences