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Perioral myoclonia with absences manifesting with rhythmic twitching of the corners of the mouth and swallowing

Perioral myoclonia with absences manifesting with rhythmic twitching of the corners of the mouth and swallowing

Video-EEG of an absence with moderate impairment of consciousness and rhythmic twitching of the corners of the mouth with simultaneous rhythmic swallowing. This 22-year-old woman had a half-sister who died at 19 years of age from a type of “epilepsy.” At 10 years of age the patient had onset of TAS with a pyknolepsy frequency. These were brief, caused her to “miss conversation,” and were more frequent in the morning and after sleep deprivation. At 20 years of age, she drank a lot of alcohol at an all-night party, slept for an hour, and awoke to feel “strange, dizzy in the head.” This episode of absence status lasted a few hours and terminated in a GTCS. Previously, she had failed to respond to carbamazepine and ethosuximide. Valproate helped, but compliance was poor. (Used with permission from: Panayiotopoulos CP. Perioral myoclonia with absences. In: Panayiotopoulos CP, editor. The Epilepsies: Seizures, Syndromes and Management. Oxford: Bladon Medical Publishing, 2005:324-7.)

Associated Disorders

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