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.)