Neuromyotonia and myokymia
May. 03, 2021
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Samples of video-EEG of a boy in myoclonic-atonic status epilepticus. (A) The EEG showed electrical status epilepticus during wakefulness and sleep. This consisted of nearly continuous 2.5 to 3 Hz generalized spike/polyspike-wave discharges. This pattern occasionally alternated with relatively normal background activity lasting less than 30 seconds. The main clinical manifestations were frequent facial subtle myoclonias (eyelid fluttering, upwards deviation of the eyes with spontaneous eye opening associated with fast eyelid fluttering, subtle facial twitches), and a few massive myoclonic jerks, occasionally with some atonic components. Clinically, there was no apparent impairment of consciousness. (B) Details of the EEG shown in (A) (note calibration: higher sensitivity and faster speed). (C) The video-EEG 1 month later was normal during wakefulness, with a few myoclonic jerks only during sleep. (Contributed by C P Panayiotopoulos.)