Eyelid Myoclonia with Absence
A spectrum of absence seizures (AS) is associated with generalized, symmetric, bifrontal pre-dominant, 3 Hz spike-and-waves on EEG (figure A). Prominent eyelid flutter, EEG, and photosensitivity suggests Jeavons syndrome (JS). JS is characterized by eyelid myoclonia with and without AS1. A reflex epilepsy, there is prominent photosensitivity (figure B) and activation of 3-Hz GSW on EEG during eyelid closure. JS is typically occurs in girls who are otherwise normal and represents a unique type of genetic epilepsy with AS. It peaks in childhood and may have an onset early on in life before childhood onset absence epilepsy and is typically a life-long condition. Video-EEG monitoring is essential for a definitive diagnosis, to quantify seizures, and confirm photosensitivity. EEG shows 3-Hz or faster GSW that may contain polyspikes within the bursts upon eye closure. Occipital abnormalities were not encountered in our patient though has focal initiation of the thalamo-cortical system in some patients has been suggested2. Treatment of the eyelid myoclonia is difficult with drug-resistance common.
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