Eyelid myoclonia with typical absences: an epilepsy syndrome.
Five unrelated patients are described with the clinical and electrical features of eyelid myoclonia with absences (EMA). In this syndrome brief, typical absences occur with rapid eyelid myoclonia associated with retropulsive movements of the eyeballs and occasionally of the head. The seizures are of shorter duration than in childhood absence epilepsy, and are accompanied by less profound impairment of consciousness. The electroencephalogram demonstrates high amplitude discharges consisting of spikes, multiple spikes and slow waves at a fluctuating frequency of 3-5 Hz and following eye closure, which disappear in darkness. Photosensitivity is also seen. Onset is in early childhood and EMA appears to persist into adult life. Treatment is sodium valproate in combination with either ethosuximide or a benzodiazepine. On the basis of the clinical features, EEG findings, and the response to treatment and prognosis, it is suggested that EMA be classified as a specific epilepsy syndrome.
Appleton
R E
RE
Department of Neurology and Neurophysiology, Royal Liverpool Children's NHS Trust Alder Hey, UK.
Panayiotopoulos
C P
CP
Acomb
B A
BA
Beirne
M
M
D000293:Adolescent; D001921:Brain / Q000503:physiopathology*; D002675:Child, Preschool; D004569:Electroencephalography; D004832:Epilepsy, Absence / Q000503:physiopathology*; D005143:Eyelids / Q000503:physiopathology*; D005260:Female; D006801:Humans; D008297:Male; D009207:Myoclonus / Q000503:physiopathology*; D013577:Syndrome