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 br ief, typical absences occur with rapid eyelid myoclonia associated wit h retropulsive movements of the eyeballs and occasionally of the head. The seizures are of shorter duration than in childhood absence epilep sy, and are accompanied by less profound impairment of consciousness. The electroencephalogram demonstrates high amplitude discharges consis ting of spikes, multiple spikes and slow waves at a fluctuating freque ncy of 3-5 Hz and following eye closure, which disappear in darkness. Photosensitivity is also seen. Onset is in early childhood and EMA app ears to persist into adult life. Treatment is sodium valproate in comb ination 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 epile psy syndrome.