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Epileptic Disorders

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Absence status epilepticus in monozygotic twins with Jeavons syndrome Volume 10, numéro 3, September 2008

Auteurs
Department of Neurology, West China Hospital, Si Chuan University, Chengdu, Sichuan, P.R. China

As a generalized form of nonconvulsive status epilepticus (NCSE), absence status epilepticus is the most common form. It manifests as prolonged, confusional states of varying severity, and continuous or repetitive generalized discharges of spikes, multiple spikes, and slow waves on EEG. Jeavons syndrome (JS) is a new type of epilepsy syndrome. Hitherto, only four sets of monozygotic twin with JS have been reported. Absence status epilepticus occurring in monozygotic twins with JS have not been reported. Here we report on monozygotic male twins of Chinese origin with JS. Both of them presented with status epilepticus with eyelid myoclonia and absences. [Published with video sequences]