A Case of Electrical Status Epilepticus during Sleep

Song, Hong Ki

Abstract

Electrical status epilepticus during sleep (ESES), an EEG defined syndrome characterized by the occurrence of almost continuous spike and/or slow waves during nonREM sleep, is considered to be rare in incidence. It broadly overlaps with benign rolandic epilepsy, pseudo-Lennox syndrome, and Landau-Kleffner syndrome. A 13-year-old boy has been followed up for 11 years because of intractable epilepsy. He is an adopted child and first seizure occurred at the age of 14 months. Seizures were described as tonic, atypical absence and partial motor, occurred daytime and nocturnal, more frequent in the latter. He was retarded in development and had failed to acquire speech. Serial EEG showed moderately developed posterior dominant rhythm during wakefulness, and, however, continuous 2.5 to 3 Hz bilaterally synchronous centro-temporal sharp waves were observed during sleep state. The EEG features were similar, regardless of spontaneous or induced sleep, and remained essentially unchanged during a 12 year follow up period. Brain MRI was not significant. During follow-up period, carbamazepine and vigabatrin worsened his seizures in frequency and intensity, resulting in frequent generalization. On the contrary, there were rare seizures with combination therapy of valproate, clonazepam and lamotrigine and seizures were nearly disappeared after topiramate add-on. This is a case of ESCS with cognitive, behavioral and language disturbances. Clinical and EEG features of related syndromes will be briefly reviewed.