Author(s): Kramer U, Sagi L, GoldbergStern H, Zelnik N, Nissenkorn A,
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Abstract PURPOSES: To describe the clinical spectrum and to evaluate the efficacy of different therapeutic agents in children with electrical status epilepticus in sleep (ESES). METHODS: Clinical data of all patients with ESES (not including patients with Landau-Kleffner syndrome) in four pediatric neurology outpatient clinics were analyzed. Thirty patients with ESES had been treated between 1994 and 2007. RESULTS: Eleven (37\%) children had benign partial epilepsies of childhood, five (17\%) had cerebral palsy, five (17\%) had hydrocephalus, one (3\%) had schizencephaly, one (3\%) had prenatal parenchymal bleeding, and the etiology was unclear in seven (23\%). The duration of ESES ranged between 2 and 60 months. The antiepileptic drugs that were found to be efficacious were: levetiracetam (41\%), clobazam (31\%), and sulthiame (17\%). Valproic acid, lamotrigine, topiramate, and ethosuximide showed no efficacy. Steroids were efficacious in 65\%; immunoglobulins were efficacious in 33\%. High-dose diazepam was efficacious in 37\%, but all the children had temporary response. Seventeen patients (57\%) had cognitive deterioration, whereas the rest presented with regression in attention, speech, communication, and behavior. Fourteen children had permanent cognitive deficit. There was a significant correlation (p = 0.029) between the duration of ESES and residual intellectual deficit at follow-up. CONCLUSIONS: ESES reflects an evolution of benign partial epilepsy of childhood in more than one-third of the patients, whereas there is an underlying structural brain anomaly in another one-third. The most efficacious antiepileptic drugs (AEDs) are levetiracetam and clobazam. The duration of ESES correlated significantly with residual intellectual deficit at follow-up.
This article was published in Epilepsia
and referenced in Epilepsy Journal