title: Clinical spectrum and medical treatment of children with electrical status epilepticus in sleep (ESES).
abstract: OBJECTIVE
  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.
authors.affiliation: Pediatric Neurology Unit, Dana Children's Hospital, Tel Aviv Medical Center, Tel Aviv, Israel. umkramer@netvision.net.il
authors.forename: Uri
authors.initials: U
authors.lastname: Kramer
authors.forename: Liora
authors.initials: L
authors.lastname: Sagi
authors.forename: Hadassa
authors.initials: H
authors.lastname: Goldberg-Stern
authors.forename: Nathanel
authors.initials: N
authors.lastname: Zelnik
authors.forename: Andreea
authors.initials: A
authors.lastname: Nissenkorn
authors.forename: Bruria
authors.initials: B
authors.lastname: Ben-Zeev
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000145:classification / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D012890:Sleep / Q000187:drug effects / Q000502:physiology*; D013226:Status Epilepticus / Q000188:drug therapy* / Q000503:physiopathology*; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants