title: Children with ESES: variability in the syndrome.
abstract: OBJECTIVE
  We undertook a retrospective study of children who present with significant activation of paroxysmal discharges during sleep to examine the clinical spectrum of disorders that present with such an EEG abnormality.
  
  
  BACKGROUND
  Electrical status epilepticus in sleep (ESES) is an electrographic pattern characterized by nearly continuous spike-wave discharges in slow wave sleep, usually with a frequency of 1.5-3 Hz and usually diffuse and bilateral in distribution. A variety of neurocognitive and behavioral problems have been associated with this EEG pattern.
  
  
  METHODS
  We conducted a retrospective review of 1497 EEG records of patients admitted to University of California, Los Angeles (UCLA) for overnight video-EEG monitoring during a 5 year interval. Demographic, clinical and electroencephalographic variables were evaluated.
  
  
  RESULTS
  EEG records for 102 patients meeting criteria were identified. Clinical information was available for 90 of those patients. Eighteen of these patients could be diagnosed with Landau-Kleffner syndrome (LKS). Key findings include: (1) neuroimaging abnormalities were uncommon in our LKS patients; (2) among children who do not fit the specific diagnostic criteria for LKS, a spike-wave index (SWI) >50% was more likely to be associated with global developmental disturbances than SWI < or =50% (p<0.05); (3) Children with generalized discharges were more likely to experience severe or global developmental disturbance than those with focal abnormalities, without reaching statistical significance (p=0.07).
  
  
  CONCLUSIONS
  Severity of ESES can vary over time between and within patients and clinical status does not always directly correlate with SWI. However, the prognosis of LKS is substantially better than CSWS and these two disorders could be classified in a dichotomous manner rather than be seen as two points along a continuum.
authors.affiliation: Department of Pediatrics and Neurology, David Geffen School of Medicine at UCLA, Mattel Children's Hospital at UCLA, Los Angeles, CA, USA.
authors.forename: Michele
authors.initials: M
authors.lastname: Van Hirtum-Das
authors.forename: Eliot A
authors.initials: EA
authors.lastname: Licht
authors.forename: Susan
authors.initials: S
authors.lastname: Koh
authors.forename: Joyce Y
authors.initials: JY
authors.lastname: Wu
authors.forename: W Donald
authors.initials: WD
authors.lastname: Shields
authors.forename: Raman
authors.initials: R
authors.lastname: Sankar
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003072:Cognition Disorders / Q000209:etiology*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D018887:Landau-Kleffner Syndrome / Q000150:complications / Q000503:physiopathology*; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D012640:Seizures / Q000145:classification*; D012890:Sleep / Q000502:physiology*; D013226:Status Epilepticus / Q000150:complications / Q000188:drug therapy / Q000503:physiopathology*; D013577:Syndrome; D016896:Treatment Outcome; D014741:Video Recording
chemical_list: D000927:Anticonvulsants