title: Epileptic encephalopathy with electrical status epilepticus: an electroclinical study of 59 patients.
abstract: OBJECTIVE
  To evaluate the electroclinical features, treatment effectiveness, and outcome of 59 patients with epileptic encephalopathy with electrical status epilepticus during sleep.
  
  
  METHODS
  Medical-files of 59 patients with electrical status epilepticus during sleep were retrospectively evaluated for data concerning: history, physical and neurological examinations, sleep and awake EEG's, psychometric tests and brain MRI.
  
  
  RESULTS
  A total of 31 boys and 28 girls were identified. Patients were evaluated in two groups: symptomatic/structural and idiopathic group. There was no significant difference between the etiological groups in term of mean age at ESES onset, mean interval between the first seizure and the onset of ESES. The mean age at seizure onset was earlier in the symptomatic/structural group than the idiopathic ones. The mean follow-up time after the ESES onset was 4.5 years for all patients. The most effective antiepileptic drugs in our series were clobazam and levetiracetam. In refractory patients, steroid treatment was found effective during the early course of the disease. In the idiopathic group, cognitive decline has improved. However in the symptomatic group, patients did not respond to the treatment and cognitive deterioration did not improve in one third of the group.
  
  
  CONCLUSIONS
  The long-term outcome of ESES is highly variable and usually depends on etiology and the duration of ESES. The most efficious antiepileptic drugs in our study are clobazam and levetiracetam.
authors: Gazi University Faculty of Medicine, Pediatric Neurology, Turkey. Electronic address: mehmetarhan@gmail.com.
authors: Ebru
authors: E
authors: Arhan
authors: Gazi University Faculty of Medicine, Pediatric Neurology, Turkey.
authors: Ayse
authors: A
authors: Serdaroglu
authors: Gazi University Faculty of Medicine, Pediatric Neurology, Turkey.
authors: Kursad
authors: K
authors: Aydin
authors: Gazi University Faculty of Medicine, Pediatric Neurology, Turkey.
authors: Tugba
authors: T
authors: Hırfanoglu
authors: Gazi University Faculty of Medicine, Pediatric Neurology, Turkey.
authors: A Sebnem
authors: AS
authors: Soysal
mesh_terms: D000927:Anticonvulsants / Q000627:therapeutic use*; D001921:Brain / Q000187:drug effects / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D008137:Longitudinal Studies; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D012890:Sleep; D013226:Status Epilepticus / Q000150:complications* / Q000188:drug therapy*; D016896:Treatment Outcome
chemical_list: D000927:Anticonvulsants
keywords: Epileptic encephalopathy with electrical status epilepticus during sleep; Neuropsychological aspects; Symptomatic and idiopathic; Treatment