title: Sulthiame add-on therapy in children with focal epilepsies associated with encephalopathy related to electrical status epilepticus during slow sleep (ESES).
abstract: OBJECTIVE
  In children with symptomatic or idiopathic focal epilepsies, their disease may evolve into an epileptic encephalopathy related to continuous spike and wave during slow sleep (CSWS) or electrical status epilepticus during slow sleep (ESES). ESES syndrome implies serious risks of neuropsychologic impairment, and its treatment has frequently been disappointing. The aim of this study is to present our experience using sulthiame as add-on treatment in 53 patients with ESES syndrome that was refractory to other antiepileptic drugs (AEDs).
  
  
  METHODS
  Neurologic examinations, cerebral magnetic resonance imaging (MRI), and repeated prolonged sleep electroencephalography (EEG) studies were performed in all cases. Data about school achievements and or neuropsychological evaluations were obtained repeatedly during the follow-up of 1.5-16 years. Sulthiame was added in doses ranging between 5 and 30 mg/kg/day.
  
  
  RESULTS
  Since add-on of sulthiame, 10 of 28 patients in the symptomatic group became seizure free: 4 patients with normal EEG studies and 6 with residual spikes. Nine of 28 patients showed a significant reduction in number of seizures and presented spikes but no ESES on EEG. The other nine cases showed neither clinical nor EEG improvement. A striking result was that 3 of 11 children with unilateral polymicrogyria and ESES syndrome became seizure free, and in another six a significant improvement in frequency of seizures and in EEG abnormalities seemed to be related to the add-on of sulthiame. Twenty-one of the 25 patients in the idiopathic group became seizure free and without ESES in <3 months after add on of sulthiame. In two of the patients the changes were seen in a few days.
  
  
  CONCLUSIONS
  We understand that sulthiame may be effective as add-on treatment in children with ESES syndrome.
authors.affiliation: Department of Neurology, Juan P. Garrahan Pediatric Hospital, Buenos Aires, Argentina. natalio@fejerman.com
authors.forename: Natalio
authors.initials: N
authors.lastname: Fejerman
authors.forename: Roberto
authors.initials: R
authors.lastname: Caraballo
authors.forename: Ricardo
authors.initials: R
authors.lastname: Cersósimo
authors.forename: Stella Maris
authors.initials: SM
authors.lastname: Ferraro
authors.forename: Santiago
authors.initials: S
authors.lastname: Galicchio
authors.forename: Hernán
authors.initials: H
authors.lastname: Amartino
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D009483:Neuropsychological Tests; D012893:Sleep Wake Disorders / Q000188:drug therapy* / Q000209:etiology; D013226:Status Epilepticus / Q000150:complications / Q000188:drug therapy*; D013843:Thiazines / Q000627:therapeutic use*; D000096926:Benzenesulfonamides
chemical_list: D000927:Anticonvulsants; D013843:Thiazines; C084593:sulthiame; D000096926:Benzenesulfonamides