title: Ketogenic diet in patients with epileptic encephalopathy with electrical status epilepticus during slow sleep.
abstract: Epileptic encephalopathy with electrical status epilepticus during slow sleep (ESES) belongs to the group of epileptic encephalopathies that often prove refractory to AED treatment. The ketogenic diet (KD) has been used as an alternative to antiepileptic drugs (AEDs) for patients with refractory epileptic encephalopathies.
  
  
  OBJECTIVE
  In this retrospective study we assess the efficacy and tolerability of the KD in patients with ESES syndrome.
  
  
  METHODS
  Between March 1, 1990 and April 1, 2013, 65 patients who met diagnostic criteria of ESES syndrome were seen at our department. Twelve of them were placed on the KD and followed for a minimum of 18 months.
  
  
  RESULTS
  The children had previously received a mean of 5.5 different AEDs and were on a mean of 3 AEDs when the diet was started. Eighteen months after initiating the diet, seven of the initial patients (58%) remained on the diet; one patient (8.3%) had become seizure free, one (8.3%) had a 75-99% decrease in seizures, two (16.6%) had a 50-74% decrease in seizures, and the remaining three children (24.9%) had a <50% decrease in seizures. In the patient who had become seizure free and in the one who had a 75-99% seizure decrease AEDs were reduced.
  
  
  CONCLUSIONS
  The KD is a well-tolerated treatment option for patients with ESES syndrome, not only for structural cases but also for those with an unknown etiology. The diet should be considered in the management of this syndrome.
authors.affiliation: Department of Neurology, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Gabriela
authors.initials: G
authors.lastname: Reyes
authors.affiliation: Department of Neurology, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Santiago
authors.initials: S
authors.lastname: Flesler
authors.affiliation: Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Marisa
authors.initials: M
authors.lastname: Armeno
authors.affiliation: Department of Neurology, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Sebastian
authors.initials: S
authors.lastname: Fortini
authors.affiliation: Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Agustinho
authors.initials: A
authors.lastname: Ariela
authors.affiliation: Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Araceli
authors.initials: A
authors.lastname: Cresta
authors.affiliation: Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.
authors.forename: Graciela
authors.initials: G
authors.lastname: Mestre
authors.affiliation: Department of Neurology, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina. Electronic address: rhcaraballo@arnet.com.ar.
authors.forename: Roberto Horacio
authors.initials: RH
authors.lastname: Caraballo
mesh_terms: D002648:Child; D055423:Diet, Ketogenic / Q000379:methods*; D004569:Electroencephalography; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D012189:Retrospective Studies; D012893:Sleep Wake Disorders / Q000150:complications / Q000178:diet therapy*; D013226:Status Epilepticus / Q000150:complications / Q000178:diet therapy*; D016896:Treatment Outcome
keywords: ESES syndrome; Epileptic encephalopathy; Ketogenic diet; Refractory epilepsy; Seizures