title: Efficacy and tolerability of the ketogenic diet in Dravet syndrome - Comparison with various standard antiepileptic drug regimen.
abstract: There is strong evidence for the use of the ketogenic diet (KD) in Dravet syndrome (DS). The purpose of this study was to evaluate both effectiveness and tolerability in comparison with various antiepileptic drugs (AEDs).
  
  
  METHODS
  32 children (19 males) with genetically confirmed DS treated at our center since 1999 were analyzed retrospectively. Data collected from patients' files included type of mutation, age at treatment initiation and treatment lag, overall seizure frequency and frequency of different seizure types, especially prolonged seizures and status epilepticus (SE). Efficacy and safety of the KD were evaluated. In addition, the effect on seizure count was compared with that of various AED regimen and the vagus nerve stimulation (VNS).
  
  
  RESULTS
  Overall response to the KD was 70% at 3 months and 60% at 12 months. No SE occurred while patients were on the diet, and the frequencies of prolonged generalized and myoclonic seizures were reduced. No severe side effects requiring withdrawal of the KD were observed. Although the effect of the KD was independent of age at initiation, it had to be withdrawn due to noncompliance more frequently in solid fed older children compared with infants treated with the liquid ketogenic formula. The KD was not significantly inferior to the current gold standard AED triple combination of Stiripentol+Valproate+Clobazam (89%), Bromides (78%), Valproate alone (48%), Topiramate (35%) and VNS (37%) and significantly more effective than Levetiracetam (30%; p=0.037, Pearson's Chi-square).
  
  
  CONCLUSIONS
  These data suggest that the KD ranks among currently used AEDs as an effective treatment for seizures in DS. According to our results (good effect on SE and prolonged seizures, good tolerability, less compliance problems due to formula treatment) the KD should be considered as an early treatment option in infants with DS.
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
authors.forename: Anastasia
authors.initials: A
authors.lastname: Dressler
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
authors.forename: Petra
authors.initials: P
authors.lastname: Trimmel-Schwahofer
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
authors.forename: Eva
authors.initials: E
authors.lastname: Reithofer
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
authors.forename: Angelika
authors.initials: A
authors.lastname: Mühlebner
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
authors.forename: Gudrun
authors.initials: G
authors.lastname: Gröppel
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
authors.forename: Edith
authors.initials: E
authors.lastname: Reiter-Fink
authors.affiliation: Department of Child and Adolescent Neuropsychiatry, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
authors.forename: Franz
authors.initials: F
authors.lastname: Benninger
authors.affiliation: Department of Child and Adolescent Neuropsychiatry, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria.
authors.forename: Roland
authors.initials: R
authors.lastname: Grassl
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria. Electronic address: martha.feucht@meduniwien.ac.at.
authors.forename: Martha
authors.initials: M
authors.lastname: Feucht
mesh_terms: D000293:Adolescent; D000927:Anticonvulsants / Q000627:therapeutic use*; D001569:Benzodiazepines / Q000627:therapeutic use; D001965:Bromides / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D000078306:Clobazam; D055423:Diet, Ketogenic; D004148:Dioxolanes / Q000627:therapeutic use; D004831:Epilepsies, Myoclonic / Q000235:genetics / Q000628:therapy*; D005260:Female; D005632:Fructose / Q000031:analogs & derivatives / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D000077287:Levetiracetam; D008297:Male; D010889:Piracetam / Q000031:analogs & derivatives / Q000627:therapeutic use; D012189:Retrospective Studies; D012640:Seizures / Q000628:therapy; D000077236:Topiramate; D016896:Treatment Outcome; D055536:Vagus Nerve Stimulation; D014635:Valproic Acid / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000927:Anticonvulsants; D001965:Bromides; D004148:Dioxolanes; D000077236:Topiramate; D001569:Benzodiazepines; D000078306:Clobazam; D005632:Fructose; D000077287:Levetiracetam; D014635:Valproic Acid; C021092:stiripentol; D010889:Piracetam
keywords: Dravet syndrome; Epilepsy; Ketogenic diet; Status epilepticus