Epilepsy Research (2015) 109, 81—89 jo ur nal ho me p ag e: www.elsevier.com/locate/epilepsyres Efficacy and tolerability of the ketogenic diet in Dravet syndrome — Comparison with various standard antiepileptic drug regimen Anastasia Dressler a , Petra Trimmel-Schwahofer a , Eva Reithofer a , Angelika Mühlebner a , Gudrun Gröppel a , Edith Reiter-Fink a , Franz Benninger b , Roland Grassl b , Martha Feucht a,* a Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria b Department of Child and Adolescent Neuropsychiatry, Medical University Vienna, Waehringer Guertel 18-20, 1090 Vienna, Austria Received 7 September 2014; received in revised form 6 October 2014; accepted 18 October 2014 Available online 28 October 2014 KEYWORDS Dravet syndrome; Ketogenic diet; Epilepsy; Status epilepticus Summary 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 dif- ferent 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 Although the effect of the KD was independent of age at initiation, it had to be withdrawn due to noncompliance more frequently in solid fed older children compared with infants treated with the liquid ketogenic formula.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 ∗ Corresponding author at: Epilepsy Monitoring Unit, Department of Pediatrics and Adolescent Medicine, Medical University Vienna, Waehringer Guertel 18-20, A-1090 Wien, Austria. Tel.: +43 1 40400 38050; fax: +43 1 40400 22770. E-mail address: martha.feucht@meduniwien.ac.at (M. Feucht). http://dx.doi.org/10.1016/j.eplepsyres.2014.10.014 0920-1211/© 2014 Elsevier B.V. All rights reserved.