We aimed to test the efficacy of ketogenic diet (KD) in patients with Dravet syndrome (DS) not satisfactorily controlled by antiepileptic drugs (AEDs). We included prospectively 15 DS patients aged >3 years with partial response to AEDs including stiripentol. All patients had a seizure diary and clinical examination with Conners and Achenbach scales before KD, at 1 month following onset and every 3 months thereafter. At 1 month, 10 patients (66%) had a decrease of seizure frequency ≥75%. Efficacy was maintained in eight responders at 3 and 6 months and in six responders at 9 months. Five patients (33%) remained on KD over 12 months, and one was seizure-free. In addition to efficacy on seizure frequency, KD was beneficial on behavior disturbances including hyperactivity. This effect was reported in all responders and in a few nonresponders. KD might have a double effect, on seizure control and on hyperactivity and behavior disturbances in patients with DS.
Wiley Periodicals, Inc. © 2011 International League Against Epilepsy.