Ketogenic diet also benefits Dravet syndrome patients receiving stiripentol: A prospective pilot study

Abstract

SUMMARYWe 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 seizurefree. 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.

The ketogenic diet in infants – Advantages of early use

“…Good efficacy in DS was observed by several centers (65-66% responders and 0-6% seizure free), respectively (Laux and Blackford, 2013;Nabbout et al, 2011) and also by our group with 70% response and 10% seizure freedom (Dressler et al, 2015).…”

Efficacy and tolerability of the ketogenic diet in Dravet syndrome – Comparison with various standard antiepileptic drug regimen

“…There is strong evidence that the KD effectively controls seizures in patients with DS (Caraballo, 2011;Caraballo et al, 2005;Kang et al, 2005;Korff et al, 2007;Laux and Blackford, 2013), especially when added to the gold standard triple combination (Nabbout et al, 2011). Further, the diet has been reported to exhibit neuroprotective effects (Dutton et al, 2011;Luan et al, 2012) and to control long lasting SE refractory to conventional AED treatment (Nabbout et al, 2010).…”

SCN2A mutation in a Chinese boy with infantile spasm - response to Modified Atkins Diet

“…The ketogenic diet was showed to improve the seizure thresholds in mice with SCN1A mutation which is the major cause of Dravet syndrome [14]. Previous studies demonstrated a significant reduction of seizure frequency in patients with Dravet syndrome who were maintained on this diet [15,16]. Recent evidence showed that the ketones produced after MAD could reduce synaptic transmission and neuron excitability by inhibiting glutamate transport and activation of ATP-sensitive potassium channels [17].…”