Ketogenic Diet in Patients with Dravet Syndrome

Abstract

Summary:Purpose: The ketogenic diet (KD) has been used as a therapeutic alternative to antiepileptic drugs (AEDs) for refractory epilepsy. Severe myoclonic epilepsy in infants or Dravet syndrome (DS) is one of the most malignant epileptic syndromes. In this retrospective study, we evaluated the efficacy and tolerability of the KD in patients with diagnostic criteria of DS.Methods: Between March 1, 1990, and August 31, 2004, 52 patients who met diagnostic criteria for DS were enrolled in a study at our department. Twenty of them were placed on the KD with the Hopkins protocol and followed up for a minimum of 1 year.Results: Three of the 20 original children stayed on the diet for 12 months, four children for 2 years, four children for 3 years, and two children for 4 years. One year after initiating the diet, 13 (65%) of the initial patients remained on the diet. Two (15%) patients were seizure free, eight (61.7%) children had a 75-99% decrease in seizures, and the remaining three (23%) children had a 50-74% decrease in seizures. Thus 1 year after starting the diet, 10 (77%) children had achieved a >75% decrease in their seizures. Four patients have been off the diet for >2 years; one of them is seizure free, two have sporadic seizures, and one, who abandoned the diet after 2 years of adhering to it, relapsed. No differences in seizure control when compared with age, sex, or seizure type were found.Conclusions: Considering the severity and intractability of seizures in patients with DS, the fact that 10 of the 13 children who remained on the diet had a significant reduction in number of seizures shows that the KD is at present an interesting therapeutic alternative. Even in patients in whom seizure reduction was not dramatic, quality of life improved, and in all of them, the number of AEDs was reduced to one or two. We consider that children with DS should be offered the KD immediately after three adequate trials of AEDs have failed.

Neuroprotective and disease-modifying effects of the ketogenic diet

“…Although the diet is useful in people of all ages, clinical experience suggests that it may be more valuable in children, if only because adults have greater difficulty adhering to it. Importantly, the diet is often effective in pharmacoresistant forms of common epilepsies as well as in the difficult to treat catastrophic epilepsy syndromes of infancy and early childhood such as West Syndrome, Lennox-Gastaut Syndrome, and Dravet Syndrome (Crumrine, 2002;Trevathan, 2002;Caraballo et al, 2005).…”

Advanced Treatments for Childhood Epilepsy

“…9,10 The ketogenic diet might be particularly helpful for certain epilepsy syndromes, for example Dravet syndrome, 11 myoclonic atonic epilepsy, 10 and infantile spasms. 12 The ketogenic diet can be efficacious for focal and generalized epilepsies, 13 but children with focal epilepsies whose seizures do not respond to conventional treatment should undergo evaluation for epilepsy surgery if at all possible, because surgery could be curative.…”

Non-pharmacologic Therapeutic Alternatives for Children with Pharmacoresistant Epilepsy: Vagus Nerve Stimulation and Ketogenic Diet

“…Ketojenik diyetin özellikle faydalı ya da kontrendike olduğu düşünülen sendrom ve durumlar [14] Kullanımında özellikle faydalı olduğu kabul edilen Kullanımının kontrendike olduğu kabul edilen [20][21][22] Son çalışmalarda, diyetin infantil spazmlarda, şiddetli miyoklonik epilepside ve tuberozkleroz kompleksinde özel-likle etkin olduğu vurgulanmakta, pediatrik status olguların-da da etkinliğine dikkat çeken olgu sunumları bildirilmektedir. [23][24][25][26][27] Az sayıdaki çalışmalar da, çocuklarda epilepsinin erken dönemlerinde de KD'ye başvurulabileceği konusunda öneriler getirmekte, özellikle iki beyin enerji metabolizma bozukluğu hastalığı olan GLUT 1 Eksikliği Sendromu ve Piruvat Dehidrogenaz Eksikliği'nde (PDHD) erken evrelerde başlanılmasının faydalarından bahsedilmektedir. [28][29][30] Kullanımında özellikle fayda görülen ya da kontrendike bulunan sendrom ve durumlar Tablo 1'de görülmektedir.…”