Ketogenic diet in pediatric epilepsies

The ketogenic diet (KD), an individually calculated and tightly controlled high-lipid, adequate-protein, low-carbohydrate diet, is an established treatment option for children with refractory or drug-resistant epilepsy (DRE), and it is the gold standard treatment for two specific metabolic disorders: GLUT-1 and pyruvate dehydrogenase deficiency. Recent studies supported its extension also in adults and in patients with status epilepticus. Since its introduction in the clinical practice, about 100years ago, four variants of ketogenic diet were developed: classical ketogenic diet, medium-chain triglycerides (MCT) diet and modified MCT diet, modified Atkins diet, and low glycemic index treatment (LGIT). This chapter discusses the structure of the different ketogenic diets, their mechanisms of action, and their role in pediatric epilepsies (including the actual criteria for the selection of candidate patients and their clinical monitoring).

Epilepsy is a disorder of the brain characterized by an enduring predisposition to generate epileptic seizures, and by several neurobiological, cognitive, psychological, and social consequences. The incidence of epilepsy in pediatric age ranges from 41 to 187/ 100,000 (Camfield & Camfield, 2015). About 1/3 of the patients with epilepsy showhypocaloric vegetarian diet associated with intermittent fasting and purging resulting in a significant reduction in the frequency of seizures and in an improvement in cognition (H€ohn et al., 2019). In 1921, Wilder proposed KD as an alternative strategy to obtain the benefits of fasting in patients with epilepsy (H€ohn et al., 2019).