XASIM May p141-146

141 M ost practicing child neurologists possess a basic understanding of the ketogenic diet, which is a high-fat, low-protein, low-carbohydrate diet for treating children with epilepsy who do not respond to or cannot tolerate drugs. This diet mimics the biochemical changes associated with starvation and induces, among other changes, production of ketone bodies (mainly beta-hydroxybutyrate, and to a lesser extent, acetoacetate and acetone), which have been implicated in the mechanisms of seizure control. Before the historic first announcement of results with the ketogenic diet by the Mayo Clinic, bromides and phenobarbital were the only effective options for antiepileptic therapy. Fasting as a method for controlling epilepsy had been reported sporadically long before this time. However, it was only after such reports in the clinical journals that other medical centers began adopting the ketogenic diet as an effective treatment for intractable cases of epilepsy. Still, the diet remained generally underutilized— mainly employed at institutions such as the Mayo Clinic and Johns Hopkins—until the 1990s, when a national television program aired a report on a child whose epilepsy was cured by the ketogenic diet. Later, the Charlie Foundation (named after the young patient) was formed and a television movie recounting another young boy’s success with the ketogenic diet was produced. The movie prompted a flood of inquiries to pediatric neurologists and epileptologists about this treatment option. Today, after a long history of clinical use and the recent surge of professional interest and research, many questions remain unanswered about the ketogenic diet. The purpose of this review is to update neurologists by highlighting key issues and controversies pertaining to this important diet-based therapy for epilepsy. In particular, the review will focus on the potential role of ketone bodies in energy metabolism and in seizure control, the indications and contraindications for the ketogenic diet in pediatrics, and other practical clinical questions involving efficacy, patient selection, monitoring, and side effects.