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Most children with epilepsy in childhood achieve seizure freedom with anticonvulsant medication, and many outgrow their epilepsy and can be seizure-free and medication-free in adulthood. However, up to one third of patients can have intractable epilepsy, usually defined as continuing to have debilitating seizures despite the use of 2 or 3, appropriately chosen anticonvulsant medications at high therapeutic doses. For some children who have focal epilepsy, surgical resection of the responsible focus offers an alternative approach, and possibly cure of epilepsy. For others who do not have a surgical option, dietary manipulation and new techniques of neurostimulation offer approaches that may improve or even achieve seizure control. For those children whose seizure control improves, a reduction in medication dose or number can improve quality of life by reducing adverse effects.

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The Ketogenic Diet (KD)

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The ketogenic diet simulates the biochemical changes of fasting by providing the majority of calories in the diet from fat. The dietary fat is metabolized via acetyl coenzyme A to ketone bodies (acetoacetate 3-hydroxybutyrate and acetone) and fatty acids.1 In the context of reduced glucose availability, the body utilizes ketone bodies and free fatty acids for energy production. On a ketogenic diet, approximately 90% of calories consumed are from fat in contrast to a standard Western diet in which approximately 30% of calories are from fat. A ketogenic diet is high in fat, low in carbohydrate (10 to 15 gm/day), and provides adequate protein for health and growth (~ 1 gm/kg/day in children); typically it involves a 3:1 to 4:1 ratio of fat (grams) to carbohydrate and protein (grams).2In order to maintain ketosis, the components of each meal, snack, or beverage must preserve this ratio. The carbohydrate content of medications must be considered and minimized, usually by eliminating sugary liquid or chewable tablets in favor of standard tablet formulations. The diet can be provided using formula preparations for young infants and for patients using feeding tubes. The successful use of the ketogenic diet requires medical supervision, and the input ofa specifically trained, knowledgeable nutritionist for diet design, adjustment, and surveillance of growth and nutritional status. Parent education for the practicalities of weighing and measuring food components, managing food preferences and feeding behaviors, and adjusting for intercurrent illnesses, holidays, and variable energy demands, is essential . An intangible additional component of successful ketogenic diet programs is the ability to enthusiastically support families over the long term, who are engaged in a difficult, life-altering, but potentially rewarding process.

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The ketogenic diet fell out of favor in most centers with the discovery of phenytoin and remained unpopular as newer drugs came to market. However, it has become clear that though safer than the older anticonvulsant drugs, the new drugs are not necessarily more effective. This has led to a resurgence of interest in diet treatment. In patients with epilepsy who fail to be controlled with a first anticonvulsant, the use of an alternative ...

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