The ketogenic diet is a medically supervised high-fat, low-carbohydrate diet that has been found useful in patients with refractory epilepsy. It has been shown to be effective in treating multiple seizure types and epilepsy syndromes. In this paper, we review the use of the ketogenic diet in epileptic encephalopathies such as Ohtahara syndrome, West syndrome, Dravet syndrome, epilepsy with myoclonic atonic seizures, and Lennox-Gastaut syndrome. 1. Introduction Epileptic encephalopathies are disorders in which the presence of frequent and difficult-to-control seizures along with unremitting interictal epileptiform activity contributes to progressive neurodevelopmental deterioration [1]. These have been described based on their electroclinical features (age of onset, seizure type, and EEG pattern). These include Ohtahara syndrome, early myoclonic encephalopathy, epilepsy of infancy with migrating focal seizures, West syndrome, Dravet syndrome, Lennox-Gastaut syndrome, epilepsy with myoclonic atonic seizures, and Landau-Kleffner syndrome. These epilepsies are often described as “catastrophic epilepsies” as they are difficult to treat despite the use of multiple anticonvulsant drugs. They are often associated with a poor cognitive and neurodevelopmental outcome. The ketogenic diet (KD) is a high-fat, low-carbohydrate, and restricted protein diet that has been found useful in patients with refractory epilepsy [2]. One systematic review showed complete cessation of seizures in 16% of children, greater than 90% reduction in 32%, and greater than 50% reduction in 56% with the use of the KD [3]. The KD has also been found effective in children with refractory epilepsy in a randomized controlled trial [4]. A recent Cochrane review concluded that the ketogenic diet results in short- to medium-term benefits in seizure control, the effects of which are comparable to modern antiepileptic drugs [5]. The modified Atkins diet is a less restrictive alternative to KD [6]. In this diet, carbohydrates are restricted to 10 to 20 grams/day. Fats are actively encouraged, and proteins can be given unlimited. A number of studies in the last few years have shown a similar efficacy to the KD [7–10]. Approximately, half of the patients have a better than 50% improvement in seizures with this diet. The KD has been found particularly useful in some epileptic encephalopathies of young children such as West syndrome and epilepsy with myoclonic atonic seizures. In this paper, we review the use of the ketogenic diet in various epileptic encephalopathies. 2. Ohtahara Syndrome Ohtahara
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