Introduction: Epileptic encephalopathies are entities where epileptic activity is so intense that it contributes to cognitive and behavioral impairment. There are several syndromes that fall into this category such as: West syndrome, Ohtahara syndrome, Dravet syndrome, Doose syndrome and Landau-Kleffner syndrome. Objective: presentation of the clinical indications of the ketogenic diet (CD) as a therapeutic measure in childhood refractory epilepsies, demonstrating the efficacy and side effects expected from this practice. Methodology: a narrative review of the scientific literature (PUBMED and SCIELO) was carried out, with a selection of articles published in the last ten years, also considering renowned magazines and newspapers in the areas of Clinical Neurology, Neuropediatrics and epilepsy. Results: A randomized and controlled clinical trial, conducted in children aged 2 to 16 years with refractory epilepsy, demonstrated that after 3 months, 38% of patients using CD had more than 50% reduction in seizures, compared with four ( 6%) of the control group (p <00001). Corroborating this finding, a Brazilian study demonstrated that 60% of patients with refractory epilepsy and using CD had more than 50% reduction in seizure frequency and 10% were seizure-free. Conclusions: The use of the ketogenic diet demonstrated a favorable result in children with epilepsy refractory to drug treatment. In cases of deficiency in the type 1 glucose transporter and deficiency of pyruvate dehydrogenase, CD should be the treatment of first choice. In cases such as Dravet’s Syndrome, West’s Syndrome, Ohtahara’s Syndrome, Lennox- Gastaut Syndrome and DC Dose Syndrome is part of the therapeutic arsenal.
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