Because the ketogenic diet alters the body's metabolism, it is a first-line therapy in children with certain congenital metabolic diseases such as pyruvate dehydrogenase (E1) deficiency and glucose transporter 1 deficiency syndrome, which prevent the body from using carbohydrates as fuel, leading to a dependency on ketone bodies. The ketogenic diet is beneficial in treating the seizures and some other symptoms in these diseases and is an absolute indication. However, it is absolutely contraindicated in the treatment of other diseases such as pyruvate carboxylase deficiency, porphyria, and other rare genetic disorders of fat metabolism. Persons with a disorder of fatty acid oxidation are unable to metabolise fatty acids, which replace carbohydrates as the major energy source on the diet. On the ketogenic diet, their bodies would consume their own protein stores for fuel, leading to ketoacidosis, and eventually coma and death.
The modified Atkins diet reduces seizure frequency by more than 50% in 43% of patients who try it and by more than 90% in 27% of patients. Few adverse effects have been reported, though cholesterol is increased and the diet has not been studied long term. Although based on a smaller data set (126 adults and children from 11 studies over five centres), these results from 2009 compare favourably with the traditional ketogenic diet.
A systematic review in 2018 looked at 16 studies on the ketogenic diet in adults. It concluded that the treatment was becoming more popular for that group of patients, that the efficacy in adults was similar to children, the side effects relatively mild. However, many patients gave up with the diet, for various reasons, and the quality of evidence was inferior to studies on children. Health issues include high levels of low-density lipoprotein, high total cholesterol, and weight loss.
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