If the ketogenic diet is ineffective for seizure control and cognitive improvement, the ketogenic diet should be gradually reduced from a 4:1 to 3:1 to 2:1 fat/(protein + carbohydrate) ratio in all food intake until ketosis disappears. If effective, the ketogenic diet can be maintained for 2-3 years. Treatment should be prolonged in patients with glucose carrier deficiency, pyruvate dehydrogenase deficiency, and tuberous sclerosis. In patients with complete seizure control, 80% remain seizure-free after discontinuation of the ketogenic diet.
Treatment should also be discontinued if it is effective but the following conditions occur: immediate discontinuation in the presence of gastrointestinal distress, especially in acute pancreatitis; prompt adjustment of the ketogenic diet formula from 4:1-3:1-2:1 in the presence of hypertriglyceridemia with values >11.3 mmol/L. If the value remains high, the ketogenic diet should be discontinued; in the presence of hypoproteinemia, the protein supply should be adjusted, i.e., increase protein intake and further decrease carbohydrate intake, while the ratio remains unchanged; in the presence of persistent metabolic acidosis, as well as cardiomyopathy and decreased myocardial contractile function, the ketogenic diet should be discontinued.