Abstract
The ketogenic diet (KD) is the first line intervention for glucose transporter 1 deficiency syndrome and pyruvate dehydrogenase deficiency, and is recommended for refractory epilepsy. It is a normo-caloric, high-fat, adequate-protein, and low-carbohydrate diet aimed at switching the brain metabolism from glucose dependence to the utilization of ketone bodies. Several variants of KD are currently available. Depending on the variant, KDs require the almost total exclusion, or a limited consumption of carbohydrates. Thus, there is total avoidance, or a limited consumption of cereal-based foods, and a reduction in fruit and vegetable intake. KDs, especially the more restrictive variants, are characterized by low variability, palatability, and tolerability, as well as by side-effects, like gastrointestinal disorders, nephrolithiasis, growth retardation, hyperlipidemia, and mineral and vitamin deficiency. In recent years, in an effort to improve the quality of life of patients on KDs, food companies have started to develop, and commercialize, several food products specific for such patients. This review summarizes the foods themselves, including sweeteners, and food products currently available for the ketogenic dietary treatment of neurological diseases. It describes the nutritional characteristics and gives indications for the use of the different products, taking into account their metabolic and health effects.
Highlights
The ketogenic diet (KD) is a normo-caloric, high-fat, adequate-protein, and low-carbohydrate dietary treatment that, according to recent international guidelines, is recommended for children and adults with drug-refractory epilepsy and two rare neurometabolic disorders: glucose transporter 1 deficiency syndrome (GLUT1-DS) and pyruvate dehydrogenase (PDH) deficiency [1,2,3]
The present review summarizes the foods and the food products available on the Italian market for the ketogenic dietary treatment of neurological diseases
We think that this article is helpful for clinicians trying to promote KDs to patients, and researchers that want to implement the diet into their studies
Summary
The ketogenic diet (KD) is a normo-caloric, high-fat, adequate-protein, and low-carbohydrate dietary treatment that, according to recent international guidelines, is recommended for children and adults with drug-refractory epilepsy and two rare neurometabolic disorders: glucose transporter 1 deficiency syndrome (GLUT1-DS) and pyruvate dehydrogenase (PDH) deficiency [1,2,3]. LCT, allowing a decrement of total fat and a higher carbohydrate and protein intake, compared to from medium-chain triglyceride (MCT) oil, which yields more ketone per kilocalorie of energy than. A recent meta-analysis of 11 studies on adult patients reveals a compliance rate of 45% for KDs [34] Such adherence decreases to 38% when considering only the CKD [35]. In order to increase KDs compliance and reduce the diet’s side effects, improving the quality of life and diet of patients treated with KDs, there has, in recent years, been an exponential increment in the availability of special food products usable for KDs. This review summarizes the food, including sweeteners, and food products for the ketogenic dietary treatment of neurological diseases, describing their nutritional characteristics, giving indications for the use of the different products, and considering their metabolic and health effects. A description is given of food products designed for KDs, and we illustrate food alternative to traditional cereal-based food
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