Food and Food Products on the Italian Market for Ketogenic Dietary Treatment of Neurological Diseases

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.

The Effects of Ketogenic Diet Treatment in Kcna1-Null Mouse, a Model of Sudden Unexpected Death in Epilepsy

“…Despite lack of direct clinical evidence about the efficacy of KD in SUDEP in Humans, KD reveals an effective treatment for refractory epilepsy. It can reduce seizure frequency (14), decrease seizure severity, and improve cognitive ability (15), cardiac dysfunction and quality of life (16). Thus, the KD appears to be highly effective in treating refractory epilepsy.…”

“…Kv1.1 alpha subunit protein is mainly expressed in the brain, but it reveals lower levels in heart and almost is not expressed in lung tissue in mice (14). Due to Kv1.1 alpha subunit protein highly expressed in neural tissue, its presence and function are established in the vagal and phrenic nerves.…”

“…Furthermore, other researchers have showed that the KD has a protection against oxidative stress and mitochondrial dysfunction derived from epilepsy. These effects might be caused by diminishing reactive oxygen species and raise the mitochondrial uncoupling protein activity and the biosynthesis of glutathione (14, 38, 39).…”

Neuroendocrine and Metabolic Effects of Low-Calorie and Non-Calorie Sweeteners

“…Sugar alcohols (also referred to as polyols) are characterized by a lower calorie content (2 to 4 kcal/g) (44) than sucrose (55)(56)(57)(58)(59)(60)(61)(62)(63)(64)(65)(66)(67)(68)(69), or high GI (≥70) (47). c Source (1,48). d Source (45).…”

“…According to the most commonly used GI classification, foods are categorized as having a low ( ≤ 55), medium (55–69), or high GI (≥70) ( 47 ) . c Source ( 1 , 48 ) . d Source ( 45 ) .…”