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BioMed Research International
Volume 2014, Article ID 474296, 10 pages
http://dx.doi.org/10.1155/2014/474296
Review Article

Ketogenic Diet in Neuromuscular and Neurodegenerative Diseases

1Department of Biomedical Sciences, University of Padova, Via Marzolo 3, 35031 Padova, Italy
2Sport and Exercise Sciences Research Unit, University of Palermo, Via Eleonora Duse 2, 90146 Palermo, Italy

Received 24 April 2014; Accepted 30 May 2014; Published 3 July 2014

Academic Editor: Giuseppe D’Antona

Copyright © 2014 Antonio Paoli et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

An increasing number of data demonstrate the utility of ketogenic diets in a variety of metabolic diseases as obesity, metabolic syndrome, and diabetes. In regard to neurological disorders, ketogenic diet is recognized as an effective treatment for pharmacoresistant epilepsy but emerging data suggests that ketogenic diet could be also useful in amyotrophic lateral sclerosis, Alzheimer, Parkinson’s disease, and some mitochondriopathies. Although these diseases have different pathogenesis and features, there are some common mechanisms that could explain the effects of ketogenic diets. These mechanisms are to provide an efficient source of energy for the treatment of certain types of neurodegenerative diseases characterized by focal brain hypometabolism; to decrease the oxidative damage associated with various kinds of metabolic stress; to increase the mitochondrial biogenesis pathways; and to take advantage of the capacity of ketones to bypass the defect in complex I activity implicated in some neurological diseases. These mechanisms will be discussed in this review.