Ketogenic diet in neuromuscular and neurodegenerative diseases

Biomed Res Int. 2014:2014:474296. doi: 10.1155/2014/474296. Epub 2014 Jul 3.


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.

Publication types

  • Review

MeSH terms

  • 3-Hydroxybutyric Acid / metabolism*
  • Alzheimer Disease / diet therapy
  • Alzheimer Disease / metabolism
  • Amyotrophic Lateral Sclerosis / diet therapy
  • Amyotrophic Lateral Sclerosis / metabolism
  • Brain / metabolism
  • Diet, Ketogenic*
  • Glucose / metabolism*
  • Glycogen Storage Disease / diet therapy
  • Glycogen Storage Disease / metabolism
  • Humans
  • Mitochondrial Diseases / diet therapy*
  • Mitochondrial Diseases / metabolism
  • Parkinson Disease / diet therapy
  • Parkinson Disease / metabolism


  • Glucose
  • 3-Hydroxybutyric Acid