Glut1 Deficiency Syndrome and Novel Ketogenic Diets. (August 2013)

• Record Type: Journal Article
• Authors: Klepper, Joerg; Leiendecker, Baerbel
• Is Part Of: Journal of child neurology. Volume 28:Number 8(2013)
• Page Start: 1045
• Page End: 1048
• Publication Date: 2013-08
• Subjects: Glut1 deficiency syndrome -- ketogenic diet -- modified Atkins diet -- low glycemic index treatment -- intractable childhood epilepsy; Nervous system -- Diseases -- Periodicals; 618.928
• DOI: 10.1177/0883073813487600
• Languages: English
• ISSNs: 0883-0738

Abstract

The classical ketogenic diet has been used for refractory childhood epilepsy for decades. It is also the treatment of choice for disorders of brain energy metabolism, such as Glut1 deficiency syndrome. Novel ketogenic diets such as the modified Atkins diet and the low glycemic index treatment have significantly improved the therapeutic options for dietary treatment. Benefits of these novel diets are increased palatability, practicability, and thus compliance—at the expense of lower ketosis. As high ketones appear essential to meet the brain energy deficit caused by Glut1 deficiency syndrome, the use of novel ketogenic diets in this entity may be limited. This article discusses the current data on novel ketogenic diets and the implications on the use of these diets in regard to Glut1 deficiency syndrome.