title: Glut1 deficiency syndrome and novel ketogenic diets.
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.
authors.affiliation: Children's Hospital Aschaffenburg, Am Hasenkopf, Aschaffenburg, Germany. joerg.klepper@klinikum-aschaffenburg.de
authors.forename: Joerg
authors.initials: J
authors.lastname: Klepper
authors.forename: Baerbel
authors.initials: B
authors.lastname: Leiendecker
mesh_terms: D020157:Amino Acid Transport Disorders, Inborn / Q000178:diet therapy*; D055423:Diet, Ketogenic / Q000379:methods*; D051272:Glucose Transporter Type 1 / Q000172:deficiency*; D006801:Humans
chemical_list: D051272:Glucose Transporter Type 1
keywords: Glut1 deficiency syndrome; intractable childhood epilepsy; ketogenic diet; low glycemic index treatment; modified Atkins diet