title: Modified Atkins diet therapy for a case with glucose transporter type 1 deficiency syndrome.
abstract: Glucose transporter type 1 deficiency syndrome (GLUT-1 DS), giving rise to impaired glucose transport across the blood-brain barrier, is characterized by infantile seizures, complex motor disorders, global developmental delay, acquired microcephaly, and hypoglycorrhachia. GLUT-1 DS can be treated effectively with a ketogenic diet because it can provide an alternative fuel for brain metabolism; however, the excessive restriction of food intake involved frequently makes it difficult for patients to initiate or continue the diet. Recently, the modified Atkins diet, which is much less restrictive in terms of the total calorie and protein intake than the classical ketogenic diet, has been shown to be effective and well tolerated in children with intractable epilepsy. We successfully introduced the modified Atkins diet to a 7-year-old boy with GLUT-1 DS, whose caregivers refused ketogenic diet treatment because of strong concerns over restricting the diet. The modified Atkins diet should be considered for patients with GLUT-1 DS as an alternative to the traditional ketogenic diet.
authors.affiliation: Department of Pediatrics, Tokyo Women's Medical University, 8-1, Tokyo 162-8666, Japan. susumu.ito@nifty.com
authors.forename: Susumu
authors.initials: S
authors.lastname: Ito
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Oguni
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Ito
authors.forename: Keiko
authors.initials: K
authors.lastname: Ishigaki
authors.forename: Junko
authors.initials: J
authors.lastname: Ohinata
authors.forename: Makiko
authors.initials: M
authors.lastname: Osawa
mesh_terms: D002239:Carbohydrate Metabolism, Inborn Errors / Q000178:diet therapy* / Q000378:metabolism*; D002648:Child; D050528:Diet, Carbohydrate-Restricted / Q000379:methods*; D051272:Glucose Transporter Type 1 / Q000172:deficiency*; D006801:Humans; D008297:Male; D013577:Syndrome
chemical_list: D051272:Glucose Transporter Type 1