title: Oxidative stress markers and phosphorus magnetic resonance spectroscopy in a patient with GLUT1 deficiency treated with modified Atkins diet.
abstract: Glucose transporter type 1 deficiency syndrome is an inborn error of glucose transport across blood-tissue barriers, and the modified Atkins diet is an effective and well-tolerated treatment. To investigate the effects of the modified Atkins diet, we examined the cerebrospinal fluid markers and performed phosphorus magnetic resonance spectroscopy in a patient with glucose transporter type 1 deficiency syndrome before and after the modified Atkins diet. Cerebrospinal fluid levels of the oxidative stress markers, 8-hydroxy-2'-deoxyguanosine and hexanoyl-lysine adduct, were markedly increased above the cutoff index and were normalized 18 months after the modified Atkins diet. Phosphorus magnetic resonance spectroscopy measurements showed 18% increase of PCr/γ-ATP ratio after the modified Atkins diet. These results suggest that the modified Atkins diet may reduce oxidative stress in the brain and improve energy reserve capacity, which is important in sustaining electrophysiological activities essential for performing brain functions.
authors: Department of Pediatrics, Juntendo University School of Medicine, Japan. yuri-kitamura@hotmail.co.jp
authors: Yuri
authors: Y
authors: Kitamura
authors: Akihisa
authors: A
authors: Okumura
authors: Masaharu
authors: M
authors: Hayashi
authors: Harushi
authors: H
authors: Mori
authors: Satoru
authors: S
authors: Takahashi
authors: Keiko
authors: K
authors: Yanagihara
authors: Rie
authors: R
authors: Miyata
authors: Naoyuki
authors: N
authors: Tanuma
authors: Takashi
authors: T
authors: Mimaki
authors: Shinpei
authors: S
authors: Abe
authors: Toshiaki
authors: T
authors: Shimizu
mesh_terms: D015415:Biomarkers / Q000134:cerebrospinal fluid; D001921:Brain / Q000378:metabolism; D002239:Carbohydrate Metabolism, Inborn Errors / Q000178:diet therapy* / Q000378:metabolism*; D002675:Child, Preschool; D050528:Diet, Carbohydrate-Restricted / Q000379:methods*; D005260:Female; D051272:Glucose Transporter Type 1 / Q000172:deficiency*; D006801:Humans; D009682:Magnetic Resonance Spectroscopy / Q000379:methods*; D018384:Oxidative Stress / Q000502:physiology*
chemical_list: D015415:Biomarkers; D051272:Glucose Transporter Type 1; C495677:SLC2A1 protein, human