New paradigm for the treatment of glucose transporter 1 deficiency syndrome: Low glycemic index diet and modified high amylopectin cornstarch

Pediatric Neurology

Volumn 53, Issue 3, 2015, Pages 243-246

  Almuqbil, Mohammed ^a,b,c   Go, Cristina ^a   Nagy, Laura L ^a   Pai, Nisha ^a   Mamak, Eva ^a   Mercimek Mahmutoglu, Saadet ^a,d  

^a HOSPITAL FOR SICK CHILDREN   (Canada)

^b MONTREAL CHILDREN S HOSPITAL   (Canada)

^c KING SAUD BIN ABDULAZIZ UNIVERSITY FOR HEALTH SCIENCES   (Saudi Arabia)

^d HOSPITAL FOR SICK CHILDREN RESEARCH INSTITUTE   (Canada)

Author keywords

compliance; GLUT 1 deficiency syndrome; ketogenic diet; low glycemic index diet; modified high amylopectin cornstarch (Glycosade)

Indexed keywords

AMYLOPECTIN; CLOBAZAM; GLUCOSE; GLUCOSE TRANSPORTER 1; LAMOTRIGINE; MEDIUM CHAIN TRIACYLGLYCEROL; STARCH; GLUCOSE TRANSPORTER; SLC2A1 PROTEIN, HUMAN;

ADULT; ANTICONVULSANT THERAPY; ARTICLE; AUTOSOMAL DOMINANT DISORDER; CASE REPORT; COGNITION; DRUG WITHDRAWAL; FEMALE; GLUCOSE BLOOD LEVEL; GLUCOSE TRANSPORTER 1 DEFICIENCY SYNDROME; GLYCEMIC INDEX; HUMAN; KETOGENIC DIET; MOTOR PERFORMANCE; NEUROPSYCHOLOGICAL TEST; PATIENT COMPLIANCE; PRIORITY JOURNAL; SEIZURE; YOUNG ADULT; DEFICIENCY; DIET THERAPY; DISORDERS OF CARBOHYDRATE METABOLISM; GENETICS; PATHOPHYSIOLOGY; PSYCHOLOGY; SYNDROME; TREATMENT OUTCOME;

CARBOHYDRATE METABOLISM, INBORN ERRORS; GLUCOSE TRANSPORTER TYPE 1; GLYCEMIC INDEX; HUMANS; KETOGENIC DIET; MONOSACCHARIDE TRANSPORT PROTEINS; NEUROPSYCHOLOGICAL TESTS; STARCH; SYNDROME; TREATMENT OUTCOME; YOUNG ADULT;

EID: 84940452782     PISSN: 08878994     EISSN: 18735150     Source Type: Journal    
DOI: 10.1016/j.pediatrneurol.2015.06.018     Document Type: Article

References (15)

1. D.C. De Vivo, R.R. Trifiletti, R.I. Jacobson, G.M. Ronen, R.A. Behmand, and S.I. Harik Defective glucose transport across the blood-brain barrier as a cause of persistent hypoglycorrhachia, seizures, and developmental delay N Engl J Med 325 1991 703 709
2. D. Wang, J. Pascual, and D. De Vivo Glucose Transporter Type 1 Deficiency Syndrome R. Pagon, M. Adam, H. Ardinger, GeneReviews 2002 University of Washington, Seattle Seattle (WA)
3. J. Klepper, M. Garcia-Alvarez, K.R. O'Driscoll, and et al. Erythrocyte 3-O-methyl-D-glucose uptake assay for diagnosis of glucose-transporter-protein syndrome J Clin Lab Anal 13 1999 116 121
4. D.C. De Vivo, L. Leary, and D. Wang Glucose transporter 1 deficiency syndrome and other glycolytic defects J Child Neurol 17 Suppl 3 2002 3S15-23; discussion 13S24-15
5. R. Pons, A. Collins, M. Rotstein, K. Engelstad, and D.C. De Vivo The spectrum of movement disorders in Glut-1 deficiency Mov Disord 25 2010 275 281
6. H. Yang, D. Wang, K. Engelstad, and et al. Glut1 deficiency syndrome and erythrocyte glucose uptake assay Ann Neurol 70 2011 996 1005
7. T.S. Pearson, C. Akman, V.J. Hinton, K. Engelstad, and D.C. De Vivo Phenotypic spectrum of glucose transporter type 1 deficiency syndrome (Glut1 DS) Curr Neurol Neurosci Rep 13 2013 342
8. P.F. Chinnery Defining neurogenetic phenotypes (or how to compare needles in haystacks) Brain 133 2010 649 651
9. W.G. Leen, J. Klepper, M.M. Verbeek, and et al. Glucose transporter-1 deficiency syndrome: the expanding clinical and genetic spectrum of a treatable disorder Brain 133 2010 655 670
10. J. Klepper GLUT1 deficiency syndrome in clinical practice Epilepsy Res 100 2012 272 277
11. H.H. Pfeifer, and E.A. Thiele Low-glycemic-index treatment: A liberalized ketogenic diet for treatment of intractable epilepsy Neurology 65 2005 1810 1812
12. R. Gallop, and R. Gallop The Family G.I. Diet 2005 Random House Of Canada Canada
13. Coppens S, van Bogaert P. Evaluation of the efficacy of Glycosade on epilepsy in 2 children with glucose transporter GLUT-1 deficiency. 10th European Paediatric Neurology Society Congress; 25-28 September, 2013; Brussels, Belgium.
14. M. Maes, R. Van Coster, M. Van Driessche, and et al. Glut-1 deficiency syndrome: 3 case reports demonstrate clinical improvement due to a trial with glycosade, a high-amylopectin maize starch J Inherit Metab Dis 36 Suppl 2 2013 S225
15. J. Klepper, and B. Leiendecker Glut1 deficiency syndrome and novel ketogenic diets J Child Neurol 28 2013 1045 1048