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Pediatr Neurol. 2015 Sep;53(3):243-6. doi: 10.1016/j.pediatrneurol.2015.06.018. Epub 2015 Jun 26.

New Paradigm for the Treatment of Glucose Transporter 1 Deficiency Syndrome: Low Glycemic Index Diet and Modified High Amylopectin Cornstarch.

Author information

1
Division of Clinical and Metabolic Genetics, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Canada; Division of Pediatric Neurology, Montreal Children's Hospital-McGill University Health Center, Montreal, Canada; Division of Pediatric Neurology, King Saud bin Abdulaziz University for Health Sciences, Riyadh, Saudi Arabia.
2
Division of Neurology, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Canada.
3
Division of Clinical and Metabolic Genetics, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Canada; Department of Clinical Dietetics, University of Toronto, The Hospital for Sick Children, Toronto, Canada.
4
Department of Psychology, University of Toronto, The Hospital for Sick Children, Toronto, Canada.
5
Division of Clinical and Metabolic Genetics, Department of Pediatrics, University of Toronto, The Hospital for Sick Children, Toronto, Canada; Genetics and Genome Biology Research Program, Research Institute, The Hospital for Sick Children, Toronto, Canada. Electronic address: saadet.mahmutoglu@sickkids.ca.

Abstract

OBJECTIVE:

Glucose transporter 1 deficiency syndrome is an autosomal, dominantly inherited neurometabolic disorder caused by mutations in the SLC2A1 gene. Decreased glucose transport into the brain results in seizures and cognitive dysfunction. The ketogenic diet is the treatment of choice, but complicated with compliance problems. Stabilization of blood glucose levels by low glycemic index diet and modified high amylopectin cornstarch would provide steady-state glucose transport into the brain to prevent seizures and cognitive dysfunction in patients with glucose transporter 1 deficiency syndrome as an alternative treatment.

PATIENT:

We report a new glucose transporter 1 deficiency syndrome patient (c.988C>T; p. Arg330X in the SLC2A1) treated with modified high amylopectin cornstarch (Glycosade) and low glycemic index diet because of compliance problems with the ketogenic diet. She was diagnosed at 11.5 years of age and was treated with the ketogenic diet between ages 12 and 18 years.

RESULTS:

She was started on modified high amylopectin cornstarch at bedtime and low glycemic index diet with meals and snacks every 3-4 hours. Within the first 6 months of therapy, she improved in her seizures and cognitive functions, but experienced compliance problems afterwards. Neuropsychological assessment was stable at 12 months of therapy.

CONCLUSION:

This diet was easy to apply compared with the ketogenic diet and resulted in stable neuropsychological functioning of this glucose transporter 1 deficiency syndrome patient. Modified high amylopectin cornstarch and low glycemic index diet might be an alternative treatment in glucose transporter 1 deficiency syndrome patients with compliance problems to the ketogenic diet treatment, but additional patients should be treated to prove usefulness of this new treatment.

KEYWORDS:

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

[Indexed for MEDLINE]

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