Movement disorders in GLUT1 deficiency syndrome respond to the modified Atkins diet.

Abstract

BACKGROUND:

Movement disorders are a prominent feature of glucose transporter-1 (GLUT1) deficiency syndrome (GLUT1DS). First-choice treatment is a ketogenic diet, but compliance is poor. We have investigated the effect of the modified Atkins diet as an alternative treatment for movement disorders in GLUT1DS.

METHODS:

Four patients with GLUT1DS ages 15 to 30 years who had movement disorders as the most prominent feature were prospectively evaluated after initiation of the modified Atkins diet. Movement disorders included dystonia, ataxia, myoclonus, and spasticity, either continuous or paroxysmal, triggered by action or exercise. Duration of treatment ranged from 3 months to 16 months.

RESULTS:

All patients reached mild to moderate ketosis and experienced remarkable improvement in the frequency and severity of paroxysmal movement disorders. Cognitive function also improved subjectively.

CONCLUSIONS:

The modified Atkins diet is an effective and feasible alternative to the ketogenic diet for the treatment of GLUT1DS-related paroxysmal movement disorders in adolescence and adulthood.

• Authors: Wilhelmina G Leen, Leena Mewasingh, Marcel M Verbeek, Erik-Jan Kamsteeg, Bart P van de Warrenburg, Michel A Willemsen
• Journal: Movement disorders : official journal of the Movement Disorder Society (Mov Disord) Vol. 28 Issue 10 Pg. 1439-42 (Sep 2013) ISSN: 1531-8257 [Electronic] United States
• PMID: 23801573 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2013 International Parkinson and Movement Disorder Society.

Chemical References

• Monosaccharide Transport Proteins

Topics

• Adolescent
• Adult
• Ataxia (diet therapy, etiology)
• Carbohydrate Metabolism, Inborn Errors (diet therapy, genetics)
• Cognition Disorders (diet therapy, etiology, psychology)
• Diet, Carbohydrate-Restricted (adverse effects)
• Female
• Humans
• Ketosis (diet therapy, etiology)
• Male
• Monosaccharide Transport Proteins (deficiency, genetics)
• Movement Disorders (diet therapy, genetics)
• Myoclonus (diet therapy, etiology)
• Patient Compliance
• Seizures (etiology)
• Treatment Outcome
• Young Adult