Funding agencies: The first author has received a grant for performing research on the subject of GLUT1 deficiency syndrome (NWO, ZonMW, “AGIKO-stipendium”; grant number 92003529 to W. G. Leen).
Movement disorders in GLUT1 deficiency syndrome respond to the modified Atkins diet
Article first published online: 25 JUN 2013
© 2013 International Parkinson and Movement Disorder Society
Volume 28, Issue 10, pages 1439–1442, September 2013
How to Cite
Leen, W. G., Mewasingh, L., Verbeek, M. M., Kamsteeg, E.-J., van de Warrenburg, B. P. and Willemsen, M. A. (2013), Movement disorders in GLUT1 deficiency syndrome respond to the modified Atkins diet. Mov. Disord., 28: 1439–1442. doi: 10.1002/mds.25515
Relevant conflicts of interest/financial disclosures: Nothing to report.
Full financial disclosures and author roles may be found in the online version of this article.
- Issue published online: 23 SEP 2013
- Article first published online: 25 JUN 2013
- Manuscript Accepted: 17 APR 2013
- Manuscript Revised: 15 APR 2013
- Manuscript Received: 12 FEB 2013
- GLUT1 deficiency syndrome;
- modified Atkins diet;
- paroxysmal exercise induced dyskinesia;
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.
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.
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.
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. © 2013 International Parkinson and Movement Disorder Society