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

Authors

  • Wilhelmina G. Leen MD,

    Corresponding author
    1. Department of Neurology, Radboud University Medical Centre, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, the Netherlands
    • Correspondence to: Dr. Wilhelmina G. Leen, Department of Neurology, Radboud University Medical Centre, 935 Neurology, PO Box 9101, 6500 HB Nijmegen, the Netherlands; w.leen@neuro.umcn.nl.

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  • Leena Mewasingh MD,

    1. Department of Paediatric Neurology, Imperial College Healthcare National Health Service Trust, London, United Kingdom
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  • Marcel M. Verbeek PhD,

    1. Department of Neurology, Radboud University Medical Centre, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, the Netherlands
    2. Department of Laboratory Medicine, Radboud University Medical Centre, Nijmegen, the Netherlands
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  • Erik-Jan Kamsteeg PhD,

    1. Department of Genetics, Radboud University Medical Centre, the Netherlands
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  • Bart P. van de Warrenburg MD, PhD,

    1. Department of Neurology, Radboud University Medical Centre, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, the Netherlands
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  • Michel A. Willemsen MD, PhD

    1. Department of Paediatric Neurology, Radboud University Medical Centre, Donders Institute for Brain, Cognition, and Behaviour, Nijmegen, the Netherlands
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  • 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).

  • 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.

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. © 2013 International Parkinson and Movement Disorder Society

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