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Bilateral pallidal stimulation for Wilson's disease

Authors

  • Christos Sidiropoulos MD,

    Corresponding author
    1. Parkinson's Disease and Movement Disorders Program, Henry Ford Hospital, West Bloomfield, Michigan, USA
    2. Department of Physiology, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada
    • Movement Disorders Centre, Toronto Western Hospital, Toronto, Ontario, Canada
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  • William Hutchison PhD,

    1. Department of Physiology, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada
    2. Department of Surgery, Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada
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  • Tiago Mestre MD, MSc,

    1. Movement Disorders Centre, Toronto Western Hospital, Toronto, Ontario, Canada
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  • Elena Moro MD, PhD,

    1. Movement Disorders Centre, Toronto Western Hospital, Toronto, Ontario, Canada
    2. Department of Psychiatry and Neurology, Movement Disorders Unit, University Hospital Center (CHU) of Grenoble, Grenoble, France
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  • Ian A. Prescott MSc,

    1. Department of Physiology, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada
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  • Alejandro Valencia Mizrachi NP,

    1. Movement Disorders Centre, Toronto Western Hospital, Toronto, Ontario, Canada
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  • Melanie Fallis RN,

    1. Movement Disorders Centre, Toronto Western Hospital, Toronto, Ontario, Canada
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  • Anand I. Rughani MD,

    1. Department of Surgery, Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada
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  • Suneil K. Kalia MD, PhD,

    1. Department of Surgery, Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada
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  • Andres Lozano MD, PhD, FRCSC,

    1. Department of Surgery, Division of Neurosurgery, University of Toronto, Toronto Western Hospital, Toronto, Ontario, Canada
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  • Susan Fox MD, PhD

    1. Movement Disorders Centre, Toronto Western Hospital, Toronto, Ontario, Canada
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  • 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.

Correspondence to: Dr. Christos Sidiropoulos, Parkinson's Disease and Movement Disorders Program, Henry Ford Hospital, 6777 West Maple Road, West Bloomfield, MI 48322; csidiro1@hfhs.org

ABSTRACT

Background

To report on the clinical efficacy of bilateral globus pallidus internus deep brain stimulation in a 29-year-old patient with severe generalized dystonia secondary to Wilson's disease.

Methods

The primary outcome measure was the Burke-Fahn-Marsden Dystonia Scale motor severity score (blinded assessment) and the secondary outcome measures were the Abnormal Involuntary Movement Scale (blinded assessment) and the Zaritt Caregiver Burden Interview score, at 20-week postoperative follow up.

Results

There was a 14% improvement in the Burke-Fahn-Marsden Dystonia Scale motor severity score. Abnormal Involuntary Movement Scale score remained unchanged while the Zaritt Caregiver Burden Interview score improved by 44.4%.

Conclusions

Bilateral globus pallidus deep brain stimulation can be effective in ameliorating dystonia and caregiver burden in Wilson's disease. Outcomes may depend on the stage of the disease at which the surgical procedure is completed. © 2013 Movement Disorder Society

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