Deep brain stimulation in myoclonus–dystonia syndrome

Authors

  • Laura Cif MD,

    1. Department of Neurosurgery (Research Group on Movement Disorders in Children) Gui de Chauliac University Hospital, Montpellier, France
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  • Enza Maria Valente MD, PhD,

    1. IRCCS CSS, San Giovanni Rotondo and CSS Mendel Institute, Rome, Italy
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  • Simone Hemm BME,

    1. Department of Neurosurgery (Research Group on Movement Disorders in Children) Gui de Chauliac University Hospital, Montpellier, France
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  • Christine Coubes MD,

    1. Department of Medical Genetics Arnaud de Villeneuve, University Hospital, Montpellier, France
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  • Nathalie Vayssiere PhD,

    1. Department of Neurosurgery (Research Group on Movement Disorders in Children) Gui de Chauliac University Hospital, Montpellier, France
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  • Stéphanie Serrat CRA,

    1. Department of Neurosurgery (Research Group on Movement Disorders in Children) Gui de Chauliac University Hospital, Montpellier, France
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  • Annalisa Di Giorgio BSc,

    1. IRCCS CSS, San Giovanni Rotondo and CSS Mendel Institute, Rome, Italy
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  • Philippe Coubes MD, PhD

    Corresponding author
    1. Department of Neurosurgery (Research Group on Movement Disorders in Children) Gui de Chauliac University Hospital, Montpellier, France
    • Service de Neurochirurgie Pédiatrique (Unité de Recherche sur les Mouvements Anormaux de l'Enfant), Hôpital Gui de Chauliac, 80, Avenue Augustin Fliche, 34295 Montpellier, France
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  • A videotape accompanies this article.

Abstract

Myoclonus–dystonia syndrome (MDS) is an autosomal dominant disorder characterized by bilateral myoclonic jerks. An 8-year-old boy presenting with early onset, medically intractable, MDS due to a mutation in the ϵ-sarcoglycan gene (SGCE) underwent chronic bilateral stimulation of the globus pallidus internus, which eliminates both myoclonus and dystonia. We conclude that deep brain stimulation can be an effective and safe treatment for MDS. © 2004 Movement Disorder Society

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