Long-term follow-up of DYT1 dystonia patients treated by deep brain stimulation: An open-label study

Authors

  • Laura Cif MD,

    Corresponding author
    1. CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France
    2. INSERM, U661, Montpellier, France
    3. Université de Montpellier 1, Montpellier, France
    4. CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier, France
    • Service de Neurochirurgie, Hôpital Gui de Chauliac, 80, Avenue Augustin Fliche, Montepellier, 34295 Cedex 5, France===

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  • Xavier Vasques PhD,

    1. INSERM, U661, Montpellier, France
    2. Université de Montpellier 1, Montpellier, France
    3. CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier, France
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  • Victoria Gonzalez MD,

    1. CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France
    2. INSERM, U661, Montpellier, France
    3. Université de Montpellier 1, Montpellier, France
    4. CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier, France
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  • Patrice Ravel PhD,

    1. Université de Montpellier 1, Montpellier, France
    2. CNRS UMR5048, Centre de Biochimie Structurale, Montpellier, France
    3. INSERM, U554, Montpellier, France
    4. Université de Montpellier 2, Montpellier, France
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  • Brigitte Biolsi MD,

    1. CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France
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  • Gwenaelle Collod-Beroud PhD,

    1. Université de Montpellier 1, Montpellier, France
    2. INSERM, U827, Montpellier, France
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  • Sylvie Tuffery-Giraud PhD,

    1. Université de Montpellier 1, Montpellier, France
    2. INSERM, U827, Montpellier, France
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  • Hassan Elfertit MD,

    1. CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France
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  • Mireille Claustres MD, PhD,

    1. Université de Montpellier 1, Montpellier, France
    2. INSERM, U827, Montpellier, France
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  • Philippe Coubes MD, PhD

    1. CHRU Montpellier, Hôpital Gui de Chauliac, Service de Neurochirurgie, Montpellier, France
    2. INSERM, U661, Montpellier, France
    3. Université de Montpellier 1, Montpellier, France
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  • Potential conflict of interest: Nothing to report

Abstract

Long-term efficacy of internal globus pallidus (GPi) deep-brain stimulation (DBS) in DYT1 dystonia and disease progression under DBS was studied. Twenty-six patients of this open-label study were divided into two groups: (A) with single bilateral GPi lead, (B) with a second bilateral GPi lead implanted owning to subsequent worsening of symptomatology. Dystonia was assessed with the Burke Scale. Appearance of new symptoms and distribution according to body region were recorded. In the whole cohort, significant decreases in motor and disability subscores (P < 0.0001) were observed at 1 year and maintained up to 10 years. Group B showed worsening of the symptoms. At 1 year, there were no significant differences between Groups A (without subsequent worsening) and B; at 5 years, a significant difference was found for motor and disability scores. Within Group B, four patients exhibited additional improvement after the second DBS surgery. In the 26 patients, significant difference (P = 0.001) was found between the number of body regions affected by dystonia preoperatively and over the whole follow-up. DBS efficacy in DYT1 dystonia can be maintained up to 10 years (two patients). New symptoms appear with long-term follow-up and may improve with additional leads in a subgroup of patients. © 2009 Movement Disorder Society

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