Interruption of deep brain stimulation of the globus pallidus in primary generalized dystonia

Authors

  • David Grabli MD, PhD,

    Corresponding author
    1. Neurology Department and Centre de Référence des Maladies Neurogénétiques, Hôpital de la Salpétrière, Paris, France
    2. INSERM/Pierre et Marie Curie University UMR 679, Experimental Neurology and Therapeutics, Hôpital de la Salpétrière, Paris, France
    • Fédération des Maladies du Système Nerveux, Hopital de la Salpétrière, 47–83 Boulevard de l'Hôpital, Paris, Cedex 13 75651, France
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    • David Grabli and Claire Ewenczyk contributed equally to this work.

  • Claire Ewenczyk MD,

    1. Neurology Department and Centre de Référence des Maladies Neurogénétiques, Hôpital de la Salpétrière, Paris, France
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    • David Grabli and Claire Ewenczyk contributed equally to this work.

  • Maria-Clara Coelho-Braga MD,

    1. Neurology Department, Grenoble University Hospital, Grenoble, France
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  • Christelle Lagrange PhD,

    1. Neurology Department, Grenoble University Hospital, Grenoble, France
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  • Valerie Fraix MD,

    1. Neurology Department, Grenoble University Hospital, Grenoble, France
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  • Philippe Cornu MD,

    1. Neurosurgery Department, Hôpital de la Salpétrière, Paris, France
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  • Alim-Louis Benabid MD, PhD,

    1. Neurosurgery Department, Grenoble University Hospital, Grenoble, France
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  • Marie Vidailhet MD,

    1. Neurology Department and Centre de Référence des Maladies Neurogénétiques, Hôpital de la Salpétrière, Paris, France
    2. INSERM/Pierre et Marie Curie University UMR 679, Experimental Neurology and Therapeutics, Hôpital de la Salpétrière, Paris, France
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  • Pierre Pollak MD

    1. Neurology Department, Grenoble University Hospital, Grenoble, France
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  • Potential conflict of interest: P. Cornu reports receiving consulting fees from Medtronic. A.L. Benabid reports receiving lecture fees and grant support from Medtronic. P. Pollak reports receiving consulting fees from Medtronic.

Abstract

Stimulation (DBS) of the globus pallidus (GP) is effective to treat generalized dystonia. Little is known about the evolution of dystonia in case of arrest after a long period of stimulation. This study describes the course of dystonia during a 48 hours period without stimulation followed by a 24 hours period after turning ON the stimulator. 14 patients with generalized dystonia treated with bilateral GP DBS for 3 years or more were recruited. Blinded video-based analysis was performed using Burke-Fahn-Marsden scale at (1) baseline (ON stimulation), (2) up to 48 hours after the stimulator was turned OFF, and (3) 24 hours after the stimulator was turned ON. 13 patients completed the 48 hours OFF-stimulation period. The dystonia movement score progressively worsened from 24.3 ± 13.9 at baseline to 48.9 ± 19.8 after 48 hours (P < 0.00001). The disability score also worsened from 4.4 ± 1.2 at baseline to 5.7 ± 1.5 after 48 hours without stimulation (P < 0.001). When the neurostimulator was turned ON, the dystonia scores returned to baseline level after 10 hours. The interruption of GP DBS in dystonia results in a progressive worsening which is rapidly reversible once the neurostimulator is turned ON. © 2009 Movement Disorder Society

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