Pallidal stimulation improves pantothenate kinase–associated neurodegeneration

Authors

  • Pierre Castelnau MD, PhD,

    1. Pediatric Neurology and Institut National de la Sante et de la Recherche Médicale UB19 Hôpital Gatien de Clocheville, Centre Hospitalo-Universitaire, Tours, France 619
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  • Laura Cif MD,

    1. Research Unit on Movement Disorders, Department of Neurosurgery, Hôpital Gui de Chauliac, Centre Hospitalo-Universitaire, Montpellier, France
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  • Enza Maria Valente MD, PhD,

    1. Instituto di Ricovero e cura a Carattere Scientifico, Casa Sollievo della Sofferenza, San Giovanni Rotondo and CSS Mendel Institute, Rome, Italy
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  • Nathalie Vayssiere PhD,

    1. Research Unit on Movement Disorders, Department of Neurosurgery, Hôpital Gui de Chauliac, Centre Hospitalo-Universitaire, Montpellier, France
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  • Simone Hemm PhD,

    1. Research Unit on Movement Disorders, Department of Neurosurgery, Hôpital Gui de Chauliac, Centre Hospitalo-Universitaire, Montpellier, France
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  • Amandine Gannau,

    1. Research Unit on Movement Disorders, Department of Neurosurgery, Hôpital Gui de Chauliac, Centre Hospitalo-Universitaire, Montpellier, France
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  • Annalisa DiGiorgio BSc,

    1. Instituto di Ricovero e cura a Carattere Scientifico, Casa Sollievo della Sofferenza, San Giovanni Rotondo and CSS Mendel Institute, Rome, Italy
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  • Philippe Coubes MD, PhD

    Corresponding author
    1. Research Unit on Movement Disorders, Department of Neurosurgery, Hôpital Gui de Chauliac, Centre Hospitalo-Universitaire, Montpellier, France
    • Research Unit on Movement Disorders, Department of Pediatric Neurosurgery, Hôpital Gui de Chauliac, Centre Hospitalo-Universitaire, Montpellier, France
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Abstract

Pantothenate kinase–associated neurodegeneration (PKAN) causes a progressive generalized dystonia which remains pharmacologically intractable. We performed bilateral internal globus pallidus stimulation in six patients with genetically confirmed PKAN who obtained a major and long-lasting improvement of their painful spasms, dystonia, and functional autonomy. This study shows the benefits of pallidal DBS for the dystonia of PKAN patients. Ann Neurol 2005;57:738–741

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