Speech disturbances in patients with dystonia or chorea due to neurometabolic disorders

Authors

  • Constance Flamand-Rouvière ST,

    Corresponding author
    1. Service de Neurologie, CHU de Bicêtre, AP-HP, Le Kremlin Bicêtre, France
    • Service de Neurologie, Centre, Hospitalier Universitaire de Bicêtre, 78 rue du Général Leclerc, Le Kremlin Bicêtre Cedex 94275, France
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  • Emilie Guettard MD,

    1. Service de médecine physique et de réadaptation, Hôpital Fernand Widal, AP-HP, Paris, France
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  • Caroline Moreau MD,

    1. Service de neurologie et pathologie du mouvement, IMPRT, Lille EA 2683, IFR 114, France
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  • Nadia Bahi-Buisson MD, PHD,

    1. Service Neurologie Pédiatrique et Maladies métaboliques, Hôpital Necker Enfants Malades, Université Paris-Descartes, AP-HP, Paris, France
    2. Commission Mouvements Anormaux, de la Société Française de Neurologie Pédiatrique, France
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  • Vassili Valayannopoulos MD,

    1. Service Neurologie Pédiatrique et Maladies métaboliques, Hôpital Necker Enfants Malades, Université Paris-Descartes, AP-HP, Paris, France
    2. Centre de Référence des Maladies Héréditaires du Métabolisme, Hôpital Necker Enfants Malades, Université Paris-Descartes, Paris, France
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  • David Grabli MD, PHD,

    1. Pôle des maladies du système nerveux, Fédération de Neurologie, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris, France
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  • Jacques Motte MD, PHD,

    1. Unité de neurologie pédiatrique, American Memorial Hospital, CHU Reims, France
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  • Diana Rodriguez MD, PHD,

    1. Service de neuropédiatrie, Université Pierre et Marie Curie, Paris VI, Hôpital Armand Trousseau, AP-HP, Paris, France
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  • Agathe Roubertie MD, PHD,

    1. Service de Neurologie Pédiatrique, CHU, Montpellier, France
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  • Lisa Maintigneux ST,

    1. Service de Neurologie, CHU de Bicêtre, AP-HP, Le Kremlin Bicêtre, France
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  • Isabelle Kemlin MD,

    1. Service de neuropédiatrie, Université Pierre et Marie Curie, Paris VI, Hôpital Armand Trousseau, AP-HP, Paris, France
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  • Irène Ceballos-Picot PHARMD, PHD,

    1. Service de Biochimie Métabolique, Hôpital Necker Enfants Malades, Université Paris-Descartes, AP-HP, Paris, France
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  • David Adams MD, PHD,

    1. Service de Neurologie, CHU de Bicêtre, AP-HP, Le Kremlin Bicêtre, France
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  • Marie Vidailhet MD,

    1. Pôle des maladies du système nerveux, Fédération de Neurologie, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris, France
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  • Emmanuel Roze MD, PHD

    1. Commission Mouvements Anormaux, de la Société Française de Neurologie Pédiatrique, France
    2. Pôle des maladies du système nerveux, Fédération de Neurologie, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris, France
    3. Centre d'Investigation Clinique, 9503, INSERM, AP-HP, Paris, France
    4. CNRS UMRS 952, INSERM UMR 7224, Université Pierre et Marie Curie Paris VI, Paris, France
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  • Potential conflict of interest: Nothing to report.

Abstract

Speech disturbances are frequent and potentially disabling in patients with dystonia or chorea due to neurometabolic disorders (DCND), but their precise characteristics are poorly documented. We prospectively studied 29 consecutive patients with DCND. A detailed description of their speech patterns was obtained by using the Frenchay dysarthria assessment test and the apraxia of speech evaluation test of Wertz. Gross motor function and intelligibility were each scored on 5-point scales to identify a possible correlation between the severity of the speech and motor disorders. All the patients were found to have complex speech alterations with combined features of hyperkinetic dysarthria and speech apraxia. We also noted a correlation between the severity of the speech disorders and the motor disorders. These findings have important implications for speech rehabilitation, and may provide new insights into the pathophysiology of dystonia due to neurometabolic disorders. © 2010 Movement Disorder Society

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