Speech disturbances in patients with dystonia or chorea due to neurometabolic disorders
Identifieur interne : 001998 ( Main/Curation ); précédent : 001997; suivant : 001999Speech disturbances in patients with dystonia or chorea due to neurometabolic disorders
Auteurs : Constance Flamand-Rouvière [France] ; Emilie Guettard [France] ; Caroline Moreau [France] ; Nadia Bahi-Buisson [France] ; Vassili Valayannopoulos [France] ; David Grabli [France] ; Jacques Motte [France] ; Diana Rodriguez [France] ; Agathe Roubertie [France] ; Lisa Maintigneux [France] ; Isabelle Kemlin [France] ; Irène Ceballos-Picot [France] ; David Adams [France] ; Marie Vidailhet [France] ; Emmanuel Roze [France]Source :
- Movement Disorders [ 0885-3185 ] ; 2010-08-15.
English descriptors
- KwdEn :
- Adolescent, Adult, Brain Diseases, Metabolic (complications), Child, Child, Preschool, Chorea (complications), Chorea (etiology), Dystonia (complications), Dystonia (etiology), Family Health, Female, GM1 gangliosidosis type 3, Humans, Lesch‐Nyhan disease, Male, Neurologic Examination (methods), Speech Disorders (etiology), Young Adult, dystonia, glutaric aciduria type 1, hyperkinetic dysarthria, speech apraxia.
- MESH :
- complications : Brain Diseases, Metabolic, Chorea, Dystonia.
- etiology : Chorea, Dystonia, Speech Disorders.
- methods : Neurologic Examination.
- Adolescent, Adult, Child, Child, Preschool, Family Health, Female, Humans, Male, Young Adult.
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
Url:
DOI: 10.1002/mds.23134
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<term>Child</term>
<term>Child, Preschool</term>
<term>Chorea (complications)</term>
<term>Chorea (etiology)</term>
<term>Dystonia (complications)</term>
<term>Dystonia (etiology)</term>
<term>Family Health</term>
<term>Female</term>
<term>GM1 gangliosidosis type 3</term>
<term>Humans</term>
<term>Lesch‐Nyhan disease</term>
<term>Male</term>
<term>Neurologic Examination (methods)</term>
<term>Speech Disorders (etiology)</term>
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<term>glutaric aciduria type 1</term>
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<term>speech apraxia</term>
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<front><div type="abstract" xml:lang="en">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</div>
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