Speech disturbances in patients with dystonia or chorea due to neurometabolic disorders.
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.
Flamand-Rouvière
Constance
C
Service de Neurologie, CHU de Bicêtre, AP-HP, Le Kremlin Bicêtre, France. constance.rouviere@bct.aphp.fr
Guettard
Emilie
E
Moreau
Caroline
C
Bahi-Buisson
Nadia
N
Valayannopoulos
Vassili
V
Grabli
David
D
Motte
Jacques
J
Rodriguez
Diana
D
Roubertie
Agathe
A
Maintigneux
Lisa
L
Kemlin
Isabelle
I
Ceballos-Picot
Irène
I
Adams
David
D
Vidailhet
Marie
M
Roze
Emmanuel
E
D000293:Adolescent; D000328:Adult; D001928:Brain Diseases, Metabolic / Q000150:complications*; D002648:Child; D002675:Child, Preschool; D002819:Chorea / Q000150:complications* / Q000209:etiology*; D004421:Dystonia / Q000150:complications* / Q000209:etiology*; D005192:Family Health; D005260:Female; D006801:Humans; D008297:Male; D009460:Neurologic Examination / Q000379:methods; D013064:Speech Disorders / Q000209:etiology*; D055815:Young Adult