Oral mobility reflects rate of progression in advanced Friedreich’s ataxia - Réhabilitation Chirurgicale mini-Invasive et Robotisée de l'Audition Access content directly
Journal Articles Annals of Clinical and Translational Neurology Year : 2019

Oral mobility reflects rate of progression in advanced Friedreich’s ataxia

Abstract

Our objective was to identify a sensitive marker of disease progression in Friedreich’s ataxia. We prospectively evaluated speech, voice, and oromotor function in 40 patients at two timepoints. The mean disease duration was 20.8 ± 9.8 years and mean SARA score 23.7 ± 8.6 at baseline. Oral motor mobility, assessed by a combination of movements of the face, eyes, cheeks, lips, and tongue, decreased significantly after 1 year (P < 0.0001). The standardized response mean over 12 months was considered as large for oral mobility (1.26) but small for SARA (0.12). Oral mobility could therefore be a sensitive marker in therapeutic trials.
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Dates and versions

hal-02289471 , version 1 (16-09-2019)

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Stéphanie Borel, Peggy Gatignol, Mustapha Smail, Marie-Lorraine Monin, Claire Ewenczyk, et al.. Oral mobility reflects rate of progression in advanced Friedreich’s ataxia. Annals of Clinical and Translational Neurology, inPress, ⟨10.1002/acn3.50879⟩. ⟨hal-02289471⟩
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