Oral mobility reflects rate of progression in advanced Friedreich's ataxia.
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ABSTRACT:
SUBMITTER: Borel S
PROVIDER: S-EPMC6764486 | biostudies-other | 2019 Sep
REPOSITORIES: biostudies-other
Publications
Oral mobility reflects rate of progression in advanced Friedreich's ataxia.
Borel Stéphanie S Gatignol Peggy P Smail Mustapha M Monin Marie-Lorraine ML Ewenczyk Claire C Bouccara Didier D Durr Alexandra A
Annals of clinical and translational neurology 20190825 9
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 considere ...[more]
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