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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.

SUBMITTER: Borel S 

PROVIDER: S-EPMC6764486 | biostudies-other | 2019 Sep

REPOSITORIES: biostudies-other

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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]