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

Borel, S. , Gatignol, P. , Smail, M. , Monin, M. , Ewenczyk, C. , Bouccara, D. and Durr, A. (2019). Ann Clin Transl Neurol. doi:10.1002/acn3.50879

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.

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