Oral mobility reflects rate of progression in advanced Friedreich's ataxia. Issue 9 (25th August 2019)

• Record Type: Journal Article
• Authors: Borel, Stéphanie; Gatignol, Peggy; Smail, Mustapha; Monin, Marie‐Lorraine; Ewenczyk, Claire; Bouccara, Didier; Durr, Alexandra
• Is Part Of: Annals of clinical and translational neurology. Volume 6:Issue 9(2019)
• Page Start: 1888
• Page End: 1892
• Publication Date: 2019-08-25
• Subjects: Nervous system -- Diseases -- Periodicals; Neurology -- Periodicals; 616.8005
• DOI: 10.1002/acn3.50879
• Languages: English
• ISSNs: 2328-9503

Abstract

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.