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

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 mobi...

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Autores principales: Borel, Stéphanie, Gatignol, Peggy, Smail, Mustapha, Monin, Marie‐Lorraine, Ewenczyk, Claire, Bouccara, Didier, Durr, Alexandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley and Sons Inc. 2019
Materias:
Brief Communications
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6764486/
https://www.ncbi.nlm.nih.gov/pubmed/31448573
http://dx.doi.org/10.1002/acn3.50879
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author Borel, Stéphanie
Gatignol, Peggy
Smail, Mustapha
Monin, Marie‐Lorraine
Ewenczyk, Claire
Bouccara, Didier
Durr, Alexandra
author_facet Borel, Stéphanie
Gatignol, Peggy
Smail, Mustapha
Monin, Marie‐Lorraine
Ewenczyk, Claire
Bouccara, Didier
Durr, Alexandra
author_sort Borel, Stéphanie
collection PubMed
description 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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spelling pubmed-67644862019-09-30 Oral mobility reflects rate of progression in advanced Friedreich’s ataxia Borel, Stéphanie Gatignol, Peggy Smail, Mustapha Monin, Marie‐Lorraine Ewenczyk, Claire Bouccara, Didier Durr, Alexandra Ann Clin Transl Neurol Brief Communications 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. John Wiley and Sons Inc. 2019-08-25 /pmc/articles/PMC6764486/ /pubmed/31448573 http://dx.doi.org/10.1002/acn3.50879 Text en © 2019 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association. This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
spellingShingle Brief Communications
Borel, Stéphanie
Gatignol, Peggy
Smail, Mustapha
Monin, Marie‐Lorraine
Ewenczyk, Claire
Bouccara, Didier
Durr, Alexandra
Oral mobility reflects rate of progression in advanced Friedreich’s ataxia
title Oral mobility reflects rate of progression in advanced Friedreich’s ataxia
title_full Oral mobility reflects rate of progression in advanced Friedreich’s ataxia
title_fullStr Oral mobility reflects rate of progression in advanced Friedreich’s ataxia
title_full_unstemmed Oral mobility reflects rate of progression in advanced Friedreich’s ataxia
title_short Oral mobility reflects rate of progression in advanced Friedreich’s ataxia
title_sort oral mobility reflects rate of progression in advanced friedreich’s ataxia
topic Brief Communications
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6764486/
https://www.ncbi.nlm.nih.gov/pubmed/31448573
http://dx.doi.org/10.1002/acn3.50879
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