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

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 m...

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Main Authors: Stéphanie Borel, Peggy Gatignol, Mustapha Smail, Marie‐Lorraine Monin, Claire Ewenczyk, Didier Bouccara, Alexandra Durr
Format: Article
Language:English
Published: Wiley 2019-09-01
Series:Annals of Clinical and Translational Neurology
Online Access:https://doi.org/10.1002/acn3.50879
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spelling doaj-4994d13e313c4c6181d97476f4fa1fb12021-05-02T14:20:13ZengWileyAnnals of Clinical and Translational Neurology2328-95032019-09-01691888189210.1002/acn3.50879Oral mobility reflects rate of progression in advanced Friedreich’s ataxiaStéphanie Borel0Peggy Gatignol1Mustapha Smail2Marie‐Lorraine Monin3Claire Ewenczyk4Didier Bouccara5Alexandra Durr6Sorbonne Université, INSERM, UMRS1159 Réhabilitation Chirurgicale Mini‐Invasive et Robotisée de l’Audition Paris FranceAP‐HP, Service d’ORL, Hôpital Universitaire Pitié-Salpêtrière Paris FranceAP‐HP, Service d’ORL, Hôpital Universitaire Pitié-Salpêtrière Paris FranceSorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP‐HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière Paris FranceSorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP‐HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière Paris FranceAP‐HP, Service d’ORL, Hôpital Universitaire Pitié-Salpêtrière Paris FranceSorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP‐HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière Paris FranceAbstract 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.https://doi.org/10.1002/acn3.50879
collection DOAJ
language English
format Article
sources DOAJ
author Stéphanie Borel
Peggy Gatignol
Mustapha Smail
Marie‐Lorraine Monin
Claire Ewenczyk
Didier Bouccara
Alexandra Durr
spellingShingle Stéphanie Borel
Peggy Gatignol
Mustapha Smail
Marie‐Lorraine Monin
Claire Ewenczyk
Didier Bouccara
Alexandra Durr
Oral mobility reflects rate of progression in advanced Friedreich’s ataxia
Annals of Clinical and Translational Neurology
author_facet Stéphanie Borel
Peggy Gatignol
Mustapha Smail
Marie‐Lorraine Monin
Claire Ewenczyk
Didier Bouccara
Alexandra Durr
author_sort Stéphanie Borel
title 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_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_sort oral mobility reflects rate of progression in advanced friedreich’s ataxia
publisher Wiley
series Annals of Clinical and Translational Neurology
issn 2328-9503
publishDate 2019-09-01
description 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.
url https://doi.org/10.1002/acn3.50879
work_keys_str_mv AT stephanieborel oralmobilityreflectsrateofprogressioninadvancedfriedreichsataxia
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AT marielorrainemonin oralmobilityreflectsrateofprogressioninadvancedfriedreichsataxia
AT claireewenczyk oralmobilityreflectsrateofprogressioninadvancedfriedreichsataxia
AT didierbouccara oralmobilityreflectsrateofprogressioninadvancedfriedreichsataxia
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