Oral mobility reflects rate of progression in advanced Friedreich's ataxia.


Journal

Annals of clinical and translational neurology
ISSN: 2328-9503
Titre abrégé: Ann Clin Transl Neurol
Pays: United States
ID NLM: 101623278

Informations de publication

Date de publication:
09 2019
Historique:
received: 17 06 2019
revised: 18 07 2019
accepted: 06 08 2019
pubmed: 27 8 2019
medline: 19 5 2020
entrez: 27 8 2019
Statut: ppublish

Résumé

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.

Identifiants

pubmed: 31448573
doi: 10.1002/acn3.50879
pmc: PMC6764486
doi:

Types de publication

Journal Article Research Support, Non-U.S. Gov't

Langues

eng

Sous-ensembles de citation

IM

Pagination

1888-1892

Subventions

Organisme : AFAF French Friedreich Ataxia Association
Pays : International

Informations de copyright

© 2019 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association.

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Auteurs

Stéphanie Borel (S)

Sorbonne Université, INSERM, UMRS1159 Réhabilitation Chirurgicale Mini-Invasive et Robotisée de l'Audition, Paris, France.
AP-HP, Service d'ORL, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.

Peggy Gatignol (P)

AP-HP, Service d'ORL, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.
Sorbonne Université, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Paris, France.

Mustapha Smail (M)

AP-HP, Service d'ORL, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.

Marie-Lorraine Monin (ML)

Sorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP-HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.

Claire Ewenczyk (C)

Sorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP-HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.

Didier Bouccara (D)

AP-HP, Service d'ORL, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.

Alexandra Durr (A)

Sorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP-HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.

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