Your browser doesn't support javascript.
loading
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.
Affiliation
  • Borel S; Sorbonne Université, INSERM, UMRS1159 Réhabilitation Chirurgicale Mini-Invasive et Robotisée de l'Audition, Paris, France.
  • Gatignol P; AP-HP, Service d'ORL, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.
  • Smail M; AP-HP, Service d'ORL, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.
  • Monin ML; Sorbonne Université, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Paris, France.
  • Ewenczyk C; AP-HP, Service d'ORL, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.
  • Bouccara D; Sorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP-HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.
  • Durr A; Sorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP-HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.
Ann Clin Transl Neurol ; 6(9): 1888-1892, 2019 09.
Article in En | MEDLINE | ID: mdl-31448573
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.
Subject(s)
Fulltext
Print
XML
PubMed Links
Search on Google

Full text: 1 Database: MEDLINE Main subject: Friedreich Ataxia / Range of Motion, Articular / Facial Muscles Type of study: Observational_studies / Prognostic_studies Limits: Adult / Aged / Female / Humans / Male / Middle aged Language: En Year: 2019 Type: Article
Fulltext
Print
XML
PubMed Links
Search on Google

Full text: 1 Database: MEDLINE Main subject: Friedreich Ataxia / Range of Motion, Articular / Facial Muscles Type of study: Observational_studies / Prognostic_studies Limits: Adult / Aged / Female / Humans / Male / Middle aged Language: En Year: 2019 Type: Article