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Ann Clin Transl Neurol. 2019 Aug 25. doi: 10.1002/acn3.50879. [Epub ahead of print]

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

Author information

1
Sorbonne Université, INSERM, UMRS1159 Réhabilitation Chirurgicale Mini-Invasive et Robotisée de l'Audition, Paris, France.
2
AP-HP, Service d'ORL, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.
3
Sorbonne Université, INSERM, UMRS1158 Neurophysiologie Respiratoire Expérimentale et Clinique, Paris, France.
4
Sorbonne Université, Institut du Cerveau et de la Moelle épinière (ICM), AP-HP, INSERM, CNRS, Hôpital Universitaire Pitié-Salpêtrière, Paris, France.

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.

PMID:
31448573
DOI:
10.1002/acn3.50879
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