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

被引：5

作者：

Borel, Stephanie ^{[1
,2
]}

Gatigno, Peggy ^{[2
,3
]}

Smail, Mustapha ^{[2
]}

Monin, Marie-Lorraine ^{[4
]}

Ewenczyk, Claire ^{[4
]}

Bouccara, Didier ^{[2
]}

Durr, Alexandra ^{[4
]}

机构：

[1] Sorbonne Univ, INSERM, UMRS1159, Rehabil Chirurg Mini Invas & Robotisee Audit, Paris, France

[2] Hop Univ Pitie Salpetriere, AP HP, Serv ORL, Paris, France

[3] Sorbonne Univ, INSERM, UMRS1158, Neurophysiol Resp Expt & Clin, Paris, France

[4] Sorbonne Univ, Hop Univ Pitie Salpetriere, AP HP, INSERM,CNRS,Inst Cerveau & Moelle Epiniere ICM, Paris, France

来源：

ANNALS OF CLINICAL AND TRANSLATIONAL NEUROLOGY | 2019年 / 6卷 / 09期

关键词：

DYSARTHRIA; ABNORMALITIES; SCALE;

D O I：

10.1002/acn3.50879

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

摘要：

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.

引用

页码：1888 / 1892

页数：5

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