口腔运动能力反映晚期弗里德里希共济失调的进展速度。
Oral mobility reflects rate of progression in advanced Friedreich's ataxia.
机构信息
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.
出版信息
Ann Clin Transl Neurol. 2019 Sep;6(9):1888-1892. doi: 10.1002/acn3.50879. Epub 2019 Aug 25.
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.
摘要
我们的目的是确定弗里德里希共济失调疾病进展的敏感标志物。我们前瞻性地在两个时间点评估了 40 名患者的言语、语音和口运动功能。平均疾病持续时间为 20.8 ± 9.8 年，基线时 SARA 评分为 23.7 ± 8.6。通过面部、眼睛、脸颊、嘴唇和舌头的运动的组合评估口腔运动能力，在 1 年后显著下降（P < 0.0001）。12 个月内的标准化反应均值被认为在口腔运动能力方面较大（1.26），而在 SARA 方面较小（0.12）。因此，口腔运动能力可能是治疗试验中的一个敏感标志物。
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