面肩肱型肌营养不良症的肌电图检查：一项为期 1 年的随访研究。
Electrical impedance myography in facioscapulohumeral muscular dystrophy: A 1-year follow-up study.
机构信息
Department of Neurology, University of Kansas Medical Center, 4330 Shawnee Mission Parkway, Suite 323 Fairway, Kansas, 66205, USA.
Department of Neurology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
出版信息
Muscle Nerve. 2018 Aug;58(2):213-218. doi: 10.1002/mus.26127. Epub 2018 Apr 17.
INTRODUCTION
Electrical impedance myography (EIM) is a noninvasive technique for measuring muscle composition and a potential physiological biomarker for facioscapulohumeral muscular dystrophy (FSHD).
METHODS
Thirty-two participants with genetically confirmed and clinically affected FSHD underwent EIM in 7 muscles bilaterally. Correlations between EIM and baseline clinical measures were used to select EIM variables of interest in FSHD, and EIM and clinical measures were followed for 1 year.
RESULTS
There were no significant changes in the EIM variables. Although 50-kHZ reactance correlated the strongest with clinical measures at baseline, the 50-211-kHZ phase ratio demonstrated lower within-subject 12-month variability, potentially offering sample size savings for FSHD clinical trial planning.
DISCUSSION
EIM did not identify significant disease progression over 12 months. It is currently unclear whether this is because of limitations of the technology or the slow rate of disease progression in this cohort of FSHD patients over this period of time. Muscle Nerve 58: 213-218, 2018.
简介
电阻抗肌描记法（EIM）是一种用于测量肌肉成分的非侵入性技术，也是面肩肱型肌营养不良症（FSHD）的潜在生理生物标志物。
方法
32 名经基因证实且临床表现为 FSHD 的患者接受了双侧 7 块肌肉的 EIM 检查。EIM 与基线临床指标的相关性用于选择 FSHD 中感兴趣的 EIM 变量，并对 EIM 和临床指标进行了 1 年的随访。
结果
EIM 变量无显著变化。虽然 50-kHz 电抗在基线时与临床指标相关性最强，但 50-211-kHz 相位比的个体内 12 个月变异性较低，这可能为 FSHD 临床试验规划节省样本量。
讨论
EIM 未在 12 个月内发现显著的疾病进展。目前尚不清楚这是由于技术限制，还是由于在此期间，这组 FSHD 患者的疾病进展速度较慢。肌肉神经 58:213-218,2018.
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