Electrical impedance myography in facioscapulohumeral muscular dystrophy: A 1-year follow-up study.
Electrical impedance myography in facioscapulohumeral muscular dystrophy: A 1-year follow-up study.
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面肩肱型肌营养不良症的电阻抗肌电图：一项为期一年的随访研究。
DOI：
10.1002/mus.26127
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发表时间：
2018
期刊：
影响因子：
3.4
通讯作者：
Statland,JeffreyM
中科院分区：
文献类型：
--
作者：
Mul,Karlien;Heatwole,Chad;Eichinger,Katy;Dilek,Nuran;Martens,WilliamB;VanEngelen,BazielGM;Tawil,Rabi;Statland,JeffreyM
简介：电阻抗肌电图 (EIM) 是一种用于测量肌肉成分的无创技术，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受影响的 FSHD 参与者在双侧 7 块肌肉中接受了 EIM。 EIM 和基线临床测量之间的相关性用于选择 FSHD 中感兴趣的 EIM 变量，并对 EIM 和临床测量进行为期 1 年的跟踪。结果：EIM 变量没有显着变化。尽管 50-kHZ 电抗与基线时的临床测量相关性最强，但 50-211-kHZ 相位比表现出较低的受试者内 12 个月变异性，可能为 FSHD 临床试验计划节省样本量。讨论：EIM 未发现 12 个月内有显着的疾病进展。目前尚不清楚这是否是由于技术的局限性或这组 FSHD 患者在这段时间内疾病进展速度缓慢所致。 Muscle Nerve58: 213–218, 2018
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‐kHZreactance correlated the strongest with clinical measures at baseline, the 50–211‐kHZphase 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 Nerve58: 213–218, 2018