Fasciculations demonstrate daytime consistency in amyotrophic lateral sclerosis.

Fasciculations demonstrate daytime consistency in amyotrophic lateral sclerosis.
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肌萎缩性侧索硬化症的肌束颤动表现出白天的一致性。

DOI:
10.1002/mus.26864
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发表时间:
2020
期刊:
影响因子:
3.4
通讯作者:
Bashford J
Bashford J
中科院分区:
医学3区
文献类型:
--
作者:
Bashford J

文献摘要

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肌萎缩侧索硬化症(ALS)患者的束状纹表现为早期神经元的高兴奋性。为了帮助校准作为疾病生物标志物,我们开始表征白天的束状跳动变异性。方法收集19例ALS患者的肌束控制意识评分。此外,10例ALS患者在一天内的三个时间点接受了二头肌和腓肠肌的高密度表面肌电图(HDSEMG)记录。结果白天肌束意识评分较低(平均0.28个肌群),表现出显著的变异性(变异系数:303%)。二头肌HDSEMG记录在束状电位频率(类内相关系数[ICC] = 95%, n = 19)和束状电位幅度的四分位数范围(ICC = 95%, n = 19)上高度一致。这些参数对观测到的数据质量参数波动表现出鲁棒性。腓肠肌整体表现出更适度的一致性(44%至62%,n = 20)。肌萎缩侧索硬化症患者的二头肌肌束放电在白天具有显著的一致性,尽管在患者的叙述中有稀疏和间歇性的意识。
IntroductionFasciculations represent early neuronal hyperexcitability in amyotrophic lateral sclerosis (ALS). To aid calibration as a disease biomarker, we set out to characterize the daytime variability of fasciculation firing.MethodsFasciculation awareness scores were compiled from 19 ALS patients. In addition, 10 ALS patients prospectively underwent high‐density surface electromyographic (HDSEMG) recordings from biceps and gastrocnemius at three time‐points during a single day.ResultsDaytime fasciculation awareness scores were low (mean: 0.28 muscle groups), demonstrating significant variability (coefficient of variation: 303%). Biceps HDSEMG recordings were highly consistent for fasciculation potential frequency (intraclass correlation coefficient [ICC] = 95%, n = 19) and the interquartile range of fasciculation potential amplitude (ICC = 95%, n = 19). These parameters exhibited robustness to observed fluctuations in data quality parameters. Gastrocnemius demonstrated more modest levels of consistency overall (44% to 62%, n = 20).DiscussionThere was remarkable daytime consistency of fasciculation firing in the biceps of ALS patients, despite sparse and intermittent awareness among patients’ accounts.