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运动单位数量指数(MUNIX)在新诊断的肌萎缩侧索硬化症患者中的预后价值

Prognostic Usefulness of Motor Unit Number Index (MUNIX) in Patients Newly Diagnosed with Amyotrophic Lateral Sclerosis.

作者信息

Risi Barbara, Cotti Piccinelli Stefano, Gazzina Stefano, Labella Beatrice, Caria Filomena, Damioli Simona, Poli Loris, Padovani Alessandro, Filosto Massimiliano

机构信息

Department of Clinical and Experimental Sciences, University of Brescia, 25121 Brescia, Italy.

Unit of Neurology, ASST Spedali Civili, 25123 Brescia, Italy.

出版信息

J Clin Med. 2023 Jul 31;12(15):5036. doi: 10.3390/jcm12155036.

DOI:10.3390/jcm12155036
PMID:37568439
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC10420094/
Abstract

UNLABELLED

The MUNIX technique allows us to estimate the number and size of surviving motor units (MUs). Previous studies on ALS found correlations between MUNIX and several clinical measures, but its potential role as a predictor of disease progression rate (DPR) has not been thoroughly evaluated to date. We aimed to investigate MUNIX's ability to predict DPR at a six-month follow up.

METHODS

24 ALS patients with short disease duration (<24 months from symptoms' onset) were enrolled and divided according to their baseline DPR into two groups (normal [DPR-N] and fast [DPR-F] progressors). MUNIX values were obtained from five muscles (TA, APB, ADM, FDI, Trapezius) and averaged for each subject.

RESULTS

MUNIX was found to predict DPR at follow up in a multivariable linear regression model; namely, patients with lower MUNIX values were at risk of showing greater DPR scores at follow up. The result was replicated in a simple logistic regression analysis, with the dichotomic category "MUNIX-Low" as the independent variable and the outcome "DPR-F" as the dependent variable.

CONCLUSIONS

our results pave the way for the use of the MUNIX method as a prognostic tool in early ALS, enabling patients' stratification according to their rates of future decline.

摘要

未标注

MUNIX技术使我们能够估计存活运动单位(MU)的数量和大小。先前关于肌萎缩侧索硬化症(ALS)的研究发现MUNIX与几种临床指标之间存在相关性,但迄今为止,其作为疾病进展率(DPR)预测指标的潜在作用尚未得到充分评估。我们旨在研究MUNIX在六个月随访中预测DPR的能力。

方法

纳入24例病程较短(症状出现后<24个月)的ALS患者,并根据其基线DPR分为两组(正常进展者[DPR-N]和快速进展者[DPR-F])。从五块肌肉(胫骨前肌、拇短展肌、小指展肌、第一骨间背侧肌、斜方肌)获取MUNIX值,并计算每个受试者的平均值。

结果

在多变量线性回归模型中发现MUNIX可预测随访时的DPR;也就是说,MUNIX值较低的患者在随访时出现更高DPR评分的风险更大。在简单逻辑回归分析中重复了该结果,将二分变量“MUNIX低”作为自变量,将结果“DPR-F”作为因变量。

结论

我们的结果为将MUNIX方法用作早期ALS的预后工具铺平了道路,能够根据患者未来的衰退率进行分层。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b886/10420094/2c3fc8ad0fa2/jcm-12-05036-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b886/10420094/426f9e9cf3e1/jcm-12-05036-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b886/10420094/2c3fc8ad0fa2/jcm-12-05036-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b886/10420094/426f9e9cf3e1/jcm-12-05036-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/b886/10420094/2c3fc8ad0fa2/jcm-12-05036-g002.jpg

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Motor unit number index (MUNIX) loss of 50% occurs in half the time of 50% functional loss according to the D50 disease progression model of ALS.根据 ALS 的 D50 疾病进展模型,运动单位数量指数(MUNIX)损失 50% 的发生时间是功能性损失 50% 的一半。
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Motor Unit Number Index of the Upper Trapezius: A Meta-Analysis and Cross-sectional Study of Its Reliability.
肌萎缩侧索硬化症的神经生理学创新:加强诊断、监测和治疗评估
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Sci Rep. 2022 Sep 26;12(1):15997. doi: 10.1038/s41598-022-19911-0.
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Hastening the Diagnosis of Amyotrophic Lateral Sclerosis.加速肌萎缩性侧索硬化症的诊断。
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