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慢性炎症性脱髓鞘性多发性神经病中MScanFit运动单位数量估计与临床功能及免疫球蛋白治疗反应之间的关联

Association Between MScanFit Motor Unit Number Estimation and Clinical Function and Response to Immunoglobulin Therapy in Chronic Inflammatory Demyelinating Polyneuropathy.

作者信息

Hansen Peter N, Mohammed Abdullahi A, Markvardsen Lars K, Andersen Henning, Tankisi Hatice, Sindrup Søren H, Krøigård Thomas

机构信息

Neurology Research Unit, Odense University Hospital, Odense, Denmark, University of Southern Denmark, Odense, Denmark.

Department of Neurology, Aarhus University Hospital, Aarhus, Denmark.

出版信息

J Peripher Nerv Syst. 2025 Mar;30(1):e70001. doi: 10.1111/jns.70001.

DOI:10.1111/jns.70001
PMID:39887824
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11783579/
Abstract

BACKGROUND AND AIMS

Loss of motor units in chronic inflammatory demyelinating polyneuropathy is difficult to assess by conventional nerve conduction due to collateral innervation. We aimed to assess the association between a motor unit number estimate (MUNE) derived from the compound muscle action potential (CMAP) scan using MScanFit and hand function and the clinical response to intravenous immunoglobulin (IVIG).

METHODS

Forty-nine CIDP patients and 52 control subjects were included. CMAP scan recordings were obtained from the right abductor pollicis brevis muscle. The primary outcome was the correlation between MUNE and the duration of the nine-hole-peg test (9-HPT) at baseline and the change in the duration of the 9-HPT following treatment with IVIG. Secondary outcomes were grip strength, 10-m-walk test, six-spot-step test, medical research council sum score, inflammatory neuropathy cause and treatment sensory sum score, overall neuropathy limitations scale, and the Rasch-built overall disability scale (R-ODS).

RESULTS

MScanFit analysis suggested both loss of motor units (reduced MUNE (p = 0.022) and N50 (p < 0.0001)) and collateral reinnervation (increased median amplitude (p < 0.0001) and size of the largest unit (p < 0.0001)) in CIDP patients compared to controls. In CIDP patients, there was a statistically significant correlation between MUNE and the duration of the 9-HPT (Spearman's r = -0.342; p = 0.016). Further, patients with a low MUNE had the largest reduction in the duration of the 9-HPT following IVIG treatment (r = -0.577; p = 0.043). MUNE also correlated significantly with R-ODS (r = -0.722; p = 0.007).

INTERPRETATION

MScanFit MUNE could be a useful method for assessing motor axonal loss in CIDP, which correlates with the clinical function and treatment response.

摘要

背景与目的

由于侧支神经支配,慢性炎症性脱髓鞘性多发性神经病中运动单位的丧失难以通过传统神经传导来评估。我们旨在评估使用MScanFit从复合肌肉动作电位(CMAP)扫描得出的运动单位数量估计值(MUNE)与手功能以及静脉注射免疫球蛋白(IVIG)临床反应之间的关联。

方法

纳入49例CIDP患者和52例对照受试者。从右侧拇短展肌获取CMAP扫描记录。主要结局是基线时MUNE与九孔插针试验(9-HPT)持续时间之间的相关性,以及IVIG治疗后9-HPT持续时间的变化。次要结局包括握力、10米步行试验、六点步态试验、医学研究委员会总分、炎性神经病病因和治疗感觉总分、总体神经病限制量表以及Rasch构建的总体残疾量表(R-ODS)。

结果

与对照组相比,MScanFit分析表明CIDP患者存在运动单位丧失(MUNE降低(p = 0.022)和N50降低(p < 0.0001))以及侧支神经再支配(中位幅度增加(p < 0.0001)和最大单位大小增加(p < 0.0001))。在CIDP患者中,MUNE与9-HPT持续时间之间存在统计学显著相关性(Spearman相关系数r = -0.342;p = 0.016)。此外,MUNE较低的患者在IVIG治疗后9-HPT持续时间的降低幅度最大(r = -0.577;p = 0.043)。MUNE与R-ODS也显著相关(r = -0.722;p = 0.007)。

解读

MScanFit MUNE可能是评估CIDP中运动轴突丧失的一种有用方法,其与临床功能和治疗反应相关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/842d832a2df1/JNS-30-0-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/e747b5afd074/JNS-30-0-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/179b18887b62/JNS-30-0-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/873484be1994/JNS-30-0-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/842d832a2df1/JNS-30-0-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/e747b5afd074/JNS-30-0-g003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/179b18887b62/JNS-30-0-g004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/873484be1994/JNS-30-0-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/68ab/11783579/842d832a2df1/JNS-30-0-g001.jpg

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本文引用的文献

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Clin Neurophysiol. 2023 Jul;151:92-99. doi: 10.1016/j.clinph.2023.04.008. Epub 2023 May 8.
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Changes in axonal and clinical function during intravenous and subcutaneous immunoglobulin therapy in chronic inflammatory demyelinating polyneuropathy.慢性炎症性脱髓鞘性多发性神经病患者静脉及皮下注射免疫球蛋白治疗期间轴突和临床功能的变化
J Peripher Nerv Syst. 2023 Sep;28(3):425-435. doi: 10.1111/jns.12563. Epub 2023 Jun 7.
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Neurophysiological Hallmarks of Axonal Degeneration in CIDP Patients: A Pilot Analysis.
慢性炎性脱髓鞘性多发性神经病(CIDP)患者轴索性变性的神经生理学特征:一项初步分析
Brain Sci. 2022 Nov 7;12(11):1510. doi: 10.3390/brainsci12111510.
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The value of MUNIX as an objective electrophysiological biomarker of disease progression in chronic inflammatory demyelinating polyneuropathy.MUNIX 作为慢性炎症性脱髓鞘性多发性神经病疾病进展的客观电生理学生物标志物的价值。
Muscle Nerve. 2022 Apr;65(4):433-439. doi: 10.1002/mus.27498. Epub 2022 Jan 31.
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Axonal damage determines clinical disability in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): A prospective cohort study of different CIDP subtypes and disease stages.轴突损伤决定慢性炎症性脱髓鞘性多发性神经病(CIDP)的临床残疾:不同 CIDP 亚型和疾病阶段的前瞻性队列研究。
Eur J Neurol. 2022 Feb;29(2):583-592. doi: 10.1111/ene.15156. Epub 2021 Nov 13.
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