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慢性炎症性脱髓鞘性多发性神经病(CIDP)治疗结果与首次神经传导变化的相关性:确定初始和长期反应者。

CIDP Treatment Outcomes Correlation With First Nerve Conduction Changes: Ascertainment of Initial and Long-Term Responders.

作者信息

Wannarong Thapat, Skolka Michael P, Rattanathamsakul Natthapon, Swart Grace, Dyck James B, Berini Sarah E, Dubey Divyanshu, Shouman Kamal, Pinto Marcus V, Mauermann Michelle L, Windebank Anthony J, Staff Nathan P, Klein Christopher J

机构信息

Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.

Division of Neurology, Department of Medicine, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand.

出版信息

J Peripher Nerv Syst. 2025 Jun;30(2):e70017. doi: 10.1111/jns.70017.

DOI:10.1111/jns.70017
PMID:40189868
Abstract

BACKGROUND AND AIMS

Nerve conduction studies (NCS) are integral to diagnosing chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but their role in predicting treatment outcomes remains underexplored. This study evaluates NCS changes at first follow-up (first NCS changes) as predictors of treatment success in CIDP, focusing on their correlation with clinical outcomes over time.

METHODS

Newly diagnosed CIDP patients meeting the 2021 EAN/PNS criteria were retrospectively evaluated. Baseline and first follow-up NCS parameters were compared with clinical outcomes, assessed by the Neuropathy Impairment Score (NIS) and Inflammatory Neuropathy Cause and Treatment (INCAT) disability score. All patients received first-line immunotherapy (intravenous immunoglobulin, corticosteroids, or plasma exchange).

RESULTS

Of 39 treated patients, 26 (66.7%) were responders based on improving NIS trends, while 13 (33.3%) were nonresponders. Responders showed significant improvements at the first follow-up in fibular compound muscle action potential (CMAP) amplitude, ulnar CMAP amplitude, summated CMAP amplitudes, and fibular motor conduction velocity. Changes in fibular CMAP amplitude consistently correlated with NIS (R = -0.8 to -0.6, p ≤ 0.004) and INCAT disability score improvements (R = -0.6 to -0.3, p ≤ 0.032) across all follow-up intervals up to 60 months. Ulnar and summated CMAP amplitude changes also correlated with clinical outcomes, though their associations were less sustained than those of fibular CMAP amplitude.

INTERPRETATION

The first change in fibular CMAP amplitude is a reliable biomarker for predicting CIDP treatment response, with ulnar and summated CMAP amplitudes as alternatives when the fibular response is absent. Our findings highlight the utility of first NCS changes in monitoring and predicting treatment outcomes in CIDP.

摘要

背景与目的

神经传导研究(NCS)对于慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)的诊断不可或缺,但其在预测治疗结果方面的作用仍未得到充分探索。本研究评估首次随访时的NCS变化(首次NCS变化)作为CIDP治疗成功的预测指标,重点关注其与不同时间临床结果的相关性。

方法

对符合2021年欧洲神经病学学会/周围神经学会(EAN/PNS)标准的新诊断CIDP患者进行回顾性评估。将基线和首次随访时的NCS参数与临床结果进行比较,临床结果通过神经病损伤评分(NIS)和炎症性神经病病因与治疗(INCAT)残疾评分进行评估。所有患者均接受一线免疫治疗(静脉注射免疫球蛋白、皮质类固醇或血浆置换)。

结果

在39例接受治疗的患者中,基于NIS趋势改善,26例(66.7%)为反应者,13例(33.3%)为无反应者。反应者在首次随访时腓骨复合肌肉动作电位(CMAP)波幅、尺神经CMAP波幅、总CMAP波幅和腓骨运动传导速度方面有显著改善。在长达60个月的所有随访间隔中,腓骨CMAP波幅的变化始终与NIS(R = -0.8至-0.6,p≤0.004)和INCAT残疾评分改善(R = -0.6至-0.3,p≤0.032)相关。尺神经和总CMAP波幅变化也与临床结果相关,尽管它们的相关性不如腓骨CMAP波幅持久。

解读

腓骨CMAP波幅的首次变化是预测CIDP治疗反应的可靠生物标志物,当缺乏腓骨反应时,尺神经和总CMAP波幅可作为替代指标。我们的研究结果突出了首次NCS变化在监测和预测CIDP治疗结果中的作用。

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