多灶性运动神经病中除传导阻滞外的神经传导异常。对诊断标准准确性的影响。

Nerve Conduction Abnormalities Beyond Conduction Block in Multifocal Motor Neuropathy. Impact on Diagnostic Criteria Accuracy.

作者信息

Doneddu Pietro Emiliano, Gallo Chiara, Falzone Yuri, Matà Sabrina, Cosentino Giuseppe, Di Stefano Vincenzo, Filosto Massimiliano, Leonardi Luca, Ricciardi Dario, Mazzeo Anna, Benedetti Luana, Luigetti Marco, Solla Paolo, Inghilleri Maurizio, Cocito Dario, Habetswallner Francesco, De Lorenzo Alberto, Sorrenti Benedetta, Spalletti Maddalena, Vegezzi Elisa, Messina Christian, Risi Barbara, Forcina Francesca, Fasolino Alessandra, Gentile Luca, Beronio Alessandro, Vitali Francesca, Moret Federica, Iabichella Giacomo, Gazzina Stefano, Cabona Corrado, Cutellè Claudia, Bianchi Elisa, Nobile-Orazio Eduardo

机构信息

Neuromuscular and Neuroimmunology Unit, IRCCS Humanitas Research Hospital, Milano, Italy.

Department of Biomedical Sciences, Humanitas University, Milan, Italy.

出版信息

Eur J Neurol. 2025 Sep;32(9):e70300. doi: 10.1111/ene.70300.

DOI:10.1111/ene.70300

PMID:40984777

Abstract

BACKGROUND

Multifocal motor neuropathy (MMN) is a rare motor neuropathy diagnosed by identifying motor conduction block (CB), which may be absent or transient. This study aimed to evaluate nerve conduction abnormalities beyond CB and their diagnostic value.

METHODS

A retrospective analysis included patients fulfilling the 2010 EFNS/PNS clinical criteria for MMN and controls with axonal polyneuropathy, lower motor neuron disease, or chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Electrophysiological studies evaluated motor distal latency prolongation, reduced motor conduction velocity, prolonged F-wave latency, absence of F-waves, abnormal temporal dispersion, and distal CMAP duration prolongation. The 2010 EFNS/PNS criteria for MMN were compared to an 'extended' set incorporating additional electrophysiological parameters.

RESULTS

A total of 70 MMN patients and 359 controls were included. At least one nerve conduction abnormality, excluding motor CB, was detected in one or more nerve segments unaffected by CB in 71% of MMN patients, significantly more frequently than in axonal polyneuropathy or lower motor neuron disease patients. These parameters included abnormal temporal dispersion (47%), distal CMAP duration prolongation (31%), reduced motor conduction velocity (26%), absence of F-waves (9%), prolonged F-wave latency (6%), and motor distal latency prolongation (3%). Incorporating these parameters increased sensitivity for diagnosing probable/definite MMN by 26% (p < 0.001; compared to EFNS/PNS criteria), with minimal impact on specificity, even when compared to CIDP patients.

CONCLUSION

Including additional electrophysiological parameters into the diagnostic criteria for MMN may enhance diagnostic sensitivity while maintaining comparable specificity. The observation of nerve conduction abnormalities beyond CB indicates a broader electrophysiological profile for MMN.

摘要

背景

多灶性运动神经病（MMN）是一种罕见的运动神经病，通过识别运动传导阻滞（CB）来诊断，而运动传导阻滞可能不存在或为短暂性。本研究旨在评估除CB之外的神经传导异常及其诊断价值。

方法

一项回顾性分析纳入了符合2010年欧洲神经病学学会/周围神经学会（EFNS/PNS）MMN临床标准的患者，以及患有轴索性多发性神经病、下运动神经元疾病或慢性炎症性脱髓鞘性多发性神经根神经病（CIDP）的对照。电生理研究评估运动远端潜伏期延长、运动传导速度降低、F波潜伏期延长、F波缺失、异常时间离散以及远端复合肌肉动作电位（CMAP）时限延长。将2010年EFNS/PNS的MMN标准与纳入了额外电生理参数的“扩展”标准进行比较。

结果

共纳入70例MMN患者和359例对照。在71%的MMN患者中，在一个或多个未受CB影响的神经节段检测到至少一项排除运动CB的神经传导异常，其发生频率显著高于轴索性多发性神经病或下运动神经元疾病患者。这些参数包括异常时间离散（47%）、远端CMAP时限延长（31%）、运动传导速度降低（26%）、F波缺失（9%）、F波潜伏期延长（6%）以及运动远端潜伏期延长（3%）。纳入这些参数使诊断可能/确诊MMN的敏感性提高了26%（p<0.001；与EFNS/PNS标准相比），即使与CIDP患者相比，对特异性的影响也最小。