神经病理学鉴别局灶性运动性慢性炎性脱髓鞘性多发神经根神经病与多灶性运动神经病。
Nerve Pathology Distinguishes Focal Motor Chronic Inflammatory Demyelinating Polyradiculoneuropathy From Multifocal Motor Neuropathy.
机构信息
Department of Neurology, Mayo Clinic, Rochester, MN.
Department of Neurological Research, Menzies Institute for Medical Research, University of Tasmania, Tasmania, Australia.
出版信息
J Clin Neuromuscul Dis. 2020 Sep;22(1):1-10. doi: 10.1097/CND.0000000000000279.
OBJECTIVES
The objective of the study is to distinguish the mechanisms of disease for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN), which we believe to be fundamentally different. However, distinguishing the mechanisms is more difficult when the presentation of CIDP is motor-predominant, focal, or asymmetric.
METHODS
We describe 3 focal, motor-predominant, representative cases that could be interpreted on clinical and/or electrophysiological grounds as either MMN or focal CIDP, and present pathological findings.
RESULTS
We highlight pathological differences in these cases, and provide an argument that CIDP and MMN are distinct entities with different pathophysiological mechanisms-chronic demyelination for CIDP, and an immune-mediated attack on paranodal motor axons for MMN.
CONCLUSIONS
Based on clinical evaluation, electrophysiology, and nerve biopsy pathology, we can divide the conditions into inflammatory demyelinating neuropathy (focal CIDP) versus chronic axonal neuropathy (MMN). The divergent pathological findings provide further evidence that CIDP and MMN are fundamentally different disorders.
目的
本研究旨在区分慢性炎症性脱髓鞘性多发神经根神经病（CIDP）和多发运动神经病（MMN）的发病机制，我们认为这两种疾病在根本上有所不同。然而，当 CIDP 表现为以运动为主、局灶性或不对称时，区分其发病机制就变得更加困难。
方法
我们描述了 3 例具有代表性的局灶性、以运动为主的病例，这些病例在临床和/或电生理基础上可被解释为 MMN 或局灶性 CIDP，并介绍了其病理学发现。
结果
我们强调了这些病例的病理学差异，并提出了一个论点，即 CIDP 和 MMN 是具有不同病理生理机制的不同实体——CIDP 为慢性脱髓鞘，MMN 为免疫介导的运动轴突旁攻击。
结论
基于临床评估、电生理学和神经活检病理学，我们可以将这些疾病分为炎症性脱髓鞘性神经病（局灶性 CIDP）和慢性轴索性神经病（MMN）。不同的病理学发现进一步证明 CIDP 和 MMN 是两种截然不同的疾病。
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