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Nerve pathology distinguishes focal motor chronic inflammatory demyelinating polyradiculoneuropathy from multifocal motor neuropathy

Tracy, JA, Taylor, BV, Kiernan, M, Dyck, PJ, Crum, BA, Mauermann, ML, Amrami, KK, Spinner, RJ and Dyck, PJB 2020 , 'Nerve pathology distinguishes focal motor chronic inflammatory demyelinating polyradiculoneuropathy from multifocal motor neuropathy' , Journal of Clinical Neuromuscular Disease, vol. 22, no. 1 , pp. 1-10 , doi: 10.1097/CND.0000000000000279.

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Abstract

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.

Item Type: Article
Authors/Creators:Tracy, JA and Taylor, BV and Kiernan, M and Dyck, PJ and Crum, BA and Mauermann, ML and Amrami, KK and Spinner, RJ and Dyck, PJB
Keywords: chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, peripheral nerve pathology, chronic axonal neuropathy, peripheral neuropathy
Journal or Publication Title: Journal of Clinical Neuromuscular Disease
Publisher: Lippincott Williams & Wilkins
ISSN: 1522-0443
DOI / ID Number: 10.1097/CND.0000000000000279
Copyright Information:

Copyright © 2020 Wolters Kluwer Health, Inc.

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