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

Citation

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, Nerve pathology distinguishes focal motor chronic inflammatory demyelinating polyradiculoneuropathy from multifocal motor neuropathy, Journal of Clinical Neuromuscular Disease, 22, (1) pp. 1-10. ISSN 1522-0443 (2020) [Refereed Article]

Copyright Statement

Copyright 2020 Wolters Kluwer Health, Inc.

DOI: doi:10.1097/CND.0000000000000279

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 Details

Item Type:Refereed Article
Keywords:chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy, peripheral nerve pathology, chronic axonal neuropathy, peripheral neuropathy
Research Division:Biomedical and Clinical Sciences
Research Group:Neurosciences
Research Field:Neurology and neuromuscular diseases
Objective Division:Health
Objective Group:Clinical health
Objective Field:Treatment of human diseases and conditions
UTAS Author:Taylor, BV (Professor Bruce Taylor)
ID Code:141819
Year Published:2020
Deposited By:Menzies Institute for Medical Research
Deposited On:2020-11-24
Last Modified:2021-03-15
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