Nerve Pathology Distinguishes Focal Motor Chronic Inflammatory Demyelinating Polyradiculoneuropathy From Multifocal Motor Neuropathy. Issue 1 (September 2020)

• Record Type: Journal Article
• Authors: Tracy, Jennifer A.; Taylor, Bruce V.; Kiernan, Matthew; Dyck, Peter J.; Crum, Brian A.; Mauermann, Michelle L.; Amrami, Kimberly K.; Spinner, Robert J.; Dyck, P. James B.
• Is Part Of: Journal of clinical neuromuscular disease. Volume 22:Issue 1(2020:Sep.)
• Publication Date: 2020-09
• Subjects: chronic inflammatory demyelinating polyradiculoneuropathy -- multifocal motor neuropathy -- peripheral nerve pathology -- chronic axonal neuropathy -- peripheral neuropathy; Neuromuscular diseases -- Periodicals; 616.744005
• DOI: 10.1097/CND.0000000000000279
• Languages: English
• ISSNs: 1522-0443

Abstract

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.