Chronic inflammatory demyelinating polyneuropathy: The spectrum and immunopathogenesis deciphered by electrophysiology and neuroimaging

Chronic inflammatory demyelinating polyneuropathy: The spectrum and immunopathogenesis deciphered... Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder having a wide range of clinical phenotypes, and is currently classified into “typical CIDP” and variants, such as multifocal acquired demyelinating sensory and motor neuropathy. Typical CIDP is clinically characterized by symmetric polyneuropathy involving proximal as well as distal muscle weakness, whereas electrophysiology shows evidence of demyelination predominant in the distal nerve terminals, and magnetic resonance neurography frequently shows prominent hypertrophy of the nerve roots. The pattern of demyelination distribution strongly suggests primary involvement of the nerve terminals and roots, where the blood–nerve barrier is anatomically deficient, and the importance of an antibody‐mediated mechanism. In contrast, multifocal acquired demyelinating sensory and motor neuropathy is multiple mononeuropathy or asymmetric polyneuropathy with multifocal conduction blocks and focal nerve enlargement in the intermediate nerve trunks at the site of conduction block; such distribution of lesions is reasonably explained by breakdown of the blood–nerve barrier by activated lymphocytes, suggesting a multiple sclerosis‐like cellular mechanism. Clinical features are likely to be determined by the immunopathogenesis, and therefore different immunological treatment would be required for each CIDP subtype. The present review focuses on current concepts of the disease spectrum of “CIDP syndrome” deciphered by electrophysiology and neuroimaging. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Clinical and Experimental Neuroimmunology Wiley

Chronic inflammatory demyelinating polyneuropathy: The spectrum and immunopathogenesis deciphered by electrophysiology and neuroimaging

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Publisher
Wiley
Copyright
Copyright © 2018 Japanese Society for Neuroimmunology
ISSN
1759-1961
eISSN
1759-1961
D.O.I.
10.1111/cen3.12431
Publisher site
See Article on Publisher Site

Abstract

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder having a wide range of clinical phenotypes, and is currently classified into “typical CIDP” and variants, such as multifocal acquired demyelinating sensory and motor neuropathy. Typical CIDP is clinically characterized by symmetric polyneuropathy involving proximal as well as distal muscle weakness, whereas electrophysiology shows evidence of demyelination predominant in the distal nerve terminals, and magnetic resonance neurography frequently shows prominent hypertrophy of the nerve roots. The pattern of demyelination distribution strongly suggests primary involvement of the nerve terminals and roots, where the blood–nerve barrier is anatomically deficient, and the importance of an antibody‐mediated mechanism. In contrast, multifocal acquired demyelinating sensory and motor neuropathy is multiple mononeuropathy or asymmetric polyneuropathy with multifocal conduction blocks and focal nerve enlargement in the intermediate nerve trunks at the site of conduction block; such distribution of lesions is reasonably explained by breakdown of the blood–nerve barrier by activated lymphocytes, suggesting a multiple sclerosis‐like cellular mechanism. Clinical features are likely to be determined by the immunopathogenesis, and therefore different immunological treatment would be required for each CIDP subtype. The present review focuses on current concepts of the disease spectrum of “CIDP syndrome” deciphered by electrophysiology and neuroimaging.

Journal

Clinical and Experimental NeuroimmunologyWiley

Published: Jan 1, 2018

Keywords: ; ; ; ;

References

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