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Chronic inflammatory demyelinating polyneuropathy: The spectrum and immunopathogenesis deciphered by electrophysiology and neuroimaging
Author(s) -
Kuwabara Satoshi,
Misawa Sonoko,
Mori Masahiro
Publication year - 2018
Publication title -
clinical and experimental neuroimmunology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.297
H-Index - 15
ISSN - 1759-1961
DOI - 10.1111/cen3.12431
Subject(s) - chronic inflammatory demyelinating polyneuropathy , medicine , polyneuropathy , magnetic resonance neurography , pathology , neuroscience , multifocal motor neuropathy , mononeuropathy , magnetic resonance imaging , peripheral neuropathy , immunology , mismatch negativity , antibody , electroencephalography , psychology , diabetes mellitus , endocrinology , psychiatry , radiology
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.