Chronic inflammatory demyelinating polyradiculoneuropathy

Betty Soliven MD (Dr. Soliven of the University of Chicago has no relevant financial relationships to disclose.)
Raymond P Roos MD, editor. (Dr. Roos of the University of Chicago owns stock in Amgen, Best Doctors, Express Scripts, Ionis, and Merck.)
Originally released May 8, 1995; last updated March 12, 2017; March 12, 2020

This article includes discussion of chronic inflammatory demyelinating polyradiculoneuropathy, chronic immune demyelinating polyneuropathy, chronic inflammatory demyelinating polyneuropathy, chronic inflammatory polyradiculoneuropathy, chronic relapsing Guillain-Barre polyneuritis, chronic relapsing polyneuritis, CIDP, relapsing corticosteroid-dependent polyneuritis, relapsing hypertrophic neuritis, steroid-responsive recurrent polyneuropathy, chronic inflammatory demyelinating polyradiculoneuropathy associated with monoclonal gammopathy of undetermined significance, chronic inflammatory demyelinating polyradiculoneuropathy with severe axonal loss, distal acquired demyelinating symmetric neuropathy, multifocal acquired demyelinating sensory and motor neuropathy, and sensory chronic inflammatory demyelinating polyradiculoneuropathy. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Chronic inflammatory demyelinating polyradiculoneuropathy is sometimes referred to as the peripheral nervous system counterpart of multiple sclerosis because of similarities in clinical course and immunopathogenesis. In this article, the author explains the clinical features, criteria for the diagnosis, and updates in the pathogenesis of chronic inflammatory demyelinating polyradiculoneuropathy. Variants of chronic inflammatory demyelinating polyradiculoneuropathy are described briefly. In addition, advances in the treatment of this disorder are discussed.

Key points

 

• The Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society has revised the consensus guidelines for the definition and treatment of chronic inflammatory demyelinating polyradiculoneuropathy.

 

• Additionally, information on the immunopathogenetic mechanisms has been updated.

 

• A cluster of an immune-mediated polyradiculoneuropathy in swine abattoir workers highlights the potential of respiratory or mucosal exposure in this disorder in an occupational setting.

Historical note and terminology

The first case of recurrent neuritis was published by Eichhorst in 1890 (Eichhorst 1890), but it was Austin who first noted the response of this condition to glucocorticoid treatment (Austin 1958). Later on, the frequent occurrence of elevated CSF protein levels in this disorder was observed. The term "chronic inflammatory polyradiculoneuropathy" was used first by Dyck and colleagues to describe 53 patients with relapsing, slow monophasic, relapsing-progressive, or steadily progressive forms of acquired nonfamilial neuropathy (Dyck et al 1975). In 1984, Dyck and Arnason acknowledged the demyelinating nature of this disorder by proposing the final definition of "chronic inflammatory demyelinating polyradiculoneuropathy" (Dyck and Arnason 1984).

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