Chronic inflammatory demyelinating polyradiculoneuropathy

Betty Soliven MD (Dr. Soliven of the University of Chicago has no relevant financial relationships to disclose.)
Francesc Graus MD PhD, editor. (

Dr. Graus, Emeritus Professor, Laboratory Clinical and Experimental Neuroimmunology, Institut D’Investigacions Biomédiques August Pi I Sunyer, Hospital Clinic, Spain, has no relevant financial relationships to disclose.

Originally released May 8, 1995; last updated August 20, 2019; expires August 20, 2022

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.


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.


• Intravenous immunoglobulins or oral steroids remain the first-line treatment of chronic inflammatory demyelinating polyradiculoneuropathy.


• Alternative diagnosis or associated antibodies against paranodal antigens should be considered in nonresponders.


• A subset of patients have IgG4 antibodies against nodal and paranodal proteins that usually respond to rituximab.

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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