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  • Updated 09.05.2022
  • Released 05.08.1995
  • Expires For CME 09.05.2025

Chronic inflammatory demyelinating polyradiculoneuropathy

Introduction

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 briefly described. 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.

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

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

• Patients with IgG4 antibodies against nodal and paranodal proteins and a good response to rituximab are now classified as having autoimmune nodopathies.

Historical note and terminology

The first case of recurrent neuritis was published by Eichhorst in 1890 (31), but it was Austin who first noted the response of this condition to glucocorticoid treatment (03). 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 (27). In 1984, Dyck and Arnason acknowledged the demyelinating nature of this disorder by proposing the final definition of "chronic inflammatory demyelinating polyradiculoneuropathy" (25).

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