ARTHUR ASBURY LECTURE: Chronic inflammatory demyelinating polyradiculoneuropathy: clinical aspects and new animal models of auto-immunity to nodal components

J Peripher Nerv Syst. 2017 Dec;22(4):418-424. doi: 10.1111/jns.12237. Epub 2017 Nov 15.


Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder of the peripheral nerves with clinical and immunological heterogeneity. Both cellular and humoral immune mechanisms against peripheral nerve antigens are considered to contribute to the pathogenesis of the disorder. Currently, the diagnosis of CIDP is based on clinical, laboratory and electrophysiological criteria. The field of CIDP recently underwent a major change with the identification of autoantibodies directed against paranodal (CNTN1, CASPR1 and NF155) and nodal (NF186/140) proteins. Over the last 5 years, correlations have been found between these autoantibodies and CIDP clinical subtypes including the likelihood of response to specific immunotherapies. Additionally, during this time a series of experimental studies have unraveled the underlying immunopathogenesis for CNTN1 and NF155 antibody associated CIDP. Although paranodal and nodal autoantibodies are only found in a small subset of patients with CIDP, the detection of these immune biomarkers should be incorporated in the evaluation of patients, considering the implications of their presence on prognosis, follow-up, and treatment decisions.

Keywords: ANTI NF155 antibodies; ANTI-CASPR-1; ANTI-CNTN antibodies; ANTI-NF 186 antibodies; CIDP; antibodies; autoimmunity; immune therapy; nodopathies.

Publication types

  • Review
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Autoantibodies / immunology*
  • Humans
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / immunology*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / therapy
  • Ranvier's Nodes / immunology*


  • Autoantibodies