Electrophysiological features of chronic inflammatory demyelinating polyradiculoneuropathy associated with IgG4 antibodies targeting neurofascin 155 or contactin 1 glycoproteins

Clin Neurophysiol. 2020 Apr;131(4):921-927. doi: 10.1016/j.clinph.2020.01.013. Epub 2020 Feb 6.


Objective: Chronic inflammatory demyelinating polyradiculoneuropathies (CIDP) with antibodies against neurofascin 155 (Nfasc155) or contactin-1 (CNTN1) have distinctive clinical features. Knowledge on their electrophysiological characteristics is still scarce. In this study, we are investigating whether these patients have specific electrophysiological characteristics.

Methods: The electrophysiological data from 13 patients with anti-Nfasc155 IgG4 antibodies, 9 with anti-CNTN1 IgG4 antibodies were compared with those of 40 consecutive CIDP patients without antibodies.

Results: All the patients with antibodies against Nfasc155 or CNTN1 fulfilled the EFNS/PNS electrodiagnostic criteria for definite CIDP. There was no electrophysiological difference between patients with anti-CNTN1 and anti-Nfasc155 antibodies. Nerve conduction abnormalities were heterogeneously distributed along nerves trunks and roots. They were more pronounced than in CIDP without antibodies. Motor conduction velocity on median nerve <24 m/s or motor velocity on ulnar nerve <26 m/s or motor distal latency on ulnar nerve >7.4 ms were predictive of positive antibodies against the node of Ranvier with a sensitivity of 59% and a specificity of 93%.

Conclusions: Marked conduction abnormalities may suggest the presence of positive antibodies against the node of Ranvier.

Significance: Anti-Nfasc155 and anti-CNTN1 antibodies target the the paranodal axo-glial domain but are associated with nerve conduction abnormalities mimicking a "demyelinating" neuropathy.

Keywords: CIDP; Contactin 1; Electrophysiology; Neurofascin 155.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Autoantibodies / immunology
  • Cell Adhesion Molecules / immunology*
  • Contactin 1 / immunology*
  • Female
  • Humans
  • Immunoglobulin G / immunology*
  • Male
  • Median Nerve / physiopathology*
  • Middle Aged
  • Nerve Growth Factors / immunology*
  • Neural Conduction / physiology*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / immunology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / physiopathology*


  • Autoantibodies
  • Cell Adhesion Molecules
  • Contactin 1
  • Immunoglobulin G
  • NFASC protein, human
  • Nerve Growth Factors