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

Abstract

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.

• Authors: Ludivine Kouton, José Boucraut, Jérome Devaux, Yusuf A Rajabally, David Adams, Jean Christophe Antoine, Frédéric Bourdain, Alexandre Brodovitch, Jean-Philippe Camdessanché, Cécile Cauquil, Jonathan Ciron, Thierry Dubard, Andoni Echaniz-Laguna, Aude-Marie Grapperon, Raul Juntas-Morales, Laurent Kremer, Thierry Kuntzer, Céline Labeyrie, Luca Lanfranco, Jean-Marc Léger, Thierry Maisonobe, Nicolas Mavroudakis, Sylvie Mecharles-Darrigol, Philippe Merle, Jean-Baptiste Noury, Violaine Rouaud, Céline Tard, Marie Théaudin, Jean-Michel Vallat, Karine Viala, Shahram Attarian, Emilien Delmont
• Journal: Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology (Clin Neurophysiol) Vol. 131 Issue 4 Pg. 921-927 (04 2020) ISSN: 1872-8952 [Electronic] Netherlands
• PMID: 32078921 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: Copyright © 2020 International Federation of Clinical Neurophysiology. All rights reserved.

Chemical References

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

Topics

• 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, physiopathology)