Electrophysiological features of chronic inflammatory demyelinating polyradiculoneuropathy associated with IgG4 antibodies targeting neurofascin 155 or contactin 1 glycoproteins. Issue 4 (April 2020)

• Record Type: Journal Article
• Authors: Kouton, Ludivine; Boucraut, José; Devaux, Jérome; Rajabally, Yusuf A.; Adams, David; Antoine, Jean Christophe; Bourdain, Frédéric; Brodovitch, Alexandre; Camdessanché, Jean-Philippe; Cauquil, Cécile; Ciron, Jonathan; Dubard, Thierry; Echaniz-Laguna, Andoni; Grapperon, Aude-Marie; Juntas-Morales, Raul; Kremer, Laurent; Kuntzer, Thierry; Labeyrie, Céline; Lanfranco, Luca; Léger, Jean-Marc; Maisonobe, Thierry; Mavroudakis, Nicolas; Mecharles-Darrigol, Sylvie; Merle, Philippe; Noury, Jean-Baptiste; Rouaud, Violaine; Tard, Céline; Théaudin, Marie; Vallat, Jean-Michel; Viala, Karine; Attarian, Shahram; Delmont, Emilien;
• Is Part Of: Clinical neurophysiology. Volume 131:Issue 4(2020:Apr.)
• Page Start: 921
• Page End: 927
• Publication Date: 2020-04
• Subjects: Neurofascin 155 -- Contactin 1 -- CIDP -- Electrophysiology; Neurophysiology -- Periodicals; Electroencephalography -- Periodicals; Electromyography -- Periodicals; Neurology -- Periodicals; 612.8
• DOI: 10.1016/j.clinph.2020.01.013
• Languages: English
• ISSNs: 1388-2457

Abstract

Highlights: Patients with antibodies against the node of Ranvier fulfil electrodiagnostic criteria for definite CIDP. Patients with anti-CNTN1 and anti-NfascC155 antibodies have similar electrophysiological patterns. Electrophysiological abnormalities are more marked in patients with antibodies. 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.