期刊
CLINICAL NEUROPHYSIOLOGY
卷 131, 期 4, 页码 921-927出版社
ELSEVIER IRELAND LTD
DOI: 10.1016/j.clinph.2020.01.013
关键词
Neurofascin 155; Contactin 1; CIDP; Electrophysiology
资金
- Agence Nationale pour la Recherche
- Association Francaise contre les Myopathies [21532]
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. (C) 2020 International Federation of Clinical Neurophysiology. Published by Elsevier B.V. All rights reserved.
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