Antibodies against the node of Ranvier: a real-life evaluation of incidence, clinical features and response to treatment based on a prospective analysis of 1500 sera.

Details

Serval ID
serval:BIB_3AF6527FF0D1
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Antibodies against the node of Ranvier: a real-life evaluation of incidence, clinical features and response to treatment based on a prospective analysis of 1500 sera.
Journal
Journal of neurology
Author(s)
Delmont E., Brodovitch A., Kouton L., Allou T., Beltran S., Brisset M., Camdessanché J.P., Cauquil C., Cirion J., Dubard T., Echaniz-Laguna A., Grapperon A.M., Jauffret J., Juntas-Morales R., Kremer L.D., Kuntzer T., Labeyrie C., Lanfranco L., Maisonobe T., Mavroudakis N., Mecharles-Darrigol S., Nicolas G., Noury J.B., Perie M., Rajabally Y.A., Remiche G., Rouaud V., Tard C., Salort-Campana E., Verschueren A., Viala K., Wang A., Attarian S., Boucraut J.
ISSN
1432-1459 (Electronic)
ISSN-L
0340-5354
Publication state
Published
Issued date
16/07/2020
Peer-reviewed
Oui
Language
english
Notes
Publication types: Journal Article
Publication Status: aheadofprint
Abstract
IgG4 antibodies against neurofascin (Nfasc155 and Nfasc140/186), contactin (CNTN1) and contactin-associated protein (Caspr1) are described in specific subtypes of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Our objective was to assess, in a real-life practice, the incidence, the clinical features and the response to treatment of these forms of CIDP.
1500 sera of patients suspected of having CIDP from France, Belgium and Switzerland were prospectively tested using a flow cytometry technique. The characteristics of patients with antibodies against the node of Ranvier were compared to 100 seronegative CIDP from our department.
IgG4 antibodies against Nfasc155, CNTN1, and Caspr1 were, respectively, detected in 15 (prevalence 1%), 10 (0.7%) and 2 (0.2%) sera. Antibodies specific of the Nfasc140/186 were not detected. All subjects with antibodies against the node of Ranvier fulfilled diagnostic criteria for CIDP. CIDP with anti-Nfasc155 were younger, had more sensory ataxia and postural tremor than seronegative CIDP. CIDP with anti-CNTN1 had more frequent subacute onset and facial paralysis, commoner renal involvement with membranous glomerulonephritis and greater disability, than seronegative CIDP. CIDP with anti-Caspr1 had more frequent respiratory failure and cranial nerve involvement but not more neuropathic pain than seronegative CIDP. Intravenous immunoglobulins were ineffective in most seropositive patients. Rituximab produced dramatic improvement in disability and decreased antibodies titres in 13 seropositive patients (8 with anti-Nfasc155 and 5 with anti-CNTN1 antibodies).
Although rare, anti-paranodal antibodies are clinically valuable, because they are associated with specific phenotypes and therapeutic response.
Keywords
CIDP, Caspr1, Contactin, Neurofascin 140/186, Neurofascin 155, Node of Ranvier
Pubmed
Create date
24/07/2020 13:22
Last modification date
31/07/2020 5:26
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