Autoimmune Nodopathies

Lyou, Hyun Ji; Shin, Ha Young

J Mult Scler Neuroimmunol > Volume 13(2); 2022 > Article

Review Article

Journal of Multiple Sclerosis and Neuroimmunology 2022;13(2):23-29.
Published online December 31, 2022.

자가면역결절병증

유현지, 신하영

연세대학교 의과대학 신경과학교실

Autoimmune Nodopathies

Hyun Ji Lyou, Ha Young Shin

Department of Neurology, Yonsei University College of Medicine, Seoul, Korea

Correspondence: Ha Young Shin, Tel: +82-2-2228-1600, Fax: +82-2-393-0705,
Email: hayshin@yuhs.ac

Received: 9 August 2022 • Revised: 23 September 2022 • Accepted: 15 October 2022

Abstract

Autoantibodies against proteins at the node of Ranvier such as neurofascin-155, contactin-1, contactin-associated protein-1, and neurofascin-140/186 have been discovered in a proportion of patients with peripheral neuropathy fulfilling the previous diagnostic criteria for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) (for example, European Federation of Neurological Societies and the Peripheral Nerve Society [EFNS/PNS] 2010 criteria). Although peripheral neuropathies with these autoantibodies can meet the criteria, they show distinct clinical characteristics, such as poor response to intravenous immunoglobulin and/ or corticosteroids and no macrophage-mediated demyelination or overt inflammation, which are not typical of CIDP. For these reasons, a new concept of? “autoimmune nodopathy”? has been proposed and distinguished from CIDP. This review briefly summarized the structure of the node of Ranvier and autoimmune nodopathies associated with autoantibodies against neurofascin-155, contactin-1, contactin-associated protein-1, and neurofascin-140/186. Journal of Multiple Sclerosis and Neuroimmunology 13(2):23-29, 2022

Key Words: Chronic inflammatory demyelinating polyradiculoneuropathy, Ranvier’s nodes, Autoantibodies