@article{d92ff643069f4a9cbd58ffcd886834df,
title = "ELectron microscopic abnormality and therapeutic efficacy in chronic inflammatory demyelinating polyneuropathy with anti-neurofascin155 immunoglobulin G4 antibody",
abstract = "Introduction: Neurofascin155 (NF155) is a target antigen for autoantibodies in a subset of chronic inflammatory demyelinating polyneuropathy (CIDP). Methods: We report the cases of 4 patients with anti-NF155 immunoglobulin G4 (IgG4) antibody-positive CIDP who underwent sural nerve biopsies. Results: All patients were relatively young at onset. Three patients experienced tremors, and 2 patients had severe ataxia. Although the response to intravenous immunoglobulin was poor in all patients, plasma exchange and corticosteroids were at least partially effective. Immunoadsorption plasmapheresis was performed in 1 patient but was ineffective. Electron microscopic examination of sural nerve biopsies revealed loss of paranodal transverse bands in all patients. Discussion: Anti-NF155 IgG4 antibody-positive CIDP shows distinctive clinicopathological features, indicating that the IgG4 antibody is directly associated with the pathogenic mechanisms of anti-NF155 IgG4 antibody-positive CIDP. Muscle Nerve 57: 498–502, 2018.",
author = "Motoi Kuwahara and Hidekazu Suzuki and Nobuyuki Oka and Hidenori Ogata and Satoshi Yanagimoto and Shuji Sadakane and Yuta Fukumoto and Masaki Yamana and Yoshiko Yuhara and Keisuke Yoshikawa and Miyuki Morikawa and Shigeru Kawai and Masahiro Okazaki and Toru Tsujimoto and Kira, {Jun Ichi} and Susumu Kusunoki",
note = "Funding Information: Abbreviations: CIDP, chronic inflammatory demyelinating polyneuropathy; CV, conduction velocity; CyA, cyclosporine A; DL, distal latency; GBS, Guillain-Barr{\'e}syndrome; IAPP, immunoadsorption plasmapheresis; IgG4, immunoglobulin G4; IVIg, intravenous immunoglobulin; IVMP, intravenous methylprednisolone; MG, myasthenia gravis; NCS, nerve conduction studies; NF155, neurofascin155; PE, plasma exchange; PSL, prednisolone; SNAP, sensory nerve action potential Key words: antibody; chronic inflammatory demyelinating polyneuropathy; electron microscopy; IgG4; immunotherapy; neurofascin155 Funding: This work was supported in part by the Ministry of Education, Culture, Sports, Science, and Technology of Japan (Grants-in-Aid for Scientific Research, 15H04845); Practical Research Project for Rare/Intractable Diseases from the Japan Agency for Medical Research and Development (16ek0109056h0003, 16ek0109115h0002); and the Ministry of Health, Labour, and Welfare of Japan (Health and Labour Sciences Research Grant on Rare and Intractable Diseases, Evidence-based Early Diagnosis and Treatment Strategies for Neuroimmunological Diseases). Conflicts of Interest: None of the authors have any conflicts of interest. Correspondence to: S. Kusunoki; e-mail: kusunoki-tky@umin.ac.jp VC 2017 Wiley Periodicals, Inc. Published online 10 August 2017 in Wiley Online Library (wileyonlinelibrary. com). DOI 10.1002/mus.25757 Publisher Copyright: {\textcopyright} 2017 Wiley Periodicals, Inc.",
year = "2018",
month = mar,
doi = "10.1002/mus.25757",
language = "English",
volume = "57",
pages = "498--502",
journal = "Muscle and Nerve",
issn = "0148-639X",
publisher = "John Wiley and Sons Inc.",
number = "3",
}