ELectron microscopic abnormality and therapeutic efficacy in chronic inflammatory demyelinating polyneuropathy with anti‐neurofascin155 immunoglobulin G4 antibody

• Published in: Muscle & nerve Vol. 57; no. 3; pp. 498 - 502
• Main Authors: Kuwahara, Motoi, Suzuki, Hidekazu, Oka, Nobuyuki, Ogata, Hidenori, Yanagimoto, Satoshi, Sadakane, Shuji, Fukumoto, Yuta, Yamana, Masaki, Yuhara, Yoshiko, Yoshikawa, Keisuke, Morikawa, Miyuki, Kawai, Shigeru, Okazaki, Masahiro, Tsujimoto, Toru, Kira, Jun‐Ichi, Kusunoki, Susumu
• Format: Journal Article
• Language: English
• Published: United States Wiley Subscription Services, Inc 01.03.2018
• Subjects: Adrenal Cortex Hormones - therapeutic use; Adult; antibody; Ataxia; Autoantibodies; Autoantibodies - blood; Biopsy; Cell Adhesion Molecules - immunology; chronic inflammatory demyelinating polyneuropathy; Corticoids; Corticosteroids; Demyelination; electron microscopy; Female; Humans; IgG4; Immunoglobulin G; Immunoglobulin G4; Immunoglobulins; Immunoglobulins, Intravenous - therapeutic use; Immunotherapy; Inflammation; Intravenous administration; Male; Microscopy, Electron; Muscles; Nerve Growth Factors - immunology; neurofascin155; Patients; Plasma Exchange; Plasmapheresis; Polyneuropathy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - drug therapy; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - immunology; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating - therapy; Sural nerve; Treatment Outcome; Tremors; Young Adult; chronic inflammatory demyelinating polyneuropathy; IgG4; electron microscopy; antibody; neurofascin155; immunotherapy
• ISSN: 0148-639X; 1097-4598
• DOI: 10.1002/mus.25757

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