Jérôme J Devaux1, Yumako Miura1, Yuki Fukami1, Takayuki Inoue1, Constance Manso1, Maya Belghazi1, Kenji Sekiguchi1, Norito Kokubun1, Hiroo Ichikawa1, Anna Hiu Yi Wong1, Nobuhiro Yuki2. 1. From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan. 2. From Aix-Marseille Université (J.J.D., C.M., M.B.), CNRS, CRN2M-UMR 7286, Marseille, France; Departments of Medicine (Y.M., Y.F., T.I., A.H.Y.W., N.Y.) and Physiology (N.Y.), Yong Loo Lin School of Medicine, National University of Singapore; Brain and Mind Centre (N.Y.), University of Sydney, Australia; Division of Neurology (K.S.), Kobe University Graduate School of Medicine; Department of Neurology (N.K.), Dokkyo Medical University, Tochigi; and Department of Neurology (H.I.), Brain Nerve Center, Showa University Fujigaoka Hospital, Tokyo, Japan. gbs.yuki.cidp@gmail.com.
Abstract
OBJECTIVE: We report the clinical and serologic features of Japanese patients with chronic inflammatory demyelinating polyneuropathy (CIDP) displaying anti-neurofascin-155 (NF155) immunoglobulin G4 (IgG4) antibodies. METHODS: In sera from 533 patients with CIDP, anti-NF155 IgG4 antibodies were detected by ELISA. Binding of IgG antibodies to central and peripheral nerves was tested. RESULTS: Anti-NF155 IgG4 antibodies were identified in 38 patients (7%) with CIDP, but not in disease controls or normal participants. These patients were younger at onset as compared to 100 anti-NF155-negative patients with CIDP. Twenty-eight patients (74%) presented with sensory ataxia, 16 (42%) showed tremor, 5 (13%) presented with cerebellar ataxia associated with nystagmus, 3 (8%) had demyelinating lesions in the CNS, and 20 of 25 (80%) had poor response to IV immunoglobulin. The clinical features of the antibody-positive patients were statistically more frequent as compared to negative patients with CIDP (n = 100). Anti-NF155 IgG antibodies targeted similarly central and peripheral paranodes. CONCLUSION: Anti-NF155 IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset, ataxia, tremor, CNS demyelination, and a poor response to IV immunoglobulin. The autoantibodies may serve as a biomarker to improve patients' diagnosis and guide treatments.
OBJECTIVE: We report the clinical and serologic features of Japanese patients with chronic inflammatory demyelinating polyneuropathy (CIDP) displaying anti-neurofascin-155 (NF155) immunoglobulin G4 (IgG4) antibodies. METHODS: In sera from 533 patients with CIDP, anti-NF155 IgG4 antibodies were detected by ELISA. Binding of IgG antibodies to central and peripheral nerves was tested. RESULTS: Anti-NF155 IgG4 antibodies were identified in 38 patients (7%) with CIDP, but not in disease controls or normal participants. These patients were younger at onset as compared to 100 anti-NF155-negative patients with CIDP. Twenty-eight patients (74%) presented with sensory ataxia, 16 (42%) showed tremor, 5 (13%) presented with cerebellar ataxia associated with nystagmus, 3 (8%) had demyelinating lesions in the CNS, and 20 of 25 (80%) had poor response to IV immunoglobulin. The clinical features of the antibody-positive patients were statistically more frequent as compared to negative patients with CIDP (n = 100). Anti-NF155 IgG antibodies targeted similarly central and peripheral paranodes. CONCLUSION: Anti-NF155 IgG4 antibodies were associated with a subgroup of patients with CIDP showing a younger age at onset, ataxia, tremor, CNS demyelination, and a poor response to IV immunoglobulin. The autoantibodies may serve as a biomarker to improve patients' diagnosis and guide treatments.
Authors: P Y K Van den Bergh; R D M Hadden; P Bouche; D R Cornblath; A Hahn; I Illa; C L Koski; J-M Léger; E Nobile-Orazio; J Pollard; C Sommer; P A van Doorn; I N van Schaik Journal: Eur J Neurol Date: 2010-03 Impact factor: 6.089
Authors: Richard A C Hughes; Peter Donofrio; Vera Bril; Marinos C Dalakas; Chunqin Deng; Kim Hanna; Hans-Peter Hartung; Norman Latov; Ingemar S J Merkies; Pieter A van Doorn Journal: Lancet Neurol Date: 2008-02 Impact factor: 44.182
Authors: W I McDonald; A Compston; G Edan; D Goodkin; H P Hartung; F D Lublin; H F McFarland; D W Paty; C H Polman; S C Reingold; M Sandberg-Wollheim; W Sibley; A Thompson; S van den Noort; B Y Weinshenker; J S Wolinsky Journal: Ann Neurol Date: 2001-07 Impact factor: 10.422