title: Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy.
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.
  
  
  CONCLUSIONS
  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: 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.
authors: Jérôme J
authors: JJ
authors: Devaux
authors: 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.
authors: Yumako
authors: Y
authors: Miura
authors: 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.
authors: Yuki
authors: Y
authors: Fukami
authors: 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.
authors: Takayuki
authors: T
authors: Inoue
authors: 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.
authors: Constance
authors: C
authors: Manso
authors: 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.
authors: Maya
authors: M
authors: Belghazi
authors: 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.
authors: Kenji
authors: K
authors: Sekiguchi
authors: 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.
authors: Norito
authors: N
authors: Kokubun
authors: 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.
authors: Hiroo
authors: H
authors: Ichikawa
authors: 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.
authors: Anna Hiu Yi
authors: AH
authors: Wong
authors: 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.
authors: Nobuhiro
authors: N
authors: Yuki
mesh_terms: D000293:Adolescent; D000328:Adult; D017677:Age Distribution; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood* / Q000276:immunology; D015415:Biomarkers / Q000097:blood*; D015815:Cell Adhesion Molecules / Q000097:blood* / Q000276:immunology; D002648:Child; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood* / Q000276:immunology; D015994:Incidence; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009414:Nerve Growth Factors / Q000097:blood* / Q000276:immunology; D020277:Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / Q000097:blood* / Q000175:diagnosis / Q000453:epidemiology*; D018570:Risk Assessment / Q000379:methods; D055815:Young Adult
chemical_list: D001323:Autoantibodies; D015415:Biomarkers; D015815:Cell Adhesion Molecules; D007074:Immunoglobulin G; C054305:NFASC protein, human; D009414:Nerve Growth Factors