Neurofascin-155 IgG4 in chronic inflammatory demyelinating polyneuropathy


  • Devaux Jérôme J.
  • Miura Yumako
  • Fukami Yuki
  • Inoue Takayuki
  • Manso Constance
  • Belghazi Maya
  • Sekiguchi Kenji
  • Kokubun Norito
  • Ichikawa Hiroo
  • Wong Anna Hiu Yi
  • Yuki Nobuhiro


  • Autoantibodies
  • Biomarkers
  • Cell Adhesion Molecules
  • Child
  • Female
  • Humans
  • Immunoglobulin G
  • Incidence
  • Japan
  • Male
  • Middle Aged
  • Nerve Growth Factors
  • Polyradiculoneuropathy
  • Chronic Inflammatory Demyelinating
  • Risk Assessment
  • Young Adult
  • Adolescent
  • Adult
  • Aged
  • 80 and over
  • Age Distribution


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.ăMETHODS: 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.ăRESULTS: 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.ăCONCLUSION: 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.

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