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

Muscle Nerve. 2018 Mar;57(3):498-502. doi: 10.1002/mus.25757. Epub 2017 Aug 23.


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.

Keywords: IgG4; antibody; chronic inflammatory demyelinating polyneuropathy; electron microscopy; immunotherapy; neurofascin155.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Adult
  • Autoantibodies / blood
  • Cell Adhesion Molecules / immunology*
  • Female
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Male
  • Microscopy, Electron
  • Nerve Growth Factors / immunology*
  • Plasma Exchange*
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / drug therapy
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / immunology
  • Polyradiculoneuropathy, Chronic Inflammatory Demyelinating / therapy*
  • Treatment Outcome
  • Young Adult


  • Adrenal Cortex Hormones
  • Autoantibodies
  • Cell Adhesion Molecules
  • Immunoglobulins, Intravenous
  • NFASC protein, human
  • Nerve Growth Factors