GFAP IgG associated inflammatory polyneuropathy

J Neuroimmunol. 2020 Jun 15:343:577233. doi: 10.1016/j.jneuroim.2020.577233. Epub 2020 Apr 1.

Abstract

Background: GFAP (glial fibrillary acidic protein)-IgG is predominantly associated with meningoencephalomyelitis, and neuropathy presentations are rare.

Methods: We reviewed clinical, electrodiagnostic and histopathological presentations of GFAP-IgG associated peripheral neuropathy.

Results: We identified six cases, five of whom had peripheral neuropathy as the initial presentation. Acute/subacute polyradicluoneuropathy or proximal nerve involvement was a common presentation. Three had demyelinating neuropathies on electrophysiological studies. Nerve biopsies (n = 2) demonstrated T-cell predominant perivascular inflammatory collections, and all patients with clinical follow up responded favorably to immunotherapy.

Conclusion: GFAP neuropathy represents a potentially treatable immune-mediated neuropathy and can occur with or without co-existing meningoencephalomyelitis.

Keywords: Antibodies; GFAP; Peripheral neuropathy; Polyradiculoneuropathy.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Autoantibodies / immunology*
  • Autoantigens / immunology*
  • Child
  • Glial Fibrillary Acidic Protein / immunology*
  • Humans
  • Immunoglobulin G / immunology
  • Immunosuppressive Agents / therapeutic use
  • Male
  • Middle Aged
  • Polyneuropathies / drug therapy
  • Polyneuropathies / immunology*
  • Polyneuropathies / pathology

Substances

  • Autoantibodies
  • Autoantigens
  • GFAP protein, human
  • Glial Fibrillary Acidic Protein
  • Immunoglobulin G
  • Immunosuppressive Agents