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GFAP IgG associated inflammatory polyneuropathy.

Pritikanta Paul1, Andrew McKeon2, Sean J Pittock2

  • 1Department of Neurology, Mayo Clinic, Rochester, MN, United States of America.

Journal of Neuroimmunology
|April 10, 2020
PubMed
Summary
This summary is machine-generated.

Glial fibrillary acidic protein (GFAP)-IgG associated neuropathy is a rare but treatable immune-mediated condition. Early diagnosis and immunotherapy can lead to favorable outcomes in patients with GFAP neuropathy.

Keywords:
AntibodiesGFAPPeripheral neuropathyPolyradiculoneuropathy

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Area of Science:

  • Neuroimmunology
  • Neuropathology

Background:

  • Glial fibrillary acidic protein (GFAP)-IgG is typically linked to meningoencephalomyelitis.
  • Peripheral neuropathy is an uncommon manifestation of GFAP-IgG.

Observation:

  • Reviewed six cases of GFAP-IgG associated peripheral neuropathy.
  • Five patients presented initially with neuropathy, often acute/subacute polyradiculoneuropathy or proximal nerve involvement.
  • Electrophysiological studies indicated demyelinating neuropathies in three patients.

Findings:

  • Nerve biopsies revealed T-cell predominant perivascular inflammatory infiltrates.
  • All patients who received immunotherapy showed a positive clinical response.

Implications:

  • GFAP-IgG neuropathy is a distinct, treatable immune-mediated peripheral neuropathy.
  • It can manifest independently of or concurrently with meningoencephalomyelitis.
  • Highlights the importance of considering GFAP-IgG in the differential diagnosis of immune-mediated neuropathies.