GFAP astrocytopathy presenting with profound intracranial hypertension and vision loss

J Neuroimmunol. 2022 Dec 15:373:577976. doi: 10.1016/j.jneuroim.2022.577976. Epub 2022 Oct 3.

Abstract

Background: Glial fibrillary acidic protein (GFAP) astrocytopathy is a steroid-responsive autoimmune meningoencephalomyelitis commonly preceded by a viral illness. It is clinically characterized by encephalopathy, myelopathy and papillitis without significant effect on visual acuity. It can be associated with an underlying malignancy or autoimmune condition.

Objective: To report a novel case of GFAP astrocytopathy presenting with profound intracranial hypertension and bilateral vision loss.

Methods: Case report.

Results and conclusion: GFAP astrocytopathy should be considered when evaluating patients with intracranial hypertension or bilateral vision loss, particularly when other features of autoimmune encephalitis are present.

Keywords: Autoimmune encephalitis; GFAP astrocytopathy; Glial fibrillary acidic protein; Intracranial hypertension; Optic neuropathy.

Publication types

  • Case Reports

MeSH terms

  • Astrocytes / metabolism
  • Autoantibodies
  • Autoimmune Diseases* / pathology
  • Encephalitis* / pathology
  • Glial Fibrillary Acidic Protein / metabolism
  • Humans
  • Intracranial Hypertension* / complications
  • Intracranial Hypertension* / diagnosis
  • Intracranial Hypertension* / metabolism
  • Vision Disorders / etiology

Substances

  • Glial Fibrillary Acidic Protein
  • Autoantibodies