Autoimmune glial fibrillary acidic protein astrocytopathy

Amy Kunchok, Anastasia Zekeridou, Andrew McKeon

Research output: Contribution to journalReview article

12 Scopus citations

Abstract

Purpose of reviewTo describe a recently characterized autoimmune, inflammatory central nervous system (CNS) disorder known as autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.Recent findingsAffected patients present with symptoms of one or more of meningitis (headache and neck ache), encephalitis (delirium, tremor, seizures, or psychiatric symptoms), and myelitis (sensory symptoms and weakness). Optic disc papillitis (blurred vision) is common. CNS inflammation is evident in characteristic T1 postgadolinium enhancement of GFAP-enriched CNS regions, and lymphocytic cerebrospinal fluid (CSF) white cell count elevation. CSF is more reliable than serum for GFAP-immunoglobulin G (IgG) testing. Ovarian teratoma commonly coexists, particularly among patients with accompanying N-methyl-D-aspartate receptor or aquaporin-4 autoimmunity. Parainfectious autoimmunity is suspected in some other patients, though the culprit organism is rarely verified. Pathophysiologic relevance of T cells is underscored by neuropathology and cases of dysregulated T-cell function (HIV or checkpoint inhibitor cancer therapy). Corticosteroid-responsiveness is a hallmark of the disease. Relapses occur in approximately 20% of patients, necessitating transition to a steroid-sparing drug. Reported outcomes vary, though in the authors' experience, early and sustained intervention usually portends recovery.SummaryAutoimmune GFAP astrocytopathy is a treatable autoimmune CNS disease diagnosable by GFAP-IgG testing in CSF. This disease presents opportunities to explore novel mechanisms of CNS autoimmunity and inflammation.

Original languageEnglish (US)
Pages (from-to)452-458
Number of pages7
JournalCurrent opinion in neurology
Volume32
Issue number3
DOIs
StatePublished - Jun 1 2019

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Keywords

  • encephalitis
  • glial fibrillary acidic protein
  • meningitis
  • myelitis
  • steroids

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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