Autoimmune glial fibrillary acidic protein astrocytopathy

Amy Kunchok, Anastasia Zekeridou, Andrew B McKeon

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

PURPOSE OF REVIEW: To describe a recently characterized autoimmune, inflammatory central nervous system (CNS) disorder known as autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy. RECENT FINDINGS: Affected 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. SUMMARY: Autoimmune 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

Fingerprint

Glial Fibrillary Acidic Protein
Autoimmunity
Cerebrospinal Fluid
Central Nervous System
Central Nervous System Diseases
Autoimmune Diseases of the Nervous System
Immunoglobulin G
Aquaporin 4
Myelitis
Inflammation
T-Lymphocytes
Patient Transfer
Papilledema
Delirium
Neck Pain
Optic Disk
Tremor
Encephalitis
N-Methyl-D-Aspartate Receptors
Meningitis

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Autoimmune glial fibrillary acidic protein astrocytopathy. / Kunchok, Amy; Zekeridou, Anastasia; McKeon, Andrew B.

In: Current opinion in neurology, Vol. 32, No. 3, 01.06.2019, p. 452-458.

Research output: Contribution to journalArticle

Kunchok, Amy ; Zekeridou, Anastasia ; McKeon, Andrew B. / Autoimmune glial fibrillary acidic protein astrocytopathy. In: Current opinion in neurology. 2019 ; Vol. 32, No. 3. pp. 452-458.
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