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Review
. 2019 Jun;32(3):452-458.
doi: 10.1097/WCO.0000000000000676.

Autoimmune glial fibrillary acidic protein astrocytopathy

Amy Kunchok  1 , Anastasia Zekeridou  1   2 , Andrew McKeon  1   2
Affiliations
Review

Autoimmune glial fibrillary acidic protein astrocytopathy

Amy Kunchok et al. Curr Opin Neurol. 2019 Jun.

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.

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Figures

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Box 1
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FIGURE 1
FIGURE 1
Indirect immunofluorescence assay: staining pattern of glial elements within a composite of adult mouse brain and gastric tissues produced by patient GFAP-IgG in CSF. Patient IgG produces filamentous staining of: (a) brain parenchyma adjacent to the lateral ventricle (LV, 20×), most prominently in the subependymal zone (arrow); (b) pia and subpial region of cerebral cortex (Cx, 20×) and adjacent myenteric plexus glial cells (arrow heads); (c) pial and subpial midbrain, but sparing cerebellum (Cb, 20×). (d) 40× magnification of midbrain demonstrates filamentous staining around small blood vessels. CSF, cerebrospinal fluid; GFAP, glial fibrillary acidic protein; IgG, immunoglobulin G.
FIGURE 2
FIGURE 2
Characteristic T1 postgadolinium MR images of autoimmune GFAP astrocytopathy (axial brain, a–d; sagittal spine, e). Patterns of brain enhancement include: (a) radial periventricular; (b) leptomeningeal and punctate; (c) serpiginous; and (d), periependymal. Spinal cord enhancement, e, is characteristically central, often adjacent to the canal (arrow heads). GFAP, glial fibrillary acidic protein; MR, magnetic resonance.
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References

    1. Fang B, McKeon A, Hinson SR, et al. Autoimmune glial fibrillary acidic protein astrocytopathy: a novel meningoencephalomyelitis. JAMA Neurol 2016; 73:1297–1307. - PubMed

    2. The original study describing autoimmune GFAP astrocytopathy.

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