Encephalitis with radial perivascular emphasis: Not necessarily associated with GFAP antibodies.
Animals
Astrocytes
/ pathology
Autoantibodies
/ blood
Autoimmune Diseases of the Nervous System
/ diagnosis
Encephalitis
/ diagnosis
Glial Fibrillary Acidic Protein
/ immunology
Glucocorticoids
/ administration & dosage
Glymphatic System
/ diagnostic imaging
Humans
Magnetic Resonance Imaging
Male
Methylprednisolone
/ administration & dosage
Mice
Middle Aged
Syndrome
Journal
Neurology(R) neuroimmunology & neuroinflammation
ISSN: 2332-7812
Titre abrégé: Neurol Neuroimmunol Neuroinflamm
Pays: United States
ID NLM: 101636388
Informations de publication
Date de publication:
05 03 2020
05 03 2020
Historique:
received:
04
09
2019
accepted:
26
11
2019
entrez:
6
2
2020
pubmed:
6
2
2020
medline:
7
5
2021
Statut:
epublish
Résumé
Autoimmune steroid-responsive meningoencephalomyelitis with linear perivascular gadolinium enhancement in brain MRI is regarded as glial fibrillary acidic protein (GFAP) astrocytopathy characterized by anti-GFAP antibodies (ABs). We questioned whether anti-GFAP ABs are necessarily associated with this syndrome. Two patients with a strikingly similar disease course suggestive of autoimmune GFAP astrocytopathy are reported. Clinical examination, MRI, laboratory, and CSF analysis were performed. Neuropathologic examination of brain tissue was obtained from one patient. Serum and CSF were additionally tested using mouse brain slices, microglia-astrocyte cocultures, and a GFAP-specific cell-based assay. Both patients presented with subacute influenza-like symptoms and developed severe neurocognitive and neurologic deficits and impaired consciousness. MRIs of both patients revealed radial perivascular gadolinium enhancement extending from the lateral ventricles to the white matter suggestive of autoimmune GFAP astrocytopathy. Both patients responded well to high doses of methylprednisolone. Only one patient had anti-GFAP ABs with a typical staining pattern of astrocytes, whereas serum and CSF of the other patient were negative and showed neither reactivity to brain tissue nor to vital or permeabilized astrocytes. Neuropathologic examination of the anti-GFAP AB-negative patient revealed infiltration of macrophages and T cells around blood vessels and activation of microglia without obvious features of clasmatodendrosis. The GFAP-AB negative patient had both a striking (para)clinical similarity and an immediate response to immunotherapy. This supports the hypothesis that the clinical spectrum of steroid-responsive meningoencephalomyelitis suggestive of autoimmune GFAP astrocytopathy may be broader and may comprise also seronegative cases.
Identifiants
pubmed: 32019875
pii: 7/2/e670
doi: 10.1212/NXI.0000000000000670
pmc: PMC7051210
pii:
doi:
Substances chimiques
Autoantibodies
0
GFAP protein, human
0
Glial Fibrillary Acidic Protein
0
Glucocorticoids
0
Methylprednisolone
X4W7ZR7023
Types de publication
Case Reports
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Informations de copyright
Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.
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