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Neuroimmunomodulation. 2018;25(1):1-6. doi: 10.1159/000488879. Epub 2018 May 22.

Brain Immunohistopathology in a Patient with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy.

Author information

1
Department of Neurology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
2
Department of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China.
3
Department of Pathology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
4
Centre for Neuromuscular and Neurological Disorders, Department of Neurology, Sir Charles Gairdner Hospital, University of Western Australia, Queen Elizabeth II Medical Centre, Perth, Washington, Australia.

Abstract

BACKGROUND:

Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel meningoencephalomyelitis. However, the pathogenesis of this disease is unclear. We therefore examined a brain biopsy from a patient with autoimmune GFAP astrocytopathy by immunohistopathology.

METHODS:

We examined brain biopsy sections from a patient with autoimmune GFAP astrocytopathy using hematoxylin and eosin (HE) and Luxol fast blue (LFB) staining, and immunostaining with antibodies for CD4, CD8, CD3, CD20, CD68, CD138, Neu-N, GFAP, myelin oligodendrocyte glycoprotein (MOG), and aquaporin-4 (AQP4).

RESULTS:

HE staining revealed extensive inflammatory cells (marked lymphocytes) around brain vessels, and LFB showed no signs of demyelination or axon loss. Immunohistochemical analysis showed CD3+ and CD4+ T cells cuffing around brain vessels, accompanied by CD8+ T cells, CD20+ B cells, and CD138+ plasma cells, while some macrophages (CD68+) were scattered throughout the brain parenchyma. There was no loss of AQP4 or MOG expression in this patient, while GFAP was abundantly expressed.

CONCLUSIONS:

These findings suggest that inflammatory cells, including T cells, B cells, plasma cells, and macrophages, are involved in autoimmune GFAP astrocytopathy. Demyelination and astrocyte loss may not necessarily occur in this disease.

KEYWORDS:

Antibody; Astrocytopathy; B cells; Glial fibrillary acidic protein; T cells

PMID:
29788018
DOI:
10.1159/000488879
[Indexed for MEDLINE]

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