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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.

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Department of Neurology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
Department of Neurology, The Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China.
Department of Pathology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
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.



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.


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).


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.


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.


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

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