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

作者：Shu, Yaqing; Long, Youming; Chang, Yanyu; Li, Rui; Sun, Xiaobo; Wang, Yuge; Huang, Yinong; Li, Jing; Chen, Jianning; Yang, Yu; Lu, Zhengqi; Hu, Xueqiang; Kermode, Allan G.; Qiu, Wei^*

来源：Neuroimmunomodulation, 2018, 25(1): 1-6.

DOI：10.1159/000488879

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

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.

• 出版日期2018
• 单位中山大学; 广州医科大学