Brain Immunohistopathology in a Patient with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy
Brain Immunohistopathology in a Patient with Autoimmune Glial Fibrillary Acidic Protein Astrocytopathy
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自身免疫性胶质纤维酸性蛋白星形细胞病患者的脑免疫组织病理学
DOI：
10.1159/000488879
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发表时间：
2018-01-01
影响因子：
2.4
通讯作者：
Qiu, Wei
中科院分区：
文献类型：
--
作者：
Shu, Yaqing;Long, Youming;Qiu, Wei
背景：自身免疫胶质纤维酸性蛋白（GFAP）星形细胞病是一种新型脑膜脑脊髓炎。然而，该病的发病机制尚不清楚。因此，我们通过免疫组织病理学检查了一名自身免疫性GFAP星形细胞病患者的脑活检。方法：我们使用苏木精伊红（HE）和Luxol快速蓝（LFB）染色，以及CD4、CD8、CD3、CD20、CD68、CD138、Neu-N、GFAP、髓鞘少突胶质细胞糖蛋白（MOG）和水通道蛋白-4 （AQP4）抗体进行免疫染色，对自身免疫性GFAP星形细胞病患者的脑活检切片进行检查。结果：HE染色显示脑血管周围有广泛的炎症细胞（淋巴细胞明显），LFB未见脱髓鞘或轴突丢失迹象。免疫组化分析显示，CD3+和CD4+ T细胞围绕在脑血管周围，伴有CD8+ T细胞、CD20+ B细胞和CD138+浆细胞，部分巨噬细胞（CD68+）分散在脑实质内。在该患者中，AQP4和MOG的表达均未减少，而GFAP则大量表达。结论：这些发现提示炎症细胞，包括T细胞、B细胞、浆细胞和巨噬细胞，参与自身免疫性GFAP星形细胞病。脱髓鞘和星形胶质细胞丢失不一定发生在本病中。
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.