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

• DOI: 10.1159/000488879
• Publication Date: 2018-05-22T21:01:22Z
• Authors: Yaqing Shu; Youming Long; Yanyu Chang; Rui Li; Xiaobo Sun; Yuge Wang; Yinong Huang; Jing Li; Jianning Chen; Yu Yang; Zhengqi Lu; Xueqiang Hu; Allan G. Kermode; Wei Qiu
• Keywords: GFAP stain; Neuroimmunology

<b><i>Background:</i></b> Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy is a novel meningoencephalomyelitis. However, the pathogenesis of this disease unclear. We therefore examined brain biopsy from patient with autoimmune GFAP by immunohistopathology. <b><i>Methods:</i></b> sections using hematoxylin and eosin (HE) Luxol fast blue (LFB) staining, immunostaining antibodies for CD4, CD8, CD3, CD20, CD68, CD138, Neu-N, GFAP, myelin oligodendrocyte glycoprotein (MOG), aquaporin-4 (AQP4). <b><i>Results:</i></b> HE staining revealed extensive inflammatory cells (marked lymphocytes) around vessels, LFB showed no signs demyelination or axon loss. Immunohistochemical analysis CD3<sup>+</sup> CD4<sup>+</sup> T cuffing accompanied CD8<sup>+</sup> cells, CD20<sup>+</sup> B CD138<sup>+</sup> plasma while some macrophages (CD68<sup>+</sup>) were scattered throughout parenchyma. There was loss AQP4 MOG expression in patient, abundantly expressed. <b><i>Conclusions:</i></b> These findings suggest that including macrophages, are involved astrocytopathy. Demyelination astrocyte may not necessarily occur disease.