Zhongmin Yuan1,2,3, Huilu Li1,4, Lu Huang1,4, Congcong Fu1,4, Yaotang Chen5, Cheng Zhi6, Wei Qiu7, Youming Long1,4. 1. Institute of Neuroscience and Department of Neurology, Second Affiliated Hospital of Guangzhou Medical University, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Guangzhou, China. 2. Guangdong Province Key Laboratory of Brain Function and Disease, Guangzhou, China. 3. Guangdong-Hong Kong-Macao Greater Bay Area Center for Brain Science and Brain-Inspired Intelligence, Guangzhou, China. 4. Department of Neurology, Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China. 5. Department of Radiology, Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China. 6. Department of Pathology, Second Affiliated Hospital of Guangzhou Medical University, Guangzhou, China. 7. Department of Neurology, Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, China.
Abstract
BACKGROUND AND OBJECTIVE: We aimed to report the pathological features of T lymphocytes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A). METHODS: A retrospective pathological analysis of patients with GFAP-A was performed. RESULTS: Eight patients with GFAP-immunoglobulin G (IgG) and pathological data were included. Their biopsy findings were similar, and all showed marked lymphocytic infiltration in the white matter, with perivascular predominance. The lymphocytic infiltration was predominantly composed of CD8+ T lymphocytes rather than CD4+ T lymphocytes, except in one patient who had overlapping positive myelin oligodendrocyte glycoprotein-IgG. Unlike CD4+ T cells, CD8+ T cells were frequently observed adjacent to dystrophic neurons and astrocytes. There was also diffuse infiltration by CD68+ and CD163+ macrophages. CD8+ astrocytes were identified in two samples, but no CD4+ astrocytes were observed. CONCLUSIONS: A predominance of CD8+ T cells may be an important pathological and diagnostic feature in GFAP-A.
BACKGROUND AND OBJECTIVE: We aimed to report the pathological features of T lymphocytes in autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A). METHODS: A retrospective pathological analysis of patients with GFAP-A was performed. RESULTS: Eight patients with GFAP-immunoglobulin G (IgG) and pathological data were included. Their biopsy findings were similar, and all showed marked lymphocytic infiltration in the white matter, with perivascular predominance. The lymphocytic infiltration was predominantly composed of CD8+ T lymphocytes rather than CD4+ T lymphocytes, except in one patient who had overlapping positive myelin oligodendrocyte glycoprotein-IgG. Unlike CD4+ T cells, CD8+ T cells were frequently observed adjacent to dystrophic neurons and astrocytes. There was also diffuse infiltration by CD68+ and CD163+ macrophages. CD8+ astrocytes were identified in two samples, but no CD4+ astrocytes were observed. CONCLUSIONS: A predominance of CD8+ T cells may be an important pathological and diagnostic feature in GFAP-A.