Department of Neurology, Gifu Prefectural General Medical Center, Gifu, Japan.
Department of Neurology, Gifu University Graduate School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan.
BMC Neurol. 2023 Apr 4;23(1):145. doi: 10.1186/s12883-023-03194-7.
Autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy (GFAP-A) is an autoimmune inflammatory central nervous system disorder characterized by the detection of autoantibodies that recognize GFAP in CSF. The pathogenesis of GFAP-A is poorly understood. Some patients had a neoplasm detected and GFAP expressed by neoplasms is plausible as immunogen triggering paraneoplastic neurological autoimmunity.
We report a case of 76-year-old female patient with GFAP-A complicated with breast cancer. She presented with altered consciousness, nuchal rigidity, speech disturbances, and weakness. Her clinical symptoms were improved by immunotherapy and cancer treatments. Immunohistochemical analysis showed that the restricted tumor expressed GFAP. The infiltration of CD3 + T cells were observed in the peritumoral and intratumoral areas. The most common infiltrating lymphocytes were CD8 + T cells. CD4 + T cells and CD20 + B cells were also observed in the predominant peritumoral area.
These results suggest that GFAP-A may occur in a paraneoplastic neurological syndrome associated with breast cancer.
自身免疫性神经胶质纤维酸性蛋白（GFAP）星形胶质细胞病（GFAP-A）是一种自身免疫性炎症性中枢神经系统疾病，其特征是在 CSF 中检测到识别 GFAP 的自身抗体。GFAP-A 的发病机制尚不清楚。一些患者检测到肿瘤，并且肿瘤表达的 GFAP 作为触发副肿瘤性神经自身免疫的免疫原是合理的。
我们报告了一例 76 岁女性 GFAP-A 合并乳腺癌患者。她表现为意识改变、颈项强直、言语障碍和无力。她的临床症状通过免疫治疗和癌症治疗得到改善。免疫组织化学分析显示，受限制的肿瘤表达 GFAP。在肿瘤周围和肿瘤内区域观察到 CD3+T 细胞浸润。最常见的浸润淋巴细胞是 CD8+T 细胞。在主要的肿瘤周围区域也观察到 CD4+T 细胞和 CD20+B 细胞。
这些结果表明，GFAP-A 可能发生在与乳腺癌相关的副肿瘤性神经综合征中。
