PDF(Pubmed)
Abstract：
A 54-year-old Japanese man presented with headache and fever the day after SARS-CoV-2 vaccination. He became deeply unconscious within a week. Brain MRI showed periventricular linear enhancements and a few spotty lesions in the cerebral white matter. Cerebrospinal fluid (CSF) testing showed mild pleocytosis. He was treated with intravenous methylprednisolone and plasma exchange. However, the white matter lesions enlarged to involve the brainstem and cerebellum, and long cord spinal lesions appeared. Anti-glial fibrillary acidic protein (GFAP) antibody was positive in the CSF and serum, and he was therefore diagnosed as autoimmune GFAP-astrocytopathy (GFAP-A). In addition, high-dose immunoglobulin therapy was administered twice, but his symptoms did not improve; the white matter lesions enlarged further, and modified Rankin Scale score increased to 5. A brain biopsy specimen showed infiltration of macrophages and CD4 + lymphocytes together with neuron and oligodendrocytic injuries and glial scar. Although GFAP-A generally responds well to steroids, the present case developed GFAP-A following SARS-CoV-2 vaccination, with refractory to intensive immunosuppressive therapy and atypical pathologic findings of infiltration of CD4 + lymphocytes and demyelination.
摘要：
一名54岁的日本男子在接种SARS-CoV-2疫苗后的第二天出现头痛和发烧。他在一周内变得非常无意识。脑MRI显示脑室周围线性增强和脑白质中的一些斑点病变。脑脊液（CSF）测试显示轻度细胞增多。他接受静脉注射甲基强的松龙和血浆置换治疗。然而,脑白质病变扩大，累及脑干和小脑，并出现脊髓长型病变。脑脊液和血清中抗胶质纤维酸性蛋白(GFAP)抗体阳性,因此，他被诊断为自身免疫性GFAP-星形细胞病（GFAP-A）。此外,大剂量免疫球蛋白治疗两次，但是他的症状没有改善；白质病变进一步扩大，改良Rankin量表评分增至5分。脑活检标本显示巨噬细胞和CD4淋巴细胞浸润，神经元和少突胶质细胞损伤以及神经胶质瘢痕。虽然GFAP-A通常对类固醇反应良好，本病例在SARS-CoV-2疫苗接种后发展为GFAP-A，难以接受强化免疫抑制治疗和CD4+淋巴细胞浸润和脱髓鞘的非典型病理结果。
