Neurology, University of California, La Jolla, California, USA
University of California, La Jolla, California, USA.
BMJ Case Rep. 2023 Mar 10;16(3):e248921. doi: 10.1136/bcr-2022-248921.
Autoimmune glial fibrillar acidic protein (GFAP) astrocytopathy is a rare autoimmune neuroinflammatory disorder that affects the central nervous system. We present a case of GFAP astrocytopathy in a middle-aged male who presented with constitutional symptoms, encephalopathy and lower extremity weakness and numbness. Initially MRI of the spine was normal, but he subsequently developed longitudinally extensive myelitis and meningoencephalitis. Workup for infectious aetiologies was negative and the patient's clinical course worsened despite broad antimicrobial coverage. Ultimately, he was found to have anti-GFAP antibodies in his cerebral spinal fluid consistent with GFAP astrocytopathy. He was treated with steroids and plasmapheresis with clinical and radiographic improvement. This case demonstrates the temporal evolution of myelitis on MRI in a case of steroid-refractory GFAP astrocytopathy.
自身免疫性神经胶质纤维酸性蛋白 (GFAP) 星形胶质细胞病是一种罕见的自身免疫性神经炎症性疾病，影响中枢神经系统。我们报告了一例中年男性的 GFAP 星形胶质细胞病，其表现为全身症状、脑病和下肢无力和麻木。最初的脊柱 MRI 正常，但随后他发展为长节段横贯性脊髓炎和脑膜脑炎。尽管进行了广泛的抗菌治疗，但对感染病因的检查均为阴性，且患者的临床病程恶化。最终，他的脑脊液中发现了抗 GFAP 抗体，符合 GFAP 星形胶质细胞病。他接受了类固醇和血浆置换治疗，临床和影像学均有改善。该病例显示了类固醇难治性 GFAP 星形胶质细胞病中脊髓炎的 MRI 时间演变。
