Wu Chengji, Han Xuefei, Yang Fen, Zhang Guangyun, Zhao Cong
Department of Neurology, Air Force Medical Center of PLA, Beijing, 100142, China.
Sixth Ward, Beijing Xicheng District Pingan Hospital, Beijing, 100035, China.
Neurol Sci. 2025 May 29. doi: 10.1007/s10072-025-08258-9.
Autoimmune glial fibrillary acidic protein astrocytopathy (A-GFAP-A) is a rare autoimmune central nervous system disorder associated with anti-GFAP IgG, presenting with meningoencephalitis or myelitis. Differential diagnosis from infectious causes, such as tuberculous meningitis (TBM), is challenging due to overlapping clinical and radiological features.
A 24-year-old Chinese female presented with acute headache, fever, and vomiting. The cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis, elevated protein and decreased glucose level. Brain magnetic resonance imaging (MRI) showed diffuse leptomeningeal enhancement. She was initially diagnosed with infectious meningitis and emperically treated with antibiotics and anti-tuberculosis therapy. However, her symptoms progressed with seizures, urinary retention, and tremor. Subsequent MRI revealed the involvement of the whole spinal cord. CSF analysis identified anti-GFAP IgG (titer 1:32). Bacterial, viral and tuberculous infection were excluded through bacterial culturing, metagenomic next-generation sequencing and Xpert MTB/RIF assay. The patient responded well to intravenous immunoglobulin and corticosteroids, achieving full remission. Finally, the diagnosis of A-GFAP-A was confirmed.
A-GFAP-A mimics infectious meningitis such as TBM due to similar CSF abnormalities and neuroimaging findings. This case underscores the importance of GFAP-IgG testing in differential diagnosis of patients with meningitis who have negative microbiological studies and atypical symptoms such as urinary retention and tremor.
自身免疫性胶质纤维酸性蛋白星形细胞病(A-GFAP-A)是一种罕见的自身免疫性中枢神经系统疾病,与抗GFAP IgG相关,表现为脑膜脑炎或脊髓炎。由于临床和影像学特征重叠,与感染性病因如结核性脑膜炎(TBM)进行鉴别诊断具有挑战性。
一名24岁中国女性出现急性头痛、发热和呕吐。脑脊液(CSF)分析显示淋巴细胞增多、蛋白升高和葡萄糖水平降低。脑磁共振成像(MRI)显示软脑膜弥漫性强化。她最初被诊断为感染性脑膜炎,并经验性地接受抗生素和抗结核治疗。然而,她的症状进展为癫痫发作、尿潴留和震颤。随后的MRI显示整个脊髓受累。CSF分析发现抗GFAP IgG(1:32)。通过细菌培养、宏基因组下一代测序和Xpert MTB/RIF检测排除了细菌、病毒和结核感染。患者对静脉注射免疫球蛋白和皮质类固醇反应良好,实现了完全缓解。最终,A-GFAP-A的诊断得以确诊。
由于脑脊液异常和神经影像学表现相似,A-GFAP-A可模仿感染性脑膜炎如TBM。该病例强调了GFAP-IgG检测在鉴别诊断微生物学检查阴性且有尿潴留和震颤等非典型症状的脑膜炎患者中的重要性。