自身免疫性胶质纤维酸性蛋白星形细胞病的脑膜脊膜病变研究

目的 探讨自身免疫性胶质纤维酸性蛋白星形细胞病的脑膜脊膜病变特点，旨在提高临床医师对自身免疫性胶质纤维酸性蛋白星形细胞病（GFAP-A）的认识与诊治。方法一项回顾性病例对照研究。通过免疫荧光的方法检测出阳性的GFAP-IgG 55例，其中28例患者完成头颅MRI的平扫和增强(25例同时完成脊髓MRI平扫和增强)。对照组为同期住院的27例AQP4-IgG阳性的视神经脊髓炎谱系疾病患者，均完成头颅和脊髓MRI平扫和增强。结果纳入28例GFAP-A，主要临床症状包括头痛（464%，13/28）、发热（571%，16/28）、脊髓炎（429%，12/28）、视觉异常（393%，11/28）、行为异常（286%，8/28）、共济失调（250%，7/28）、意识障碍（143%，4/28）、癫痫发作(107%, 3/28)、运动障碍（71%，2/28）、认知障碍(107%，3/28)和其他症状（25%，7/28）。MRI增强下15例（536%)出现脑膜和（或）脊膜的异常信号，其中脑膜异常8例（286%)，脊膜异常3例(107%)，脑膜与脊膜同时异常4例（143%）。两例病理资料显示脑膜增厚，慢性炎症改变。与视神经脊髓炎谱系疾病对比显示性别比例、发热、头痛、行为异常、视觉异常、MRI放射性血管样强化、脊髓异常、长节段脊髓炎、脑膜和（或）脊膜异常差异均有统计学意义（P<005）。结论脑膜/脊膜异常在GFAP-A比较常见，但临床或者放射学医生可能容易忽视，需提高对GFAP-A影像学特点的认识。

Leptomeningeal abnormality in autoimmune glial fibrillary acidic protein -IgG disease

Objective To investigate the leptomeningeal abnormality in patients with autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A). Methods A retrospective analysis of patients with GFAP-IgG was performed. CSF GFAP-IgG were measured via tissue-based assay and/or cell-based assays. Results Twenty-eight patients with headache (464%, 13/28), fever (571%, 16/28), myelitis (429%, 12/28), abnormal vision (393%, 11/28), ataxia (25%, 7/28), abnormal behavior (286%, 8/28), coma (143%, 4/28), epilepsy (107%, 3/28), dyskinesia (71%, 2/28), dementia (107%, 3/28) and other manifestations (25%, 7/28) were included. There were 15 patients (536%) with leptomeningeal enhancement, including eight (286%) with brain meninges abnormality, three (107%) with spinal meninge abnormality, and four (143%) with both abnormalities. GFAP-A and NMOSD were different in ratio of sex, fever, headache, vision abnormality, abnormal behavior, and radial enhancement and leptomeningeal enhancement in MRI (P<005). Conclusion Leptomeningeal abnormality is usually found in GFAP-A but may be overlooked by clinician and radiologist, which requires more attentions.