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  

Leptomeningeal abnormality in autoimmune glial fibrillary acidic protein-IgG disease

作　　者：姚海燕[1] 江丽红 李惠璐 刘思 高聪[1] 龙友明[1] 杨新光[1] YAO Hai-yan;JIANG Li-hong;LI Hui-lu;LIU Si;GAO Cong;LONG You-ming;YANG Xin-guang(Department of Neurology,the Second Affiliated Hospital of Guangzhou Medical Unirersity,Guangzhou 510260,Guangdong,China)

机构地区：[1]广州医科大学附属第二医院神经内科、神经科学研究所,广东广州510260

出　　处：《广东医学》2021年第10期1203-1206,共4页Guangdong Medical Journal

基　　金：国家自然科学基金资助项目(81771302);广东省医学科学技术研究基金项目(B2021235);广州市民生科技计划项目(201803010123,202002020021);广州医科大学附属第二医院新技术、新业务临床研究项目(2019-hs-11,2020-hs-54)。

摘　　要：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-IgG55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。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.

关 键 词：抗体 星形细胞 胶质纤维酸性蛋白 磁共振 

分 类 号：R74[医药卫生—神经病学与精神病学] R445[医药卫生—临床医学]