以中枢系统炎症为主要表现的14例自身免疫性胶质纤维酸性蛋白星形细胞病患者的临床特征分析  

Clinical characteristics of 14 patients with autoimmune glial fibrillary acidic protein astrocytosis with central nervous system inflammation as the main manifestation

作　　者：叶锦龙 张伟劲[2] 龙友明[2] 刘晓蓉[2] 刘宝珠 李波 潘梦秋 匡祖颖 陈伯勇 王展航 YE Jinlong;ZHANG Weijing;LONG Youming(Guang Dong 999 Brain Hospital,Guangzhou 510510,China)

机构地区：[1]广东三九脑科医院神经内科,广东广州510510 [2]广州医科大学附属第二医院神经内科,广东广州510260

出　　处：《中风与神经疾病杂志》2020年第12期1101-1104,共4页Journal of Apoplexy and Nervous Diseases

摘　　要：目的探索以中枢系统炎症为主要表现的自身免疫性胶质纤维酸性蛋白(GFAP)星形细胞病患者的临床特征。方法收集2017年1月-2020年4月在广东三九脑科医院住院治疗的21例脑脊液GFAP抗体阳性的自身免疫性GFAP星形细胞病患者,回顾性分析其中以脑脊髓膜炎为主要表现的14例患者的临床资料。结果14例患者中,女性4例,男性10例,发病年龄(42±16)岁。患者的主要临床表现为头痛(13/14)、发热(13/14)、意识障碍(11/14)、精神症状(10/14)、癫痫发作(4/10)、排便障碍(8/14)、低氧血症(6/14)、肢体瘫痪(2/14)等。MRI提示颅内脑膜异常强化13例(13/14),累及脊髓5例(5/14)。结论以脑脊髓膜炎等中枢系统炎症为主的自身免疫性GFAP星形细胞病患者男性多于女性,MRI表现形式复杂,颅内和脊髓可以同时受累,颅内病变主要以脑膜受累为主,可合并大脑皮质损害。在脊髓病变中脊髓膜及脊髓均可受累,并且以累及脊髓膜为主。Objective To explore the clinical characteristics of patients with autoimmune glial fibrillary acidic protein(GFAP)astrocytosis with central nervous system inflammation as the main manifestation.Methods Twenty-one patients with autoimmune GFAP astrocytosis with positive cerebrospinal fluid GFAP antibody were collected from January 2017 to April 2020.The clinical data of 14 patients with cerebrospinal fluid GFAP antibody positive were retrospectively analyzed.Results Among the 14 patients,4 were female and 10 were male.The age of onset was(42±16)years old.The main clinical manifestations of the patients were headache(13/14),fever(13/14),disturbance of consciousness(11/14),mental symptoms(10/14),epilepsy(4/10),defecation disorder(8/14),hypoxemia(6/14),limb paralysis(2/14),etc.MRI showed abnormal enhancement of intracranial meninges in 13 cases(13/14)and spinal cord involvement in 5 cases(5/14).Conclusion There are more males than females with autoimmune GFAP astrocytosis with cerebrospinal meningitis and other central system inflammation.MRI manifestations are complex.Intracranial and spinal cord can be involved at the same time.Intracranial lesions mainly involve meninges,which can be combined with cerebral cortex damage.In myelopathy,both the spinal cord and the spinal cord are involved,and the spinal cord membrane is mainly involved.

关 键 词：自身免疫疾病 胶质纤维酸性蛋白星形细胞 脑膜炎 脑脊髓炎 脊髓炎 

分 类 号：R742[医药卫生—神经病学与精神病学]