自身免疫性胶质纤维酸性蛋白星形细胞病16例临床特征分析  

Clinical analysis of 16 cases of autoimmune glial fibrillary acidic protein astrocytopathy

作　　者：江佳佳 杨伊 尹梓曈 赵辉[1] 霍颖浩[1] 卜晖[1] JIANG Jiajia;YANG Yi;YIN Zitong;ZHAO Hui;HUO Yinghao;BU Hui(The Second Hospital of Hebei Medical University,Shijiazhuang 050004,China)

机构地区：[1]河北医科大学第二医院神经内科,石家庄050004

出　　处：《中国神经精神疾病杂志》2023年第8期475-482,共8页Chinese Journal of Nervous and Mental Diseases

摘　　要：目的探讨自身免疫性胶质纤维酸性蛋白(glial fibrillary acidic protein,GFAP)星形细胞病患者的临床特征。方法回顾性分析2021年1月至2023年5月在河北医科大学第二医院就诊的脑脊液GFAP阳性的自身免疫性GFAP星形细胞病患者病历资料。结果16例患者发病年龄21~66岁,平均(40.56±12.04)岁,其中男10例,女6例。主要首发表现为发热(14/16)、头痛(12/16)、尿便障碍(12/16)、恶心呕吐(7/16)、双下肢无力(7/16)、意识障碍(4/16)、记忆力减退(2/16),其中9例患者最初考虑诊断为结核性脑膜炎,给予诊断性抗结核治疗。2例因呼吸衰竭需机械通气。无患者发现肿瘤。14例患者对激素和免疫球蛋白治疗反应好,2例患者预后不佳。结论自身免疫性GFAP星形细胞病临床表现多样,早期易误诊为结核性脑膜脑炎,对激素及免疫治疗敏感。Objective To analyze the clinical characteristics of autoimmune glial fibrillary acidic protein(GFAP)astrocytopathy.Methods The clinical data of 16 patients with autoimmune GFAP astrocytopathy confirmed by detection of GFAP-IgG in cerebrospinal fluid(CSF)admitted and diagnosed in the second Hospital of Hebei Medical University from January 2021 to May 2023 were retrospectively analyzed.Results The average age of these 16 patients was 40.56±12.04 years old(range 21~66),including 10 males and 6 females.The main initial manifestations included fever(14/16),headache(12/16),urination and defecation function disturbance(12/16),nausea and vomiting(7/16),limb weakness(7/16),disturbance of consciousness(4/16),and hypomnesia(2/16).9 patients initially considered diagnosis of tuberculous meningitis and were treated with diagnostic anti-tuberculosis treatment.2 patients with respiratory failure needed mechanical ventilation.No tumor was found in patients.Fourteen patients responded well to corticosteroid and immunotherapy.The median follow-up time was 12.5 months.2 patients had poor prognosis.Conclusion Autoimmune glial fibrillary acidic protein astrocytopathy has diverse clinical manifestations.In the early stage,it is easy to be misdiagnosed as tuberculous meningoencephalitis.Many patients were complicated with hyponatremia.Most patients are good response to corticosteroid and immunotherapy.

关 键 词：自身免疫性胶质纤维酸性蛋白星形细胞病 GFAP-IgG 重叠的自身免疫综合征 脑膜脑炎 结核性脑膜炎 激素治疗 免疫治疗 呼吸衰歇 

分 类 号：R741[医药卫生—神经病学与精神病学]