自身免疫性胶质纤维酸性蛋白星形胶质细胞病临床特征分析

Clinical characteristics of autoimmune glial fibrillary acidic protein astrocytopathy

目的:探讨自身免疫性胶质纤维酸性蛋白星形胶质细胞病(GFAP-A)的临床特征。方法:回顾性分析自2020年12月1日至2022年5月1日郴州市第一人民医院神经内科收治的14例自身免疫性GFAP-A患者的临床资料。结果:14例患者的年龄为(46±31)岁,其中男性明显多于女性(10∶4)。14例患者均为急性起病,临床表现包括头晕头痛(10例)、发热(8例)、大小便障碍(5例)、精神行为异常(4例)、肢体无力(4例)、肢体震颤(3例)等。所有患者均未合并肿瘤,但有8例合并低钠血症。12例患者对激素和(或)免疫抑制剂治疗反应良好;2例患者因个人原因拒绝激素或免疫抑制剂治疗,出院时病情无好转。结论:自身免疫性GFAP-A患者临床表现异质性较大,合并低钠血症比例较高,部分患者的临床表现类似神经系统感染性疾病,免疫治疗反应性较好。

Objective To analyze the clinical features of autoimmune glial fibrillary acidic protein astrocytopathy(GFAP-A).Methods A retrospective analysis was performed.Data of 14 patients with GFAP-A,admitted to Department of Neurology,Chenzhou First People's Hospital from December 1,2020 to May 1,2022,were collected.Results The mean age of these 14 patients was(46±31)years,and the male patients were more than female ones(10∶4).All patients had acute onset,and the main clinical symptoms included dizziness and headache(n=10),fever(n=8),urination and defecation function disturbance(n=5),mental and behavioral abnormality(n=4),limb weakness(n=4),and tremor(n=3).No tumors were found;8 patients were with hyponatremia.Twelve patients responded well to hormone and/or immunosuppressive therapy;2 patients refused corticosteroid and/or immunosuppressive therapy for personal reasons,and their condition did not improve at discharge.Conclusion In these GFAP-A patients,relatively heterogeneous clinical manifestations are noted;many are complicated with hyponatremia,and some have clinical manifestations similar to nervous system infectious diseases;the prognosis is good after immunotherapy.