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 Features of Multicenter Autoimmune Glial Fibrillary Acidic Protein Astrocytosis

作　　者：郑秀君 林麒[1] 舒崖清[2] 孙晓渤[2] 钟晓南[2] 李蕊[2] 常艳宇 邱伟[2] 王玉鸽[2] ZHENG Xiujun;LIN Qi;SHU Yaqing;SUN Xiaobo;ZHONG Xiaonan;LI Rui;CHANG Yanyu;QIU Wei;WANG Yuge(Department of Neurology,Shantou Central Hospital,Shangtou 515031,China;Department of Neurology,the Third Affiliated Hospital,SUN Yat-sen University,Guangzhou 510630,China)

机构地区：[1]汕头市中心医院神经内科,汕头515031 [2]中山大学附属第三医院神经内科,广州510630

出　　处：《罕见病研究》2022年第2期137-141,共5页Journal of Rare Diseases

摘　　要：目的探讨自身免疫性胶质纤维酸性蛋白(GFAP)星形胶质细胞病的临床特征。方法纳入多中心脑脊液检测GFAP-IgG证实为自身免疫性GFAP星型胶质细胞病患者15例,收集其临床及实验室检查资料,分析总结其临床特征和诊治。结果自身免疫性GFAP星形胶质细胞病首次发病年龄平均为39.73岁(4~65岁),性别无显著差异;临床表现中全脑症状如精神行为异常、意识障碍、痫性发作(50%以上),脑膜炎(66.7%),脊髓炎(53.3%),肢体震颤(53.3%),视力下降(33.3%),全身症状如发热(100.0%)和乏力(86.7%),其中46.7%的患者最初诊断考虑为结核性脑膜脑炎,予诊断性抗结核治疗。46.7%的患者MRI表现为典型的垂直于脑室的脑白质内血管周围线性放射性线样征。结论自身免疫性GFAP星形胶质细胞病常为急性或亚急性起病,临床表现多样,可表现为脑炎、脑膜炎、脊髓炎、视神经炎等,临床早期识别较困难,易误诊为结核性脑膜脑炎,诊断依赖于脑脊液抗GFAP抗体,且早期即可出现进展性意识障碍,危及生命。Objective To investigate the clinical features of autoimmune glial fibrillary acidic protein(GFAP)astrocytopathy.Methods We collected and analyzed the clinical and laboratory data and obtained the clinical characteristics of diagnosis and treatment from fifteen patients with positive GFAP antibody tested by cerebrospinal fluid and diagnosed autoimmune GFAP astrocytopathy by the multi-centers.Results The mean age of the first onset of autoimmune GFAP astrocytopathy was 39.73 years old(range 4-65 years),with no significant gender difference.In terms of clinical manifestations,we found the whole brain symptoms including abnormal mental behavior,disturbance of consciousness,epileptic attack accounting for more than 50,,meningitis accounting for 66.7%,myelitis(53.3%),limb tremor(53.3%),vision loss(33.3%);systemic symptoms including fever(100%)and fatigue(86.7%).46.7%of patients were initially diagnosed with tuberculous meningoencephalitis and were treated with diagnostic antituberculous therapy.The MRI showed 46.7%of patients showedbrain linear perivascular radial gadolinium enhancement in the white matter perpendicular to the ventricle.Conclusions Autoimmune GFAP astrocytopathy are acute or subacute dieases and the main clinical features include encephalitis,meningitis,myelitis and optic neuritis.They are likely to be misdiagnosed as tuberculous meningoencephalitis and can manifest progressive loss of consciousness in early phase,which is even life threatening.

关 键 词：自身免疫性胶质纤维酸性蛋白星形胶质细胞病 抗胶质纤维酸性蛋白抗体 进展性意识障碍 脑膜炎 

分 类 号：R73[医药卫生—肿瘤]