成人自身免疫性胶质纤维酸性蛋白星形细胞病12例临床特征分析  

Analysis of clinical characteristicssof 12 cases of adult autoimmune glial fibrillary acidic protein astrocytopathy

作　　者：张颖[1] 卢宏[1] ZHANG Ying;LU Hong(Department of Neurology,the First Affliated Hospital of Zhengzhou University,Zhengzhou 450000,China)

机构地区：[1]郑州大学第一附属医院神经内一科,450000

出　　处：《临床神经病学杂志》2023年第3期180-184,共5页Journal of Clinical Neurology

摘　　要：目的 分析成人自身免疫性胶质纤维酸性蛋白(GFAP)星形细胞病的临床特点、辅助检查、治疗及预后,以期提高对该疾病的认识。方法 回顾性分析郑州大学第一附属医院2019年9月至2022年5月确诊的12例成人自身免疫性GFAP星形细胞病患者的临床资料。结果 12例患者中,男性9例,女性3例,主要表现为发热、头痛、肢体无力、肢体麻木等。头颅MRI提示多发点状或斑片状T2WI、Flair高信号,可累及侧脑室旁、额叶、基底节区等。部分患者脊髓受累,脊髓MRI显示条片状T2WI高信号。CSF GFAP抗体均为阳性,CSF白细胞、淋巴细胞、蛋白含量、白蛋白商值及24 h IgG鞘内合成率多升高,部分患者寡克隆区带阳性。5例患者合并其他自身抗体,6例合并低钠血症,8例血尿酸下降,4例血清IL-6下降,3例EMG存在异常。给予经验性免疫治疗,大多患者预后良好,部分留有功能障碍。结论 成人自身免疫性GFAP星形细胞病临床表现具有异质性,重症患者进展较快。头颅MRI和(或)脊髓MRI及CSF检查可辅助诊断,免疫治疗效果好。Objective To analyze the clinical characteristics,ancillary examinations,treatment and prognosis of autoimmune glial fibrillary acidic protein(GFAP)astrocytopathy in adults,in order to improve the understanding of the disease.Methods The clinical data of 12 adult patients with autoimmune GFAP astrocytopathy diagnosed in the First Affiliated Hospital of Zhengzhou University from September 2019 to May 2022 were retrospectively analyzed.Results There were 9 males and 3 females in 12 patients,mainly presenting with fever,headache,limb weakness,limb numbness,etc.The cranial MRI suggested multiple dots or patches of T,WI and Flair hyperintensities,which involved the lateral ventricle,frontal lobe and basal ganglia,etc.The spinal cord was involved in some patients and the spinal MRI showed strips of T,WI hyperintensities.GFAP antibody was positive in CSF,with leukocytes,lymphocytes,protein,albumin quotient and 24 h intrathecal IgG synthesis rate elevated in CSF,and oligoclonal bands were positive in some patients.Five patients were combined with other autoantibodies,6 patients with hyponatremia,8 patients with decreased blood uric acid,4 patients with decreased serum IL-6 and three had abnormalities in the EMG.Empirical immunotherapy was given and most patients had good prognosis,while some stayed with functional impairment.Conclusions The clinical manifestations of adult autoimmune GFAP astrocytopathy are heterogeneous,with rapid progression in severe patients.The cranial MRI and/or spinal MRI and CSF examination can assist diagnosis,and immunotherapy has good efficacy.

关 键 词：胶质纤维酸性蛋白 自身免疫性星形细胞病 临床特征 

分 类 号：R744.5[医药卫生—神经病学与精神病学]