自身免疫性GFAP星形细胞病儿童患者的临床特征和短期预后研究  

Clinical Characteristics and Short-Term Prognosis of Pediatric Patients with Autoimmune GFAP Astrocytopathy

作　　者：王亚丽[1] 张婉婉 张璐瑶[3] 徐凯丽 宋丽芳 乔平云 王莉[1] WANG Yali;ZHANG Wanwan;ZHANG Luyao(Department of Neurology,Zhengzhou University Children's Hospital,Zhengzhou Henan 450000)

机构地区：[1]郑州大学附属儿童医院神经内科,河南郑州450000 [2]郑州大学第一附属医院神经内科五病区,河南郑州450000 [3]郑州大学附属儿童医院儿科研究所,河南郑州450000

出　　处：《医学临床研究》2025年第4期578-582,共5页Journal of Clinical Research

基　　金：河南省科技攻关联合共建项目(编号:LHGJ20220730)。

摘　　要：【目的】探讨儿童自身免疫性胶质纤维酸性蛋白星形细胞病(GFAP-A)患儿的临床特征和短期预后。【方法】回顾性分析15例脑脊液胶质纤维酸性蛋白(GFAP)阳性的GFAP-A患儿的临床资料,分析患儿的临床特征和预后情况。【结果】患儿发病时的中位年龄为8岁,主要临床症状为发热、四肢无力、头痛、食欲不振等;主要临床表型为脑炎、脑膜炎、脊髓炎等。磁共振检查显示,病变累及脑实质、脑膜、视神经和脊髓,表现为斑片状、线状、点状和条片状T 2高信号。13例患儿脑脊液白细胞升高,以淋巴细胞为主,8例伴有蛋白升高,4例伴有低血糖。14例对包括皮质类固醇和免疫球蛋白在内的一线免疫疗法有效,1例水通道蛋白-4(AQP-4)抗体重叠的患者复发,且对一线免疫疗法反应不佳。【结论】儿童GFAP-A的临床症状及磁共振影像多样,无明显特异性,一线免疫治疗效果良好,AQP-4抗体重叠的患者预后不良。【Objective】To investigate the clinical characteristics and short-term prognosis of pediatric patients with autoimmune glial fibrillary acidic protein astrocytopathy(GFAP-A).【Methods】A retrospective analysis was conducted on the clinical data of 15 pediatric patients with cerebrospinal fluid positivity for glial fibrillary acidic protein(GFAP).【Results】The median age of onset was 8 years.The main clinical symptoms included fever,limb weakness,headache,and loss of appetite.The predominant clinical phenotypes were encephalitis,meningitis,and myelitis.Magnetic resonance imaging(MRI)revealed lesions involving brain parenchyma,meninges,optic nerves,and spinal cord,presenting as patchy,linear,punctate,and strip-like T 2 hyperintensities.Among 15 patients,13 cases had elevated cerebrospinal fluid white blood cells,mainly lymphocytes,8 with elevated protein,and 4 with decreased sugar.Fourteen patients responded well to first-line immunotherapy,including corticosteroids and immunoglobulins,while one patient with overlapping aquaporin(AQP-4)antibody experienced relapse and had a poor response to first-line therapy.【Conclusion】Pediatric GFAP-A patients have diverse clinical symptoms and MRI findings,lacking distinct specificity.First-line immunotherapy is generally effective in treatment,although patients with overlapping AQP-4 antibody tend to have a poor prognosis.

关 键 词：星形细胞瘤 神经胶质原纤维酸性蛋白质 自身免疫疾病 儿童 预后 

分 类 号：R730.264.1[医药卫生—肿瘤]