11例自身免疫性GFAP星形胶质细胞病患儿的临床特征分析  

Clinical characteristics of 11 children with autoimmune glial fibrillary acidic protein astrocytopathy

作　　者：王越[1] 罗强 禚志红[1] 王怀立[1] Wang Yue;Luo Qiang;Zhuo Zhihong;Wang Huaili(Department of Pediatrics,First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院儿科,郑州450052

出　　处：《中华神经医学杂志》2022年第5期478-485,共8页Chinese Journal of Neuromedicine

摘　　要：目的探讨儿童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。方法收集自2020年1月至2022年2月在郑州大学第一附属医院儿科住院治疗的11例血清和(或)脑脊液中GFAP抗体阳性的自身免疫性GFAP星形胶质细胞病患儿的临床资料进行回顾性分析。结果11例患儿中,男性6例,女性5例;发病年龄3岁10个月~12岁;主要临床表现为发热(8例)、头痛(5例)、呕吐(6例)、共济失调(2例)、肢体无力(4例)、颅神经受累(6例)、意识障碍(4例)、精神行为异常(3例)、抽搐发作(1例)、自主神经功能紊乱(3例);临床类型为脑膜脑脊髓炎1例、脑膜脑炎1例、脑脊髓炎7例、脑炎2例。11例患儿MRI均提示颅内受累,脊髓长节段受累8例,视神经受累1例,4例行增强扫描患儿中3例显示脑叶、脑膜、脑沟、视神经、脊髓不同部位强化。4例患儿脑脊液和血清GFAP抗体阳性,3例脑脊液GFAP抗体阳性,4例血清GFAP抗体阳性;4例合并多重抗体,主要为髓鞘少突胶质细胞糖蛋白抗体;肿瘤筛查均阴性。11例患儿均对免疫治疗有反应,但有2例复发,1例遗留视力、运动功能损伤。结论儿童自身免疫性GFAP星形胶质细胞病的临床表现多样、无特异性,病变主要累及脑膜、脑、脊髓和视神经。大多数患儿对糖皮质激素治疗反应良好,预后也良好,但仍有一定的复发率,部分患儿可能遗留神经系统损伤。Objective To explore the clinical characteristics of autoimmune glial fibrillary acidic protein(GFAP)astrocytopathy in children.Methods Eleven children with autoimmune GFAP astrocytopathy with positive GFAP antibody in serum and/or cerebrospinal fluid were collected in our hospital from January 2020 to February 2022.The clinical data of these children were analyzed retrospectively.Results Among the 11 children,there were 6 males and 5 females,and the age of onset ranged from 3 years old and 10 months to 12 years old.The main clinical manifestations included fever(n=8),headache(n=5),vomiting(n=6),ataxia(n=2),limb weakness(n=4),cranial nerve involvement(n=6),disturbance of consciousness(n=4),abnormal mental behavior(n=3),seizures(n=1),and autonomic nervous dysfunction(n=3).Meningoencephalomyelitis was noted in one child,meningoencephalitis in one,encephalomyelitis in 7,and encephalitis was noted in two children.MRI showed brain involvement in all children,spinal cord involvement in 8 children,and optic nerve involvement in one child.Abnormal enhancement in different parts of cerebral lobe,meninges,sulcus,optic nerve and spinal cord were found in 3 children.Four children were positive for GFAP antibody in cerebrospinal fluid and serum,3 patients were positive for GFAP antibody in cerebrospinal fluid,and 4 children were positive for GFAP antibody in serum.Four children were complicated with multiple antibodies,mainly myelin oligodendrocyte glycoprotein antibody.Tumor screening was all negative.All of the 11 children responded to immunotherapy,but two of them relapsed;one left visual and motor function impairment.Conclusions The clinical manifestations of autoimmune GFAP astrocytopathy in children are diverse and non-specific,and the lesions mainly involve meninx,brain,spinal cord and optic nerve.Most children respond well to glucocorticoid treatment and have a good prognosis;but there is still a certain recurrence rate,and some children may leave neurological damage.

关 键 词：自身免疫性胶质纤维酸性蛋白星形胶质细胞病 儿童 脑膜脑脊髓炎 

分 类 号：R725.9[医药卫生—儿科]