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作 者:黄海霞[1] 符跃强[1] 白科[1] 周昉[1] 陈应富[1] 党红星[1] 李静[1] HUANG Haixia;FU Yueqiang;BAI Ke;ZHOU Fang;CHEN Yingfu;DANG Hongxing;LI Jing(Department of Critical Care Medicine,Children’s Hospital of Chongqing Medical University,Ministry of Education Key Laboratory of Child Development and Disorders,China International Science and Technology Cooperation Base of Child Development and Critical Disorders,Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China)
机构地区:[1]重庆医科大学附属儿童医院重症医学科,儿童发育疾病研究教育部重点实验室,国家儿童健康与疾病临床医学研究中心,儿童发育重大疾病国家国际科技合作基地,儿科学重庆市重点实验室,重庆400014
出 处:《临床儿科杂志》2021年第3期209-212,共4页Journal of Clinical Pediatrics
摘 要:目的分析儿童自身免疫性胶质纤维酸性蛋白胶质细胞病(GFAP-A)的诊断及治疗。方法回顾分析1例自身免疫性GFAP-A患儿的临床资料,并复习相关文献。结果女性患儿,10岁2个月。以发热起病,病程早期出现呼吸困难、尿储留,其后出现进行性意识障碍及惊厥。脑脊液及血液胶质纤维酸性蛋白抗体阳性。头颅磁共振成像(MRI)示双侧丘脑及基底节病变。脊髓平扫加增强示胸段部分脊膜及部分神经根线状强化。患儿最终确诊为GFAP-A,予以丙种球蛋白及激素治疗后病情好转。结论GFAP-A可以脑膜脑炎伴或不伴脊髓炎起病,MRI示脑膜脊膜强化、多发高信号病灶、血管样放射强化,抗体检测有助诊断。Objective To explore the diagnosis and treatment of autoimmune glial fibrillary acidic protein astrocytopathy(GFAP-A)in children.Methods The clinical data of an autoimmune GFAP-A in a child were retrospectively analyzed,and the related literature was reviewed.Results In a girl,aged 10 years and 2 months,it had an onset with fever.The child developed dyspnea and urine retention early in the course of the disease,followed by progressive disturbance of consciousness and convulsions.Her glial fibrillary acidic protein(GFAP)antibody in cerebrospinal fluid and serum were positive.Brain magnetic resonance imaging(MRI)showed bilateral thalamus and basal ganglia lesions.Plain and enhanced MRI scan of the spinal cord showed linear enhancement in the thoracic part of the spinal membrane and nerve roots.The child was finally diagnosed with GFAP-A,and his condition improved after treatment with gamma globulin and hormones.Conclusion GFAP-A begins with meningoencephalitis with or without myelitis.MRI showed meningeal enhancement,multiple hyperintensity lesions,and vascular-like radiologic enhancement.Antibody detection is helpful for diagnosis.
关 键 词:自身免疫性胶质纤维酸性蛋白星形细胞病 脑膜脑炎 儿童
分 类 号:R748[医药卫生—神经病学与精神病学]
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