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.