胶质纤维酸性蛋白星型细胞病的病例特点  

Clinical features of patients with autoimmune glial fibrillary acidic protein astrocytopathy

作　　者：袁泉[1] 谭淑慧 YUAN Quan;TAN Shuhui(Innovation Center for Neurological Disorders,Department of Neurology,Xuanwu Hospital,Capital Medical University,Beijing 100053,China)

机构地区：[1]首都医科大学宣武医院神经疾病高创中心,北京100053 [2]聊城市人民医院神经内科,山东聊城252000

出　　处：《中风与神经疾病杂志》2021年第6期501-504,共4页Journal of Apoplexy and Nervous Diseases

摘　　要：目的通过分析5例GFAP星型胶质细胞病患者的临床资料,参考国内外文献报道,探讨GFAP星型胶质细胞病患者的临床特点。方法通过对宣武医院2018年-2020年收治的5例GFAP星型胶质细胞病患者一般临床信息、临床症状、脑脊液结果、脑电图结果、影像学特征及治疗结果进行回顾性分析及相关文献复习。结果5例患者平均发病年龄为57岁,临床症状主要表现为肢体乏力、记忆力下降、睡眠增多、尿便障碍、发热等,有1例患者除上述表现外还伴有癫痫发作;脑脊液检查均显示蛋白及细胞数不同程度的增高,4例患者出现脑脊液寡克隆区带及脑脊液特异性寡克隆区带阳性、24 h鞘内合成率不同程度的增高。脑电图显示不同程度的慢波;头部核磁可见颞叶、侧脑室旁、基底节及胼胝体异常信号,有3例患者延髓、颈髓及胸髓可见异常信号。5例患者均给予激素治疗,且症状均不同程度改善。结论GFAP星型细胞病患者临床症状主要为脑病、脑膜炎及脑脊髓炎等表现,且脑脊液均有不同程度的免疫介导的炎症反应表现,影像学检查可见脑、脑膜、脊髓和视神经受累,大部分对激素反应良好。Objective To explore the clinical features through analyzing the clinical data of 5 patients with autoimmune glial fibrillary acidic protein(GFAP)astrocytopathy and reviewing the literatures.Methods We retrospectively analyzed the general data,clinical symptoms,cerebrospinal fluid results,electroencephalogram results,imaging data and treatment results of 5 patients with GFAP astrocytopathy at our hospital and reviewed the literatures.Result The average age of onset was 57 in five patients.The main clinical symptoms were limb fatigue,memory loss,increased sleep,urine and stool disorders,fever and so on,and 1 patient was accompanied by epileptic seizure in addition to the above manifestations.Cerebrospinal fluid examination showed that protein and cell number were increased in different degrees,4 patients showed positive oligoclonal bands and specific oligoclonal bands in cerebrospinal fluid,and 24 hours intrathecal synthesis rate increased in different degrees.The electroencephalogram showed varying degrees of slow waves.Abnormal signals in the temporal lobe,lateral ventricle,basal ganglia and corpus callosum were observed by craniocerebral MRI.Abnormal signals in the medulla oblongata,cervical medulla and thoracic medulla were observed in 3 patients.All the 5 patients received hormone therapy,and their symptoms were improved to varying degrees.Conclusions The clinical symptoms of patients with GFAP astrocytosis were mainly encephalopathy,meningitis and encephalomyelitis,and the cerebrospinal fluid had different degrees of immune-mediated inflammatory reaction.Imaging examination showed that the brain,meninges,spinal cord and optic nerve were involved,and most of patients respond well to hormones.

关 键 词：GFAP星型细胞病 脑脊液 脑电图 治疗 

分 类 号：R741[医药卫生—神经病学与精神病学]