摘要
目的 分析国人自身免疫性胶质纤维酸性蛋白(GFAP)星形细胞病患者的脊髓病变的磁共振成像(MRI)特征及其相关临床特点。 方法 使用基于组织法及细胞法检测2013年3月至2018年6月在广州医科大学附属第二医院神经内科收集的1 040份脑脊液和血清标本,发现42例GFAP抗体阳性患者,回顾性分析脑脊液GFAP抗体阳性同时存在脊髓病灶的19例自身免疫性GFAP星形细胞病患者的脊髓MRI资料及其临床资料。 结果 19例患者中,女性12例,男性7例;发病年龄(44±17)岁。患者的主要临床表现为肢体麻木无力(14/19)、视力障碍(5/19)、头痛(4/19)、癫痫发作(4/19)、认知障碍(3/19)等。脊髓MRI提示,脊髓病灶仅累及颈段脊髓者5例(5/19),仅累及胸段脊髓者8例(8/19),同时累及颈胸段脊髓者6例(6/19);脊髓受累长度超过3个脊髓节段者15例(15/19);脊髓病灶有强化者7例(7/19);脊髓病灶合并有颅内病灶者8例(8/19);病灶累及脊髓灰质19例(19/19)。 结论 自身免疫性GFAP星形细胞病患者女性多于男性,患者的脑部和脊髓可以同时受累,患者的脊髓MRI表现形式复杂,脊髓受累节段一般较长,并且以累及脊髓灰质为主,脊髓病灶多累及胸段脊髓,仅累及颈段脊髓较少,腰段脊髓基本不受累。
Objective To analyze the magnetic resonance imaging (MRI) of the spinal cord and clinical characteristics in patients with autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.Methods A total of 1 040 samples of cerebrospinal fluid (CSF) and sera collected in the Second Affiliated Hospital of Guangzhou Medical University from March 2013 to June 2018 were tested with tissue- and cell-based assays, and 42 patients were found positive for GFAP-IgG. The clinical data and MRI characteristics of the spinal cord of 19 patients who were positive for GFAP-IgG in CSF with autoimmune GFAP astrocytopathy and lesions in the spinal cord were retrospectively reviewed. Results There were 12 females and seven males among the 19 patients, with onset age of (44±17) years. The main manifestations of these patients included limb weakness (14/19), abnormal vision (5/19), headache (4/19), seizure (4/19), dementia (3/19), etc. On MRI of the spinal cord, five patients showed involvement in the cervical cord alone, eight showed involvement in the thoracic cord alone and six had both cervical and thoracic segment involvement. Fifteen patients had longitudinally extensive myelitic abnormalities (≥3 vertebral segments long). Seven enhancement patterns were encountered. Lesions were displayed in the spinal cord and brain in eight patients. Central gray matter involvement in the spinal cord was found in all the 19 patients. Conclusions Autoimmune GFAP astrocytopathy more frequently presents in females than in males. MRI of the spinal cord has complex presentations and longitudinally extensive myelitic abnormalities usually. Patients often show central gray matter involvement in the spinal cord. Myelitic abnormalities present more often in thoracic segment than in cervical segment. Abnormalities in lumbar segment are less encountered.
作者
徐辉明
黄清梅
肖小玉
刘天妮
陈百铿
杨华才
刘思
杨洁
黄莉
龙友明
高聪
Xu Huiming;Huang Qingmei;Xiao Xiaoyu;Liu Tianni;Chen Baikeng;Yang Huacai;Liu Si;Yang Jie;Huang Li;Long Youming;Gao Cong(Department of Neurology,Institute of Neuroscience,the Second Affiliated Hospital of Guangzhou Medical University,Guangzhou 510260,China;Department of Nephrology,the First Affiliated Hospital,Guangdong Pharmaceutical University,Guangzhou 510080,China)
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2019年第2期92-97,共6页
Chinese Journal of Neurology
基金
国家自然科学基金面上项目(81771302)
广东省科技计划项目(2015A030302093)
广州市科技计划项目(201803010123).
关键词
自身免疫疾病
胶质纤维酸性蛋白
星形细胞
脊髓
磁共振成像
Autoimmune diseases
Glial fibrillary acidic protein
Astrocytes
Spinal cord
Magnetic resonance
