可逆性后部白质脑病综合征临床及影像学特点分析  

Analysis of clinical and imaging characteristics of rever-sible posterior leukoencephalopathy syndrome

作　　者：韩丽娟[1] 李政[2] 黄嵘[1] 徐运[1] 陈燕婷[1] HAN Lijuan;LI Zheng;HUANG Rong;XU Yun;CHEN Yanting(Department of Neurology,Nanjing Drum Tower Hospital,the Affiliated Hospital of Nanjing University Medical School,Jiangsu Province,Nanjing 210008,China;Department of Rehabilitation Medicine,Nanjing Drum Tower Hospital,the Affiliated Hospital of Nanjing University Medical School,Jiangsu Province,Nanjing 210008,China)

机构地区：[1]南京大学医学院附属鼓楼医院神经内科,江苏南京210008 [2]南京大学医学院附属鼓楼医院康复医学科,江苏南京210008

出　　处：《中国医药导报》2022年第2期117-120,125,共5页China Medical Herald

基　　金：国家自然科学基金青年科学基金项目(81601016、81400971)。

摘　　要：目的分析可逆性后部白质脑病综合征临床及影像学特点。方法回顾性分析南京大学医学院附属鼓楼医院2017年5月至2020年6月收治的17例可逆性后部白质脑病综合征患者的临床特征、影像学特点、治疗及预后情况。结果17例可逆性后部白质脑病综合征患者均为女性,平均年龄(31.06±12.84)岁,最常见的临床表现包括癫痫、意识障碍、头痛及视物模糊,癫痫持续状态少见。绝大多数患者发病时血压增高。子痫/子痫前期、产褥期、酪氨酸激酶抑制剂使用、系统性红斑狼疮、输血反应和肾病综合征是可逆性后部白质脑病综合征常见发病诱因。白质病变以顶叶、枕叶为主,其次为额叶及颞叶,小部分病变累及基底节、小脑及脑干。病灶信号提示血管源性水肿,强化少见。患者总体预后良好,在1周内大部分患者的症状有明显改善,大部分病灶可明显吸收。共随访14例患者,最长随访时间为发病后39个月,无再发可逆性后部白质脑病综合征,无癫痫发作。结论多种疾病可合并可逆性后部白质脑病综合征,其临床表现多样,有影像学特征。及时治疗可获得较好预后,可逆性后部白质脑病综合征患者长期癫痫概率小。Objective To analyze the clinical and imaging characteristics of rever-sible posterior leukoencephalopathy syndrome.Methods The clinical features,imaging features,treatment and prognosis of 17 patients with rever-sible posterior leukoencephalopathy syndrome admitted in Nanjing Drum Tower Hospital,the Affiliated Hospital of Nanjing University Medical School from May 2017 to June 2020 were retrospectively analyzed.Results All the 17 rever-sible posterior leukoencephalopathy syndrome patients were female with a mean age of(31.06±12.84)years.The most common clinical manifestations included epilepsy,disturbance of consciousness,headache and blurred vision,and epileptic persistence was rare.The vast majority of patients have elevated blood pressure at onset.Eclampsia/preeclampsia,puerperium,tyrosine kinase inhibitor use,systemic lupus erythematosus,blood transfusion reactions,and nephrotic syndrome were common causes of rever-sible posterior leukoencephalopathy syndrome.The white matter lesions were mainly parietal and occipital lobes,followed by frontal and temporal lobes,and a small number of lesions involved basal ganglia,cerebellum and brainstem.The signal of the lesion suggested vasogenic edema with rare enhancement.The overall prognosis of the patients was good.Within one week,the symptoms of most patients improved significantly,and most lesions were clearly absorbed.A total of 14 patients were followed up,and the longest follow-up time was 39 months after onset.There was no recurrence of rever-sible posterior leukoencephalopathy syndrome and no seizures.Conclusion Rever-sible posterior leukoencephalopathy syndrome can be associated with many diseases,and its clinical manifestations are diverse with imaging features.Timely treatment can obtain a better prognosis,and patients with rever-sible posterior leukoencephalopathy syndrome have a low probability of long-term epilepsy.

关 键 词：可逆性后部白质脑病综合征 高血压 子痫/子痫前期 输血 癫痫发作 

分 类 号：R742[医药卫生—神经病学与精神病学]