脊髓受累的可逆性后部脑病综合征的临床特征及预后  

Clinical characteristics and prognosis of posterior reversible encephalopathy syndrome with spinal cord involvement

作　　者：赵璐[1] 宋波[1] 曹爽 朱永梅 许予明[1] Zhao Lu;Song Bo;Cao Shuang;Zhu Yongmei;Xu Yuming(Department of Neurology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China;Department of Neurology,the First People′s Hospital of Lingbao City,Sanmenxia 472599,China)

机构地区：[1]郑州大学第一附属医院神经内科,郑州450052 [2]灵宝市第一人民医院神经内科,三门峡472599

出　　处：《中华神经科杂志》2023年第4期427-433,共7页Chinese Journal of Neurology

摘　　要：目的探讨脊髓受累的可逆性后部脑病综合征(PRES-SCI)的临床表现、影像特征及预后。方法分析郑州大学第一附属医院神经内科2021年11月收治的1例PRES-SCI患者的临床资料,并检索国内外数据库,对已报道的38例PRES-SCI患者及本例患者进行汇总分析。结果39例PRES-SCI患者(包括本例)主要临床表现为头痛(79.5%,31/39)、视力障碍(79.5%,31/39)、呕吐(46.2%,18/39)、意识障碍(38.5%,15/39)、肢体无力(28.2%,11/39)、癫痫发作(23.1%,9/39)。97.4%(38/39)的患者有血压明显升高。影像特征性表现为以中央灰质受累为主的长节段脊髓病变,89.7%(35/39)的脊髓病灶起源于延髓颈髓交界处,病变累及椎体所对应的脊髓节段数中位数为9(最短为4,最长为全脊髓)。38例合并颅脑病变,最常累及的部位为延髓(82.1%,32/39)、枕叶(46.2%,18/39)、脑桥(43.6%,17/39)、顶叶(41.0%,16/39)、小脑(38.5%,15/39)。38例完成随访的患者中,31例(81.6%)临床痊愈。结论可逆性后部脑病综合征可累及脊髓,出现长节段脊髓病变、合并血压明显增高或其他危险因素,应考虑到PRES-SCI。及时识别和治疗,大部分患者预后良好。Objective To investigate the clinical manifestations,imaging features and prognosis of posterior reversible encephalopathy syndrome with spinal cord involvement(PRES-SCI).Methods The clinical data of 1 patient with PRES-SCI admitted to the Department of Neurology of the First Affiliated Hospital of Zhengzhou University in November 2021 were analyzed,and the data of 38 patients with PRES-SCI reported in domestic and foreign databases and this patient were collected for pooled analysis.Results The main clinical manifestations of 39 PRES-SCI patients(including this patient)included headache(79.5%,31/39),visual disturbance(79.5%,31/39),vomiting(46.2%,18/39),disturbance of consciousness(38.5%,15/39),limb weakness(28.2%,11/39)and seizure(23.1%,9/39).There were up to 97.4%(38/39)of patients who had significantly elevated blood pressure.The imaging feature was long-segment spinal cord lesion involving central gray matter.Approximately 89.7%(35/39)of the spinal cord lesions originated from the junction of the medulla oblongata and the cervical spinal cord.The median number of abnormal spinal cord segments corresponding to the vertebral body was 9(the shortest was 4 and the longest was the entire spinal cord).Thirty-eight patients had brain lesions,and the most frequently involved sites were medulla oblongata(82.1%,32/39),occipital lobe(46.2%,18/39),pons(43.6%,17/39),parietal lobe(41.0%,16/39),and cerebellum(38.5%,15/39).Among 38 patients completing the follow-up,31 patients(81.6%)were clinically recovered.Conclusions Posterior reversible encephalopathy syndrome could involve the spinal cord.For patients with long-segment spinal cord lesions and significantly increased blood pressure or other risk factors,PRES-SCI should be considered.Timely identification and treatment could make most patients recovery.

关 键 词：后部白质脑病综合征 脊髓 预后 临床表现 影像特征 

分 类 号：R741[医药卫生—神经病学与精神病学]