干燥综合征合并中枢神经系统脱髓鞘疾病的临床特点分析  

Clinical analysis of Sjgren′ s syndrome complicated with central nervous system demyelinating disease

作　　者：顾寒英[1,2] 施若非[3] 陈迎春[2] 王刚[2] 

机构地区：[1]上海交通大学医学院附属瑞金医院卢湾分院急诊科,上海200025 [2]上海交通大学医学院附属瑞金医院神经内科,上海200025 [3]上海交通大学医学院附属瑞金医院皮肤科,上海200025

出　　处：《诊断学理论与实践》2014年第1期86-89,共4页Journal of Diagnostics Concepts & Practice

摘　　要：目的:探讨干燥综合征合并中枢神经系统脱髓鞘疾病的临床特点。方法:回顾分析2007年至2011年收治的原发性干燥综合征(primary Sj觟gren′s syndrome,pSS)合并中枢神经系统脱髓鞘疾病患者的相关资料。结果:297例pSS患者中,有4例(1.35%)合并中枢神经系统脱髓鞘疾病。4例患者中,脱髓鞘病变在脑部者2例,在脊髓者2例。脱髓鞘病变位于脑部的患者主要表现为头晕、肢体乏力,而病变位于脊髓的主要表现为肢体乏力、损伤平面以下感觉减退、括约肌功能障碍。病变发生在脊髓的2例患者全部累及颈胸髓,1例患者纵向脊髓节段累及超过4个,为国内外较罕见。经激素及环磷酰胺治疗后,4例患者均好转出院。结论:pSS合并脱髓鞘疾病具有异质性特点,应结合临床特点及体液神经免疫学指标、神经影像具体分析诊断。Objective： To investigate the clinical features and incidence of Sjoegren＇s syndrome complicated with central nervous system demyelinating disease. Methods： Two hundred and ninety-seven patients with Sjoegren＇s syndrome were enrolled, of them 4 cases were diagnosed as having central nervous system demyelinating disease. Results： Clinical data from these ,4 patients （one male and 3 females） were analyzed. Demyelinating lesions were found to be located at the brain in 2 cases and at spinal cord in the other 2 cases. Patients with brain lesions were manifested as dizziness, limb weakness, and patients with spinal cord lesions mainly as limb weakness, hypoesthesia below the level of spinal cord injury and sphincter dysfunction. Two cases with spinal cord lesions had cervical and thoracic spinal cord involvement. One of these 2 patients had involvement of more than 4 spinal segments, this was rarely seen both in home and abroad. After corticosteroids and cyclophosphamide treatment, all 4 patients had their disease improved and discharged from hospital. Conclusions： Sjoegren＇ s syndrome complicated with central nervous system demyelinating diseases has heterogeneous characteristics, and combination of clinical features, neuro-humoral immunological parameters and neuro- imaging should be used for its diagnosis.

关 键 词：干燥综合征 脱髓鞘疾病 诊断 

分 类 号：R442.8[医药卫生—诊断学]