摘要:
Objective To summarize the neurological manifestations in patients with primary Sj?gren′s syndrome (pSS). Methods A total of 68 patients were diagnosed as pSS in neurology department of Peking Union Medical College Hospital from March 2014 to February 2018, among whom sixteen cases were excluded due to modified final diagnoses of primary neurological diseases. Therefore 52 pSS patients with neurological involvement were enrolled and retrospectively analyzed. They were divided into two groups as extensive group in which both central and peripheral nervous system were involved, non?extensive group in which either central or peripheral nervous system was involved. Results Neurological manifestations were presented as primary symptoms in 98.1%(51/52) patients, while 35 had neurological involvement as their only extraglandular manifestations. Thirteen cases were in extensive group. The other 39 in non?extensive group including 22 cases with only peripheral nervous system involved and 17 cases with only single central nervous system involved. Compared to non?extensive group, the proportion of woman patients [13/13 vs.71.8% (28/39),P=0.047], serum IgG level [17.73(11.11,22.41)g/L vs. 11.49(9.58,13.40)g/L, P=0.017] and positive rates of oligoclonal band (OB) in cerebral spinal fluid (CSF) [7/13 vs. 22.6%(7/31), P=0.042)] were significantly higher in extensive involvement group. Conclusions Neurological manifestations in pSS patients could be extensive, both central and peripheral nervous system might be associated. Female patients, high serum IgG level and positive OB in CSF are risk factors of extensive neurological involvement, suggesting that the immune system may be generally over?stimulated.%目的 通过分析原发性干燥综合征(pSS)相关神经系统损害的临床特征,提高医生对其的认识水平,避免漏诊和误诊.方法 回顾性分析2014年3月至2018年2月北京协和医院神经内科收治的诊断为pSS相关神经系统损害的52例患者的临床资料,并进一步分析神经系统广泛受累(同时累及中枢神经系统与周围神经系统)和非广泛受累(单纯周围神经系统受累或中枢神经系统受累)的临床特征.结果 51例患者以神经系统症状为首发临床表现,35例患者以神经系统受累为唯一腺体外临床表现.神经系统广泛受累者13例,非广泛受累者39例.非广泛受累者中,22例单纯周围神经系统损害,17例单纯中枢神经系统损害.广泛受累者女性患者比例[13/13比71.8%(28/39), P=0.047]、血IgG水平[17.73(11.11,22.41)g/L比11.49(9.58,13.40)g/L,P=0.017]、脑脊液特异性寡克隆区带阳性患者比例[7/13比22.6%(7/31),P=0.042]高于非广泛受累者.结论 神经系统受累可以是pSS首发症状,甚至是唯一的临床表现.对神经系统受累部位广泛,同时累及中枢神经系统与周围神经系统者需警惕pSS.神经系统广泛受累者中女性多见,且外周血IgG水平、脑脊液寡克隆区带阳性率高于非广泛受累者,提示免疫系统的激活与泛化可能在pSS神经系统受累中发挥重要作用.