原发性干燥综合征神经系统损害.docVIP

原发性干燥综合征神经系统损害 原发性干燥综合征的神经系统损害 2010-03-24 刘布骏　张杏书 　　【摘　要】　目的　观察原发性干燥综合征(SS)的神经系统病变及临床特征。方法　选择了18例在我院就诊的SS患者，合并神经系统损害不能以其他疾病解释。观察期间给予神经系统、病理及免疫学各项检查。结果　显示了神经及精神病的多种多样临床表现。血清学检查免疫球蛋白升高，自身抗体阳性，病理检查脑血管炎表现。结论　SS因累及的神经系统部位不同，临床表现多种多样。中枢神经系统病变临床过程大多较轻且迁延，脑血管炎为主要致病因素，并可形成脑软化灶及脑梗死。抗SS-A/SS-B抗体的出现可能更多的伴有内脏损害。出现中枢神经系统损害应立即给予糖皮质激素及免疫抑制剂治疗。 　　【关键词】　干燥综合征　　神经系统　　损害 Nervous system lesion of Sjgren syndrome 　　LIU Bujun,ZHANG Xingshu.Nanjing Gulou Hospital,Nanjing 210008 　　【Abstract】　Objective　To investigate the lesion of nervous system and its clinical features of Sjgren syndrome (SS).Methods　Eighteen SS patients with nervous system injuries which could not be explained by other diseases have been selected.Tests of nervous system,pathology and immunology were carried out during the observational period.Results　There were a variety of neuropsychiatric presentations.Serological examination showed that the immunoglobulin was elevated and autoantibodies were positive and pathological examination showed cerebral vasculitis.Conclusion　SS shows a variety of clinical manifestations because of the different positions of nervous system involvement.The clinical process of central nervous system pathological changes is usually protractive and not serious.Cerebral vasculitis is the main pathogenesis,which maybe forms encephalomalacia and cerebral infarction.The presentation of anti-SSA and anti-SSB may be companied with more lesions of organs.Corticosteroid and immunosuppressive agents must be given immediately when the lesions of the central nervous system are present. 　　【Key words】　Sjgren′s syndrome　　Nervous system　　Lesion 　　原发性干燥综合征(Sjgrens syndrome，SS)是一种主要侵犯外分泌腺的慢性自身免疫性疾病，也可累及多系统和器官，造成多种多样的临床表现。其神经系统病变早在40年代已有报道，甚至以神经系统病变为主要表现，而口、眼干燥症状仅在追问病史中发现。本文就我科收集的18例SS合并神经系统损害病例作一分析。 1　资料和方法 1.1　对象 　　本组18例患者于1988年—1997年就诊于我院免疫科及神经科。符合Fox的SS诊断标准三条以上，而不伴有另一种肯定的结缔组织病。其中男性2例，女性16例，年龄21～60岁，平均40.5岁。平均病程12.5年。神经系统损害都不能以其他疾病来解释。 1.2　临床表现 1.2.1　头痛4例，表现为反复发作性头痛。发作时伴有恶心及耳鸣，发作时间及间隙不定，常因情绪激动、劳累及受