原发性干燥综合征中枢神经系统损害八例临床分析  

Primary Sjogren syndrome combined with central nervous system damage： a clinical analysis of 8 cases

作　　者：易婷玉[1] 吴宗忠[1] 吴燕敏[1] 詹阿来[1] 陈跃鸿[1] 张梅芳[1] 陈文伙[1] 

机构地区：[1]福建医科大学附属漳州市医院神经内科,漳州363000

出　　处：《中华神经医学杂志》2011年第9期938-941,共4页Chinese Journal of Neuromedicine

摘　　要：目的探讨原发性干燥综合征的中枢神经系统损害的临床表现、MRI特点、治疗方法。方法回顾性分析漳州市福建医科大学附属漳州市医院确诊的8例原发性干燥综合征合并中枢神经系统损害的临床表现、MRI特点。结果临床可表现为延髓背外侧综合征、肢体无力、智能改变，可类似多发性硬化、视神经脊髓炎，视神经较经常累及；MRI检查示5例出现脊髓损害：病变部位以颈胸髓为主，颈髓2例，胸髓2例，1例同时累及颈髓和胸髓，病灶可大片状、散在斑片状，可轻度、明显强化或不强化，5例出现颅内损害：病变部位可见于延髓背外侧、丘脑、海马、中脑导水管、第四脑室旁、侧脑室旁，可表现为皮质下及脑室旁白质多发小缺血灶，可类似韦尼克脑病，1例出现颈动脉夹层，7例使用了激素后病情有一定改善。结论原发性干燥综合征中枢神经系统损害的症状、体征及影像学表现多种多样。Objective To investigate the clinical manifestations, MRI features and therapeutic methods of patients with central nervous system damage secondary to primary Sjogren syndrome. Methods The clinical features, MRI manifestations and therapeutic method of 8 patients with central nervous system damage secondary to primary Sjogren syndrome, confirmed in our hospital, were retrospectively reviewed and analyzed. Results Wallenberg syndrome, limb weakness and mentality alteration appeared in these patients, some manifested as having multiple sclerosis and neuromyelitis optica, and optic nerve was frequently involved. MRI results indicated that 5 patients had lesion in the spinal cord, mainly located in the cervical and thoracic cords： 2 in the cervical cord, 2 in the thoracic cord, and 1 both in the cervical and thoracic cords; the lesions manifested as sheet lamellar, scattered plaques with mild/obvious enhancement or without enhancement; 5 patients had brain damage： lesions could be detected in the dorsolateral medulla oblongata, thalamus, hippocampus, aqueduct of midbrain, and sides of fourth ventricle and lateral ventricles, and these lesions could manifest as multiple minor ischemic focus white matter in the subcortex and cerebral ventricles, which might be similar to Wemicke eneephalopathy; 1 patient appeared cervical artery dissection, and 7 patients got improvement after the treatment of hormone. Conclusion The clinical features and MRI manifestations of patients with central nervous system damage secondary to primary Sjogren syndrome are diverse.

关 键 词：原发性干燥综合征 中枢神经系统 颅神经 MRI 

分 类 号：R593.2[医药卫生—内科学]