摘要： 回顾分析4 例原发性干燥综合征合并中枢神经系统病变患者临床与影像学资料，表现为发热（2 例）、视物模糊（2 例）、双侧下肢无力（2 例）、单侧下肢无力（1 例）、偏侧肢体无力（1 例）、偏身感觉减退（1 例）、双侧外展神经麻痹（1 例）和周围神经病变（3 例）；脑脊液压力升高（210 ~ 270 mm H2O，3 例）、白细胞计数增加［（50 ~ 380）× 106/L，3 例］、蛋白定量升高（1.30 ~ 2.56 g/L，3 例）；MRI 以颅内多发异常信号为特征。经大剂量激素冲击治疗3 例恢复良好、1 例于6 个月后复发。原发性干燥综合征合并中枢神经系统病变临床鲜见，腰椎穿刺脑脊液检查和头部MRI检查对诊断具有重要意义，大剂量激素冲击治疗效果良好。

关键词: 干燥综合征, 中枢神经系统

Abstract: Clinical and imaging data of 4 cases of primary Sjögren's syndrome (pSS) combined with central nervous system (CNS) lesions were retrospectively analyzed. Clinical symptoms of 4 patients were as follows: 2 cases had fever; 2 cases had blurred vision; 2 cases had lower extremity weakness; one case had single lower limb weakness; one case had unilateral limb weakness; one case had hemisensory reduction; one case had bilateral abducens paralysis; 3 cases had peripheral neuropathy. Analysis of cerebrospinal fluid (CSF) revealed increased CSF pressure (210-270 mm H2O) in 3 cases, increased number of leukocyte [(50-380) × 10 6/L] in 3 cases, and increased protein (1.30-2.56 g/L) in 3 cases. Brain MRI showed multiple lesions in white matters. After high-dose steroid therapy, 3 cases had good prognosis, and one had recurrent lower extremity weakness after 6 months. pSS combined with CNS lesions is rare. Lumbar puncture and brain MRI is important for diagnosis, and high-dose steroid therapy may have good therapeutic effect.

Key words: Sjogren's syndrome, Central nervous system