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目的了解原发性干燥综合征(pSS)患者中枢神经系统病变(CNS-SS)的临床特征。方法对北京协和医院21例CNS-SS患者进行回顾性分析。结果CNS-SS临床表现多样,脑损害12例,占57.1%,脊髓损害9例,占42.9%;7例合并周围神经系统病变,占33.3%;CNS症状出现时pSS的平均病程(4±6)年,10例CNS-SS以CNS病变为首发表现,占47.6%,在pSS诊断后第1、2、3年内出现CNS症状的分别为1、2和0例,第4年至17年的共8例。核磁共振成像(MRI)检查异常13例,占61.9%,其中脑白质脱髓鞘病变4例,占19.0%,脑缺血病变2例,占9.5%,灰质病变1例,占4.8%,脊髓脱髓鞘病变4例,占19.0%,脊髓空洞症2例,占9.5%。脑脊液检查异常11例,占52.3%.其中蛋白、压力和白细胞数增高者分别为10、6和5例,分别占47.6%、28.6%和23.8%。结论pSS可以合并中枢神经系统受累,其中脱髓鞘病变最常见,诊断CNS-SS最好同时行MRI和脑脊液检查。
Objective To investigate the clinical features of central nervous system lesions (CNS-SS) in patients with primary Sjogren’s syndrome (SS). Methods 21 cases of CNS-SS in Peking Union Medical College Hospital were retrospectively analyzed. Results The clinical manifestations of CNS-SS were diverse. Brain damage was found in 12 cases (57.1%), spinal cord injury in 9 cases (42.9%), peripheral neuropathy in 7 cases (33.3%), mean duration of CNS symptoms (4 ± 6 ) Years, 10 CNS-SS patients showed CNS disease as the first manifestation, accounting for 47.6%. In the first, second and third years after pSS diagnosis, symptoms of CNS were 1, 2 and 0 cases respectively, and in the 4th to 17th years A total of 8 cases. Magnetic resonance imaging (MRI) abnormalities in 13 cases, accounting for 61.9%, including demyelinating lesions in 4 cases, accounting for 19.0%, cerebral ischemic lesions in 2 cases, accounting for 9.5%, gray matter lesions in 1 case, accounting for 4.8%, spinal cord Demyelinating lesions in 4 cases, 19.0%, syringomyelia in 2 cases, accounting for 9.5%. Cerebrospinal fluid abnormalities were detected in 11 cases (52.3%), including 10, 6 and 5 cases, accounting for 47.6%, 28.6% and 23.8% respectively. Conclusion pSS can be involved in the central nervous system involvement, of which demyelinating lesions are the most common, the best diagnosis of CNS-SS MRI and cerebrospinal fluid simultaneously.