Alexander E L
Scand J Rheumatol Suppl. 1986;61:161-5.
Central nervous system (CNS) complications occur in approximately 25% of patients with primary Sjögren's syndrome (CNS-SS) seen at our institution. We describe herein the clinical, neurodiagnostic, and immunopathologic features of CNS-SS. The neurologic manifestations can be protean and affect the entire neuroaxis. Characteristically, CNS-SS is multifocal, recurrent, and progressive resulting in cumulative neurologic impairment over time. In addition to specific neurologic deficits, more subtle abnormalities of personality and mentation may occur. Whereas, computed axial tomography of the head and cerebral angiography are relatively insensitive techniques in detecting abnormalities, brain magnetic resonance imaging shows multiple small areas of increased intensity in two thirds of CNS-SS patients. Electrophysiologic studies or cerebrospinal fluid analyses are abnormal in two thirds of CNS-SS patients. Preliminary histopathologic studies show several types of CNS inflammatory involvement suggesting an immunologically mediated autoimmune disorder.
在我们机构就诊的原发性干燥综合征合并中枢神经系统病变（CNS-SS）患者中，约25%会出现中枢神经系统（CNS）并发症。我们在此描述CNS-SS的临床、神经诊断及免疫病理特征。其神经表现多样，可累及整个神经轴。典型的CNS-SS具有多灶性、复发性和进行性特点，随时间推移会导致累积性神经功能损害。除了特定的神经功能缺损外，还可能出现更细微的人格和精神异常。头部计算机断层扫描和脑血管造影在检测异常方面相对不敏感，而脑磁共振成像显示三分之二的CNS-SS患者有多个小面积强化区。三分之二的CNS-SS患者电生理研究或脑脊液分析异常。初步组织病理学研究显示中枢神经系统有几种炎症累及类型，提示为免疫介导的自身免疫性疾病。
