Niu Bing, Zou Zhenzhen, Shen Yuqin, Cao Bingzhen
Department of Neurology, General Hospital of Jinan Military Region, Jinan, Shandong Province, China.
Medicine (Baltimore). 2017 Apr;96(17):e6715. doi: 10.1097/MD.0000000000006715.
Peripheral neurological complications in primary Sjögren's syndrome (pSS) seem the most common, however the involvement of central nervous system (CNS) remains unclear. While abnormalities in pSS revealed by brain magnetic resonance imaging (MRI) are usually small discrete hyperintense areas in the white matter on T2-FLAIR weighted MRI, massive brain lesions have been rarely reported, particularly in bilateral basal ganglia.
A 51-year-old woman exhibited dizziness, slurred speech and hemiplegia as a manifestation of pSS. Brain MRI revealed bilateral and symmetrical lesions extending into the basal ganglia, corona radiata and corpus callosum.
Primary Sjögren's syndrome was diagnosed on the basis of clinical features, abnormal Schirmer's test and tear break-up time (BUT) findings, high levels of anti-Sjögren's-syndrome-related antigen A (anti-SSA) (Ro) and anti-Sjögren's-syndrome-related antigen B (anti-SSB) (La) antibodies, and positive labial minor salivary gland biopsy results.
She was treated with intravenous methylprednisolone and discharged on oral steroid therapy of prednisolone acetate.
The patient had an excellent response to steroid therapy.
The present case suggests that symmetry bilateral lesions can occur as a symptom of pSS, which could be induced by an autoimmune mechanism.
原发性干燥综合征（pSS）的周围神经并发症似乎最为常见，然而中枢神经系统（CNS）受累情况仍不明确。虽然脑磁共振成像（MRI）显示的pSS异常通常是T2-FLAIR加权MRI上白质中的小而离散的高信号区，但大量脑病变很少被报道，尤其是双侧基底节病变。
一名51岁女性表现出头晕、言语不清和偏瘫，为pSS的表现。脑MRI显示双侧对称病变延伸至基底节、放射冠和胼胝体。
根据临床特征、Schirmer试验和泪膜破裂时间（BUT）检查结果异常、抗干燥综合征相关抗原A（抗SSA）（Ro）和抗干燥综合征相关抗原B（抗SSB）（La）抗体水平升高以及唇小唾液腺活检结果阳性，诊断为原发性干燥综合征。
给予静脉注射甲泼尼龙治疗，出院时接受醋酸泼尼松口服类固醇治疗。
患者对类固醇治疗反应良好。
本病例表明，对称性双侧病变可作为pSS的症状出现，可能由自身免疫机制诱发。
