Department of Neurology, Tongde Hospital of Zhejiang Province, 234 Gucui Road, 310012, Hangzhou, Zhejiang, China.
Department of Cardiology, Hangzhou Hospital of Traditional Chinese Medicine, Hangzhou, Zhejiang, China.
BMC Neurol. 2022 Nov 8;22(1):414. doi: 10.1186/s12883-022-02945-2.
Primary Sjögren's syndrome (pSS) is an autoimmune inflammatory disease characterized by dryness of the eyes, mouth and other mucous membranes. Patients with pSS can also present with extraglandular manifestations, such as pulmonary, kidney and nervous system involvement. Central nervous system (CNS) manifestations have rarely been described in pSS.
A 33-year-old man was admitted with a one-month history of dizziness, speech disturbance, and walking instability. His brain enhanced magnetic resonance imaging (MRI) showed symmetrical, enhanced "salt-and-pepper-like" speckled lesions in the brainstem, basal ganglia, and subcortical regions, and his diagnosis was considered possible chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Further examination revealed that anti-SSA antibody was positive, and the Schirmer test and labial salivary gland histopathology were abnormal, which supported the diagnosis of pSS.
pSS is a chronic systemic autoimmune disease that involves neurological complications. This case suggests that CNS lesions of pSS can present with clinical and MRI findings similar to those of CLIPPERS.
原发性干燥综合征（pSS）是一种以眼睛、口腔和其他黏膜干燥为特征的自身免疫性炎症性疾病。pSS 患者还可能出现腺外表现，如肺部、肾脏和神经系统受累。中枢神经系统（CNS）表现在 pSS 中很少被描述。
一名 33 岁男性因头晕、言语障碍和行走不稳入院，病史为一个月。他的脑部增强磁共振成像（MRI）显示脑干、基底节和皮质下区域存在对称、增强的“椒盐状”斑点样病变，考虑可能为对类固醇有反应的慢性淋巴细胞性炎症伴脑桥血管周围强化（CLIPPERS）。进一步检查显示抗 SSA 抗体阳性，Schirmer 试验和唇腺组织病理学异常，支持 pSS 的诊断。
pSS 是一种慢性全身性自身免疫性疾病，涉及神经系统并发症。本病例提示 pSS 的中枢神经系统病变可表现出与 CLIPPERS 相似的临床和 MRI 表现。
