目的·总结以神经系统损伤为首发症状的2例原发性干燥综合征（primary Sj?gren's syndrome，pSS）患者的临床特点并复习相关文献。方法·对以神经系统受累为首发症状的2例pSS患者的临床表现、实验室检查结果、影像学检查结果、唇腺活检结果、治疗效果等进行总结和分析。结果·病例1，女性，自50岁起出现进行性行走不稳伴下肢远端感觉障碍，表现为痉挛步态、感觉性共济失调、传导束性感觉障碍和二便障碍；58岁起无法独立行走。实验室检查提示抗核抗体阳性（1∶1 000着丝点型），抗干燥综合征抗原A（SSA）/Ro-52抗体、抗SSA/Ro-60抗体、抗着丝点抗体阳性；唇腺活检可见唾液腺组织、导管间及小叶内淋巴细胞浸润灶2个/4 mm²（淋巴细胞>50个）。头颅磁共振成像可见“蛇眼征”；肌电图显示右侧腓神经运动纤维轴索损伤。在院期间予以大剂量糖皮质激素冲击、联合免疫抑制剂治疗后明显好转。3个月后可推助行器行走，日常生活基本自理。病例2，女性，自81岁起出现颈部不自主右斜，既往有轻度口干、眼干、伴膝关节疼痛1年。实验室检查提示抗SSA/Ro-52抗体、抗SSA/Ro-60抗体阳性，血红蛋白86 g/L；唇腺活检可见小唾液腺组织部分腺泡萎缩，间质内见淋巴细胞、浆细胞浸润灶2个/4 mm²（淋巴细胞>50个）。周围神经电生理检查结果提示右侧正中神经传导速度减慢。经免疫抑制剂、解痉、肌松药物治疗后症状较前好转。结论·以复杂型痉挛性截瘫样表现、颈部肌张力障碍为首发症状的病例拓展了pSS的临床表型谱；临床上应注意鉴别继发于pSS的神经系统损伤与其他原发神经系统疾病；对于具有无明显原因的神经系统损伤症状的患者，应当筛查相关自身免疫抗体。

Objective ·To study the clinical features of two primary Sj?gren's syndrome (pSS) patients with neurological symptoms as initial manifestations and review the related literature. Methods ·The clinical data, response to treatment as well as prognosis of 2 cases were analyzed and followed up. Results ·Case 1, female, initially presented progressive gait instability and distal sensory impairment at the age of 50. Clinical manifestations included spastic gait, sensory ataxia, conduction fascicular sensory impairment, as well as urination and defecation dysfunction. At the age of 58, this patient was unable to walk independently. The laboratory findings revealed a positive result for anti-nuclear antibody with a titer of 1∶1 000, anti-Sj?gren's syndrome A (SSA)/Ro-52, anti-SSA/Ro-60, and anti-centromere antibodies. Labial salivary gland biopsy showed lymphocytes and plasma cells infiltration into the glandular tissues, interstitium, and lobules, with 2 foci/4 mm² (lymphocytes >50). The cranial magnetic resonance imaging exhibited bilateral symmetric hyperintensity in the brainstem, characterized by the "snake-eye sign". Electromyography examination revealed axonal impairment of the right peroneal nerve. The patient was treated with high-dose corticosteroid therapy in combination with immunosuppressants. She experienced remarkable improvement. After three months, she was able to walk with aids and take care of herself in daily life. Case 2, female, presented spasmodic torticollis since the age of 81 with unknown reason. She had a history of mild mucosal dryness of mouth and eyes, and painful knee for one year. Laboratory findings revealed positive results for anti-SSA/Ro-52 and anti-SSA/Ro-60 antibodies, as well as hemoglobin of 86 g/L. Labial salivary gland biopsy demonstrated partial atrophy of the acini and the presence of 2 foci/4 mm² of lymphocytes and plasma cells infiltration into the stroma (lymphocytes>50). Electromyography examination showed reduced conduction velocity in the right median nerve. She got a significant relief after the treatment of immunosuppressants, antispasmodics, and muscle relaxants. Conclusion ·Patients presenting initial symptoms such as complex forms of spastic paraplegia and cervical dystonia expand the clinical spectrum of pSS. In clinical practice, it is important to distinguish neurological involvement secondary to pSS from other primary neurological disorders. For patients with neurological impairments but without apparent etiology, it is crucial to screen relevant series of autoimmune antibodies.