To investigate the clinical characteristics of primary Sj-gren's Syndrome （pSS） patients with central nervous system（CNS） damage as the initial manifestation.Methods Fifteen pSS patients with CNS damage as the initial manifestation were recruited.Clinical manifestations，laboratory results and imaging findings were retrospectively analyzed.Descriptive statistics was used for statistical analysis.Results Among the 15 patients，the ratio of male to female was 1∶4，and the first onset age was （45.53±16.19） years old.The diagnosis was confirmed after an average of 32.37 months.The initial symptoms included headache （6.67%），dizziness （20%），vomiting （20%），slurred speech （6.67%），facial paralysis （6.67%），hemilateral limb weakness （26.67%），numbness and weakness of both lower limbs （13.33 %） or the limbs （46.67%），chest and back pain（26.67%），blurred vision （6.67%）.The lesions involved the cerebral hemisphere （33.33%），brain stem （53.33%），cerebellum （6.67%），spinal cord （53.33%），and optic nerve （6.67%），presenting as cerebral infarction（33.33%），SAH（6.67%），transverse myelitis（6.67%），bulbar lesion（6.67%），neuromyelitis optica spectrum disorder （NMOSD）（53.33%） and NMOSD complicated with cerebral infarction in 1 case （6.67%）.CNS symptoms recurred in 3 cases （20%） before pSS was diagnosed.At the time of diagnosis，the positive rate of autoimmune antibodies was anti-SSA antibody 100%，anti-nuclear antibody 100%，anti-SSB antibody 73.33%，anti-R0-52 antibody 80%，anti-ds-DNA antibody 6.67%，respectively.The positive rate of RF was 20%.The increase rate of IgG and IgA were 20%，and the decrease rate of complement C3 was 20%.The cerebrospinal fluid study showed：CSF pressure （80-240） mmH-2O，white blood cells cout （4-130）×106/L，protein （0.27-1.46） g/L；CSF IgG increased in 5 cases，and IgM increased in 2 cases，and IgA increased in 4 cases.Conclusion CNS damage can be the initial manifestation of pSS.The lack of timely screening and monitoring of autoimmune antibody is an important reason for the delayed diagnosis of pSS.For unexplained CNS lesions，especially those presenting as recurrent demyelinating events or cerebral infarction，autoimmune antibodies such as anti-SSA and SSB should be examed actively，while the symptoms such as dry eyes and dry mouth should be questioned.It is important for early diagnosis.