To summarize the neurological manifestations in patients with primary Sjögren′s syndrome (pSS).

A total of 68 patients were diagnosed as pSS in neurology department of Peking Union Medical College Hospital from March 2014 to February 2018, among whom sixteen cases were excluded due to modified final diagnoses of primary neurological diseases. Therefore 52 pSS patients with neurological involvement were enrolled and retrospectively analyzed. They were divided into two groups as extensive group in which both central and peripheral nervous system were involved, non-extensive group in which either central or peripheral nervous system was involved.

Neurological manifestations were presented as primary symptoms in 98.1%(51/52) patients, while 35 had neurological involvement as their only extraglandular manifestations. Thirteen cases were in extensive group. The other 39 in non-extensive group including 22 cases with only peripheral nervous system involved and 17 cases with only single central nervous system involved. Compared to non-extensive group, the proportion of woman patients [13/13 vs.71.8% (28/39), P=0.047], serum IgG level [17.73(11.11,22.41)g/L vs. 11.49(9.58,13.40)g/L, P=0.017] and positive rates of oligoclonal band (OB) in cerebral spinal fluid (CSF) [7/13 vs. 22.6%(7/31), P=0.042)] were significantly higher in extensive involvement group.

Neurological manifestations in pSS patients could be extensive, both central and peripheral nervous system might be associated. Female patients, high serum IgG level and positive OB in CSF are risk factors of extensive neurological involvement, suggesting that the immune system may be generally over-stimulated.

Sjögren′s syndrome; Neurological involvement; Central nervous system injury; Peripheral nervous system injury