The kaleidoscope of neurological manifestations in primary Sjögren's syndrome

Neurologic involvement is a common extraglandular manifestation of primary Sjögren's syndrome (pSS), is varied and can be divided anatomically into 3 categories: central nervous system, peripheral neuropathies and autonomous nervous system manifestations. According to different study cohorts, neurological manifestations can occur in 18-45% of pSS patients, with the peripheral nervous system being the most frequent site of involvement compared to the central nervous system and autonomic system. Some neurologic complications share convergent pathophysiology, although the pathological basis of other conditions, namely cognitive impairment in pSS, is less clear. The heterogeneity of neurologic manifestations in pSS complicates the diagnosis and approach to treatment, which should be directed toward the underlying neuro-pathologic mechanism. The diagnosis and treatment of these manifestations must be optimised in order to avoid severe disability. However, for the majority of the complications, evidence for treatment efficacy is limited and requires further investigation.