Background: Sjögren's syndrome is an autoimmune disease characterised by
exocrinopathy mainly involving the salivary and lacrimal glands. In addition, it is a multisystemic
condition (i.e., affecting multiple organs and systems). Neurological involvement has been reported
in ~20% of cases, with peripheral manifestations being the most frequent.
Methods: We analysed four cases in which neurological manifestations were the first symptoms of
Results: In all four cases, neurological symptoms preceded sicca symptoms. In addition, immunosuppressive
treatment with steroids and, in some cases, cyclophosphamide showed improvement.
Conclusion: Neurological involvement in Sjögren's syndrome is common and often occurs as the
first clinical manifestation. Since evidence is limited, more studies are required in order to determine
appropriate diagnostic methods and treatments for each manifestation of Sjögren's syndrome.