Abstract
Sjögren's syndrome (SS) is less frequently diagnosed in childhood than in adults, and central nervous system (CNS) disease is among the rarest systemic involvements. Thus, the clinical spectrum of CNS diseases and their management strategies have not been fully defined. In this article, we present the case of a 16-year-old girl who was referred for severe headache and diagnosed with SS with CNS involvement. Several immunosuppressive treatments failed to improve her neurological symptoms until the rituximab treatment. When we systematically reviewed the literature on cases of CNS involvement in childhood-onset SS, we found that CNS involvement was the presenting feature at the diagnosis of SS in the majority of published cases. While headache and fever were the most frequent complaints at presentation, most of the children displayed features of neuromyelitis optica spectrum disorder. CNS disease showed a variable response to immunosuppressives, and residual neurological deficits were not rare. Additionally, a significant number of cases required treatment with rituximab due to the treatment failures or subsequent flares. Sjögren's syndrome should be considered in children presenting with predominant neurological symptoms, and careful evaluation of glandular features might help in the prompt diagnosis of childhood-onset SS in children with CNS disease.