Background: Although Sjögren's syndrome has been known to complicate with white matter lesions, encephalopathy, or stroke, reports of cerebral venous sinus thrombosis due to Sjögren's syndrome with atypical antibodies are rare.
Case report: A 50-year-old woman was admitted to our neurological ward with nausea and vomiting following acute onset of severe headache in the left occipital region. Brain computed tomography revealed no abnormalities. The patient was fully conscious, with normal cognitive functioning, but exhibited unsteady tandem gait. Both magnetic resonance venography and computed tomography venography suggested left transverse sinus blockage. Intravenous enoxaparin, followed by oral warfarin, was initiated as treatment for cerebral venous sinus thrombosis. After investigation, Sjögren's syndrome was diagnosed and lupus anticoagulant antibody test was positive. The patient was treated with hydroxychloroquine, and appeared fully recovered at the 6-month follow-up, with no clinical or radiological signs of relapse.
Conclusion: This case reports the relationship between cerebral venous sinus thrombosis and Sjögren's syndrome. It is necessary to screen autoimmune disorders in patients with cerebral venous sinus thrombosis that present with no common risk factors of venous thrombosis in order to prevent inappropriate management, and potentially adverse outcomes.