A 39-year-old woman presented at the emergency department with three weeks of progressive, constant and pulsatile right-sided headache. She said her headache was worse in the morning and when she would bend forward. She reported associated nausea and vomiting.
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December 2018On initial assessment, she did not have any focal neurological deficits. Her medical history was significant for tobacco use, alcohol abuse, depression and recent diagnosis of Behçet’s disease. Behçet’s was diagnosed on the basis of many years of recurrent oral ulcers, one year of recurrent genital ulcers, diffuse arthralgia, positive pathergy, history suggestive of prior iritis and pustular skin eruption with an otherwise negative rheumatologic workup. At the time of presentation, she was not on any treatment for Behçet’s disease due to financial limitations.
In the emergency department, she was promptly evaluated with magnetic resonance imaging (MRI) and magnetic resonance venography (MRV), and was found to have extensive sinus thrombosis involving the right internal jugular vein, right sigmoid sinus, right transverse sinus and right superior sagittal sinus (see Figures 1 and 2). She did not have any findings suggestive of infarction or hemorrhage.
Her ophthalmologic evaluation did not show any evidence of subclinical uveitis or optic neuritis. The findings of cerebral venous sinus thrombi (CVST) were attributed to Behçet’s disease, and a hypercoagulable workup, including factor V Leiden, prothrombin 20210 mutation and antiphospholipid antibodies, was later unrevealing.