A 68-year-old woman with polycythemia vera experienced a global, nonpositional headache for one day. She subsequently became comatose. On emergent evaluation she exhibited decorticate posturing. Her Glasgow coma score was 6. There was no preceding history of trauma, infection, or dehydration. She was not on anticoagulants or antiplatelet agents. Her polycythemia vera had been managed with therapeutic phlebotomy and hydroxyurea (500 mg daily). She had no prior history of thromboembolic events. Noncontrasted head CT showed extensive left 20 mm acute SDH with 16 mm of midline shift []. She underwent emergent craniotomy and hematoma evacuation. On careful inspection of the exposed brain, a prominent and engorged cortical vein observed. No other vascular abnormalities or potential bleeding sources were identified. CT venography and MR venography detected partial superior sagittal sinus thrombosis, which leads to the empty delta sign (the intraluminal thrombus prevents central filling of the sinus; ). She was managed conservatively with hydroxyurea, hydration, and aspirin. Laboratory analyses were notable for normal coagulation panel, normal platelet function assays, hematocrit of 45.9, negative thrombophilia evaluation (protein S, protein C, antithrombin III, anticardiolipin antibody, homocysteine, lupus anticoagulant, beta-2 glycoprotein antibodies, Factor V Leiden mutation, prothrombin gene mutation). Analysis of DNA extracted from her blood revealed the presence of the V617F mutation within the JAK2 gene. Her 3-month outcome was poor (modified Rankin score 4).
A 40-year-old female presented to an emergency in altered sensorium with right hemiplegia with a history of a severe headache and multiple episodes of vomiting 1 day prior to admission. She was on oral contraceptives for last 2 years. Her Glasgow Coma Scale at admission was E1M3V1 and left pupil was dilated and not reacting to light. Computed tomography (CT) of head revealed venous infarct in left temporo-occipito-parietal region with linear area of hemorrhage within it with large left fronto-temporo-parietal acute subdural hematoma with midline shift of 9 mm toward right side with subfalcine herniation with descending transtentorial herniation with sub-arachnoid hemorrhage in the left parietal and occipital sulci with diffuse cerebral edema [].\nCT venography revealed thrombosis in superior sagittal sinus extending to adjacent cortical veins []. She was immediately taken for surgery, and a left fronto-temporo-parietal decompressive craniectomy was performed with the evacuation of acute subdural hematoma and lax duroplasty. However, she continued to deteriorate in the postoperative period and died on the first postoperative day.