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 59-year-old male patient suffered from rapidly progressive and pulsatile headache over the right parietal region characterized as thunderclap and nausea. He had no fever. These symptoms were followed by generalized seizure. Neurological examination showed a normal level of consciousness. On examination, meningism was seen. The rest of the physical examination was unremarkable. CT scan head was normal. MRI revealed right parasagittal high fronto-parietal subarachnoid hemorrhage with mild gyral edema. MRV showed superior sagittal sinus and bilateral transverse sinus thrombosis [Figures and ]. DSA confirmed [Figures and ] the diagnosis of DST without any potential cause of SAH. Coagulation testing, including prothrombin time, activated prothrombin time, anticardiolipin antibody titer, antiphospholipid antibody titer, homocysteine titer, and levels of protein C and S, antithrombin III, and fibrinogen were all within normal limits and done simultaneously with MRI, MR Venography, DSA within one to two days of admission. Subcutaneous LMW heparin therapy was given. The patient's condition stabilized after nine days of treatment. Oral warfarin maintained an INR of 2.0-3.0. Patient's improvement was clinically satisfactory within six weeks, Repeat DSA done after six weeks was normal, except minimal filling defect. Patient was followed up at two-weeks intervals for three months