Protein S deficiency associated with progressive loss of vision and intracranial venous sinus thrombosis

We report a 27-year-old male who presented with headache and rapid visual impairment. He had been diagnosed with venous sinus thrombosis 3 months earlier, when he had diffuse headache, nausea and vomiting, which subsided after incomplete thrombolytic therapy. Warfarin was then prescribed without screening for coagulopathy. Ophthalmic examination revealed bilateral papilloedema, splinter hemorrhage and lipid exudates. Neuroradiological studies including magnetic resonance imaging and cerebral angiography revealed chronic partial thrombosis over superior sagittal sinus, left side transverse sinus, right side transverse and venous confluence with engorged cortical veins and secondary dural arteriovenous malformation (AVM) and reversed flow over bilateral superior orbital veins, and thrombolytic therapy was considered not feasible. Clot-assay protein S activity was decreased (25%, normal range: 65-140%). No underlying connective tissue diseases or other coagulopathies were noted. The patient's vision failed to respond to aggressive medical treatment, and he received lumboperitoneal shunt in another hospital. His vision was improved. For young patients with occlusive cerebrovascular disorder, extensive hematological investigation for coagulopathy is strongly recommended.