Abstract
The case of a 27-year-old man with sinus thrombosis caused by protein C deficiency is reported. He suffered seizure, headache and higher brain functional disorder. T2 weighted MR image showed disappearance of flow void in right sigmoid sinus and magnetic resonance venography (MRV) revealed dural sinus thrombosis from the superior sagittal sinus to the right sigmoid sinus. Cerebral angiography confirmed these findings and delay of cerebral circulation time with collateral venous drainage. Xenon-enhanced computed tomographic measurement of cerebral blood flow demonstrated whole brain ischemia. Coagulation studies revealed decreases in both protein C antigen level and activity. The same results were obtained from a study of the patient's mother. Prior to obtaining coagulation study results, he was treated with glycerol and continuous intravenous heparin injection. Headache diminished but did not disappear. After administration of continuous intravenous urokinase, headache disappeared. Magnetic resonance venography demonstrated sinus re-canalization. Management of patients with protein C deficiency is often problematic due to the risk of warfarin-induced skin necrosis. To avoid this complication, oral administration of warfarin was started at low dosage, progressively increasing daily doses with co-administration of heparin. Treatment of this rare disease thus proved successful, and he has been followed up with no subsequent problems.
