A Case of Arterial Cerebral Thrombosis with Congenital Protein C Deficiency

Abstract

Deficiency of protein C has been considered as an increased risk factor of thrombotic disease so far. Deficiency of this protein predisposes to venous thrombosis, but have been very rarely associated with arterial thrombosis.In this study we described a case of multiple cerebral infarction and cerebral arterial thrombosis in a 46-year-old man with heterozygous protein C deficiency (type 1 deficiency). Cranial computed tomography showed multiple hypodensity areas in bilateral basal ganglia, and cerebral angiography demonstrated occlusion in the territory of left posterior cerebral artery.Routine coagulation tests containing Fbg, AT III, Plg, PS and Lp (a) were within normal limits. Hepatic function was also found to be normal. No other risk factors (smoking, dyslipidemia and diabetes) but hypertension for arterial thrombotic disease were present in the propositus. Three other members of this family (mother and two sons) showed the same defect, but they had been asymptomatic.It is considered that congenital protein C deficiency may also be a risk factor for cerebral arterial thrombosis. Protein C activity should be evaluated in patients with juvenile onset of cerebral infarction especially in ones without any known risk factors.