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Cerebral vein thrombosis (CVT) is an infrequent condition with a large variety of causes that can lead to serious disabilities. However, in 20% to 35% of cases, no cause is found. In this study we evaluated the hereditary (P & C Proteins, antithrombin, mutation of prothrombin G20210A and factor V Leiden), other risk factors (hyperhomocycteinemia, factor VIII, ACL-ab, APL-ab, and OCP) and clinical manifestations among a population of Iranian patients with CVT. 18 women and 10 men aged 16 to 50 years with CVT were screened for inherited and acquired coagulation risk factors. No one had an abnormal ACL-ab, APL-ab or antithrombin III deficiency. One had prothrombin G20210A mutation (heterozygot) (3.6%). Hyperhomocycteinemia was observed in 5 patients (17.9%). APC-R was decreased in 3 (10.7%). 2 had positive factor V Leiden mutation (heterozygot) (7.1%). 17 had an increased of factor VIII (60.7). PS and PC deficiencies were each detected in two cases (7.1%). Conclusion: Our study suggests that screening for inherited thrombophilia may be an integral part in the diagnostic workup and duration of treatment in patients with CVT.