Cerebral Vein and Dural Venous Sinus Thrombosis: Risk Factors, Prognosis and Treatment—a Modern Approach

Abstract

ABSTRACTCerebral vein and dural venous sinus thrombosis is a rare cause of stroke. It affects between three and four patients per million adults; although the incidence is higher in children and young women. Our understanding of this disease has evolved over the last two decades. Thrombophilia, particularly factor V Leiden and prothrombin gene mutations, as well as oral contraceptive use have been identified as predominant risk factors in contemporary studies compared to infection and puerperium in earlier series. Women of childbearing age account for 75% of cases. Compared to mortality rates in the past, which were as high as 50%, the outcome has improved considerably in recent years—likely due to better management and improved diagnostic capabilities. Approximately 80% of patients will have a meaningful recovery, and in patients receiving anticoagulation the risk of recurrence is about 2%. Magnetic resonance imaging, magnetic resonance venography and computerized tomographic venography have become the methods of choice for diagnosing cerebral vein and dural sinus thrombosis. Anticoagulation with unfractionated heparin and low molecular weight heparin is a well-accepted standard of care even in the presence of concurrent intracranial haemorrhage, while thrombolysis has been used only in selected cases and remains investigational. This review will focus on the presentation and natural history of cerebral vein and sinus thrombosis, risk factors and recommendations for treatment.