MEET THE EXPERT Cerebral sinus-venous thrombosis Ida Martinelli • Serena Maria Passamonti • Elena Rossi • Valerio De Stefano Ó SIMI 2012 Abstract Cerebral sinus-venous thrombosis (CSVT) is a rare life-threatening disease with an estimated annual incidence of 3–4 cases per million in adults and 7 cases per million in neonates. Brain tumors, cerebral infections and traumas are local risk factors for CSVT, but the commonest encountered risk factors are oral contraceptive use, preg- nancy and puerperium that make the disease predominant in female sex. In 15–20 % of patients, the disease remains unprovoked, i.e., occurring in the absence of predisposing factors. Thrombophilic abnormalities either inherited [deficiency of the natural anticoagulant proteins anti- thrombin, protein C or protein S, mutations in the factor V gene (factor V Leiden) or prothrombin gene (prothrombin G20210A)] or acquired (antiphospholipid antibodies) are worthy to be investigated in patients with CSVT, as well as hyperhomocysteinemia. In a small proportion of patients, CSVT is the first manifestation of a myeloproliferative neoplasm. The proportion of patients with recurrent CSVT is low, but venous thromboembolism (deep vein thrombosis in the lower limbs or pulmonary embolism) can develop particularly in patients with a first idiopathic CSVT. In the past decade, there has been increasing evidence that early diagnosis and anticoagulant treatment reduce morbidity of CSVT and improve survival. However, the optimal duration of anticoagulant treatment is not well established, because limited information is available on the rate of CSVT recurrence after anticoagulant discontinuation. Keywords Cerebral sinus Á Venous thrombosis Á Thrombophilia Á Risk factors Á Anticoagulant therapy Introduction Venous thrombosis affects mainly the lower extremities but may rarely involve other venous districts, such as cerebral veins and sinuses, and splanchnic and upper extremity veins. In a nationwide epidemiological study, rare venous thromboses (defined as migrating thrombo- phlebitis, abdominal and cerebral venous thrombosis, and renal vein thrombosis) accounted for 4 % of hospitalized cases with venous thrombosis, with an overall incidence lower than one per 100,000 person-years [1]. Among the rare sites of venous thrombosis, the cerebral circulation is one of the most life threatening. Because in the vast majority of patients thrombosis develops concomitantly in sinuses and veins, the disease is commonly named cerebral sinus-venous thrombosis (CSVT). Epidemiology and clinical manifestations The incidence of CSVT is uncertain, because of the absence of epidemiological studies. At variance with arterial stroke, CSVT affects mainly young adults and children, with an estimated annual incidence of 3–4 cases per 1 million adults and 7 cases per 1 million neonates and children [2, 3]. The most frequent locations of thrombosis are the superior sagittal (62 % of patients) and the trans- verse sinus (40–45 %), but in two-thirds of cases more than I. Martinelli (&) Á S. M. Passamonti Department of Internal Medicine and Medical Specialities, Angelo Bianchi Bonomi Hemophilia and Thrombosis Center, Fondazione IRCCS Ca’ Granda, Ospedale Maggiore Policlinico, Via Pace, 9, 20122 Milan, Italy e-mail: martin@policlinico.mi.it E. Rossi Á V. De Stefano Institute of Hematology, Catholic University, Rome, Italy 123 Intern Emerg Med (2012) 7 (Suppl 3):S221–S225 DOI 10.1007/s11739-012-0806-9