Opinion statement
Patients with recent cerebral venous sinus thrombosis (CVT) should be fully anticoagulated with heparin as soon as the diagnosis is confirmed, even in the presence of cerebral hemorrhagic venous infarcts. Fixed-dose subcutaneous low-molecular-weight heparins (LMWH) in therapeutic dosage have a better safety profile and are probably more effective than dose-adjusted intravenous unfractionated heparin. After the patient is stabilized , oral anticoagulant treatment is started, aimed at an INR value between 2.0 and 3.0 for at least three months after the acute phase. Since about 40 % of patients with CVT have epileptic seizures at onset or during the first days, many require treatment with anti-epileptics. Prophylactic anti-epileptic treatment is an option, but its efficacy has not been investigated. If no new seizures occur after the acute phase, anti-epileptics can be tapered after three to six months. The efficacy of endovascular treatment, with mechanical and/or pharmacologic thrombolysis, has only been published in small case series, and cannot be regarded as proven effective treatment for CVT. Its theoretical advantage of more rapid clearance of thrombi may be offset by severe bleeding complications, and the limited availability and higher cost of the procedure. Patients who deteriorate because of large space-occupying venous infarcts have a high risk of dying from cerebral herniation. In such cases an emergent decompressive hemicraniectomy is often life-saving. Experience from different centers with this procedure shows a good clinical outcome in the majority of these patients. Patients with CVT may develop – and sometimes present with - chronic intracranial hypertension with headache and papilledema. In such patients the priority is prevention of visual function loss; intracranial hypertension should be controlled with acetazolamide, and occasionally with repeated lumbar punctures if vision is threatened. Refractory cases will need a CSF shunting procedure.
Similar content being viewed by others
References and Recommended Reading
Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance
Kalbach RM, Woolf AL. Cerebral venous thrombosis. London: Oxford University Press; 1967.
Coutinho JM, Zuurbier SM, Stam J. Declining mortality in cerebral venous thrombosis: a systematic review; Stroke 2014; in press.
Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med. 2005;352:1791–8. This review article provides a general overview of cerebral venous thrombosis.
Bousser MG, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol. 2007;6:162–70.
Coutinho JM, Zuurbier SM, Aramideh M, et al. The incidence of cerebral venous thrombosis: a cross-sectional study. Stroke. 2012;43:3375–7. This study found that the incidence of CVT among adults is approximately 4 times higher than previously believed.
Janghorbani M, Zare M, Saadatnia M, et al. Cerebral vein and dural sinus thrombosis in adults in Isfahan, Iran: frequency and seasonal variation. Acta Neurol Scand. 2008;117:117–21.
Coutinho JM, Ferro JM, Canhão P, et al. Cerebral venous and sinus thrombosis in women. Stroke. 2009;40:2356–61. This study highlights the differences in clinical manifestations, risk factor profile and outcome between men and women with CVT.
Coutinho JM, van den Berg R, Zuurbier SM, et al. Small juxtacortical hemorrhages in cerebral venous thrombosis. Ann Neurol. 2014. doi:10.1002/ana.24180.
Leach JL, Fortuna RB, Jones BV, Gaskill-Shipley MF. Imaging of cerebral venous thrombosis: current techniques, spectrum of findings, and diagnostic pitfalls. Radiographics. 2006;26 Suppl 1:S19–41. This review article provides a comprehensive overview of the available radiological modalities to diagnose CVT.
Saposnik G, Barinagarrementeria F, Brown Jr RD, et al. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke. 2011;42:1158–92. Most recent version of the AHA guidelines for the treatment of cerebral venous thrombosis.
Einhäupl K, Stam J, Bousser MG, et al. European Federation of Neurological Societies. EFNS guideline on the treatment of cerebral venous and sinus thrombosis in adult patients. Eur J Neurol. 2010;17:1229–35. Most recent version of the EFNS guidelines for the treatment of cerebral venous thrombosis.
Lebas A, Chabrier S, Fluss J, et al. EPNS/SFNP guideline on the anticoagulant treatment of cerebral sinovenous thrombosis in children and neonates. Eur J Paediatr Neurol. 2012;16:219–28.
Coutinho J, de Bruijn SF, Deveber G, et al. Anticoagulation for cerebral venous sinus thrombosis. Cochrane Database Syst Rev. 2011;CD002005. Cochrane meta-analysis on the efficacy and safety of anticoagulation for cerebral venous thrombosis
Einhäupl KM, Villringer A, Meister W, et al. Heparin treatment in sinus venous thrombosis. Lancet. 1991;338:597–600.
de Bruijn SF, Stam J. Randomized, placebo-controlled trial of anticoagulant treatment with low-molecular-weight heparin for cerebral sinus thrombosis. Stroke. 1999;30:484–8.
Coutinho JM, Ferro JM, Zuurbier SM, et al. Thrombolysis or anticoagulation for cerebral venous thrombosis: rationale and design of the TO-ACT trial. Int J Stroke. 2013;8:135–40.
Ferro JM, Canhão P, Stam J, et al. ISCVT Investigators. Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Stroke. 2004;35:664–70. This paper reports the main results of the ISCVT study, the largest international prospective cohort study on CVT.
Erkens PM, Prins MH. Fixed dose subcutaneous low molecular weight heparins versus adjusted dose unfractionated heparin for venous thromboembolism. Cochrane Database Syst Rev. 2010;(9):CD001100.
Coutinho JM, Ferro JM, Canhão P, et al. ISCVT Investigators. Unfractionated or low-molecular weight heparin for the treatment of cerebral venous thrombosis. Stroke. 2010;41:2575–80. The results of this non-randomized study indicate that low-molecular weight heparins are superior to unfractionated heparin for the treatment of CVT.
Misra UK, Kalita J, Chandra S, et al. Low molecular weight heparin versus unfractionated heparin in cerebral venous sinus thrombosis: a randomized controlled trial. Eur J Neurol. 2012;19:1030–6. Data from this small randomized study indicate that low-molecular weight heparins are superior to unfractionated heparin for the treatment of CVT.
Fennerty AG, Thomas P, Backhouse G, et al. Audit of control of heparin treatment. BMJ. 1985;290:27–8.
Aarab R, van Es J, de Pont AC, et al. Monitoring of unfractionated heparin in critically ill patients. Neth J Med. 2013;71:466–71.
Zuurbier SM, Coutinho JM, Majoie CB, et al. Decompressive hemicraniectomy in severe cerebral venous thrombosis: a prospective case series. J Neurol. 2012;259:1099–105.
Ferro JM, Crassard I, Coutinho JM, et al. Second International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT 2) Investigators. Decompressive surgery in cerebrovenous thrombosis: a multicenter registry and a systematic review of individual patient data. Stroke. 2011;42:2825–31. Retrospective multi-center study on decompressive craniotomy for cerebral venous thrombosis.
Canhão P, Cortesão A, Cabral M, et al. Are steroids useful to treat cerebral venous thrombosis? Stroke. 2008;39:105–10.
Compliance with Ethics Guidelines
Conflict of Interest
Dr. Coutinho declares that he has received research grants from The Netherlands Organisation for Scientific Research, Thrombosis Foundation Holland, the Dutch Heart Foundation, and the Netherlands Brain Foundation. He also declares that he has received speaker fees Boehringer Ingelheim, but that all of these fees have been donated to the “Stichting Klinische Neurologie”, a foundation that supports research in the field of neurological disorders.
Dr. Middeldorp declares that she has passed consultancy fees to her institution from Boehringer Ingelheim, Bayer, BMS/Pfizer Alliance, and Daiichi Sankyo. She also declares research grant funding from GSK and BMS/Pfizer Alliance, as well as honoraria payments from Boehringer Ingelheim, Bayer, BMS/Pfizer Alliance, and Daiichi Sankyo. Finally, she declares that she has received reimbursement of travel expenses from BMS/Pfizer Alliance and Daiichi Sankyo.
Dr. Stam declares research grant funding from Thrombosis Foundation Holland, the Dutch Heart Foundation, and the Netherlands Brain Foundation.
Human and Animal Rights and Informed Consent
This article does not contain any studies with human or animal subjects performed by any of the authors.
Author information
Authors and Affiliations
Corresponding author
Additional information
This article is part of the Topical Collection on Cerebrovascular Disorders
Rights and permissions
About this article
Cite this article
Coutinho, J.M., Middeldorp, S. & Stam, J. Advances in the Treatment of Cerebral Venous Thrombosis. Curr Treat Options Neurol 16, 299 (2014). https://doi.org/10.1007/s11940-014-0299-0
Published:
DOI: https://doi.org/10.1007/s11940-014-0299-0