Skip to main content

Advertisement

SpringerLink
Log in

Cerebral Venous Thrombosis Headache

  • Uncommon and/or Unusual Headaches and Syndromes (J Ailani, Section Editor)
  • Published:
Current Pain and Headache Reports Aims and scope Submit manuscript

Abstract

Purpose of Review

Cerebral venous thrombosis (CVT) is a neurovascular disease caused by a thrombotic occlusion of either a dural sinus or cerebral vein. CVT results in a variety of neurological symptoms with the most common being headache. The purpose of this review is to characterize CVT, describe the headache pattern, and, finally, provide an update to date review of diagnostic and treatment options for this condition.

Recent Findings

CVT is a very difficult disease to diagnose due to the variability in both patient presentation and imaging findings. Recent literature has attempted to standardize its risk factors, diagnosis, and treatment modalities. Additionally, new laboratory studies are being investigated for CVT patients who present with isolated headaches.

Summary

CVT is a debilitating disease requiring immediate medical or surgical intervention. Because the disease can mask as a multitude of neurological deficits, patients are not properly diagnosed. Headache is the most common patient presentation. The quality of this headache is highly variable with no specific location or pattern. New literature has provided insight into potential diagnostic and treatment options for CVT patients. However, further large-scale cohort studies are necessary to standardize the care for this disease.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance •• Of major importance

  1. Ribes MF. Des recherches faites sur la phlébite. Revue Médicale Française et Etrangère et Journal de Clinique de l’Hôtel-Dieu et de la Charité de Paris. 1825;3:5–41.

    Google Scholar 

  2. Abercrombie J. ART. XV. Pathological and practical researches on the diseases of the brain and the spinal cord. Am J Med Sci. 1828;4:384–9. https://doi.org/10.1097/00000441-182808000-00015.

    Article  Google Scholar 

  3. Bousser MG, Chiras J, Bories J, Castaigne P. Cerebral venous thrombosis--a review of 38 cases. Stroke. 1985;16(2):199–213. https://doi.org/10.1161/01.str.16.2.199.

    Article  CAS  PubMed  Google Scholar 

  4. Ferro JM, Canhão P, Stam J, Bousser M, Barinagarrementeria F. Prognosis of cerebral vein and dural sinus thrombosis. Stroke. 2004;35(3):664–70. https://doi.org/10.1161/01.str.0000117571.76197.26.

    Article  Google Scholar 

  5. Kamal MK. Computed tomographic imaging of cerebral venous thrombosis. J Pak Med Assoc. 2006;56(11):519–22.

    PubMed  Google Scholar 

  6. Mattle HP, Wentz KU, Edelman RR, Wallner B, Finn JP, Barnes P, … Hoogewoud HM. Cerebral venography with MR. Radiology 1991;178(2):453–458. doi:https://doi.org/10.1148/radiology.178.2.1987608.

    Article  CAS  Google Scholar 

  7. • Silvis SM, Sousa DA, Ferro JM, Coutinho JM. Cerebral venous thrombosis. Nat Rev Neurol. 2017;13(9):555–65. https://doi.org/10.1038/nrneurol.2017.104 Excellent CVT review article providing the reader with an overview of the epidemiology, pathophysiology, clinical presentation, diagnostics, and treatment of the disease.

    Article  PubMed  Google Scholar 

  8. Coutinho JM. Cerebral venous thrombosis. J Thromb Haemost. 2015;13:S238–44. https://doi.org/10.1111/jth.12945.

    Article  PubMed  Google Scholar 

  9. Luo Y, Tian X, Wang X. Diagnosis and treatment of cerebral venous thrombosis: a review. Front Aging Neurosci. 2018;10. https://doi.org/10.3389/fnagi.2018.00002.

  10. Saposnik, G., Barinagarrementeria, F., Brown, R. D., Bushnell, C. D., Cucchiara, B., Cushman, M., … Tsai, F. Y.. Diagnosis and management of cerebral venous thrombosis. Stroke 2011;42(4):1158–1192. doi:https://doi.org/10.1161/str.0b013e31820a8364.

    Article  Google Scholar 

  11. Bousser M, Ferro JM. Cerebral venous thrombosis: an update. Lancet Neurol. 2007;6(2):162–70. https://doi.org/10.1016/s1474-4422(07)70029-7.

    Article  CAS  PubMed  Google Scholar 

  12. Silvis S, Middeldorp S, Zuurbier S, Cannegieter S, Coutinho J. Risk factors for cerebral venous thrombosis. Semin Thromb Hemost. 2016;42(06):622–31. https://doi.org/10.1055/s-0036-1584132.

    Article  PubMed  Google Scholar 

  13. Coutinho J, Middeldorp S, Lauw M, Barco S. Cerebral venous thrombosis and thrombophilia: a systematic review and meta-analysis. Semin Thromb Hemost. 2013;39(08):913–27. https://doi.org/10.1055/s-0033-1357504.

    Article  CAS  Google Scholar 

  14. Couturier M, Huguet F, Chevallier P, Suarez F, Thomas X, Escoffre-Barbe M, … Tanguy-Schmidt A. Cerebral venous thrombosis in adult patients with acute lymphoblastic leukemia or lymphoblastic lymphoma during induction chemotherapy with l-asparaginase: the GRAALL experience. Am J Hematol 2015;90(11):986–991. doi:https://doi.org/10.1002/ajh.24130.

    Article  CAS  Google Scholar 

  15. Zuurbier SM, Arnold M, Middeldorp S, Broeg-Morvay A, Silvis SM, Heldner MR, … Coutinho JM. Risk of cerebral venous thrombosis in obese women. JAMA Neurol 2016;73(5):579-584. doi:https://doi.org/10.1001/jamaneurol.2016.0001.

    Article  Google Scholar 

  16. Stolz E, Valdueza JM, Grebe M, Schlachetzki F, Schmitt E, Madlener K, … Kaps M. Anemia as a risk factor for cerebral venous thrombosis? An old hypothesis revisited. J Neurol 2007;254(6):729–734. doi:https://doi.org/10.1007/s00415-006-0411-9.

    Article  Google Scholar 

  17. Cognat E, Crassard I, Denier C, Vahedi K, Bousser M. Cerebral venous thrombosis in inflammatory bowel diseases: eight cases and literature review. Int J Stroke. 2011;6(6):487–92. https://doi.org/10.1111/j.1747-4949.2011.00620.x.

    Article  PubMed  Google Scholar 

  18. Verberne HJ, Fliers E, Prummel MF, Stam J, Brandjes DP, Wiersinga WM. Thyrotoxicosis as a predisposing factor for cerebral venous thrombosis. Thyroid. 2000;10(7):607–10. https://doi.org/10.1089/thy.2000.10.607.

    Article  CAS  PubMed  Google Scholar 

  19. Saadoun D, Wechsler B, Resche-Rigon M, Trad S, Huong DL, Sbai A, … Piette JC. Cerebral venous thrombosis in Behçets disease. Arthritis Rheum 2009;61(4):518–526. doi:https://doi.org/10.1002/art.24393.

    Article  CAS  Google Scholar 

  20. Vidailhet M, Piette JC, Wechsler B, Bousser MG, Brunet P. Cerebral venous thrombosis in systemic lupus erythematosus. Stroke. 1990;21(8):1226–31. https://doi.org/10.1161/01.str.21.8.1226.

    Article  CAS  PubMed  Google Scholar 

  21. Aidi S, Chaunu M, Biousse V, Bousser M. Changing pattern of headache pointing to cerebral venous thrombosis after lumbar puncture and intravenous high-dose corticosteroids. Headache. 1999;39(8):559–64. https://doi.org/10.1046/j.1526-4610.1999.3908559.x.

    Article  CAS  PubMed  Google Scholar 

  22. Sinnaeve L, Vanopdenbosch L, Paemeleire K. Association of cerebral venous thrombosis and intracranial hypotension: review of 3 cases. J Stroke Cerebrovasc Dis. 2017;26(8):e165–9. https://doi.org/10.1016/j.jstrokecerebrovasdis.2017.05.015.

    Article  Google Scholar 

  23. Ueda K, Nakase H, Miyamoto K, Otsuka H, Sakaki T. Impact of anatomical difference of the cerebral venous system on microcirculation in a gerbil superior sagittal sinus occlusion model. Acta Neurochir. 2000;142(1):75–82. https://doi.org/10.1007/s007010050010.

    Article  CAS  PubMed  Google Scholar 

  24. Schaller B, Graf R. Cerebral venous infarction: the pathophysiological concept. Cerebrovasc Dis. 2004;18(3):179–88. https://doi.org/10.1159/000079939.

    Article  CAS  PubMed  Google Scholar 

  25. Leach J, Meyer K, Jones B, Tomsick T. Large arachnoid granulations involving the dorsal superior sagittal sinus: findings on MR imaging and MR venography. Am J Neuroradiol. 2008;29(7):1335–9. https://doi.org/10.3174/ajnr.a1093.

    Article  CAS  Google Scholar 

  26. Schaller B, Graf R, Sanada Y, Tolnay M, Rosner G, Wienhard K, et al. Hemodynamic changes after occlusion of the posterior superior sagittal sinus: an experimental PET study in cats. Am J Neuroradiol. 2003;24:1876–80.

    PubMed  Google Scholar 

  27. Mullins ME, Grant P, Wang B, Gonzalez R, Schaefer PW. Parenchymal abnormalities associated with cerebral venous sinus thrombosis: assessment with diffusion-weighted MR imaging. Am J Neuroradiol. 2004;25(10):1666–75.

    PubMed  Google Scholar 

  28. Ferro JM, Canhão P, Bousser M, Stam J, Barinagarrementeria F. Early seizures in cerebral vein and dural sinus thrombosis. Stroke. 2008;39(4):1152–8. https://doi.org/10.1161/strokeaha.107.487363.

    Article  PubMed  Google Scholar 

  29. Bousser MG, Russel RR. Cerebral venous thrombosis. London: Saunders; 1997. p. 15–7.

    Google Scholar 

  30. Villringer A, Mehraein S, Einhaupl KM. Pathophysiological aspects of cerebral sinus venous thrombosis (SVT). J Neuroradiol. 1994;21:72–80.

    CAS  PubMed  Google Scholar 

  31. Kate MP, Thomas B, Sylaja PN. Cerebral venous thrombosis in post-lumbar puncture intracranial hypotension: case report and review of literature. F1000Res. 2014;11(3):41.

    Article  Google Scholar 

  32. Caso, V., Agnelli, G., Paciaroni, M., (eds). Handbook on cerebral venous thrombosis. Front Neurol Neurosci. Basel, Karger. 2008; Vol 23, pp 89–95.

  33. •• Botta R, Donirpathi S, Yadav R, Kulkamim GB, Kumar VK, Nagaraja D. Headache patterns in cerebral venous sinus thrombosis. J Neurosci Rural Pract. 2017;8(1):72–7. Prospective cohort study assessing clinical characteristics of headaches associated with CVT. Adds support to the previous studies that report a large variability in CVT headaches. Many of the findings in this study were contrary to previous studies (see Wasay, M., Kojan, S., Dai, A.I., Bobustuc, G., Sheikh, Z. (2010) Headache in cerebral venous thrombosis: incidence, pattern and location in 200 consecutive patients. J Headache Pain, 11:137–139. doi: 10.1007/s10194-010-0186-3.) and highlight the need for larger studies into CVT headache characteristics.

    Google Scholar 

  34. •• Nascimento FA, Soria MG, Rizelio V, Kowacs PA. Cerebral venous thrombosis with migraine-like headache and the trigeminovascular system. Case Rep Neurol Med. 2016. https://doi.org/10.1155/2016/2059749 Although a case report, it is relevant in exploring the pathophysiology and treatment of CVT headaches, two aspects of this disease that are relatively unexplored. This study explains how CVT headaches could occur independently of raised intracranial pressure and be due to activation of the trigeminovascular system similarly to a migraine headache. The study also reports that treatment with dihydroergotamine was effective which could lead to more studies looking into treating CVT headaches similarly to migraine headaches.

    Article  Google Scholar 

  35. Agostoni E. Headache in cerebral venous thrombosis. Neurol Sci. 2004;25:206–S210. https://doi.org/10.1007/s10072-004-0287-3.

    Article  Google Scholar 

  36. Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarrementeria F, 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(3):664–70.

    Article  Google Scholar 

  37. Iurlaro S, Beghi E, Massetto N, Guccione A, Autunno M, Colombo B, et al. Cerebral vein and dural sinus thrombosis in Portugal: 1980-1998. Cerebrovasc Dis. 2001;11(3):177–82.

    Article  Google Scholar 

  38. de Bruijn SF, de Haan FJ, Stam J. Clinical features and prognostic factors of cerebral venous thrombosis in a prospective series of 59 patients. J Neurol Neurosurg Psychiatry. 2001;70:105–8.

    Article  Google Scholar 

  39. Cumurciuc R, Crassard I, Sarov M, Valade D, Bousser MG. Headache as the only neurological sign of cerebral venous thrombosis: a series of 17 cases. J Neurol Neurosurg Psychiatry. 2005;76(8):1084–7.

    Article  CAS  Google Scholar 

  40. Ferro JM, Canhao P, Stam J, Bousser MG, Barinagaarrementeria F. Cerebral vein and dural sinus thrombosis in elderly patients. Stroke. 2005;36(9):1927–32.

    Article  Google Scholar 

  41. Cumurciuc R, Crassard I, Sarov M, Valade D, Boussier M. Headache as the only neurological sign of cerebral venous thrombosis: a series of 17 cases. Neurol Neurosurg Psychiatry. 2005;76(8):1084–7. https://doi.org/10.1136/jnnp.2004.056275.

    Article  CAS  Google Scholar 

  42. Wasay M, Kojan S, Dai AI, Bobustuc G, Sheikh Z. Headache in cerebral venous thrombosis: incidence, pattern and location in 200 consecutive patients. J Headache Pain. 2010;11:137–9. https://doi.org/10.1007/s10194-010-0186-3.

    Article  PubMed  PubMed Central  Google Scholar 

  43. Alberti A, Venti M, Biagini S. Headache and cerebral vein and sinus thrombosis. Handbook on Cerebral Venous Thrombosis. Front Neurol Neurosci. Basel, Karger. 2008;23:89–95.

    PubMed  Google Scholar 

  44. Tanislav C, Siekmann R, Sieweke N, Allendörfer J, Pabst W, Kaps M, et al. Cerebral vein thrombosis: clinical manifestation and diagnosis. BMC Neurol. 2011;11:69. https://doi.org/10.1186/1471-2377-11-69.

    Article  PubMed  PubMed Central  Google Scholar 

  45. Lafitte F, Boukobza M, Guichard J, Hoeffel C, Reizine D, Ille O, … Merland J. MRI and MRA for diagnosis and follow-up of cerebral venous thrombosis (CVT). Clin Radiol 1997;52(9), 672–679. doi:https://doi.org/10.1016/s0009-9260(97)80030-x.

    Article  CAS  Google Scholar 

  46. Boukobza M, Crassard I, Bousser M, Chabriat H. MR imaging features of isolated cortical vein thrombosis: diagnosis and follow-up. Am J Neuroradiol. 2008;30(2):344–8. https://doi.org/10.3174/ajnr.a1332.

    Article  PubMed  Google Scholar 

  47. Roland T, Jacobs J, Rappaport A, Vanheste R, Wilms G, Demaerel P. Unenhanced brain CT is useful to decide on further imaging in suspected venous sinus thrombosis. Clin Radiol. 2010;65(1):34–9. https://doi.org/10.1016/j.crad.2009.09.008.

    Article  CAS  PubMed  Google Scholar 

  48. Virapongse C, Cazenave C, Quisling R, Sarwar M, Hunter S. The empty delta sign: frequency and significance in 76 cases of dural sinus thrombosis. Radiology. 1987;162(3):779–85. https://doi.org/10.1148/radiology.162.3.3809494.

    Article  CAS  PubMed  Google Scholar 

  49. Rizzo L, Crasto SG, Rudà R, Gallo G, Tola E, Garabello D, et al. Cerebral venous thrombosis: role of CT, MRI and MRA in the emergency setting. Radiol Med. 2010;115(2):313–25. https://doi.org/10.1007/s11547-010-0493-4.

    Article  CAS  PubMed  Google Scholar 

  50. Linn J, Ertl-Wagner B, Seelos KC, Strupp M, Reiser M, Brückmann H, et al. Diagnostic value of multidetector-row CT angiography in the evaluation of thrombosis of the cerebral venous sinuses. Am J Neuroradiol. 2007;28:946–52.

    CAS  PubMed  Google Scholar 

  51. Coutinho JM, Berg RV, Zuurbier SM, Vanbavel E, Troost D, Majoie CB, et al. Small juxtacortical hemorrhages in cerebral venous thrombosis. Ann Neurol. 2014;75(6):908–16. https://doi.org/10.1002/ana.24180.

    Article  PubMed  Google Scholar 

  52. Patel SI, Obeid H, Matti L, Ramakrishna H, Shamoun FE. Cerebral venous thrombosis current and newer anticoagulant treatment options. Neurologist. 2015;20(5):80–8. https://doi.org/10.1097/nrl.0000000000000049.

    Article  PubMed  Google Scholar 

  53. Hon SF, Li HL, Cheng PW. Use of direct thrombin inhibitor for treatment of cerebral venous thrombosis. J Stroke Cerebrovasc Dis. 2012;21(8):915.e11–5. https://doi.org/10.1016/j.jstrokecerebrovasdis.2012.02.004.

    Article  Google Scholar 

  54. Alons IM, Jellema K, Wermer MJ, Algra A. D-dimer for the exclusion of cerebral venous thrombosis: a meta-analysis of low risk patients with isolated headache. BMC Neurol. 2015;15(1):118. https://doi.org/10.1186/s12883-015-0389-y.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  55. Geisbüsch C, Richter D, Herweh C, Ringleb PA, Nagel S. Novel factor Xa inhibitor for the treatment of cerebral venous and sinus thrombosis. Stroke. 2014;45(8):2469–71. https://doi.org/10.1161/strokeaha.114.006167.

    Article  PubMed  Google Scholar 

  56. Demir R, Saritemur M, Ozel L, Ozdemir G, Emet M, Ulvi H. Red cell distribution width identifies cerebral venous sinus thrombosis in patients with headache. Clin Appl Thromb Hemost. 2013;21(4):354–8. https://doi.org/10.1177/1076029613505764.

    Article  PubMed  Google Scholar 

  57. Breteau G, Mounier-Vehier F, Godefroy O, Gauvrit JY, Mackowiak- Cordoliani MA, Girot M, et al. Cerebral venous thrombosis 3-year clinical outcome in 55 consecutive patients. J Neurol. 2003;250:29–35.

    Article  CAS  Google Scholar 

  58. Wu YW, Hamrick SE, Miller SP, Haward MF, Lai MC, Callen PW, et al. Intraventricular hemorrhage in term neonates caused by sinovenous thrombosis. Ann Neurol. 2003;54:123–6.

    Article  Google Scholar 

  59. Ferro JM, Canhao P. Cerebral venous sinus thrombosis: update on diagnosis and management. Stroke. 2014;16:523.

    Google Scholar 

  60. Saposnik G, Barinagarrementeria F, Brown RD, Bushnell CD, Cucchiara B, Cushman M, 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(4):1158–92.

    Article  Google Scholar 

  61. Ferro JM, Bousser MG, Canhão P, Coutinho JM, Crassard I, Dentali F, et al. European Stroke Organization guideline for the diagnosis and treatment of cerebral venous thrombosis – endorsed by the European Academy of Neurology. Eur J Neurol. 2017;24:1203–13.

    Article  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Neurology, Yale School of Medicine, New Haven, CT, USA

    Amit Mehta, Julius Danesh & Deena Kuruvilla

Authors
  1. Amit Mehta
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Julius Danesh
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Deena Kuruvilla
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Amit Mehta.

Ethics declarations

Conflict of Interest

Amit Mehta, Julius Danesh, and Deena Kuruvilla declare no conflict of interest.

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.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

This article is part of the Topical Collection on Uncommon and/or Unusual Headaches and Syndromes

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Mehta, A., Danesh, J. & Kuruvilla, D. Cerebral Venous Thrombosis Headache. Curr Pain Headache Rep 23, 47 (2019). https://doi.org/10.1007/s11916-019-0786-9

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11916-019-0786-9

Keywords

Search

Navigation