Reversible Cerebral Vasoconstriction Syndrome: Updates and New Perspectives

  • Huma U. Sheikh
  • Paul G. Mathew
Uncommon Headache Syndromes (J Ailani, Section Editor)
Part of the following topical collections:
  1. Topical Collection on Uncommon Headache Syndromes


Reversible cerebral vasoconstriction syndrome (RCVS) is an important cause of headaches that can lead to other neurological complications, including stroke, if not recognized early. Over the past few years, there has been great progress in the recognition of this entity. However, there is still much to be learned about its pathophysiology and optimal treatment strategies. RCVS occurs mostly in middle-aged adults, and there is a female preponderance with an increased incidence during the postpartum period. A consistent, predominating feature is a sudden-onset, severe headache that is frequently recurrent, usually over the span of a week. Less common presentations include seizures or focal neurological symptoms. Important causative factors include vasoactive medications, as well as illicit drugs like marijuana and cocaine. The current underlying pathophysiology is thought to be a disturbance in cerebrovascular tone leading to vasoconstriction. The diagnosis is based on history, physical examination, and cerebrovascular imaging findings that demonstrate multifocal, segmental areas of vasoconstriction in large- and medium-sized arteries. An important criterion for making the diagnosis is the eventual reversibility of symptoms and imaging findings.


Reversible cerebral vasoconstriction syndrome Thunderclap headache Pathophysiology Vasoconstriction Cerebrovascular imaging 


Compliance with Ethics Guidelines

Conflict of Interest

Dr. Huma U. Sheikh and Dr. Paul G. Mathew each declare no potential conflicts of interest relevant to this article.

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.


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

  1. 1.
    •• Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM. Reversible cerebral segmental vasoconstriction. Stroke. 1988;19(9):1159–70. This was the first known case series of RCSV.PubMedCrossRefGoogle Scholar
  2. 2.
    Snyder BD, McClelland RR. Isolated benign cerebral vasculitis. Arch Neurol. 1978;35(9):612–4.PubMedCrossRefGoogle Scholar
  3. 3.
    Garcin B, Clouston J, Saines N. Reversible cerebral vasoconstriction syndrome. J Clin Neurosci. 2009;16(1):147–50.PubMedCrossRefGoogle Scholar
  4. 4.
    Velez A, McKinney JS. Reversible cerebral vasoconstriction syndrome: a review of recent research. Curr Neurol Neurosci Rep. 2013;13(1):319-012-0319-y.Google Scholar
  5. 5.
    Yancy H, Lee-Iannotti JK, Schwedt TJ, Dodick DW. Reversible cerebral vasoconstriction syndrome. Headache. 2013;53(3):570–6.PubMedCrossRefGoogle Scholar
  6. 6.
    Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146(1):34–44.PubMedCrossRefGoogle Scholar
  7. 7.
    Sattar A, Manousakis G, Jensen MB. Systematic review of reversible cerebral vasoconstriction syndrome. Expert Rev Cardiovasc Ther. 2010;8(10):1417–21.PubMedCentralPubMedCrossRefGoogle Scholar
  8. 8.
    Robert T, Kawkabani Marchini A, Oumarou G, Uske A. Reversible cerebral vasoconstriction syndrome identification of prognostic factors. Clin Neurol Neurosurg. 2013;115:2351–7.PubMedCrossRefGoogle Scholar
  9. 9.
    •• Ducros A, Boukobza M, Porcher R, Sarov M, Valade D, Bousser MG. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain. 2007;130(Pt 12):3091–101. Comprehensive Review.PubMedCrossRefGoogle Scholar
  10. 10.
    •• Chen SP, Fuh JL, Wang SJ. Reversible cerebral vasoconstriction syndrome: an under-recognized clinical emergency. Ther Adv Neurol Disord. 2010;3(3):161–71. Comprehensive Review.PubMedCentralPubMedCrossRefGoogle Scholar
  11. 11.
    Singhal AB, Hajj-Ali RA, Topcuoglu MA, Fok J, Bena J, Yang D, et al. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol. 2011;68(8):1005–12.PubMedCrossRefGoogle Scholar
  12. 12.
    Moustafa RR, Allen CM, Baron JC. Call-Fleming syndrome associated with subarachnoid haemorrhage: three new cases. BMJ Case Rep. 2009. doi: 10.1136/bcr.09.2008.0989.Google Scholar
  13. 13.
    Chen SP, Chung YT, Liu TY, Wang YF, Fuh JL, Wang SJ. Oxidative stress and increased formation of vasoconstricting F2-isoprostanes in patients with reversible cerebral vasoconstriction syndrome. Free Radic Biol Med. 2013;61C:243–8.PubMedCrossRefGoogle Scholar
  14. 14.
    •• Chen SP, Fuh JL, Wang SJ. Reversible cerebral vasoconstriction syndrome: current and future perspectives. Expert Rev Neurother. 2011;11(9):1265–76. Important for review of recent literature.PubMedCrossRefGoogle Scholar
  15. 15.
    Ducros A, Fiedler U, Porcher R, Boukobza M, Stapf C, Bousser MG. Hemorrhagic manifestations of reversible cerebral vasoconstriction syndrome: frequency, features, and risk factors. Stroke. 2010;41(11):2505–11.PubMedCrossRefGoogle Scholar
  16. 16.
    Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11(10):906–17.PubMedCrossRefGoogle Scholar
  17. 17.
    Davies G, Wilson H, Wilhelm T, Bowler J. The reversible cerebral vasoconstriction syndrome in association with venlafaxine and methenamine. BMJ Case Rep. 2013. doi: 10.1136/bcr-2013-009701.Google Scholar
  18. 18.
    Matano F, Murai Y, Adachi K, Koketsu K, Kitamura T, Teramoto A, et al. Reversible cerebral vasoconstriction syndrome associated with subarachnoid hemorrhage triggered by hydroxyzine pamoate. Clin Neurol Neurosurg. 2013;115(10):2189–91.PubMedCrossRefGoogle Scholar
  19. 19.
    Chen SP, Fuh JL, Wang SJ, Tsai SJ, Hong CJ, Yang AC. Brain-derived neurotrophic factor gene Val66Met polymorphism modulates reversible cerebral vasoconstriction syndromes. PLoS One. 2011;6(3):e18024.PubMedCentralPubMedCrossRefGoogle Scholar
  20. 20.
    Marder CP, Donohue MM, Weinstein JR, Fink KR. Multimodal imaging of reversible cerebral vasoconstriction syndrome: a series of 6 cases. AJNR Am J Neuroradiol. 2012;33(7):1403–10.PubMedCrossRefGoogle Scholar
  21. 21.
    Villablanca JP, Rodriguez FJ, Stockman T, Dahliwal S, Omura M, Hazany S, et al. MDCT angiography for detection and quantification of small intracranial arteries: comparison with conventional catheter angiography. AJR Am J Roentgenol. 2007;188(2):593–602.PubMedCrossRefGoogle Scholar
  22. 22.
    Muehlschlegel S, Kursun O, Topcuoglu MA, Fok J, Singhal AB. Differentiating reversible cerebral vasoconstriction syndrome with subarachnoid hemorrhage from other causes of subarachnoid hemorrhage. JAMA Neurol. 2013.Google Scholar
  23. 23.
    Hammad TA, Hajj-Ali RA. Primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome. Curr Atheroscler Rep. 2013;15(8):346-013-0346-4.Google Scholar
  24. 24.
    Ducros A. L37. Reversible cerebral vasoconstriction syndrome: distinction from CNS vasculitis. Presse Med. 2013;42(4 Pt 2):602–4.PubMedCrossRefGoogle Scholar
  25. 25.
    Mandell DM, Matouk CC, Farb RI, Krings T, Agid R, terBrugge K, et al. Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: preliminary results. Stroke. 2012;43(3):860–2.PubMedCrossRefGoogle Scholar
  26. 26.
    Bartynski WS, Boardman JF. Catheter angiography, MR angiography, and MR perfusion in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol. 2008;29(3):447–55.PubMedCrossRefGoogle Scholar
  27. 27.
    Levin M. The International Classification of Headache Disorders, 3rd edition (ICHD III)—changes and challenges. Headache. 2013;53(8):1383–95.CrossRefGoogle Scholar
  28. 28.
    Olesen J. ICHD-3 beta is published. Use it immediately. Cephalalgia. 2013;33(9):627–8.PubMedCrossRefGoogle Scholar
  29. 29.
    Takis C, Kwan ES, Pessin MS, Jacobs DH, Caplan LR. Intracranial angioplasty: experience and complications. AJNR Am J Neuroradiol. 1997;18(9):1661–8.PubMedGoogle Scholar
  30. 30.
    Fugate JE, Ameriso SF, Ortiz G, Schottlaender LV, Wijdicks EF, Flemming KD, et al. Variable presentations of postpartum angiopathy. Stroke. 2012;43(3):670–6.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Brigham and Women’s Hospital, Department of Neurology, John R. Graham Headache CenterHarvard Medical SchoolBostonUSA
  2. 2.Cambridge Health Alliance, Division of NeurologyHarvard Medical SchoolCambridgeUSA

Personalised recommendations