Skip to main content

Advertisement

SpringerLink
Log in

Reversible Cerebral Vasoconstriction Syndrome: A Review of Recent Research

  • Stroke (HP Adams Jr, Section Editor)
  • Published:
Current Neurology and Neuroscience Reports Aims and scope Submit manuscript

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a collective term used for transient noninflammatory, nonatherosclerotic segmental constriction of cerebral arteries. The angiopathies of RCVS have previously been defined by several nomenclatures. Current opinion favors the unification of these pathophysiologically related angiopathies because of their similar angiographic features and clinical course. RCVS typically presents acutely as headache, delirium, seizure, cerebral ischemia, and/or hemorrhage. The angiographic features make RCVS an important mimic of CNS vasculitides. In contrast to CNS vasculitis, RCVS is typically a transient condition with relatively good clinical outcomes. Although a complete understanding of the etiological and pathological features of RCVS has not yet been achieved, alterations in vascular tone lead to the observed arterial changes. In this review, we aim to provide a summary of RCVS and provide insight into current perspectives of the underlying pathophysiological processes, diagnosis, and treatment.

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

Fig. 1

Similar content being viewed by others

References

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

  1. Call GK, Fleming MC, Sealfon S, et al. Reversible cerebral segmental vasoconstriction. Stroke. 1988;19:1159–70.

    Article  PubMed  CAS  Google Scholar 

  2. Chen SP, Fuh JL, Lirng JF, et al. Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology. 2006;67:2164–9.

    Article  PubMed  Google Scholar 

  3. Ducros A, Boukobza M, Porcher R, et al. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain. 2007;130:3091–101.

    Article  PubMed  Google Scholar 

  4. •• Singhal AB, Hajj-Ali RA, Topcuoglu MA, et al. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol. 2011;68:1005–12. Describe the features of RCVS in patients requiring hospitalization. In addition to the clinical features of 139 cases, the authors discuss RCVS treatment and outcome.

  5. • Chen SP, Fuh JL, Wang SJ. Reversible cerebral vasoconstriction syndrome: current and future perspectives. Expert Rev Neurother. 2011;11:1265–76. Provide a comprehensive review of RCVS based on their experience with a large cohort of patients presenting to an outpatient headache center.

  6. 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;33:1403–11.

    Article  PubMed  CAS  Google Scholar 

  7. Williams TL, Lukovits TG, Harris BT, Harker Rhodes C. A fatal case of postpartum cerebral angiopathy with literature review. Arch Gynecol Obstet. 2007;275:67–77.

    Article  PubMed  Google Scholar 

  8. Thakur JD, Chittiboina P, Khan IS, Nanda A. Unique case of postpartum cerebral angiopathy requiring surgical intervention: case report and review of literature. Neurol India. 2011;59:891–4.

    Article  PubMed  Google Scholar 

  9. Levine RJ, Maynard SE, Qian C, et al. Circulating angiogenic factors and the risk of preeclampsia. N Engl J Med. 2004;350:672–83.

    Article  PubMed  CAS  Google Scholar 

  10. Levine RJ, Lam C, Qian C, et al. Soluble endoglin and other circulating antiangiogenic factors in preeclampsia. N Engl J Med. 2006;355:992–1005.

    Article  PubMed  CAS  Google Scholar 

  11. McKinney JS, Messe SR, Pukenas BA, et al. Intracranial vertebrobasilar artery dissection associated with postpartum angiopathy. Stroke Res Treat. 2010;2010:320627.

    PubMed  Google Scholar 

  12. Macgregor EA, Rosenberg JD, Kurth T. Sex-related differences in epidemiological and clinic-based headache studies. Headache. 2011;51:843–59.

    Article  PubMed  Google Scholar 

  13. Chen SP, Fuh JL, Wang SJ, et al. Brain-derived neurotrophic factor gene Val66Met polymorphism modulates reversible cerebral vasoconstriction syndromes. PLoS One. 2011;6:e18024.

    Article  PubMed  CAS  Google Scholar 

  14. Palma JA, Fontes-Villalba A, Irimia P, et al. Reversible cerebral vasoconstriction syndrome induced by adrenaline. Cephalalgia. 2012;32:500–4.

    Article  PubMed  Google Scholar 

  15. Schellinger PD, Bryan RN, Caplan LR, et al. Evidence-based guideline: the role of diffusion and perfusion MRI for the diagnosis of acute ischemic stroke: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2010;75:177–85.

    Article  PubMed  CAS  Google Scholar 

  16. Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146:34–44.

    PubMed  Google Scholar 

  17. Lu SR, Liao YC, Fuh JL, et al. Nimodipine for treatment of primary thunderclap headache. Neurology. 2004;62:1414–6.

    Article  PubMed  CAS  Google Scholar 

  18. Ducros A, Fiedler U, Porcher R, et al. Hemorrhagic manifestations of reversible cerebral vasoconstriction syndrome: frequency, features, and risk factors. Stroke. 2010;41:2505–11.

    Article  PubMed  Google Scholar 

  19. Elstner M, Linn J, Muller-Schunk S, Straube A. Reversible cerebral vasoconstriction syndrome: a complicated clinical course treated with intra-arterial application of nimodipine. Cephalalgia. 2009;29:677–82.

    Article  PubMed  CAS  Google Scholar 

  20. Theeler BJ, Krasnokutsky MV, Scott BR. Exertional reversible cerebral vasoconstriction responsive to verapamil. Neurol Sci. 2010;31:773–5.

    Article  PubMed  Google Scholar 

  21. Chik Y, Hoesch RE, Lazaridis C, et al. A case of postpartum cerebral angiopathy with subarachnoid hemorrhage. Nat Rev Neurol. 2009;5:512–6.

    Article  PubMed  Google Scholar 

  22. Grande PO, Lundgren A, Bjartmarz H, Cronqvist M. Segmental cerebral vasoconstriction: successful treatment of secondary cerebral ischaemia with intravenous prostacyclin. Cephalalgia. 2010;30:890–5.

    Article  PubMed  Google Scholar 

  23. Farid H, Tatum JK, Wong C, et al. Reversible cerebral vasoconstriction syndrome: treatment with combined intra-arterial verapamil infusion and intracranial angioplasty. AJNR Am J Neuroradiol. 2011;32:E184–187.

    Article  PubMed  CAS  Google Scholar 

  24. Bouchard M, Verreault S, Gariepy JL, Dupre N. Intra-arterial milrinone for reversible cerebral vasoconstriction syndrome. Headache. 2009;49:142–5.

    Article  PubMed  Google Scholar 

  25. Takis C, Kwan ES, Pessin MS, et al. Intracranial angioplasty: experience and complications. AJNR Am J Neuroradiol. 1997;18:1661–8.

    PubMed  CAS  Google Scholar 

  26. Singhal AB, Kimberly WT, Schaefer PW, Hedley-Whyte ET. Case records of the Massachusetts General Hospital. Case 8-2009. A 36-year-old woman with headache, hypertension, and seizure 2 weeks post partum. N Engl J Med. 2009;360:1126–37.

    Article  PubMed  CAS  Google Scholar 

Download references

Disclosure

No potential conflicts of interest relevant to this article were reported.

Author information

Authors and Affiliations

  1. Department of Neurology, Robert Wood Johnson Medical School, University of Medicine and Dentistry of New Jersey, 125 Paterson St, Suite 6200, New Brunswick, NJ, 08901, USA

    Arnaldo Velez & James S. McKinney

Authors
  1. Arnaldo Velez
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. James S. McKinney
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to James S. McKinney.

Additional information

This article is part of the Topical Collection on Stroke

Rights and permissions

Reprints and permissions

About this article

Cite this article

Velez, A., McKinney, J.S. Reversible Cerebral Vasoconstriction Syndrome: A Review of Recent Research. Curr Neurol Neurosci Rep 13, 319 (2013). https://doi.org/10.1007/s11910-012-0319-y

Download citation

  • Published:

  • DOI: https://doi.org/10.1007/s11910-012-0319-y

Keywords

Search

Navigation