Reversible Cerebral Vasoconstriction Syndromes: What the Cardiologist Should Know

Cerebrovascular Disease and Stroke (D Greer, Section Editor)
First Online:
Part of the following topical collections:
  1. Topical Collection on Cerebrovascular Disease and Stroke

Opinion statement

Over the past decade, the reversible cerebral vasoconstriction syndromes (RCVS) have emerged as a group of conditions with easily recognizable clinical-angiographic features and a usually benign prognosis. The RCVS affect young individuals, mostly women, and the majority present with recurrent, severe, ‘thunderclap’ headaches. Vascular imaging studies show dynamic and reversible narrowing and dilatation of multiple intracerebral arteries. Brain imaging usually shows no parenchymal lesions, however, approximately one-third of patients develop ischemic or hemorrhagic strokes or reversible brain edema. The etiopathogenesis of this syndrome remains unclear. It has been associated with diverse conditions such as pregnancy, vasoconstrictive drug use, and neurovascular procedures. Recent studies characterizing RCVS have made it relatively easy to exclude mimics such as aneurysmal subarachnoid hemorrhage and primary angiitis of the central nervous system. There is no proven treatment, although calcium channel blockers may help to reduce the intensity of headaches. Empiric glucocorticoid treatment should be avoided. Since most patients do well with simple observation alone, invasive strategies such as pharmacologically-induced hypertension, balloon angioplasty, and direct intra-arterial vasodilator infusion should be reserved for patients showing clear clinical progression.

Keywords

Vasoconstriction Vasospasm Vasculitis Ischemic stroke Hemorrhagic Stroke Subarachnoid hemorrhage Cerebral edema Thunderclap headache Migraine Illicit drugs Pregnancy Postpartum Cerebral angiography Calcium-channel blockers 
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Notes

Compliance with Ethics Guidelines

Conflict of interest

Dr. Aneesh Singhal has given expert testimonial for Mediolegal Firms and received honoraria from American Academy of Neurology

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.

References and Recommended Reading

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

  1. 1.
    Armstrong FS, Hayes GJ. Segmental cerebral arterial constriction associated with pheochromocytoma. J Neurosurg. 1961;18:843–6.PubMedCrossRefGoogle Scholar
  2. 2.
    Buckle RM, Smith B. Death due to cerebral vasospasm. J Neurol Neurosurg Psychiatry. 1964;27:440–4.PubMedCrossRefGoogle Scholar
  3. 3.
    Rascol AGB, Manelfe C, Clanet M. Accidents vasculaires cerebraux de la grossesse et du post partum. In: II Conference de la Salpetriere sue les maladies vasculaires cerebrales: 1979; Hospital de la Salpetriere, Paris; JB Balliere. 1979;84–127.Google Scholar
  4. 4.
    Serdaru M, Chiras J, Cujas M, Lhermitte F. Isolated benign cerebral vasculitis or migrainous vasospasm? J Neurol Neurosurg Psychiatry. 1984;47(1):73–6.PubMedCrossRefGoogle Scholar
  5. 5.
    Jackson M, Lennox G, Jaspan T, Jefferson D. Migraine angiitis precipitated by sex headache and leading to watershed infarction. Cephalalgia. 1993;13(6):427–30.PubMedCrossRefGoogle Scholar
  6. 6.
    Bogousslavsky J, Despland PA, Regli F, Dubuis PY. Postpartum cerebral angiopathy: reversible vasoconstriction assessed by transcranial Doppler ultrasounds. Eur Neurol. 1989;29(2):102–5.PubMedCrossRefGoogle Scholar
  7. 7.
    Kaye BR, Fainstat M. Cerebral vasculitis associated with cocaine abuse. JAMA. 1987;258(15):2104–6.PubMedCrossRefGoogle Scholar
  8. 8.
    Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM. Reversible cerebral segmental vasoconstriction. Stroke. 1988;19(9):1159–70.PubMedCrossRefGoogle Scholar
  9. 9.
    Singhal AB, Koroshetz WJ, Caplan LR. Cerebral Vasoconstriction Syndromes. In: Bogousslavsky J, Caplan LR, editor. Uncommon Causes of Stroke. Cambridge: Cambridge University Press; 2001. p. 114–23.Google Scholar
  10. 10.
    Singhal AB, Bernstein RA. Postpartum angiopathy and other cerebral vasoconstriction syndromes. Neurocrit Care. 2005;3(1):91–7.PubMedCrossRefGoogle Scholar
  11. 11.••
    Calabrese LH, Dodick DW, Schwedt TJ, Singhal AB. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med. 2007;146(1):34–44. Comprehensive review article with a discussion of differential diagnosis and the major characteristic features for diagnosing RCVS.PubMedCrossRefGoogle Scholar
  12. 12.
    Ducros A, Boukobza M, Porcher R, Sarov M, Valade D, Bousser MGT. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain. 2007;130(Pt 12):3091–101.PubMedCrossRefGoogle Scholar
  13. 13.
    Chen SP, Fuh JL, Chang FC, Lirng JF, Shia BC, Wang SJ. Transcranial color doppler study for reversible cerebral vasoconstriction syndromes. Ann Neurol. 2008;63(6):751–7.PubMedCrossRefGoogle Scholar
  14. 14.••
    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. Detailed description of RCVS-associated lobar and subarachnoid hemorrhage.PubMedCrossRefGoogle Scholar
  15. 15.••
    Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11(10):906–17. Comprehensive review article.PubMedCrossRefGoogle Scholar
  16. 16.••
    Singhal AB, Hajj-Ali RA, Topcuoglu MA, et al. Reversible cerebral vasoconstriction syndromes: analysis of 139 cases. Arch Neurol. 2011;68(8):1005–12. Retrospective 2-center study of RCVS patients encountered in both the inpatient and outpatient settings.PubMedCrossRefGoogle Scholar
  17. 17.
    Hajj-Ali RA, Furlan A, Abou-Chebel A, Calabrese LH. Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum. 2002;47(6):662–9.PubMedCrossRefGoogle Scholar
  18. 18.
    Kirton A, Diggle J, Hu W, Wirrell E. A pediatric case of reversible segmental cerebral vasoconstriction. Can J Neurol Sci. 2006;33(2):250–3.PubMedGoogle Scholar
  19. 19.
    Liu HY, Fuh JL, Lirng JF, Chen SP, Wang SJ. Three paediatric patients with reversible cerebral vasoconstriction syndromes. Cephalalgia. 2010;30(3):354-9. doi: 10.1111/j.1468-2982.2009.01955.x.Google Scholar
  20. 20.
    Singhal AB. Cerebral vasoconstriction syndromes. Top Stroke Rehabil. 2004;11(2):1–6.PubMedCrossRefGoogle Scholar
  21. 21.
    Singhal AB, Caviness VS, Begleiter AF, Mark EJ, Rordorf G, Koroshetz WJ. Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology. 2002;58(1):130–3.PubMedCrossRefGoogle Scholar
  22. 22.
    Walsh RD, Floyd JP, Eidelman BH, Barrett KM. Balint syndrome and visual allochiria in a patient with reversible cerebral vasoconstriction syndrome. J Neuroophthalmol. 2012;32(4):302–6.PubMedCrossRefGoogle Scholar
  23. 23.
    Singhal AB. Thunderclap headache, reversible cerebral arterial vasoconstriction, and unruptured aneurysms. J Neurol Neurosurg Psychiatry. 2002;73(1):96. author reply 96–7.PubMedCrossRefGoogle Scholar
  24. 24.
    Williams TL, Lukovits TG, Harris BT, Harker RC. A fatal case of postpartum cerebral angiopathy with literature review. Arch Gynecol Obstet. 2007;275(1):67–77.PubMedCrossRefGoogle Scholar
  25. 25.••
    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 postpartum. N Engl J Med. 2009;360(11):1126–37. Instructive clinical-pathological conference of a patient with fatal outcome; highlights the risks of intra-arterial vasodilator approaches to treat RCVS.PubMedCrossRefGoogle Scholar
  26. 26.
    Iancu-Gontard D, Oppenheim C, Touze E, et al. Evaluation of hyperintense vessels on FLAIR MRI for the diagnosis of multiple intracerebral arterial stenoses. Stroke. 2003;34(8):1886–91.PubMedCrossRefGoogle Scholar
  27. 27.•
    Mawet J, Boukobza M, Franc J, et al. Reversible cerebral vasoconstriction syndrome and cervical artery dissection in 20 patients. Neurology. 2013;81(9):821–4. This article highlights the frequent occurrence of cerebral artery dissection in RCVS.PubMedCrossRefGoogle Scholar
  28. 28.
    Bayer-Karpinska A, Patzig M, Adamczyk C, et al. Reversible cerebral vasoconstriction syndrome with concurrent bilateral carotid artery dissection. Cephalalgia. 2013;33(7):491–5.PubMedCrossRefGoogle Scholar
  29. 29.
    Mitchell LA, Santarelli JG, Singh IP, Do HM. Reversible cerebral vasoconstriction syndrome and bilateral vertebral artery dissection presenting in a patient after cesarean section. BMJ Case Reports. 2013. doi: 10.1136/bcr-2012-010521.
  30. 30.
    Singhal AB. Postpartum angiopathy with reversible posterior leukoencephalopathy. Arch Neurol. 2004;61(3):411–6.PubMedCrossRefGoogle Scholar
  31. 31.••
    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;70(10):1254-1260 Comparision of subarachnoid hemorrhage resulting from RCVS, aneurysmal rupture, and cryptogenic causes.Google Scholar
  32. 32.
    Rosenbloom MH, Singhal AB. CT angiography and diffusion-perfusion MR imaging in a patient with ipsilateral reversible cerebral vasoconstriction after carotid endarterectomy. Am J Neuroradiol. 2007;28(5):920–2.PubMedGoogle Scholar
  33. 33.
    Agostoni E, Rigamonti A, Aliprandi A. Thunderclap headache and benign angiopathy of the central nervous system: a common pathogenetic basis. Neurol Sci. 2011;32 Suppl 1:S55–9.PubMedCrossRefGoogle Scholar
  34. 34.
    Hammad TA, Hajj-Ali RA. Primary angiitis of the central nervous system and reversible cerebral vasoconstriction syndrome. Curr Atheroscler Rep. 2013;15(8):346.PubMedCrossRefGoogle Scholar
  35. 35.
    Schwedt TJ, Matharu MS, Dodick DW. Thunderclap headache. Lancet Neurol. 2006;5(7):621–31.PubMedCrossRefGoogle Scholar
  36. 36.
    Bettoni L, Juvarra G, Bortone E, Lechi A. Isolated benign cerebral vasculitis. Case report and review Acta Neurol Belg. 1984;84(4):161–73.Google Scholar
  37. 37.
    Snyder BD, McClelland RR. Isolated benign cerebral vasculitis. Arch Neurol. 1978;35(9):612–4.PubMedCrossRefGoogle Scholar
  38. 38.
    van Calenbergh F, van den Bergh V, Wilms G. Benign isolated arteritis of the central nervous system. Clin Neurol Neurosurg. 1986;88(4):267–73.PubMedCrossRefGoogle Scholar
  39. 39.
    Calado S, Vale-Santos J, Lima C, Viana-Baptista M. Postpartum cerebral angiopathy: vasospasm, vasculitis, or both? Cerebrovasc Dis. 2004;18(4):340–1.PubMedCrossRefGoogle Scholar
  40. 40.
    Mandell DM, Matouk CC, Farb RI, 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
  41. 41.
    Muehlschlegel S, Rordorf G, Bodock M, Sims JR. Dantrolene mediates vasorelaxation in cerebral vasoconstriction: a case series. Neurocrit Care. 2009;10(1):116–21.PubMedCentralPubMedCrossRefGoogle Scholar
  42. 42.
    Ringer AJ, Qureshi AI, Kim SH, Fessler RD, Guterman LR, Hopkins LN. Angioplasty for cerebral vasospasm from eclampsia. Surg Neurol. 2001;56(6):373–8. discussion 378–9.PubMedCrossRefGoogle Scholar
  43. 43.
    Klein M, Fesl G, Pfister HW, et al. Intra-arterial nimodipine in progressive postpartum cerebral angiopathy. Cephalalgia. 2009;29(2):279–82.PubMedCrossRefGoogle Scholar
  44. 44.
    Linn J, Fesl G, Ottomeyer C, Straube A, Dichgans M, Bruckmann H, Pfefferkorn T. Intra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: a diagnostic tool in select cases? Cephalalgia. 2011;31(10):1074-81. doi: 10.1177/0333102410394673. Epub 2011 Jan 10.
  45. 45.
    Ioannidis I, Nasis N, Agianniotaki A, Katsouda E, Andreou A. Reversible cerebral vasoconstriction syndrome: treatment with multiple sessions of intra-arterial nimodipine and angioplasty. Interv Neuroradiol. 2012;18(3):297–302.PubMedCentralPubMedGoogle Scholar
  46. 46.
    French KF, Hoesch RE, Allred J, et al. Repetitive use of intra-arterial verapamil in the treatment of reversible cerebral vasoconstriction syndrome. J Clin Neurosci. 2012;19(1):174–6.PubMedCrossRefGoogle Scholar
  47. 47.
    Song JK, Fisher S, Seifert TD, et al. Postpartum cerebral angiopathy: atypical features and treatment with intracranial balloon angioplasty. Neuroradiology. 2004;46(12):1022–6.PubMedCrossRefGoogle Scholar
  48. 48.
    Singhal AB. Diagnostic challenges in RCVS, PACNS, and other cerebral arteriopathies. Cephalalgia. 2011;31(10):1067-70. doi: 10.1177/0333102411410084.Google Scholar
  49. 49.•
    Fugate JE, Wijdicks EF, Parisi JE, et al. Fulminant postpartum cerebral vasoconstriction syndrome. Arch Neurol. 2012;69(1):111–7. This article suggests that poor outcome may be more frequent when RCVS occurrs in the postpartum setting.PubMedCrossRefGoogle Scholar
  50. 50.
    Fugate JE, Ameriso SF, Ortiz G, et al. Variable presentations of postpartum angiopathy. Stroke. 2012;43(3):670–6.PubMedCrossRefGoogle Scholar
  51. 51.
    Ursell MR, Marras CL, Farb R, Rowed DW, Black SE, Perry JR. Recurrent intracranial hemorrhage due to postpartum cerebral angiopathy: implications for management. Stroke. 1998;29(9):1995–8.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  1. 1.Harvard Medical School, Department of Neurology, Stroke ServiceMassachusetts General Hospital ACC-729CBostonUSA

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