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Reversible cerebral vasoconstriction syndrome: A thunderclap headache-associated condition

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Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by a sudden, severe headache at onset, vascular narrowing involving the circle of Willis and its immediate branches, and angiographic evidence of vasoconstriction reversibility within minutes to weeks of onset. RCVS is underrecognized and often misdiagnosed; it can defy clinical detection because it can mimic common conditions such as migraine and ischemic stroke. A lack of shared nosology has hampered awareness and understanding of the syndrome. Clinicians must consider primary angiitis of the central nervous system because of its high rates of morbidity and mortality if left untreated. RCVS has a number of primary and secondary associations (cerebral hemorrhage, vasoactive substances, the peripartum period, bathing, and physical exertion) but also occurs in isolation. RCVS can present in conjunction with hypertensive encephalopathy, preeclampsia, and reversible posterior leukoencephalopathy. This review provides an up-to-date account of RCVS.

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References and Recommended Reading

  1. Call GK, Fleming MC, Sealfon S, et al.: Reversible cerebral segmental vasoconstriction. Stroke 1988, 19:1159–1170.

    PubMed  CAS  Google Scholar 

  2. Nowak DA, Rodiek SO, Henneken S, et al.: Reversible segmental cerebral vasoconstriction (Call-Fleming syndrome): are calcium channel inhibitors a potential treatment option? Cephalalgia 2003, 23:218–222.

    Article  PubMed  CAS  Google Scholar 

  3. Younger DS, Calabrese LH, Hays AP: Granulomatous angiitis of the nervous system. Neurol Clin 1997, 15:821–834.

    Article  PubMed  CAS  Google Scholar 

  4. Slivka A, Philbrook B: Clinical and angiographic features of thunderclap headache. Headache 1995, 35:1–6.

    Article  PubMed  CAS  Google Scholar 

  5. Al-Sous W, Bohlega S, Al-Kawi Z, et al.: Post-partum cerebral angiopathy. A rare cerebrovascular complication. Eur J Neurology 1998, 5:411–416.

    Article  Google Scholar 

  6. Hajj-Ali RA, Furlan A, Abou-Chebel A, Calabrese LH: Benign angiopathy of the CNS: cohort of 16 patients with clinical course and long-term follow-up. Arthritis Rheum 2002, 47:662–669.

    Article  PubMed  Google Scholar 

  7. Snyder BD, McClelland RR: Isolated benign cerebral vasculitis. Arch Neurol 1978, 35:612–614.

    PubMed  CAS  Google Scholar 

  8. Solomon S, Lipton RB, Harris PY: Arterial stenosis in migraine spasm or arteriopathy? Headache 1990, 30:52–61.

    Article  PubMed  CAS  Google Scholar 

  9. Schwedt TJ, Matharu MS, Doddick DW: Thunderclap headache. Lancet Neurol 2006, 5:621–631.

    Article  PubMed  Google Scholar 

  10. Gerretsen P, Kern RZ: Reversible cerebral vasoconstriction syndrome or primary angiitis of the central nervous system? Can J Neurol Sci 2007, 34:467–477.

    PubMed  Google Scholar 

  11. Chen SP, Fuh JL, Lirng JF, et al.: Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology 2006, 67:2164–2169.

    Article  PubMed  Google Scholar 

  12. Chen SP, Fuh JL, Chang FC, et al.: Transcranial color Doppler study for reversible cerebral vasoconstriction syndromes. Ann Neurol 2008, 63:751–757.

    Article  PubMed  Google Scholar 

  13. 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 

  14. Iu PP, Lam HS: Migrainous spasm simulating carotid dissection: a pitfall in MR arteriographic findings. AJNR Am J Neuroradiol 2001, 22:1550–1552.

    PubMed  CAS  Google Scholar 

  15. Singhal AB: Posterior angiopathy with reversible posterior leukoencephalopathy. Arch Neurol 2004, 61:411–416.

    Article  PubMed  Google Scholar 

  16. Noskin O, Jafarimojarrad E, Libman RB, Nelson JL: Diffuse cerebral vasoconstriction (Call-Fleming syndrome) and stroke associated with antidepressants. Neurology 2006, 67:159–160.

    Article  PubMed  Google Scholar 

  17. Dodick DW: Thunderclap headache. J Neurol Neurosurg Psychiatry 2002, 72:6–11.

    Article  PubMed  CAS  Google Scholar 

  18. Hinchey J, Chaves C, Appignani B, et al.: A reversible posterior leukoencephalopathy syndrome. N Engl J Med 1996, 334:494–500.

    Article  PubMed  CAS  Google Scholar 

  19. Heo K, Park SA, Lee JY, et al.: Post-transfusion posterior leukoencephalopathy with cytotoxic and vasogenic edema precipitated by vasospasm. Cerebrovasc Dis 2005, 15:230–233.

    Article  Google Scholar 

  20. Obeid T, Shami A, Karsou S: The role of seizures in reversible posterior leukoencephalopathy. Seizure 2004, 13:277–281.

    Article  PubMed  CAS  Google Scholar 

  21. Tajima Y, Isonishi K, Kashiwaba T, Tashiro K: Two similar cases of encephalopathy, possibly a reversible posterior leukoencephalopathy syndrome: serial findings of magnetic resonance imaging, SPECT and angiography. Intern Med 1999, 38:54–58.

    Article  PubMed  CAS  Google Scholar 

  22. Chen SP, Fuh JL, Lirng JF, Wang SJ: Is vasospasm requisite for posterior leukoencephalopathy in patients with primary thunderclap headaches? Cephalalgia 2005, 26:530–536.

    Article  Google Scholar 

  23. Dodick DW, Eross EJ, Drazkowski JF, Ingall TJ: Thunderclap headache associated with reversible vasospasm and posterior leukoencephalopathy syndrome. Cephalalgia 2003, 2233:994–997.

    Article  Google Scholar 

  24. Chen TH, Huang CC, Chang YY, et al.: Vasoconstriction as the etiology of hypercalcemia-induced seizures. Epilepsia 2004, 45:551–554.

    Article  PubMed  Google Scholar 

  25. Dodick DW: Thunderclap headache. Headache 2002, 42:309–315.

    Article  PubMed  Google Scholar 

  26. Headache Classification Subcommittee of the International Headache Society: The international classification of headache disorders: 2nd edition. Cephalalgia 2004, 24(Suppl 1):9–160.

    Google Scholar 

  27. Dodick DW, Brown RD Jr, Britton JW, Huston J 3rd: Nonaneurysmal thunderclap headache with diffuse, multifocal, segmental and reversible vasospasm. Cephalalgia 1999, 19:118–123.

    Article  PubMed  CAS  Google Scholar 

  28. Sturm JW, Macdonnell RAL: Recurrent thunderclap headache associated with reversible intracerebral vasospasm causing stroke. Cephalalgia 2000, 20:132–135.

    Article  PubMed  CAS  Google Scholar 

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

    PubMed  Google Scholar 

  30. Valença MM, Valença LP, Bordini CA, et al.: Cerebral vasospasm and headache during sexual intercourse and masturbatory orgasms. Headache 2004, 44:244–248.

    Article  PubMed  Google Scholar 

  31. Schlegel D, Cucchiara B.: Orgasmic headache with transient basilar artery vasospasm. Headache 2004, 44:710–712.

    Article  PubMed  Google Scholar 

  32. Kirton A, Diggle J, Hu W, Wirrell E: A pediatric case of reversible segmental cerebral vasoconstriction. Can J Neurol Sci 2006, 33:250–253.

    PubMed  Google Scholar 

  33. Liao YC, Fuh JL, Lirng JF, et al.: Bathing headache: a variant of idiopathic thunderclap headache. Cephalalgia 2003, 23:854–859.

    Article  PubMed  Google Scholar 

  34. 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, 130:3091–3101.

    Article  PubMed  Google Scholar 

  35. Moustafa RR, Allen CM, Baron JC: Call-Fleming syndrome associated with subarachnoid haemorrhage: three new cases. J Neurol Neurosurg Psychiatry 2008, 79:602–605.

    Article  PubMed  CAS  Google Scholar 

  36. Mak W, Tsang KL, Tsoi TH, et al.: Bath-related headache. Cephalalgia 2005, 25:191–198.

    Article  PubMed  CAS  Google Scholar 

  37. Tani E: Molecular mechanisms involved in development of cerebral vasospasm. Neurosurg Focus 2002, 12(3):ECP1.

    Article  Google Scholar 

  38. Salvarani C, Brown RD Jr, Calamia KT, et al.: Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol 2007, 62:442–451.

    Article  PubMed  Google Scholar 

  39. Younger DS: Headaches and vasculitis. Neurol Clin 2004, 22:207–228.

    Article  PubMed  Google Scholar 

  40. Wynne PJ, Younger DS, Khandji A, Sliver AJ: Radiographic features of central nervous system vasculitis. Neurol Clin 1997, 15:779–804.

    Article  PubMed  CAS  Google Scholar 

  41. Hajj-Ali RA, Ghamande S, Calabrese LH, Arroliga AC: Central nervous system vasculitis in the intensive care unit. Crit Care Clin 2002, 18:897–914.

    Article  PubMed  Google Scholar 

  42. Younger D: Vasculitis of the nervous system. Curr Opin Neurol 2004, 17:317–336.

    Article  PubMed  Google Scholar 

  43. Calabrese LH, Furlan AJ, Gragg LA, Ropos TJ: Primary angiitis of the central nervous system. Cleve Clin J Med 1992, 59:293–306.

    PubMed  CAS  Google Scholar 

  44. Calabrese LH: Diagnostic strategies in vasculitis affecting the central nervous system. Cleve Clin J Med 2002, 69(Suppl 2): SII105–SII108.

    Google Scholar 

  45. Singhal AB, Caviness VS, Begleiter AF, et al.: Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology 2002, 58:130–133.

    PubMed  CAS  Google Scholar 

  46. Aikins PT, Levy KJ, Cross AH, et al.: Postpartum cerebral vasospasm treated with hypervolemic therapy. Am J Obstet Gynecol 1996, 175:1386–1388.

    Article  Google Scholar 

  47. Gomez CR, Gomez SM, Puricelli MS, Malik MM: Transcranial Doppler in reversible migrainous vasospasm causing cerebellar infarction: report of a case. Angiology 1991, 42:152–156.

    Article  PubMed  CAS  Google Scholar 

  48. Ikeda T, Urabe H, Matsukage S, et al.: Serial assessment in eclampsia of cerebral hemodynamics by combined transcranial Doppler and magnetic resonance angiography. Gynecol Obstet Invest 2001, 53:65–67.

    Article  Google Scholar 

  49. Dodick DW: Reversible segmental cerebral vasoconstriction (Call-Fleming syndrome): the role of calcium antagonists. Cephalalgia 2003, 23:163–165.

    Article  PubMed  CAS  Google Scholar 

  50. Singhal AB, Bernstein RA: Postpartum angiopathy and other cerebral vasoconstriction syndromes. Neurocrit Care 2005, 3:91–97.

    Article  PubMed  Google Scholar 

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Correspondence to Ralph Z. Kern.

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Gerretsen, P., Kern, R.Z. Reversible cerebral vasoconstriction syndrome: A thunderclap headache-associated condition. Curr Neurol Neurosci Rep 9, 108–114 (2009). https://doi.org/10.1007/s11910-009-0018-5

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