Abstract
Introduction
Reversible cerebral vasoconstriction syndrome (RCVS) is a rare vasculopathy of unknown etiology. Ischemic stroke and intracerebral hemorrhage are well-documented sequelae, but subarachnoid hemorrhage is an uncommon complication of RCVS.
Methods and results
We report six cases of RCVS associated with subarachnoid hemorrhage. Two cases occurred in postpartum women, two in women with a history of migraines, one in a woman who recently stopped taking her anti-hypertensive medications, and one in a man after sexual intercourse. All six patients presented with the classic thunderclap headache. Two patients experienced generalized tonic-clonic seizures, and two patients had small ischemic infarcts. Segmental vasoconstriction was demonstrated on cerebral angiography in all six cases. Aneurysmal subarachnoid hemorrhage and other etiologies were excluded. Reversibility of the segmental vasoconstriction was confirmed by follow-up angiography in four patients and by transcranial Doppler sonography in two patients. All six patients had an excellent neurological outcome.
Conclusions
Reversible cerebral vasoconstriction syndrome may be associated with subarachnoid hemorrhage. RCVS should be included in the differential diagnosis of non-aneurysmal subarachnoid hemorrhage.
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References
Call GK, Fleming MC, Sealfon S, et al. Reversible cerebral segmental vasoconstriction. Stroke 1988;19:1159–70.
Slivka A, Philbrook B. Clinical and angiographic features of thunderclap headache. Headache 1995;35:1–6.
Singhal AB. Cerebral vasoconstriction syndromes. Top Stroke Rehabil 2004;11:1–6.
Bogousslavsky J, Despland PA, Regli F, et al. Postpartum cerebral angiopathy: reversible vasoconstriction assessed by transcranial Doppler ultrasounds. Eur Neurol 1989;29:102–5.
Silbert PL, Hankey GJ, Prentice DA, et al. Angiographically demonstrated arterial spasm in a case of benign sexual headache and benign exertional headache. Aust N Z J Med 1989;19:466–8.
Singhal AB, Bernstein RA. Postpartum angiopathy and other cerebral vasoconstriction syndromes. Neurocrit Care 2005;3:91–7.
Rousseaux P, Scherpereel B, Bernard MH, et al. Acute benign cerebral angiopathy. 6 cases. Presse Med 1983;12:2163–8.
Al-Sous W, Bohlega S, Al-Kawi Z, et al. Post-partum cerebral angiopathy. A rare cerebrovascular complication. Eur J Neurol 1998;5:411–6.
Sato S, Shimizu M, Endo K, et al. Postpartum cerebral angiopathy—a case report the vasculopathy associated with co-administration of two vasoconstrictives, methylergometrine maleate and sumatriptan. Rinsho Shinkeigaku 2004;44:96–101.
Crippa G, Sverzellati E, Pancotti D, et al. Severe postpartum hypertension and reversible cerebral angiopathy associated with ergot derivative (methergoline) administration Ann Ital Med Int 2000;15:303–5.
Comabella M, Alvarez-Sabin J, Rovira A, et al. Bromocriptine and postpartum cerebral angiopathy: a causal relationship? Neurology 1996;46:1754–6.
Loewen AH, Hudon ME, Hill MD. Thunderclap headache and reversible segmental cerebral vasoconstriction associated with use of oxymetazoline nasal spray. Cmaj 2004;171:593–4.
Meschia JF, Malkoff MD, Biller J. Reversible segmental cerebral arterial vasospasm and cerebral infarction: possible association with excessive use of sumatriptan and Midrin. Arch Neurol 1998;55:712–4.
Granier I, Garcia E, Geissler A, et al. Postpartum cerebral angiopathy associated with the administration of sumatriptan and dihydroergotamine––a case report. Intensive Care Med 1999;25:532–4.
Singhal AB, Caviness VS, Begleiter AF, et al. Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology 2002;58:130–3.
Janssens E, Hommel M, Mounier-Vehier F, et al. Postpartum cerebral angiopathy possibly due to bromocriptine therapy. Stroke 1995;26:128–130.
Calabrese LH, Dodick DW, Schwedt TJ, et al. Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med 2007;146:34–44.
Rubiera del Fueyo M, Molina Cateriano CA, Arenillas Lara JF, et al. Reversible segmental cerebral vasoconstriction: the value of duplex transcranial Doppler in its diagnosis and follow up. Rev Neurol 2004;38:530–3.
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.
Kubo S, Nakata H, Tatsumi T, et al. Headache associated with postpartum cerebral angiopathy: monitoring with transcranial color-coded sonography. Headache 2002;42:297–300.
Gladstone JP, Dodick DW, Evans R. The young woman with postpartum “thunderclap” headache. Headache 2005;45:70–4.
Calado S, Viana-Baptista M. Benign cerebral angiopathy; postpartum cerebral angiopathy: characteristics and treatment. Curr Treat Options Cardiovasc Med 2006;8:201–212.
Sengoku R, Iguchi Y, Yaguchi H, et al. A case of postpartum cerebral angiopathy with intracranial hemorrhage and subarachnoid hemorrhage immediately after delivery. Rinsho Shinkeigaku 2005;45:376–9.
Ursell MR, Marras CL, Farb R, et al. Recurrent intracranial hemorrhage due to postpartum cerebral angiopathy: implications for management. Stroke 1998;29:1995–8.
Saito I, Ueda Y, Sano K. Significance of vasospasm in the treatment of ruptured intracranial aneurysms. J Neurosurg 1977;47:412–429.
Qureshi AI, Sung GY, Suri MA, et al. Prognostic value and determinants of ultraearly angiographic vasospasm after aneurysmal subarachnoid hemorrhage. Neurosurgery 1999;44:967–973.
Baldwin ME, Macdonald RL, Huo D, et al. Early vasospasm on admission angiography in patients with aneurysmal subarachnoid hemorrhage is a predictor for in-hospital complications and poor outcome. Stroke 2004;35:2506–11.
Byrom FB. The pathogenesis of hypertensive encephalopathy and its relation to the malignant phase of hypertension; experimental evidence from the hypertensive rat. Lancet 1954;267:201–211.
Day JW, Raskin NH. Thunderclap headache: symptom of unruptured cerebral aneurysm. Lancet 1986;2:1247–8.
Singhal AB. Thunderclap headache, reversible cerebral arterial vasoconstriction, and unruptured aneurysms [letter]. J Neurol Neurosurg Psychiatry 2002;73:96; author reply 96–7.
Neudecker S, Stock K, Krasnianski M. Call-Fleming postpartum angiopathy in the puerperium: a reversible cerebral vasoconstriction syndrome. Obstet Gynecol 2006;107:446–9.
Konstantinopoulos PA, Mousa S, Khairallah R, et al. Postpartum cerebral angiopathy: an important diagnostic consideration in the postpartum period. Am J Obstet Gynecol 2004;191:375–7.
Donaldson JO. Eclampsia and postpartum cerebral angiopathy. J Neurol Sci 2000;178:1.
Varner MW. Cerebral vasculopathies masquerading as eclampsia. Obstet Gynecol 2006;107:437–8.
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.
Acknowledgments
We would like to thank Natasha Wehrli for translating articles in French, and Benjamin Meyer for translating articles in Japanese.
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Edlow, B.L., Kasner, S.E., Hurst, R.W. et al. Reversible Cerebral Vasoconstriction Syndrome Associated with Subarachnoid Hemorrhage. Neurocrit Care 7, 203–210 (2007). https://doi.org/10.1007/s12028-007-0058-0
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DOI: https://doi.org/10.1007/s12028-007-0058-0