Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches and reversible cerebral vasoconstrictions. RCVS is more common than previously thought and should be differentiated from aneurismal subarachnoid hemorrhage. RCVS can be spontaneous or evoked by pregnancy or exposure to vasoactive substances. Patients tend to be middle-aged women but pediatric patients have been seen. Up to 80% of sufferers have identifiable triggers. Thunderclap headaches tend to recur daily and last for a period of around 2 weeks, while the vasoconstrictions may last for months. About one-third of patients have blood pressure surges accompanying headache attacks. The potential complications of RCVS include posterior reversible encephalopathy syndrome, ischemic strokes over watershed zones, cortical subarachnoid hemorrhage and intracerebral hemorrhage. Magnetic resonance images including angiography and venography and lumbar punctures are the studies of choice, whereas catheter angiography should not be implemented routinely. Patients with a mean flow velocity of the middle cerebral artery greater than 120 cm/s shown by transcranial color-coded sonography have a greater risk of ischemic complications than those without. The pathophysiology of RCVS remains unknown; sympathetic hyperactivity may play a role. Open-label trials showed calcium channel blockers, such as nimodipine may be an effective treatment in prevention of thunderclap headache attacks. In severe cases, intra-arterial therapy may be considered. Most patients with RCVS recover without sequelae; however, relapse has been reported in a small proportion of patients.
Aaslid, R. ( 2002) Transcranial Doppler assessment of cerebral vasospasm . Eur J Ultrasound16: 3-10.
2.
Armstrong, F.S. and Hayes, G.J. ( 1961) Segmental cerebral arterial constriction associated with pheochromocytoma: report of a case with arteriograms. J Neurosurg18: 843-846.
3.
Bartynski, W.S. ( 2008) Posterior reversible encephalopathy syndrome, part 2: controversies surrounding pathophysiology of vasogenic edema. AJNR Am J Neuroradiol29: 1043-1049.
4.
Bartynski, W.S. and Boardman, J.F. (2008) Catheter angiography, MR angiography, and MR perfusion in posterior reversible encephalopathy syndrome. AJNR Am J Neuroradiol29: 447-455.
5.
Beausang-Linder, M. and Bill, A. ( 1981) Cerebral circulation in acute arterial hypertension - protective effects of sympathetic nervous activity. Acta Physiol Scand111: 193-199.
6.
Birnbaum, J. and Hellmann, D.B.(2009) Primary angiitis of the central nervous system. Arch Neurol66: 704-709.
7.
Bogousslavsky, J., Despland, P.A., Regli, F. and Dubuis, P.Y. ( 1989) Postpartum cerebral angiopathy: reversible vasoconstriction assessed by transcranial Doppler ultrasounds. Eur Neurol29: 102-105.
8.
Bouchard, M., Verreault, S., Gariepy, J.L. and Dupre, N.(2009) Intra-arterial milrinone for reversible cerebral vasoconstriction syndrome . Headache49: 142-145.
9.
Brisman, J.L., Song, J.K. and Newell, D.W. ( 2006) Cerebral aneurysms. N Engl J Med355: 928-939.
10.
Calabrese, L.H., Dodick, D.W., Schwedt, T.J. and Singhal, A.B. ( 2007) Narrative review: reversible cerebral vasoconstriction syndromes. Ann Intern Med146: 34-44.
11.
Calabrese, L.H., Gragg, L.A. and Furlan, A.J. ( 1993) Benign angiopathy: a distinct subset of angiographically defined primary angiitis of the central nervous system. J Rheumatol20: 2046-2050.
Chen, S.P., Fuh, J.L., Chang, F.C., Lirng, J.F., Shia, B.C. and Wang, S.J. ( 2008) Transcranial color doppler study for reversible cerebral vasoconstriction syndromes. Ann Neurol63: 751-757.
14.
Chen, S.P., Fuh, J.L., Lirng, J.F., Chang, F.C. and Wang, S.J. ( 2006a) Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology67: 2164-2169.
15.
Chen, S.P., Fuh, J.L., Lirng, J.F. and Wang, S.J. ( 2006b) Is vasospasm requisite for posterior leukoencephalopathy in patients with primary thunderclap headaches? Cephalalgia26: 530-536.
16.
Chen, S.P., Fuh, J.L., Wang, S.J., Chang, F.C., Lirng, J.F., Fang, Y.C. et al. (2009) Magnetic resonance angiography study in reversible cerebral vasoconstriction syndromes. Ann Neurol (DOI: 10.1002/ ana.21951).
17.
Chik, Y., Hoesch, R.E., Lazaridis, C., Weisman, C.J. and Llinas, R.H.(2009) A case of postpartum cerebral angiopathy with subarachnoid hemorrhage . Nat Rev Neurol5: 512-516.
18.
Dietrich, H.H. and Dacey Jr, R.G. ( 2000) Molecular keys to the problems of cerebral vasospasm. Neurosurgery46: 517-530.
Dodick, D.W., Brown Jr, R.D., Britton, J.W. and Huston 3rd, J. ( 1999) Nonaneurysmal thunderclap headache with diffuse, multifocal, segmental, and reversible vasospasm. Cephalalgia19: 118-123.
21.
Dodick, D.W., Eross, E.J., Drazkowski, J.F. and Ingall, T.J. ( 2003) Thunderclap headache associated with reversible vasospasm and posterior leukoencephalopathy syndrome. Cephalalgia23: 994-997.
Ducros, A., Boukobza, M., Porcher, R., Sarov, M., Valade, D. and Bousser, M.G. ( 2007) The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients. Brain130: 3091-3101.
24.
Ducros, A. and Bousser, M.G. ( 2009) Reversible cerebral vasoconstriction syndrome. Pract Neurol9: 256-267.
25.
Ducros, A., Fiedler, U., Stapf, C., Boukobza, M., Porcher, R., Valade, D. et al. (2009) Hemorrhagic complications in reversible cerebral vasoconstriction syndrome are more frequent in women and in migrainers. Cephalalgia29: 153.
26.
Elstner, M., Linn, J., Muller-Schunk, S. and Straube, A.(2009) Reversible cerebral vasoconstriction syndrome: a complicated clinical course treated with intra-arterial application of nimodipine. Cephalalgia29: 677-682.
27.
Fletcher, J.J., Kramer, A.H., Bleck, T.P. and Solenski, N.J.(2009) Overlapping features of eclampsia and postpartum angiopathy. Neurocrit Care11: 199-209.
28.
Forsting, M. ( 2005) CTA of the ICA bifurcation and intracranial vessels. Eur Radiol15(Suppl 4): D25-D27.
29.
Hajj-Ali, R.A., Furlan, A., Abou-Chebel, A. and Calabrese, L.H. ( 2002) Benign angiopathy of the central nervous system: cohort of 16 patients with clinical course and long-term followup. Arthritis Rheum47: 662-669.
30.
Headache Classification Subcommittee of the International Headache Society. (2004) The International Classification of Headache Disorders, 2nd edition. Cephalalgia24(Suppl 1): 9-160.
31.
Heo, Y.E., Kwon, H.M. and Nam, H.W.(2009) Thunderclap headache as an initial manifestation of phaeochromocytoma . Cephalalgia29: 388-390.
32.
Im, S.H. and Kim, N.H. ( 2008) Thunderclap headache after micturition in bladder pheochromocytoma . Headache48: 965-967.
33.
Jackson, M., Lennox, G., Jaspan, T. and Jefferson, D. ( 1993) Migraine angiitis precipitated by sex headache and leading to watershed infarction. Cephalalgia13: 427-430.
34.
Kaye, B.R. and Fainstat, M. ( 1987) Cerebral vasculitis associated with cocaine abuse. JAMA258: 2104-2106.
35.
Kirton, A., Diggle, J., Hu, W. and Wirrell, E. ( 2006) A pediatric case of reversible segmental cerebral vasoconstriction . Can J Neurol Sci33: 250-253.
36.
Klein, M., Fesl, G., Pfister, H.W., Straube, A., Bruckmann, H., Hoffmann, L.A. et al. (2009) Intra-arterial nimodipine in progressive postpartum cerebral angiopathy. Cephalalgia29: 279-282.
37.
Lee, V.H., Brown Jr, R.D., Mandrekar, J.N. and Mokri, B. ( 2006) Incidence and outcome of cervical artery dissection: a population-based study. Neurology67: 1809-1812.
38.
Levine, R.J., Lam, C., Qian, C., Yu, K.F., Maynard, S.E., Sachs, B.P. et al. (2006) Soluble endoglin and other circulating antiangiogenic factors in preeclampsia. N Engl J Med355: 992-1005.
39.
Levine, R.J., Maynard, S.E., Qian, C., Lim, K.H., England, L.J., Yu, K.F. et al. (2004) Circulating angiogenic factors and the risk of preeclampsia. N Engl J Med350: 672-683.
40.
Liao, Y.C., Fuh, J.L., Lirng, J.F., Lu, S.R., Wu, Z.A. and Wang, S.J. ( 2003) Bathing headache: a variant of idiopathic thunderclap headache. Cephalalgia23: 854-859.
41.
Liu, H.Y., Fuh, J.L., Lirng, J.F., Chen, S.P. and Wang, S.J. ( 2009) Three paediatric patients with reversible cerebral vasoconstriction syndromes. Cephalalgia (DOI: 10.1111/j.1468-2982.2009.01955.x).
42.
Lu, S.R., Liao, Y.C., Fuh, J.L., Lirng, J.F. and Wang, S.J. ( 2004) Nimodipine for treatment of primary thunderclap headache . Neurology62: 1414-1416.
43.
Lysakowski, C., Walder, B., Costanza, M.C. and Tramer, M.R. ( 2001) Transcranial Doppler versus angiography in patients with vasospasm due to a ruptured cerebral aneurysm: A systematic review. Stroke32: 2292-2298.
Nishizawa, S. and Laher, I. ( 2005) Signaling mechanisms in cerebral vasospasm. Trends Cardiovasc Med15: 24-34.
46.
Pluta, R.M. ( 2005) Delayed cerebral vasospasm and nitric oxide: review, new hypothesis, and proposed treatment. Pharmacol Ther105: 23-56.
47.
Rana, S., Karumanchi, S.A., Levine, R.J., Venkatesha, S., Rauh-Hain, J.A., Tamez, H. et al. (2007) Sequential changes in antiangiogenic factors in early pregnancy and risk of developing preeclampsia. Hypertension50: 137-142.
48.
Salvarani, C., Brown Jr, R.D., Calamia, K.T., Christianson, T.J., Weigand, S.D., Miller, D.V. et al. (2007) Primary central nervous system vasculitis: analysis of 101 patients. Ann Neurol62: 442-451.
49.
Schlegel, D. and Cucchiara, B. ( 2004) Orgasmic headache with transient basilar artery vasospasm . Headache44: 710-712.
50.
Schwedt, T.J. ( 2007) Clinical spectrum of thunderclap headache. Expert Rev Neurother7: 1135-1144.
Singhal, A.B. and Bernstein, R.A. ( 2005) Postpartum angiopathy and other cerebral vasoconstriction syndromes. Neurocrit Care3: 91-97.
54.
Singhal, A.B., Caviness, V.S., Begleiter, A.F., Mark, E.J., Rordorf, G. and Koroshetz, W.J. ( 2002) Cerebral vasoconstriction and stroke after use of serotonergic drugs. Neurology58: 130-133.
55.
Singhal, A.B., Kimberly, W.T., Schaefer, P.W. and Hedley-Whyte, E.T.(2009) 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 Med360: 1126-1137.
56.
Sloan, M.A., Alexandrov, A.V., Tegeler, C.H., Spencer, M.P., Caplan, L.R., Feldmann, E. et al. (2004) Assessment: transcranial Doppler ultrasonography: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology62: 1468-1481.
57.
Tang-Wai, D.F., Phan, T.G. and Wijdicks, E.F. ( 2001) Hypertensive encephalopathy presenting with thunderclap headache. Headache41: 198-200.
58.
Wang, S.J., Fuh, J.L., Wu, Z.A., Chen, S.P. and Lirng, J.F. ( 2008) Bath-related thunderclap headache: a study of 21 consecutive patients. Cephalalgia28: 524-530.
