Preview

Russian Sklifosovsky Journal "Emergency Medical Care"

Advanced search

Reversible Cerebral Vasoconstriction Syndrome

https://doi.org/10.23934/2223-9022-2024-13-3-492-500

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a collective term used to describe pathological conditions with a similar clinical and radiological picture which is characterized by thunderclap headaches with a sudden onset and reversible segmental (multifocal) constriction of the cerebral arteries. The article is devoted to issues of terminology, genetic and clinical features of RSCV, and also discusses risk factors, differential diagnosis, complications and prognosis. Modern pathogenetic mechanisms and possible approaches to the treatment of this condition are presented.

About the Authors

G. R. Ramazanov
N.V. Sklifosovsky Research Institute for Emergency Medicine
Russian Federation

Ganipa R. Ramazanov, Candidate of Medical Sciences, Deputy Director – Head, Regional Vascular Center; Head, Scientific Department of Emergency Neurology and Rehabilitation

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090



T. A. Magomedov
N.V. Sklifosovsky Research Institute for Emergency Medicine
Russian Federation

Timur A. Magomedov, Neurologist, Neurological Department for Patients with Acute Cerebrovascular Accidents

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090



M. S. Solovtsova
N.V. Sklifosovsky Research Institute for Emergency Medicine
Russian Federation

Margarita S. Solovtsova, Neurologist, Neurological Department for Patients with Acute Cerebrovascular Accidents

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090



E. V. Shevchenko
N.V. Sklifosovsky Research Institute for Emergency Medicine
Russian Federation

Evgeny V. Shevchenko, Candidate of Medical Sciences, Head, Neurological Department for Patients with Acute Cerebrovascular Accidents; Senior Researcher, Scientific Department of Emergency Neurology and Rehabilitation

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090



E. A. Kovaleva
N.V. Sklifosovsky Research Institute for Emergency Medicine
Russian Federation

Ella A. Kovaleva, Candidate of Medical Sciences, Senior Lecturer, Educational Department; Senior Researcher, Scientific Department of Emergency Neurology and Rehabilitation

Bolshaya Sukharevskaya Sq. 3, Moscow, 129090



References

1. Singhal AB. Posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome as syndromes of cerebrovascular dysregulation. Continuum (Minneap Minn). 2021;27(5):1301–1320. PMID: 34618761 https://doi.org/10.1212/CON.0000000000001037

2. Call GK, Fleming MC, Sealfon S, Levine H, Kistler JP, Fisher CM. Reversible cerebral segmental vasoconstriction. Stroke. 1988;19(9):1159–1170. PMID: 3046073 https://doi.org/10.1161/01.str.19.9.1159

3. Burton TM, Bushnell CD. Reversible Cerebral Vasoconstriction Syndrome. Stroke. 2019;50(8):2253–2258. PMID: 31272323 https://doi.org/10.1161/STROKEAHA.119.024416

4. Afanasyeva MYu, Goldobin VV, Klocheva EG. Clinical manifestations and pathogenesis of reversible cerebral vasoconstriction syndrome. Medical alphabet. 2020;(22):22–26. (In Russ.) https://doi.org/10.33667/2078-5631-2020-22-22-26

5. Boitet R, Gaillard N, Bendiab E, Corti L, Roos C, Reynes J, et al. Concomitant reversible cerebral vasoconstriction syndrome and transient global amnesia. J Neurol. 2020;267(2):390–394. PMID: 31650256 https://doi.org/10.1007/s00415-019-09594-5

6. Kim T, Ahn S, Sohn CH, Seo DW, Kim WY. Reversible cerebral vasoconstriction syndrome at the emergency department. Clin Exp Emerg Med. 2015;2(4):203–209. PMID: 27752599 https://doi.org/10.15441/ceem.15.099

7. Singhal AB. Reversible cerebral vasoconstriction syndrome: A review of pathogenesis, clinical presentation, and treatment. Int J Stroke. 2023 Jun 12:17474930231181250. PMID: 37246916 https://doi.org/10.1177/17474930231181250 Online ahead of print.

8. Chen SP, Fuh JL, Lirng JF, Chang FC, Wang SJ. Recurrent primary thunderclap headache and benign CNS angiopathy: spectra of the same disorder? Neurology. 2006;67(12):2164–2169. PMID: 17190937 https://doi.org/10.1212/01.wnl.0000249115.63436.6d

9. Klocheva EG, Goldobin VV. Diagnostics of Reversible Cerebral Vasoconstriction Syndrome: Clinical Manifestation and Imaging Patterns. Diagnostic radiology and radiotherapy. 2018;(3):50–55. (In Russ.). https://doi.org/10.22328/2079-5343-2018-9-3-50-55

10. Caria F, Zedde M, Gamba M, Bersano A, Rasura M, Adami A, et al.; Italian Project on Stroke at Young Age (IPSYS) Investigators. The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS). Cephalalgia. 2019;39(10):1267–1276. PMID: 31060368 https://doi.org/10.1177/0333102419849013

11. Patel SD, Topiwala K, Otite Oliver F, Saber H, Panza G, Mui G, et al. Outcomes among patients with reversible cerebral vasoconstriction syndrome: a nationwide United States analysis. Stroke. 2021;52(12):3970–3977.

12. Erhart DK, Ludolph AC, Althaus K. RCVS: by clinicians for clinicians-a narrative review. J Neurol. 2023;270(2):673–688. PMID: 36305970 https://doi.org/10.1007/s00415-022-11425-z

13. Lin PT, Wang YF, Fuh JL, Lirng JF, Ling YH, Chen SP, et al. Diagnosis and classification of headache associated with sexual activity using a composite algorithm: a cohort study. Cephalalgia. 2021;41(14):1447–1457. PMID: 34275353 https://doi.org/10.1177/03331024211028965

14. Kato Y, Hayashi T, Sano H, Kato R, Tanahashi N, Takao M. Cough headache presenting with reversible cerebral vasoconstriction syndrome. Intern Med. 2018;57(10):1459–1461. PMID: 29321411 https://doi.org/10.2169/internalmedicine.0061-17

15. Hu CM, Lin YJ, Fan YK, Chen SP, Lai TH. Isolated thunderclap headache during sex: orgasmic headache or reversible cerebral vasoconstriction syndrome? J Clin Neurosci. 2010;17(10):1349–1351. PMID: 20655230 https://doi.org/10.1016/j.jocn.2010.01.052

16. Ribas MZ, Paticcié GF, de Medeiros SDP, de Oliveira Veras A, Noleto FM, Dos Santos JCC. Reversible cerebral vasoconstriction syndrome: literature review. Egypt J Neurol Psychiatr Neurosurg. 2023;59(1):5. PMID: 36647436 https://doi.org/10.1186/s41983-023-00607-9

17. Valencia-Mendoza M, Ramírez-Rodríguez N, Vargas-Avila N, Peña-Ortiz A, Corzo-Villamizar M, Serna-Ramírez L, et al. Fatal reversible cerebral vasoconstriction syndrome: a systematic review of case series and case reports. J Clin Neurosci. 2019;70:183–188. PMID: 31416730 https://doi.org/10.1016/j.jocn.2019.08.014

18. Kato Y, Fujita S, Osada T, Takahashi S, Takao M. Reversible cerebral vasoconstriction syndrome triggered by typhoon Hagibis in 2019: report of 2 cases. Headache. 2020;60(4):781–786. PMID: 32100287 https://doi.org/10.1111/head.13775.

19. Lozupone E, Distefano M, Calandrelli R, Della Marca G, Pedicelli A, Pilato F. Reversible cerebral vasoconstriction syndrome: a severe neurological complication in postpartum period. Neurol India. 2020;68(1):192. PMID: 32129278 https://doi.org/10.4103/0028-3886.279674

20. Chen SP, Wang SJ. Pathophysiology of reversible cerebral vasoconstriction syndrome. J Biomed Sci. 2022;29(1):72. PMID: 36127720 https://doi.org/10.1186/s12929-022-00857-4

21. Chen SP, Chang YA, Chou CH, Juan CC, Lee HC, Chen LK, et al. Circulating microRNAs associated with reversible cerebral vasoconstriction syndrome. Ann Neurol. 2021;89(3):459–473. PMID: 33314303 https://doi.org/10.1002/ana.25965

22. Hsu WH, Wang SJ, Chao YM, Chen CJ, Wang YF, Fuh JL, et al. Urine metabolomics signatures in reversible cerebral vasoconstriction syndrome. Cephalalgia. 2020;40(7):735–747. PMID: 31910660 https://doi.org/10.1177/0333102419897621

23. Shnyakin PG, Loseva AS. Development of the syndrome of reversible cerebral vasoconstriction after clipping of an asymptomatic aneurysm of the middle cerebral artery. Russian Neurosurgical Journal named after Professor A. L. Polenov. 2023;15(2):134–139. (In Russ.). https://doi.org/10.56618/2071-2693_2023_15_2_134

24. Arrigan MT, Heran MK, Shewchuk JR. Reversible cerebral vasoconstriction syndrome: an important and common cause of thunderclap and recurrent headaches. Clin Radiol. 2018;73(5):417–427. PMID: 29274685 https://doi.org/10.1016/j.crad.2017.11.017

25. Wolff V, Ducros A. Reversible Cerebral Vasoconstriction Syndrome without Typical Thunderclap Headache. Headache. 2016;56(4):674–687. PMID: 27016378 https://doi.org/10.1111/head.12794

26. Arnold M, Camus-Jacqmin M, Stapf C, Ducros A, Viswanathan A, Berthet K, et al. Postpartum cervicocephalic artery dissection. Stroke. 2008;39(8):2377–2379. PMID: 18535274 https://doi.org/10.1161/STROKEAHA.107.510107

27. Sheikh HU, Mathew PG. Reversible cerebral vasoconstriction syndrome: updates and new perspectives. Curr Pain Headache Rep. 2014;18(5):414. PMID: 24658747 https://doi.org/10.1007/s11916-014-0414-7

28. Kulkarni M, Chauhan V, Shetty S. Reversible cerebral vasoconstriction syndrome. J Assoc Physicians India. 2016;64(6):76–78. PMID: 27739275

29. Perdices M, Herkes G. Reversible cerebral vasoconstriction syndrome. Neuropsychol Rehabil. 2018;28(2):223–233. PMID: 27915588 https://doi.org/10.1080/09602011.2016.1257434

30. Ling YH, Chen SP. Narrative review: headaches after reversible cerebral vasoconstriction syndrome. Cerr Pain Headache Rep. 2020;24(12):74. PMID: 33161482 https://doi.org/10.1007/s11916-020-00908-1

31. Boitet R, de Gaalon S, Duflos C, Marin G, Mawet J, Burcin C, et al. Long-term outcomes after reversible cerebral vasoconstriction syndrome. Stroke. 2020;51(2):670–673. PMID: 31842705 https://doi.org/10.1161/STROKEAHA.119.027703

32. Ramineni KK, Jakkani RK, Girgani S, Balani A, Satyanarayan S. Triptan-induced reversible cerebral vasoconstriction syndrome presenting with thunderclap headache and paraparesis. Neurologist. 2018;23(5):160–162. PMID: 30169369 https://doi.org/10.1097/NRL.0000000000000193

33. Garg A, Starr M, Rocha M, Ortega-Gutierrez S. Predictors and outcomes of ischemic stroke in reversible cerebral vasoconstriction syndrome. J Neurol. 2021;268(8):3020–3025. PMID: 33646329 https://doi.org/10.1007/s00415-021-10456-2

34. Topcuoglu MA, Singhal AB. Hemorrhagic Reversible Cerebral Vasoconstriction Syndrome: Features and Mechanisms. Stroke. 2016;47(7):1742–1747. PMID: 27272485 https://doi.org/10.1161/STROKEAHA.116.013136.

35. Patel SD, Topiwala K, Saini V, Patel N, Pervez M, Al-Mufti F, et al. Hemorrhagic reversible cerebral vasoconstriction syndrome: a retrospective observational study. J Neurol. 2021;268(2):632–639. PMID: 32894331 https://doi.org/10.1007/s00415-020-10193-y

36. 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):2505–2511. PMID: 20884871 https://doi.org/10.1161/strokeaha.109.572313

37. 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. PMID: 23939614 https://doi.org/10.1001/jamaneurol.2013.3484

38. Singhal AB, Topcuoglu MA, Fok JW, Kursun O, Nogueira RG, Frosch MP, et al. Reversible cerebral vasoconstriction syndromes and primary angiitis of the central nervous system: clinical, imaging, and angiographic comparison. Ann Neurol. 2016;79(6):882–894. PMID: 27043703 https://doi.org/10.1002/ana.24652

39. Chen SP, Fuh JL, Wang SJ, Chang FC, Lirng JF, Fang YC, et al.Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes. Ann Neurol. 2010;67(5):648–656. PMID: 20437562 https://doi.org/10.1002/ana.21951

40. Mills JN, Mehta V, Russin J, Amar AP, Rajamohan A, Mack WJ. Advanced imaging modalities in the detection of cerebral vasospasm. Neurol Res Int. 2013;2013:415960. PMID: 23476766 https://doi.org/10.1155/2013/415960

41. Perillo T, Paolella C, Perrotta G, Serino A, Caranci F, Manto A. Reversible cerebral vasoconstriction syndrome: review of neuroimaging findings. Radiol Med. 2022;127(9):981–990. PMID: 35932443 https://doi.org/10.1007/s11547-022-01532-2

42. Fifi JT, Meyers PM, Lavine SD, Cox V, Silverberg L, Mangla S, et al. Complications of modern diagnostic cerebral angiography in an academic medical center. J Vasc Interv Radiol. 2009;20(4):442–447. PMID: 19246211 https://doi.org/10.1016/j.jvir.2009.01.012

43. Shen J, Karki M, Jiang T, Zhao B. Complications associated with diagnostic cerebral angiography: a retrospective analysis of 644 consecutive cerebral angiographic cases. Neurol India. 2018;66(4):1154–1158. PMID: 30038108 https://doi.org/10.4103/0028-3886.237018

44. 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(7):1403–1410. PMID: 22422190 https://doi.org/10.3174/ajnr.A2964

45. Miller TR, Shivashankar R, Mossa-Basha M, Gandhi D. Reversible cerebral vasoconstriction syndrome, part 2: diagnostic work-up, imaging evaluation, and differential diagnosis. AJNR Am J Neuroradiol. 2015;36(9):1580–1588. PMID: 25614476 https://doi.org/10.3174/ajnr.A4215

46. Mandell DM, Matouk CC, Farb RI, Krings T, Agid R, terBrugge K, et al. Vessel wall MRI to differentiate between reversible cerebral vasoconstriction syndrome and central nervous system vasculitis: preliminary results. Stroke. 2012;43:860–862. https://doi.org/10.1161/STROKEAHA.111.626184

47. Pilato F, Distefano M, Calandrelli R. Posterior Reversible Encephalopathy Syndrome and Reversible Syndrome: Clinical and Radiological Considerations. Front Neurol. 2020;11:34. PMID: 32117007 https://doi.org/10.3389/fneur.2020.00034 eCollection 2020.

48. Merli N, Padroni M, Azzini C, Bernardoni A, Marcialis C, Tugnoli V, et al. Reversible cerebral vasoconstriction syndrome: strategies to early diagnosis and the role of transcranial color-coded doppler ultrasonography (TCCD). Neurol Sci. 2023;44(7):2541–2545. PMID: 37014565 https://doi.org/10.1007/s10072-023-06755-3

49. Liu L, Tan Q, Huang R, Hu Z. Analysis of postpartum reversible cerebral vasoconstriction syndrome in China. Medicine (Baltimore). 2019;98(45):e17170. PMID: 31702607 https://doi.org/10.1097/MD.0000000000017170

50. Ducros A. Reversible cerebral vasoconstriction syndrome. Lancet Neurol. 2012;11(10):906–917. PMID: 22995694 https://doi.org/10.1016/S1474-4422(12)70135-7

51. Saini M, Jeerakathil T, Butcher K. Reversible cerebral vasoconstriction syndrome. Neurol India. 2009;57(1):63–65. PMID: 19305081 https://doi.org/10.4103/0028-3886.48816

52. Hajj-Ali RA, Singhal AB, Benseler S, Molloy E, Calabrese LH. Primary angiitis of the CNS. Lancet Neurol. 2011;10(6):561–572. PMID: 21601163 https://doi.org/10.1016/s1474-4422(11)70081-3

53. Rocha EA, Topcuoglu MA, Silva GS, Singhal AB. RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome. Neurology. 2019;92(7):e639–e647. PMID: 30635475 https://doi. org/10.1212/wnl.0000000000006917

54. Singhal AB, Rocha EA. Author response: RCVS2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome. Neurology. 2020;94(21):946. https://doi.org/10.1212/WNL.0000000000009500

55. Collins L, Lam L, Kleinig O, Proudman W, Zhang R, Bagster M, et al. Verapamil in the treatment of reversible cerebral vasoconstriction syndrome: A systematic review. J Clin Neurosci. 2023;113:130–141. https://doi.org/10.1016/j.jocn.2023.05.013 PMID: 37267876

56. Cho S, Lee MJ, Chung CS. Effect of nimodipine treatment on the clinical course of reversible cerebral vasoconstriction syndrome. Front Neurol. 2019;10:644. PMID: 31275233 https://doi.org/10.3389/fneur.2019.00644 eCollection 2019.

57. Song TJ, Lee KH, Li H, Kim JY, Chang K, Kim SH, et al. Reversible cerebral vasoconstriction syndrome: a comprehensive systematic review. Eur Rev Med Pharmacol Sci. 2021;25(9):3519–3529. PMID: 34002826 https://doi.org/10.26355/eurrev_202105_25834

58. Hadhiah KM, Alshagawi ZA, Alzahrani SK, Alrayes MM, Aldandan HW. Reversible cerebral vasoconstriction syndrome in a background of eclampsia responding to milrinone infusion. Am J Case Rep. 2021;22: e934528. PMID: 34784343 https://doi.org/10.12659/AJCR.934528

59. Zeitouni D, Parish JM, Smith M, Stetler WR, Bernard JD. Reversible cerebral vasoconstriction syndrome successfully treated by intrathecal nicardipine. Clin Neurol Neurosurg. 2021;206:106705 PMID: 34053805 https://doi.org/10.1016/j.clineuro.2021.10670

60. Farid H, Tatum JK, Wong C, Halbach VV, Hetts SW. Reversible Cerebral Vasoconstriction Syndrome: Treatment with Combined Intra-Arterial Verapamil Infusion and Intracranial Angioplasty. AJNR Am J Neuroradiol. 2011;32(10):E184–E187. PMID: 21273351 https://doi.org/10.3174/ajnr.A2341


Review

For citations:


Ramazanov G.R., Magomedov T.A., Solovtsova M.S., Shevchenko E.V., Kovaleva E.A. Reversible Cerebral Vasoconstriction Syndrome. Russian Sklifosovsky Journal "Emergency Medical Care". 2024;13(3):492-500. (In Russ.) https://doi.org/10.23934/2223-9022-2024-13-3-492-500

Views: 884


Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.


ISSN 2223-9022 (Print)
ISSN 2541-8017 (Online)