Skip to content
2000
Volume 21, Issue 1
  • ISSN: 1573-4056
  • E-ISSN: 1875-6603
file format text download EPUB
file format pdf download PDF
side by side viewer icon HTML

Abstract

Introduction

Reversible cerebral vasoconstriction syndrome (RCVS) is a condition characterized by thunderclap headaches, which are sudden and severe headaches that peak within a few seconds. These headaches present diagnostic difficulties due to their diversity and low specificity, often leading to misdiagnoses and patient dissatisfaction.

Case Presentation

We present the case of a 52-year-old woman with a 10-day history of recurrent thunderclap headaches. Initial imaging revealed no abnormalities, but she experienced further episodes of thunderclap headaches during hospitalization. Subsequent neurovascular imaging revealed multiple intracranial stenoses with a “string of beads” appearance, confirming the diagnosis of reversible cerebral vasoconstriction syndrome. She was treated with nimodipine, and most symptoms had resolved upon discharge, with no recurrence of headache reported during a 3-month follow-up.

Discussion

Prior reviews on reversible cerebral vasoconstriction syndrome predominantly emphasized isolated symptoms or advanced neuroimaging findings, offering limited applicability in primary care services. More attention should be given to identifying clinical manifestations warranting heightened reversible cerebral vasoconstriction syndrome suspicion.

Conclusion

Early recognition of reversible cerebral vasoconstriction syndrome counts in primary care services. We proposed a revised diagnostic routine that begins with clinical suspicion prompted by typical manifestations, like recurrent thunderclap headaches, female sex, and specific triggers, and recommends advanced neurovascular imaging when accessible. Extreme headache severity or deviation from prior migraine patterns should raise suspicion for reversible cerebral vasoconstriction syndrome, while diagnostic consideration should still remain in patients with transient neurological deficits, seizures, or cerebrovascular events.

© The Author(s). Published by Bentham Science Publisher.
This is an open access article distributed under the terms of the Creative Commons Attribution 4.0 International Public License (CC-BY 4.0), a copy of which is available at: https://creativecommons.org/licenses/by/4.0/legalcode. This license permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Loading

Article metrics loading...

/content/journals/cmir/10.2174/0115734056366051250604054327
2025-06-17
2025-09-04

  • From This Site

    /content/journals/cmir/10.2174/0115734056366051250604054327
    dcterms_title,dcterms_subject,pub_keyword
    -contentType:Contributor -contentType:Concept -contentType:Institution
    10
    5
Loading full text...

Full text loading...

/deliver/fulltext/cmir/21/1/CMIR-21-E15734056366051.html?itemId=/content/journals/cmir/10.2174/0115734056366051250604054327&mimeType=html&fmt=ahah

References

  1. SinghalA.B. CavinessV.S. BegleiterA.F. MarkE.J. RordorfG. KoroshetzW.J. Cerebral vasoconstriction and stroke after use of serotonergic drugs.Neurology200258113013310.1212/WNL.58.1.13011781419
     [Google Scholar]
  2. RazaviM. BendixenB. MaleyJ.E. ShoaibM. ZargarianM. RazaviB. AdamsH.P. CNS pseudovasculitis in a patient with pheochromocytoma.Neurology19995251088109010.1212/WNL.52.5.108810102439
     [Google Scholar]
  3. CalabreseL.H. DodickD.W. SchwedtT.J. SinghalA.B. Narrative review: Reversible cerebral vasoconstriction syndromes.Ann. Intern. Med.20071461344410.7326/0003‑4819‑146‑1‑200701020‑0000717200220
     [Google Scholar]
  4. ChenS.P. FuhJ.L. WangS.J. ChangF.C. LirngJ.F. FangY.C. ShiaB.C. WuJ.C. Magnetic resonance angiography in reversible cerebral vasoconstriction syndromes.Ann. Neurol.201067564865610.1002/ana.2195120437562
     [Google Scholar]
  5. SinghalA.B. Hajj-AliR.A. TopcuogluM.A. FokJ. BenaJ. YangD. CalabreseL.H. Reversible cerebral vasoconstriction syndromes: Analysis of 139 cases.Arch. Neurol.20116881005101210.1001/archneurol.2011.6821482916
     [Google Scholar]
  6. PerilloT. PaolellaC. PerrottaG. SerinoA. CaranciF. MantoA. Reversible cerebral vasoconstriction syndrome: Review of neuroimaging findings.Radiol. Med.2022127998199010.1007/s11547‑022‑01532‑235932443
     [Google Scholar]
  7. DucrosA. Reversible cerebral vasoconstriction syndrome.Lancet Neurol.2012111090691710.1016/S1474‑4422(12)70135‑722995694
     [Google Scholar]
  8. SinghalA.B. Posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome as syndromes of cerebrovascular dysregulation.Continuum20212751301132010.1212/CON.000000000000103734618761
     [Google Scholar]
  9. ShimodaM. OdaS. ShigematsuH. HoshikawaK. ImaiM. KomatsuF. HirayamaA. OsadaT. Clinical significance of centripetal propagation of vasoconstriction in patients with reversible cerebral vasoconstriction syndrome: A retrospective case-control study.Cephalalgia201838121864187510.1177/033310241876247129495882
     [Google Scholar]
  10. BurtonT.M. BushnellC.D. Reversible cerebral vasoconstriction syndrome.Stroke20195082253225810.1161/STROKEAHA.119.02441631272323
     [Google Scholar]
  11. WangS-J. FuhJ-L. WuZ-A. ChenS-P. LirngJ-F. Bath-related thunderclap headache: A study of 21 consecutive patients.Cephalalgia200828552453010.1111/j.1468‑2982.2008.01541.x18318749
     [Google Scholar]
  12. HuC.M. LinY.J. FanY.K. ChenS.P. LaiT.H. Isolated thunderclap headache during sex: Orgasmic headache or reversible cerebral vasoconstriction syndrome?J. Clin. Neurosci.201017101349135110.1016/j.jocn.2010.01.05220655230
     [Google Scholar]
  13. SongT.J. LeeK.H. LiH. KimJ.Y. ChangK. KimS.H. HanK.H. KimB.Y. KronbichlerA. DucrosA. KoyanagiA. JacobL. KimM.S. YonD.K. LeeS.W. YangJ.M. HongS.H. GhaydaR.A. KangJ.W. ShinJ.I. SmithL. Reversible cerebral vasoconstriction syndrome: A comprehensive systematic review.Eur. Rev. Med. Pharmacol. Sci.20212593519352934002826
     [Google Scholar]
  14. DucrosA. BoukobzaM. PorcherR. SarovM. ValadeD. BousserM.G. The clinical and radiological spectrum of reversible cerebral vasoconstriction syndrome. A prospective series of 67 patients.Brain2007130123091310110.1093/brain/awm25618025032
     [Google Scholar]
  15. ChoiH.A. LeeM.J. ChoiH. ChungC.S. Characteristics and demographics of reversible cerebral vasoconstriction syndrome: A large prospective series of Korean patients.Cephalalgia201838476577510.1177/033310241771522328592180
     [Google Scholar]
  16. TopcuogluM.A. SinghalA.B. Hemorrhagic reversible cerebral vasoconstriction syndrome.Stroke20164771742174710.1161/STROKEAHA.116.01313627272485
     [Google Scholar]
  17. DucrosA. FiedlerU. PorcherR. BoukobzaM. StapfC. BousserM.G. Hemorrhagic manifestations of reversible cerebral vasoconstriction syndrome: Frequency, features, and risk factors.Stroke201041112505251110.1161/STROKEAHA.109.57231320884871
     [Google Scholar]
  18. ChenS.P. WangS.J. Pathophysiology of reversible cerebral vasoconstriction syndrome.J. Biomed. Sci.20222917210.1186/s12929‑022‑00857‑436127720
     [Google Scholar]
  19. PatelS.D. TopiwalaK. Otite OliverF. SaberH. PanzaG. MuiG. LiebeskindD.S. SaverJ.L. AlbertsM. DucrosA. Outcomes among patients with reversible cerebral vasoconstriction syndrome: A nationwide united states analysis.Stroke202152123970397710.1161/STROKEAHA.121.03442434470494
     [Google Scholar]
  20. RileyD.S. BarberM.S. KienleG.S. AronsonJ.K. von Schoen-AngererT. TugwellP. KieneH. HelfandM. AltmanD.G. SoxH. WerthmannP.G. MoherD. RisonR.A. ShamseerL. KochC.A. SunG.H. HanawayP. SudakN.L. Kaszkin-BettagM. CarpenterJ.E. GagnierJ.J. CARE guidelines for case reports: Explanation and elaboration document.J. Clin. Epidemiol.20178921823510.1016/j.jclinepi.2017.04.02628529185
     [Google Scholar]
  21. KatzB.S. FugateJ.E. AmerisoS.F. Pujol-LereisV.A. MandrekarJ. FlemmingK.D. KallmesD.F. RabinsteinA.A. Clinical worsening in reversible cerebral vasoconstriction syndrome.JAMA Neurol.2014711687310.1001/jamaneurol.2013.463924190097
     [Google Scholar]
  22. JohnS. SinghalA.B. CalabreseL. UchinoK. HammadT. TepperS. StillmanM. MillsB. ThankachanT. Hajj-AliR.A. Long-term outcomes after reversible cerebral vasoconstriction syndrome.Cephalalgia201636438739410.1177/033310241559150726088331
     [Google Scholar]
  23. LeeM.J. ChaJ. ChoiH.A. WooS.Y. KimS. WangS.J. ChungC.S. Blood–brain barrier breakdown in reversible cerebral vasoconstriction syndrome: Implications for pathophysiology and diagnosis.Ann. Neurol.201781345446610.1002/ana.2489128195428
     [Google Scholar]
  24. SinghalA.B. TopcuogluM.A. Glucocorticoid-associated worsening in reversible cerebral vasoconstriction syndrome.Neurology201788322823610.1212/WNL.000000000000351027940651
     [Google Scholar]
  25. de BoyssonH. ParientiJ.J. MawetJ. ArquizanC. BoulouisG. BurcinC. NaggaraO. ZuberM. TouzéE. AoubaA. BousserM.G. PagnouxC. DucrosA. Primary angiitis of the CNS and reversible cerebral vasoconstriction syndrome.Neurology20189116e1468e147810.1212/WNL.000000000000636730232250
     [Google Scholar]
  26. ChoS. LeeM.J. ChungC.S. Effect of nimodipine treatment on the clinical course of reversible cerebral vasoconstriction syndrome.Front. Neurol.20191064410.3389/fneur.2019.0064431275233
     [Google Scholar]
  27. RochaE.A. TopcuogluM.A. SilvaG.S. SinghalA.B. RCVS 2 score and diagnostic approach for reversible cerebral vasoconstriction syndrome.Neurology2019927e639e64710.1212/WNL.000000000000691730635475
     [Google Scholar]
  28. BoitetR. de GaalonS. DuflosC. MarinG. MawetJ. BurcinC. RoosC. FiedlerU. BousserM.G. DucrosA. Long-term outcomes after reversible cerebral vasoconstriction syndrome.Stroke202051267067310.1161/STROKEAHA.119.02770331842705
     [Google Scholar]
  29. ChoS. LeeM.J. GilY.E. ChungC.S. RCVS–TCH score can predict reversible cerebral vasoconstriction syndrome in patients with thunderclap headache.Sci. Rep.2021111775010.1038/s41598‑021‑87412‑733833341
     [Google Scholar]
  30. LingY.H. WangY.F. LirngJ.F. FuhJ.L. WangS.J. ChenS.P. Post-reversible cerebral vasoconstriction syndrome headache.J. Headache Pain20212211410.1186/s10194‑021‑01223‑933765906
     [Google Scholar]
  31. PatelS.D. TopiwalaK. SainiV. PatelN. PervezM. Al-MuftiF. HassanA.E. KhandelwalP. StarkeR.M. Hemorrhagic reversible cerebral vasoconstriction syndrome: A retrospective observational study.J. Neurol.2021268263263910.1007/s00415‑020‑10193‑y32894331
     [Google Scholar]
  32. HathidaraM. PatelN.H. FloresA. CabreraY. CabreraF. KochS. Transcranial Doppler findings in reversible cerebral vasoconstriction syndrome.J. Neuroimaging202232234535110.1111/jon.1294634784094
     [Google Scholar]
  33. StrunkD. VeltkampR. MeuthS.G. ChapotR. KraemerM. Intra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: A neuroradiological method to help differentiate from primary central nervous system vasculitis.Neurol. Res. Pract.202241810.1186/s42466‑022‑00173‑035227319
     [Google Scholar]
  34. AnsariS.A. RathT.J. GandhiD. Reversible cerebral vasoconstriction syndromes presenting with subarachnoid hemorrhage: A case series.J. Neurointerv. Surg.20113327227810.1136/jnis.2010.00424221990840
     [Google Scholar]
  35. OgunlajaO.I. CowanR. Subarachnoid Hemorrhage and Headache.Curr. Pain Headache Rep.20192364410.1007/s11916‑019‑0785‑x31123920
     [Google Scholar]
  36. PilatoF. DistefanoM. CalandrelliR. Posterior reversible encephalopathy syndrome and reversible cerebral vasoconstriction syndrome: Clinical and radiological considerations.Front. Neurol.2020113410.3389/fneur.2020.0003432117007
     [Google Scholar]
  37. LipP.Z.Y. LipG.Y.H. Patent foramen ovale and migraine attacks: A systematic review.Am. J. Med.2014127541142010.1016/j.amjmed.2013.12.00624355354
     [Google Scholar]
  38. CaoW. ShenY. ZhongJ. ChenZ. WangN. YangJ. The patent foramen ovale and migraine: Associated mechanisms and perspectives from MRI evidence.Brain Sci.202212794110.3390/brainsci1207094135884747
     [Google Scholar]
  39. AshinaM. HansenJ.M. DoT.P. Melo-CarrilloA. BursteinR. MoskowitzM.A. Migraine and the trigeminovascular system—40 years and counting.Lancet Neurol.201918879580410.1016/S1474‑4422(19)30185‑131160203
     [Google Scholar]
  40. WilmshurstP. NightingaleS. The role of cardiac and pulmonary pathology in migraine: A hypothesis.Headache200646342943410.1111/j.1526‑4610.2006.00374.x16618259
     [Google Scholar]
  41. TobisJ.M. CharlesA. SilbersteinS.D. SorensenS. MainiB. HorwitzP.A. GurleyJ.C. Percutaneous closure of patent foramen ovale in patients with migraine.J. Am. Coll. Cardiol.201770222766277410.1016/j.jacc.2017.09.110529191325
     [Google Scholar]
  42. CariaF. ZeddeM. GambaM. The clinical spectrum of reversible cerebral vasoconstriction syndrome: The Italian Project on Stroke at Young Age (IPSYS).Cephalalgia201939101267127610.1177/0333102419849013
     [Google Scholar]
/content/journals/cmir/10.2174/0115734056366051250604054327
Loading
Navigating the Diagnostic Maze: A Case Report and Narrative Review of Reversible Cerebral Vasoconstriction Syndrome
Curr. Med. Imaging 21, E15734056366051 (2025); https://doi.org/10.2174/0115734056366051250604054327
/content/journals/cmir/10.2174/0115734056366051250604054327
/content/journals/cmir/10.2174/0115734056366051250604054327
Loading

Data & Media loading...


  • Article Type:     Case Report
Keyword(s): Case report; Cerebral vascular disease; Magnetic resonance angiography; Narrative review; Reversible cerebral vasoconstriction syndrome; Thunderclap headache

Most Cited Most Cited RSS feed

This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error
Please enter a valid_number test