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Current Treatment Options in Cardiovascular Medicine: Update on Reversible Cerebral Vasoconstriction Syndrome

  • Cerebrovascular Disease and Stroke (S Silverman, Section Editor)
  • Published:
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Abstract

Purpose of review

To describe advances in the diagnosis and management of reversible cerebral vasoconstriction syndrome (RCVS), a group of conditions with reversible multifocal narrowing of intracranial arteries.

Recent findings

Over the last decade, multiple cohort studies have characterized RCVS and distinguished it from primary angiitis of the central nervous system and aneurysmal subarachnoid hemorrhage. Onset with recurrent thunderclap headaches (TCH) occurs in 85–90% of patients; most authors believe that RCVS and primary TCH are similar conditions. Rare cases with concurrent takotsubo cardiomyopathy or extracranial artery narrowing have been published. Stroke and brain edema can develop in up to 40% of inpatients; however, the discharge and 10-year outcome is invariably benign. Pregnancy-associated RCVS may have worse outcome. The pathophysiology remains relatively unknown. There is no specific treatment. Management involves pain relief and removal of identified vasoconstrictive factors. Calcium-channel blockers may help to relieve headaches. Glucocorticoids are associated with significantly worse outcome. The role of intra-arterial vasodilator infusion remains uncertain. The recently developed “RCVS2 score” enables accurate bedside diagnosis with up to 99% specificity and 90% sensitivity, obviating the need for invasive tests such as lumbar puncture, brain biopsy and catheter angiography to exclude mimics or confirm the diagnosis.

Conclusion

RCVS can now be accurately diagnosed using clinical and imaging features available upon presentation. Advances in knowledge about the risk factors, prognosis, and potential harmful effects of certain therapeutic strategies, are expected to optimize the management of this increasingly well recognized syndrome.

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Authors and Affiliations

  1. Department of Neurology, Massachusetts General Hospital, Boston, MA, USA

    Eva A. Rocha MD & Aneesh B. Singhal MD

  2. Department of Neurology, Universidade Federal de São Paulo, São Paulo, Brazil

    Eva A. Rocha MD

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Correspondence to Aneesh B. Singhal MD.

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Dr. Rocha reports the following conflicts: Medlink, Inc.

Dr. Singhal reports the following conflicts: UptoDate Medicine Inc.; Medlink, Inc.; Medicolegal Expert Witness; Biogen; Omniox; Deck Therapeutics; NIH-NINDS U24 NS107243, U01 NS095869, U01 NS110728.

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Rocha, E.A., Singhal, A.B. Current Treatment Options in Cardiovascular Medicine: Update on Reversible Cerebral Vasoconstriction Syndrome. Curr Treat Options Cardio Med 22, 18 (2020). https://doi.org/10.1007/s11936-020-00819-9

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