Current Treatment Options in Cardiovascular Medicine: Update on Reversible Cerebral Vasoconstriction Syndrome
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(2020) 22:18
Cerebrovascular Disease and Stroke (S Silverman, Section Editor)
Current Treatment Options in Cardiovascular Medicine: Update on Reversible Cerebral Vasoconstriction Syndrome Eva A. Rocha, MD1,2 Aneesh B. Singhal, MD1,* Address *,1 Department of Neurology, Massachusetts General Hospital, Boston, MA, USA Email: [email protected] 2 Department of Neurology, Universidade Federal de São Paulo, São Paulo, Brazil
* Springer Science+Business Media, LLC, part of Springer Nature 2020
This article is part of the Topical Collection on Cerebrovascular Disease and Stroke Keywords Vasoconstriction I Stroke I Thunderclap headache I Pregnancy I Stress cardiomyopathy I Posterior reversible leukoencephalopathy syndrome
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. Pregnancyassociated 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
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Curr Treat Options Cardio Med
(2020) 22:18
potential harmful effects of certain therapeutic strategies, are expected to optimize the management of this increasingly well recognized syndrome.
Introduction Reversible cerebral vasoconstriction syndrome (RCVS) includes a group of conditions characterized by reversible segmental cerebral artery narrowing and dilatation (Fig. 1), usually accompanied by severe thunderclap headaches (TCH) [1–13]. The dynamic arterial changes can culminate in ischemic or hemorrhagic strokes or brain edema. Yet, the clinical outcome remains favorable in over 90 to 95% of patients [5–13]. RCVS is an umbrella term [2, 4, 14] that include
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