WSO December 2023 – Issue 3 - 1151

1181250
WSO
International Journal of StrokeSinghal
Review
Reversible cerebral vasoconstriction
syndrome: A review of pathogenesis,
clinical presentation, and treatment
Aneesh B Singhal
Abstract
Reversible segmental narrowing of the intracranial arteries has been described since several decades in numerous clinical
settings, using variable nosology. Twenty-one years ago, we tentatively proposed the unifying concept that these entities,
based on similar clinical-imaging features, represented a single cerebrovascular syndrome. This " reversible cerebral
vasoconstriction syndrome " or RCVS has now come of age. A new International Classification of Diseases code, (ICD10,
I67.841) has been established, enabling larger-scale studies. The RCVS2 scoring system provides high accuracy in
confirming RCVS diagnosis and excluding mimics such as primary angiitis of the central nervous system. Several groups
have characterized its clinical-imaging features. RCVS predominantly affects women. Recurrent worst-ever (thunderclap)
headaches are typical at onset. While initial brain imaging is often normal, approximately one-third to half develop
complications such as convexity subarachnoid hemorrhages, lobar hemorrhages, ischemic strokes located in arterial
" watershed " territories and reversible edema, alone or in combination. Vasoconstriction evolves over hours to days,
first affecting distal and then the more proximal arteries. An overlap between RCVS and primary thunderclap headache,
posterior reversible encephalopathy syndrome, Takotsubo cardiomyopathy, transient global amnesia, and other
conditions has been recognized. The pathophysiology remains largely unknown. Management is mostly symptomatic:
headache relief with analgesics and oral calcium-channel blockers, removal of vasoconstrictive factors, and avoidance
of glucocorticoids that can significantly worsen outcome. Intra-arterial vasodilator infusions provide variable success.
Overall, 90-95% of admitted patients achieve complete or major resolution of symptoms and clinical deficits within days
to weeks. Recurrence is exceptional, although 5% can later develop isolated thunderclap headaches with or without mild
cerebral vasoconstriction.
Keywords
Thunderclap headache, cerebral vasoconstriction, arteriopathy
Received: 21 February 2023; accepted: 23 May 2023
International Journal of Stroke
2023, Vol. 18(10) 1151 -1160
© 2023 World Stroke Organization
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DOI: 10.1177/17474930231181250
journals.sagepub.com/home/wso
https://doi.org/10.1177/17474930231181250
Introduction: unity in diversity
Reversible cerebral vasoconstriction syndrome (RCVS)
refers to conditions with similar clinical-imaging features,
course, and outcome, namely, onset with recurrent thunderclap
headaches (TCHs) and multifocal, segmental, centripetally
progressive narrowing-dilatation of intracranial
arteries that resolves within days to weeks. Many patients
develop convexity subarachnoid hemorrhage (cSAH),
watershed-territory infarcts, or edema; however, clinical
outcome is excellent.
Twenty-one years ago, we tentatively proposed that
RCVS1-3 can be triggered by serotonergic drugs, and was
identical to entities described for over 50 years: migrainous
vasospasm,4 migraine-angiitis,4,5 Call-Fleming syndrome,6
TCH-associated vasospasm,7,8 vasospasm from unruptured
aneurysms,7 drug-induced arteritis,9,10 postpartum angiopathy,11,12
benign angiopathy of the central nervous system
(CNS),13 and CNS pseudovasculitis.14 Many patients had
been misinterpreted as having primary angiitis of the CNS
(PACNS) due to overlapping features such as headache,
stroke, and cerebral angiographic abnormalities, and were
Neurology Department, Massachusetts General Hospital, Boston, MA,
USA
Corresponding author:
Aneesh B Singhal, Neurology Department, Massachusetts General
Hospital, Boston, MA 02114, USA.
Email: asinghal@mgh.harvard.edu
International Journal of Stroke, 18(10)
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WSO December 2023 – Issue 3

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