Reversible cerebral vasoconstriction syndrome: A review of pathogenesis, clinical presentation, and treatment

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 ICD-10 code 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 which 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.

Reversible Cerebral Vasoconstriction Syndrome and Female Sex: A Narrative Review

“…2 Headaches during the course of a typical patient with RCVS tend to recur intermittently over a mean of ≈7 days and as noted can be triggered by sexual activity, physical exercise, or Valsalva maneuvers. 1 However, notably, the thunderclap headache occurs less commonly in pregnant women. 1 Unfortunately, RCVS is often initially missed among patients with headaches.…”

“…Projections suggest that each year, over 1000 patients with RCVS are hospitalized; most are middle-aged and ≈75% are women. 4 It is well-established that RCVS more commonly affects females than males, typically in a 2:1 to 10:1 ratio 1 ; furthermore, RCVS appears to manifest in greater clinical and radiographic severity in women. 5,6 Affected women are typically in their 40s and early 50s, while men tend to be 10 years younger.…”

“…7,14 In young adults, RCVS is a common cause of stroke. 1 However, the risk of stroke in RCVS may be potentially mitigated with a high index of clinical suspicion and appropriate treatment in someone presenting with recurrent headaches. 2 Posterior reversible encephalopathy syndrome can also occur in RCVS.…”

“…Segmental, multifocal constriction of intracranial arteries in addition to sudden painful, recurrent headaches characterizes reversible cerebral vasoconstriction syndrome (RCVS). 1 A neurovascular disorder that can result in ischemic stroke, intracranial hemorrhage (including intracerebral hemorrhage and subarachnoid hemorrhage), and posterior reversible encephalopathy, RCVS is known by a variety of eponyms, including Call-Fleming syndrome, postpartum angiopathy, drug-induced angiopathy, acute benign cerebral angiopathy, and benign angiopathy of the central nervous system. 2,3 RCVS has a well-known association with the female sex; thus, it is of utmost importance to have an understanding of the epidemiology, risk factors, clinical and imaging features, differential diagnosis, outcomes, and unanswered questions with regard to RCVS in women.…”

December highlights

“…One of these is reversible cerebral vasoconstriction syndrome (RCVS) which is comprehensively reviewed in this month’s issue of the International Journal of Stroke (IJS). 1 RCVS is characterized by the appearance on angiography of multifocal, segmental, and narrowing-dilatation of intracranial arteries which resolves within days to weeks. It predominantly affects women, and recurrent worst-ever (thunderclap type) headaches are typical at onset.…”