Narrative review: reversible cerebral vasoconstriction syndromes

Abstract

Reversible cerebral vasoconstriction syndromes (RCVS) comprise a group of diverse conditions, all characterized by reversible multifocal narrowing of the cerebral arteries heralded by sudden (thunderclap), severe headaches with or without associated neurologic deficits. Reversible cerebral vasoconstriction syndromes are clinically important because they affect young persons and can be complicated by ischemic or hemorrhagic strokes. The differential diagnosis of RCVS includes conditions associated with thunderclap headache and conditions that cause irreversible or progressive cerebral artery narrowing, such as intracranial atherosclerosis and cerebral vasculitis. Misdiagnosis as primary cerebral vasculitis and aneurysmal subarachnoid hemorrhage is common because of overlapping clinical and angiographic features. However, unlike these more ominous conditions, RCVS is usually self-limited: Resolution of headaches and vasoconstriction occurs over a period of days to weeks. In this review, we describe our current understanding of RCVS; summarize its key clinical, laboratory, and imaging features; and discuss strategies for diagnostic evaluation and treatment.