Reversible Cerebral Vasoconstriction Syndrome

Abstract

Reversible cerebral vasoconstriction syndromes (RCVS) are a group of conditions characterized by reversible multifocal cerebral artery narrowing and dilatation, typically heralded by acute, severe, and recurrent ‘thunderclap’ headaches. RCVS can develop spontaneously or in diverse clinical settings such as childbirth or after exposure to vasoconstrictive drugs. Brain imaging shows no parenchymal lesion in more than one-third of patients; the rest develop ischemic stroke, parenchymal hemorrhage, convexity subarachnoid hemorrhage, or reversible brain edema. RCVS can be instantly recognized by a characteristic constellation of acute headaches and neurovascular imaging findings in the appropriate clinical setting. It is important to exclude mimics such as aneurysmal subarachnoid hemorrhage and primary angiitis of the central nervous system. The clinical course is self-limited and benign in more than 90%, but a minority can develop progressive vasoconstriction resulting in widespread strokes, brain edema, and even death. Treatment is empirical and includes simple observation and calcium channel blockers. Recurrence of an episode of RCVS is rare.