Reversible Cerebral Vasoconstriction Syndromes: What the Cardiologist Should Know

Abstract

Over the past decade, the reversible cerebral vasoconstriction syndromes (RCVS) have emerged as a group of conditions with easily recognizable clinical-angiographic features and a usually benign prognosis. The RCVS affect young individuals, mostly women, and the majority present with recurrent, severe, 'thunderclap' headaches. Vascular imaging studies show dynamic and reversible narrowing and dilatation of multiple intracerebral arteries. Brain imaging usually shows no parenchymal lesions, however, approximately one-third of patients develop ischemic or hemorrhagic strokes or reversible brain edema. The etiopathogenesis of this syndrome remains unclear. It has been associated with diverse conditions such as pregnancy, vasoconstrictive drug use, and neurovascular procedures. Recent studies characterizing RCVS have made it relatively easy to exclude mimics such as aneurysmal subarachnoid hemorrhage and primary angiitis of the central nervous system. There is no proven treatment, although calcium channel blockers may help to reduce the intensity of headaches. Empiric glucocorticoid treatment should be avoided. Since most patients do well with simple observation alone, invasive strategies such as pharmacologically-induced hypertension, balloon angioplasty, and direct intra-arterial vasodilator infusion should be reserved for patients showing clear clinical progression.