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.