Reversible Cerebral Vasoconstriction Syndromes

The reversible cerebral vasoconstriction syndrome includes a group of conditions unified by dynamic and reversible changes in the caliber of intracranial arteries, usually accompanied by recurrent thunderclap headaches. Women are predominantly affected. The etiology is unknown; however, several triggers are recognized including vasoconstrictive drug use and childbirth. Brain imaging can remain normal, but about half develop infarcts, convexal (non-aneurysmal) subarachnoid hemorrhages, lobar hemorrhages, or reversible brain edema. The condition is invariably self-limited with resolution of clinical and angiographic abnormalities within 3. months. Glucocorticoid therapy has been associated with worse outcome. Progressive vasoconstriction can occur resulting in significant disability or death in rare cases. The vast majority have excellent long-term outcome without recurrences.