2019 Volume 36 Issue 3 Pages 241-246
During the last 10 years, reversible cerebral vasoconstriction syndrome (RCVS) has emerged as the most frequent cause of thunderclap headache (TCH) in patients without aneurysmal subarachnoid hemorrhage, and as the most frequent cause of recurrent TCHs. The typical TCHs of RCVS are multiple, recurring over a few days to weeks, excruciating, short–lived, and brought up by exertion, sexual activities, emotion, Valsalva maneuvers, or bathing, among other triggers. The pathophysiology of RCVS is unknown, though alterations in cerebral vascular tone are thought to be a key underlying mechanism.
Because randomized controlled trials do not yet exist, treatment of RCVS is currently based on empiric treatment. Current treatment recommendations for RCVS include withdrawal of any suspected exogenous triggers, including vasoactive medications, and intensive care unit level care, symptom relief with analgesics, blood pressure control, and seizure prophylaxis. Calcium channel blockers, such as nimodipine, verapamil or lomerizine, have been used in most patients. Glucocorticoid steroids have been administered to patients with RCVS, without improvement in either patient symptoms or sequelae of the disease.
Early diagnosis of RCVS in patients who present with isolated headache enables proper management and might reduce the risk of eventual stroke. Probable RCVS can be diagnosed transiently in patients in whom a repeat angiogram will disclose visible vasoconstriction after a few days, and can also be made in patients who will have no visible vasoconstriction at all. Patients with probable RCVS should be managed as patients with visible vasoconstriction. The syndrome typically follows a benign course ; however, RCVS may result in permanent disability or death in a small minority of patients secondary to complications such as ischemic stroke or intracranial hemorrhage.
We describe the characteristics, triggers, diagnosis, and management of TCH in RCVS.
