Reversible cerebral vasoconstriction syndrome - A narrative revision of the literature

Abstract

Reversible Cerebral Vasoconstriction Syndrome (RCVS) is a not very well known clinical-imaging entity; it is characterized by thunderclap headache, which mimics an aneurysmal subarachnoid haemorrhage, and a diffuse and segmental constriction of cerebral arteries, that resolves spontaneously within 3 months. The pathophysiology remains unknown. The female gender is the more affected and more than half of cases occur in the puerperium or after exposure to vasoactive substances. Typically, RCVS is self-limited and has a benign course, although it may have more serious complications with permanent neurologic sequelae and death. Treatment is predominantly supportive and directed to the symptoms.

Keywords: Complications; Female; Reversible Cerebral Vasoconstriction Syndrome; Thunderclap headache.

Fig. 1.

An algorithm for RCVS diagnosis. CTA, computed tomography angiography; MRI, magnetic resonance imaging; MRA, magnetic resonance angiography; PACNS, primary angiitis of the central nervous system (CNS); CA, catheter angiography; TCD, transcranial Doppler. Adapted from Tan et al.