Reversible Cerebral Vasoconstriction Syndrome, Part 1: Epidemiology, Pathogenesis, and Clinical Course

Abstract

Reversible cerebral vasoconstriction syndrome is a clinical and radiologic syndrome that represents a common presentation of a diverse group of disorders. The syndrome is characterized by thunderclap headache and reversible vasoconstriction of cerebral arteries, which can either be spontaneous or related to an exogenous trigger. The pathophysiology of reversible cerebral vasoconstriction syndrome is unknown, though alterations in cerebral vascular tone are thought to be a key underlying mechanism. The syndrome typically follows a benign course; however, reversible cerebral vasoconstriction syndrome may result in permanent disability or death in a small minority of patients secondary to complications such as ischemic stroke or intracranial hemorrhage.

Fig 1.

A 47-year-old woman with the sudden onset of severe headache. Initial noncontrast head CT (A) demonstrates trace sulcal subarachnoid hemorrhage (white arrow) near the vertex. CT angiography performed at the same time (B) is interpreted as having unremarkable findings. Conventional angiography (C) demonstrates mild diffuse irregularity with multifocal narrowings throughout the cerebral vasculature with a beaded appearance, most pronounced in distal right middle cerebral artery cortical branches (black arrow). Findings are most consistent with RCVS. Follow-up catheter angiogram performed 1 month later (D) demonstrates complete resolution of cerebral vasoconstriction (black arrow).

Fig 2.

A 42-year-old woman who presented with altered mental status and lethargy. FLAIR imaging (A) demonstrates signal hyperintensity involving the cortex and underlying subcortical white matter in the parietal and occipital lobes (white arrows), consistent with PRES. There is no evidence of associated diffusion restriction. Trace sulcal subarachnoid hemorrhage was also noted overlying the right frontal lobe (not shown). Note subtle irregularity and multifocal narrowings involving distal cortical branches of the bilateral middle and anterior cerebral arteries (black arrows) on cerebral angiography (B), suggestive of RCVS. The patient made a full recovery, with complete resolution of cerebral areas of abnormal FLAIR hyperintensity (C) and cerebral vasoconstriction (not shown).

Fig 3.

A 19-year-old man with a 2-day history of recurrent headaches and prior marijuana use. Noncontrast CT was negative for acute hemorrhage (not shown). Conventional angiography (A) reveals multifocal areas of moderate narrowing and irregularity involving the cerebral vasculature (white arrows, A). These areas resolved following intra-arterial administration of verapamil (white arrows, B). Clinical course and imaging findings are consistent with RCVS.