Reversible Cerebral Vasoconstriction Syndrome: an Update of Recent Research

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by recurrent thunderclap headaches (TCH) and reversible segmental and multifocal vasoconstriction of cerebral arteries. Noninvasive neuroimaging such as computed tomography (CT), and magnetic resonance imaging (MRI) are widely used to assess cerebral vasculature, ischemic or hemorrhagic stroke, and posterior reversible encephalopathy (PRES). The significant differential diagnoses of RCVS center on the clinical presentation of headache and similar radiographic features including aneurysmal subarachnoid hemorrhage (SAH), cervical artery dissection, and central nervous system vasculitis (CNS-V). In this review we present a comprehensive overview of RCVS with the newest findings in the reported literature. Pathophysiology: Several surrogate markers including plasma endothelin-1 (E-1) have been investigated to elucidate the pathogenesis of RCVS. Clinical evaluation and diagnosis: The RCVS2 score is a simple scoring system has been proposed to distinguish RCVS from other intracranial vasculopathies. Neuroimaging: High-resolution contract-enhanced MRI (HR-MRI) vessel wall imaging is a feasible tool in differentiating vessel wall patterns of RCVS and CNS-V. Treatment: Early initiation of calcium channel blockers effectively prevents recurrent TCH and shorten the clinical courses. Clinical scoring system and newer imaging tools are helpful for the early distinction of RCVS. These approaches may serve increased sensitivity and specificity for the diagnosis and lead to appropriate management in RCVS patients.