Reversible cerebral vasoconstriction syndrome: review of neuroimaging findings

Abstract

Reversible cerebral vasoconstriction syndrome (RCVS) is a group of disorders characterized by segmental narrowing and dilatation of medium-to-large cerebral arteries, clinically presenting with recurrent episodes of sudden-onset thunderclap headaches, with or without focal neurological deficits. Cerebral vasoconstriction is typically reversible, with spontaneous resolution within 3 months. Although the syndrome has generally a benign course, patients with neurological deficits may experience worse outcome. The main imaging finding is segmental constriction of intracranial arteries, which can be associated with subarachnoid hemorrhage and/or ischemic foci. Other possible findings are intracranial hemorrhage, subdural bleeding and cerebral edema. The latter may have a pattern which can resemble that of posterior reversible encephalopathy syndrome, a condition that can overlap with RCVS. New imaging techniques, such as vessel wall imaging and arterial spin labeling, are proving useful in RCVS and are giving new insights into the pathophysiology of this condition. In this paper, we aim to review neuroimaging findings of RCVS.

Keywords: Digital subtraction angiography; Magnetic resonance imaging; Reversible cerebral vasoconstriction syndrome; Subarachnoid hemorrhage; Vessel wall imaging.

Fig. 1

Non-contrast coronal reformat CT shows left frontal SAH (arrows) within cortical sulci

Fig. 2

Non-contrast CT reveals a focal intraparenchymal hemorrhage in the subcortical white matter in the area of the inferior frontal gyrus (arrow)

Fig. 3

Axial fat-suppressed FLAIR (a) and coronal reformat of 3D maximum intensity projection MR Angiography (b) showing a vast ICH in the right frontal region (short black arrows in a) with concomitant vasogenic edema (white arrows in A). There is concomitant vasoconstriction of anterior, middle and posterior cerebral arteries with alternating areas of vasodilatation (b)

Fig. 4

Axial fat-suppressed FLAIR (a) and coronal reformat of 3D maximum intensity projection MR Angiography (b) showing SAH in the frontal region bilaterally. There is also vasoconstriction of the arteries of the circle of Willis (b) which resolved after 3 months (c)

Fig. 5

Axial fat-suppressed FLAIR (a), Apparent Diffusion Coefficient (B) and coronal reformat of 3D maximum intensity projection MR Angiography (c) show an acute ischemic area in the right parietal region hyperintense in FLAIR (A) with low Apparent Diffusion Coefficient values (b). There is also vasoconstriction with “sausage on a string” appearance, more prominent in the right middle cerebral artery (arrows in c)

Fig. 6

Axial FLAIR (a), DWI (b) and Apparent Diffusion Coefficient (c) images show ischemic stroke with watershed distribution, involving left frontal parasagittal area and the left posterior parietal lobe. Note also SAH in the left parietal convexity (arrow in A)

Fig. 7

Coronal reformat of 3D maximum intensity projection MR Angiography reveals multiple and bilateral segmental arterial narrowing of anterior, middle and posterior cerebral arteries with a subtle “beaded” appearance

Fig. 8

Coronal reformat of 3D maximum intensity projection MR Angiography (a) shows bilateral stenoses of anterior and middle cerebral arteries and of the basilar artery. At follow-up MR Angiography performed 9 weeks later, there is marked improvement in vessel caliber (b)

Fig. 9

DSA of the right internal carotid artery demonstrates subtle areas of narrowing