Vascular wall imaging in reversible cerebral vasoconstriction syndrome - a 3-T contrast-enhanced MRI study

Abstract

Background: Limited histopathology studies have suggested that reversible cerebral vasoconstriction syndromes (RCVS) does not present with vascular wall inflammation. Previous vascular imaging studies have had inconsistent vascular wall enhancement findings in RCVS patients. The aim of this study was to determine whether absence of arterial wall pathology on imaging is a universal finding in patients with RCVS.

Methods: We recruited patients with RCVS from Taipei Veterans General Hospital prospectively from 2010 to 2012, with follow-up until 2017 (n = 48). We analyzed the characteristics of vascular wall enhancement in these patients without comparisons to a control group. All participants received vascular wall imaging by contrasted T1 fluid-attenuated inversion recovery with a 3-T magnetic resonance machine. The vascular wall enhancement was rated as marked, mild or absent.

Results: Of 48 patients with RCVS, 22 (45.8%) had vascular wall enhancement (5 marked and 17 mild). Demographics, clinical profiles, and cerebral artery flow velocities were similar across patients with versus without vascular wall enhancement, except that patients with vascular wall enhancement had fewer headache attacks than those without (p = 0.04). Follow-up imaging completed in 14 patients (median interval, 7 months) showed reduced enhancement in 9 patients, but persistent enhancement in 5.

Conclusion: Almost half of our RCVS patients exhibited imaging enhancement of diseased vessels, and it was persistent for approximately a third of those patients with follow-up imaging. Both acute and persistent vascular wall enhancement may be unhelpful for differentiating RCVS from central nervous system vasculitis or subclinical atherosclerosis.

Keywords: Contrast enhancement; Reversible cerebral vasoconstriction syndromes; Thunderclap headache; Vascular wall imaging.

Fig. 1

Vascular wall enhancement in patients with RCVS. a, initially mild concentric enhancement, vascular imaging obtained 10 days after disease onset in a 48-year-old female; the enhancement was completely resolved at 7 years of follow-up; b, initially mild concentric enhancement, vascular imaging obtained 9 days after disease onset in a 52-year-old female; the enhancement was partially resolved 96 days later; c, initially marked concentric enhancement, vascular imaging obtained 10 days after disease onset in a 60-year-old female; the enhancement was partially resolved at 4.5 years of follow-up. The white arrowhead in c indicates partial volume of vein. Note that the enhanced vascular wall did not concordantly present at the site of vasoconstriction; d, upper, initially mild eccentric enhancement, vascular imaging obtained 25 days after disease onset in a 49-year-old female; lower, initially mild concentric enhancement, vascular imaging obtained 15 days after disease onset in a 48-year-old female. White arrows locate vascular wall enhancement. Yellow arrows locate vasoconstriction