MRI of a patient with retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) and a rim-enhancing lesion with mass effect

The upper row shows axial T2 FLAIR sequences (A, B, C) or T2-weighted TSE sequences (D). The lower row shows contrast-enhanced T1 SE. A small, rim-enhancing lesion with perifocal T2 hyperintense white matter on FLAIR imaging can be seen at the left anterior horn with little mass effect (A). After two months the lesion has dramatically increased in size and shows massive T2 hyperintense, T1 hypointense perifocal edema with midline shifting mass effect that comprises most parts of the left frontal lobe (B). Another four weeks later, peripheral enhancement of the lesion is still prominent while the size and mass effect is decreased (C). After 20 months a vascular defect has formed with ex vacuo enlargement of the anterior horn of the left lateral ventricle (D). Blood brain barrier disruption is still evident but has changed from rim-enhancement to a central and nodular appearance.