MRI of a patient with retinal vasculopathy with cerebral leukoencephalopathy and systemic manifestations (RVCL-S) and a rim-enhancing lesion without mass effect

The upper row shows axial T2 FLAIR sequences (A,C) or T2-weighted TSE sequences (B,D). The lower row shows contrast-enhanced T1 SE. Confluent hyperintensity of the periventricular white matter can be found, predominantly at the anterior horns of the lateral ventricles. A rim enhancing lesion can be seen adjacent to the left anterior horn of the lateral ventricle without relevant mass effect. Smaller enhancing lesions can be found adjacent to the posterior part of the left lateral ventricle and in the deep white matter of the left hemisphere (A). After three months the rim-enhancing lesion at the left anterior horn increased in size (B). After five months the rim-enhancing lesion remained stable in size and appearance (C) and after another two months the lesion was reduced in size (D).