Dilated perivascular spaces and steno-occlusive changes in children and adults with moyamoya disease

Abstract

Background Dilated perivascular spaces (DPVS), known as one of imaging markers in cerebral small vessel disease, may be found in patients with moyamoya disease (MMD). However, little is known about DPVS in MMD. The purpose of this study was to investigate the distribution pattern of dPVS in children and adults with MMD and determine whether it is related to steno-occlusive changes of MMD. Methods DPVS was scored in basal ganglia (BG) and white matter (WM) on T2-weighted imaging, using a validated 4-point semi-quantitative score. The degree of dPVS was classified as high (score > 2) or low (score ≤ 2) grade. The steno-occlusive changes on MR angiography (MRA) was scored using a validated MRA grading. Asymmetry of DPVS and MRA grading was defined as a difference of 1 grade or higher between hemispheres. Results Fifty-one patients with MMD (mean age 24.9 ± 21.1 years) were included. Forty-five (88.2%) patients had high WM-DPVS grade (degree 3 or 4). BG-DPVS was found in 72.5% of all patients and all were low grade (degree 1 or 2). The distribution patterns of DPVS degree in BG (P = 1.000) and WM (P = 0.767) were not different between child and adult groups. The asymmetry of WM-DPVS (26%) and MRA grade (42%) were significantly correlated to each other (Kendall’s tau-b = 0.604, P < 0.001). Conclusions DPVS of high grade in MMD is predominantly found in WM, which was not different between children and adults. The correlation between asymmetry of WM-DPVS degree and MRA grade suggests that weak cerebral artery pulsation due to steno-occlusive changes may affect WM-DPVS in MMD.

Quantifying dilated perivascular spaces in children with sickle cell disease

“…24 In contrast, in a study of children with moyamoya disease, 88% of the children ages 1-16 years included had evidence of high dPVS burden in the CSO, albeit also on 3T MRI. 37 Finally, our analysis was completed on images acquired through a 1.5T MRI, which suggests that our results may either be underscored or represent true pathological dPVS, as those apparent on 1.5T may be truly disease-related. Thus, these results suggest that children with SCD, similarly to children with another neurovascular disorder, moyamoya disease, have a higher burden of dPVS compared to healthy children, which is consistent with the known association of neurological complications with SCD.…”