Cerebral Small Vessel Disease Imaging Markers and Disease Severity in Moyamoya Disease: Insights from MRI Analysis

Introduction: There is a lack of early non-invasive imaging markers that can accurately reflect the severity of moyamoya disease (MMD). The clinical significance of summary cerebral small vessel disease (CSVD) score in MMD was unrecognized. This research sought to investigate the clinical importance of CSVD imaging markers in MMD and assess their potential as markers that reflect the disease severity of MMD.

Methods: We prospectively included 225 MMD patients at Beijing Tiantan Hospital from July 2022 to December 2023 and 225 gender-age-matched healthy individuals. The summary CSVD score and each CSVD marker were assessed by magnetic resonance imaging (MRI). Analyses using logistic regression were conducted to identify the factors that were independently linked to CSVD imaging markers, including cerebrovascular morphology, cerebral hemodynamics, clinical manifestation, and RNF213 variant. Receiver operating characteristic (ROC) curves were created to evaluate the relationship between variables and the summary CSVD score, with the area under the curve (AUC) utilized as a measure of this association's strength.

Results: MMD patients showed significantly higher levels of summary CSVD score and each CSVD imaging marker compared with healthy controls (P < 0.05). The age (OR, 1.027 [1.002-1.054]; P=0.038), hemorrhage (OR, 4.560 [2.063-10.079]; P< 0.001), infarction (OR, 4.520 [1.899-10.758]; P=0.001), Suzuki stage≥4 (OR, 3.160 [1.586-6.293]; P=0.001), and high PLR burden (OR, 2.627 [1.278-5.401]; P=0.009) were significantly associated with severe CSVD burden after adjusting for covariates. The multivariate regression models demonstrated a significant relationship between the disease severity and summary CSVD score (AUC=0.809). Furthermore, the associations between disease severity and severe CSVD burden were more prominent among patients with elevated age, preinfarction period stage, and ischemic manifestation.

Conclusion : The CSVD imaging markers were more prominent in patients with MMD compared with healthy individuals, even in asymptomatic patients. These imaging indicators can reflect the severity and progression status of MMD.