Cerebral small vessel disease imaging markers and disease severity in moyamoya disease: Insights from magnetic resonance imaging analysis

Abstract

Background: The clinical significance of the summary cerebral small vessel disease (CSVD) score in moyamoya disease (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.

Method: We prospectively included 225 MMD patients at Beijing Tiantan Hospital from July 2022 to December 2023 and 225 gender-age-matched healthy individuals. CSVD imaging markers were assessed using T1-weighted, T2-weighted, fluid-attenuated inversion recovery, and susceptibility-weighted imaging sequences. Multivariable logistic regression analysis was performed to identify factors independently associated with CSVD imaging markers, including cerebrovascular morphology, cerebral hemodynamics, clinical manifestations, and the RNF213 variant.

Result: 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 platelet-lymphocyte ratio 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.

Keywords: Cerebral Small Vessel Diseases; Magnetic Resonance Imaging; Moyamoya disease; Stroke.