Quantification of High-Resolution Contrast-Enhanced T1-Weighted Vessel Wall MRI for Predicting Disease Progression in Moyamoya Disease

<b>Objective</b><b>:</b> In moyamoya disease (MMD), the internal carotid and proximal cerebral arteries narrow, potentially leading to stroke or hemorrhage from fragile collaterals. Disease activity and progression may be detected by contrast-enhanced (CE) high-resolution (HR) vessel wall imaging (CE-VWI) on T1-weighted MRI. However, this imaging approach needs standardization for the evaluation of signal intensity and longitudinal reproducibility. <b>Methods:</b> MMD patients with at least two separate CE-VWI examinations on the same and on different scanners were included. Signal intensity of the vessel wall, pituitary stalk, and temporal lobe white matter were measured and normalized using manually selected regions of interest. Intraindividual longitudinal reproducibility of MRI was analyzed and the clinical course was correlated with vessel wall enhancement data. <b>Results:</b> Eighty-seven patients were analyzed. Primary analysis included 60 patients with two or more CE-VWI measurements (n = 129) with median 14.8 months between examinations (range: 2-36 months) on the same scanner. Intraindividual variation in pituitary stalk enhancement (positive control) and temporal lobe white matter enhancement (negative control) showed median signal variability of 20.5% and 17.5%, respectively. The pituitary-to-temporal lobe signal intensity ratio remained stable over time (<i>p</i> = 0.843) with 9.4% median variability. Correlation analysis revealed a significant positive association between pituitary and temporal lobe signal changes (ρ = 0.717, <i>p</i> < 0.001). A total of 75% of patients showed vessel wall contrast enhancement with fluctuating signal intensity over approximately 15.9 months, likely depicting disease activity. <b>Conclusions:</b> CE-VWI is important for screening disease activity in moyamoya patients. Our findings demonstrate longitudinal intraindividual reproducibility when normalized to pituitary stalk, enabling quantified evaluation of disease progression through longitudinal vessel wall contrast-enhancement changes.