PurposeTo investigate whether moyamoya disease (MMD) and atherosclerotic moyamoya syndrome (AS-MMS) differ in vascular morphology and perfusion characteristics using T1w-CUBE imaging and multiple post-labeling delay 3D pseudo-continuous arterial spin labeling imaging (MP 3D-PcASL), and to explore the potential of the combined techniques for accurate diagnosis of both diseases. MethodThis prospective study enrolled 51 patients with moyamoya vasculopathy, including 26 with MMD and 25 with AS-MMS. All patients underwent digital subtraction angiography (DSA)/magnetic resonance angiography (MRA), T1w-CUBE imaging, and MP 3D-PCASL examinations. Morphological parameters, including the outer diameter, maximum wall thickness, luminal stenosis morphology, degree of wall enhancement, number of collateral vessels, and perfusion parameters, such as cerebral blood flow (CBF) and arterial transit time (ATT), were measured. After univariate analysis between the two groups, logistic regression models based on the derived parameters of T1w-CUBE imaging, MP 3D-PCASL, and combined imaging were implemented, and receiver operating characteristic (ROC) curves were generated to compare the discriminatory power of the different imaging methods for the diagnosis of MMD. ResultsWith T1w-CUBE imaging, MMD showed a smaller outer diameter (2.76 ± 0.39 vs. 3.07 ± 0.49 mm) and maximum wall thickness (1.27 ± 0.19 vs. 1.49 ± 0.24 mm) than AS-MMS (both P < 0.05). Using MP 3D-pcASL, the resultant CBF (36.64 ± 14.28 vs. 28.77 ± 8.63 mL/100 g/min) was higher in MMD relative to AS-MMS, while an opposite pattern was shown for ATT (1.61 ± 0.09 vs. 1.72 ± 0.13 s; both P < 0.05). Robust diagnostic efficacies for disease differentiation, confirmed by high areas under the ROC curve (AUCs) (>0.808), were separately shown with T1w-CUBE and MP 3D-pcASL derived parameters. However, the combined multivariate logistic regression model showed optimaldiagnostic efficacy(AUC: 0.938; P < 0.05). ConclusionsCombined T1w-CUBE imaging and MP 3D-PCASL provides distinctive morphological and functional features to evaluate vessel walls and cerebral perfusion, and might help distinguish MMD from AS-MMS.