Background and Purpose
Although studies have evaluated the differential imaging of moyamoya disease and atherosclerosis, none have investigated the added value of vessel-wall MRI. The current study evaluates the added diagnostic value of vessel-wall MRI in differentiating moyamoya disease (MMD), atherosclerotic-moyamoya syndrome (A-MMS) and vasculitic-moyamoya syndrome (V-MMS) with a multi-contrast protocol.
Methods
We retrospectively reviewed the carotid artery territories of patients with clinically defined vasculopathies (MMD, atherosclerosis, vasculitis) and steno-occlusive intracranial carotid disease. Two neuroradiologists, blinded to clinical data reviewed the luminal imaging of each carotid, evaluating collateral extent and making a presumed diagnosis with diagnostic confidence. After three weeks, the two readers reviewed the luminal imaging+vessel-wall MRI for presence, pattern and intensity of post-contrast enhancement, T2 signal characteristics, pattern of involvement, presumed diagnosis and confidence.
Results
Ten A-MMS, three V-MMS and eight MMD cases with 38 affected carotid segments were included. There was significant improvement in diagnostic accuracy with luminal imaging+vessel-wall MRI as compared to luminal imaging (87% vs. 32%, p<.001). The most common vessel-wall MRI findings for MMD were non-enhancing, non-remodeling lesions without T2 heterogeneity; for A-MMS eccentric, remodeling, and T2 heterogeneous lesions with mild/moderate and homogeneous/heterogeneous enhancement; and for V-MMS concentric lesions with homogeneous, moderate enhancement. Inter-reader agreement was moderate to substantial for all vessel-wall MRI characteristics (κ=0.46-0.86) and fair for collateral grading (κ=0.35). There was 11% inter-reader agreement for diagnosis on luminal imaging as compared to 82% for luminal imaging+vessel-wall MRI (p<.001).
Conclusion
Vessel-wall MRI can significantly improve the differentiation of moyamoya vasculopathies when combined with traditional imaging techniques.