Evaluation of vessel-wall contrast-enhancement on high-resolution MRI in European patients with Moyamoya disease
Introduction
Moyamoya disease (MMD) is an arteriopathy characterized by progressive stenosis and ultimately occlusion of the terminal internal carotid artery (ICA) and its proximal branches1,2. The angiographic appearance of compensatory collateral networks resembling a “puff of smoke” (“moyamoya” in Japanese) has given the disease its name. The term “Moyamoya disease” is reserved for the idiopathic form of the arteriopathy. When underlying conditions such as sickle cell anemia, neurofibromatosis or prior cranial irradiation are present, the term “Moyamoya syndrome” is often used2. Moreover, MMD needs to be differentiated from other intracranial arteriopathies, particularly atherosclerosis, which can occasionally cause similar angiographic findings3,4. MMD typically involves the carotid terminus bilaterally, but unilateral MMD has been acknowledged and can be classified as “probable MMD”5.
MMD is mostly found in East Asian patients3,6,7 and can be considered as rare in the European population8,9. Thus comparatively little is known regarding epidemiological, clinical and imaging manifestations of MMD in European patients. It has been reported that the percentage of female patients is higher and the frequency of cerebral hemorrhage is lower in patients of European origin10,11. Recently, digital subtraction angiography (DSA) findings in Europeans have been described as relatively similar to Asian MMD patients12.
Magnetic resonance imaging (MRI) with dedicated vessel wall imaging (VWI) is used to assess a variety of vasculopathies13. VWI uses different techniques to suppress the signal of intraluminal blood, thus allowing the evaluation of the vessel wall13,14. An important aspect of VWI is the detection of vessel wall contrast-enhancement (VW-CE). Initially thought of as hallmark of CNS vasculitis, VW-CE has now been identified in many intracranial vasculopathies, as recently detailed15. VW-CE has been described in several of the existing studies on VWI in MMD patients16, 17, 18, 19, 20, 21. It has been suggested that VW-CE might be caused by hyperproliferation of vessel wall components and/or neovascularization in MMD patients20,22, although this remains speculative as no direct correlation of VWI and pathological findings is available. However, there is evidence that the presence of VW-CE could be clinically relevant, as previous studies have found associations of VW-CE with clinical or imaging signs of active or progressive disease16,21,23. To our knowledge there are only a few studies describing VWI findings in non-Asian MMD patients23, 24, 25. We report our data regarding the frequency of VW-CE and its correlation with clinical and other imaging parameters in a European cohort. We hypothesized that the presence of VW-CE is dependent on the stage of the disease.
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Materials and Methods
The study was approved by the ethics committee of the medical faculty of the LMU Munich. Informed patient consent was waived due to the retrospective design of the study.
We searched the electronic medical records of the Department of Neurology of our institution from September 2008 to December 2019 for patients with suspected MMD. The time span was chosen because dedicated vessel wall MRI has been used at our institution since September 2008.
Patients were eligible for this study if they had at
Results
Eleven patients were included in the study. Eight patients were female and three were male; all were of European origin (none of Asian descent). The median age was 41 years (range 15-49 years). Clinical data are shown in Table 2.
The terminal ICA and/or its proximal branches were affected bilaterally in seven patients and unilaterally in four patients. No stenoocclusive lesions were found in the posterior circulation. Three patients had more than one MRI scan meeting the criteria to be included
Discussion
VW-CE was documented in 57 % of the affected hemispheres in MMD patients in our study. In the literature, the reported rate of VW-CE in MMD patients differs significantly. While Kathuveetil16 and Mossa-Basha25 report that VW-CE of steno-occlusive lesions was relatively rare in their respective studies, Ryoo et al20 found VW-CE in 90.6 % of cases (29 of 32 patients). Some of the differences can be explained methodically by different ways of image analysis and mathematical calculation: For
Conclusions
Our data suggest that concentric VW-CE is a relatively frequent finding in European MMD patients. There are indications that VW-CE may change over time and occur in certain stages, possibly representing “active stenosing”. Larger studies are warranted to validate these findings and determine the clinical relevance of VW-CE in MMD.
Declaration of Competing Interest
None.
Funding
There was no grant support for this study.
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Cited by (0)
The study was performed at the Institute of Diagnostic and Interventional Neuroradiology and the Department of Neurology of the Ludwig-Maximilians-University Munich.