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Moyamoya disease in the United States
Y Numaguchi1, C F Gonzalez, P C Davis
1University of Rochester Medical Center, Department of Radiology, NY 14642, USA. numaguch@rad.rochester.edu
Abstract:
The epidemiology and radiological features of Moyamoya disease (MMD) in the US were investigated. This study encompassed 98 cases; 26 were newly collected from eight US institutions and 72 were previously reported in the US literature. The patients ranged in age from 6 months to 67 years with age peaks in the first, third and fourth decades. MMD was seen in various ethnic groups and females were more commonly involved (71%) than males. A specific etiology could not be determined in most cases but arteriosclerosis and use of oral contraceptives were occasional associations. On angiography and/or magnetic resonance angiography (MRA), carotid arterial stenosis or occlusion was seen bilaterally in 95 cases (97%) and unilaterally in three. On MR or MRA, internal carotid steno-occlusive lesions were well demonstrated in all cases but Moyamoya collateral vessels (MMVs) were visualized in only 65% of the patients. MMVs in the basal ganglia and thalami were best demonstrated on T1 weighted images. Parenchymal lesions were seen in all patients and were often bilateral. With advances in MR techniques and increasing awareness of diagnostic guidelines, MMD will be diagnosed more frequently than before in the US.
Insights
Moyamoya disease (MMD) predominantly affects females and shows characteristic carotid steno-occlusive lesions. Advances in MR imaging enhance MMD diagnosis, revealing associated parenchymal lesions.
Area of Science:
- Neurology
- Radiology
- Epidemiology
Background:
- Moyamoya disease (MMD) is a rare cerebrovascular disorder characterized by progressive stenosis or occlusion of the internal carotid arteries.
- Understanding the epidemiology and radiological manifestations of MMD in the United States is crucial for timely diagnosis and management.
Purpose of the Study:
- To investigate the epidemiological characteristics and radiological features of Moyamoya disease in the US population.
- To analyze the diagnostic utility of angiography and magnetic resonance angiography (MRA) in identifying MMD features.
Main Methods:
- Retrospective analysis of 98 Moyamoya disease cases in the US (26 new, 72 literature-based).
- Review of patient demographics, clinical presentations, and radiological findings from angiography and MRA.
- Assessment of the visualization of internal carotid artery steno-occlusive lesions and Moyamoya collateral vessels (MMVs).
Main Results:
- MMD affected females more commonly (71%) across various age groups, with peaks in the first, third, and fourth decades.
- Bilateral carotid arterial stenosis or occlusion was present in 97% of cases.
- While internal carotid steno-occlusive lesions were consistently demonstrated, Moyamoya collateral vessels were visualized in 65% of patients; parenchymal lesions were universal.
Conclusions:
- MMD exhibits distinct epidemiological and radiological patterns in the US.
- Advanced MR techniques improve the detection of MMD and its associated lesions.
- Increased awareness and improved diagnostic guidelines are expected to lead to more frequent MMD diagnoses in the US.