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. 2011 Jan;76(1):73-9.

Moyamoya disease: Diagnostic imaging

Eugeniusz Tarasów  1 , Alina Kułakowska, Adam Lukasiewicz, Katarzyna Kapica-Topczewska, Alicja Korneluk-Sadzyńska, Joanna Brzozowska, Wiesław Drozdowski
Affiliations

Moyamoya disease: Diagnostic imaging

Eugeniusz Tarasów et al. Pol J Radiol. 2011 Jan.

Abstract

Moyamoya disease is a progressive vasculopathy leading to stenosis of the main intracranial arteries. The incidence of moyamoya disease is high in Asian countries; in Europe and North America, the prevalence of the disease is considerably lower. Clinically, the disease may be of ischaemic, haemorrhagic and epileptic type. Cognitive dysfunction and behavioral disturbance are atypical symptoms of moyamoya disease.Characteristic angiographic features of the disease include stenosis or occlusion of the arteries of the circle of Willis, as well as the development of collateral vasculature. Currently, magnetic resonance angiography and CT angiography with multi-row systems are the main imaging methods of diagnostics of the entire range of vascular changes in moyamoya disease.The most common surgical treatment combines the direct arterial anastomosis between the superficial temporal artery and middle cerebral, and the indirect synangiosis involving placement of vascularised tissue in the brain cortex, in order to promote neoangiogenesis. Due to progressive changes, correct and early diagnosis is of basic significance in selecting patients for surgery, which is the only effective treatment of the disease. An appropriate qualification to surgery should be based on a comprehensive angiographic and imaging evaluation of brain structures.Despite the rare occurrence of moyamoya disease in European population, it should be considered as one of causes of ischaemic or haemorrhagic strokes, especially in young patients.

Keywords: angiography; ischaemic stroke; moyamoya disease.

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Figures

Figure 1.
Figure 1.
A 39-year-old women, 3 years after stroke, preceded by TIAs (Case 1). CT examination – hypodense area attributable to malacia after stroke in the right temporal and occipital lobe.
Figure 2.
Figure 2.
Case 1. MR study, FLAIR image in axial plane. Hyperintense lesion in the right temporal and occipital lobe.
Figure 3.
Figure 3.
Case 2. A 35-year-old man with facial and arm paresis. MR study, FLAIR image in axial plane. Hyperintense lesion in the right basal ganglia (A). Cortical stroke in the left frontal lobe and ivy sign in the right frontal lobe (B).
Figure 4.
Figure 4.
Case 1, Suzuki grades III/IV. CT angiography, 3D reconstruction. Narrowing of the distal segments of both ICAs. Narrow or locally not visible segments A1 of the anterior cerebral arteries and M1 of the middle cerebral arteries (A). Moyamoya collaterals visible only in MIP reconstruction (B).
Figure 5.
Figure 5.
Case 1, Suzuki grades III/IV. MR angiography, ToF (time-of-flight) technique, MIP reconstructions in sagittal (A) and axial plane (B). No signal from either of the anterior cerebral and middle cerebral arteries. Collateral vessels of “moyamoya” type, visible at the base of the brain.
Figure 6.
Figure 6.
Case 3, Suzuki grade III. A 33-year-old man with seizures, aphasia and right-sided hemiparesis. Angiogram of the right (A) and of the left (B) internal carotid artery. Stenosis of the distal parts of the internal carotid arteries; proximal stenosis of anterior and middle cerebral arteries; single “moyamoya” collateral circulation vessels
Figure 7.
Figure 7.
Case 2, Suzuki grades V/VI. MR angiography, MIP reconstruction in axial plane. No signal from either of the anterior or middle cerebral arteries; collateral circulation vessels not visible.
See this image and copyright information in PMC

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