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. 2022 Jan-Feb;55(1):31-37.
doi: 10.1590/0100-3984.2021.0010.

Moyamoya disease and syndrome: a review

Zeferino Demartini Jr  1   2   3 , Bernardo Ca Teixeira  1   2 , Gelson Luis Koppe  2   3 , Luana A Maranha Gatto  3 , Alex Roman  4 , Renato Puppi Munhoz  5
Affiliations

Moyamoya disease and syndrome: a review

Zeferino Demartini Jr et al. Radiol Bras. 2022 Jan-Feb.

Abstract
in English, Portuguese

Moyamoya disease is a chronic occlusive cerebrovascular disease that is non-inflammatory and non-atherosclerotic. It is characterized by endothelial hyperplasia and fibrosis of the intracranial portion of the carotid artery and its proximal branches, leading to progressive stenosis and occlusion, often clinically manifesting as ischemic or hemorrhagic stroke with high rates of morbidity and mortality. On cerebral angiography, the formation of collateral vessels has the appearance of a puff of smoke (moyamoya in Japanese), which became more conspicuous with the refinement of modern imaging techniques. When there is associated disease, it is known as moyamoya syndrome. Treatments are currently limited, although surgical revascularization may prevent ischemic events and preserve quality of life. In this review, we summarize recent advances in moyamoya disease, covering aspects of epidemiology, etiology, presentation, imaging, and treatment strategies.

A doença de moyamoya, ou doença cerebrovascular oclusiva crônica, é uma afecção não inflamatória e não aterosclerótica, caracterizada por hiperplasia endotelial e fibrose dos segmentos intracranianos das artérias carótidas internas e da porção proximal de seus ramos. Isso provoca estenose progressiva e oclusão, frequentemente manifestada clinicamente como isquemia cerebral ou hemorragia intracraniana, com alta morbimortalidade. A formação compensatória de vasos colaterais produz, na angiografia encefálica, um aspecto de nuvem de fumaça (moyamoya, em japonês). Quando existe doença subjacente que possa estar relacionada, a doença recebe o nome de síndrome de moyamoya. Embora a incidência esteja aumentando graças aos novos métodos diagnósticos, as estratégias terapêuticas ainda são limitadas. O diagnóstico precoce permite cirurgias de revascularização cerebral que podem evitar novos acidentes vasculares e melhorar a qualidade de vida. Nesta revisão são apresentados os avanços recentes sobre a doença de moyamoya, citando aspectos de epidemiologia, etiologia, apresentação, exames diagnósticos e tratamento.

Keywords: Cerebral arterial diseases; Cerebral revas-cularization; Cerebrovascular disorders; Intracranial arterial diseases; Moyamoya disease.

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Figures

Figure 1
Figure 1
An 11-year-old girl with a learning disorder who developed right hemichorea. A: MRI angiography showing stenosis of the left ICA (short arrow) and collateral circulation to the right hemisphere (long arrows). Vessel wall imaging before and after contrast administration (B and C, respectively) showing diffuse thickening and enhancement at the site of the left ICA stenosis. Selective DSA of the left ICA (D) and right vertebral artery (E) showing carotid stenosis, whereas collaterals vessels have the moyamoya (“puff of smoke”) aspect.
Figure 2
Figure 2
A 46-year-old male presenting with motor deficit and TIAs. A: Diffusion-weighted MRI showing infarcts (arrows) in the territory of right middle cerebral artery and watershed area. B: Three-dimensional CT angiography showing bilateral stenosis of the terminal carotid arteries (arrows) and reduced opacification of the middle cerebral arteries. C: Arterial spin-labeling perfusion MRI showing extensive reductions in blood flow in the territories of the anterior and middle cerebral arteries, with preserved posterior circulation (asterisk).
Figure 3
Figure 3
A 22-year-old female with chronic headache, together with incipient seizures and MMD. A: Contrast-enhanced MRI showing chronic right frontotemporal infarct and subacute left parietotemporal infarct, as well as collateral circulation surrounding the middle and anterior cerebral arteries (arrow). B: Dynamic susceptibility contrast perfusion MRI showing increased time-to-maximum values in both cerebral hemispheres. Axial contrast-enhanced MRI (C) and coronal CT angiography (D) after right-side encephalo-duro-arterio-synangiosis and left-side bypass between the superficial temporal and middle cerebral arteries (long arrows) showing moyamoya collaterals (short arrow) and signs of revascularization (asterisks).
See this image and copyright information in PMC

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