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Review
. 2025 Jun;20(5):512-523.
doi: 10.1177/17474930241297031. Epub 2024 Nov 4.

Diagnosis and management of adult Moyamoya angiopathy: An overview of guideline recommendations and identification of future research directions

Nicola Rifino  1 , Dominique Hervè  2 , Francesco Acerbi  3 , Satoshi Kuroda  4 , Giuseppe Lanzino  5 , Peter Vajkoczy  6 , Anna Bersano  1
Affiliations
Review

Diagnosis and management of adult Moyamoya angiopathy: An overview of guideline recommendations and identification of future research directions

Nicola Rifino et al. Int J Stroke. 2025 Jun.

Abstract

Despite the progress made in understanding the management and outcomes of Moyamoya angiopathy (MMA), several aspects of the disease remain largely unknown. In particular, evidence on the disease history and management of MMA is lacking, mainly due to methodological and selection biases in the available studies and the lack of large, randomized prospective studies. Therefore, the care of MMA patients remains limited to a few expert centers worldwide, and management is often based on local expertise and available resources. Over the years, recommendations or expert opinions have been written to provide guidance to physicians in the treatment of this condition with the goal of reducing the risk of stroke recurrence and long-term disability. However, there is no complete agreement between the available guidelines and recommendations due to differences in the articles addressed, methodologies, expertise, and validated approaches to literature review. This lack of consensus on the management of MMA may confuse clinicians and highlight some important issues and points. The aim of this comprehensive review article is to critically examine three recent guidelines and recommendations on MMA, discussing their differences and similarities and highlighting gaps in MMA care that need to be covered.

Keywords: Moyamoya; antiplatelet therapy; arteriopathy; stenosis; stroke; therapy.

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Conflict of interest statement

Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Graphical abstract
Graphical abstract
Figure 1.
Figure 1.
(a) Abnormal net-like vessels proliferate giving the characteristic “puff of smoke” appearance on direct angiography. (b) MR angiography showing bilateral middle cerebral artery stenosis. (c) MR angiography showing unilateral stenosis of the right middle cerebral artery. (d) Anteroposterior digital subtraction angiography showing a severe stenosis of the right middle cerebral artery. (e) Perfusion CT scan showing a marked increase in T max bilaterally and (f) only in the left hemisphere.
Figure 2.
Figure 2.
Future research directions in moyamoya angiopathy: key areas to advance diagnostic precision, treatment, and patient outcomes.
See this image and copyright information in PMC

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