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

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 centres 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 not complete agreement be-tween 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 paper is to critically examine these three recent guidelines on MMA and discuss their differences and similarities, highlighting gaps in MMA care that need to be covered.