Moyamoya disease is a rare disorder of the cerebrovascular system affecting individuals in a bimodal age distribution and is characterized by progressive vascular stenosis of the bilateral supraclinoid internal carotid arteries with compensatory formation of collateral vessels at the base of the brain. Despite the disease’s initial description in the literature in 1957, little progress has been made in the development of medical and surgical therapeutics due to, in no small part, the lack of effective experimental animal models. Currently, there is a poor understanding of the pathophysiological mechanisms behind the development of the moyamoya vasculopathies. Since the description of a genetic association between moyamoya disease, few studies have investigated the impact of genetic manipulation on the development of an animal model for experimentation. To date, no one model recapitulates the precise phenotype of the moyamoya vasculopathies, although development of an appropriate model would allow for an in-depth investigation into the pathological mechanisms underlying the disease. In this review, the authors discuss the immunological, mechanical, and genetic methods used to develop moyamoya experimental models, as well as future perspectives.

Moyamoya 病是一种罕见的脑血管系统疾病，影响处于双峰年龄分布的个体，其特征是双侧颈内动脉进行性血管狭窄，并在大脑底部形成代偿性侧支血管。尽管在 1957 年的文献中对该疾病进行了初步描述，但在很大程度上由于缺乏有效的实验动物模型，医学和外科疗法的发展进展甚微。目前，人们对烟雾病血管病发展背后的病理生理机制知之甚少。由于描述了烟雾病之间的遗传关联，很少有研究调查基因操作对实验动物模型开发的影响。迄今为止，没有一个模型能够概括出烟雾病血管病的精确表型，尽管开发合适的模型将有助于深入研究该疾病的病理机制。在这篇综述中，作者讨论了用于开发烟雾病实验模型的免疫学、机械和遗传方法，以及未来的前景。