脑动静脉畸形（brain arteriovenous malformations，bAVM）是神经外科常见的血管性疾病，其组织病理学表现为动静脉之间缺乏常见的毛细血管，血液从动脉直接分流到静脉，由动脉化静脉和静脉化动脉组成的异常血管团。破裂出血是bAVM最为常见的临床表现，严重者影响神经功能甚至危及生命。尽管大多数bAVM病例是散发性的，没有家族史，但有些病例是有家族性的。大多数家族性bAVM病例与一种称为遗传性毛细血管扩张症（HHT）的疾病有关。本文综述了近年来bAVM研究的最新进展，并对bAVM发病机制中的一些重要信号通路途径进行了总结。

Brain arteriovenous malformations (BAVM) is a common vascular disease in neurosurgery. Its histopathological manifestations are the lack of common capillaries between the arteriovenous, and the blood flows directly from the artery to the vein, which is an abnormal vascular mass composed of arterialized vein and venous artery. Rupture and hemorrhage is the most common clinical manifestation of BVM, which seriously affects the nerve function and even endangers life. Although most of the cases are sporadic and have no family history, some cases are familial. Most cases of familial balavm are associated with a disease called hereditary telangiectasia (HHT). This paper reviews the latest progress in the study of BAVM in recent years, and summarizes some important signaling pathways in the pathogenesis of BAVM.