Mouse Models of Cerebral Arteriovenous Malformation

Arteriovenous (AV) malformation (AVM) is a vascular anomaly capable of both hemorrhagic and ischemic insults, leading to seizures, headaches, stroke, and even death.1 BAVM prevalence is estimated at 0.05%,2 often occurring in young people between 20 and 40 years of age.3 BAVMs account for 50% of hemorrhagic stroke in children4 and 1% to 2% of all strokes in the population.5 Brain AVMs (BAVMs) can cause life-threatening intracerebral hemorrhage (Figure 1).6 Fifty percent of patients are first diagnosed on intracerebral hemorrhage,1 with 1% and 5% annual hemorrhage rate for previously unruptured and ruptured AVMs, respectively.7,8 After BAVM rupture, reported mortality rates range from to 15% to 29%,7 and long-term morbidity rates range from 16% to 56%.1,9 Thus, BAVM is defined by vascular features and accompanying neurological deficits.1Figure 1. Features of human brain arteriovenous malformation (AVM). A , An AVM is visualized on the lateral temporal surface of a human brain. 5, 6 are landmarks placed by surgeon; 40 shows Broca's area; 48 shows Wernicke's area. B , Left internal carotid artery (ICA) angiography (lateral view) reveals a left lateral temporal AVM with a large feeding artery and draining vein. C , Cartoon of this subtype (lateral view), indicating feeding arteries and draining veins. ATA indicates anterior temporal artery. Reprinted from Lawton6 with permission of the publisher. Copyright © 2014, Thieme Medical Publishers.AVM is characterized by high-flow AV connections that shunt blood directly from arteries to veins, displacing intervening capillaries with a nidus of enlarged and tortuous vessels. BAVM clinical characteristics include (1) AV shunting, the presence of direct connections between arteries and veins, displacing intervening capillaries; (2) abnormally high blood flow through the feeding artery, AV …