Pathogenesis of non-hereditary brain arteriovenous malformation and therapeutic implications

Abstract

<jats:p> Brain arteriovenous malformations have a high risk of intracranial hemorrhage, which is a substantial cause of morbidity and mortality in patients with brain arteriovenous malformations. Although a variety of genetic factors leading to hereditary brain arteriovenous malformations have been extensively investigated, their pathogenesis is still not well elucidated, especially in sporadic brain arteriovenous malformations. The authors have reviewed the updated data of not only the genetic aspects of sporadic brain arteriovenous malformations, but also the architecture of microvasculature, the roles of the angiogenic factors, and the signaling pathways. This knowledge may allow us to infer the pathogenesis of sporadic brain arteriovenous malformations and develop pre-emptive treatments for them. </jats:p>

State of the Art in the Role of Endovascular Embolization in the Management of Brain Arteriovenous Malformations—A Systematic Review

“…Hitherto, given that 88% of patients are asymptomatic, establishing a true prevalence rate is extremely complicated if not even impossible [ 14 ]. However, due to the widespread use of advanced imaging modalities, there has been an increase in the incidental discoveries of bAVM [ 1 , 2 ]. Brain arteriovenous malformations have an incidence rate of 0.89–2.05 per 100,000 person-years in Western societies [ 15 , 16 , 17 , 18 ].…”

“…Arteriovenous malformations of the brain involve a dense tangle of anomalous connections between dilated cerebral arteries and veins located within the brain parenchyma [ 1 , 2 ]. These lesions are known to form at the interface between the arterial and venous endothelium [ 2 ].…”

“…Arteriovenous malformations of the brain involve a dense tangle of anomalous connections between dilated cerebral arteries and veins located within the brain parenchyma [ 1 , 2 ]. These lesions are known to form at the interface between the arterial and venous endothelium [ 2 ]. The abnormal vascular organization at the subarterial level of these vessels and the lack of a capillary bed lead to a high-flow, low-resistance arteriovenous shunt [ 1 , 2 ].…”

“…These lesions are known to form at the interface between the arterial and venous endothelium [ 2 ]. The abnormal vascular organization at the subarterial level of these vessels and the lack of a capillary bed lead to a high-flow, low-resistance arteriovenous shunt [ 1 , 2 ]. The resulting blood-flow conditions significantly increase the risk of intracerebral hemorrhage and seizures, with the former being the most common manifestation as well as the primary source of morbidity and mortality due to bAVMs, especially in children and young adults [ 3 , 4 ].…”

“…Early hemorrhage symptoms are often mild, with bleeding typically limited to the brain arteriovenous malformation itself or stemming from the venous side of the malformation [ 5 , 6 , 7 ]. Aside from these most common manifestations, patients may develop focal neurologic deficits and headaches independently of cerebral bleeding [ 1 , 2 ]. Generally, ruptured bAVMs manifest in intraparenchymal, subarachnoid, and intraventricular hemorrhages.…”