Moyamoya disease (MMD) is a chronic occlusive-stenosis cerebrovascular disease that characterized by the stenosis of internal carotid artery termination and the formation of net-like vessel. It is a multifactorial disease caused by genetic, inflammatory, immunological and other environmental factors. The specific pathogenesis of MMD is still unclear. The treatment modalities of revascularization and conservative management have been used in patients with MMD. However, the long-term outcomes of MMD with conservative management remain unknown. Also, some first-degree relatives who are carriers of genetic variants occasionally manifest with intracranial arterial stenosis. Therefore, it is significant to detect the long-term outcomes of conservative treatment in MMD patients and their first-degree relatives, and thus provide potential pathogenesis of MMD.
The purpose of this study is to investigate the long-term outcomes of conservative management in patients with moyamoya disease and their first-degree relatives, and provide potential pathogenesis of moyamoya disease.