烟雾病发病机制研究和诊疗进展

Comentary on mechanical investigation and progress of therapeutic methods of Moyamoya disease

烟雾病(MMD)是一类病因不明,自发性双侧颈内动脉远端慢性闭塞,继发颅底异常血管网形成的脑血管病,临床上常表现为缺血或出血性卒中发作。世界范围内东亚地区MMD的发病率最高,特别是近年来随着国内相关知识的普及,我国MMD的诊断和治疗病例数呈快速上升趋势。然而迄今为止仍有发病机制不明,颅内外搭桥手术对患者长期疗效不明确,药物治疗开发难度大等临床痛点和难点。本文将针对上述问题和争议,从MMD的发病机制研究、临床诊疗现状和治疗展望三个方面展开论述。MMD是一个进行性进展的疾病,其发生发展机制还需更多的深入研究来揭示,MMD的治疗也是综合性治疗难题,需要药物治疗,手术治疗以及心理治疗等辅助治疗来帮助患者获得最大的临床受益。

Moyamoya disease(MMD)is a group of cerebrovascular diseases of unknown etiology,with spontaneous bilateral chronic occlusion of the distal internal carotid arteries and secondary formation of an abnormal vascular network at the base of the skull,often presenting clinically as ischaemic or haemorrhagic stroke episodes.The incidence of MMD is highest in East Asia worldwide,and the number of diagnosed and treated cases of MMD in China is rapidly increasing,especially in recent years as knowledge of the disease has become more widespread in China.However,to date there are still clinical pains and difficulties such as unknown pathogenesis,unclear long-term efficacy of intracranial external bypass surgery for patients,and difficulty in developing drug therapy.In this article,we will address these issues and controversies in three areas:research into the pathogenesis of MMD,the current state of clinical management and the outlook for treatment.MMD is a progressive disease,and more research is needed to uncover the mechanisms of its development.The treatment of MMD is also a comprehensive treatment challenge,requiring pharmacological,surgical and psychological treatments to help patients achieve maximum clinical benefit.