烟雾病临床分型研究进展

doi:10.3969/j.issn.1673-5765.2022.10.014

摘要/Abstract

摘要： 烟雾病（moyamoya disease，MMD）是一种颈内动脉末端及其分支起始处进行性狭窄伴颅底异常血管网形成的脑血管疾病，临床上常将其分为缺血型、出血型和无症状型。随着诊疗技术的进步，对MMD临床分型的流行病学特征认识也在不断发展。在成年患者中，缺血型MMD占有重要地位，无症状型亦呈逐渐增多的趋势。不同临床分型MMD的发病年龄、遗传背景、病理生理机制、侧支血管代偿、受累血管分布及其预后存在显著差异。本文总结了对MMD临床分型流行病学特征认识的历史演变，不同临床分型可能的遗传背景、病理生理机制和影像学表现上的差异，为基于不同临床分型的MMD治疗决策提供新的思路。

文章导读： 不同临床分型的MMD其发病机制、影像学特点不同，目前对其易感基因、生物学标志物等方面的研究正在开展。随着技术的发展，我们对MMD的认识会更加深入。

关键词: 烟雾病; 临床分型; 遗传; 机制; 治疗

Abstract: Moyamoya disease (MMD) is a cerebrovascular disease characterized by progressive stenosis of internal carotid arteries and their proximal branches with abnormal vascular network at skull base, which is clinically divided into ischemic, hemorrhagic and asymptomatic types. With the progress of diagnosis and treatment technology, the understanding of epidemiological characteristics of different clinical classification of MMD has developed. The majority of adult patients are ischemic type and the asymptomatic type is gradually increasing. The age of onset, genetic background, pathophysiological mechanism, collateral vascular compensation, distribution of involved vessels and prognosis of MMD were significantly different among different types. This article summarized the historical evolution of epidemiological characteristics, the possible genetic background, pathophysiological mechanism and imaging manifestations of different clinical classification of MMD, to provide new ideas for clinical treatment strategy of MMD.

Key words: Moyamoya disease; Clinical classification; Genetic; Mechanism; Treatment

韩广淞, 倪俊. 烟雾病临床分型研究进展[J]. 中国卒中杂志, 2022, 17(10): 1120-1126.

HAN Guangsong, NI Jun. Advances in Clinical Classification of Moyamoya Disease [J]. Chinese Journal of Stroke, 2022, 17(10): 1120-1126.

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