单侧烟雾病的最新研究进展

单侧烟雾病是以一侧颈内动脉末端及其主要分支进行性狭窄、闭塞以致脑底出现烟雾状血管的慢性血管病。单侧烟雾病的发病率最高达17.8%,其家族发病率占6.7%。发病年龄的双峰分布、儿童及成人的两种首发症状均与典型烟雾病基本一致,但成人单侧烟雾病患者的脑出血发生率较高,且主要为脑室出血。单侧烟雾病进展为典型烟雾病的发生率最高可达58.8%。手术治疗可有效地降低缺血性卒中的发生,提高预后,但其适应证有待进一步研究。本文就单侧烟雾病的流行病学、病因学、临床特征、影像学特征、手术及预后等最新研究进展做一综述,增强认识,为诊断及治疗打下基础。

Current Research Progress of Unilateral Moyamoya Disease

Unilateral moyamoya disease is a chronic vasculopathy characterized by progressive
stenosis or occlusion of terminal and main branches of unilateral internal carotid artery, with
the formation of moyamoya vessels at the base of brain. The incidence of unilateral moyamoya
disease is up to 17.8% and familial forms account for about 6.7% of patients with the disease.
Two peaks of age distribution and two first symptoms of children and adults are basically
consistent with typical moyamoya disease, but the incidence of hemorrhage is higher in adults
with unilateral moyamoya disease, especially ventricular hemorrhage. For the most part, 58.8% of
the unilateral cases developed into definite type. Surgical revascularization procedures can help
prevent ischemic attacks effectively and improve prognosis, but operation indications need further
study. In this review, we summarize current research progress of epidemiology, aetiology, clinical
features, imaging features, treatment, and prognosis in unilateral moyamoya disease for profound
understanding, clarified diagnosis and effective treatment.