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。     

烟雾病的研究进展

烟雾病(moyamoya disease)是一种罕见的脑血管疾病,其特征是颈内动脉末端及其主要分支逐渐狭窄、闭塞,并导致在颅底部形成代偿微血管网络。该血管网在血管造影上的外观看起来就像一股烟雾在空中飘过,因此,在1969年,铃木和TAKAKU将这种新型疾病称为"烟雾病"。现在已知烟雾病病理学的主要特征是颈内动脉终末的进行性狭窄,且烟雾血管是扩张的穿支动脉,起到侧支通路的作用。本文就烟雾病的发病机制、流行病学、临床特征及诊断等作一综述。     

单侧烟雾病的临床特征

【摘要】
目的 探讨单侧烟雾病的临床特征及预后。
方法 回顾性分析2003年1月～2010年1月在中国人民解放军第307医院神经外科就诊的单侧烟雾病患者,对患者的发病情况、影像学检查结果、治疗结果及预后情况进行分析。
结果 78例患者中男52例,女26例,成人52例,儿童26例,其中2例患儿合并家族性烟雾病。首发症状中24例患者表现为脑梗死,短暂性脑缺血发作（transient ischemic attack,TIA）18例,头痛17例,9例表现为脑出血（8例为成人）,其他表现共10例。46例患者为右侧病变,32例患者为左侧病变。73例患者接受手术,69例患者接受随访。术后6例出现TIA,2例出现新发脑梗死,9例脑出血患者未再发生脑出血。32例患者接受血管造影复查,29例显示颅内外已建立良好的侧支循环。2例患者术后对侧血管出现狭窄或闭塞,按复查血管病变是否存在对侧进展,分为进展组和非进展组,其中进展比例在本组为6.3%（2/32）。在进展组儿童及伴有家族史所占比例均为100%,与非进展组这两项比较P值分别为0.043和0.002,差异具有显著性（P<0.05）。
结论 具有家族史的儿童患者可能是对侧进展的预测因素;手术后能建立明确的侧支循环,改善临床症状。     

烟雾病的病因学及临床诊治研究进展

烟雾病是由于颈内动脉末端出现进展性狭窄或闭塞以及大脑底部异常血管网形成而导致的脑缺血性或出血性疾病,目前病因未明。治疗上除外科手术外,尚无肯定的治疗方法。本文对近年来国内外关于烟雾病的发病机制及临床诊治研究进展作一简要综述。     

血管内介入治疗合并单侧烟雾病的前交通动脉瘤

目的探讨血管内介入治疗合并单侧烟雾病（MMD）的前交通动脉瘤临床效果。 方法回顾性分析吉林大学第一医院神经血管病外科自2017年1月至2019年12月行血管内介入治疗的8例合并单侧MMD的前交通动脉瘤患者的临床资料,观察其栓塞方法、术后效果及随访结果。 结果7例患者成功实施动脉瘤栓塞术,均顺利出院;1例动脉瘤术中破裂,术后死亡。临床及影像学随访6~10个月,平均（7.9±1.4）个月,7例患者均恢复良好,动脉瘤均未复发。 结论血管内介入治疗合并单侧MMD的前交通动脉临床效果良好,是一种相对安全、有效的方法,其临床效果仍需长期随访及更多病例进一步证实。     

儿童烟雾病脑梗死影响因素分析

目的 分析儿童烟雾病（Moyamoya disease,MMD）的临床及影像学特征。 方法 回顾性分析176例儿童缺血型烟雾病患者临床资料,分为脑梗死组和非梗死组。组间的特征 和影像学参数采用单因素分析和多因素分析,评估脑梗死的危险因素。 结果 儿童缺血型烟雾病中脑梗死好发于女童（P =0.006）。大脑前动脉（anterior cerebral artery, ACA）、大脑中动脉（middle cerebral artery,MCA）和颈内动脉（internal Carotid Artery,ICA）狭窄程度 是脑梗死发生的危险因素（P＜0.001,0.014和＜0.001）。多因素分析显示颈内动脉狭窄[比值比（odds ratio,OR）6.945,95%置信区间（confidence interval,CI）1.406～34.302,P =0.017）、脉络膜后动脉 代偿（OR 0.780,95%CI 0.078～0.324,P =0.000）、后交通动脉代偿（OR 3.288,95%CI 1.521～7.111, P =0.002）与脑梗死发生独立相关。 结论 儿童缺血型烟雾病中,脑血管狭窄程度以及侧支循环代偿情况与脑梗死发生有关。     

烟雾病的相关研究进展

烟雾病(Moyamoya disease)又称为颅底异常血管网病,是一种以双侧颈内动脉末端进行性狭窄或闭塞,颅底异常血管网形成为特征的慢性闭塞性脑血管病。该病1957年首先由Takeuchi与Shimizu以颈内动脉发育不全报告此病,由于床突以上颈内动脉及Willis环自发性进展性梗死,在颅底出现大量     

烟雾病颅内外血管参数评估研究进展

烟雾病(moyamoya disease,MMD)的特征性病变为双侧颈内动脉终末段及大脑中动脉、大脑前动脉起始段进行性狭窄闭塞,导致脑缺血及缺血后脆弱的血管破裂引起脑出血。基于脑-颈血管一体化,颅外及颅内不同节段血流状态相互影响,可通过检测特定节段血流参数提示疾病发展进程,从而指导临床策略的选择。深入了解烟雾病不同节段血流状态及疾病发展进程对疾病诊疗有重要作用。本文对烟雾病颅外及颅内不同节段血管参数、侧支循环及其与临床预后的相关性进行综述,为后续针对颅内动脉狭窄或闭塞引起的缺血性及出血性相关脑血管病诊疗与研究提供参考。     

烟雾病的临床特征及外科治疗的流行病学分析

目的探讨烟雾病的性别、年龄、地区分布特点、临床表现以及外科治疗效果分析。方法回顾性分析2017-01—2019-04河南省人民医院收治的542例烟雾病患者的临床资料,包括性别、年龄、地区分布、临床表现等,分析其手术效果及预后。结果542例烟雾病患者中,男女比例为1∶1.05(男265/女277);年龄(45±12.7)岁(3~75岁),年龄分布呈现双峰形态,好发于9~11岁儿童及46~51岁成人段;河南南方地区发病率明显高于其他区域,呈区域集中现象。儿童成人均以缺血性症状为主;缺血型以头痛、头晕为主要首发症状,出血型中以脑室出血最常见。432例接收手术治疗的烟雾病患者中,行间接血管重建术66例,联合血管重建术366例,18例围手术期出现并发症,其余均恢复顺利,临床症状得到改善,随访结果良好。结论河南地区烟雾病的发病无明显性别差异,年龄呈双峰分布,成人多见,多为缺血型,纳入病例中儿童均为缺血型;豫南地区发病率明显高于其他地区;个体化脑血管重建术具有明确的疗效。     

烟雾病的外科治疗

烟雾病(Moyamoya病)目前还没有理想的治疗方法.内科曾采用血管扩张剂、皮质激素、抗血小板聚集药物、改善微循环药物及神经营养剂等进行对症治疗,效果均不佳.部分文献报道,钙离子拮抗剂如尼莫地平、尼卡地平等对少数缺血型Moyamoya病患者有效,但缺乏有效的数据及长时间的随访.总之,内科治疗尚不能确切阻止病情的发展或防止缺血和出血的再次发作,通过外科手术进行血管重建是治疗Moyamoya病的主要方法.……     

Rapid Progression of Unilateral Moyamoya Disease

The detailed clinical characteristics of unilateral moyamoya disease (MMD) have not been fully elucidated. It has been reported that some patients with unilateral MMD progress to bilateral involvement, while others remain with the unilateral variant. In this series, we present a case of unilateral MMD that progressed to bilateral involvement over the course of just one month.     

增殖性脑血管病研究进展

增殖性脑血管病(cerebral proliferative angiopathy,CPA)是一种原因不明的慢性缺血性脑血管疾病,是以颅内存在弥漫性密集增强的巨大血管网状空间,同时具有弥漫的增生血管供血为影像学特征表现的脑血管病。其在颅内动静脉畸形中占3.4%的比重,但临床表现及预后与动静脉畸形明显不同。其表现主要为癫痫、头痛和进行性神经功能缺损。由于该疾病直到近几年才被单独提出来,截至2016年4月6日,国内尚无文献对其报道。本文就CPA的病理、临床表现、影像学特点等最新研究做综述,旨在提高对CPA的认识,为未来更好地诊治做铺垫。     

Arteriovenous Malformation with an Occlusive Feeding Artery Coexisting with Unilateral Moyamoya Disease

Background

Arteriovenous malformations (AVMs) with vascular abnormalities, including aneurysms, have been reported frequently. However, the coexistence of AVM and unilateral moyamoya disease is rare. We report herein an AVM patient who presented with acute ischemic stroke with unilateral moyamoya disease and occlusion of the feeding artery.

Case Report

A-41-year old man was admitted with sudden dysarthria and facial palsy. Brain computed tomography and magnetic resonance imaging revealed an acute infarction adjacent to a large AVM in the right frontal lobe. Cerebral angiography revealed occlusions of the proximal right middle cerebral and proximal anterior cerebral arteries, which were the main feeders of the AVM. Innumerable telangiectatic moyamoya-type vessels between branches of the anterior cerebral artery and dilated lenticulostriate arteries on the occluded middle cerebral artery were detected. However, a nidus of the AVM was still opacified through the distal right callosomarginal artery, which was supplied by the remaining anterior cerebral artery and leptomeningeal collaterals from the posterior cerebral artery.

Conclusions

While AVM accompanied by unilateral moyamoya disease is rare, our case suggests an association between these two dissimilar vascular diseases.     

Neurofibromatosis Type 1 Association with Moyamoya Disease

The neurofibromatoses are genetic disorders of the nervous system that primarily affect the development and growth of neural (nerve) cell tissues. The neurofibromatoses are classified as neurofibromatosis type 1 (NF1) and neurofibromatosis type 2 (NF2). NF1 is the more common type of the neurofibromatoses. The gene responsible for NF1 is located on the chromosome region 17q11.2 and for familial moyamoya disease on chromosome 17q25. This article reports on a 20-year-old female with neurofibromatosis-1 who developed moyamoya syndrome. More extensive reports and further investigations of such families having this combination will certainly provide a better understanding of this link in the near future.     

Quality of Life in Pediatric Moyamoya Disease

BackgroundMoyamoya disease (MMD) is a progressive intracranial arteriopathy with high risk of stroke. Its impact on quality of life is unstudied. We surveyed children with moyamoya disease and compared their quality of life to chronically ill children and children with stroke to better understand the impact of this diagnosis.MethodsChildren with moyamoya disease aged seven to 17 years from Stanford's Moyamoya Clinic between June 2014 and March 2015 were included. Children with syndromic neurodevelopmental diagnoses were excluded. Patients were surveyed using the Pediatric Quality of Life 4.0, in addition to the Pediatric Stroke Outcome Measure or Recovery Recurrence Questionnaire. Mean scores were compared to normative data sets. Linear regression models compared total quality of life scores in patients with and without stroke, after adjusting for confounders.ResultsThis cross-sectional study included 30 children with moyamoya disease; ten were male, and the median age was 13.5 years (range, 7 to 17 years). Twenty children (67%) had a stroke, and 14 of these had good neurological outcome (70%). Mean parent-proxy Pediatric Quality of Life scores were lower in all domains compared to healthy controls (P < 0.05), and all scores were comparable to chronically ill children and children with non–moyamoya disease stroke. There was no significant difference in total quality of life between patients with and without stroke.ConclusionsEven in the absence of stroke, children with moyamoya disease have lower quality of life than healthy controls and a similar quality of life to chronically ill children and those with non–moyamoya disease stroke. Children with moyamoya disease would benefit from mental health support beyond what a mild physical presentation may indicate.     

烟雾病的临床及影像研究

目的:研究烟雾病(moyamoya disease,MMD)的临床及影像学特征。方法:回顾性分析27例烟雾病,观察其临床及影像学特点。结果:本组病例多为儿童及青壮年起病,女性居多。病程为多发,主要有脑梗死、出血和短暂脑缺血发作等起病形式,临床表现为肢体麻木、无力、头痛、智力减退等症状;DSA在脑底动脉环发现血管闭塞、狭窄及烟雾状毛细血管网形成,MRA对本病有一定阳性发现。结论:对于儿童及青壮年反复发作的脑卒中患者要考虑MMD的可能,目前仍依靠数字减影血管造影确诊,MRA对本病有一定阳性发现。     

烟雾病的临床特点分析

目的:观察烟雾病的临床特点,为临床治疗缺血性脑血管病提供依据.方法:回顾性分析20例烟雾病患者的临床资料,观察其临床特点.结果:本组患者年龄在46～60岁,平均(51.25±4.40)岁,临床表现多样,头颅影像学有改变;脑血管数字减影血管造影(DSA)可见明显烟雾病表现.结论:烟雾病是一种较少见的慢性脑血管进行性闭塞性疾病,是缺血性脑血管病发病的重要原因之一,应尽早行脑血管DSA检查,及时治疗.     

烟雾病临床分型研究进展

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。     

出血型烟雾病的研究进展

【摘要】 烟雾病是一种以颈内动脉末端狭窄、闭塞并伴有颅底异常血管网形成为特征的脑血管病,出血型烟雾病是其中的一个重要类型,也是目前的研究热点,本文就出血型烟雾病的流行病学特征、病因及发病机制、影像学特征及预测出血的指标、治疗和疗效评价等做一综述。