大脑中动脉狭窄脑梗死颅内血流动力学及侧支循环研究

目的 探讨大脑中动脉(MCA)狭窄或闭塞的脑梗死患者颅内血流动力学改变和侧支循环的代偿及神经功能缺损的关系.方法 通过经颅多普勒(TCD)检查,计算双侧大脑前动脉(ACA)、大脑后动脉(PCA)峰流速及其比值(RVACA、RVPCA),并与正常对照组比较.结果 共观察38例单侧MCA狭窄或闭塞的脑梗死患者.(1)病例组ACA、PCA血流速度代偿性增快,以ACA代偿为主(76.3%);(2)病例组RVACA明显较对照组高﹙P<0.01﹚;(3)MCA主干及皮层支梗死患者的RVACA明显较对照组及深穿支梗死组高﹙P<0.01,P<0.05﹚;MCA重度狭窄或闭塞脑梗死患者的RVACA较对照组及中度狭窄组高﹙P<0.05﹚;(4)病例组RVACA及RVPCA与NIHSS呈负相关(P<0.01﹚.结论 皮质软脑膜侧支吻合血管开放成为MCA狭窄或闭塞脑梗死侧支循环的主要途径,其代偿程度与预后相关.     

DSA对单侧颈内动脉系统大动脉狭窄或闭塞后侧支循环建立的应用价值

目的 探讨DSA对单侧颈内动脉系统大动脉狭窄或闭塞后侧支循环建立的应用价值,探讨三级侧支循环在单侧颈内动脉开口部位狭窄或闭塞及大脑中动脉M1段狭窄或闭塞中的特点.方法 分别对56例颈内动脉开口处狭窄或闭塞及94例大脑中动脉M1段狭窄或闭塞的患者进行脑血管造影检查,根据其狭窄程度分析其侧支循环建立的情况.结果 颈内动脉开口部位闭塞组大脑动脉环开放率约38.5%,颅内外沟通开放率30.8%,软脑膜吻合支开放率约30.8%;重度狭窄组大脑动脉环开放率35.1%,软脑膜吻合支开放率16.2%,颅内外沟通开放率约5.4%;中轻度狭窄组无侧支循环建立.大脑中动脉M1段闭塞组大脑动脉环开放率5%,软脑膜吻合支开放率95%;重度狭窄组仅软脑膜吻合支开放,开放率约61%;轻中度狭窄组无侧支形成.结论 在颈内动脉开口部位重度狭窄或闭塞的病例中,一级侧支循环的开放代偿最为重要,二级侧支循环起着重要的辅助作用.在大脑中动脉M1段重度狭窄或闭塞的病例中,二级和三级侧支循环的开放起主要的代偿作用.     

缺血性卒中病变血管和侧支循环代偿的研究

目的 观察缺血性卒中患者的责任病变血管及其侧支循环代偿方式,探讨脑动脉闭塞或严重狭窄时侧支循环的代偿作用与牛津郡社区卒中项目(OCSP)临床症状分型之间的关系.方法对211例缺血性卒中患者采用OCSP分型(完全型前循环梗死36例,部分前循环梗死94例,后循环梗死31例,腔隙性梗死50例),进行数字减影全脑血管造影检查,判定梗死的责任血管、侧支循环是否建立及代偿方式.结果 检出有病变血管的患者198例,共累及病变血管206支,责任血管为颈内动脉98条、大脑中动脉54条、椎动脉27条、颈总动脉6条、基底动脉5条、锁骨下动脉4条、大脑前动脉及大脑后动脉各2条;经Willis环代偿98例,软脑膜支吻合115例,颅外代偿46例.结论脑动脉病变最多位于颈内动脉、大脑中动脉,其次位于椎动脉,前循环病变较后循环病变具有更高的梗死发生率;侧支循环代偿以Willis环最充分,软脑膜支吻合最常见;脑梗死的临床分型受病变血管与侧支循环代偿的综合影响.     

经颅多普勒超声对单侧MCA重度狭窄或闭塞及侧支循环的诊断与研究

目的探讨经颅多普勒超声（TCD）对单侧大脑中动脉（MCA）重度狭窄或闭塞的诊断价值以及颅内血流动力学的改变与相应软脑膜侧支（LMCs）开放的关系。方法将48例MCA重度狭窄或闭塞患者的TCD检查结果与DSA检查结果进行对比,计算TCD判断大脑前动脉（ACA）和大脑后动脉（PCA）的LMCs开放的特异性与敏感性（％）。结果TCD对MCA重度狭窄或闭塞的诊断与DSA检查的符合率为89．6％,Kappa值为0．79（P〈0．01）,呈高度一致性。以同侧ACA血流速度（iVACA）〉对侧MCA血流速度（cVMCA）为判断ACA的LMCs开放的标准,TCD的敏感性为90．5％,特异性为70．4％,Youden指数（YI）=0．61;以iVPCA〉cVwcA为判断PCA的LMCs开放的标准,TCD的敏感性为45．5％,特异性为84．6％,YI=0．30。结论TCD对MCA重度狭窄或闭塞的诊断准确性较高;TCD能间接判断MCA病变后的LMCs开放情况。     

脑梗死患者脑血管病变及侧支循环状态的研究

目的探讨脑梗死患者的责任病变血管及侧支循环主要方式。方法对110例脑梗死患者行牛津郡社区卒中项目（OCSP）分型,进行全脑数字减影血管造影（DSA）检查,判定梗死的责任血管、侧支循环是否存在及方式。结果各型患者,病变血管共137条,其中颈内动脉（ICA）54条（39．4％）、锁骨下．椎动脉（SUB—VA）42条（30．7％）、大脑中动脉（MCA）29条（21．2％）、颈总动脉（CCA）和基底动脉（BA）各4条（各2．9％）、无名动脉2条（1．5％）、大脑前动脉（ACA）和大脑后动脉（PCA）各1条（各0．7％）;责任血管ICA49条（44．5％）、MCA27条（24．5％）、SUB—VA19条（17．3％）及CCA3条（2．7％）、IA2条（1．8％）,余3支血管各1条（均占0．9％）。7例（6．4％）未发现异常。经软脑膜支吻合代偿59例,Willis环代偿49例,来自各种颅外代偿计27例,两种及以上方式联合代偿19例,罕见代偿3例,未发现代偿6例。结论脑动脉病变最多位于ICA、SUB-VA主干,其次位于MCA;前循环病变较后循环病变具有更高的梗死发生率。侧支循环代偿以Willis环最充分,软脑膜支吻合最常见;脑梗死的类型和预后受血管病变和侧支循环状态的综合影响。     

脑白质区域非腔隙性梗死灶与颅内外血管狭窄关系的探讨

目的 探讨脑白质区域非腔隙性梗死灶与颅内外血管狭窄的关系.方法 对30例脑白质区域非腔隙性梗死患者的头颅MRI以及主动脉弓、全脑数字减影血管造影(DSA)检查资料进行分析.结果 本组MRI示12例单侧基底节区片状异常信号中,DSA表现为一侧颈内动脉(ICA)起始部闭塞或高度狭窄9例,一侧大脑中动脉(MCA)M1段高度狭窄2例,无明确血管病变1例.6例基底节以及侧脑室旁白质区域病灶中,一侧ICA起始部闭塞或高度狭窄3例,一侧ICA C5段闭塞1例,一侧MCA M1段闭塞2例.4例侧脑室旁或半卵圆中心白质区域病灶中,一侧ICA C6段闭塞1例,一侧MCA M1段高度狭窄2例,无明确血管病变1例.8例皮质下上型或皮质下侧型分水岭脑梗死患者中,一侧ICA起始部闭塞或高度狭窄6例,双侧ICA起始部闭塞1例,一侧MCA M1段高度狭窄1例.结论 脑白质区非腔隙性梗死灶的发生与ICA系统大血管的狭窄或闭塞有密切的关系.     

CTA评估分水岭脑梗死患者侧枝循环的初步研究

目的初步探讨侧枝代偿在分水岭脑梗死(CWI)发病中的作用。方法应用CT血管成像(CTA)技术,回顾性分析于我院住院治疗的139例分水岭脑梗死(包括皮质型、内侧型及混合型)患者的颅内动脉,比较各组患者相关血管病变及侧枝代偿情况。结果 (1)CTA显示110例患者存在颈动脉系统病变,其中49例为颈内动脉(ICA)狭窄/闭塞,61例为大脑中动脉(MCA)狭窄/闭塞;(2)在内侧型分水岭梗死(IWI)患者中,存在MCA狭窄/闭塞者(46例)高于ICA狭窄/闭塞(31例)和无明显狭窄(16例),差异有统计学意义(P=0.000);(3)ICA狭窄/闭塞时,前交通动脉(ACOA)未开放者在IWI患者所占比例(25/31例)明显多于皮质型分水岭梗死患者(5/11例)和混合型分水岭梗死患者(3/7例),差别有统计学意义(P=0.033);(4)MCA狭窄/闭塞时,软脑膜侧枝代偿较差者在皮质型分水岭梗死患者所占比例(6/9例)明显多于IWI患者(11/46例)和混合型分水岭梗死患者(1/6例),差别有统计学意义(P=0.028)。结论颈动脉系统病变所致的IWI受MCA的狭窄/闭塞影响程度更大;ICA狭窄/闭塞所致IWI梗死与ACOA缺乏或发育不良有关,ACOA的存在可以明显减少IWI发生;MCA病变时软脑膜代偿的出现可增加受累皮质的血供,软脑膜侧枝代偿较差可促进皮质型分水岭脑梗死的发生。     

甲状腺功能亢进症合并烟雾病1例报告并文献复习

<正>甲状腺功能亢进症(hyperthyroidism,简称甲亢)是甲状腺腺体本身合成释放过多的甲状腺激素引起以神经、循环、消化等系统兴奋性增高和代谢亢进为主要表现的一组临床综合征。而烟雾病(moyamoya disease,MMD)又称脑底异常血管网病,是双侧颈内动脉虹吸部及大脑前动脉、大脑中动脉起始部严重狭窄或闭塞,软脑膜动脉、穿通动脉等小血管代偿增生形成的脑底异常血管网为特征的一种脑血管病。     

幕上腔隙性脑梗死的发病机制分析

目的通过数字剪影脑血管造影(DSA)及MRI成像分析幕上腔隙性脑梗死发病机制特点。方法对30例MRI确诊的急性幕上腔隙性脑梗死患者的脑血管造影结果及MRI成像结果进行分析。结果 30例患者中梗死同侧大脑中动脉(MCA)M1段狭窄(狭窄≥50%)10例,颈内动脉(ICA)末端狭窄或闭塞(狭窄≥50%)5例,大脑前动脉(ACA)A1段狭窄(狭窄≥50%)3例,颈内动脉颅外段狭窄或闭塞(狭窄≥70%)3例,烟雾病2例,仅发现同侧颈内动脉后交通动脉瘤1例,未发现可分辨的责任狭窄6例(SWI显示基底节区或皮质下微出血灶者4例,SWI显示正常者2例);SWI发现基底节区或皮质下微出血灶者10例。结论幕上腔隙性脑梗死患者前循环大血管狭窄者占有相当比例,单纯穿支小血管本身病变者比例较小。故对于临床诊断腔隙性脑梗死者,不要仅认为是穿支本身病变,需进一步评估大血管,筛查出可以进一步干预特别是内科治疗无效的血管内干预,从而改善患者预后。     

TCD动态监测软脑膜侧支循环状态评估急性缺血卒中患者预后的可行性分析

目的通过经颅多普勒超声(transcranial Doppler,TCD)动态监测大脑中动脉(middle cerebral artery,MCA) M1段慢性闭塞所致急性缺血性卒中患者病程中软脑膜侧支循环建立及动态变化情况,评价其对预后的影响。方法收集单侧MCA M1段慢性闭塞所致的急性缺血性卒中患者,分别于入院时、7 d、14 d、21 d、60 d、90 d行TCD检查,以不同软脑膜侧支存在状态分为FD(flow diversion,FD)阳性组及FD阴性组,同期行NIHSS评分,记录90 d mRS评分,比较不同FD存在状态患者预后。结果共纳入符合条件患者57例,FD阳性43例(75%),FD阴性14例(25%),病程中软脑膜侧支循环存在状态相对稳定。FD阳性组NIHSS评分低于FD阴性组(P 0. 05),90 d mRS评分优于FD阴性组(P 0. 05)。结论 CA慢性闭塞的急性缺血性卒中患者,病程中软脑膜侧支循环存在状态相对稳定,且具有良好的代偿作用,可显著改善患者近期及远期预后,降低卒中进展风险。TCD评价软脑膜侧支循环可作为此类患者预后评估的工具。     

30例烟雾病的临床及影像学特征分析

目的研究烟雾病的临床特点、影像表现及其两者之间关系。方法对30例经磁共振血管成像(MRA)和数字减影全脑血管造影(DSA)确诊的烟雾病患者,分析其临床和影像学特征。结果(1)烟雾病发病年龄在35～45岁左右,发病以脑缺血为主,临床表现以单侧肢体无力或偏瘫最为常见。(2)CT和MRI提示,脑出血11例(36.7%),脑梗死10例(33.3%),脑出血合并梗死灶5例。MRA和DSA均发现大脑前、中、后动脉狭窄或闭塞性病变,双侧病变均多于单侧病变,烟雾状异常血管网分别为5例(45.5%)和29例(100.0%);DSA发现动脉瘤3例(10.3%);后交通动脉增粗13例、前交通动脉增粗7例、眼动脉增粗6例及软脑膜吻合支增多4例。4例大面积脑梗死侧枝循环差。结论烟雾病发病高峰为35～45岁;本病临床表现多样,但以脑缺血常见;MRI和MRA为无创伤性,为本病筛选、诊断、随访的重要方法。对临床疑似病例应及早行DSA检查。     

Treatment of moyamoya syndrome in children

Moyamoya syndrome, a known cause of pediatric stroke, is a chronic cerebrovascular occlusive disorder of unknown etiology that can lead to severe, permanent neurologic disability. It is characterized by progressive stenosis of the distal intracranial carotid arteries and branches of the circle of Willis and the nearly simultaneous appearance of basal arterial collateral vessels, which vascularize hypoperfused brain distal to the occluded vessels. Most children with moyamoya syndrome present either with transient ischemic attacks or strokes. This review summarizes the current medical and surgical practices used in the treatment of moyamoya syndrome in children.     

Moyamoya disease: current concepts and future perspectives

Moyamoya disease is an uncommon cerebrovascular disease that is characterised by progressive stenosis of the terminal portion of the internal carotid artery and its main branches. The disease is associated with the development of dilated, fragile collateral vessels at the base of the brain, which are termed moyamoya vessels. The incidence of moyamoya disease is high in east Asia, and familial forms account for about 15% of patients with this disease. Moyamoya disease has several unique clinical features, which include two peaks of age distribution at 5 years and at about 40 years. Most paediatric patients have ischaemic attacks, whereas adult patients can have ischaemic attacks, intracranial bleeding, or both. Extracranial-intracranial arterial bypass, including anastomosis of the superficial temporal artery to the middle cerebral artery and indirect bypass, can help prevent further ischaemic attacks, although the beneficial effect on haemorrhagic stroke is still not clear. In this Review, we summarise the epidemiology, aetiology, clinical features, diagnosis, surgical treatment, and outcomes of moyamoya disease. Recent updates and future perspectives for moyamoya disease will also be discussed.     

Are the strokes in moyamoya syndrome associated with Down syndrome due to protein C deficiency?

Moyamoya syndrome has occasionally been seen in association with Down syndrome. We report a child with moyamoya syndrome and Down syndrome who was admitted with repeated episodes of strokes; his magnetic resonance imaging and magnetic resonance angiography findings confirmed the presence of occlusive cerebrovascular disease with basal collateral vessels. His protein C levels were significantly decreased during the stroke. Complete clinical recovery was seen during follow-up. This raises the possibility of a link between protein C deficiency and Down syndrome with moyamoya syndrome.     

Fetal posterior cerebral artery in pediatric and adult moyamoya disease: A single-center experience of 480 patients

PurposeIn past pediatric and adult cohort studies of moyamoya disease, the fetal posterior cerebral artery has received less attention. Its relationship with the clinical manifestations and collateral circulation of moyamoya disease or ipsilateral cerebral hemispheres remains unclear.MethodWe summarize the clinical features of patients with and without fetal posterior cerebral artery moyamoya disease from consecutive cases.We explored the relationship between fetal posterior cerebral arteries and collateral circulation in the ipsilateral cerebral hemispheres, as well as differences among different subgroups of patients.According to the morphology, the fetal posterior cerebral artery is divided into complete fetal posterior cerebral artery and partial fetal posterior cerebral artery. Clinical features were classified as: infarction,hemorrhage,and non-stroke in unilateral/bilateral cerebral hemispheres. Collateral circulation is divided into extracranial vascular compensation and leptomeningeal collateral circulation. Digital subtraction angiography and CT/MR were used to evaluate the blood flow status and clinical characteristics of patients with moyamoya disease.ResultA total of 960 cerebral hemispheres from 142 pediatric patients and 338 adult patients were included in the study. A total of 273 (56.9%) patients had 399 cerebral hemispheres (41.6%) with fetal posterior cerebral arteries. Adults with fetal posterior cerebral arteries had lower rates of infarction (24.6%vs37.3%, P =0.005) and were less likely to have bilateral stroke (8.4%vs11.5%, P =0.038). Cerebral hemispheres with fetal posterior cerebral artery were more likely to have anterior cerebral artery and middle cerebral artery stenosis and less likely to have occlusion (P =0.002, 0.001), and less likely to involve the posterior circulation (P < 0.001). The cerebral hemispheres of the fetal posterior cerebral artery had higher leptomeningeal collateral circulation scores. There are significant differences in extracranial vascular compensation between cerebral hemispheres with and without fetal posterior cerebral artery. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage (P =0.017).ConclusionsOur results suggest that fetal posterior cerebral artery is associated with infarct manifestations in pediatric and adult moyamoya disease. In the cerebral hemispheres, the fetal posterior cerebral artery is associated with ipsilateral hemispheric anterior and posterior circulation artery injury, extracranial vascular compensation, leptomeningeal collateral circulation compensation, and infarction. Adult patients with fetal posterior cerebral artery were more advanced in Suzuki stage.     

甲状腺功能亢进合并烟雾病发病机制的研究进展

烟雾病是一种以双侧颈内动脉末端及大脑前动脉、大脑中动脉起始部慢性进行性狭窄或闭塞为特征，并继发颅底异常血管网形成的脑血管疾病。甲状腺功能亢进继发烟雾病的发病机制尚不清楚，可能与甲状腺功能、免疫与炎症、遗传因素等有关。免疫和炎症相关的分子变化对血管内皮细胞和平滑肌细胞的影响，可能在颅内大动脉狭窄、闭塞和烟雾血管的形成中起着重要作用。本文对甲状腺功能亢进继发烟雾病的可能病理生理学机制、免疫与炎症、遗传因素等发病机制进行综述。     

Moyamoya syndrome associated with sickle cell trait in a child

Moyamoya syndrome is a chronic, progressive occlussion of cerebrovascular arteries that leads to the development of characteristic collateral vessels. It is usually accompanied with sickle cell disease and other hemoglobinopathies. We report a 7-year-old boy, who admitted to our clinic with headache, diagnosed as moyamoya syndrome associated with sickle cell trait. To date, two such cases have been reported in the literature. As far as we know, this patient is the first child reported.     

Childhood moyamoya disease and moyamoya syndrome: a pictorial review

Moyamoya disease is an uncommon chronic cerebrovasculopathy, characterized by progressive stenosis of the terminal portion of the internal carotid artery and its main branches, in association with the development of compensatory collateral vessels at the base of the brain. The etiology is unknown, and was originally considered exclusive to East Asia, with particular prevalence in Japan. Moyamoya disease is increasingly diagnosed throughout the world, and represents an important cause of childhood stroke in Western countries. In some cases, similar angiographic features are evident in children with other medical conditions, such as sickle cell disease and Down syndrome. In these instances, the term "moyamoya syndrome" is used. Diagnosing the vasculopathy, excluding possible associated conditions, and planning treatment and follow-up imaging comprise important aspects of clinical management. We review the key imaging features of childhood moyamoya disease and syndrome, present examples of its associations, and discuss new neuroradiologic methods that may be useful in management.     

Efficacy of Direct Revascularization Surgery for Hemorrhagic Moyamoya Syndrome As a Late Complication of Cranial Irradiation for Childhood Craniopharyngioma

Moyamoya syndrome (MMS) is an uncommon late complication after cranial irradiation. Its hemorrhagic presentation from the associated pseudo-aneurysm is extremely rare, and the optimal management strategy is undetermined. We herein report a 36-year-old man who developed intraventricular hemorrhage from a pseudo-aneurysm at the extended left anterior choroidal artery as an abnormal collateral of MMS 30 years after surgical removal and cranial irradiation for childhood craniopharyngioma. Catheter angiography confirmed the diagnosis of MMS, and multiple pseudo-aneurysms were evident at the ipsilateral abnormal choroidal collateral, one of which was considered to be a source of bleeding. The patient underwent left superficial temporal artery (STA)-middle cerebral artery (MCA) anastomosis with indirect pial synangiosis based on the observation that the development of choroidal collateral may be associated with a high rebleeding risk in hemorrhagic moyamoya disease. The patient was discharged without neurological deficit, and postoperative magnetic resonance angiography confirmed the STA-MCA bypass to be patent. Catheter angiography 1 year after revascularization surgery revealed the complete disappearance of the pseudoaneurysms with the apparently patent STA-MCA bypass. The patient did not exhibit any cerebrovascular events during the follow-up period of 16 months. In conclusion, hemorrhagic MMS with choroidal collateral as a dangerous anastomosis was effectively managed by STA-MCA anastomosis. Although long-term follow-up is necessary to evaluate our strategy, the favorable disappearance of pseudoaneurysms after revascularization surgery in the present case strongly suggests that STA-MCA anastomosis has a potential role for preventing rebleeding in MMS after cranial irradiation.     

Disappearance of aneurysms associated with moyamoya disease after STA-MCA anastomosis with encephaloduro myosynangiosis

Moyamoya disease is a rare cerebrovascular disease characterized by steno-occlusive vasculopathy affecting the terminal internal carotid arteries. Although the effect of direct arterial bypass on the prevention of recurrent haemorrhage or ischemic events in patients with hemorrhagic moyamoya disease has been demonstrated, disappearance of aneurysms associated with moyamoya disease has rarely been reported. In this study, we present two patients with aneurysms associated with moyamoya disease. After superficial temporal artery to middle cerebral artery anastomosis combined with encephaloduro myosynangiosis, the aneurysms on the moyamoya vessels disappeared, which was confirmed by follow-up angiography.