以视功能障碍为首发症状的脑血管病15例临床特征分析

目的 总结归纳以视力下降、视野缺损为首发症状的脑血管病临床特征。方法 对2008年1月至2009年12月间北京同仁医院神经内科收治的以视力下降、视野缺损为首发症状的脑血管病15例患者进行回顾性分析。结果 患者平均年龄为55±14岁;前循环与后循环病变的平均年龄分别为41±8岁（32～52岁）与60±13岁（42～81岁）。患者主诉包括双眼先后视力下降、单眼视力下降、双眼同时视物不见、双眼偏侧视物不见。诊断烟雾病2例、颈动脉闭塞1例、前循环脑梗死1例、脑血管畸形1例、后循环脑梗死10例。单眼视功能障碍的3例患者均为前循环病变。后循环病变中单或双侧枕、颞叶梗死7例;基底动脉尖综合征1例;脑干梗死2例。结论 以视功能障碍为首发症状的脑血管病可以是前循环病变,也可以是后循环病变;单眼症状为前循环病变,是颈内动脉缺血、闭塞导致的眼征。双眼同时起病及偏盲多是后循环疾病累及枕颞叶所致。     

后循环血管重建手术治疗椎基底动脉闭塞疗效初探

目的探讨后循环血管重建手术治疗双侧椎动脉或基底动脉闭塞的术式选择和疗效。方法分别采用枕动脉小脑后下动脉、颞浅动脉小脑上动脉、颞浅动脉大脑后动脉,以及移植桡动脉行枕动脉椎动脉寰椎上段吻合术等术式治疗9例双侧椎动脉或基底动脉闭塞患者。结果术中吲哚菁绿荧光血管造影显示所有患者吻合血管形态良好、血流通畅;术后第7天脑血管造影检查,8例吻合血管血流通畅、1例吻合血管未显影,MR(或CT)灌注成像7例血流灌注明显改善。术后患者头晕症状完全消失(2例)、构音障碍减轻及吞咽功能好转(5例)。9例患者中仅1例于术后15d死于心肺功能衰竭,其余8例随访期间均未发生脑缺血症状,4例基本恢复正常生活。结论根据椎基底动脉闭塞部位选择相应后循环血管重建手术方式,初步结果显示效果良好,但仍需大样本随机对照研究予以验证。     

烟雾病的MSCTA分析研究

目的进一步提高对烟雾病的理解和认识。方法收集本院30例证实为烟雾病患者MSCTA影片(包括原始轴位影片、VR、MIP、MPR后处理影片),结合脑血管造影结果及文献资料进行分析。结果 30例患者中均有不同程度颈内动脉系统狭窄及闭塞,单侧者10例,双侧者20例;大脑后动脉狭窄或闭塞者9例;大脑后动脉及颈外动脉系统侧支循环形成20例;合并动脉瘤者11例。所有病例均可见异常血管网,部分患者行DSA检查,结果基本一致。结论烟雾病为脑底动脉进行性狭窄、闭塞及广泛侧支循环形成的疾病,它不仅累及颈内动脉系统,还累及椎-基底动脉系统和颈外动脉系统。MSCTA可代替DSA作为烟雾病的首选检查方法。     

Moyamoya disease. Posterior cerebral artery occlusion and pattern-reversal visual-evoked potential

Although vascular abnormality in moyamoya disease predominates in the anterior and middle cerebral arteries, the posterior cerebral artery (PCA) has been found to be involved in the course of the disease. To explore PCA occlusion by noninvasive means, we studied visual-evoked potentials in the patients with PCA occlusion (occlusive group), as well as in those without PCA occlusion (nonocclusive group). The results were compared with those of other examinations that also detected an occipital lobe pathologic condition. Abnormalities of those examinations were highly specific to PCA occlusion. Positron emission tomography and pattern-reversal visual-evoked potentials yielded high incidence of abnormality in the occlusive group (86% and 75%, respectively), and expressed the side of PCA occlusion if the occlusion was unilateral. Since pattern-reversal visual-evoked potentials is popular and a low-cost examination compared with positron emission tomography, we conclude that pattern-reversal visual-evoked potentials is the most practical mean to explore PCA occlusion in the course of moyamoya disease.     

数字减影血管造影对缺血性脑血管病诊断及治疗的意义

目的:探讨数字减影血管造影检查(DSA)对缺血性脑血管病血管病变诊断的意义。方法:选取有脑缺血症状的经TCD检查发现血管狭窄的21例病人,行DSA检查。结果:颈内动脉系统TIA患者3例,DSA证实为大脑中动脉狭窄2例,烟雾病1例,椎基底动脉供血不足8例中双侧椎动脉发育不全1例,一侧或双侧椎动脉狭窄5例,锁骨下动脉盗血2例。8例脑梗塞患者中已经出现一侧颈内动脉闭塞的有两例,但均已出现侧枝循环的建立,另6例均为颈内动脉或大脑中动脉的狭窄,2例脑干梗塞患者均有椎动脉的狭窄。结论:数字减影血管造影是评价头颈部血管狭窄、闭塞和选择治疗方案的金标准,它对缺血性脑血管病的进一步治疗起着重要的作用。     

Visual disturbances in moyamoya disease

Visual disturbances in moyamoya disease are evaluated. Forty-three among our series of 178 cases of moyamoya disease were suffered from various visual symptoms such as visual field defect, attack of blindness, decreased visual acuity, scintillating scotoma. These visual manifestations were resulted from the occlusive lesions in the posterior circulation. In comparison with the cases of adult onset, those of juvenile onset tend to develop visual disturbances. STA-MCA anastomosis with EMS exerts indirect redistribution effect upon the posterior circulation in moyamoya disease. In most cases, improvement in the visual disturbances followed these bypass operations. However, in five cases, omentum transplantation to the occipital lobe as the method of direct revascularization were needed.     

Additional Revascularization Using Multiple Burr Holes for PCA Involvement in Moyamoya Disease

In specific cases of moyamoya disease (MMD), posterior cerebral artery (PCA) stenosis can develop after treatment of the anterior circulation and require additional revascularization. Here, we report two cases that underwent additional posterior indirect revascularization with multiple burr holes for PCA involvement after bilateral revascularization treatment of the anterior circulation. They presented with transient ischemic attack even after bilateral superficial temporal artery–middle cerebral artery bypass, and magnetic resonance angiography (MRA) showed that PCA stenosis had worsened. Indirect revascularization with multiple burr holes using Benz-marked skin incisions was performed. After surgery, the symptoms improved without perioperative complications, and cerebral angiography showed collateral circulation via the burr hole. Indirect revascularization for MMD is often combined with direct revascularization, and there are only a few reports on the use of multiple burr hole surgery alone. In addition, there are few reports of posterior circulation, despite the emphasis on the importance of PCA involvement in MMD. Indirect revascularization with multiple burr holes alone can be performed in multiple areas and applied to patients who cannot undergo direct revascularization using the occipital artery. The procedure is simple and less invasive than traditional direct revascularization procedures. Therefore, it can be effective, especially in pediatric cases of MMD with PCA involvement.     

Pathological and immunohistochemical findings of an autopsy case of adult moyamoya disease

Moyamoya disease is vaso‐occlusive disease involving the arteries of the circle of Willis that is accompanied by a compensatory recruitment of a vascular network. The pathological and immunohistochemical findings of an autopsy case of hemorrhagic moyamoya disease in a 69‐year‐old woman are described in the present report. The autopsy findings of the brain revealed cerebral and intraventricular hemorrhage with edema. The left anterior cerebral artery, bilateral middle cerebral arteries and left posterior cerebral artery were marked narrowing, and the other arteries revealed mild narrowing. Microscopically, the arteries of the circle of Willis showed narrowed lumen, fibrocellular intimal thickening, marked tortuousness of internal elastic lamina and attenuation of media. The thickened intima was composed of smooth muscle cells. The vessels with dilated or irregular‐shaped lumen suggested abnormal vascular networks demonstrated by angiography. In this case, no correlation between the abnormal vascular network and expression of VEGF or VEGF receptor was disclosed. It was hypothesized that abnormal vascular networks might be composed of collateral vessels in relation to various pathological changes of the arteries, such as occlusion and stenosis, and intracranial hemorrhage in patients with moyamoya disease might occur as a result of rupture of arteries including abnormal vascular networks.     

DSA在烟雾病诊断中的临床应用研究

目的探讨烟雾病DSA检查的影像学表现及临床意义。方法回顾性分析26例确诊烟雾病患者临床及DSA影像学资料。结果26例患者DSA影像学特征如下:脑底异常烟雾状血管网形成;受累动脉狭窄或闭塞;丰富侧支循环形成。其中单/双侧大脑前、中及后动脉均存在不同程度的狭窄或闭塞性病变者24例(92.3%);双侧颈内动脉床突上段狭窄或闭塞者18例(69.2%),单侧者7例(26.9%)。结论烟雾病患者有显著的DSA影像学特征,DSA是诊断烟雾病的主要手段,临床上对疑似病例应早行DSA检查明确诊断。     

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.     

Characteristics of vascular lesions in patients with posterior circulation infarction according to age and region of infarct

Patients with posterior circulation infarction underwent CT angiography and magnetic resonance angiography. Intracranial and extracranial vasculopathy was evaluated according to age group and location of stroke. Patients aged > 60 years and < 60 years had similar rates of vertebral artery dominance and vertebrobasilar artery developmental or origin anomalies. Vertebrobasilar artery stenosis or occlusion and tortuosity occurred more frequently in patients aged > 60 years than < 60 years. The rates of vertebrobasilar artery anomalies and tortuosity were high in patients with posterior circulation infarction. Vertebrobasilar artery tortuosity occurred more frequently in patients aged > 60 years, whereas vertebrobasilar artery developmental anomalies occurred with similar frequency in patients aged < 60 years and > 60 years. Patients with infarction of the brainstem or cerebellum were more likely to have vertebral artery stenosis or occlusion, basilar artery stenosis or occlusion, vertebral artery dominance or tortuosity, and basilar artery tortuosity, and patients with infarction of the thalamus, medial temporal, or occipital lobes were more likely to have stenosis or occlusion of the vertebral or basilar arteries. Vertebrobasilar artery tortuosity, vertebral artery dominance (hypoplasia), and congenital variations of the vertebrobasilar system may lead to posterior circulation infarction at different locations in different age groups.     

儿童缺血型烟雾病的临床和影像分析

【摘要】
目的 分析儿童缺血型烟雾病患者的临床和影像特点,探讨脑梗死的相关因素。
方法 回顾性分析2011年6月～2012年6月收治的27例儿童缺血型烟雾病患者的临床及影像学资料。
大脑半球分为无脑梗死半球32侧和脑梗死半球22侧,将患者性别、年龄、造影特点（包括54侧半球
的Suzuki分期、大脑后动脉分期,眼动脉、颞浅动脉、脑膜动脉、枕动脉、上颌动脉和后循环向前循
环代偿）与脑梗死的发生进行统计分析。
结果 27例患者的临床资料显示性别和年龄与脑梗死的发生差异不具有显著性（性别χ2=2.095,
P =0.148;年龄χ2=4.286,P =0.453）。根据半球计算：皮质脑梗死17侧（17/22）,额叶梗死14侧
（14/22）,皮质下脑梗死9侧（9/22）。无梗死侧半球和梗死侧半球Suzuki分期以及大脑后动脉分期
的差异具有显著性（Suzuki分期Z=－3.054,P =0.002;大脑后动脉分期Z=－4.557,P <0.001）。眼动脉、
颞浅动脉、脑膜动脉、枕动脉、上颌动脉和后循环向前循环代偿与脑梗死的发生差异不具有显著性。
结论 本研究显示儿童缺血型烟雾病皮质脑梗死较皮质下脑梗死常见。在皮质脑梗死中,额叶脑
梗死最常见。脑梗死的发生与Suzuki分期和大脑后动脉受累程度有关。     

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.     

Regional cerebral hemodynamics in childhood moyamoya disease

Regional cerebral blood flow (rCBF) and its reactivity to acetazolamide were analyzed in a series of 15 cases of childhood moyamoya disease, using the xenon-133 inhalation method and single photon emission CT (SPECT). Most of the patients had normal mean hemispheric cerebral blood flow (mCBF), comparable to that of age-matched healthy children. However, they had abnormal rCBF distribution and disturbed reactivity to acetazolamide in the territory of the internal carotid artery, especially in the frontal lobe. Surgical revascularization for the anterior circulation, which consisted of STA-MCA anastomosis and indirect synangiosis, dramatically improved theses cerebral hemodynamics in the anterior circulation, including the frontal lobe, and reactivity to acetazolamide in the whole brain. These results suggest that surgical revascularization for the anterior circulation should be carried out in order to improve cerebral hemodynamics as widely as possible, especially in the frontal lobe, and may improve cerebral perfusion reserve in the posterior circulation.     

能谱计算机断层扫描容积螺旋穿梭扫描技术在烟雾病中的初步应用研究

目的 探讨能谱计算机断层扫描（computed tomography,CT）容积螺旋穿梭扫描技术在烟雾病患者血管和灌注成像中的应用价值。 方法 对4例术前、11例术后的烟雾病患者行容积螺旋穿梭扫描检查,获得四维CT血管成像及全脑CT灌注成像。采用CT平扫分型、颈内动脉分期、侧支循环分类、烟雾状血管丰富程度分期、CT灌注异常分期及Alberta卒中早期CT评分的评估标准,分左右大脑半球综合评价烟雾病的脑实质和脑血管病变。 结果 术前4例烟雾病患者,8个半球中的5个半球（62.5%）处于颈内动脉分期的III期;本组侧支循环主要来源于颅底血管网（46.1%）;梗死分期中,8个半球（100%）出现异常,其中7个半球（87.5%）处于梗死前期的Ⅱ期,且Alberta卒中早期CT评分均在10分以上。术后11例（22个半球）患者均行颞浅动脉-大脑中动脉搭桥术,其中左侧6例,右侧4例,双侧1例,吻合血管通畅率为100%;梗死分期中19个半球（86.4%）出现异常,其中10个半球（45.5%）处于II期、8个半球（36.4%）处于Ⅲ期。 结论 能谱CT容积螺旋穿梭技术可以同时获得四维CT血管成像及全脑CT灌注成像,能对判断烟雾病患者的动脉分期、侧支循环、脑灌注情况、术后血管吻合等影像学信息发挥重要作用。     

Analysis of ischemic cerebral lesions using 3.0-T diffusion-weighted imaging and magnetic resonance angiography after revascularization surgery for ischemic disease

Background

Cerebral revascularization surgery (CRS) is increasingly recognized as an important component in the treatment of complex cerebral vascular disease and tumors. CRS requires that the incidence of perioperative neurological complications should be minimized, because CRS for ischemic disease is often not the goal of treatment, but rather a prophylactic surgery. CRS carries the risk of focal postoperative neurological deficits. Little has been established concerning mechanisms of post-CRS ischemia. We used 3.0-T diffusion-weighted magnetic resonance imaging (DWI) and magnetic resonance angiography (MRA) to analyze the incidence and mechanism of ischemic lesions.

Methods

We studied the anterior circulation territory after 20 CRS procedures involving 33 vascular anastomosis procedures (13 double anastomoses and 7 single anastomoses) in 12 men and 8 women between June 2007 and October 2011. The operations included single or double superficial temporal artery–middle cerebral artery (STA–MCA) anastomosis to treat internal carotid artery/MCA occlusions or severe MCA stenosis. A combined STA–MCA anastomosis and indirect bypass were performed for moyamoya disease. Postoperative DWI and MRA were obtained in all patients between 24 and 96 h after surgery to detect thromboembolism, hypoperfusion, or procedural ischemic complications and vasospasms of the donor STA.

Results

Follow-up DWI and MRA were carried out 1.8 ± 0.6 days after CRS (range, 1–4 days). Temporary occlusion time for anastomoses averaged 18.9 min (range, 16–32 min). Asymptomatic new hyperintensities occurred in the ipsilateral hemisphere of 2 patients on postoperative DWI (10% patients/6.0% anastomoses), and 1 moyamoya patient (5.0% patients/3.0% anastomoses) developed a symptomatic hyperintensity in the ipsilateral occipital lobe in response to the operation. Two abnormal small (<5 mm) cortical DWI lesions were caused by sacrifices of a small branch of the recipient MCA.

Conclusion

This study is the first postoperative 3.0-T DWI study of CRS and related clinical events. The incidence of symptomatic postoperative DWI abnormalities was restricted to 1 moyamoya patient representing 5.0% of total patients and 3.0% anastomoses. Although some postoperative DWI abnormalities occurred, CRS was found to be safe with a low risk of symptomatic ischemia.     

Repeated vertebrobasilar ischemic stroke caused by an intracranial vertebral artery dissection not detected on the initial angiogram: a case report]

Spontaneous dissection of the intracranial vertebral artery has been increasingly recognized as a cause of vertebrobasilar ischemic stroke. However, little is known about its natural history and clinical course. The authors report a young patient with repeated cerebral infarction in the posterior cerebral circulation caused by dissection of the vertebral artery, which was not detected on the first angiogram. The otherwise healthy 22-year-old male suddenly developed visual disturbance. Neuroradiological examination revealed right occipital infarction, but cerebral aniography revealed no caliber change or other pathological findings. He was treated with antiplatelet therapy. Two years later, he suffered vertigo, nausea, and dysarthria due to newly developed left cerebellar and medullary infarction. Cerebral angiography revealed left vertebral artery occlusion. T1-weighted MR image demonstrated Gadlinium-enhanced intramural hematoma in the occluded left vertebral artery, which was compatible to the arterial dissection. The present case argues serial neuroimaging studies especially in young patients with vertebrobasilar stroke in order to rule out the arterial dissection, even if initial angiography failed to demonstrate any radiographical evidence.     

联合血管重建术治疗烟雾病疗效评价

目的 探讨联合血管重建术治疗烟雾病的疗效.方法 23例缺血型烟雾病患者均采用颞浅动脉-大脑中动脉分支吻合与脑-硬膜-肌肉血管融合术（STA-MCA＋ EDMS）手术方法.术前、术后1周及术后3个月行mRS评分,并测量术前、术后1周手术侧大脑中动脉分布区的CT灌注参数.结果 22例患者术后1周行DSA或CTA检查示吻合口通畅,1例吻合血管闭塞.术后3个月mRS评分与术前、术后1周两组比较,差异有统计学意义（P＜0.05）.所有患者手术侧大脑中动脉分布区CT灌注成像各参数分别行分析,结果显示：术后1周示CBF增加,MTT、TTP缩短,与术前比较差异有统计学意义（P＜0.05）.结论 联合血管重建术能改善缺血型烟雾病患者症状.     

Blood supply of the posterior cerebral artery by the carotid system on angiograms

Background and Purpose Occipital lobe infarcts are traditionally attributed to vertebrobasilar disease. However, anatomical studies indicate that in some people the supply of the posterior cerebral artery is via the carotid system. We investigated how often such a developmental variant in the cerebral blood supply was present during life. Methods We retrospectively studied 212 conventional four-vessel cerebral angiograms. Eighteen subjects were excluded beforehand, because of vascular abnormalities causing important hemodynamic changes. We determined whether a fetal variant was present, and in other cases whether there was a functioning posterior communicating artery. Results In 11 % of hemispheres the posterior cerebral artery was exclusively supplied by the internal carotid artery; in a further 46 % of hemispheres the internal carotid artery might contribute, via a patent posterior communicating artery. In 75 % of subjects the internal carotid artery contributed in at least one hemisphere to the blood flow of the posterior cerebral artery. Conclusions The implication of our findings is that an occipital lobe infarct can be caused by ipsilateral carotid disease in a proportion of cases between 10 and 60 %. This implies that carotid endarterectomy might be beneficial in some patients with severe carotid stenosis and infarction in the territory of the posterior cerebral artery. Received: 21 February 2001, Received in revised form: 16 August 2001, Accepted: 1 October 2001     

A case of internal carotid artery occlusion successfully treated by encephalo-duro-arterio-synangiosis

In many cases, intracranial occlusive strokes in children are treated symptomatically, with a diagnosis of acute infantile hemiplegia. This is because angiography is more difficult in children and, even if occlusive cerebral arterial disease is diagnosed, there is no definite therapeutic procedure. A case is reported of left internal carotid artery occlusion presenting with acute infantile hemiplegia and followed by repeated transient ischemic attacks. Encephalo-duro-arterio-synangiosis — developed as an operation for moyamoya disease — was performed on this patient, resulting in a marked revascularization of the brain in 8 postoperative months with good improvement in symptoms. This suggests that the operation may be effective in chronic cerebral ischemic diseases other than moyamoya disease.