Moyamoya病发生,发展及转归实验研究

目的 研究Ｍｏｙａｍｏｙａ病的发生，发展及转归过程，方法 建立Ｍｏｙａｍｏｙａ病的实验动物模型。结果 颈动脉逐渐狭窄或闭塞的过程是内弹力纤维变性，断裂后，中膜平滑肌细胞沿断处向内膜游走，深入增生的过程，早期，因侧支循环血管建立不完善，脑组织缺血，缺氧出现多灶性脑软化坏死，随时间延长，脑内大量小动脉及毛细血管代偿性增生，其血管壁腔大壁薄，形成异网，同时５个栗粒状或囊状动脉瘤，蛛网膜下腔，脑室内及脑实     

MRA与脑梗塞（附28例分析）

对２８例脑梗塞进行ＭＲＡ检查，结果显示：大面积脑梗塞以脑动脉闭塞为主，腔隙性脑梗塞以脑动脉狭窄、变细或粗细不均为主。表明ＭＲＡ可用来了解腔隙性脑梗塞的脑内动脉粥样硬化程度及大面积脑梗塞闭塞的血管。与ＤＳＡ检查对比，ＭＲＡ可部分替代ＤＳＡ检查。     

小儿moyamoya病颞浅动脉组织学和超微结构与免疫组化研究

报告了５例小儿ｍｏｙａｍｏｙａ病（ＭＤ）颞浅动脉（ＳＴＡ）的组织学和超微结构病理，它们的变化是中膜平滑肌细胞变性、破坏，内弹力板变薄、断裂和内膜平滑肌细胞增生。这些变化与成人ＭＤ脑动脉和ＳＴＡ的病变一致。小儿ＭＤ的ＳＴＡ中能看到大量较早期和较轻的病变，如中膜平滑肌细胞空泡变性、质膜破坏、或仅见于内弹力板两侧的肌细胞坏死；内弹力板破坏轻；内膜增生轻，没有管腔狭窄，与成人不同。本文对ＭＤ动脉病变的发展过程和病因问题提出了看法。     

颞浅动脉贴附治疗Moyamoya病的远期疗效观察

颞浅动脉贴附治疗Ｍｏｙａｍｏｙａ病的远期疗效观察郝建中，徐体华，石祥恩，富兴家Ｍｏｙａｍｏｙａ病至今原因不明，以颈内动脉及其分支慢性进展性阻塞，穿动脉代偿扩张形成异常颅底血管网为特点。目前外科治疗的主要方法有颞浅动脉－大脑中动脉吻合，颞肌贴附和颈浅动...     

成人烟雾病的临床、头颅CT和DSA分析研究

目的探讨成人烟雾病的的临床、头颅CT和脑血管造影(DSA)影像学特征.方法对21例病例的临床、头颅CT和DSA表现进行综合分析.结果本组80%的患者首发症状是以出血为主,主要集中在大于40岁年龄组,头颅CT扫描示脑室系统出血、脑实质出血及蛛网膜下腔出血;30岁以下以缺血性表现为主,头部CT扫描多未见异常密度灶,仅少数可见低密度灶或软化灶.21例DSA表现为ICA末端狭窄或闭塞,脑底烟雾状血管网以及侧枝循环血管网形成.结论DSA检查是确诊烟雾病的主要方法,临床上脑室出血、蛛网膜下腔出血以及无明确病因的脑实质出血或脑缺血发作均应常规行DSA检查.     

3.0T磁共振SWI在高血压脑内微出血中的应用及临床意义

目的探讨磁敏感加权成像(SWI)在高血压脑内微出血中的应用及临床意义。方法选53例高血压患者,应用3.0T MR行常规MRI和SWI序列成像检查并结合临床资料进行分析。结果32例高血压患者脑内有微出血灶,在SWI上呈点状、圆形、椭圆形低信号。总数达887个,直径为0.3mm～7.6mm。分布于皮层、皮层下和基底节区。其中17例伴发缺血性脑血管病,5例出血性腔隙性梗塞,7例伴发出血性脑血管病,3例无临床症状。结论SWI序列可敏感的显示高血压脑内微出血灶,对伴发缺血性和出血性脑血管病的诊断治疗有重要的指导价值。     

ESWAN序列和常规FSET2WI序列对脑微出血灶检出效能的比较

目的探讨ESWAN序列对脑多发微出血灶的检出效能。方法 35例脑血管病患者,18例高血压,8例腔隙性梗死,9例患高血压合并腔隙性梗死,排除脑出血、颅内占位、外伤、手术史。采用GE3.0T磁共振FSET2WI及ESWAN序列横断位扫描,分析统计结果。结果 35例患者均存在多发微出血灶,以基底节/丘脑区多发,皮质及皮质下区次之,脑干和小脑区最少,差异有统计学意义(P0.01)。ESWAN序列对脑微出血灶的显示明显优于FSET2WI序列,差异有统计学意义(P0.01)。结论 ESWAN序列对微出血敏感,有助于临床更早期发现病变,为及早治疗提供信息。     

真性红细胞增多症致脑血管病25例临床诊治

目的探讨真性红细胞增多症(polycythemia vera,PV)致脑血管病的临床和影像学特点。方法对25例PV致脑血管病患者的临床资料进行回顾性分析。结果本组PV致脑血管病表现:头晕12例、眩晕5例,头痛8例、偏身肢体瘫痪15例、肢体麻木4例。脑梗死19例(76%),其中多发性脑梗死15例(60%);短暂性脑缺血发作2例(8%);脑出血2例(8%);蛛网膜下腔出血1例(4%);脑静脉窦血栓1例(4%)。均符合PV的临床表现和体征及血象、骨髓象改变。影像学检查显示以多发性小梗死灶多见(60%),常见于脑叶、基底节、内囊。脑出血血肿内密度不均匀,周边水肿明显。结论 PV致脑血管病以脑梗死多见,其中又以多发性脑梗死为主,并发脑出血、脑静脉窦血栓和蛛网膜下腔出血较少见。     

脑干腔隙性梗塞的MRI,TCD与临床

本文对８５例脑干腔隙性梗塞（腔梗）患者的头颅ＭＲＩ、部分ＴＣＤ及临床特征作回顾性分析。结果：本组患者发病年龄较大，７５％有长期高血压史，临床表现为复杂性、多样化特点，且多数缺乏典型的交叉或脑干孤立症征。ＭＲＩ扫描显示腔隙灶以桥脑腹侧居多，病灶＜０．５ｃｍ者可无症状或症状轻微。ＴＣＤ可很好反映椎－基底动脉系统的血管弹性及血流速度，对脑干腔梗的诊断有一定的帮助，但定位不甚准确。ＭＲＩ仍是诊断脑干腔梗的最佳手段。     

脑缺血大鼠脑皮质中前蛋白转化酶枯草溶菌素9 mRNA的经时表达

氧化的低密度脂蛋白是脑血管病的危险因素,而前蛋白转化酶枯草溶菌素9能升高低密度脂蛋白的水平。因此,课题组设想前蛋白转化酶枯草溶菌素9可能在缺血性脑血管病中发挥作用。实验以阻塞大鼠大脑中动脉100 min后进行再灌注建立短暂性局灶性脑缺血模型,原位杂交染色发现脑缺血大鼠脑皮质内前蛋白转化酶枯草溶菌素9 mRNA表达随再灌注时间延长而增高。说明局灶性短暂脑缺血可上调大鼠缺血脑皮质内前蛋白转化酶枯草溶菌素9 mRNA的表达。 关键词：前蛋白转化酶枯草溶菌素9 mRNA;皮质;脑缺血;大鼠;神经再生     

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。     

成年型孪生子Moyamoya病

目的报道一个成年型孪生子Moyamoya病。方法孪生姊妹之一的先证者为31岁,1年前出现全身乏力,检查发现糖尿病。半年前出现性格改变和抑郁,5个月前出现发作性右上肢麻木无力,2个月前出现左上肢活动不灵活和双下肢一过性无力,6d前出现大小便失禁和精神行为异常。查体显示轻度痴呆,左侧鼻唇沟浅,四肢肌力级,肌张力高,四肢锥体束征阳性。头部CT显示大脑半球多发性脑梗死。另一孪生妹妹在2年前亦发现糖尿病,曾经有过头晕发作。对先证者进行TCD和脑血管造影检查,另一孪生妹妹和无症状的2个哥哥及一对孪生女儿进行TCD检查。结果先证者DSA检查示双侧颈内动脉闭塞,双侧大脑前、中动脉主干不显影,颅底可见异常增生的血管网。TCD检查显示先证者和孪生姊妹的双侧颈内动脉末端闭塞,后循环代偿性血流增快,其孪生女儿之一存在双侧大脑中动脉起始段狭窄,无症状的2个哥哥血管无异常。结论孪生子Moyamoya病可以在成年起病,以缺血性损害为主要临床表现,合并糖尿病提示该类型Moyamoya病的发生可能和糖尿病相关基因的异常有关。当双胞胎之一出现Moyamoya病时,对另一个成员应当常规进行TCD筛查以确定其血管是否存在异常。     

Moyamoya病的临床表现与影像学特点

目的 回顾分析12例Moyamoya病的临床和影像学特征，探讨Moyamoya病的影像学诊断价值。方法 分析12例Moyamoya病患的临床资料及数字减影血管造影(DSA)、磁共振血管造影(MRA)、磁共振成像(MRI)和CT结果。结果 所有病例均表现有颈内动脉或其分支不同程度的狭窄或闭塞和颅底异常血管网(MMD血管)；其中病变呈双侧8例．单侧4例。12例中头颅CT表现有梗塞灶4例，脑出血5例，其余3例表现正常。结论 除DsA外．MRI和MRA是两种可以很好评价Moyamoya病的影像学方法。若儿童或青壮年发生脑血管病，反复出现脑梗死或出现脑室出血、脑叶出血或蛛网膜下腔出血(SAH)则要考虑Moyamoya病的可能。     

Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation

An opportunity to study cerebrovascular changes in sickle cell anemia (SCA) presented itself when a black child with this disorder died of bihemispheric strokes. Angiography demonstrated severe occlusive vascular disease involving primarily the circle of Willis and major bifurcations of both internal carotid arteries. Collateral circulation to the distal branches of the internal carotid arteries occurred through transdural anastomoses from the external carotid system and via the leptomeningeal route. Perfusion of the basal ganglia was accomplished by vessels arising from the proximal internal carotid arteries. These changes resembled those of Moyamoya disease. Autopsy showed old and recent cerebral infarcts. Two vascular processes were responsible for the arterial occlusions: (1) exuberant intimal hyperplasia, and (2) old and recent thrombi with partial recanalization. The former has been described only once before in SCA. Small vessels in the basal ganglia were exceptionally numerous and dilated. We conclude that intimal hyperplasia within large cerebral arteries may be responsible for infarction and small vessel prliferation in basal ganglia in patients with SCA.     

连续40例出血型烟雾病的DSA影像学分析

目的探讨烟雾病患者的DSA影像学诊断特征。方法回顾性分析40例烟雾病病例的DSA影像学表现,40例患者均经CT诊断为颅内出血,均经DSA确诊。结果双颈内动脉末段狭窄闭塞22例,一侧颈内动脉末段狭窄闭塞8例,一侧大脑中动脉狭窄闭塞3例,双侧大脑前动脉和一侧大脑中动脉狭窄闭塞1例,双侧大脑前动脉狭窄闭塞3例,双侧大脑中动脉狭窄闭塞1例,一侧大脑中动脉及对侧大脑前动脉狭窄闭塞2例,均伴颅底异常血管网形成。结论DSA为诊断烟雾病的金标准,DSA检查可清楚显示烟雾病血管狭窄闭塞的部位、侧支循环情况及是否合并动脉瘤,据其表现可指导进一步治疗。     

An attempt to treat cerebrovascular 'Moyamoya' disease in children.

Moyamoya formations at the base of the brain are not congenital vascular malformations but represent collateral pathways associated with chronic progressive stenosis of the carotid fork. The authors have studied 44 personal cases, 18 children under 15 years of age, and 26 adults. In children the Moyamoya vessels change through six stages: (1) carotid fork stenosis; (2) progressive carotid stenosis with initial Moyamoya collaterals and dilatations of cerebral arteries; (3) dilatation of Moyamoya collaterals and disappearance of anterior and middle cerebral arteries; (4) thinning of Moyamoya; (5) contraction of Moyamoya and disappearance of posterior cerebral arteries; (6) intracerebral vessels perfused from the external carotid and/or vertebrae. These six stages are not observed in adults. Bilateral cervical perivascular sympathectomy (PVS) was performed in 9 children and superior cervical ganglionectomy (SCG) was added unilaterally in 4 and bilaterally in 3 cases. Angiographic follow-up studies were carried out 1-7 years following surgery. Improvement was observed in most of the cases examined within the first 2 months after surgery. This was not the case in arteriograms performed more than 6 months postoperatively. It would seem that PVS and SCG can improve the progress of Moyamoya vessels but only for a short period of time. Clinical symptoms, however, seem to continue improving over a long period of time. Cerebral blood flow improved 5 weeks following surgery in a 13-year-old boy.     

Moyamoya disease in children

Moyamoya disease is an uncommon cerebrovascular disease characterized by progressive steno-occlusive changes in the terminal internal carotid arteries (ICA) and their main branches, associated with the development of moyamoya vessels. The incidence of the disease is high in east Asia, especially in Japan and Korea. The familial form accounts for 10-15%. Moyamoya disease has two age distribution peaks at around 5 and 40 years. Most pediatric patients exhibit transient ischemic attacks or infarction. Headache and involuntary movements are serious symptoms associated with pediatric moyamoya disease. MRI and MR angiography (MRA) are useful and non-invasive methods for diagnosing or monitoring moyamoya disease. Cerebral angiography is still the gold standard for a diagnosis, however, it is not mandatory when MRI and MRA show typical findings of moyamoya disease; steno-occlusive changes at the ends of ICA and an abnormal vascular network in the basal ganglia. Other MRI findings have been reported, including T2 shortening in the white matter, the ivy sign on fluid-attenuated inversion recovery (FLAIR) images, and medullary streaks on FLAIR or enhanced T1-weighted images.     

变态反应与烟雾病关系的实验研究

目的：探讨免疫复合物与烟雾病之间的关系。方法：用颈局部注射马血清方法建立烟雾病的实验动物模型。结果：颈动脉受免疫复合物损伤后，内弹力层变性、迂曲、断裂、中膜平滑肌间力纤维断裂处向内腔伸入增生，致管腔狭窄或闭塞，脑组织长期缺血，小血管及毛细血管代偿性增生形成异网。结论：变化反应性免疫复合物对颈动脉的损伤可能是引起烟雾病的原因。     

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。     

Cases similar to cerebrovascular moyamoya disease--investigation by angiography and computed tomography

The criteria of the cerebrovascular Moyamoya disease is defined by the characteristic findings of its cerebral angiograms, as follows; 1) The internal carotid siphon is narrowed or obstructed bilaterally. 2) The "Moyamoya vessels" are observed at the base of the brain or the basal ganglionic regions. 3) Main trunks of the cerebral arteries such as the anterior, the middle, and/or the posterior cerebral arteries are often not or poorly visualized. 4) Its etiology is unknown. It has been known that the occlusion of the internal carotid fork with Moyamoya vessels is not infrequently seen in patients with tuberculous meningitis, sickle cell anemia, head trauma, and so on. In the definition of the disease, patients with known etiology and/or unilateral occlusion in the carotid fork must be excluded. However, the cases who cannot fulfil its criteria of the cerebrovascular Moyamoya disease, but have its characteristic Moyamoya vessels and collateral pathways have been reported. We investigated the findings of cerebral computed tomograms in 13 patients who did not fulfil the criteria of the cerebrovascular Moyamoya disease, but revealed the Moyamoya vessels. The subjects are 5 males and 8 females, ranging 15 to 70 years old. The past histories of 9 patients among them revealed hypertension, radiation therapy for pituitary adenoma, head trauma, aplastic anemia, and the Raynaud phenomenon. By angiographic evaluations, occlusions in the unilateral carotid forks were seen in 7 patients, and stenoses in those were in 5 patients. One patient showed only a severe stenosis in the horizontal portion of the middle cerebral artery.(ABSTRACT TRUNCATED AT 250 WORDS)