烟雾病的CT,MRI,MRA,脑血管造影和临床对比分析

目的探讨烟雾病的临床表现与病因、病理、影像学特点的相关性。方法对２０例经磁共振血管造影（ＭＲＡ）或脑血管造影证实的烟雾病，从其临床表现、病理改变、影像学特点及诊断价值进行对比分析。结果２０例中有１５例（＜１５岁）表现为缺血性脑血管病变，其中５例考虑为钩端螺旋体性脑动脉炎所致。５例（＞１６岁）表现为脑出血。结论烟雾病在儿童、青少年以缺血性脑血管病变为主，成人以脑出血为主要表现。绝大多数烟雾病能通过ＭＲＡ诊断     

儿童和成人烟雾病临床及磁共振表现对照分析

目的 探讨儿童和成人烟雾病的临床和磁共振成像表现的异同.方法 回顾分析18例儿童烟雾病和12例成人烟雾病患者的临床、MRI和MRA资料,分析对比其发病诱因、临床症状、MRI和MRA表现.结果 儿童烟雾病以上呼吸道感染为主要诱发因素,成人则以情绪激动和过度劳累为主要诱发因素;临床上儿童以惊厥型和TIA发作型表现为主,成人则以出血型和缺血型表现为主;MRI显示儿童患者脑梗死部位以大脑皮质和皮质下为主,成人患者则以基底节区和放射冠为主;成人患者脑出血发生率明显高于儿童;MRA显示儿童和成人患者颈内动脉、大脑中动脉和大脑前动脉受累几率相似,但成人颈内动脉和大脑中动脉狭窄程度稍重于儿童;成人患者大脑后动脉受累多于儿童;儿童患者颅底侧支血管形成情况优于成人.结论 儿童和成人烟雾病在发病原因、磁共振成像及临床表现上均有一定差别,磁共振成像表现与临床表现具有良好的相关性和一致性.     

37例烟雾病的临床特点及影像学分析

该文回顾性分析了15例儿童烟雾病和22例成人烟雾病患者首发症状、临床表现及影像学,其中儿童患者以缺血型为主(80%),成年患者以出血型(81.8%)为主。37例均行数字减影脑血管造影(DSA),其中14例同时行头颅磁共振血管成像(MRA)检查,结果显示,成人患者的血管狭窄程度重于儿童。烟雾病临床表现多样,儿童和成人烟雾病在临床表现、发病类型、影像学表现上均有一定差别。DSA和磁共振血管成像(MRA)是诊断烟雾病有效的检查方法。     

烟雾病临床特点分析

目的分析经颅多普勒超声（TCD）问世后，烟雾病的临床特点。方法总结了北京协和医院自TCD应用于临床后（1992-2004年）的54例烟雾病的特点。35例（65％）通过数字减影血管造影（DSA）确诊，19例（35％）通过磁共振血管成像（MRA）确诊。结果（1）起病年龄有两个高峰，10岁左右和40岁左右。≤25岁年龄组19例（35％，中位数9岁），〉25岁年龄组35例（65％，中位数39岁）。（2）最常见的首发症状包括脑梗死和短暂性脑缺血发作（TIA）在内的缺血性血管病，共39例（72％）。以缺血为首发症状的发生频率在不同年龄组分别为：〈16岁年龄组87％；16—30岁年龄组55％和〉30岁年龄组71％。脑出血8例（15％），发病年龄17—49岁。其余7例首发症状表现为非典型血管病临床表现。确诊时为脑出血的10例患者均直接经DSA检查诊断，而非出血的44例患者中的37例（84％）先经TCD筛查而后行DSA或MRA确诊为烟雾病。结论TCD能够发现更多缺血性和表现为非典型血管病临床症状的烟雾病患者，我们的资料显示，成年发病的烟雾病患者较儿童更多。此外，虽然脑出血好发于成年人，但无论儿童期还是成年期，烟雾病的临床表现都以缺血症状为主，尤其是TIA。     

儿童钩端螺旋体脑动脉炎29例的脑电图观察

1978年～1981年6月我科收治儿童钩端螺旋体脑动脉炎29例,就其脑电图的检查结果报告如下。 临床诊断依据 1.病人均来自钩端螺旋体病流行区,有与病猪、羊或疫水接触史;2.发病与当地钩端螺旋体病流行季节相一致,半数病人有“上感”史,3.突然发生偏瘫、失语,部分病人瘫痪前有多次短暂脑缺血发作(TIA),其症状、体征,临床经过符合脑血管阻塞性病变;4.血清凝溶试验效价在1:400以上;5.脑血管造影显示脑动脉炎改变。     

烟雾病的临床特点及DSA分析

目的探讨烟雾病的临床特点和DSA表现。方法回顾分析我院2006年以来收治的34例烟雾病患者的临床特点和DSA表现。结果 (1)34例患者均表现为急性脑卒中,其中脑出血21例(61.8%),蛛网膜下腔出血3例(8.8%),脑梗死10例(29.4%)。(2)所有患者均经DSA确诊,DSA主要表现为单侧或双侧颈内动脉及大脑前、中动脉狭窄或闭塞,均伴有颅底不同程度烟雾状异常增生的血管网;双侧病变25例,单侧病变9例;部分病例有侧支代偿;另有4例患者检出4枚动脉瘤。结论烟雾病的临床表现以急性脑卒中为主,成人患者主要表现为脑出血,尤其是脑室出血,其次是脑梗死。DSA是诊断烟雾病的金标准,对发病年龄较轻的卒中患者应行DSA检查明确诊断。     

25例烟雾病临床影像特点观察

目的探讨烟雾病的临床特征、影像学特点。方法对25例烟雾病患者首发症状、临床表现及影像学结果进行分析。结果烟雾病发病平均年龄较轻,女性多于男性;少儿烟雾病患者以缺血改变为主(87.5%),成年烟雾病患者缺血改变(58.8%)与出血改变(41.2%)相当;临床以头痛、头晕、肢体无力或偏瘫或交替性瘫痪为主要表现,同时可伴失语、智能下降、视力下降、癫痫发作、小舞蹈发作等表现。14例行头CT检查仅示颅内病灶,均未提示烟雾病;25例均行MRI和MRA检查发现颅内病灶,显示颈内动脉虹吸末段和大脑前或中动脉近段狭窄或闭塞,并有脑基底部异常血管网及侧支循环形成,均被诊断为烟雾病。其中9例又行数字减影血管造影术(DSA),结果与其MRA相符。结论头颅MRI和MRA是诊断烟雾病理想的非介入性检查方法。     

钩端螺旋体病的神经系统并发症(文献综述)

钩端螺旋体病(以下简称钩体病)是一种常见的流行病,由它引起的神经系统并发症特别在儿童,虽早有报导,但仍未被人们重视。常见的致病性钩体有波蒙那型、犬型、黄疸出血型、秋季热型、和七日热A、B型等。在Heath报导的345例中,235例(68%)有神经系统症状和并发症。临床表现一、急性期的神经精神症状1.一般感染中毒症状:头痛,常剧烈,     

烟雾病与钩端螺旋体脑动脉炎

烟雾病又称Moyamoya病或自发性基底动脉环闭塞症，是一种以双侧颈内动脉末端及大脑前、大脑中动脉起始部动脉内膜缓慢增厚，动脉管腔逐渐狭窄以致闭塞，脑底穿通动脉代偿性扩张为特征的疾病^[1]。钩端螺旋体脑动脉炎是神经系统感染钩端螺旋体后常见的严重并发症^[2]，约占钩端螺旋体病并发症的87.16％。     

儿童烟雾病与成人烟雾病79例临床及影像学分析

目的 分析并总结儿童烟雾病与成人烟雾病的临床及影像表现的差异.方法 对回顾性分析79例烟雾病患者的临床资料,儿童30例,成人49例,对其临床症状和影像特征进行对照研究.结果 儿童组首次发病以缺血性脑血管病表现为主,显著高于成人组（P＜0.05）,成人组首次发病以出血性脑血管病表现为主,显著高于儿童组（P＜0.05）.结论 儿童烟雾病与成人烟雾病的临床症状和影像表现具有差异,临床接诊应区别对待.     

烟雾病颅内血流相关性动脉瘤的血管内治疗

目的探讨烟雾病患者颅内血流相关性动脉瘤的临床特点及血管内治疗方法。方法回顾性分析11例经全脑血管造影证实的烟雾病合并颅内血流相关性动脉瘤患者的临床表现和血管内栓塞治疗方法。结果 11例中7例表现为颅内出血,4例表现为缺血症状;9个动脉瘤位于Willis环周围,1个位于大脑后动脉顶枕支,1个位于脉络膜后内侧动脉;11个动脉瘤除1例外周型动脉瘤因超选困难外其余10例均成功行血管内栓塞治疗,随访均获良好疗效。结论烟雾病患者颅内血流相关性动脉瘤可有出血或非动脉瘤性临床表现,此类动脉瘤可分为累及Willis环的主要动脉型动脉瘤和累及侧支为主的外周动脉型动脉瘤;血管内栓塞治疗烟雾病患者的颅内动脉瘤是安全有效的,对外周动脉型动脉瘤可予对症保守治疗。     

烟雾病合并动脉瘤的临床诊治探讨

目的探讨烟雾病合并动脉瘤的临床特点及治疗方法。方法回顾性分析10例经全脑血管造影证实的烟雾病合并动脉瘤患者的临床资料。结果10例患者巾9例表现为颅内出血，1例表现为脑缺血。烟雾病合并的动脉瘤9例位于Willis动脉环附近，为主要动脉型动脉瘤。其中5例行动脉瘤夹闭术，3例行血管内栓塞治疗，均恢复良好；1例基底动脉梭形动脉瘤和1例脉络膜前动脉动脉瘤（外周动脉型动脉瘤）无法手术。结论烟雾病合并动脉瘤可导致不同类型的颅内出血，对主要动脉型动脉瘤可进行外科手术治疗，对外周动脉型动脉瘤目前尚无理想治疗方法，可予以对症保守治疗。     

Epidemiological and clinical features of Moyamoya disease in Nanjing,China

Objective

The epidemiology of Moyamoya disease in mainland China has not been documented. Therefore, the present study was designed to examine the epidemiological and clinical features of Moyamoya disease in Nanjing, a provincial capital in China.

Methods

Patient records from multiple hospitals in Nanjing from January 2000 to December 2007 were collected. The clinical features of Moyamoya disease were retrospectively analyzed.

Results

A total of 202 patients were identified. There were 94 males and 108 females, with ages ranging from 2 to 78 years. There was a dual age peak, one in the group of patients 5–9 years of age and another in the group of patients 35–39 years of age. The initial symptoms included cerebral ischemia (81 patients, 40%), cerebral hemorrhage (113 patients, 55.9%) and asymptomatic disease (8 patients, 3.9%). An increasing incidence rate of Moyamoya disease was observed during the period of 2000–2007, with an average detection rate of 0.43 cases/100,000 persons/year (prevalence 3.92/100,000 persons). The incidence of ischemia associated with the disease was 0.16 cases/100,000 people-years and the incidence of hemorrhage was 0.22 cases/100,000 people-years.

Conclusion

This first study on the epidemiological and clinical features of Moyamoya disease in mainland China indicated an increasing incidence of Moyamoya disease with bimodal incidence distribution appearing more frequently in adults.     

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。     

病理证实的淀粉样脑血管病26例临床分析

目的 总结26例尸检证实的淀粉样脑血管病的临床资料特点,以提高对本病的认识和诊断水平.方法 回顾性分析我院1983--1999年收治的经尸检证实的26例淀粉样脑血管病患者,总结其既往史、临床表现、实验室检查等临床资料.结果 26例淀粉样脑血管病中男17例,女9例,年龄45 ～78岁,有高血压病史者8例(30.7％),糖尿病病史者6例(23.1％).2例患者(7.6％)正服用抗凝或扰血小板剂,均为多发脑叶出血.26例中脑出血20例,其中单发脑叶出血2例,多灶性脑叶出血8例,壳核出血5例,丘脑及小脑出血各2例,脑干出血1例.蛛网膜下腔出血2例,出血性脑梗死、基底节区梗死、椎基底动脉闭塞及硬膜下血肿各1例.20例淀粉样脑血管病脑出血患者临床表现为头痛、肢体瘫痪、昏迷、抽搐等.结论 淀粉样脑血管病临床上常以脑血管病表现形式起病,伴有或不伴有高血压.脑叶出血是淀粉样脑血管病最常见临床表现,但少数也可见基底节、小脑和脑干出血.淀粉样脑血管病也可表现为脑梗死、蛛网膜下腔出血.抗凝剂(如华法林)及抗血小板药物(如阿司匹林)的应用对淀粉样脑血管病脑出血可能具有一定的促发作用.     

颞浅动脉-大脑中动脉搭桥术治疗成人烟雾病

目的 探讨颞浅动脉-大脑中动脉（STA-MCA）搭桥术治疗成人烟雾病的效果。方法 回顾性分析17例成人烟雾病患者（出血型13例,缺血型4例）的临床资料,均行STA-MCA搭桥术治疗,其中双侧STA-MCA搭桥术15例,单侧STA-MCA搭桥术2例。结果 术后13例出血型患者临床症状改善,2例出现过度灌注综合征。17例随访3~36个月,13例出血型患者中,12例恢复正常或临床症状改善,1例术后突发颅内出血,预后不良;4例缺血型患者术后3例未再出现短暂性脑缺血发作,1例短暂性脑缺血发作的频率和持续时间减少。结论 STA-MCA搭桥术治疗烟雾病,可以降低出血型患者的再出血率和致残率,以及缺血型患者的短暂性脑缺血发作的发生率。     

Moyamoya: Indiana University Medical Center experience

BACKGROUND: Moyamoya usually presents with cerebral ischemia in children and intracranial hemorrhage in adults. Treatment remains controversial. DESIGN AND OBJECTIVE: We reviewed our experience from June 1995 to August 1999 of 20 adult and pediatric angiographically diagnosed patients with moyamoya to report their clinical presentation, radiological findings, management, and clinical outcomes. RESULTS: The mean age of patients at symptom onset was 17 years (range, 2-54 years). Patients were divided into 2 age groups (group 1, <18 years; group 2, > or =18 years). There were 13 patients in group 1 and 7 patients in group 2. Ischemic strokes or transient ischemic attacks were the predominant initial presentations in both groups. One patient in group 2 had an intraparenchymal brain hemorrhage. Five patients received medical treatment, and 15 had surgical revascularization. The mean time from symptom onset to surgical procedure was significantly longer for patients in group 1 than for those in group 2 (P =.03). The mean follow-up time was 36 months. One patient in group 1 had an ischemic stroke. There was no difference in stroke recurrence, mortality, or modified Rankin scale score among medically or surgically treated patients. CONCLUSIONS: Moyamoya disease may have a different presentation and more benign natural history in our population than in Asian populations. Our findings emphasize the need to better understand the natural history of patients with moyamoya as well as the clinical benefit of different treatment modalities. Structured multicenter randomized clinical trials are needed to further assess the best treatment modalities for patients with moyamoya in the United States.     

硬脑膜动脉血管融通术治疗成人烟雾病

目的 探讨成人烟雾病的临床特征,观察脑硬膜动脉血管融通术(EDAS)对成人烟雾病的治疗作用.方法 回顾分析在我院接受EDAS治疗的312例成人烟雾病患者的临床资料.结果 缺血型和出血型患者分别占79.8%(249例)、20.2%(63例).2例出血型患者术后发生再出血;219例缺血型患者术后临床症状明显缓解或完全消失.术后76.2%的患者糖代谢明显改善;56.6%的患者显示良好的颅内外血管重建,糖代谢减低、低龄、缺血型患者预示更好的血管重建.结论 中国成人烟雾病患者有自身的发病特征;应用EDAS术能明显改善和预防成人烟雾病患者脑缺血发作,对出血型患者再发脑出血也可能有一定的预防作用.     

Moyamoya Disease Associated with Graves’ Disease and Down Syndrome: A Case Report and Literature Review

BackgroundMoyamoya vessels are cerebral vasculopathies characterized by net-like collateral vessel formation at the cerebral basal area and stenosis of the terminal internal carotid artery, proximal middle cerebral artery, and anterior cerebral artery. A diagnosis of Moyamoya disease depends on the bilateral presence of Moyamoya vessels. Moyamoya disease associated with Graves' disease has rarely been reported to be a cause of ischemic events due to hyperthyroidism. However, there are extremely rare cases of Moyamoya disease with concurrent Graves' disease and Down syndrome. We aimed to report such a case, and to compare these cases’ clinical features, pathogenesis, and treatment effects to those of the cases of concurrent Moyamoya disease and Graves' disease alone.MethodsWe performed an English literature search using the PubMed database and the keywords Moyamoya, quasi-Moyamoya, Graves’ disease, thyrotoxicosis, Down syndrome, and trisomy 21.ResultsOnly five cases of Moyamoya disease with Graves' disease and Down syndrome have been reported, including our own. Four patients were female (80%), and all underwent antithyroid therapy and experienced ischemic episodes, including transient ischemic attacks. At the time of their vascular accident, two patients were in a thyrotoxic state; only our patient was in a euthyroid state. The mean age was 15.6 years (standard deviation: 6.1), which was younger than the mean age of 31.4 years (standard deviation: 13) in patients with Moyamoya disease and Graves' disease alone. Down syndrome is commonly associated with abnormal vascular networks due to increased endostatin concentrations or immunological abnormalities such as those that occur in Graves' disease. Graves' disease accelerates the progression of Moyamoya disease and ischemic attacks due to atherosclerosis, enhances sympathetic nervous system activity and immunological changes. As compared to Moyamoya disease patients, patients with concurrent Graves' disease only and Moyamoya disease patients with concurrent Graves' disease and Down syndrome may experience accelerated disease progression or more frequent ischemic attacks.ConclusionEarly imaging follow-ups and strict control of thyroid function are necessary in such cases; if ischemic attacks have already occurred, revascularization surgery may be effective.     

Cerebral infarction following intracranial hemorrhage in pediatric Moyamoya disease �C A case report and brief review of literature

Moyamoya disease is a clinical entity characterized by progressive cerebrovascular occlusion with spontaneous development of a collateral vascular network called Moyamoya vessels. This disease mainly manifests as cerebral ischemia. Intracranial bleeding is another major presentation of patients with Moyamoya disease. We report here a 12-year-old male child who presented with severe headache, vomiting and meningismus. Initial neuroimaging study with noncontrast computed tomography scan revealed fresh intraventricular hemorrhage in right-sided lateral ventricle. Magnetic resonance imaging with angiography of brain was done 5 days later when the child developed right-sided hemiparesis, and the diagnosis of Moyamoya disease was confirmed along with lacunar infarction of right posterior peri and paraventricular area and in the left paraventricular area and centrum semiovale. Simultaneous presence of cerebral infarction along with intraventricular hemorrhage in adult with bleeding-type Moyamoya disease is reported in literature, but it is a rare entity in a child.