成人出血型烟雾病的临床特点分析

目的探讨成人出血型烟雾病的临床特点,为提高其诊治水平提供帮助。方法回顾性分析58例成人出血型烟雾病患者的临床资料。结果 58例患者均以中风发病,头颅CT示单纯脑室出血14例、脑室+脑实质出血34例、蛛网膜下腔出血6例、脑实质出血2例、脑实质出血+蛛网膜下腔出血1例。MRI见"常春藤"征者5例、基底节区血管留空影4例。DSA示合并动脉瘤或疑似动脉瘤8例。按GOS评定预后,恢复良好34例,中残15例,重残2例,植物生存1例,死亡6例,31例随访6月至10年,病史大于3年者11例,4例发生再次脑出血;病史小于3年者20例,2例发生再次脑出血。结论脑室出血合并脑实质出血是成年出血型烟雾病的主要出血方式;临床上对于不明原因的无血管病变危险因素的脑出血患者均应常规行脑血管造影检查。     

成人烟雾病17例临床表现和影像学特点分析

目的探讨成人烟雾病的临床和影像学改变特点。方法回顾性分析17例经常规数字减影血管造影(DSA)、磁共振血管造影(MRA)和/或MRI检查确诊的成人烟雾病患者的临床和影像学资料。结果 17例患者中表现为缺血性脑卒中12例(70.6%),主要表现为偏瘫、失语、头痛及智能减退,还可表现为短暂性脑缺血发作;出血性脑卒中5例(29.4%),主要表现为头痛、意识障碍、偏瘫及抽搐。MRI检查发现缺血性脑卒中累及额叶8例,底节区3例,深部脑白质3例,枕叶3例,颞叶1例;累及单侧半球4例,双侧半球8例;脑萎缩6例。环池内烟雾血管影1例,双侧大脑中动脉累及10例。出血性脑卒中患者中单纯脑室出血2例,蛛网膜下腔出血1例,脑室出血并脑干出血1例,脑室出血并丘脑出血1例。MRA均发现双侧颈内动脉或其分支狭窄或闭塞。全脑DSA检查7例,其中双侧颈内动脉狭窄、闭塞5例,双侧大脑前动脉狭窄或闭塞4例,双侧大脑中动脉狭窄或闭塞3例。结论成人烟雾病临床表现多样,临床特点与责任病灶及支配血管相关;MRI和/或MRA有助于烟雾病的早期诊断,确诊需行全脑DSA检查。     

成人烟雾病的临床特点及影像分析

目的　研究成人型烟雾病的临床特点、影像表现及其两者的关系。方法　 6 1例经常规血管造影 (包括 DSA)和 MRA确诊的成人型烟雾病患者 (>16岁 )。分析其临床表现及血管造影结果。结果　主要临床表现为突发剧烈头痛、意识障碍 ,伴呕吐、失语及一侧肢体瘫痪。在 CT和 MRI上 ,脑出血 4 1例 (6 7.2 % ) ,脑梗死 15例(2 4 .6 % ) ,脑萎缩 2例 ,无异常表现 3例。在 DSA和 MRA上 ,双侧病变者 32例 ,单侧病变 2 9例 ,5 1例可见到烟雾状异常血管网 ,尤其在出血型患者中。后交通动脉增粗 38支、眼动脉增粗 31支及软脑膜吻合支增多 4 4侧。 5例大面积脑梗死侧支循环很差或缺乏。结论　成人型烟雾病以脑出血多见 ,在发病率和血管造影上和儿童无明显差异。治疗应首选血管重建手术。 DSA为诊断金标准 ,为术前必要检查 ;MRA无创伤性 ,为本病筛选、诊断、随访的首选方法。     

烟雾病18例临床分析

<正>烟雾病(moyamcya disease,MMD)本文对18例确诊的MMD病人的临床及影像特点分析如下: 临床资料1.一般资料本组18例,男7例,女11例,男:女为1:1. 6。年龄3～75岁,平均28.3岁。缺血性卒中13例,出血性卒中5例。<20岁者,缺血组8例、出血组1例;>20岁者,缺血组5例、出血组4例。既往有肺结核史1例,脑炎史1例,心脏病史1例,其余无特殊病史。     

烟雾病32例临床分析

临床资料一、本组32例中男14例，女18例，4～13岁4例，22～36岁25例，47～54岁3例。二、临床表现：本组12例以TLA起病，其中3例为反复发作的一侧肢体无力，1例为发作性失语，1例为发作性晕厥，3例先为发作性一例肢体麻木后无力，并逐渐进展为持久的偏瘫，伴有运动性失语；3例为急性不完全们知肌力为’～N级，1例并有外展神经麻痹；2例为起病较急的交替性偏瘫、假性球麻痹，其中互例并双眼视力下降；5例为发作性头痛，其中3例1月～30年后出现蛛网膜下腔出血；7例表现为剧烈头痛伴呕吐、不同程度的意识障碍，1例并癫病发作，经脑CT扫描证实…     

烟雾病的临床特点分析(附4例报道)

烟雾病(Moyamoya病)于1955年在日本首次发现.因颈内动脉及其分支的进行性闭塞,深穿支动脉的膨大,在脑底部出现代偿性异常网状血管,在脑血管造影片上形似烟雾,1960年铃木医生用Moyamoya(日语烟雾的意思)命名此病.现将本院自1990年5月以来诊断的4例患者报道如下.     

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。     

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。     

41例成人烟雾病的临床特点及血管影像学分析

目的总结成人烟雾病的临床及血管影像学特点。方法回顾性分析41例成人烟雾病的临床表现及影像学资料,以观察其临床及影像学特点。结果本组患者多为青壮年起病,平均起病年龄40岁左右,无明显性别差异;均以脑卒中形式急性起病,缺血性卒中占19.5%,出血性卒中占80.5%;DSA表现为Willis环附近动脉狭窄或闭塞,脑底烟雾状血管网、侧枝循环及动脉瘤形成。结论青壮年反复发作的脑卒中,尤其是无高血压病及动脉硬化证据时,应警惕烟雾病的可能,需行全脑DSA检查以明确诊断。     

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.     

胼胝体变性的临床特点与影像学特征研究

目的　研究分析了一组胼胝体变性 (MBD)患者的临床表现和影像学特征 ,探讨不同临床分型的特点与诊断方法。方法　 4例长期酗酒男性MBD患者 ,急性型 1例 ,慢性型 3例 ,均经CT检查 ,3例经头MR检查 ,1例行尸检病理检查。结果　急性型主要表现为意识障碍、精神症状与癫 ;慢性型以痴呆、精神症状为主要表现 ;CT与MR可见胼胝体膝部和压部病灶。CT病灶为低密度 ,MR表现为T1WI低信号 ;T2 WI高信号 ;2例伴发双侧大脑半球白质对称性病灶 ,1例出现脑干对称性病灶。尸检病理提示 :胼胝体呈多灶性脱髓鞘与坏死改变。结论　急性型MBD意识障碍突出 ,病程进展迅速 ;慢性型以痴呆、精神症状和锥体束损害为主要表现 ;CT与MR为其生前诊断的主要手段 ,以胼胝体膝部、压部对称性病灶为其主要异常表现。     

68例出血性烟雾病临床分析

目的 研究成年出血性烟雾病患者的临床特点及DSA表现. 方法 对CT诊断为颅内出血,并经DSA确诊的68例烟雾病患者进行临床资料和DSA影像资料分析. 结果 68例患者中男23例,女45例,平均37.8岁.DSA表现:双侧前循环病变57例,合并后循环改变19例;单侧前循环病变9例,合并后循环改变6例;单纯后循环病变2例,单纯前循环病变41例.合并脑动脉瘤患者11例,其中7例进行了栓塞或开颅夹闭术.合并脑梗死患者13例.2次以上反复出血17例.脑室出血59例,脑实质出血7例,蛛网膜下腔出血2例.因脑出血量较大,需行开颅手术者6例. 结论 成人出血性烟雾病主要表现为脑室内出血,尤其中年女性多见.积极针对合并动脉瘤及症状的外科干预多能取得良好治疗效果,但难以防止再次出血.脑DSA是明确分析烟雾病病变特征及选择治疗方法的首要手段.     

Renal artery lesions in patients with moyamoya disease: angiographic findings

BACKGROUND AND PURPOSE: Renal artery lesions in moyamoya disease have been described sporadically in several case reports. The purpose of this study is to evaluate the angiographic findings of renal artery lesions in moyamoya disease and to determine the prevalence of renal artery lesions in patients with moyamoya disease. METHODS: Eighty-six consecutive patients with idiopathic moyamoya disease were prospectively examined with both cerebral angiography and abdominal aortography. The findings of abdominal aortography were reviewed for the presence and appearance of renal artery lesions and compared with the clinical data and cerebral angiographic findings. RESULTS: Of 86 patients with idiopathic moyamoya disease, 7 patients (8%) were found to have renal artery lesions. Six patients (7%) had stenosis in the renal artery, and 1 patient (1%) had a small saccular aneurysm in the renal artery. Two patients (2%) with a marked renal artery stenosis presented with renovascular hypertension, which resulted in an intraventricular hemorrhage in 1 patient. Furthermore, the renal artery stenosis in the 2 patients with renovascular hypertension was successfully treated with percutaneous transluminal angioplasty. There was no significant correlation between the presence of renal artery lesions and cerebral angiographic findings. CONCLUSIONS: Seven (8%) of 86 patients with moyamoya disease showed renal artery lesions, including 6 stenoses (7%) and 1 aneurysm (1%). Renal artery lesions are a clinically relevant systemic manifestation in patients with moyamoya disease.     

脑动静脉畸形的DSA、CT和MR诊断对比研究

回顾性研究1985年2月～1996年8月脑动脉畸形96例，对照分析本病的DSA、CT和MR诊断征象。结果96例DSA表现为供血动脉增粗迂曲，畸形血管团，引流静脉提早显影．58例CT平扫25例显示局部有条索状、团状高密度灶，14例显示高低混杂密度灶。显示低密度灶7例。12例显示正常．增强扫描可见弯曲，粗大明显增强高密度血管影，无水肿及占位效应．14例MR扫描均见流空信号，多为蜂窝状或蚯蚓状，其周围有粗大供血动脉和引流静脉。作者认为DSA是诊断本病的首选检查方法．MR对畸形血管的显示优于CT，但价格昂贵．     

Clinical and angiographic follow-up of childhood-onset moyamoya disease

To clarify the differences between childhood-onset moyamoya disease and that with onset in adulthood, we studied the clinical course and angiographic findings of adult patients (over 20 years of age) with moyamoya disease of childhood onset (up to 15 years of age). The clinical course in 25 patients could be assessed. The follow-up period was 5–27 years. Neurological deficits were noted in 11/23 and mental disorders in 9/21. In all except one, the illness had started before the age of 7 years. Neither neurological nor mental condition changed during or after adolescence (15–20 years of age). Two patients died of intracranial hemorrhage. The disease progressed in angiographic stage until adolescence, but had stabilized or almost stabilized by the age of 20 years. This study indicates that moyamoya disease with onset in childhood carries high morbidity and mortality. The disease advances in angiographic stage between childhood and adolescence, but stabilizes or almost stabilizes between adolescence and adulthood.     

烟雾病的临床特点及影像学分析(附28例报告)

目的探讨烟雾病的临床特点及影像学特征。方法对28例烟雾病患者的临床资料和影像学表现进行分析。结果本组病例中脑出血18例(64.3%),脑缺血10例(35.7%)。20例行磁共振血管成像(MRA)检查。28例均行数字减影脑血管造影(DSA)检查。结论烟雾病的临床表现多样,以脑出血多见。DSA是诊断该病的金标准,对临床疑似病例应尽早行DSA检查。     

Surgical results in pediatric moyamoya disease: angiographic revascularization and the clinical results

OBJECTIVE: We retrospectively reviewed the pediatric patients with moyamoya disease (MMD) who underwent bypass surgery at our institution to compare the surgical results according to the surgical procedures. PATIENTS AND METHODS: There were 24 total patients (age range: 2-15 years; mean age: 8.2 years). Twelve patients underwent encephalo-duro-arterio-synangiosis (EDAS) on 16 sides, 5 patients underwent encephalo-duro-arterio-myo-synangiosis (EDAMS) on 8 sides and 7 patients underwent combined superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis with EDAMS (STA-MCA-EDAMS) on 12 sides. The postoperative results were evaluated between 4 months and 5 years following surgery in terms of the angiographic revascularization and the clinical outcome. RESULTS: EDAMS, regardless of the combined STA-MCA anastomosis, was significantly effective for achieving a good extent of the postoperative angiographic revascularization as compared with simple EDAS (P<0.05). STA-MCA-EDAMS tended to be better with respective to the relief of preoperative ischemic symptoms as compared with simple EDAS, although there was no significant statistical difference. CONCLUSION: These results suggest that EDAMS with or without the combination of STA-MCA anastomosis was very useful for the formation of collateral circulation in comparison with simple EDAS for treating the pediatric patients with MMD, although these findings were not well correlated with the clinical outcomes.     

硬脑膜动静脉瘘CT、MRI及DSA对比研究

目的对比研究硬脑膜动静脉瘘(DAVF)的CT、MRI及DSA影像学检查结果的差异,探索DAVF在不同影像学检查中的特征性表现。方法对98例不同类型DAVF的CT、MRI及DSA检查结果进行对比研究。结果CT及MRA检查能较好地显示因DAVF所致的颅内继发性病变,扩张的引流静脉,继发性出血及静脉窦狭窄、闭塞等改变;DSA检查则能准确地显示瘘口部位、供血动脉、静脉引流情况及颅内血流分布情况,但无法显示继发的脑实质病变。结论DSA检查是诊断DAVF的金标准。CT、MRA检查亦能为诊断DAFV提供可靠依据。     

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.