【作者】 宋阳；

【导师】 张仲慧；

【作者基本信息】 大连医科大学 ， 神经病学， 2016， 硕士

【摘要】 目的:烟雾病的发病率较低,临床表现多种多样且缺乏特异性,如不提高对本病的认识及进行脑血管方面的相关检查容易出现漏诊及误诊。对本病的早发现、早治疗可以有效延缓其临床症状的进展,对患者本人、家庭和社会有巨大获益。本研究通过收集烟雾病患者的一般情况,临床表现及影像学资料来提高对本病的认知度,为日后的临床工作打下基础。方法:本文通过收集整理2000年1月至2015年12月期间就诊于大连医科大学附属第二医院神经内科及神经外科明确诊断为烟雾病的33例患者的病历资料,详细记录每一位患者的年龄,性别,临床症状、体征及影像学结果,回顾性分析其一般情况,临床表现及影像学结果。收集的数据通过SPSS 19.0软件进行统计分析,计量资料以均数±标准差(x±s)表示;率的比较采用卡方检验,P<O.05为差异有统计学意义。结果:一般情况:33名患者中年龄最小者14岁,最大者72岁,平均年龄(41.85±14.25)岁,收集到的数据表明本病于各个年龄段均可发病,但中青年患者所占的比例较大,发病率较高的年龄段为36岁至50岁之间。缺血性脑血管病及出血性脑血管病亦在各个年龄段均可出现,但是缺血性脑血管病的发病年龄与出血性脑血管病相比普遍较轻。所有患者中男性12名,女性21名,男女比例为1:1.75。1名患者存在可疑的家族遗传倾向,其余患者均未发现有本病的家族史。临床表现:33名患者中缺血性脑血管病患者10名,出血性脑血管病患者23名,两者比例为1:2.3,出血性脑血管病患者的比例明显高于缺血性脑血管病,两者的差异有统计学意义(χ2=5.121,P<0.05)。其临床表现为痛觉减退者2例,偏瘫者13例,失语者8例,认知功能障碍(具体表现为记忆力减退及精神症状)者5例,非特异性症状(头痛、眩晕、恶心及呕吐)者21例,意识障碍者5例(1)。影像学结果:本组33名患者全部应用颅脑MRI或CT进行脑组织平扫。32名患者通过脑血管造影(Digital Subtraction Angiography DSA)确诊,1名患者通过颅脑MRA确诊。10名缺血性脑血管病患者均诊断为脑梗死,梗死部位分别位于脑叶(额叶及顶叶),基底节区,脑干及小脑,由此可以看出因烟雾病继发的缺血性脑血管病患者,其梗死灶可位于颅脑的任何部位。23名出血性脑血管病患者中存在脑室出血者13名,其中5名患者合并脑实质出血,2名患者合并蛛网膜下腔出血;存在蛛网膜下腔出血者8名,除两名患者为合并脑室出血外(2),1名为合并脑实质出血,5名单独表现为蛛网膜下腔出血;另有4名患者出血部位单独位于脑实质。脑实质出血部位中位于基底节区者6人,位于脑叶者5人(3),通过以上数据可以看由烟雾病继发的出血性脑血管病患者中脑室出血及蛛网膜下腔出血所占的比例明显高于单纯脑实质出血,而脑实质出血的患者中出血部位既可以位于脑叶也可以位于基底节区。脑血管影像学检查结果显示:共有20条颈内动脉受累,其中10条为狭窄,10条为闭塞;35条大脑中动脉受累,其中狭窄为12条,闭塞为23条;26条大脑前动脉受累,其中狭窄3条,闭塞为23条;同时发现2名患者合并后循环狭窄,7名患者合并颅内动脉瘤。32名患者存在烟雾样血管,通过前交通动脉代偿供血者6例,通过后交通动脉代偿供血者15例,通过前循环内及后循环向前循环的穿支动脉的深部吻合代偿供血者10例,通过眼动脉代偿供血者3例(4)。结论:烟雾病的临床表现多种多样,缺乏特异性,既可以表现为偏瘫、失语等神经功能缺损症状,也可以表现为头痛等非特异性症状,甚至可以以认知功能障碍为主要表现。脑血管造影为本病诊断的“金标准”,现随着对本病认识的不断加深及MRA等检查技术的广泛应用,本病的诊断率也有着明显的提高。更多还原

【Abstract】 Objective:Moyamoya disease with low incidence,clinical manifestations is varied and lack of specificity,If not to raise awareness of the disease and do cerebrovascular check early will easily missed diagnosis and misdiagnosis,Do early detection,early treatment of the disease can effectively slow down the progress of the clinical symptoms of the disease,which can bring great benefit to the patients themselves,family and society.This resesrch through collecting the general situation,clinical and radiographic features to deepen our understanding of the disease.Methods:This paper collected 33 patients medical records,who were clear diagnosis of moyamoya disease during January 2000 to December 2015 in 2ad Affiliated Hospital of Dalian Medical University neurology and neurosurgery..Detail recording every patient,age,gender,clinical symptoms,signs and imaging results.Retrospectively analysis the general situation,clinical and radiographic feature.Using SPSS 19.0 software to statistical analysis,measurement data to mean + /-standard deviation(+ s);Rate compared by chi-square test,if P < 0.05,the difference has statistically significant.Results:General situation:33 patients with the youngest is 14 years old and the oldest is 72 years old,average age(41.85 + 14.25),the disease can happen all ages,but the middle-aged and young with the proportion of larger,higher onset age stage is 36 years old to 50 years old.Ischemic cerebrovascular disease and hemorrhagic cerebrovascular disease can happen in each age stage,but the onset of ischemic cerebrovascular disease age generally younger than cerebral hemorrhage.Women in patients is 21 cases,with male12 cases,the male to female ratio of 1:1.75.,1 patient is suspected of family genetic predisposition,the rest of the patients were not found to have family history of the disease.Clinical manifestations:There were 10 cases of ischemic cerebrovascular disease and 23 cases of hemorrhagic cerebrovascular disease of 33 cases,the ratio is 1:2.3,hemorrhagic cerebrovascular disease is significantly higher than the proportion of ischemic cerebrovascular disease,the difference was statistically significant(chi-square= 5.121,P < 0.05).Its clinical manifestation as pain decreased is 2 people,13 people hemiplegia,8 people aphasia,cognitive dysfunction(embodied in memory and mental symptoms)5 people,nonspecific symptoms(headache,dizziness,nausea and vomiting)21 people,consciousness disorder 5 people(1).Imaging results: this group of 33 patients all use MRI or CT brain scan.32 patients using DSA(Digital Subtraction Angiography)diagnosed,1 patient with crani ocerebral MRA diagnosis.10 is chemic cerebrovascular patients were diagnosed as cerebral infarction,infarct area located in lobes(frontal lobe and parietal lobe),basal ganglia,brainstem and cerebellum,thus it can be seen the infarct area can located in any part of the brain infarcts.In 23 hemorrhagic cerebrovascular disease patients there are 13 ventricular hemorrhage,in which 5 patients with parenchymal hemorrhage,2 patients with subarachnoid hemorrhage;There are 8 subarachn oid hemorrhage,in addition to the two patients to merge ventricular hemorrhage(2),1 with parenchymal hemorrhage,5 only performance for subarachnoid hemorrh age;Another four patients bleeding alone located in the brain parenchyma.6 pati ents,blooding areas located in the basal ganglia region,5 persons located in the cerebral lobe(3),through the above data we can see that the proportion of ventr icular hemorrhage and subarachnoid hemorrhages is higher than pure parenchymal hemorrhage,and the parenchymal hemorrhage can also be located in the basal ganglia region and the lobes.Cerebral imaging examination results: a total of 20 internal carotid artery involve ment,10 narrow,10 block;35 middle cerebral artery involvement,12 narrow,23 block;26 anterior cerebral artery involvement,3 narrow,23 block;Also fou nd two patients with posterior circulation stenosis,7 patients with intracranial an eurysms.32 patients with smoke vascular,6 cases use anterior communicating arte ry,15 cases use posterior communicating artery,10 cases through the deep match of perforating artery with anterior circulation and the posterior circulation loop;3 cases use ophthalmic artery compensatory pump blood(1).Conclusion: Moyamoya disease has various clinical manifestations and lack of specificity,and can lead to the neurologic impairment symptoms such as hemiplegi a,aphasia,nonspecific symptoms such as headaches and the cognitive dysfunctio n DSA is the "gold standard" to diagnosis the disease,and now with the deepen ing understanding of the disease and MRA examination technology widespread a pplication and the improvement of accuracy,the diagnosis of this disease also have obvious improvement.更多还原