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。

Clinical manifestations and imaging characteristics of Moyamoya disease in adult patients: Report of 17 Cases

Objective To study the clinical manifestations and imaging features of adult patients with moyamoya.Methods The clinical and imaging data of 17 adult patients with moyamoya confirmed by digital signature algorithm(DSA),magnetic resonance angiography(MRA) and/or magnetic resonance imaging(MRI) were analyzed retrospectively.Results Of all the 17 patients,ischemic stroke accounted for 70.6% with the main clinical manifestations of hemiplegia,aphasia,headache,hypophrenia and transient ischemic attack(TIA);hemorrhagic stroke accounted for 29.4% with common manifestations including headache,disturbance of consciousness,hemiplegia and convulsion.Brain MRI showed ischemic stroke involving lobus frontalis(8 patients),coxopodite(3 patients),deep part of alba(3 patients),occipital lobe(3 patients) and temporal lobe(1 patient),among which,involving unilateral hemisphere in 4 patients,bilateral hemispheres in 8 patients,and encephalatrophy in 6 patients.Collaterals in cisterna ambiens,resembling a puff of smoke,were detected in 1 patient,while middle cerebral arteries(MCA) were involved in 10 patients.Among the hemorrhagic stroke patients,there were cerebroventricular hemorrhage in 2 patients,subarachnoid hemorrhage in 1 patient,cerebroventricular hemorrhage together with brainstem hemorrhage or thalamic hemorrhage in 2 patients,respectively.Stenosis or occlusion of the bilateral internal carotid artery or its branches and moyamoya vessels were found in all patients by MRA.Of the 7 patients subjected to brain DSA,there was stenosis or occlusion of the bilateral internal carotid artery in 5 patients,bilateral Anterior cerebral artery(ACA) in 4 patients and bilateral MCA in 3 patients.Conclusions The manifestations of adult moyamoya patients were diverse,which was related with location and blood vessel involved.For the diagnosis of moyamoya disease,it is convenience to take MRI and /or MRA for the early recognition,and then DSA for confirmation.