关键词: 烟雾病, 数字减影血管造影, 磁共振血管造影

Abstract: Objective To investigate the clinical characteristics,imagery display and their correlation with moyamoya disease(MMD).Methods We summarized retrospectively a total of 113 cases with moyamoya disease,who hospitalized,and diagnosed by digital subtraction angiography(DSA) and magnetic resonance angiography(MRA) in Beijing Tiantan Hospital.All subjects were divided into teenager group(<20 years old,total 31 cases) and adult group(≥20 years old,total 82 cases).Results Onset age of patients with moyamoya disease present two peaks in present study,10～14 and 35～39 years old respectively.Among 31 subjects in teenager group,there were 15 subjects with ischemic stroke(48.4%),which was dominant,8 subjects with hemorrhagic stroke(25.8%),5 subjects with epileptic seizure repeatedly(16.1%).Among 82 subjects in adult group,there were 41 subjects with hemorrhagic stroke(50%),36 subjects with ischemic stroke(43.9%).Both DSA and MRA displayed bilateral lesions,in which we could find abnormal vascular networks just like smog.In teenager group,occlusions of bilateral middle cerebral artery(64.5%)and anterior cerebral artery(61.3%)were dominant.In adult group,occlusions of bilateral internal carotid artery(45.1%)and middle cerebral artery(46.3%)were dominant.In addition,there were 5 cases with occlusions of posterior cerebral artery and one case with vertebral artery stenosis.Conclusion Morbidity in adult group was higher than in teenager group.Ischemic stroke often occured in children with MMD,while hemorrhagic stroke often occured in adult group.DSA is diagnostic golden standard for MMD,which could accurately display site and degree of injured blood vessel.MRA is being applied more and more extensively to clinical because of its superiority,including non-invasive,non-radioactivity and no demand contrast medium,and so on.In addition,MRA also could reflect the condition on brain parenchyma and intracranial blood vessel from both morphology and function,which was first selected examination means for screening,diagnosis and follow-up of MMD.Furthermore,some scholars think that transcranial doppler(TCD) could be one of method to screen stenosis and occlusion of cerebral arteries,which could detect patients with moyamoya disease in earlier period and apply to follow-up.

Key words: moyamoya disease, digital subtraction angiography, magnetic resonance angiography