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目的探讨烟雾病的临床特征及影像学表现,以提高对本病的诊断率。方法对20例烟雾病患者的临床特征和影像学资料进行回顾性分析。结果临床表现:发病年龄为10~19岁占50%(10/20),40岁以前占90%(18/20),缺血型患者主要为偏瘫、失语,出血型患者主要为头痛、呕吐、意识障碍和偏瘫,20例中反复TIA发作者7例,其中两侧交替发作者2例;CT/MRI:脑梗死6例,脑出血13例(脑室出血4例,再出血3例);梗死或出血病灶发生在基底节6例,脑叶9例,多发性病灶6例,梗死并出血5例;DSA示颈内动脉、大脑前、中动脉狭窄或闭塞,脑基底部烟雾状血管网形成,大脑后动脉(PCA)及其分支增粗者16例,颈外动脉系统向颅内代偿8例。结论对于儿童、青年人出现中风、反复交替性TIA发作、CT/MRI呈多发性脑梗死或出血、脑叶梗死或出血、脑室出血及再出血患者应高度考虑烟雾病,应行DSA检查;DSA可清晰显示烟雾病血管狭窄的程度、闭塞部位及侧支循环的情况。
Objective To explore the clinical features and imaging findings of moyamoya disease in order to improve the diagnosis rate of this disease. Methods The clinical features and imaging data of 20 patients with moyamoya disease were retrospectively analyzed. Results The clinical manifestations were as follows: the age of onset was 50% (10/20) in 10-19 years old and 90% (18/20) before 40 years old. The main types of ischemic patients were hemiplegia, aphasia and hemorrhagic patients were mainly headache and vomiting , Disturbance of consciousness and hemiplegia. Among the 20 patients, 7 patients had repeated TIA, of which 2 patients had alternation on both sides; CT / MRI: 6 patients with cerebral infarction, 13 patients with intracerebral hemorrhage (4 patients with ventricular hemorrhage and 3 patients with rebleeding); Infarction or bleeding in the basal ganglia occurred in 6 cases, 9 cases of lobar, multiple lesions in 6 cases, 5 cases of infarction and bleeding; DSA showed internal carotid artery, cerebral artery, middle artery stenosis or occlusion, basal ganglia vascular network The formation of posterior cerebral artery (PCA) and its branches thickening in 16 cases, the external carotid artery system to compensate for 8 cases of intracranial. Conclusions For children, young people have stroke, repeated TIA episodes, CT / MRI showed multiple cerebral infarction or hemorrhage, cerebral infarction or bleeding, intraventricular hemorrhage and rebleeding patients should be highly considered moyamoya disease, DSA should be performed; DSA Can clearly show the extent of moyamoya disease stenosis, occlusion sites and collateral circulation.