烟雾病的临床特点与影像学分析

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。

Aanalysis of clinical characteristics and angiographic findings for moyamoya disease

Objective To discuss the clinical features and angiographic findings of the patients with moyamoya disease(MMD). Methods Clinical manifestations and angiographic findings of 20 patients with MMD were reviewed retrospectively. Results Ten cases (50%) aged from 10 to 19 and 18 cases (90%) were below 40 years old. Hemiplegia and aphasia were the most common presentations in ischemic cases while hemorrhagic cases often manifested headache, vomiting, disturbance of consciousness and hemiplegia. Recurrent attacks of ischemia were found in 7 cases and alternately bilateral attacks in 2 cases. Six cases showed infarction on CT/MRI, while 13 cases showed hemorrhage. Four cases manifested intraventricular hemorrhage and 3 cases had rebleeding episodes. Lesions of 6 cases were located in the basal ganglia and 9 patients in cerebral lobes. Six cases showed infarcts in various distributions and 5 cases had hemorrhagic infarcts. DSA revealed stenosis or occlusions of ICA, ACA and MCA and development of basal moyamoya vessels. Dilation and branch extention of the PCA were found in 16 cases. Blood flows from ECA system to ICA systems were noted in 8 cases. Conclusions Moyamoya disease should be considered and DSA should be performed in all young patients presenting with stroke, recurrent and alterate attacks of ischemia, atypical lesions and intraventricular hemorrhage on CT/MRI. DSA could show clearly the stenotic degree, occlusive site and collateral circulations of the MMD.