25例烟雾病临床影像特点观察

The Clinical and Imaging Observation on Moyamoya Disease: with 25 Patients

目的探讨烟雾病的临床特征、影像学特点。方法对25例烟雾病患者首发症状、临床表现及影像学结果进行分析。结果烟雾病发病平均年龄较轻,女性多于男性;少儿烟雾病患者以缺血改变为主(87.5%),成年烟雾病患者缺血改变(58.8%)与出血改变(41.2%)相当;临床以头痛、头晕、肢体无力或偏瘫或交替性瘫痪为主要表现,同时可伴失语、智能下降、视力下降、癫痫发作、小舞蹈发作等表现。14例行头CT检查仅示颅内病灶,均未提示烟雾病;25例均行MRI和MRA检查发现颅内病灶,显示颈内动脉虹吸末段和大脑前或中动脉近段狭窄或闭塞,并有脑基底部异常血管网及侧支循环形成,均被诊断为烟雾病。其中9例又行数字减影血管造影术(DSA),结果与其MRA相符。结论头颅MRI和MRA是诊断烟雾病理想的非介入性检查方法。

Objective To elucidate the clinical features and imaging characters of moyamoya disease. Methods The first onset symptoms ,clinical manifestations and imaging results of 25 cases of Moyamoya patients were studied. Results The average onset age of moyamoya disease was very young. Female was dominant in our studied. Younger patients with moyamoya disease mainly suffered cerebral isehemia （87.5%）. Adult patients suffered more cerebral hemorrhage （41.2%）. Among clinical manifestations, headache, vertigo, paralysis, hemiplegia or alternative hemiplcgia were common symptoms. Sometimes aphasia, dementia, visual acuity were decreasing, epilepsy and chorea minor might occur. 14 cases had received CT examination, but none was diagnosed moyamoya discase by CT. All patients received MR1 and MRA examinations were diagnosed as moyamoya disease. Nine cases received further DSA examinations and the results were compatible with MRA.Conclusions Head MR1 and MRA were the ideal noninvasive examing methods.