2012-08-012024-05-13https://scholars.lib.ntu.edu.tw/handle/123456789/645800摘要：毛毛樣病是一種慢性、進行性腦血管閉塞的疾病，因為顱內大血管閉塞，而導致小血管代償性擴張及亂長而形成異常的血管網，會引起腦部血液灌流障礙。兒童的毛毛樣病與成人的毛毛樣病的臨床表現不太相同，以腦短暫缺血及癲癇等症狀居多，長期下來可能造成智力低下或認知功能異常等。此病的正確診斷需倚賴血管攝影評估顱內血管閉塞嚴重度，並需腦部血液灌流評估工具輔佐，決定手術的時機點及術式。腦部灌流量化值與兒童病患智力的相關及成人認知功能的相關已有文獻報導，不同術式對不同區域腦血流的影響及預後的影響也已有研究證實，但是腦部灌流量的縱向追蹤則少有相關研究，罹患毛毛症兒童在術後至成人期的變化如何、以及不同術式的長期影響都仍未有清楚的研究。本計畫是一個三年的觀察性追蹤研究，選擇磁振造影灌流檢查做為兒童毛毛樣症患者主要的縱向追蹤工具，並佐以上頸動脈超音波-穿顱彩色超音波檢查其頸部與腦部血流的變化狀況。希望縱向的腦灌流的變化資訊，可以增進學界對毛毛病患者腦部長期變化的了解，尤其是兒童患者手術後至成人期之變化。是否在適當時機應該再進行某些部位的手術以改善病患的病情，非常重要。未來這項技術將除了可以做為改良兒童與成人毛毛樣病血管吻合方式的指引。並可以做為其他腦血管疾病的研究工具<br> Abstract: Moyamoya disease (MMD) is characterized by chronic stenoocclusive vasculopathyaffecting the terminal portion of internal carotid artery (ICA) in association with abnormalvascular networks (moyamoya vessels) at basal brain. Clinical presentation of pediatric MMDis different from adult MMD, with ischemic symptoms predominating and resulting inimpairment of intelligence and cognitive function. Conventional angiography is essential inmorphological evaluation of the extent of intracerebral vascular occlusion and the conditionof external cerebral arteries. Cerebral perfusion, a physiological evaluation, plays its role inselection of patients for revascularization surgery and also postoperative evaluation of theclinical outcome.Correlation between intellectual outcome and surgical revascularization in children withMMD has been reported. Improvement in cognitive function and cerebral perfusion aftersurgery in adults with MMD has been reported, too. But the correlation of cognitive functionand hemodynamic disturbances on perfusion study hasn’t been reported in children withMMD. And, not much has been known in the longitudinal and dynamic changes of cerebralperfusion in children with MMD after the revascularization surgery. The long term changes ofcerebral perfusion when the children with MMD grow up haven’t been clarified, either.This 3-year study is aimed to evaluate the longitudinal cerebral hemodynamic changes ofthe children with MMD using the MR angiography/MR perfusion and the color and powerDoppler ultrasonography. With the quantification of longitudinal hemodynamic changes, wemay investigate the vasculogenesis process in MMD patients. By correlating thehemodynamic quantification with the conventional angiographic results, the clinical andneuro-psychological studies of the MMD patients, we will determine whether MR perfusionor ultrasonography serves as a biomarker to predict the functional outcome of the patientswith MMD. Furthermore, we may use this measurement to modify the surgicalrevascularization method to improve cerebral perfusion and clinical outcome. In the future,the model can be extended to the studies of other pediatric or adult cerebrovascular diseases.毛毛樣病磁振造影灌流檢查頸動脈超音波穿顱彩色超音波Moyamoya disease (MMD)MR PerfusionDopplersonography