肝豆状核变性的影像学诊断  

Diagnostic imaging of Wilson's disease

作　　者：黄福气[1] 陈义辉[1] 郑燕琴[1] 

机构地区：[1]武警福建总队医院放射科,福州350019

出　　处：《武警医学》2001年第8期451-454,共4页Medical Journal of the Chinese People's Armed Police Force

摘　　要：目的　了解肝豆状变性的影像学表现 ,明确病变范围及程度。方法　采用普通X线摄片 ,MRI或CT对颅脑进行扫描。结果　X线表现为四肢骨远端、椎体、骨盆、腕关节、指间关节等均可受累 ,表现形式为骨质脱钙、软化、碎裂、稀疏和骨质增生 ,CT表现为多发双侧对称性豆状核及周围低密度病变以及其它脑组织萎缩等表现 ;MRI表现为豆状核、丘脑、尾状核及齿状核在T2 加权图像上呈对称异常高信号 ,在T1加权图像上呈低信号。结论　Wilson’s病在普通X线摄片、CT及MRI扫描均能检出病变 ,CT或MRI更能明确病变范围。Objective To evaluate the manifestations of diagnostic imaging,including the range and extent,of hepatolenticulat degeneration (Wilson's disease).Methods Radiograpby,MRI or CT scanning were performed in 42 patients with Wilson's disease.Results Radiograpby showed extensive bone involvement at distal end of extremities,vertebrae,pelvis,carpal joints,interphalangeal joints,etc,manifested as decalcification,malacia,cleavage,rarefaction and hyperosteogeny.On CT scanning,multiple,symmetrical low density foci were foumd in lenticular nucleus and adjoining area,and atrophy foci were found in other regions of brain.In MRI,t 2 WI presented syrnmetrical hyperinten sive signals in lenticular nucleus,thalamus,caudate and dendate nuclei.Conclusions R adiography,MRI and CT scanning can detect abnormalities and determine the extent of lesions in Wilson's disease.

关 键 词：肝豆状核变性 X线 影像学诊断 代谢障碍病 隐性遗传病 

分 类 号：R742.4[医药卫生—神经病学与精神病学] R445[医药卫生—临床医学]