肝豆状核变性脑CT和MRI诊断

目的分析肝豆状核变性(HLD)的脑CT和MRI征象,进一步提高对HLD病的认识及诊断的正确率。方法对临床和生化检查证实36例HLD的脑CT和MRI征象及临床表现进行回顾性分析。结果 CT检查26例中主要表现为对称性基底节区低密度影。30例MRI呈对称性条状或新月形长T1长T2信号,FIAIR高信号、或稍短T1短T2信号,典型者依受累部位不同分别为"啄木鸟"、"八字","展翅蝴蝶"样改变等,其中病灶位于壳核30例,尾状核16例,丘脑15例,苍白球、红核各3例,黑质、大脑脚网状结构、脑桥桥核、额叶白质各2例,15例有轻度或重度脑萎缩。结论 HLD脑CT及MRI最常见征象是基底节区低密度影或异常信号和不同程度脑萎缩,以双侧豆状核区对称性低密度或异常信号最具特征性。

CT and MRI diagnosis of (on) hepatolenticular degeneration

Objective To improve the MRI diagnostic accuracy and the under Standing of hepatolenticular degeneration(HLD) by analyzing the magnetic rescn ance imaging(MRI) features.Methods MRI of brain and abdomen was performed in 36 patients with clinically proved HLD,Results Positive signs were found by CT soans.It was important manifestaion that low density in brain occurred bilaterally and symmetrically.The putamen was affected on MR in all cases,the caudate nucleus in 16.The thalami in 15,the globus pallidus in 3,the red nucleus in 3,the substantis nigra in 2,the midbrain in 2,the pons in 2,the white matter of frontal lobi in 2,According to the different basal ganglia involved in brain,resembling "woodpecker"or "butterfly spreading the wing"in appearance were showed on the MR images respectively,Splenomegaly was present in 15 patients.Conclusion The familiar craniocerebra1 MRI fealures of HLD include encephalatrophy and abnormal signals in basal seg ment region especially the symmetrical long T1 signal,long T2 signal and hyper intensity on FLAIR in bilateral lentiform nucleus.