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Imagiologic features and the relative imaging factors in hepatolenticular degeneration
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- Author:
- No author available
- Journal Title:
- CHINESE JOURNAL OF RADIOLOGY
- Issue:
- 5
- DOI:
- 10.3760/j.issn:1005-1201.2002.05.005
- Key Word:
- 肝豆状核变性;磁共振成像;体层摄影术,X线计算机
Abstract： 目的研究肝豆状核变性(HLD)的CT、MR和B超表现,探讨影响影像表现的相关因素.方法 54例HLD患者中,男35例,女19例,年龄3～40岁.作CT检查29例, MR检查 11例,其中两者同时检查者5例.B超检查26例.结果 (1)脑部MRI呈长T1、长T2信号9例,稍短T1、短T2信号2例.病灶位于壳核11例,尾状核8例,丘脑5例, 苍白球2例,红核2例,黑质3 例,大脑脚网状结构1例,脑桥桥核2例,额叶白质1例.典型者依受累部位不同,分别表现为"啄木鸟"、"八字"、"展翅蝴蝶"样改变等.(2)脑部CT检查25例中,阳性18例(72%),阴性 7例(28%).主要表现为双侧基底节区对称性低密度影.(3)肝脏B超26例均示慢性肝病声像学改变,伴肝硬化12例,脾肿大11例,腹水9例,胆囊水肿8例,双边影5例,胆囊结石3例.结论 (1)MRI T2信号和CT密度改变与临床表现往往不相平行. T1信号可用于随访比较,但不易定量.(2)HLD几乎都有肝损害,肝损害先于脑损害;早期肝损害呈可逆性,脑损害呈对称性;中期肝损害呈静止性;后期脑损害呈萎缩性.(3) 提出影响CT、MR图像表现的4对矛盾因素:代谢性全身性疾病与基因选择性亲和性;铜沉积与细胞损伤;内源性自主排铜与组织细胞修复;外源性驱铜治疗与组织细胞修复.
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