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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 5 4例HLD患者中 ,男 35例 ,女 19例 ,年龄 3～ 40岁。作CT检查 2 9例 ,MR检查 11例 ,其中两者同时检查者 5例。B超检查 2 6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 2 5例中 ,阳性 18例 (72 % ) ,阴性 7例 (2 8% )。主要表现为双侧基底节区对称性低密度影。 (3)肝脏B超 2 6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)MRIT2 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
Objective To study the CT, MR and B ultrasound findings of hepatolenticular degeneration (HLD) and to explore the related factors that affect the imaging performance. Method 5 4 cases of HLD patients, 35 males and 19 females, aged 3 to 40 years. 29 cases were examined by CT, 11 cases by MR examination, and 5 cases were examined by both. B ultrasound examination of 26 cases. Results (1) Brain MRI showed long T1, long T2 signal in 9 cases, short T1, short T2 signal in 2 cases. The lesions were located in 11 cases of putamen, 8 cases of caudate nucleus, 5 cases of thalamus, 2 cases of globus pallidus, 2 cases of red nucleus, 3 cases of substantia nigra, 1 case of brain foot and reticular structure, 2 cases of pontine nucleus and 2 cases of frontal lobe white matter example. Typical people are affected by the different parts of the performance, respectively, “Woodpecker”, “Character”, “Butterfly” and other changes. (2) Brain CT examination of 25 cases, positive in 18 cases (72%), negative in 7 cases (28%). Mainly for bilateral basal ganglia symmetry of low density. (3) Liver B ultrasound 26 cases showed chronic liver disease, acoustic image changes, with cirrhosis in 12 cases, splenomegaly in 11 cases, ascites in 9 cases, gallbladder edema in 8 cases, bilateral bilateral shadow in 5 cases, gallbladder stones in 3 cases. Conclusion (1) MRIT2 signal and CT density changes and clinical manifestations are often not parallel. T1 signal can be used for follow-up comparison, but not quantitative. (2) HLD almost always has liver damage, and liver damage precedes brain damage. Early liver damage is reversible and brain damage is symmetrical. Mid-term liver damage is still, while late brain damage is atrophic. (3) Four pairs of contradictory factors affecting CT and MR images were proposed: metabolic systemic diseases and gene selective affinity; copper deposition and cell injury; endogenous autologous copper and tissue repair; exogenous flooding Copper rule