Hepatolenticular变性;动力障碍现象分析;肝损害程度与病程的关系普通肝病中的神经紊乱。

E. Herz, A. Drew
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引用次数: 20

摘要

肝豆状核变性的临床诊断是基于运动现象的评估,肝功能障碍的证据和角膜边缘色素环的存在。Wilson, westphal2和strumpell3的经典描述以及随后的大量报道使得在大多数病例中可以将这种疾病与其他病理实体区分开来。然而,对运动障碍症状的详细分析仍然缺乏,肝功能检查对诊断的重要性仍在讨论中。根据最近对人类和动物中与各种肝脏疾病相关的神经紊乱的观察,需要再次强调临床图像的特异性,以及脑干病理变化的特异性。这些情况与肝豆状核变性的比较只能建立在
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Hepatolenticular degeneration; analysis of dyskinetic phenomena; relation of degree of hepatic damage to course of the disease; nervous disorders in ordinary disease of the liver.
THE CLINICAL diagnosis of hepatolenticular degeneration is made on the basis of the evaluation of the motor phenomena, the evidence of hepatic dysfunction and the presence of the ring of a pigment at the margin of the cornea. The classic descriptions of Wilson,1Westphal2and Strumpell3and the numerous subsequent reports make it possible to delimit this disease from other pathologic entities in the majority of cases. However, a detailed analysis of the dyskinetic symptoms is still wanting and the importance of liver function tests for diagnostic consideration is still under discussion. The specificity of the clinical picture, as well as that of the pathologic changes in the brain stem, needs to be stressed again in the light of recent observations on nervous disorders associated with various hepatic diseases in man and in animals. A comparison of these conditions with hepatolenticular degeneration can be established only after
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