Abstract：
Hepatic encephalopathy (HE) is a complex syndrome of neurological and psychiatric signs and symptoms that is caused by portosystemic venous shunting with or without liver disease irrespective of its etiology. The most common presentation of Wilson disease (WD) is liver disease and is frequently associated with a wide spectrum of neurological and psychiatric symptoms. The genetic defect in WD leads to copper accumulation in the liver and later in other organs including the brain. In a patient presenting with Wilsonian cirrhosis neuropsychiatric symptoms may be caused either by the metabolic consequences of liver failure or by copper toxicity. Thus, in clinical practice a precise diagnosis is a great challenge. Contrary to HE in neurological WD consciousness, is very rarely disturbed and pyramidal signs, myoclonus dominate. Asterixis and many other clinical symptoms may be present in both disease conditions and are quite similar. However details of neurological assessment as well as additional examinations could help in differential diagnosis.
摘要：
肝性脑病（HE）是神经和精神体征和症状的复杂综合征，是由有或没有肝病的门体静脉分流引起的，无论其病因如何。Wilson病（WD）最常见的表现是肝病，通常与广泛的神经和精神症状有关。WD的遗传缺陷导致铜在肝脏中积累，后来在包括大脑在内的其他器官中积累。在患有Wilsonian肝硬化的患者中，神经精神症状可能是由肝功能衰竭的代谢后果或铜毒性引起的。因此，在临床实践中，精确诊断是一个巨大的挑战。与神经WD意识中的HE相反，很少受到干扰和锥体的迹象，肌阵鸣占主导地位。Asterixis和许多其他临床症状可能存在于两种疾病中，并且非常相似。然而，神经系统评估的细节以及其他检查可能有助于鉴别诊断。
