Services of Gastroenterology and Hepatology, Hospital Universitario Puerta de Hierro, Madrid, Spain.
Rev Esp Enferm Dig. 2009 Nov;101(11):806-11. doi: 10.4321/s1130-01082009001100009.
Acquired chronic hepatocerebral degeneration, acquired hepatolenticular degeneration or pseudo-Wilson is an infrequent disorder with a hepatic origin. Cases in the literature are scarce and it is frequently confused with hepatic encephalopathy and Wilson s disease. The aim of this essay is to report a patient suffering from this disorder due to cirrhosis from non-alcoholic steatohepatitis.
We present a 54-year-old man diagnosed from cirrhosis grade B9 of the Child Pugh classification. He progressively developed a picture with bradylalia, mild postural and action tremor and spatial and temporal disorientation. Further studies demonstrated an increase of the values of hepatic transaminases and a hyperintensity in the basal nuclei in the cerebral magnetic resonance imaging. Clinical and radiological data established the diagnosis of hepatocerebral degeneration.
Acquired chronic hepatocerebral degeneration is a disorder rarely reported in the literature that it is usually confused with other diseases. We alert about the need of having this diagnosis into account with patients developing neurological symptoms after hepatic disease.
获得性慢性肝脑变性、获得性肝豆状核变性或假性威尔逊病是一种罕见的肝源性疾病。文献中的病例很少,常与肝性脑病和威尔逊病相混淆。本文旨在报告一例因非酒精性脂肪性肝炎导致肝硬化的患者患有这种疾病。
我们报告了一名 54 岁的男性,根据 Child-Pugh 分类为 B9 级肝硬化诊断。他逐渐出现了言语缓慢、轻度姿势性和动作性震颤以及空间和时间定向障碍。进一步的研究显示肝转氨酶值升高,大脑磁共振成像显示基底核区出现高信号。临床和影像学资料确立了肝脑变性的诊断。
获得性慢性肝脑变性是一种罕见的文献报道疾病,常与其他疾病混淆。我们提醒注意,对于患有肝脏疾病后出现神经系统症状的患者,需要考虑这种诊断。