获得性肝脑变性的临床与影像学特点

目的分析获得性肝脑变性的临床和影像学特征，探讨其规律性。方法分析9例获得性肝脑变性病例的临床表现、实验室检查和影像学改变的数据。结果5例既往有明确的肝硬化病史，4例没有明确的肝病病史，发病后检查分别发现肝血管病、肝胰脾等弥漫钙化或者肝硬化。主要临床表现为：精神异常、认知能力下降、帕金森病样综合征、构音障碍、共济失调、睡眠障碍。肝酶学轻中度异常，而血氨均升高。脑电图提示脑电活动呈弥漫性减慢，4例出现三相波节律。影像学改变为苍白球、大脑脚、小脑和大脑皮层下对称性短T1加权像信号，以及基底节、中脑、脑桥、延髓橄榄核对称性长T2加权像信号。结论获得性肝脑变性是肝细胞和肝血管病变引起的神经功能损害综合征，肝酶改变、血氮升高和脑电图符合代谢性疾病改变，结合特征性影像学改变，对疾病的诊断具有重要意义。

The characteristics of the clinical and imaging findings in acquired hepatocerebral degeneration

Objective To explore the regularities of acquired hepatocerebral degeneration(AHD)by analyz- ing the clinical and neuroimaging characteristics.Methods The data of clinical manifestation,laboratory examina- tion and neuroimaging findings in 9 cases of AHD had been collected.Results 5 cases had definite previous cirrho- sis,while 4 cases had no previous chronic liver disease,which afterward turned out to be hepato-vascular disease, diffuse calcification of liver,pancreas and spleen,and cirrhosis.The main clinical manifestation included mental dis- turbance,cognitive abilities decline,Parkinson-like syndrome,dysarthria,ataxia,and sleep disorder.Hepato-enzy- mology revealed mild or moderate elevation,and all cases had high level of blood ammonia.EEG showed diffuse slow electrical activity of brain,4 of which had triphasic wave.Magnetic resonance imaging of the brain revealed symmetrical high T_1signals in the bilateral globus pallidus,cerebral peduncle,cerebellum and subcortex and T_2high- signal intensities in the bilateral basal ganglia,midbrain,pons,and medulla olivary nucleus.Conclusion AHD is a neurologic syndrome associated with hepatocellular degeneration and hepato-vascular diseases.Hepato-enzymologic changes,elevated level of blood ammonia,EEG consistent with metabolic disorder,combined with typical manifesta- tion of neuroimaging,have pivotal significance in the diagnosis of AHD.