Miranda M, Caballero L
Departamento Neurología, Hospital Clínico de la Universidad de Chile, Santiago de Chile.
Rev Med Chil. 2001 Sep;129(9):1051-5.
Chronic hepatic encephalopathy (CHE) is a disabling complication of chronic liver failure and porto-systemic shunt. The pathogenesis of CHE remains unclear but increased levels of ammonia are a basic feature. Several clinical and experimental observations support a role for manganese (Mn) in the pathogenesis of this disorder. Increased blood levels of Mn have been described in patients with CHE and this could lead to its accumulation on the basal ganglia and characteristic hyperintensities of basal ganglia as seen on magnetic resonance imaging (MRI) of the brain. We report on the clinical features and characteristic radiologic findings of a patient who presented with the neurologic syndrome of CHE and who had very high blood levels of Mn in the absence of an occupational exposure to this metal. Our report supports the hypothesis that Mn has a role in the pathogenesis of CHE and also suggests that brain MRI is a useful marker of the brain metabolic repercussion due to CHE.
慢性肝性脑病(CHE)是慢性肝衰竭和门体分流的一种致残性并发症。CHE的发病机制尚不清楚,但氨水平升高是其基本特征。多项临床和实验观察结果支持锰(Mn)在该疾病发病机制中的作用。CHE患者的血液中锰水平升高,这可能导致其在基底神经节积聚,并在脑部磁共振成像(MRI)上出现基底神经节特征性高信号。我们报告了一名表现出CHE神经综合征且血液中锰水平极高但无职业性接触该金属情况的患者的临床特征和特征性放射学表现。我们的报告支持锰在CHE发病机制中起作用的假说,也表明脑部MRI是CHE所致脑代谢影响的有用标志物。