Spahr L, Butterworth R F, Fontaine S, Bui L, Therrien G, Milette P C, Lebrun L H, Zayed J, Leblanc A, Pomier-Layrargues G
Liver Unit, Hopital Saint-Luc, University of Montreal, Canada.
Hepatology. 1996 Nov;24(5):1116-20. doi: 10.1002/hep.510240523.
Increasing evidence suggests that manganese deposition is responsible for the T1-weighted magnetic resonance imaging (MRI) signal hyperintensity consistently observed in pallidum of cirrhotic patients. However, the relationship between blood manganese and the etiology or severity of liver disease, as well as the neurological symptomatology in these patients, has not been well established. In the present study, blood manganese concentrations were measured by atomic absorption spectrometry together with MRI and neurological evaluation in 57 cirrhotic patients with various etiologies and severity of liver disease. Blood manganese concentrations were elevated in 67% of cirrhotic patients and were significantly higher in patients with previous portacaval anastomoses or transjugular intrahepatic portosystemic shunt (TIPS). Pallidal signal hyperintensity was observed in 88% of patients, and significant correlations were demonstrated between blood manganese and pallidal index (PI) (a measure of pallidal signal hyperintensity), as well as Child-Pugh score. Assessment of extrapyramidal symptoms using the Columbia rating scale revealed a significant incidence of tremor, rigidity, or akinesia in up to 89% of cirrhotic patients. However, there was no significant correlation between blood manganese and extrapyramidal symptoms, although severity of akinesia was significantly greater in Child-Pugh C patients. Extrapyramidal symptoms could result from a toxic effect of manganese on basal ganglia dopaminergic function. These findings further support a role for manganese in the etiology of pallidal MRI signal hyperintensity in patients with chronic liver disease.
越来越多的证据表明，锰沉积是肝硬化患者苍白球中持续观察到的T1加权磁共振成像（MRI）信号高强化的原因。然而，血锰与肝病病因或严重程度以及这些患者的神经症状之间的关系尚未完全明确。在本研究中，采用原子吸收光谱法对57例不同病因和肝病严重程度的肝硬化患者进行血锰浓度测定，并同时进行MRI检查和神经学评估。67%的肝硬化患者血锰浓度升高，既往有门腔静脉吻合术或经颈静脉肝内门体分流术（TIPS）的患者血锰浓度显著更高。88%的患者出现苍白球信号高强化，血锰与苍白球指数（PI，一种衡量苍白球信号高强化的指标）以及Child-Pugh评分之间存在显著相关性。使用哥伦比亚评定量表评估锥体外系症状发现，高达89%的肝硬化患者有震颤、强直或运动不能的显著发生率。然而，血锰与锥体外系症状之间无显著相关性，尽管Child-Pugh C级患者的运动不能严重程度显著更高。锥体外系症状可能是锰对基底节多巴胺能功能产生毒性作用的结果。这些发现进一步支持了锰在慢性肝病患者苍白球MRI信号高强化病因中的作用。
