Masui Y, Mozai T, Kakehi K
J Neurol. 1985;232(1):15-9. doi: 10.1007/BF00314034.
In routine liver function tests, 23 of 44 patients with motor neuron disease (MND) had abnormal findings, and there was disturbance of unconjugated bilirubin metabolism in 10 of the 33 patients tested. Liver-biopsy specimens from 10 MND patients were compared by electron microscopic examination with specimens from age-matched controls who had chronic persistent hepatitis. The MND patients had a higher incidence of intramitochondrial inclusions, less abundant mitochondria in a given area of cytoplasm and enlarged mitochondria. Electron-probe X-ray microanalysis of hepatocytic lysosomes found copper in 8 of 13 MND patients, but not in the controls. These findings suggest that the pathogenetic processes in MND may involve not only motor neurons but also hepatic cells.
在常规肝功能检查中，44例运动神经元病（MND）患者中有23例出现异常结果，在接受检测的33例患者中，有10例存在非结合胆红素代谢紊乱。对10例MND患者的肝活检标本与年龄匹配的慢性持续性肝炎对照患者的标本进行电子显微镜检查比较。MND患者线粒体内含物的发生率更高，在给定细胞质区域中线粒体数量较少且线粒体增大。对肝细胞溶酶体进行电子探针X射线微分析发现，13例MND患者中有8例存在铜，但对照患者中未发现。这些发现表明，MND的发病机制可能不仅涉及运动神经元，还涉及肝细胞。
