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尽管肌萎缩性侧索硬化(ALS)的发病机理仍不清楚。近来研究认为,可能与营养代谢因素有关。在未经选择的21例ALS患者中,进行肝活检发现有下述超微结构改变:(1)巨大的线粒体;(2)线粒体内的类晶体(Paracrystalline)包涵体;(3)粗糙内质网的结构破坏;(4)滑面内质网的增生;和/或(5)窦状隙周围纤维化。这表明,在普遍的ALS患者中都有肝脏形态学的改变。尤其是类晶体包涵体的出现,与有肝病的对照组相比,对照组仅发现2.5%,而ALS患者则高达66.7%。已知线粒体内类晶体包涵体可在多种疾病中发现,如氟烷性肝炎、酒精性肝病、高脂蛋白血症、
Although the pathogenesis of amyotrophic lateral sclerosis (ALS) remains unclear. Recent studies suggest that may be related to nutrient metabolism. In 21 patients with unselected ALS, liver biopsies were found to have the following ultrastructural changes: (1) giant mitochondria; (2) mitochondrial Paracrystalline inclusions; (3) rough endoplasmic reticulum Disruption of the structure of the network; (4) proliferation of the slip surface endoplasmic reticulum; and / or (5) fibrosis around the sinusoids. This shows that in the general ALS patients have liver morphological changes. In particular, the presence of crystal-like inclusions in the control group was found to be only 2.5% compared to the control group with liver disease, compared with 66.7% for patients with ALS. It is known that intra-mitochondrial crystalloid inclusion bodies are found in a variety of diseases such as halothane, alcoholic liver disease, hyperlipoproteinemia,