Tandan R, Bradley W G
Ann Neurol. 1985 Oct;18(4):419-31. doi: 10.1002/ana.410180402.
The pathogenesis of the motor neuronal degeneration in amyotrophic lateral sclerosis (ALS) is unclear, though several possible etiological factors are currently being investigated. A unifying hypothesis will have to explain the diverse geographical occurrence, clinical features, and selective vulnerability and relative resistance of different neuronal populations in the disease. It is possible that different biochemical defects underlie this diversity, or alternatively that the many factors incriminated in the etiology may act upon an underlying genetic-biochemical abnormality to trigger premature neuronal death. Viruses, metals, endogenous toxins, immune dysfunction, endocrine abnormalities, impaired DNA repair, altered axonal transport, and trauma have all been etiologically linked with ALS, but convincing research evidence of a causative role for any of these factors is yet to be demonstrated.
肌萎缩侧索硬化症(ALS)中运动神经元变性的发病机制尚不清楚,尽管目前正在研究几种可能的病因。一个统一的假说必须解释该疾病在不同地理区域的发生情况、临床特征以及不同神经元群体的选择性易损性和相对抗性。有可能不同的生化缺陷是这种多样性的基础,或者在病因学中涉及的许多因素可能作用于潜在的遗传 - 生化异常,从而引发神经元过早死亡。病毒、金属、内源性毒素、免疫功能障碍、内分泌异常、DNA修复受损、轴突运输改变和创伤都在病因学上与ALS有关,但尚未证实这些因素中任何一个具有致病作用的令人信服的研究证据。