Amyotrophic lateral sclerosis (ALS) is a fatal neurological disease associated with neurodegeneration and intracellular pathological 43-kDa transactive response sequence DNA-binding protein (TDP-43) positive inclusions. The various clinical symptoms, such as motor disorders and cognitive impairment, reflect the degeneration of certain areas of the nervous system. Since the discovery of the significance of pathological TDP-43 for human disease including ALS, there has been an increasing number of studies reporting on the distribution and severity of neurodegeneration. These have rekindled the old debate about whether the first or second motor neuron is the primary site of degeneration in ALS. To shed light on this question, the following is a review of the relevant neuropathological studies.

肌萎缩侧索硬化症 (ALS) 是一种致命的神经系统疾病，与神经变性和细胞内病理性 43-kDa 反式反应序列 DNA 结合蛋白 (TDP-43) 阳性包涵体有关。各种临床症状，如运动障碍和认知障碍，反映了神经系统某些区域的退化。自从发现病理性 TDP-43 对包括 ALS 在内的人类疾病的重要性以来，越来越多的研究报告了神经变性的分布和严重程度。这些重新点燃了关于第一个或第二个运动神经元是否是 ALS 退化的主要部位的旧争论。为了阐明这个问题，以下是对相关神经病理学研究的回顾。