Amyotrophic lateral sclerosis : the progression mechanism across neuronal junctions

肌萎缩侧索硬化症（ALS）的运动外神经元系统的变性是否继发于运动神经元的丧失？与此相关的是，在一名56岁的男性中，不仅在前角而且在颈脊髓的中间区发现了神经元的损失，该男性在死亡前38年意外截肢了右上臂。这一发现表明，ALS患者脊髓中间区神经元的丢失至少部分是由前角细胞的丢失引起的，ALS患者胸段中间外侧核的神经元主要参与疾病过程，而没有Bunina小体的形成，并且在呼吸机支持的患者中比非呼吸机支持的患者更严重地降低。我们阐明了关岛ALS的神经元变性的机制与关岛的帕金森痴呆综合征的神经元变性的机制基本不同，关岛肌萎缩侧索硬化症最初是典型的肌萎缩侧索硬化症。

Was the degeneration in the extra-motor neuron system of the amyotrophic lateral sclerosis (ALS), induced secondarily to the loss of motor neurons? In relation to this, loss of neurons was found not only in the anterior horn but also in the intermediate zone of the cervical spinal cord in a 56-year-old man who had suffered accidental amputation of the right upper arm 38 years prior to death. This finding indicates that the loss of neurons in the intermediate zone of the spinal cord in ALS patients is sequentially induced at least partly by loss of the anterior horn cells.The neurons in the intermediolateral nucleus at the thoracic segment of the ALS patients, were involved primarily in the disease process without the formation of Bunina bodies, and were more severely decreased in the respirator-supported patients than non-respirator-supported patients.We elucidated that the mechanism of neuronal degeneration of Guam ALS is basically different from that of the parkinsonism-dementia complex of Guam, and that Guam ALS occurs initially as classic ALS.