Messer A, Strominger N L, Mazurkiewicz J E
J Neurogenet. 1987 Jun;4(4):201-13.
The motor neuron degeneration (Mnd) is characterized by a progressive deterioration of motor function (stiff-legged gait, abnormal limb placements and grasping, and finally paralysis; moving from rear to forelimbs). There is a dramatic degeneration of spinal cord motor neurons, more severe in the lumbosacral than in the other regions, as well as variable pathology in the lower cranial nerves. Upper motor neurons of the red nucleus, reticular formation of the pons and medulla, and restricted areas of the cerebral cortex are also affected. Degenerating motor neurons share many characteristics seen in the human disease amyotropic lateral sclerosis, including loss of Nissl substance, increases in lipofuscin and abnormal cytoplasmic inclusions. Additionally, Mnd, like ALS, is a disease of later life.
运动神经元变性（Mnd）的特征是运动功能逐渐恶化（腿部僵硬步态、肢体放置和抓握异常，最终瘫痪；从后肢发展到前肢）。脊髓运动神经元发生显著变性，腰荐部比其他区域更严重，同时下颅神经也存在不同程度的病变。红核、脑桥和延髓的网状结构以及大脑皮层的受限区域的上运动神经元也受到影响。退化的运动神经元具有许多在人类疾病肌萎缩侧索硬化症中看到的特征，包括尼氏体丧失、脂褐素增加和异常的细胞质内含物。此外，Mnd与ALS一样，是一种老年疾病。
