推荐回答（4个）
运动神经元病是一系列以上下运动神经元损害为突出表现的慢性进行性神经系统变性疾病。临床表现为上下运动神经元损害的不同组合，特征表现为肌无力和肌萎缩，延髓麻痹及锥体束征。临床上运动神经元病通常分为四种：第一种，进行性肌萎缩，损害仅限于下运动神经元，表现为无力和肌萎缩，而无锥体束征；第二种、进行性延髓麻痹，单独损害延髓运动神经核而表现为咽喉肌和舌肌无力，萎缩；第三种、原发性侧索硬化，仅累及锥体束而表现为无力和锥体束征；第四种、肌萎缩侧索硬化，上下运动神经元均有损害，表现为肌无力，肌萎缩和锥体束征。其中，肌萎缩侧索硬化症最为常见。
1.神经毒性物质累积，谷氨酸堆积在神经细胞之间，久而久之，造成神经细胞的损伤。
2.自由基使神经细胞膜受损。
3.神经生长因子缺乏，使神经细胞无法持续生长、发育。
临床表现
早期症状轻微，易与其他疾病混淆。患者可能只是感到有一些无力、肉跳、容易疲劳等一些症状，渐渐进展为全身肌肉萎缩和吞咽困难。最后产生呼吸衰竭。
依临床症状大致可分为两型：
1.肢体起病型
2.延髓起病型
先期出现吞咽、讲话困难，很快进展为呼吸衰竭。
检查
要早期诊断肌萎缩侧索硬化，除了神经科临床检查外，还需做肌电图、神经传导速度检测、血清特殊抗体检查、腰穿脑脊液检查、影像学检查，甚至肌肉活检。
1、下运动神经元症候群: 以手脚肌肉萎缩为主,四肢肌腱反射正常或消失,患者体内能存在某种自体抗体如GMI等,属一种自体免疫疾病。
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