肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是一种发病机制不明的致死性神经系统变性疾病,主要累及大脑皮质锥体细胞、锥体束、脑干运动神经元及脊髓前角细胞,临床上兼有上和(或)下运动神经元受累表现。由于损害部位不同,常表现为肌无力、肌萎缩、吞咽困难、锥体束征等,感觉系统一般不受累。ALS发病率约为1.5/10万,患病率约为4~6/10万,多数患者3年左右因呼 Amyotrophic lateral sclerosis (ALS) is a fatal neurological degenerative disease with unknown pathogenesis. It mainly involves cerebral cortex pyramidal cells, pyramidal tract, brainstem motor neurons and spinal cord anterior horn cells, and clinical Upper and / or lower motor neuron involvement performance. Due to the different parts of damage, often manifested as muscle weakness, muscle atrophy, dysphagia, pyramidal tract signs, etc., the general feeling of the system is not involved. The incidence of ALS is about 1.5 / 10 million, the prevalence of about 4 to 6/10 million, most patients due to call about 3 years