肌萎缩侧索硬化(ALS)是一种运动神经元病,可侵犯脊髓前角、脑干及皮质运动神经元等,而致上下运动神经元合并受损。ALS人群患病率(0.8～7.3)/106,年发病率约(0.2～2.4)/106,男女之比为(1.4～2.5):1,平均存活时间一般为31～43个月[1]。ALS主要为散发型,5%～10%为家族型,在20%的家族型患者中,有明确的SOD1基因突变。关于ALS的病因假说主要包括兴奋性氨基酸假说、遗传基因假说、自体 Amyotrophic lateral sclerosis (ALS) is a motor neuron disease that can invade the spinal cord anterior horn, brainstem and cortical motor neurons, resulting in damage to the upper and lower motor neurons. The prevalence of ALS is between 0.8 and 7.3 per 106, with an annual incidence of about 0.2 to 2.4 / 106 and a ratio of men to women of 1.4 to 2.5: 1, with an average survival of 31-43 months [1] . ALS mainly sporadic, 5% to 10% of the family type, in 20% of familial patients, there is a clear SOD1 gene mutation. Etiology hypothesis about ALS mainly includes excitatory amino acid hypothesis, genetic hypothesis, self