肌萎缩侧索硬化 (ａｍｙｏｔｒｏｐｈｉｃｌａｔｅｒａｌｓｃｌｅｒｏｓｉｓ,ＡＬＳ)是一种以脑和脊髓中选择性的大运动神经元的变性为特征的神经系统变性疾病。 10 %～ 2 0 %的患者有家族性。家族性与散发性的ＡＬＳ具有共同的临床和组织病理学特点。临床表现为缓慢起病 ,进行 Amyotrophic lateral sclerosis (ALS) is a degenerative disease of the nervous system characterized by the degeneration of selective large motor neurons in the brain and spinal cord. 10% ~ 20% of patients have familial. Familial and sporadic ALS share common clinical and histopathological features. Clinical manifestations of slow onset, carried out