肌萎缩侧索硬化( amyotrophic lateral sclerosis,ALS)是一种尚无有效治疗方法的慢性进展性神经退行性疾病,其病因不明但与遗传、饮食、环境、生活习惯等许多因素有关。ALS发病机制包括谷氨酸兴奋性毒性、氧化应激、线粒体功能障碍和轴突运输破坏等。目前获批的ALS治疗药物有利鲁唑、依达拉奉,右美沙芬和奎尼丁组合以及Tofersen,处于临床研究期间的药物较多,有效治疗选择有限。本综述概括阐述了ALS的病因与发病机制,详细总结了ALS的中西医治疗方法,在临床实践中具有较大实用性。

Amyotrophic lateral sclerosis (ALS) is a chronic progressive neurodegenerative disease with no effective treatment. The etiology of ALS is unknown but it is related to many factors such as genetics, diet, environment, and lifestyle habits. The pathogenesis of ALS includes glutamate excitatory toxicity, oxidative stress, mitochondrial dysfunction, and axon transport disruption. Currently, the approved ALS treatment drugs include Riluzole, Edaravone, Dextromethorphan/Quinidine and Tofersen injection. There are many drugs in clinical trails, but effective treatment options are still limited. This review summarizes the etiology and pathogenesis of ALS, and summarizes in detail the traditional Chinese and Western medicine treatment methods for ALS, which has great practicability in clinical practice.