Jackson C E, Bryan W W
Department of Medicine/Neurology, University of Texas Health Science Center at San Antonio 78284-7883, USA.
Semin Neurol. 1998;18(1):27-39. doi: 10.1055/s-2008-1040859.
Amyotrophic lateral sclerosis (ALS) is a motor neuron disease with evidence of both anterior horn cell and corticospinal tract degeneration. The incidence of ALS is 1 to 2.5 cases per 100,000 population and the disease occurs primarily in adult life. The etiology of sporadic ALS remains unknown, although 5 to 10% of cases are familial. The diagnosis of ALS requires the presence of both upper and lower motor neuron findings and progressive motor dysfunction. Several theories regarding the pathogenesis of ALS have emerged including glutamate excitotoxicity, free radical oxidative stress, neurofilament accumulation, and autoimmunity. Clinical trials involving antiglutamate agents, antioxidants, immunosuppressants, and growth factors have shown no substantial benefit in slowing progression, with death usually occurring 2 to 5 years following the onset of symptoms. The management of ALS patients requires a multidisciplinary team that can provide the numerous medical and physical interventions necessary to treat weakness and fatigue, bulbar dysfunction, spasticity and pain, depression, and respiratory failure.
肌萎缩侧索硬化症（ALS）是一种运动神经元疾病，有证据表明存在前角细胞和皮质脊髓束变性。ALS的发病率为每10万人中有1至2.5例，该疾病主要发生在成年人中。散发性ALS的病因尚不清楚，尽管5%至10%的病例为家族性。ALS的诊断需要同时存在上运动神经元和下运动神经元表现以及进行性运动功能障碍。关于ALS发病机制的几种理论已经出现，包括谷氨酸兴奋性毒性、自由基氧化应激、神经丝积聚和自身免疫。涉及抗谷氨酸药物、抗氧化剂、免疫抑制剂和生长因子的临床试验表明，在减缓疾病进展方面没有显著益处，症状出现后通常在2至5年内死亡。ALS患者的管理需要一个多学科团队，该团队能够提供治疗肌无力和疲劳、延髓功能障碍、痉挛和疼痛、抑郁以及呼吸衰竭所需的众多医疗和物理干预措施。
