Yanagisawa N, Shindo M
Department of Medicine (Neurology), Shinshu University School of Medicine.
Rinsho Shinkeigaku. 1996 Dec;36(12):1329-30.
Amyotrophic lateral sclerosis (ALS) is a typical intractable disease affecting the primary and secondary motoneurones resulting in generalized muscular atrophy and weakness with or without spasticity. Dysphagia, dysarthria, and respiratory difficulty are symptoms which cause restriction of ADL and death. Recent achievement in understanding neuronal death in ALS has invited trials on various drugs aiming at neuroprotection and prolongation of the course of ALS. They include inhibition of excitotoxicity of amino acids, suppression of free radicals by lecithinized SOD and various neurotrophic factors. Significant prolongation of life span was obtained by riluzole in a US-Europe trial, but the effects were insignificant in the Japanese nation-wide trial.
肌萎缩侧索硬化症（ALS）是一种典型的难治性疾病，会影响初级和次级运动神经元，导致全身性肌肉萎缩和无力，伴有或不伴有痉挛。吞咽困难、构音障碍和呼吸困难是导致日常生活活动受限和死亡的症状。近期在理解ALS中神经元死亡方面取得的成果引发了针对各种旨在神经保护和延长ALS病程的药物的试验。这些药物包括抑制氨基酸的兴奋性毒性、用卵磷脂化超氧化物歧化酶和各种神经营养因子抑制自由基。在美国-欧洲的一项试验中，利鲁唑使寿命显著延长，但在日本全国性试验中效果不显著。
