Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis
复制标题
DOI：
10.1007/s00115-007-2354-5
复制
发表时间：
2007-12-01
期刊：
影响因子：
1.1
通讯作者：
Ludolph, A. C.
中科院分区：
文献类型：
--
作者：
Gastl, R.;Ludolph, A. C.
肌萎缩性侧索硬化症（ALS）是一种进行性、退行性运动系统疾病，其特征是上肢和下肢运动功能障碍的体征和症状。这导致局灶性肌萎缩和麻痹影响随意肌的存在。病人在几年后死亡，在大多数情况下死于呼吸衰竭。肌萎缩侧索硬化症是最常见的运动神经元疾病；然而，其病因和发病机制尚不清楚。其遗传基础仅被部分理解，其发病机制与选择性易感性的基本原理及其潜在的治疗后果的理解仍存在重大差距。
Amyotrophic lateral sclerosis (ALS) is a progressive, degenerative disease of the motor system characterized by signs and symptoms of upper and lower motor dysfunction. This results in the presence of focal amyotrophies and pareses affecting voluntary muscles. Patients die after a few years, in most cases by respiratory failure. ALS is the most frequent motor neuron disease; however, its etiology and pathogenesis are only known in fragments. Its genetic basis is only partially understood and major gaps remain in the understanding of its pathogenesis with the basic principle of selective vulnerability and potentially resulting therapeutic consequences.