Mathis Stéphane, Couratier Philippe, Julian Adrien, Vallat Jean-Michel, Corcia Philippe, Le Masson Gwendal
a Department of Neurology , Neuro-Muscular Unit and ALS Center, CHU de Bordeaux, groupe hospitalier Pellegrin , Bordeaux , France.
b Department of Neurology , ALS center, Centre de compétence SLA-fédération Tours-Limoges, CHU de Limoges , Limoges , France.
Expert Rev Neurother. 2017 Mar;17(3):263-276. doi: 10.1080/14737175.2016.1227705. Epub 2016 Sep 20.
Amyotrophic lateral sclerosis (ALS) is a fatal progressive neurodegenerative disorder affecting both upper and lower motor neurons. Despite much research and effort, no clear insights into a unifying hypothesis for the pathogenesis has so far emerged for this disease. Areas covered: We review the main pathophysiological hypotheses and the potential therapeutic targets in ALS, as well as the management of these patients (in order to improve their survival and quality of life). Expert commentary: ALS is a complex neurodegenerative disease, these days considered as a multisystem disorder with predominant motor symptoms (and various clinical forms). Further comprehension of the pathophysiology of this disease is required, although pathophysiological mechanisms (such as TDP-43) show promise in the search for new therapies. There is still no curative treatment for ALS, but the emergence of multidisciplinary specialized ALS clinics has increased both the quality of life and the survival of these patients.
肌萎缩侧索硬化症(ALS)是一种致命的进行性神经退行性疾病,会影响上下运动神经元。尽管进行了大量研究并付出了诸多努力,但迄今为止,对于该疾病发病机制的统一假说仍未得出明确见解。涵盖领域:我们综述了ALS的主要病理生理假说、潜在治疗靶点以及这些患者的管理(以提高其生存率和生活质量)。专家评论:ALS是一种复杂的神经退行性疾病,如今被视为一种以运动症状为主(且有多种临床形式)的多系统疾病。尽管病理生理机制(如TDP - 43)在寻找新疗法方面显示出前景,但仍需要对该疾病的病理生理学有更深入的理解。目前尚无治愈ALS的方法,但多学科专业ALS诊所的出现提高了这些患者的生活质量和生存率。