Strong M J, Grace G M, Orange J B, Leeper H A
Department of Clinical Neurological Sciences, University of Western Ontario, London, Canada.
J Clin Exp Neuropsychol. 1996 Apr;18(2):291-303. doi: 10.1080/01688639608408283.
Amyotrophic lateral sclerosis (ALS) is a progressive, adult-onset neurodegenerative disorder manifesting as a relentless loss of motor capabilities and, ultimately, death. Traditionally thought to affect solely the lower motor neurons and corticospinal tracts, recent studies suggest that the pathogenic process of ALS is more extensive, involving dysfunction of cortical grey and white matter with clinical correlates of impairment in cognition and language. The impact of speech and motor deficits are discussed in relation to the issues of assessment of cognition and language. Three case studies are presented for illustrative purposes. Finally, direction for future research to investigate cognitive dysfunction in ALS are presented.
肌萎缩侧索硬化症(ALS)是一种进行性、成年起病的神经退行性疾病,表现为运动能力持续丧失,最终导致死亡。传统上认为该疾病仅影响下运动神经元和皮质脊髓束,但最近的研究表明,ALS的致病过程更为广泛,涉及皮质灰质和白质功能障碍,并伴有认知和语言障碍的临床相关性。本文将结合认知和语言评估问题讨论言语和运动缺陷的影响。为说明目的,本文介绍了三个病例研究。最后,本文提出了未来研究ALS认知功能障碍的方向。