King's Health Partners Centre for Neurodegeneration Research, Department of Psychology, Institute of Psychiatry, King's College London, London SE5 8AF, UK.
Lancet Neurol. 2013 Apr;12(4):368-80. doi: 10.1016/S1474-4422(13)70026-7. Epub 2013 Mar 18.
Increased awareness of cognitive and behavioural change in amyotrophic lateral sclerosis has been driven by various clinic-based and population-based studies. A frontotemporal syndrome occurs in a substantial proportion of patients, a subgroup of whom present with frontotemporal dementia. Deficits are characterised by executive and working-memory impairments, extending to changes in language and social cognition. Behaviour and social cognition abnormalities are closely similar to those reported in behavioural variant frontotemporal dementia, implying a clinical spectrum linking amyotrophic lateral sclerosis and frontotemporal dementia. Cognitive impairment should be considered in clinical management, but few specialist assessment resources are available, and thus the cognitive status of most patients is unknown. Standard assessment procedures are not appropriate to detect dysfunction due to progressive physical disability; techniques that better measure the problems encountered by this group of patients are needed to further establish disease effects. Screening instruments are needed that are validated specifically for amyotrophic lateral sclerosis, encompass the heterogeneity of impairment, and accommodate physical disability.
由于各种基于临床和基于人群的研究，人们对肌萎缩侧索硬化症的认知和行为变化的认识有所提高。相当一部分患者出现额颞叶综合征，其中一部分患者表现为额颞叶痴呆。这些缺陷的特征是执行功能和工作记忆受损，语言和社会认知也发生变化。行为和社会认知异常与行为变异型额颞叶痴呆报告的异常非常相似，这意味着肌萎缩侧索硬化症和额颞叶痴呆之间存在临床谱系。在临床管理中应考虑认知障碍，但可用的专业评估资源很少，因此大多数患者的认知状况未知。标准评估程序不适合检测因进行性身体残疾而导致的功能障碍；需要更好地测量这组患者所遇到的问题的技术，以进一步确定疾病的影响。需要专门针对肌萎缩侧索硬化症进行验证的筛查工具，涵盖损伤的异质性，并适应身体残疾。
