肌萎缩侧索硬化症（ALS）特异性认知和行为变化与疾病进展阶段相关。
ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS.
机构信息
From Human Cognitive Neuroscience (C.C., J.N., T.B., R.R., S.A.), Psychology, Philosophy, Psychology and Language Sciences, Euan MacDonald Centre for Motor Neurone Disease Research (C.C., S.A.), and Alzheimer Scotland Dementia Research Centre (R.R.), University of Edinburgh; Anne Rowling Regenerative Neurology Clinic (J.N., S.C., S.P., R.R., L.S., R.S., S.A.), Royal Infirmary of Edinburgh, UK; Academic Unit of Neurology (K.L., M.H., I.M., M.P.-G., A.V., O.H.), Trinity College Dublin; Departments of Psychology (K.L., I.M., N.P., M.P.-G.) and Neurology (O.H.), Beaumont Hospital, Dublin, Ireland; and Maurice Wohl Clinical Neuroscience Institute (T.C., C.E.S., A.A.-C.), Department of Basic and Clinical Neuroscience, King's College London, UK.
出版信息
Neurology. 2018 Oct 9;91(15):e1370-e1380. doi: 10.1212/WNL.0000000000006317. Epub 2018 Sep 12.
OBJECTIVE
To elucidate the relationship between disease stage in amyotrophic lateral sclerosis (ALS), as measured with the King's Clinical Staging System, and cognitive and behavioral change, measured with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS).
METHODS
A large multicenter observational cohort of 161 cross-sectional patients with ALS and 80 healthy matched controls were recruited across 3 research sites (Dublin, Edinburgh, and London). Participants were administered the ECAS and categorized into independent groups based on their King's clinical disease stage at time of testing.
RESULTS
Significant differences were observed between patients and controls on all subtests of the ECAS except for visuospatial functioning. A significant cross-sectional effect was observed across disease stages for ALS-specific functions (executive, language, letter fluency) and ECAS total score but not for ALS-nonspecific functions (memory, visuospatial). Rates of ALS-specific impairment and behavioral change were also related to disease stage. The relationship between cognitive function and disease stage may be due to letter fluency impairment, whereas higher rates of all behavioral domains were seen in later King's stage. The presence of bulbar signs, but not site of onset, was significantly related to ALS-specific, ECAS total, and behavioral scores.
CONCLUSION
ALS-specific cognitive deficits and behavioral impairment are more frequent with more severe disease stage. By end-stage disease, only a small percentage of patients are free of neuropsychological impairment. The presence of bulbar symptoms exaggerates the differences observed between disease stages. These findings suggest that cognitive and behavioral change should be incorporated into ALS diagnostic criteria and should be included in future staging systems.
目的
阐明肌萎缩侧索硬化症（ALS）的疾病阶段（采用 King 临床分期系统进行评估）与认知和行为变化之间的关系，认知和行为变化通过爱丁堡认知和行为 ALS 筛查（ECAS）进行评估。
方法
在 3 个研究地点（都柏林、爱丁堡和伦敦）招募了 161 名横断面 ALS 患者和 80 名健康匹配对照的大型多中心观察队列。对参与者进行 ECAS 测试，并根据他们在测试时的 King 临床疾病阶段分为独立组。
结果
除视觉空间功能外，ECAS 的所有子测试在患者和对照组之间均存在显著差异。在 ALS 特异性功能（执行功能、语言功能、字母流畅性）和 ECAS 总分方面，在疾病阶段之间观察到显著的横断面效应，但在 ALS 非特异性功能（记忆功能、视觉空间功能）方面没有观察到。ALS 特异性损伤和行为改变的发生率也与疾病阶段有关。认知功能与疾病阶段之间的关系可能与字母流畅性损伤有关，而在更高的 King 分期中，所有行为领域的发生率更高。延髓体征的存在，而不是发病部位，与 ALS 特异性、ECAS 总分和行为评分显著相关。
结论
随着疾病阶段的加重，ALS 特异性认知缺陷和行为障碍更为常见。在终末期疾病中，只有一小部分患者没有神经心理损伤。延髓症状的存在夸大了疾病阶段之间的差异。这些发现表明，认知和行为变化应纳入 ALS 诊断标准，并应包含在未来的分期系统中。
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