ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS.
ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS.
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DOI：
10.1212/wnl.0000000000006317
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发表时间：
2018-10-09
期刊：
影响因子：
9.9
通讯作者：
Abrahams S
中科院分区：
文献类型：
--
作者：
Crockford C;Newton J;Lonergan K;Chiwera T;Booth T;Chandran S;Colville S;Heverin M;Mays I;Pal S;Pender N;Pinto-Grau M;Radakovic R;Shaw CE;Stephenson L;Swingler R;Vajda A;Al-Chalabi A;Hardiman O;Abrahams S
目的：探讨肌萎缩侧索硬化症(ALS)患者的King‘s临床分期与爱丁堡认知行为筛查(ECAS)的认知行为改变之间的关系。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名健康对照的大型多中心观察队列。参与者接受了ECAS，并根据他们在测试时的King临床疾病阶段被分为独立的小组。除了视觉空间功能，患者和对照组在ECAS的所有分项测试上都观察到了显著的差异。在ALS特异性功能(执行、语言、字母流利性)和ECAS总分方面，观察到了跨疾病阶段的显著横断面效应，但对于ALS非特异性功能(记忆、视觉空间)则没有。ALS特异性损伤率和行为改变也与疾病分期有关。认知功能与疾病分期之间的关系可能与字母流畅性受损有关，而所有行为领域的发生率在King‘s病程后期较高。球部体征的存在，但与发病部位无关，与ALS特异性、ECAS总分和行为评分显著相关。ALS特异性认知障碍和行为障碍的发生率随疾病分期的加重而增加。在终末期疾病中，只有一小部分患者没有神经心理障碍。球部症状的存在夸大了疾病不同阶段之间的差异。这些发现表明，认知和行为改变应纳入ALS诊断标准，并应包括在未来的分期系统中。
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). 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. 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. 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.