Abstract：
Introduction: Amyotrophic lateral sclerosis (ALS) has heterogeneous manifestations ranging from motor neuron degeneration to cognitive and behavioral impairment. This study aims to clarify the interactions between cognition and behavioral symptoms with relevant disease predictors and with cognitive reserve (CR), quantified through education, physical activity, and occupation proxies. Methods: A prospective sample of 162 ALS patients and 61 controls were evaluated with the Edinburgh Cognitive and Behavioral ALS Screen (ECAS) (dependent variable), a Cognitive Reserve Index questionnaire (CRIq) and demographic data (age and sex), and, for patients, clinical variables: disease duration, site of onset, the ALS Functional Rating Scale (ALSFRS), forced vital capacity (FVC), and gene mutation chromosome 9 open reading frame 72 (C9orf72) (independent variables). Multiple regression and mediation analyses were performed to predict cognitive and behavioral symptoms. Results: For the ALS group, the statistical model explained 38.8% of variance in ECAS total (p < 0.001), 59.4% of executive functions (p < 0.001), and 55% of behavioral symptoms (p < 0.001). For controls, it accounted for 52.8% of variance in ECAS total (p < 0.001). Interaction effects and mediation analysis showed CR is an ECAS total modulator, with a differential effect within groups (p < 0.001). Verbal fluency was the single best cognitive score to differentiate patients from controls (p = 0.004), and the gene mutation C9orf72 was found to be a behavioral symptom\' predictor in patients (p = 0.009). Conclusion: This study supports the proposed concept that CR acts as a cognitive modulator in ALS patients and healthy individuals. Moreover, CR also modulates behavioral manifestations in ALS.
摘要：
简介：肌萎缩侧索硬化症（ALS）具有从运动神经元变性到认知和行为障碍的异质性表现。本研究旨在阐明认知和行为症状与相关疾病预测因子和认知储备（CR）之间的相互作用。通过教育量化，身体活动,和职业代理人。方法：通过爱丁堡认知和行为ALS筛查（ECAS）（因变量）评估了162名ALS患者和61名对照的前瞻性样本，认知储备指数问卷(CRIq)和人口统计数据(年龄和性别)，and,对于患者来说，临床变量：疾病持续时间，发病部位,ALS功能评定量表(ALSFRS)，强迫肺活量(FVC)，和基因突变9号染色体开放阅读框72(C9orf72)(自变量)。进行多元回归和中介分析以预测认知和行为症状。结果：对于ALS组，统计模型解释了ECAS总方差的38.8%(p<0.001)，执行功能的59.4％（p<0.001），和55%的行为症状(p<0.001)。对于控件，它占ECAS总方差的52.8%(p<0.001)。相互作用效应和中介分析显示，CR是一种ECAS总调节剂，组内效果不同（p<0.001）。言语流畅性是区分患者与对照组的最佳认知评分（p=0.004），发现基因突变C9orf72是患者行为症状的预测因子(p=0.009)。结论：这项研究支持提出的概念，即CR在ALS患者和健康个体中充当认知调节剂。此外，CR还调节ALS中的行为表现。
