Faculty of Medicine, Institute of Molecular Medicine, University of Lisbon, Lisbon, Portugal.
Faculty of Medicine, Centre of Studies Egas Moniz, University of Lisbon, Lisbon, Portugal, and.
Amyotroph Lateral Scler Frontotemporal Degener. 2024 Nov;25(7-8):726-736. doi: 10.1080/21678421.2024.2385684. Epub 2024 Aug 5.
: 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. : 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 () (independent variables). Multiple regression and mediation analyses were performed to predict cognitive and behavioral symptoms. : For the ALS group, the statistical model explained 38.8% of variance in ECAS total ( < 0.001), 59.4% of executive functions ( < 0.001), and 55% of behavioral symptoms ( < 0.001). For controls, it accounted for 52.8% of variance in ECAS total ( < 0.001). Interaction effects and mediation analysis showed CR is an ECAS total modulator, with a differential effect within groups ( < 0.001). Verbal fluency was the single best cognitive score to differentiate patients from controls ( = 0.004), and the gene mutation was found to be a behavioral symptom' predictor in patients ( = 0.009). : 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.
肌萎缩侧索硬化症（ALS）表现多样，从运动神经元变性到认知和行为损伤均有涉及。本研究旨在阐明认知和行为症状与相关疾病预测因子以及认知储备（CR）之间的相互作用，CR 通过教育、身体活动和职业等指标来量化。
本前瞻性研究纳入了 162 名 ALS 患者和 61 名对照组，采用爱丁堡认知和行为 ALS 筛查（ECAS）（因变量）、认知储备指数问卷（CRIq）和人口统计学数据（年龄和性别），以及患者的临床变量（疾病持续时间、发病部位、肌萎缩侧索硬化功能评定量表（ALSFRS）、用力肺活量（FVC）和 9 号染色体开放阅读框 72 基因（））进行评估。采用多元回归和中介分析来预测认知和行为症状。
对于 ALS 组，统计学模型解释了 ECAS 总分的 38.8%（<0.001）、执行功能的 59.4%（<0.001）和行为症状的 55%（<0.001）的变异性。对于对照组，该模型解释了 ECAS 总分的 52.8%（<0.001）的变异性。交互作用效应和中介分析表明，CR 是 ECAS 总分的调节剂，在组内具有不同的效应（<0.001）。言语流畅性是区分患者和对照组的最佳认知评分（=0.004），而基因突变被发现是患者行为症状的预测因子（=0.009）。
本研究支持了这样一个概念，即 CR 是 ALS 患者和健康个体认知的调节剂。此外，CR 还调节 ALS 患者的行为表现。
