Cognitive and behavioral impairments in German and Chinese ALS populations – a post-hoc comparison of national study data

This study aimed to clarify whether differences between German and Chinese studies using the standardized Edinburgh Cognitive and Behavioural ALS Screen (ECAS), might be explained by differences in translated versions of ECAS, by patient demographic or clinical characteristics or by population-specific factors. Comparisons were performed on data from two previous studies in Germany and China. We found except for spelling task (p = 0.05), no differences between control groups of two countries were detected after adjusting for demographics. In contrast, differences were observed in scores on total ECAS, ALS-specific function such as different executive functions (all p < 0.01) and language (p = 0.02), even after correcting for demographic and clinical variables. Chinese ALS cohort performed worse in executive subfunction scores for sentence completion, alternation, social cognition, digit span and language comprehension; they performed better in spelling. Chinese ALS cohort more frequently exhibited disinhibition (p = 0.02), whereas German cohort more frequently exhibited loss of sympathy (p = 0.01) and stereotyped behavior (p = 0.03). Chinese and German ALS cohorts showed a distinctly different pattern in executive and language function. Most of the differences might be related to distinct differences between populations as only spelling might be affected by different language versions of ECAS. Socio-cultural factors might explain behavioral profile differences.

本研究旨在阐明，使用标准化爱丁堡认知与行为肌萎缩侧索硬化症（Amyotrophic Lateral Sclerosis, ALS）筛查量表（Edinburgh Cognitive and Behavioural ALS Screen, ECAS）开展的德国与中国相关研究之间的差异，是否可由ECAS翻译版本差异、患者人口统计学或临床特征差异，抑或人群特异性因素加以解释。本研究针对德国与中国两项既往研究的数据集开展对比分析。结果显示，在对人口统计学因素进行校正后，两国对照组未检出显著差异，仅拼写任务（p = 0.05）除外。与之形成对比的是，即便在校正人口统计学与临床变量后，两国患者在ECAS总分、ALS特异性功能（如各类执行功能（所有p < 0.01）及语言功能（p = 0.02））得分上仍存在显著差异。中国ALS队列在句子完成、交替任务、社会认知、数字广度及语言理解等执行亚功能得分上表现更差，但在拼写任务中表现更佳。中国ALS队列更常出现行为脱抑制（p = 0.02），而德国队列则更常出现同情心丧失（p = 0.01）与刻板行为（p = 0.03）。中德两国ALS队列在执行功能与语言功能方面呈现出显著不同的模式。大部分差异可能与人群间的固有差异相关，仅拼写任务可能受ECAS不同语言版本的影响。社会文化因素或可解释行为特征方面的差异。