Abstract

Objective

Amyotrophic lateral sclerosis (ALS) patients might present with cognitive and behavioural abnormalities resembling frontotemporal dementia (FTD). The Edinburgh Cognitive and Behavioural ALS Screen (ECAS) was developed as an easy to administer cognitive screen for detecting these symptoms. The aim of the present study was to develop and validate a Japanese version of the ECAS.

Methods

In this single centre observational study, 35 ALS patients and 28 healthy controls were enrolled. Three patients in the ALS group fulfilled the criteria for behavioural variant FTD (ALS-FTD) and the rest were grouped as ALS without FTD. Participants were subjected to the Japanese version of the ECAS. ALS patients were also subjected to the Montreal Cognitive Assessment, Frontal Assessment Battery, ALS Functional Rating Scale-Revised, and respiratory function testing. Demographic and disease characteristics (e.g., sex, age at examination, and years of education) were also recorded.

Results

Internal consistency and correlations with general cognitive screenings were sufficient in the Japanese adaptation. Executive functions were the most commonly affected ECAS domain, followed by fluency and language. Compared to control subjects, ALS patients without FTD had low scores in the ECAS ALS-specific functions but not in ALS-nonspecific functions. Meanwhile ALS-FTD patients markedly underperformed both in the ECAS ALS-specific and ALS-nonspecific functions. Furthermore, the Japanese ECAS score correlated positively with years of education and negatively with age at onset.

Conclusion

The Japanese version of the ECAS is a valid and useful screening tool to identify multiple types of cognitive impairment in ALS patients.

中文翻译：

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针对日本 ALS 和 FTD 患者的爱丁堡认知和行为 ALS 筛查 (ECAS)

摘要

客观的

肌萎缩侧索硬化 (ALS) 患者可能会出现类似于额颞叶痴呆 (FTD) 的认知和行为异常。爱丁堡认知和行为 ALS 筛查 (ECAS) 被开发为一种易于管理的认知筛查，用于检测这些症状。本研究的目的是开发和验证日本版的 ECAS。

方法

在这项单中心观察性研究中，招募了 35 名 ALS 患者和 28 名健康对照者。ALS 组中的 3 名患者符合行为变异 FTD (ALS-FTD) 的标准，其余患者被分组为没有 FTD 的 ALS。参与者接受了日本版的 ECAS。ALS 患者还接受了蒙特利尔认知评估、额叶评估电池、ALS 功能评定量表修订版和呼吸功能测试。还记录了人口统计学和疾病特征（例如，性别、考试年龄和受教育年限）。

结果

在日语适应中，内部一致性和与一般认知筛查的相关性是足够的。执行功能是最常受影响的 ECAS 领域，其次是流畅性和语言。与对照受试者相比，没有 FTD 的 ALS 患者在 ECAS ALS 特异性功能方面得分较低，但在 ALS 非特异性功能方面得分较低。同时，ALS-FTD 患者在 ECAS ALS 特异性和 ALS 非特异性功能方面的表现明显不佳。此外，日本的 ECAS 分数与受教育年限呈正相关，与发病年龄呈负相关。

结论

日语版 ECAS 是一种有效且有用的筛查工具，可用于识别 ALS 患者的多种类型的认知障碍。