Abstract

Objectives

To examine the usefulness of the Edinburgh Cognitive and Behavioral Amyotrophic Lateral Sclerosis (ALS) Screen (ECAS) as a cognitive screening tool for the detection of behavioral variant frontotemporal dementia (bvFTD). A secondary aim was to determine whether people with FTD combined with ALS (ALS-FTD) exhibit a similar ECAS profile to that of people with bvFTD alone. Methods: Patients with ALS-FTD and bvFTD and healthy controls were recruited. Participants were administered the ECAS, which comprises tests of language, verbal fluency, executive functions, memory, and visual-spatial functions. They also carried out analogous, full-length cognitive tests that examine naming, spelling, sentence completion, and social cognition skills. Results: The study cohort comprised 20 ALS-FTD patients, 23 with bvFTD, and 30 controls. Highly significant group differences were elicited for all cognitive domains, reflecting poorer performance in patients compared to controls. No significant differences in overall test scores were found between ALS-FTD and bvFTD patients, although ALS-FTD patients showed a higher frequency of impairment on verbal fluency. Correlative analyses revealed inter-relationships in patients (but not controls) between scores in different domains, most marked in bvFTD. There were strong correlations between performance on ECAS subtests and analogous cognitive tasks. Conclusion: The ECAS is a sensitive and valuable tool for the assessment of FTD. Executive, language and behavioral breakdown may, however, compromise performance in other cognitive domains, reducing the specificity of the ‘frontotemporal’ cognitive profile. Subtle differences observed between ALS-FTD and bvFTD raise questions regarding the precise relationship between bvFTD with and without ALS.

中文翻译：

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额颞叶痴呆的爱丁堡认知和行为 ALS 筛查 (ECAS)。

摘要

目标

研究爱丁堡认知和行为肌萎缩侧索硬化 (ALS) 筛查 (ECAS) 作为检测行为变异额颞叶痴呆 (bvFTD) 的认知筛查工具的有效性。第二个目的是确定 FTD 合并 ALS (ALS-FTD) 的人是否表现出与单独 bvFTD 的人相似的 ECAS 特征。方法：招募了 ALS-FTD 和 bvFTD 患者和健康对照。参与者接受了 ECAS，其中包括语言、语言流畅性、执行功能、记忆和视觉空间功能的测试。他们还进行了类似的、完整的认知测试，检查命名、拼写、句子完成和社会认知技能。结果：研究队列包括 20 名 ALS-FTD 患者、23 名 bvFTD 患者和 30 名对照组。所有认知领域都引发了非常显着的组别差异，反映了与对照组相比患者的表现较差。ALS-FTD 和 bvFTD 患者的总体测试分数没有显着差异，尽管 ALS-FTD 患者的语言流畅性受损频率更高。相关分析揭示了患者（而非对照组）不同领域评分之间的相互关系，最明显的是 bvFTD。ECAS 子测试的表现与类似的认知任务之间存在很强的相关性。结论：ECAS 是评估 FTD 的敏感且有价值的工具。然而，执行、语言和行为崩溃可能会影响其他认知领域的表现，降低“额颞”认知特征的特异性。在 ALS-FTD 和 bvFTD 之间观察到的细微差异引发了关于 bvFTD 与 ALS 之间的确切关系的问题。