Cognitive-behavioral longitudinal assessment in ALS: the Italian Edinburgh Cognitive and Behavioral ALS screen (ECAS)

<i>Objective</i>: The study presents data on the longitudinal administration of the Italian Edinburgh Cognitive and Behavioral ALS Screen (ECAS). We investigated cognitive-behavioral performance in a group of ALS patients over time and the feasibility of repeating the ECAS longitudinally compared with standard neuropsychological tests. Finally, correlations between clinical/genetic and cognitive/behavioral data were considered. <i>Methods</i>: One hundred and sixty-eight ALS patients were tested at baseline (T₀). Among these, 48 patients performed the ECAS after 6 months (T₁), 18 patients performed it at T₂ (12 months), and five patients were assessed after 24 months (T₃). Participants were also administered two cognitive test (FAB; MoCA) and psychological questionnaires (BDI; STAI/Y). The FBI was carried out with caregivers. <i>Results</i>: No cognitive deterioration was found across follow-ups. In contrast, although scores did not change between T₀ and T₁, scores improved significantly for ECAS Total/ALS Non-specific and Memory domains when the ECAS was repeated on three occasions (T₀, T₁, T₂). Apathy/Inertia was the most common behavioral symptom, but no worsening of behavioral scores was detected over time. After 12–24 months, patients were still able to perform the ECAS in total, in contrast to FAB and MoCA, which were only partially administrable. <i>Conclusions</i>: The significant improvement of some ECAS scores over time supports the presence of possible practice effects, particularly in the memory domain, highlighting the need to accommodate for these in longitudinal assessments, through healthy controls groups or alternate versions. This work represents the first Italian ECAS follow-up study and confirms ECAS feasibility in patients with increasing physical disability.

【研究目的】本研究提供了意大利版爱丁堡认知与行为ALS筛查量表（Edinburgh Cognitive and Behavioral ALS Screen, ECAS）的纵向施测数据。本研究旨在追踪一组肌萎缩侧索硬化症（Amyotrophic Lateral Sclerosis, ALS）患者的认知行为表现随时间的变化情况，并对比标准神经心理学测试，探讨纵向重复施测ECAS的可行性。此外，本研究还分析了临床/遗传数据与认知/行为数据之间的相关性。
【研究方法】本研究共纳入168例ALS患者，于基线（T₀）时完成首次测评。其中48例患者于6个月后（T₁）再次完成ECAS测评，18例患者于12个月（T₂）时完成测评，另有5例患者在24个月（T₃）时接受了评估。所有受试者同时完成了两项认知测试：额叶评估量表（Frontal Assessment Battery, FAB）与蒙特利尔认知评估量表（Montreal Cognitive Assessment, MoCA），以及两份心理问卷：贝克抑郁量表（Beck Depression Inventory, BDI）与状态-特质焦虑问卷（State-Trait Anxiety Inventory, STAI-Y）。FBI测评由照料者协助完成。
【研究结果】随访期间未发现患者出现认知功能减退。与之相对，尽管T₀与T₁两次测评间ECAS得分无显著变化，但当在三次时间点（T₀、T₁、T₂）重复施测ECAS时，其总分、ALS非特异性维度以及记忆维度的得分均出现显著提升。淡漠/行为迟缓是最常见的行为症状，但随访期间行为量表得分未出现恶化。在12~24个月随访阶段，患者仍可独立完成完整的ECAS测评，而FAB与MoCA仅能部分完成施测。
【研究结论】部分ECAS得分随时间出现的显著提升提示存在练习效应，尤其在记忆维度更为明显，这表明纵向评估中需通过设置健康对照组或采用替代版本量表来抵消练习效应的影响。本研究是首项针对意大利版ECAS的随访研究，证实了ECAS在躯体残疾程度逐渐加重的ALS患者中仍具备良好的施测可行性。