Poletti Barbara, Solca Federica, Carelli Laura, Faini Andrea, Madotto Fabiana, Lafronza Annalisa, Monti Alessia, Zago Stefano, Ciammola Andrea, Ratti Antonia, Ticozzi Nicola, Abrahams Sharon, Silani Vincenzo
a Department of Neurology and Laboratory of Neuroscience , IRCCS Istituto Auxologico Italiano , Milan , Italy.
b Department of Pathophysiology and Transplantation , "Dino Ferrari" Center, Università degli Studi di Milano , Milan , Italy.
Amyotroph Lateral Scler Frontotemporal Degener. 2018 Aug;19(5-6):387-395. doi: 10.1080/21678421.2018.1473443. Epub 2018 May 26.
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.
One hundred and sixty-eight ALS patients were tested at baseline (T). Among these, 48 patients performed the ECAS after 6 months (T), 18 patients performed it at T (12 months), and five patients were assessed after 24 months (T). Participants were also administered two cognitive test (FAB; MoCA) and psychological questionnaires (BDI; STAI/Y). The FBI was carried out with caregivers.
No cognitive deterioration was found across follow-ups. In contrast, although scores did not change between T and T, scores improved significantly for ECAS Total/ALS Non-specific and Memory domains when the ECAS was repeated on three occasions (T, T, T). 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.
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.
本研究展示了意大利版爱丁堡认知与行为性肌萎缩侧索硬化症筛查量表（ECAS）纵向应用的数据。我们调查了一组肌萎缩侧索硬化症（ALS）患者随时间推移的认知行为表现，以及与标准神经心理学测试相比纵向重复使用ECAS的可行性。最后，考虑了临床/基因数据与认知/行为数据之间的相关性。
168例ALS患者在基线（T0）时接受测试。其中，48例患者在6个月后（T1）进行了ECAS测试，18例患者在12个月时（T2）进行了测试，5例患者在24个月后（T3）接受了评估。参与者还接受了两项认知测试（FAB；MoCA）和心理问卷（BDI；STAI/Y）。FBI由护理人员进行。
随访期间未发现认知功能恶化。相比之下，虽然T0和T1之间分数没有变化，但当ECAS在三个时间点（T0、T1、T2）重复进行时，ECAS总分/ALS非特异性和记忆领域的分数显著提高。冷漠/惰性是最常见的行为症状，但随着时间推移未检测到行为分数恶化。12 - 24个月后，患者仍能完整地进行ECAS测试，而FAB和MoCA只能部分进行。
随着时间推移，一些ECAS分数显著提高，这支持了可能存在练习效应，特别是在记忆领域，突出了在纵向评估中通过健康对照组或替代版本来考虑这些效应的必要性。这项工作是意大利首次进行的ECAS随访研究，并证实了ECAS在身体残疾程度增加的患者中的可行性。
