Objective: Patients with amyotrophic lateral sclerosis (ALS) not only show motor deficits, but may also have cognitive and/or behavioral impairments. Recognizing these impairments is crucial as they are associated with lower quality of life, shorter survival, and increased caregiver burden. Therefore, ALS-specific neuropsychological screening instruments have been developed that can account for motor and speech difficulties. This study provides an overview and comparison of these screeners. Methods: A systematic review was conducted using Medline and Embase. Articles describing cognitive/behavioral screening instruments assessed in ALS patients were included. Screening instruments were compared on multiple factors, such as domains, adaptability, required time, and validation. Results: We included 99 articles, reporting on nine cognitive screeners (i.e. ACE-R, ALS-BCA, ALS-CBS, ECAS, FAB, MMSE, MoCA, PSSFTS, and UCSF-SB), of which five ALS-specific. Furthermore, eight behavioral screeners (i.e. ALS-FTD-Q, AES, BBI, DAS, FBI, FrSBe, MiND-B, and NPI) were reported on, of which three ALS-specific. Conclusion: Considering the broad range of cognitive domains, adaptability, and satisfying validity, the ALS-CBS and ECAS appear to be the most suitable screeners to detect cognitive and behavioral changes in ALS. The BBI appears to be the best option to screen for behavioral changes in ALS, since all relevant domains are assessed, motor-related problems are considered, and has a satisfactory validity. The MiND-B and ALS-FTD-Q are promising as well. In general, all screening instruments would benefit from additional validation research to gain greater insights into test characteristics and to aid clinicians in selecting screening tools for use in clinical practice.

目的：肌萎缩侧索硬化 (ALS) 患者不仅表现出运动缺陷，还可能有认知和/或行为障碍。认识到这些损害至关重要，因为它们与较低的生活质量、较短的生存期和增加的护理人员负担有关。因此，已经开发了 ALS 特定的神经心理学筛查工具，可以解决运动和语言困难。本研究对这些筛选器进行了概述和比较。方法：使用 Medline 和 Embase 进行系统评价。包括描述在 ALS 患者中评估的认知/行为筛查工具的文章。筛选工具在多个因素上进行了比较，例如领域、适应性、所需时间和验证。结果：我们纳入了 99 篇文章，报告了 9 种认知筛查工具（即 ACE-R、ALS-BCA、ALS-CBS、ECAS、FAB、MMSE、MoCA、PSSFTS 和 UCSF-SB），其中 5 种是 ALS 特异性的。此外，还报告了八种行为筛查工具（即 ALS-FTD-Q、AES、BBI、DAS、FBI、FrSBe、MiND-B 和 NPI），其中三个是 ALS 特定的。结论：考虑到广泛的认知领域、适应性和令人满意的有效性，ALS-CBS 和 ECAS 似乎是检测 ALS 认知和行为变化的最合适的筛选器。BBI 似乎是筛查 ALS 行为变化的最佳选择，因为评估了所有相关领域，考虑了运动相关问题，并且具有令人满意的有效性。MiND-B 和 ALS-FTD-Q 也很有前景。一般而言，所有筛查工具都将受益于额外的验证研究，以更深入地了解测试特征并帮助临床医生选择用于临床实践的筛查工具。