Abstract

Objective: Detecting and monitoring cognitive and behavioral deficits in motor neuron diseases (MND) is critical due to their considerable clinical impact. In this scenario, computer-based batteries may play an important role. In this study, we investigated the progression of cognitive and behavioral deficits in MND patients using both standard and computer-based neuropsychological batteries. Methods: This is a retrospective study on 74 MND patients (52 amyotrophic lateral sclerosis [ALS], 12 primary lateral sclerosis [PLS], and 10 progressive muscular atrophy [PMA]) who were followed up for 12 months and underwent up to three cognitive/behavioral assessments, 6 months apart, including standard and/or computerized based (the Test of Attentional Performance [TAP]) batteries. Behavioral/cognitive changes were investigated over time using generalized linear model for longitudinal data accounting for time and revised-ALS Functional Rating Scale. Results: Over 12 months, ALS patients showed a global cognitive decline (Mini Mental State Examination) at the standard battery and reduced performance in the alertness, sustained and divided attention, go/nogo, cross-modal and incompatibility TAP tasks. Most of these findings remained significant when ALSFRS-R changes over time were included as covariate in the analyses. ALS patients did not show significant behavioral abnormalities over time. No cognitive and behavioral changes were found in PLS and PMA cases. Conclusions: Computer-based neuropsychological evaluations are able to identify subtle cognitive changes in ALS, unique to this condition. This study highlights the need of specific, accurate and well-tolerated tools for the monitoring of cognitive deficits in MND.

摘要

目的：检测和监测运动神经元疾病 (MND) 的认知和行为缺陷至关重要，因为它们具有相当大的临床影响。在这种情况下，基于计算机的电池可能会发挥重要作用。在这项研究中，我们使用标准和基于计算机的神经心理学电池调查了 MND 患者认知和行为缺陷的进展。方法：这是一项对 74 名 MND 患者（52 名肌萎缩侧索硬化 [ALS]、12 名原发性侧索硬化 [PLS] 和 10 名进行性肌萎缩 [PMA]）进行的回顾性研究，这些患者接受了 12 个月的随访，并接受了多达 3 次认知/行为评估，间隔 6 个月，包括标准和/或基于计算机的（注意力表现测试 [TAP]）电池。行为/认知变化随着时间的推移使用广义线性模型进行调查，纵向数据占时间和修订的 ALS 功能评定量表。结果：超过 12 个月，ALS 患者在标准电池组中表现出整体认知能力下降（迷你精神状态检查），并且在警觉性、持续和分散注意力、去/不去、跨模式和不兼容 TAP 任务方面的表现降低。当 ALSFRS-R 随时间的变化作为协变量包含在分析中时，这些发现中的大多数仍然显着。随着时间的推移，ALS 患者没有表现出明显的行为异常。在 PLS 和 PMA 病例中未发现认知和行为改变。结论：基于计算机的神经心理学评估能够识别 ALS 的细微认知变化，这是这种情况所独有的。本研究强调需要特定、准确和耐受性良好的工具来监测 MND 的认知缺陷。