Ph.D. Program in Neuroscience, School of Medicine and Surgery, University of Milano-Bicocca, Via Cadore 48, 20900, Monza, Italy.
Istituti Clinici Scientifici Maugeri, IRCCS Milano, Milan, Italy.
Neurol Sci. 2022 Sep;43(9):5397-5402. doi: 10.1007/s10072-022-06157-x. Epub 2022 May 24.
Amyotrophic lateral sclerosis (ALS) is phenotypically heterogeneous in motor manifestations, and the extent of upper vs. lower motor neuron involvement is a widespread descriptor. This study aimed to examine cognition across different ALS motor phenotypes.
ALS patients (N = 124) were classified as classical (N = 66), bulbar (N = 13), predominant-upper motor neuron (PUMN; N = 19), and predominant-lower motor neuron (PLMN; N = 26) phenotypes. Cognition was assessed with the Edinburgh Cognitive and Behavioural ALS Screen (ECAS) and function with the ALS Functional Rating Scale-Revised (ALSFRS-R). Revised ALS-FTD consensus criteria were applied for cognitive/behavioral phenotyping.
Defective ECAS-total scores were detected in all groups - bulbar: 15.4%, classical: 30.3%, PLMN: 23.1%, and PUMN: 36.8%. Classical and PUMN ALS patients performed worse than PLMN ones on ECAS-total, ALS-specific, Fluency, and Executive measures. No other difference was detected. Worse ASLFRS-R scores correlated with poorer ECAS-total scores in classical ALS patients.
Frontotemporal cognitive deficits are more prevalent in PUMN and classical ALS and linked to disease severity in the latter, but occur also in PLMN phenotypes.
肌萎缩侧索硬化症（ALS）在运动表现方面表现出明显的异质性，而上、下运动神经元受累的程度是一个广泛的描述。本研究旨在研究不同 ALS 运动表型的认知情况。
将 ALS 患者（N=124）分为经典型（N=66）、延髓型（N=13）、以皮质下运动神经元为主型（PUMN；N=19）和以皮质下运动神经元为主型（PLMN；N=26）。使用爱丁堡认知和行为 ALS 筛查（ECAS）评估认知，使用修订版 ALS 功能评定量表（ALSFRS-R）评估功能。应用修订版 ALS-FTD 共识标准进行认知/行为表型分析。
所有组均存在 ECAS 总分异常-延髓：15.4%，经典型：30.3%，PLMN：23.1%，PUMN：36.8%。经典型和 PUMN ALS 患者在 ECAS 总分、ALS 特异性、流畅性和执行能力方面的表现均较 PLMN 患者差。未检测到其他差异。ALSFRS-R 评分越低，ECAS 总分越低，经典型 ALS 患者的相关性越显著。
额颞叶认知缺陷在 PUMN 和经典型 ALS 中更为常见，与后者的疾病严重程度相关，但也发生在 PLMN 表型中。
