Poletti Barbara, Solca Federica, Maffi Sabrina, Torre Silvia, Carelli Laura, Aiello Edoardo Nicolò, Ferrucci Roberta, Priori Alberto, Monti Alessia, Verde Federico, Ticozzi Nicola, Migliore Simone, Scaricamazza Eugenia, Casella Melissa, Squitieri Ferdinando, Ciammola Andrea, Silani Vincenzo
Department of Neurology and Laboratory of Neuroscience, IRCCS Istituto Auxologico Italiano, Milan, Italy.
Huntington and Rare Diseases Unit, CSS-Mendel Institute, Fondazione IRCCS Casa Sollievo della Sofferenza Research Hospital, San Giovanni Rotondo, Italy.
Front Aging Neurosci. 2022 Nov 2;14:1031908. doi: 10.3389/fnagi.2022.1031908. eCollection 2022.
The semiology and determinants of apathy are largely unknown across amyotrophic lateral sclerosis (ALS), Parkinson's disease (PD), and Huntington's disease (HD), due to both motor and non-motor confounders. This study thus aimed at (1) profiling apathy in ALS, PD, and HD and (2) exploring its clinical determinants.
Consecutive ALS ( = 99), PD ( = 73), and HD ( = 25) patients underwent a motor-free assessment of apathy (Dimensional Apathy Scale, DAS), global cognition, anxiety and depression. Function was assessed through disease-specific scales. The DAS was also completed by = 101 healthy controls (HCs). Between-group comparisons on DAS scores were implemented by covarying for all applicable confounders. Predictive models on DAS scores were built through multiple, stepwise regressions.
Parkinson's disease and HD, but not ALS, patients were more apathetic than HCs-with HD patients also selectively showing lower initiation and poorer goal-directed planning than HCs. Higher apathetic features were detected in PD and HD as compared to ALS. Education was a protective factor against apathy in ALS. Anxiety was a risk factor for global apathy in ALS, HD, and to a lesser extent, in PD, whereas, protective only toward affective disintegration in PD and ALS. Cognitive inefficiency was a risk factor toward apathy in both PD and ALS. Depression was a risk factor for executive-related apathy in PD.
This study provides unprecedented insights into the heterogeneous semiology and determinants of apathy across ALS, PD, and HD the DAS, in turn informing clinical practice and research.
由于运动和非运动方面的混杂因素,肌萎缩侧索硬化症(ALS)、帕金森病(PD)和亨廷顿舞蹈病(HD)中冷漠的症状学及决定因素在很大程度上尚不明确。因此,本研究旨在(1)描绘ALS、PD和HD中的冷漠情况,以及(2)探究其临床决定因素。
连续纳入99例ALS患者、73例PD患者和25例HD患者,对其进行无运动干扰的冷漠评估(维度冷漠量表,DAS)、整体认知、焦虑和抑郁评估。通过疾病特异性量表评估功能。101名健康对照者(HCs)也完成了DAS评估。通过对所有适用的混杂因素进行协变量调整,对DAS评分进行组间比较。通过多元逐步回归建立DAS评分的预测模型。
与HCs相比,PD患者和HD患者更冷漠,而ALS患者无此现象——HD患者还选择性地表现出比HCs更低的启动能力和更差的目标导向计划能力。与ALS相比,PD和HD中检测到更高的冷漠特征。在ALS中,受教育程度是预防冷漠的保护因素。焦虑是ALS、HD以及在较小程度上是PD中整体冷漠的危险因素,而仅对PD和ALS中的情感解体有保护作用。认知效率低下是PD和ALS中冷漠的危险因素。抑郁是PD中与执行功能相关冷漠的危险因素。
本研究为ALS、PD和HD中冷漠的异质症状学及决定因素提供了前所未有的见解——DAS量表反过来也为临床实践和研究提供了参考。