Faculty of Psychology, University of Warsaw, Warsaw, Poland.
Institute of Psychiatry and Neurology, Sobieskiego 9, 02-957, Warsaw, Poland.
Neurol Sci. 2022 Feb;43(2):1385-1394. doi: 10.1007/s10072-021-05366-0. Epub 2021 Jun 14.
Apathy is one of the neuropsychiatric symptoms of Wilson's disease (WD) which typically affects the brain's fronto-basal circuits. Lack of agreed diagnostic criteria and common use of self-description assessment tools lead to underestimation of this clinical phenomenon. The aim of this study was to investigate whether subjective and informant-based clinical features of apathy in patients with WD enable clinicians to make a valid diagnosis.
Multiple aspects of goal-oriented behavior were assessed in 30 patients with the neurological form of WD and 30 age-matched healthy participants using two questionnaires, the Lille Apathy Rating Scale (LARS) and the Dysexecutive Questionnaire (DEX). Both included a self-descriptive and a caregiver/proxy version. Cognitive functioning was estimated with the use of Addenbrooke's Cognitive Examination-Revised.
Patients obtained significantly worse scores on all clinical scales when more objective measures were considered. Features of apathy and executive dysfunction were revealed in patients' caregiver versions of LARS and DEX, which may indicate poor self-awareness of patients with WD. Roughly 30% of participants were likely to present with clinically meaningful symptoms, independent of cognitive dysfunction.
Methods relying on self-description appear inferior to informant-based scales when diagnosing apathy. More objective criteria and measurement tools are needed to better understand this clinical syndrome.
淡漠是肝豆状核变性(WD)的神经精神症状之一,主要影响大脑额基底节回路。由于缺乏公认的诊断标准和常用的自我描述评估工具,导致对这一临床现象的低估。本研究旨在探讨 WD 患者的淡漠的主观和知情者为基础的临床特征是否能使临床医生做出有效的诊断。
使用两个问卷,即 Lille 淡漠评定量表(LARS)和执行功能障碍问卷(DEX),评估 30 例神经型 WD 患者和 30 名年龄匹配的健康参与者的目标导向行为的多个方面。两者都包括自我描述和照顾者/代理人版本。使用 Addenbrooke 的认知测验修订版(Addenbrooke's Cognitive Examination-Revised)评估认知功能。
当考虑到更客观的测量方法时,患者在所有临床量表上的得分明显更差。在 LARS 和 DEX 的患者照顾者版本中发现了淡漠和执行功能障碍的特征,这可能表明 WD 患者自我意识较差。大约 30%的参与者可能存在有临床意义的症状,而与认知功能障碍无关。
依赖自我描述的方法在诊断淡漠时不如知情者为基础的量表。需要更客观的标准和测量工具来更好地理解这一临床综合征。