PMID- 34125323 OWN - NLM STAT- MEDLINE DCOM- 20220127 LR - 20220422 IS - 1590-3478 (Electronic) IS - 1590-1874 (Print) IS - 1590-1874 (Linking) VI - 43 IP - 2 DP - 2022 Feb TI - Clinical significance of self-descriptive apathy assessment in patients with neurological form of Wilson's disease. PG - 1385-1394 LID - 10.1007/s10072-021-05366-0 [doi] AB - BACKGROUND AND AIM: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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. CI - (c) 2021. The Author(s). FAU - Lesniak, Marcin AU - Lesniak M AD - Faculty of Psychology, University of Warsaw, Warsaw, Poland. FAU - Roessler-Gorecka, Magdalena AU - Roessler-Gorecka M AD - Institute of Psychiatry and Neurology, Sobieskiego 9, 02-957, Warsaw, Poland. FAU - Czlonkowska, Anna AU - Czlonkowska A AD - Institute of Psychiatry and Neurology, Sobieskiego 9, 02-957, Warsaw, Poland. FAU - Seniow, Joanna AU - Seniow J AUID- ORCID: 0000-0002-1017-4819 AD - Institute of Psychiatry and Neurology, Sobieskiego 9, 02-957, Warsaw, Poland. seniowj@ipin.edu.pl. LA - eng GR - 501-15-021-20016/Institute of Psychiatry and Neurology/ GR - 501-D125-01-1250000 zlec*. 5011000206/Ministerstwo Nauki i Szkolnictwa Wyzszego/ PT - Journal Article DEP - 20210614 PL - Italy TA - Neurol Sci JT - Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology JID - 100959175 SB - IM MH - *Apathy MH - Caregivers MH - Cognition MH - *Cognitive Dysfunction/diagnosis/etiology MH - *Hepatolenticular Degeneration/diagnosis MH - Humans PMC - PMC8789726 OTO - NOTNLM OT - Apathy syndrome OT - Cognitive impairment OT - Psychopathology OT - Self-description OT - Wilson's disease COIS- The authors declare no competing interests. EDAT- 2021/06/15 06:00 MHDA- 2022/01/28 06:00 PMCR- 2021/06/14 CRDT- 2021/06/14 12:38 PHST- 2021/02/15 00:00 [received] PHST- 2021/05/29 00:00 [accepted] PHST- 2021/06/15 06:00 [pubmed] PHST- 2022/01/28 06:00 [medline] PHST- 2021/06/14 12:38 [entrez] PHST- 2021/06/14 00:00 [pmc-release] AID - 10.1007/s10072-021-05366-0 [pii] AID - 5366 [pii] AID - 10.1007/s10072-021-05366-0 [doi] PST - ppublish SO - Neurol Sci. 2022 Feb;43(2):1385-1394. doi: 10.1007/s10072-021-05366-0. Epub 2021 Jun 14.