Zhang Xin, Irish Muireann, Piguet Olivier, Ahmed Rebekah M
Department of Clinical Neurosciences, Memory and Cognition Clinic, Royal Prince Alfred Hospital, Sydney, NSW, Australia.
UCL Queen Square Institute of Neurology, University College London, London, United Kingdom.
J Neurol. 2025 Mar 21;272(4):279. doi: 10.1007/s00415-025-13025-z.
Longitudinal comparative characterisation of dementia syndromes may aid differential diagnosis, prognostication and intervention implementation.
We compared the behavioural and cognitive characteristics of 84 behavioural variant frontotemporal dementia (bvFTD), 29 left and 14 right-dominant semantic dementia (SDL and SDR) and 49 Alzheimer's disease (AD) patients over a follow-up period of 2.4 ± 1.6 years using the Cambridge Behavioural Inventory Revised (CBI-R) and Addenbrooke's Cognitive Examination third edition (ACE-III).
Linear mixed modelling of time effects found progression of all CBI-R domains, aside from sleep, beliefs and mood domains, and all ACE-III domains. Modelling of group effects found that bvFTD had greater symptoms than AD in most CBI-R domains. Notably, SDL and SDR compared differently with AD and bvFTD; whilst SDR did not differ significantly from bvFTD in any CBI-R domain, SDL had less severe symptoms than bvFTD in everyday skills, motivation, sleep and eating habits; whilst SDL had greater disturbances in abnormal behaviour and stereotypic behaviour than AD, SDR had greater disturbances in addition in motivation and eating habits. Motivation, eating habits, abnormal behaviour and stereotypic behaviour were the most frequently different behavioural domains between groups.
We have shown that the combined, longitudinal use of existing behavioural and cognitive assessments could capture distinct clinical profiles of common and rare dementia syndromes. Our findings also highlight the importance of select behavioural domains such as motivation and the usefulness of separate clinical characterisations of SDL and SDR.
对痴呆综合征进行纵向比较特征分析有助于鉴别诊断、预后评估及干预措施的实施。
我们使用修订后的剑桥行为量表(CBI-R)和第三版Addenbrooke认知检查量表(ACE-III),对84例行为变异型额颞叶痴呆(bvFTD)、29例左侧优势和14例右侧优势语义性痴呆(SDL和SDR)以及49例阿尔茨海默病(AD)患者在2.4±1.6年的随访期内的行为和认知特征进行了比较。
对时间效应的线性混合模型分析发现,除睡眠、信念和情绪领域外,所有CBI-R领域以及所有ACE-III领域均有进展。对组间效应的模型分析发现,在大多数CBI-R领域中,bvFTD的症状比AD更严重。值得注意的是,SDL和SDR与AD和bvFTD的比较结果不同;虽然SDR在任何CBI-R领域与bvFTD均无显著差异,但SDL在日常技能、动机、睡眠和饮食习惯方面的症状比bvFTD轻;虽然SDL在异常行为和刻板行为方面的紊乱比AD更严重,但SDR在动机和饮食习惯方面也有更严重的紊乱。动机、饮食习惯、异常行为和刻板行为是组间最常出现差异的行为领域。
我们已经表明,联合使用现有的行为和认知评估方法进行纵向分析,可以捕捉常见和罕见痴呆综合征的不同临床特征。我们的研究结果还强调了如动机等特定行为领域的重要性,以及对SDL和SDR进行单独临床特征分析的实用性。