Foxe David, Irish Muireann, Cheung Sau Chi, D'Mello Mirelle, Hwang Yun Tae, Muggleton James, Cordato Nicholas J, Piguet Olivier
The University of Sydney School of Psychology Sydney Australia.
The University of Sydney Brain and Mind Centre Sydney Australia.
Alzheimers Dement (Amst). 2024 Nov 15;16(4):e70028. doi: 10.1002/dad2.70028. eCollection 2024 Oct-Dec.
This study investigated the changes in functional capacity with disease progression in a well-characterised cohort of patients diagnosed with frontotemporal dementia (FTD) and Alzheimer's disease (AD) presentations.
We recruited 126 behavioural variant FTD (bvFTD), 40 progressive nonfluent aphasia (PNFA), 64 semantic dementia (SD), 45 logopenic progressive aphasia (LPA), and 115 AD patients. Functional capacity was measured annually over ∼7 years using the Disability Assessment for Dementia.
Linear mixed effects models revealed the bvFTD group demonstrated disproportionate functional impairment at baseline and over the study period. Functional capacity among the other syndromes showed a more uniform pattern of decline, with less severe functional impairment at baseline and ∼7%-10% mean annual decline. Baseline correlations indicated different mechanisms supporting basic and complex functional proficiency among the groups.
Our findings demonstrate distinct functional profiles across dementia syndromes with disease progression. Identifying progression milestones across syndromes will improve clinical management.
bvFTD shows severe functional impairment at baseline and over time.PNFA, SD, LPA, AD: less severe baseline functional impairment; more uniform decline.General cognition is related to IADLs, but not BADLs, in all groups.Behavioural disturbances relate to IADLs and BADLs in bvFTD and SD.Behavioural-ADL relations are more mixed in PNFA, LPA, and AD.
本研究调查了一组特征明确的被诊断为额颞叶痴呆(FTD)和阿尔茨海默病(AD)表现的患者,其功能能力随疾病进展的变化情况。
我们招募了126例行为变异型FTD(bvFTD)患者、40例进行性非流畅性失语(PNFA)患者、64例语义性痴呆(SD)患者、45例语音性进行性失语(LPA)患者以及115例AD患者。使用痴呆症残疾评估量表在约7年的时间里每年测量一次功能能力。
线性混合效应模型显示,bvFTD组在基线时和研究期间表现出不成比例的功能损害。其他综合征组的功能能力下降模式更为一致,基线时功能损害较轻,平均每年下降约7%-10%。基线相关性表明,各组中支持基本和复杂功能熟练程度的机制不同。
我们的研究结果表明,随着疾病进展,不同痴呆综合征具有不同的功能特征。确定各综合征的进展里程碑将改善临床管理。
bvFTD在基线时和随时间推移均表现出严重的功能损害。PNFA、SD、LPA、AD:基线功能损害较轻;下降更为一致。在所有组中,一般认知与工具性日常生活活动能力(IADLs)相关,但与基本日常生活活动能力(BADLs)无关。行为障碍与bvFTD和SD中的IADLs和BADLs相关。在PNFA、LPA和AD中,行为与BADLs的关系更为复杂。