Hopkins Thomas, Kwon Eunbi, Lapins Allison, Gill Nathan, Roberts Angela, Rogalski Emily
Department of Neurology Biologic Sciences Division, Healthy Aging and Alzheimer's Research Care Center University of Chicago Chicago Illinois USA.
Mesulam Center for Cognitive Neurology and Alzheimer's Disease Northwestern University Feinberg School of Medicine Chicago Illinois USA.
Alzheimers Dement (N Y). 2024 Nov 26;10(4):e12499. doi: 10.1002/trc2.12499. eCollection 2024 Oct-Dec.
Measurements of health-related quality of life (HRQoL) are important for capturing disease impact beyond physical health and relative to other diseases but have rarely been assessed in primary progressive aphasia (PPA).
HRQoL was characterized overall, by sex and subtype in PPA ( = 118) using the Health Utilities Index-2/3 (HUI2/3). Multiple linear regression assessed associations between HRQoL and language severity.
Multi-attribute HUI2/3 summary scores indicated moderate to severe impairment. Scores did not differ by sex and were more severe for semantic than non-semantic PPA. Language severity scores showed significant associations with HUI multi-attribute scores and select single-attribute measures (hearing, sensation, cognition, and speech) with less language impairment associated with better functional capacity related to HRQoL.
This study identified poor HRQoL in a relatively large PPA cohort. HRQoL measures aid in determining patient perspective, policy decision making, and resource allocation. Results may be used to advocate for PPA support.
Primary progressive aphasia (PPA) negatively impacts health-related quality of life.Health utilities index scores are associated with Western Aphasia Battery performance in PPA.Severity of language impairment in PPA is associated with poorer quality of life.
健康相关生活质量（HRQoL）的测量对于了解疾病对身体健康之外的影响以及与其他疾病相比的影响非常重要，但在原发性进行性失语症（PPA）中很少进行评估。
使用健康效用指数-2/3（HUI2/3）对118例PPA患者的总体、按性别和亚型的HRQoL进行了特征描述。多元线性回归评估了HRQoL与语言严重程度之间的关联。
多属性HUI2/3汇总评分表明存在中度至重度损害。评分在性别上没有差异，语义性PPA比非语义性PPA更严重。语言严重程度评分与HUI多属性评分以及选定的单属性测量（听力、感觉、认知和言语）显示出显著关联，语言损害越少，与HRQoL相关的功能能力越好。
本研究在一个相对较大的PPA队列中发现HRQoL较差。HRQoL测量有助于确定患者的观点、政策决策和资源分配。结果可用于倡导对PPA的支持。
原发性进行性失语症（PPA）对健康相关生活质量有负面影响。健康效用指数评分与PPA中的西方失语症成套测验表现相关。PPA中语言损害的严重程度与较差的生活质量相关。
