Supplementary Material for: Survival in Frontotemporal Dementia Phenotypes: A Meta-Analysis

<b><i>Background:</i></b> Survival in frontotemporal dementia (FTD) is not well understood. We conducted a mixed effects meta-analysis of survival in FTD to examine phenotype differences and contributory factors. <b><i>Methods:</i></b> The PubMed, Medline, EMBASE, CINAHL, PsycINFO and Cochrane databases were searched for studies describing survival or natural history of behavioral variant FTD (bvFTD), progressive non-fluent aphasia (PNFA), semantic dementia (SD), FTD with amyotrophic lateral sclerosis (FTD-ALS), progressive supranuclear palsy and corticobasal degeneration. There were no language restrictions. <b><i>Results:</i></b> We included 27 studies (2,462 subjects). Aggregate mean and median survival were derived for each phenotype and, for comparison, Alzheimer's disease (AD) (using data from the selected studies). Survival was shortest in FTD-ALS (2.5 years). Mean survival was longest in bvFTD and PNFA (8 years) and median survival in SD (12 years). AD was comparable in survival to all except FTD-ALS. Age and sex did not affect survival; the education effect was equivocal. Heterogeneity in FTD survival was largely, but not wholly, explained by phenotypes. <b><i>Conclusions:</i></b> Survival differs for FTD phenotypes but, except for FTD-ALS, compares well to AD survival. Elucidating the potential causes of within-phenotype heterogeneity in survival (such as complicating features and comorbidities) may open up opportunities for tailored interventions.背景：额颞叶痴呆（frontotemporal dementia, FTD）患者的生存预后目前尚未得到充分阐明。本研究针对额颞叶痴呆的生存情况开展混合效应meta分析，旨在探讨不同表型间的差异及其影响因素。 方法：我们检索了PubMed、Medline、EMBASE、CINAHL、PsycINFO及Cochrane数据库，筛选报道行为变异型额颞叶痴呆（behavioral variant FTD, bvFTD）、进行性非流利性失语（progressive non-fluent aphasia, PNFA）、语义性痴呆（semantic dementia, SD）、额颞叶痴呆合并肌萎缩侧索硬化症（FTD with amyotrophic lateral sclerosis, FTD-ALS）、进行性核上性麻痹以及皮质基底节变性患者生存情况或自然病程的相关研究，未对研究语言设置限制。 结果：本研究最终纳入27项研究，共计2462名受试者。我们针对每种表型计算了汇总平均生存期与中位生存期，并以阿尔茨海默病（Alzheimer's disease, AD）作为对照（采用纳入研究中的相关数据）。其中FTD-ALS患者的生存期最短，仅为2.5年；行为变异型额颞叶痴呆与进行性非流利性失语患者的平均生存期最长，达8年；语义性痴呆患者的中位生存期为12年。除FTD-ALS外，阿尔茨海默病患者的生存期与其余各表型均无显著差异。年龄与性别对患者生存期无显著影响，受教育程度的影响则尚不明确。额颞叶痴呆患者生存情况的异质性在很大程度上可由表型差异解释，但并非全部。 结论：不同表型的额颞叶痴呆患者生存期存在差异，但除FTD-ALS外，其余表型患者的生存期与阿尔茨海默病患者相当。阐明额颞叶痴呆患者生存情况在表型内存在异质性的潜在诱因（如并发症与合并症），可为定制化干预手段的开发提供思路。