ABSTRACT Background: Individuals with primary progressive aphasia (PPA) and their caregivers want to know what to expect so that they can plan support appropriately. The ability to predict decline in naming and semantic knowledge, and advise individuals with PPA and their caregivers regarding future planning, would be invaluable clinically. Aims: The aims of this study were to investigate patterns of decline in naming and semantic knowledge in each of the clinical variants of PPA (logopenic variant PPA, lvPPA; nonfluent agrammatic PPA, nfaPPA; and semantic variant PPA, svPPA) and to examine the effects of other variables on rate of decline. We hypothesized that speech-language rehabilitation, higher education, and higher baseline test scores would be associated with slower decline, and older age with faster decline. Methods and Procedures: A total of 94 participants with PPA underwent language testing, including 36 participants with lvPPA, 31 participants with nfaPPA, and 27 participants with svPPA. All participant groups were similar in age and education. We focused on decline on three tests: the short form of the Boston Naming Test (BNT), the Hopkins Assessment of Naming Actions (HANA), and the short form of the Pyramids and Palm Trees Test (PPTT). Outcome and Results: Across language tests, the most precipitous rates of decline (loss of points per month) occurred in nfaPPA, followed by svPPA, then lvPPA. Female sex, longer symptom duration, higher baseline test score, and speech-language rehabilitation were associated with slower decline. Conclusions: PPA variants were distinguishable by rapidity of decline, with nfaPPA having the most precipitous decline. As hypothesized, higher baseline test scores and speech-language rehabilitation were associated with slower decline. Surprisingly, age and education were not important prognostically for individuals in this study. Further study of prognostically-relevant variables in PPA is indicated in this population.

中文翻译：

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原发性进行性失语症中命名和语义知识下降的模式

摘要背景：患有原发性进行性失语症 (PPA) 的个人及其照顾者想知道会发生什么，以便他们能够适当地计划支持。预测命名和语义知识下降的能力，以及就未来计划向 PPA 患者及其护理人员提供建议的能力，在临床上将是无价的。目的：本研究的目的是调查 PPA 的每个临床变体（对数开放变体 PPA，lvPPA；非流利文法 PPA，nfaPPA；语义变体 PPA，svPPA）中命名和语义知识下降的模式，并检查其他变量对下降率的影响。我们假设语言康复、高等教育和较高的基线测试分数与下降速度较慢有关，而年龄较大的下降速度较快。方法和程序：共有 94 名 PPA 参与者接受了语言测试，其中 36 名 lvPPA 参与者、31 名 nfaPPA 参与者和 27 名 svPPA 参与者。所有参与者组的年龄和教育程度相似。我们专注于三个测试的下降：波士顿命名测试 (BNT) 的简短形式、命名行为的霍普金斯评估 (HANA) 以及金字塔和棕榈树测试 (PPTT) 的简短形式。结果和结果：在所有语言测试中，nfaPPA 的下降率（每月损失分数）最为急剧，其次是 svPPA，然后是 lvPPA。女性、较长的症状持续时间、较高的基线测试分数和语言康复与较慢的下降有关。结论：PPA 变体可通过下降速度来区分，其中 nfaPPA 下降幅度最大。正如假设的那样，较高的基线测试分数和语言康复与较慢的下降有关。令人惊讶的是，年龄和教育对本研究中的个体的预后并不重要。在该人群中需要进一步研究 PPA 中与预后相关的变量。