Primary progressive aphasia (PPA) and primary progressive apraxia of speech (PPAOS) are neurodegenerative syndromes characterized by progressive decline in language or speech. There is a growing number of studies investigating speech-language interventions for PPA/PPAOS. An updated systematic evaluation of the treatment evidence is warranted to inform best clinical practice and guide future treatment research. We systematically reviewed the evidence for behavioral treatment for speech and language in this population. Reviewed articles were published in peer-reviewed journals through 31 May 2021. We evaluated level of evidence, reporting quality, and risk of bias using a modified version of the American Speech-Language Hearing Association (ASHA) Levels of Evidence, an appraisal point system, additional reporting quality and internal/external validity items, and, as appropriate, the Single Case Experimental Design Scale or the Physiotherapy Evidence Database – PsycBITE Rating Scale for Randomized and Non-Randomized Controlled Trials. Results were synthesized using quantitative summaries and narrative review. A total of 103 studies reported treatment outcomes for 626 individuals with PPA; no studies used the diagnostic label PPAOS. Most studies evaluated interventions for word retrieval. The highest-quality evidence was provided by 45 experimental and quasi-experimental studies (16 controlled group studies, 29 single-subject designs). All (k = 45/45) reported improvement on a primary outcome measure; most reported generalization (k = 34/43), maintenance (k = 34/39), or social validity (k = 17/19) of treatment for at least one participant. The available evidence supports speech-language intervention for persons with PPA; however, treatment for PPAOS awaits systematic investigation. Implications and limitations of the evidence and the review are discussed.

原发性进行性失语症（PPA）和原发性进行性言语失用症（PPAOS）是神经退行性综合征，其特征是语言或言语进行性衰退。越来越多的研究调查 PPA/PPAOS 的言语干预。有必要对治疗证据进行更新的系统评估，以告知最佳临床实践并指导未来的治疗研究。我们系统地回顾了针对该人群的言语和语言行为治疗的证据。经评审的文章已在同行评审期刊上发表，截至 2021 年 5 月 31 日。我们使用美国言语听力协会 (ASHA) 证据水平（一种评估点系统）的修改版本评估了证据水平、报告质量和偏倚风险、额外的报告质量和内部/外部有效性项目，以及酌情使用单例实验设计 量表或物理治疗证据数据库 - 用于随机和非随机对照试验的 PsycBITE 评级量表。使用定量总结和叙述性回顾来综合结果。共有 103 项研究报告了 626 名 PPA 患者的治疗结果；没有研究使用诊断标签 PPAOS。大多数研究评估了单词检索的干预措施。 45 项实验和准实验研究（16 项对照组研究，29 项单受试者设计）提供了最高质量的证据。所有 ( k  = 45/45) 报告主要结果指标均有所改善；报告最多的 是至少一名参与者的治疗的概括性（k  = 34/43）、维持性（k  = 34/39）或社会有效性（k = 17/19）。现有证据支持对 PPA 患者进行言语干预；然而，PPAOS 的治疗有待系统研究。讨论了证据和审查的含义和局限性。