Primary Progressive Aphasias and Apraxia of Speech.
Primary Progressive Aphasias and Apraxia of Speech.
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DOI：
10.1212/con.0000000000000699
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发表时间：
2019-02-01
期刊：
影响因子：
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通讯作者：
Josephs, Keith A
中科院分区：
文献类型：
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作者：
Botha, Hugo;Josephs, Keith A
审查目的：本文综述了两种原发性进行性失语症（PPA），其特征是早期和主要的语言障碍，以及原发性进行性言语失用症，一种与PPA密切相关的退行性运动性言语障碍。概述了如何将这些疾病分类的历史和争议之前，文章重点介绍了每种疾病的临床和影像学features.Recent Findings：在过去的十年中，退化性言语和语言障碍的分类已经得到了完善。临床、影像学和病理学证据表明，原发性进行性言语失用症是一种明显的退行性疾病。此外，多种证据强调了非流利/无语法变体PPA的问题，这使得这种疾病的诊断，预后和研究变得复杂。语义变体PPA，而不是没有争议，仍然是最明确的疾病之一，具有良好的临床病理correlations.SUMMARY：准确的分类和诊断这些退行性言语和语言障碍是至关重要的临床实践和正在进行的研究工作。对于非流利/语法缺失变异型PPA，作者建议强调语法缺失作为核心入选标准，注意不要纳入孤立性或主要言语失用症患者。孤立性言语失用症可能是退行性疾病的表现，基于不同的预后，应该被认为是与PPA不同的。最后，重要的是要认识到，一些语义性痴呆患者，尽管共享相同的病理协会，可能不符合PPA的标准。
PURPOSE OF REVIEW: This article reviews two of the primary progressive aphasias (PPAs), disorders characterized by the early and predominant impairment of language, and primary progressive apraxia of speech, a degenerative motor speech disorder that is closely related to PPA. An outline of the history and controversy surrounding how these disorders are classified is provided before the article focuses on each disorder's clinical and imaging features.RECENT FINDINGS: Over the past decade, the classification of degenerative speech and language disorders has been refined. Clinical, imaging, and pathologic evidence suggests that primary progressive apraxia of speech is a distinct degenerative disorder. Furthermore, multiple lines of evidence have highlighted issues with nonfluent/agrammatic variant PPA, which complicates the diagnosis, prognosis, and study of this disorder. Semantic variant PPA, while not without controversy, remains one of the most well-defined disorders, with good clinicopathologic correlation.SUMMARY: Accurate classification and diagnosis of these degenerative speech and language disorders is crucial in clinical practice and ongoing research efforts. For nonfluent/agrammatic variant PPA, the authors suggest emphasizing agrammatism as the core inclusion criterion and taking care not to include patients with isolated or predominant apraxia of speech. Isolated apraxia of speech can be the manifestation of a degenerative disease and, based on the different prognosis, should be recognized as distinct from PPA. Finally, it is important to recognize that some patients with semantic dementia, despite sharing the same pathologic associations, may not meet criteria for PPA.