Purpose of Review

The term primary progressive aphasia (PPA) refers to a diverse group of dementias that present with prominent and early problems with speech and language. They present considerable challenges to clinicians and researchers.

Recent Findings

Here, we review critical issues around diagnosis of the three major PPA variants (semantic variant PPA, nonfluent/agrammatic variant PPA, logopenic variant PPA), as well as considering ‘fragmentary’ syndromes. We next consider issues around assessing disease stage, before discussing physiological phenotyping of proteinopathies across the PPA spectrum. We also review evidence for core central auditory impairments in PPA, outline critical challenges associated with treatment, discuss pathophysiological features of each major PPA variant, and conclude with thoughts on key challenges that remain to be addressed.

Summary

New findings elucidating the pathophysiology of PPA represent a major step forward in our understanding of these diseases, with implications for diagnosis, care, management, and therapies.

中文翻译：

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原发性进行性失语症：病理生理学综合

审查目的

原发性进行性失语症 (PPA) 一词是指一组表现出突出的早期言语和语言问题的痴呆症。它们给临床医生和研究人员带来了相当大的挑战。

最近的发现

在这里，我们回顾了围绕三种主要 PPA 变体（语义变体 PPA、不流利/语法变体 PPA、语词减少变体 PPA）诊断的关键问题，以及考虑“碎片”综合征。接下来，我们将考虑评估疾病阶段的问题，然后再讨论 PPA 谱中蛋白质病的生理表型。我们还回顾了 PPA 核心中枢听觉障碍的证据，概述了与治疗相关的关键挑战，讨论了每个主要 PPA 变体的病理生理学特征，并总结了对仍有待解决的关键挑战的想法。

概括

阐明 PPA 病理生理学的新发现代表了我们对这些疾病的理解向前迈出了一大步，对诊断、护理、管理和治疗都有影响。