Clinical and neuroimaging characteristics of primary progressive aphasia.
Clinical and neuroimaging characteristics of primary progressive aphasia.
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DOI：
10.1016/b978-0-12-823384-9.00016-5
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发表时间：
2022
影响因子：
--
通讯作者：
Keser, Zafer
中科院分区：
文献类型：
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作者：
Tippett, Donna C;Keser, Zafer
本章涵盖了原发性进行性失语症（PPA）的临床综合征，这种罕见的神经退行性疾病的人口统计学，定义每种PPA变体的临床和神经解剖学特征，疾病进展和行为特征。本章以简短的介绍开始，包括对定义这种临床综合征及其三种变体的开创性论文的引用。本章中讨论的经典PPA子类型是语义变体PPA（svPPA）、非流利/语法变体PPA（nfaPPA）和逻辑开放变体PPA（lvPPA）。主要的语言和认知特征，以及语言任务，可以引起这些语言障碍，详细说明。重叠的PPA变异，这使得鉴别诊断的挑战，在临床资料的解释。描述了疾病进展，揭示了PPA变体随着时间的推移变得更加相似。虽然PPA是一种语言主导型痴呆，但也有行为表现，特别是在svPPA中。该变体中的行为变化以及nfaPPA和lvPPA中的行为变化被解决，这些变化不太好识别。在左颞叶，顶叶，和/或额叶皮质萎缩的模式独特的每一个PPA的变体进行了描述。讨论了PPA变体的潜在神经病理学，特别是与svPPA和nfaPPA相关的tau蛋白病和非tau蛋白病以及lvPPA中的阿尔茨海默病病理学。
The chapter covers the clinical syndrome of primary progressive aphasia (PPA), the demographics of this rare neurodegenerative disease, defining clinical and neuroanatomical characteristics of each PPA variant, disease progression, and behavioral features. The chapter begins with a brief introduction that includes references to seminal papers that defined this clinical syndrome and its three variants. The classic PPA subtypes discussed in the chapter are semantic variant PPA (svPPA), nonfluent/agrammatic PPA (nfaPPA), and logopenic variant PPA (lvPPA). The key language and cognitive characteristics, and language tasks that can elicit these language impairments, are detailed. Overlap in the clinical profiles of the PPA variants, which make differential diagnosis challenging, are explained. Disease progression is described, revealing that the PPA variants become more similar over time. Although PPA is a language-predominant dementia, there are behavioral manifestations, particularly in svPPA. Changes in behavior in this variant are addressed as well as behavioral changes in nfaPPA and lvPPA that are less well recognized. The patterns of atrophy in the left temporal, parietal, and/or frontal cortices unique to each PPA variant are described. The underlying neuropathologies of the PPA variants are discussed, specifically tau-opathies and non-tau-opathies associated with svPPA and nfaPPA and Alzheimer’s disease pathology in lvPPA.