Primary progressive aphasia
Primary progressive aphasia
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DOI：
10.1002/ana.91.abs
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发表时间：
2001-04-01
影响因子：
11.2
通讯作者：
Mesulam, MM
中科院分区：
文献类型：
--
作者：
Mesulam, MM
原发性进行性失语症（PPA）是一种局灶性痴呆，其特征是语言功能孤立且逐渐丧失。这种疾病始于找词障碍（失序症），并经常损害语言的语法结构（句法）和理解（语义）。 PPA 中的语音输出可以是流利的，也可以是非流利的。记忆、视觉处理和个性在晚期阶段之前仍保持相对良好的状态，有助于将 PPA 与额叶痴呆和阿尔茨海默氏病的典型形式区分开来。 “语义性痴呆”一词最初是用来指代一组具有语言和视觉处理缺陷的不同患者。然而，在实践中，这种诊断也被用于不同的含义，以表示即使没有视觉处理缺陷，也具有流利言语和理解能力受损的 PPA 亚型。由于语义痴呆的诊断可以具有这两种不同的含义，因此重要的是要明确它是按原始含义使用还是表示 PPA 的亚型。结构和生理神经影像学证实了 PPA 对左半球的选择性偏好，尤其是其语言相关皮质。少数 PPA 患者表现出阿尔茨海默病的神经病理学标志物，但分布不寻常。 PPA 中的大多数尸检显示皮克氏病或脑叶萎缩，但没有独特的组织病理学。有人建议，PPA 和额叶痴呆构成“皮克脑叶萎缩”谱系内单一疾病过程的表型变异。 17 号染色体连锁痴呆的最新进展证明了对散发性 PPA 中 tau 多态性和 tau 病进行严格研究的合理性。针对这种综合征的明智方法可以提高临床医生解决与 PPA 诊断和护理相关的独特挑战的有效性。
Primary progressive aphasia (PPA) is a focal dementia characterized by an isolated and gradual dissolution of language function. The disease starts with word-finding disturbances (anomia) and frequently proceeds to impair the grammatical structure (syntax) and comprehension (semantics) of language. The speech output in PPA can be fluent or nonfluent. Memory, visual processing, and personality remain relatively well-preserved until the advanced stages and help to distiguish PPA from frontal lobe dementia and the typical forms of Alzheimer's disease. The term "semantic dementia" was originally introduced to designate a different group of patients with a combination of verbal and visual processing deficits. In practice, however, this diagnosis is also being used in a variant sense to denote a subtype of PPA with fluent speech and impaired comprehension, even in the absence of visual processing deficits. Insofar as the diagnosis of semantic dementia can have these two different meanings, it is important to specify whether it is being used in the original sense or to denote a subtype of PPA. Structural and physiological neuroimaging confirms the selective predilection of PPA for the left hemisphere, especially for its language-related cortices. A few patients with PPA display the neuropathological markers of Alzheimer's disease, but in an unusual distribution. The majority of the autopsies in PPA have shown either Pick's disease or lobar atrophy without distinctive histopathology. The suggestion has been made that PPA and frontal lobe dementia constitute phenotypical variations of a unitary disease process within the "Pick-lobar atrophy" spectrum. Recent advances in chromosome 17-linked dementias justify a rigorous search for tau polymorphisms and tauopathy in sporadic PPA. An informed approach to this syndrome mill increase the effectiveness with which clinicians can address the unique challenges associated with the diagnosis and care of PPA.