From the Department of Pathology and Molecular Medicine, Thomayer Hospital, and the Department of Pathology, Third Faculty of Medicine, Charles University, Prague, Czech Republic.
Arch Pathol Lab Med. 2014 Jan;138(1):132-8. doi: 10.5858/arpa.2012-0510-RS.
Frontotemporal lobar degenerations are clinically, genetically, and molecularly heterogeneous diseases characterized by mainly frontal and temporal atrophy and affecting behavioral, language, cognitive, and motor functions. The term frontotemporal dementia incorporates 3 distinct clinical syndromes seen in frontotemporal degenerations: behavioral variant of frontotemporal dementia, progressive nonfluent aphasia, and semantic dementia. Progressive supranuclear palsy syndrome, corticobasal syndrome, and motor neuron disease syndrome are also associated with frontotemporal lobar degenerations. The neuropathologic hallmark of frontotemporal lobar degenerations is accumulation of abnormal proteins in the cytoplasm and nuclei of neurons and glial cells. Proteins involved in pathologic processes that represent the basis for frontotemporal lobar degeneration classification are tau protein, transactive response DNA-binding protein of 43 kDa, and "fused in sarcoma" protein. The aim of this review is to provide a summary of practical approaches for neuropathologic diagnostics of the rapidly evolving classifications of frontotemporal lobar degenerations.
额颞叶变性是一种临床、遗传和分子表现多样化的疾病，其特征为额颞叶萎缩，主要影响行为、语言、认知和运动功能。额颞痴呆这一术语包含了额颞变性中出现的 3 种不同的临床综合征：行为变异型额颞痴呆、进行性非流利性失语和语义性痴呆。进行性核上性麻痹综合征、皮质基底节综合征和运动神经元病综合征也与额颞叶变性有关。额颞叶变性的神经病理学特征是神经元和神经胶质细胞的细胞质和核内异常蛋白的积累。参与病理过程的蛋白代表了额颞叶变性分类的基础，这些蛋白包括tau 蛋白、43kDa 的反式激活反应 DNA 结合蛋白和“肉瘤融合”蛋白。本文的目的是提供额颞叶变性快速发展的分类中神经病理学诊断的实用方法的概述。
