Jankowicz E, Drozdowski W, Halicka D
Kliniki Neurologii Akademii Medycznej w Białymstoku.
Neurol Neurochir Pol. 2000 May-Jun;34(3):553-64.
Frontotemporal dementias are the second largest degenerative dementia group after Alzheimer's disease. It is a clinical syndrome corresponding to at least three histological entities: Pick's disease, non-specific frontotemporal degeneration, frontal lobe abnormalities associated with motor neuron disease. There are four group of symptoms in the clinical description of FTD: behavioural disorder, affective symptoms, speech disorders, neurological signs. FTD is associated with primary degeneration of the frontal and temporal lobes. Histologically there was neuronal loss, microvacuolation, tau- and ubiquitin-immunoreactive inclusions. The ballooned cortical neurons and tau- and ubiquitin-immunoreactive, argyrophilic inclusions have been called Pick-type histology. There are many descriptions of association of FTD and Pick's disease with motor neuron disease and amyotrophic lateral sclerosis. Histological changes were similar to cortical ones. In this study, we described clinical characteristic features of frontotemporal dementia and difficulties in its identification. The distinctive histopathological pattern in the FTD patients and its value to differentiate frontotemporal degeneration from other degenerative dementias is discussed.
额颞叶痴呆是仅次于阿尔茨海默病的第二大退行性痴呆组。它是一种临床综合征，对应至少三种组织学实体：匹克病、非特异性额颞叶变性、与运动神经元病相关的额叶异常。在额颞叶痴呆的临床描述中有四组症状：行为障碍、情感症状、言语障碍、神经体征。额颞叶痴呆与额叶和颞叶的原发性变性有关。组织学上有神经元丢失、微空泡形成、tau蛋白和泛素免疫反应性包涵体。气球样皮质神经元以及tau蛋白和泛素免疫反应性、嗜银性包涵体被称为匹克型组织学。有许多关于额颞叶痴呆和匹克病与运动神经元病及肌萎缩侧索硬化症关联的描述。组织学变化与皮质的相似。在本研究中，我们描述了额颞叶痴呆的临床特征及其识别中的困难。讨论了额颞叶痴呆患者独特的组织病理学模式及其在将额颞叶变性与其他退行性痴呆区分开来方面的价值。
