Focal cortical atrophy syndromes
Focal cortical atrophy syndromes
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DOI：
10.1006/brcg.1996.0042
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发表时间：
1996-07-01
影响因子：
2.5
通讯作者：
Black, SE
中科院分区：
文献类型：
--
作者：
Black, SE
简要回顾阿尔茨海默病（AD）的地形及其对语言、感知和实践的影响，作为局灶性皮质萎缩综合征的背景，包括原发性进行性失语（PPA）、后皮质萎缩（PCA）和皮质基底节变性（CBD）。简单地说，有两种主要的病理和神经影像表型与这些症状相关 综合症。一种是额颞叶变性（FTD），一种非特异性海绵状变性，伴有神经胶质增生和神经元损失，有时伴有皮克细胞和体，通常对额叶和前颞叶皮层具有选择性。另一种是阿尔茨海默病，淀粉样蛋白斑和神经原纤维缠结最初在海马区形成，并扩散到外侧颞叶和顶叶新皮质，然后扩散到额叶皮质。对于 PPA，左侧裂周围功能障碍通常在临床表现和神经影像学上很明显。不流利进行性失语往往向前进展，通常与 FTD 相关。在流利进行性失语中，病理学可能因 FTD 而向前进展，也可能因 AD 而向后进展。在 PCA 中，视觉关联皮层的目标是双侧，通常是右侧，病理学通常表明 AD。 CBD是一种与失用症相关的不对称性运动不能-强直综合征，在光学显微镜下，额顶皮质和黑质中存在肿胀的消色差神经元，这表明它可能属于FTD和皮克病的范围。然而，新分子生物学技术的应用表明，CBD、FTD和皮克病在病理上可能是不同的。回顾了每种综合征的临床病理特征，并将其与典型的 AD 特征进行比较，并说明了每种综合征的代表单光子发射计算机断层扫描。 (C) 1996 学术出版社
The topography of Alzheimer's disease (AD) and its effects on language, perception, and praxis are briefly reviewed as background to the focal cortical atrophy syndromes, including primary progressive aphasia (PPA), posterior cortical atrophy (PCA), and corticobasal degeneration (CBD), Simplistically speaking, there are two main pathological and neuroimaging phenotypes associated with these syndromes. One is frontotemporal degeneration (FTD), a nonspecific spongiform degeneration, with gliosis and neuronal loss, sometimes with Pick cells and bodies, which is usually selective for frontal and anterior temporal cortex. The other is Alzheimer's disease, in which amyloid plaques and neurofibrillary tangles initially develop in the hippocampal region, and spread to the lateral temporal and parietal neocortex and then to the frontal cortex. In the ease of PPA, left perisylvian dysfunction is usually evident at presentation clinically and on neuroimaging. Nonfluent progres sive aphasia tends to progress anteriorly and is usually associated with FTD. In fluent progressive aphasia, the pathology may progress anteriorly due to FTD or posteriorly, reflecting AD. In PCA, the visual association cortex is targeted bilaterally, often mon so on the right, and the pathology is usually indicative of AD. CBD is an asymmetric akinetic-rigid syndrome associated with apraxia, in which swollen achromatic neurons are present in the frontoparietal cortex and substantia nigra on light microscopy, suggesting to some that it may fall within the spectrum of FTD and Pick's disease, The application of new molecular biological techniques, however, suggest that CBD, FTD, and Pick's Disease may be pathologically distinct. The clinico-pathological features of each syndrome are reviewed and compared to those typical for AD, and single photon emission computerized tomography scans representative for each are illustrated. (C) 1996 Academic Press, Inc.