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行为变异额颞叶痴呆的分子病理学影像学特征。

Imaging signatures of molecular pathology in behavioral variant frontotemporal dementia.

机构信息

Department of Radiology, Mayo Clinic Rochester, Rochester, MN, USA.

出版信息

J Mol Neurosci. 2011 Nov;45(3):372-8. doi: 10.1007/s12031-011-9533-3. Epub 2011 May 10.

DOI:10.1007/s12031-011-9533-3
PMID:21556732
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC3401589/
Abstract

Pathology underlying behavioral variant frontotemporal dementia (bvFTD) is heterogeneous, with the most common pathologies being Pick's disease (PiD), corticobasal degeneration (CBD), and FTLD-TDP type 1. Clinical features are unhelpful in differentiating these pathologies. We aimed to determine whether imaging atrophy patterns differ across these pathologies in bvFTD subjects. We identified 15 bvFTD subjects that had volumetric MRI during life and autopsy: five with PiD, five CBD, and five FTLD-TDP type 1. Voxel-based morphometry was used to assess atrophy patterns in each bvFTD group compared to 20 age- and gender-matched controls. All three pathological groups showed gray matter loss in frontal lobes, although specific patterns of atrophy differed across groups: PiD showed widespread loss in frontal lobes with additional involvement of anterior temporal lobes; CBD showed subtle patterns of loss involving posterior lateral and medial superior frontal lobe; and FTLD-TDP type 1 showed widespread loss in frontal, temporal, and parietal lobes. Greater parietal loss was observed in FTLD-TDP type 1 compared to both other groups, and greater anterior temporal and medial frontal loss was observed in PiD compared to CBD. Imaging patterns of atrophy in bvFTD vary according to pathological diagnosis and may therefore be helpful in predicting these pathologies in bvFTD.

摘要

行为变异额颞叶痴呆(bvFTD)的病理学基础具有异质性,最常见的病理学改变包括 Pick 病(PiD)、皮质基底节变性(CBD)和 FTLD-TDP 型 1。临床特征无助于区分这些病理学改变。我们旨在确定在 bvFTD 患者中,这些病理学改变的影像学萎缩模式是否存在差异。我们确定了 15 名在生前进行过容积 MRI 检查且有尸检结果的 bvFTD 患者:5 名 PiD、5 名 CBD 和 5 名 FTLD-TDP 型 1。我们使用基于体素的形态测量学方法来评估每个 bvFTD 组与 20 名年龄和性别匹配的对照组之间的萎缩模式。所有三组病理学改变患者的额叶都出现灰质丢失,尽管萎缩模式存在差异:PiD 患者表现为额叶广泛丢失,伴有前颞叶额外受累;CBD 患者表现为涉及后外侧和内侧额上回的细微丢失模式;FTLD-TDP 型 1 患者则表现为额叶、颞叶和顶叶广泛丢失。与其他两组相比,FTLD-TDP 型 1 患者的顶叶丢失更明显,与 CBD 相比,PiD 患者的前颞叶和内侧额叶丢失更明显。bvFTD 的影像学萎缩模式根据病理诊断而有所不同,因此可能有助于预测 bvFTD 的这些病理学改变。

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本文引用的文献

1
The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene.最常见的 FTLD-FUS 类型(aFTLD-U)与特定的额颞叶痴呆临床形式相关,但与 FUS 基因突变无关。
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Does TDP-43 type confer a distinct pattern of atrophy in frontotemporal lobar degeneration?TDP-43 型是否在额颞叶变性中导致独特的萎缩模式?
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TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia.TDP-43 亚型与额颞叶痴呆的不同萎缩模式相关。
Neurology. 2010 Dec 14;75(24):2204-11. doi: 10.1212/WNL.0b013e318202038c.
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Neurology. 2010 Nov 23;75(21):1879-87. doi: 10.1212/WNL.0b013e3181feb2e8.
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FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration.FUS 病理学定义了大多数 tau 和 TDP-43 阴性额颞叶变性。
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Brain. 2009 Nov;132(Pt 11):2932-46. doi: 10.1093/brain/awp232. Epub 2009 Sep 17.
8
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes.额颞叶痴呆-泛素阳性型(FTLD-U)亚型的皮质下病理及临床相关性评估
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MRI correlates of protein deposition and disease severity in postmortem frontotemporal lobar degeneration.死后额颞叶变性中蛋白质沉积与疾病严重程度的磁共振成像相关性
Neurodegener Dis. 2009;6(3):106-17. doi: 10.1159/000209507. Epub 2009 Mar 19.
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Ann Neurol. 2008 Jul;64(1):4-14. doi: 10.1002/ana.21426.