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剖析额颞叶痴呆的多面性:影像学视角。

Dissecting the Many Faces of Frontotemporal Dementia: An Imaging Perspective.

机构信息

Department of Molecular and Translational Medicine, University of Brescia, 25123 Brescia, Italy.

Stroke Unit, Department of Neurological and Vision Sciences, ASST Spedali Civili, 25123 Brescia, Italy.

出版信息

Int J Mol Sci. 2022 Oct 25;23(21):12867. doi: 10.3390/ijms232112867.

Abstract

Frontotemporal dementia (FTD) is a heterogeneous clinical and neuropathological disorder characterized by behavioral abnormalities, executive dysfunctions and language deficits. FTD encompasses a wide range of different pathological entities, associated with the accumulation of proteins, such as tau and TPD-43. A family history of dementia is found in one third of cases, and several genes causing autosomal dominant inherited disease have been identified. The clinical symptoms are preceded by a prodromal phase, which has been mainly studied in cases carrying pathogenetic mutations. New experimental strategies are emerging, in both prodromal and clinical settings, and outcome markers are needed to test their efficacy. In this complex context, in the last few years, advanced neuroimaging techniques have allowed a better characterization of FTD, supporting clinical diagnosis, improving the comprehension of genetic heterogeneity and the earliest stages of the disease, contributing to a more detailed classification of underlying proteinopathies, and developing new outcome markers on clinical grounds. In this review, we briefly discuss the contribution of brain imaging and the most recent techniques in deciphering the different aspects of FTD.

摘要

额颞叶痴呆(FTD)是一种异质性的临床和神经病理学疾病,其特征为行为异常、执行功能障碍和语言缺陷。FTD 包含广泛的不同病理实体,与蛋白质的积累有关,如 tau 和 TPD-43。三分之一的病例有痴呆家族史,已经确定了几个导致常染色体显性遗传疾病的基因。临床症状前有前驱期,主要在携带发病突变的病例中进行了研究。在前驱期和临床环境中出现了新的实验策略,需要有结局标志物来检验其疗效。在这种复杂的情况下,在过去几年中,先进的神经影像学技术使得 FTD 的特征得到了更好的描述,支持了临床诊断,提高了对遗传异质性和疾病早期阶段的理解,有助于更详细地分类潜在的蛋白病,并基于临床开发新的结局标志物。在这篇综述中,我们简要讨论了脑成像和最近的技术在解析 FTD 不同方面的贡献。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1c4b/9654520/943c0fc0469a/ijms-23-12867-g001.jpg

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