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临床前和前驱性额颞叶痴呆定义的概念框架。

Conceptual framework for the definition of preclinical and prodromal frontotemporal dementia.

作者信息

Benussi Alberto, Alberici Antonella, Samra Kiran, Russell Lucy L, Greaves Caroline V, Bocchetta Martina, Ducharme Simon, Finger Elizabeth, Fumagalli Giorgio, Galimberti Daniela, Jiskoot Lize C, Le Ber Isabelle, Masellis Mario, Nacmias Benedetta, Rowe James B, Sanchez-Valle Raquel, Seelaar Harro, Synofzik Matthis, Rohrer Jonathan D, Borroni Barbara

机构信息

Neurology Unit, Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy.

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

出版信息

Alzheimers Dement. 2022 Jul;18(7):1408-1423. doi: 10.1002/alz.12485. Epub 2021 Dec 7.

Abstract

The presymptomatic stages of frontotemporal dementia (FTD) are still poorly defined and encompass a long accrual of progressive biological (preclinical) and then clinical (prodromal) changes, antedating the onset of dementia. The heterogeneity of clinical presentations and the different neuropathological phenotypes have prevented a prior clear description of either preclinical or prodromal FTD. Recent advances in therapeutic approaches, at least in monogenic disease, demand a proper definition of these predementia stages. It has become clear that a consensus lexicon is needed to comprehensively describe the stages that anticipate dementia. The goal of the present work is to review existing literature on the preclinical and prodromal phases of FTD, providing recommendations to address the unmet questions, therefore laying out a strategy for operationalizing and better characterizing these presymptomatic disease stages.

摘要

额颞叶痴呆(FTD)的症状前阶段仍定义不清,包括长时间累积的渐进性生物学(临床前)变化,随后是临床(前驱)变化,早于痴呆症的发作。临床表现的异质性和不同的神经病理表型使得之前无法对临床前或前驱FTD进行清晰描述。治疗方法的最新进展,至少在单基因疾病方面,需要对这些痴呆前阶段进行恰当定义。显然,需要一个共识词汇表来全面描述早于痴呆症的阶段。本研究的目的是回顾关于FTD临床前和前驱阶段的现有文献,针对未解决的问题提供建议,从而制定出一个对这些症状前疾病阶段进行操作化和更好特征描述的策略。

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