Diagnosis and management of frontotemporal dementia: a narrative review.

IF 2.8 Q3 CLINICAL NEUROLOGY
Neurodegenerative disease management Pub Date : 2026-08-01 Epub Date: 2026-06-17 DOI:10.1080/17582024.2026.2687481
Valentina Cenacchi, Tiziana Lombardo, Federica Palacino, Tatiana Cattaruzza, Paolo Manganotti, Alberto Benussi
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引用次数: 0

Abstract

Frontotemporal dementia (FTD) is an umbrella term that encompasses a group of clinically heterogeneous neurodegenerative disorders. It is biologically referred to as Frontotemporal lobar degeneration (FTLD) and is characterized by progressive degeneration of frontal and/or temporal lobes. FTD poses substantial diagnostic and therapeutic challenges due to its heterogeneous clinical presentations, limited biomarker specificity, and overlap with other neurodegenerative and psychiatric diseases. This review synthesizes updated knowledge on the clinical phenotypes of FTD, their prognostic elements, and the underlying genetic and molecular mechanisms. Advances in diagnostic strategies are discussed, including structural and functional neuroimaging, fluid biomarkers, and genetic testing, together with emerging digital and AI-assisted tools that may enhance diagnostic precision. Evidence-based management approaches are examined, alongside the role of multidisciplinary care and caregiver support. Promising therapeutic strategies, including gene-targeted interventions, antisense oligonucleotides, progranulin restoration strategies, immunotherapies, and neuromodulation techniques, are discussed considering recent clinical and preclinical developments. Despite the absence of approved disease-modifying therapies, rapid progress in biomarker development, patient stratification, and personalized approaches offers encouraging prospects for more effective interventions across the FTD spectrum. PubMed/MEDLINE was searched for peer-reviewed articles on FTD diagnosis and management; reference lists of key articles were screened to identify additional sources.

额颞叶痴呆的诊断和治疗:一个叙述性的回顾。
额颞叶痴呆(FTD)是一个总称,包括一组临床异质性神经退行性疾病。它在生物学上被称为额颞叶变性(FTLD),其特征是额叶和/或颞叶的进行性变性。由于其临床表现的异质性、有限的生物标志物特异性以及与其他神经退行性和精神疾病的重叠,FTD给诊断和治疗带来了巨大的挑战。本文综述了FTD的临床表型、预后因素以及潜在的遗传和分子机制方面的最新知识。讨论了诊断策略的进展,包括结构和功能神经成像、流体生物标志物和基因检测,以及可能提高诊断精度的新兴数字和人工智能辅助工具。循证管理方法,以及多学科护理和护理人员支持的作用进行了审查。有希望的治疗策略,包括基因靶向干预,反义寡核苷酸,颗粒前恢复策略,免疫疗法和神经调节技术,讨论了最近的临床和临床前发展。尽管缺乏经批准的疾病改善疗法,但生物标志物开发、患者分层和个性化方法的快速进展为更有效的FTD干预提供了令人鼓舞的前景。在PubMed/MEDLINE检索关于FTD诊断和管理的同行评议文章;筛选了关键文章的参考文献列表,以确定其他来源。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
4.30
自引率
0.00%
发文量
35
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