Behavioral Variant Frontotemporal Dementia.
Behavioral Variant Frontotemporal Dementia.
复制标题
DOI：
10.1212/con.0000000000001105
复制
发表时间：
2022-06-01
期刊：
影响因子：
--
通讯作者：
Boeve, Bradley F
中科院分区：
文献类型：
--
作者：
Boeve, Bradley F
本文回顾了行为变异型额颞叶痴呆（bvFTD）和额颞叶变性（FTLD）的诸多复杂方面。特别聚焦于提高诊断准确性，以缩短众多患者及其家属所经历的漫长而艰难的诊断过程。文中还探讨了提高诊断准确性的策略以及处理棘手症状的方法。
尽管bvFTD的国际共识标准在十多年前就已发布，且临床病理研究也证实了其效用，但诊断混淆的情况依然存在。本文呈现了最新数据及实例，以强调对临床医生最为实用的临床要点。尽管准确预测潜在的蛋白病仍颇具挑战，但如今区分bvFTD与非典型阿尔茨海默病、精神障碍及其他类似病症的能力已有提升。对相当一部分家族性FTLD个体遗传基础的了解，有助于实现早期准确诊断。治疗前景也更加乐观，尤其是在家族性FTLD方面，目前有几项临床试验正在进行，还有更多计划中的试验，其中一些旨在减缓疾病进展或推迟症状发作，或二者兼具。
bvFTD的诊断与治疗对临床医生而言颇具挑战，对患者及其家属来说更是如此。尽管近年来已取得诸多进展，但仍存在几个关键的研究问题。能显著改善症状或改变FTLD病程的治疗方法尚未问世，但随着对疾病病理生物学的深入理解以及新疗法的不断研发，前景愈发乐观。
This article reviews many of the complex facets of behavioral variant frontotemporal dementia (bvFTD) and frontotemporal lobar degeneration (FTLD). A particular focus is on improving diagnostic accuracy to reduce the arduous diagnostic odyssey that so many patients and families endure. Strategies to promote diagnostic accuracy and approach the management of problematic symptoms are also discussed. Although the International Consensus Criteria for bvFTD were published more than a decade ago and clinicopathologic studies have confirmed their utility, diagnostic confusion continues. This article presents updated data along with illustrative cases to emphasize the clinical pearls that are most useful for clinicians. Although accurate prediction of the underlying proteinopathy remains a challenge, the ability to differentiate bvFTD from atypical Alzheimer disease, psychiatric disorders, and other mimickers has improved. Knowledge about the genetic underpinnings in a significant minority of individuals with familial FTLD is enabling early and accurate diagnosis. Therapeutic optimism has also increased, particularly in familial FTLD, with a few clinical trials in progress and several more planned, some of which are designed to slow progression or delay the onset of symptoms, or both. The diagnosis and management of bvFTD is challenging for clinicians and particularly for patients and their families. Although much progress has been gained over recent years, several key research questions persist. Treatments that significantly improve symptoms or alter the course of FTLD remain elusive, but optimism is increasing as pathobiology is better understood and novel therapies are being developed.