Continuum (Minneap Minn). 2022 Jun 1;28(3):702-725. doi: 10.1212/CON.0000000000001105.
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.
本文回顾了行为变异额颞叶痴呆（bvFTD）和额颞叶变性（FTLD）的许多复杂方面。重点特别放在提高诊断准确性上，以减少许多患者和家庭所经历的艰难诊断之旅。还讨论了促进诊断准确性和处理有问题症状的策略。
尽管国际行为变异额颞叶痴呆共识标准（bvFTD）在十多年前发布，临床病理研究也证实了其有效性，但诊断上的混淆仍在继续。本文提供了最新数据和说明性病例，强调了对临床医生最有用的临床要点。尽管准确预测潜在的蛋白病仍然是一个挑战，但区分 bvFTD 与非典型阿尔茨海默病、精神障碍和其他类似疾病的能力有所提高。少数家族性 FTLD 患者遗传基础的相关知识使早期和准确诊断成为可能。治疗的乐观情绪也有所增加，特别是在家族性 FTLD 中，一些临床试验正在进行中，还有更多的计划，其中一些旨在减缓进展或延迟症状发作，或两者兼而有之。
bvFTD 的诊断和管理对临床医生，尤其是对患者及其家属来说具有挑战性。尽管近年来取得了许多进展，但仍存在一些关键的研究问题。能够显著改善症状或改变 FTLD 病程的治疗方法仍难以捉摸，但随着对发病机制的更好理解和新疗法的开发，乐观情绪正在增强。
