Department of Neurology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.
Int Rev Psychiatry. 2013 Apr;25(2):145-58. doi: 10.3109/09540261.2013.763341.
Frontotemporal lobar degeneration (FTLD) can manifest as a spectrum of clinical syndromes, ranging from behavioural impairment to language or motor dysfunction. Recently, revised diagnostic criteria have been proposed for the behavioural and progressive aphasia syndromes associated with frontotemporal degeneration. The present review will summarize these diagnostic guidelines and highlight some lingering controversies in the classification of FTLD clinical syndromes. We will discuss common tools and methods used to identify the insidious changes of behavioural variant frontotemporal dementia (bvFTD), the value of new, patient-based tasks of orbitofrontal function, and the issue of a benign or 'phenocopy' variant of bvFTD. With regard to primary progressive aphasia (PPA), we will discuss the scope of the semantic disorder in semantic-variant PPA, the nature of the speech disorder in non-fluent, agrammatic PPA, and the preliminary utility of a logopenic PPA classification.
额颞叶变性（FTLD）可表现为一系列临床综合征，从行为障碍到语言或运动功能障碍。最近，提出了与额颞叶变性相关的行为和进行性失语综合征的修订诊断标准。本综述将总结这些诊断指南，并强调额颞叶变性临床综合征分类中的一些持续存在的争议。我们将讨论用于识别行为变异额颞叶痴呆（bvFTD）隐匿性变化的常用工具和方法、基于新患者的眶额功能任务的价值，以及 bvFTD 的良性或“表型”变异的问题。关于原发性进行性失语症（PPA），我们将讨论语义变异型 PPA 中语义障碍的范围、非流利性语法障碍型 PPA 中言语障碍的性质，以及失读型 PPA 分类的初步应用。
