Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Tanz Building, 6 Queen's Park Cres West, Room 231, Toronto, ON, Canada M5S 3H2.
Neuroimaging Clin N Am. 2012 Feb;22(1):83-97, viii. doi: 10.1016/j.nic.2011.11.009.
Frontotemporal dementia (FTD) describes a group of clinical syndromes united by underlying frontotemporal lobar degeneration (FTLD) pathology. The clinical syndromes associated with FTLD are heterogeneous and are based on whether the patients present with behavioral, language, or motor impairments. FTLD is at the center of a paradigm shift in neurodegenerative diseases, with thought being given at diagnosis of underlying disease. There is pathologic heterogeneity of certain clinical syndromes such as behavioral variant FTD. Differentiation between the proteinopathies will become imperative as protein-specific treatments become available. This review provides an overview of FTLD, with an update of recent discoveries.
额颞叶痴呆（FTD）描述了一组以额颞叶变性（FTLD）病理学为基础的临床综合征。与 FTLD 相关的临床综合征具有异质性，并且基于患者是否表现出行为、语言或运动障碍。FTLD 是神经退行性疾病范式转变的核心，在诊断时就考虑到了潜在疾病。某些临床综合征（如行为变异型额颞叶痴呆）存在病理学异质性。随着特定于蛋白质的治疗方法的出现，区分蛋白病将变得至关重要。本文综述了 FTLD 的最新发现。
