Department of Psychiatry, Jikei University School of Medicine, Tokyo 105-8461, Japan.
Int Rev Psychiatry. 2013 Apr;25(2):138-44. doi: 10.3109/09540261.2012.743877.
Frontotemporal lobar degeneration (FTLD) is a heterogeneous syndrome characterized by a progressive decline in behaviour or language associated with degeneration of the frontal and anterior temporal lobes. FTLD can be classified into three clinical syndromes based on the early and predominant symptoms: behavioural variant frontotemporal dementia (bvFTD); semantic dementia (SD); and progressive non-fluent aphasia (PNFA). Patients with bvFTD present with marked changes in personality and behaviour such as disinhibition, apathy, loss of sympathy, compulsive behaviours, hyperorality. Neuroimaging studies have highlighted frontal atrophy, hypometabolism and hypoperfusion. SD is characterized by a fluent anomic aphasia and behavioural changes with degeneration of the anterior temporal lobes. Patients with left-sided SD present with progressive loss of word knowledge and meaning about words, objects and concepts. Patients with PNFA present slow, effortful speech, impaired production and comprehension of grammar. PNFA is associated with atrophy, hypometabolism and hypoperfusion of the left perisylvian area. However, overlap between the syndromes can occur, particularly later in the course. In the absence of definitive biomarkers, the diagnosis is dependent on clinical symptoms. A new diagnostic criterion of bvFTD was published in 2011. Identification of the syndrome's core symptoms is important in clinical diagnosis.
额颞叶变性（FTLD）是一种异质性综合征，其特征是行为或语言逐渐下降，与额极和前颞叶变性有关。FTLD 可以根据早期和主要症状分为三种临床综合征：行为变异型额颞叶痴呆（bvFTD）；语义性痴呆（SD）；进行性非流利性失语症（PNFA）。bvFTD 患者表现为明显的人格和行为改变，如抑制障碍、冷漠、同情心丧失、强迫行为、过度嗜食。神经影像学研究强调了额极萎缩、代谢和灌注减少。SD 的特征是流畅性命名性失语症和行为改变，与前颞叶变性有关。左侧 SD 患者表现为逐渐丧失单词知识和对单词、物体和概念的理解。PNFA 患者表现为语速缓慢、费力，语法生成和理解受损。PNFA 与左大脑外侧裂周围区域的萎缩、代谢和灌注减少有关。然而，这些综合征之间可能存在重叠，特别是在病程后期。在缺乏明确的生物标志物的情况下，诊断依赖于临床症状。2011 年发表了 bvFTD 的新诊断标准。识别综合征的核心症状对临床诊断很重要。
