B' Department of Neurology, AHEPA University Hospital, Thessaloniki, Greece.
J Neurol Sci. 2012 Jul 15;318(1-2):171-3. doi: 10.1016/j.jns.2012.04.002. Epub 2012 Apr 26.
Frontotemporal lobar degeneration (FTLD) comprises of behavioral variant frontotemporal dementia (bvFTD) and primary progressive aphasia (PPA) with its 3 main variants, namely nonfluent/agrammatic (naPPA), semantic (svPPA) and logopenic (lvPPA). Recently a clinical syndrome with predominant right temporal atrophy was recognized (rvFTD). FTLD often overlaps with parkinsonism plus syndromes such as corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP), as well as with motor neuron disease (FTD-MND). While FTLD syndromes were thought to be rare and difficult to diagnose ante mortem, revised diagnostic criteria as well as recent studies highlighted the plausibility of accurate clinical diagnosis.
232 FTLD patients were assessed from January 1, 2003 to December 31, 2010 in the Neurology Department of a tertiary referral center. Patients were classified as bvFTD, naPPA, svPPA, lvPPA, CBD/PSP and rvFTD and their demographic characteristics were analyzed.
From the 232 patients, 111 (47.8%) were diagnosed with bvFTD, 56 (24.1%) with naPPA, 21 (9.1%) with svPPA, 6 (2.6%) with lvPPA, 20 (8.6%) with CBD or PSP and 18 (7.8%) with rvFTD. 44% of the patients were under 65 years old at onset of symptoms, while only 4.3% reported family history of dementia. FTLD subgroups did not differ with respect to demographic characteristics, but early onset cases had higher educational level.
FTLD represents a syndrome with different but clinically distinguishable phenotypes. Cultural, educational and socioeconomic status differences might regulate patients' access to medical care and therefore influence age of reported onset and prevalence of FTLD in clinical studies. High clinical alertness and sensitive neuropsychological tests could lead to timely clinical diagnosis in a common presenile type of dementia.
额颞叶变性（FTLD）包括行为变异额颞叶痴呆（bvFTD）和原发性进行性失语症（PPA），其主要有 3 种变体，即非流利/语法障碍型（naPPA）、语义型（svPPA）和命名性流畅型（lvPPA）。最近，一种以右侧颞叶萎缩为主的临床综合征（rvFTD）被识别出来。FTLD 常与帕金森病加综合征重叠，如皮质基底节变性（CBD）和进行性核上性麻痹（PSP），以及运动神经元病（FTD-MND）。虽然 FTLD 综合征被认为是罕见且难以在生前做出诊断的，但修订后的诊断标准以及最近的研究强调了准确临床诊断的可能性。
2003 年 1 月 1 日至 2010 年 12 月 31 日，在一家三级转诊中心的神经内科对 232 名 FTLD 患者进行评估。患者被分类为 bvFTD、naPPA、svPPA、lvPPA、CBD/PSP 和 rvFTD，并分析了他们的人口统计学特征。
在 232 名患者中，111 名（47.8%）被诊断为 bvFTD，56 名（24.1%）为 naPPA，21 名（9.1%）为 svPPA，6 名（2.6%）为 lvPPA，20 名（8.6%）为 CBD/PSP，18 名（7.8%）为 rvFTD。44%的患者在症状出现时年龄在 65 岁以下，而只有 4.3%的患者报告有痴呆家族史。FTLD 亚组在人口统计学特征方面没有差异，但早期发病的患者教育程度较高。
FTLD 代表一种具有不同但临床上可区分的表型的综合征。文化、教育和社会经济地位的差异可能会影响患者获得医疗保健的机会，从而影响临床研究中 FTLD 的发病年龄和患病率。高临床警觉性和敏感的神经心理学测试可以导致在一种常见的早发性痴呆中及时进行临床诊断。
