Laboratoire de neuropathologie Escourolle, pôle des maladies du système nerveux, CHU Pitié Salpétrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France; Équipe causes de la SLA et mécanismes des maladies du motoneurone, CRICM, UPMC UMR_S975, Inserm U 975, CNRS UMR 7225, 75013 Paris, France.
Rev Neurol (Paris). 2013 Oct;169(10):786-92. doi: 10.1016/j.neurol.2013.07.015. Epub 2013 Sep 12.
Frontotemporal lobar degeneration (FTLD) is a heterogeneous group including both sporadic and familial diseases, characterized by a macroscopic alteration. It may correspond to various cognitive syndromes: behavioral variant of frontotemporal dementia (bvFTD), progressive nonfluent aphasia, and semantic dementia. The neuropathologic classification is now based on identification of the protein that accumulates in neurons and glia: Tau, TAR DNA Binding Protein 43 (TDP-43), and FUsed in Sarcoma (FUS). The disorders in which the corresponding proteins accumulate have been named FTLD-Tau, FTLD-TDP, and FTLD-FUS. FTLD-Tau includes sporadic cases (e.g. Pick's disease) and Tau mutations. FTLD-TDP are subdivided within four types (A, B, C, D) according to the shape and distribution of TDP-43 positive lesions within the associative frontal cortex. The FTLD-FUS group includes atypical FTLD with ubiquitinated lesions (FTLD-U), Neuronal Intermediate Filament Inclusion Disease (NIFID) and Basophilic Inclusion Body Disease (BIBD).
额颞叶变性（FTLD）是一组异质性疾病，包括散发性和家族性疾病，以宏观改变为特征。它可能对应于各种认知综合征：额颞叶痴呆的行为变异型（bvFTD）、进行性非流利性失语症和语义性痴呆。神经病理学分类现在基于在神经元和神经胶质中积累的蛋白质的鉴定：Tau、TAR DNA 结合蛋白 43（TDP-43）和肉瘤 Fused（FUS）。相应蛋白质积累的疾病已被命名为 FTLD-Tau、FTLD-TDP 和 FTLD-FUS。FTLD-Tau 包括散发性病例（如皮克病）和 Tau 突变。FTLD-TDP 根据在额联系皮质内 TDP-43 阳性病变的形状和分布分为四个类型（A、B、C、D）。FTLD-FUS 组包括具有泛素化病变的非典型 FTLD（FTLD-U）、神经元中间丝包涵体病（NIFID）和嗜碱性包涵体病（BIBD）。
