title: Frontotemporal lobar degeneration: diversity of FTLD lesions.
abstract: 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).
authors.affiliation: 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. Electronic address: danielle.seilhean@upmc.fr.
authors.forename: D
authors.initials: D
authors.lastname: Seilhean
authors.forename: F
authors.initials: F
authors.lastname: Bielle
authors.forename: I
authors.initials: I
authors.lastname: Plu
authors.forename: C
authors.initials: C
authors.lastname: Duyckaerts
mesh_terms: D057174:Frontotemporal Lobar Degeneration / Q000145:classification* / Q000175:diagnosis / Q000235:genetics; D018740:Genetic Heterogeneity; D006801:Humans; D036341:Intercellular Signaling Peptides and Proteins / Q000235:genetics; D010641:Phenotype; D000077153:Progranulins; D034702:RNA-Binding Protein FUS / Q000235:genetics; D024801:Tauopathies / Q000150:complications / Q000235:genetics; D016875:tau Proteins / Q000235:genetics
chemical_list: C492314:GRN protein, human; D036341:Intercellular Signaling Peptides and Proteins; D000077153:Progranulins; D034702:RNA-Binding Protein FUS; D016875:tau Proteins
keywords: BIBD; C9ORF72; Dégénérescence lobaire frontotemporale; Démence frontotemporale; FUS; Frontotemporal dementia; Frontotemporal lobar degeneration; Maladie de Pick; Multisystem proteinopathy; NIFID; PCV; Pick's disease; Progranulin; Progranuline; TDP-43; Tau; VCP