Article

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).
CI - Copyright (c) 2013. Published by Elsevier Masson SAS.

Langue : ANGLAIS