Neuropathology of frontotemporal lobar degeneration: A review

ABSTRACT Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia. Three main clinical variants are widely recognized within the FTLD spectrum: the behavioural variant of frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). FTLD represents a highly heterogeneous group of neurodegenerative disorders which are best classified according to the main protein component of pathological neuronal and glial inclusions. The most common pathological class of FTLD is associated with the TDP-43 protein (FTLD-TDP), while FTLD-Tau is considered slightly less common while the FTLD-FUS (Fused in sarcoma protein) pathology is rare. In this review, these three major pathological types of FTLD are discussed.

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Main Authors: Bahia,Valéria Santoro, Takada,Leonel Tadao, Deramecourt,Vincent
Format: Digital revista
Language:English
Published: Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento 2013
Online Access:http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642013000100019
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spelling oai:scielo:S1980-576420130001000192016-06-16Neuropathology of frontotemporal lobar degeneration: A reviewBahia,Valéria SantoroTakada,Leonel TadaoDeramecourt,Vincent frontotemporal lobar degeneration pathology TAU TDP FUS ABSTRACT Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia. Three main clinical variants are widely recognized within the FTLD spectrum: the behavioural variant of frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). FTLD represents a highly heterogeneous group of neurodegenerative disorders which are best classified according to the main protein component of pathological neuronal and glial inclusions. The most common pathological class of FTLD is associated with the TDP-43 protein (FTLD-TDP), while FTLD-Tau is considered slightly less common while the FTLD-FUS (Fused in sarcoma protein) pathology is rare. In this review, these three major pathological types of FTLD are discussed.info:eu-repo/semantics/openAccessAcademia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e EnvelhecimentoDementia & Neuropsychologia v.7 n.1 20132013-03-01info:eu-repo/semantics/articletext/htmlhttp://old.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642013000100019en10.1590/S1980-57642013DN70100004
institution SCIELO
collection OJS
country Brasil
countrycode BR
component Revista
access En linea
databasecode rev-scielo-br
tag revista
region America del Sur
libraryname SciELO
language English
format Digital
author Bahia,Valéria Santoro
Takada,Leonel Tadao
Deramecourt,Vincent
spellingShingle Bahia,Valéria Santoro
Takada,Leonel Tadao
Deramecourt,Vincent
Neuropathology of frontotemporal lobar degeneration: A review
author_facet Bahia,Valéria Santoro
Takada,Leonel Tadao
Deramecourt,Vincent
author_sort Bahia,Valéria Santoro
title Neuropathology of frontotemporal lobar degeneration: A review
title_short Neuropathology of frontotemporal lobar degeneration: A review
title_full Neuropathology of frontotemporal lobar degeneration: A review
title_fullStr Neuropathology of frontotemporal lobar degeneration: A review
title_full_unstemmed Neuropathology of frontotemporal lobar degeneration: A review
title_sort neuropathology of frontotemporal lobar degeneration: a review
description ABSTRACT Frontotemporal lobar degeneration (FTLD) is the second most common cause of presenile dementia. Three main clinical variants are widely recognized within the FTLD spectrum: the behavioural variant of frontotemporal dementia (bvFTD), semantic dementia (SD) and progressive non-fluent aphasia (PNFA). FTLD represents a highly heterogeneous group of neurodegenerative disorders which are best classified according to the main protein component of pathological neuronal and glial inclusions. The most common pathological class of FTLD is associated with the TDP-43 protein (FTLD-TDP), while FTLD-Tau is considered slightly less common while the FTLD-FUS (Fused in sarcoma protein) pathology is rare. In this review, these three major pathological types of FTLD are discussed.
publisher Academia Brasileira de Neurologia, Departamento de Neurologia Cognitiva e Envelhecimento
publishDate 2013
url http://old.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642013000100019
work_keys_str_mv AT bahiavaleriasantoro neuropathologyoffrontotemporallobardegenerationareview
AT takadaleoneltadao neuropathologyoffrontotemporallobardegenerationareview
AT deramecourtvincent neuropathologyoffrontotemporallobardegenerationareview
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