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...
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Associação Neurologia Cognitiva e do Comportamento
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doaj-5bbdf40b611749cd931d7d76e55395152020-11-24T23:32:22ZengAssociação Neurologia Cognitiva e do ComportamentoDementia & Neuropsychologia1980-576471192610.1590/S1980-57642013DN70100004S1980-57642013000100019Neuropathology of frontotemporal lobar degeneration: A reviewValéria Santoro BahiaLeonel Tadao TakadaVincent DeramecourtABSTRACT 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.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642013000100019&lng=en&tlng=enfrontotemporal lobar degenerationpathologyTAUTDPFUS |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Valéria Santoro Bahia Leonel Tadao Takada Vincent Deramecourt |
spellingShingle |
Valéria Santoro Bahia Leonel Tadao Takada Vincent Deramecourt Neuropathology of frontotemporal lobar degeneration: A review Dementia & Neuropsychologia frontotemporal lobar degeneration pathology TAU TDP FUS |
author_facet |
Valéria Santoro Bahia Leonel Tadao Takada Vincent Deramecourt |
author_sort |
Valéria Santoro Bahia |
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 |
publisher |
Associação Neurologia Cognitiva e do Comportamento |
series |
Dementia & Neuropsychologia |
issn |
1980-5764 |
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. |
topic |
frontotemporal lobar degeneration pathology TAU TDP FUS |
url |
http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1980-57642013000100019&lng=en&tlng=en |
work_keys_str_mv |
AT valeriasantorobahia neuropathologyoffrontotemporallobardegenerationareview AT leoneltadaotakada neuropathologyoffrontotemporallobardegenerationareview AT vincentderamecourt neuropathologyoffrontotemporallobardegenerationareview |
_version_ |
1725534501052874752 |