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Cited by 48Dementia and Cognitive Impairment ResearchAmyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatments

Neuropathology of frontotemporal lobar degeneration: A review

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Bahia, Valéria Santoro, Takada, Leonel Tadao, Deramecourt, Vincent·2013·Dementia & Neuropsychologia
DOI 10.1590/s1980-57642013dn70100004PMC PMC5619540
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Abstract

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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Authors
Bahia, V. S., Takada, L. T., & Deramecourt, V.
Affiliations
  • Universidade de São Paulo (BR)
  • Université Lille Nord de France (FR)
Corresponding author
Bahia, Valéria Santoro
ORCID
Venue
Dementia & Neuropsychologia
Publisher
FapUNIFESP (SciELO)
Published
2013-03
Volume
7
Issue
1
Pages
19-26
ISSN
1980-5764 (ISSN-L)
DOI
10.1590/s1980-57642013dn70100004
PMC
PMC5619540
OpenAlex
W1562489172
Cited by
48
References
79
Citations by year
2026: 5 · 2025: 7 · 2024: 12 · 2023: 3 · 2022: 3 · 2021: 5 · 2020: 7 · 2019: 3 · 2018: 1 · 2016: 1
FWCI
0.05
Type
journal article
Language
en
Open access
Licence
cc-by
Locations
  • Dementia & Neuropsychologia (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • LA Referencia (Red Federada de Repositorios Institucionales de Publicaciones Científicas) (publishedVersion, other-oa)
  • Americanae (AECID Library) (submittedVersion, other-oa)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
  • Redalyc (Universidad Autónoma del Estado de México) (submittedVersion)
Topics
  • Health Sciences › Medicine › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
Keywords
frontotemporal lobar degeneration, behavioral variant frontotemporal dementia, semantic dementia, progressive nonfluent aphasia, TDP-43, FTLD-tau, FUS, neurodegeneration, proteinopathies, glial inclusions, early-onset dementia

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