Advanced search
Start date
Betweenand


Extending the neuropathological spectrum of frontotemporal lobar degenerations: review of 833 prospectively assessed dementia cases

Full text
Author(s):
Lea Tenenholz Grinberg
Total Authors: 1
Document type: Doctoral Thesis
Press: São Paulo.
Institution: Universidade de São Paulo (USP). Faculdade de Medicina (FM/SBD)
Defense date:
Examining board members:
Wilson Jacob Filho; Gyorgy Myklós Bohm; Leila Maria Cardão Chimelli; Carmen Lucia Penteado Lancellotti; Ricardo Nitrini
Advisor: Wilson Jacob Filho
Abstract

Frontotemporal dementia (FTD) encompasses two clinical phenotypes: progressive behavioral change and language disorder. Collectively, FTD may be caused by a diverse group of neurodegenerative diseases called frontotemporal lobar degenerations (FTLDs). New entities have been described and the nosology of FTLDs continues to evolve. To determine the type and frequency of FTLDs in a series using contemporary immunohistochemical methods. Eight hundred and thirtythree dementia cases were prospectively assessed at Washington University Alzheimer Disease Research Center (WUADRC) and cases with clinical FTD were identified using existing diagnostic criteria and neuropathologic entities were ascertained using immunohistochemistry and contemporary diagnostic criteria. Of the dementia cases, 53(6.3%) met clinical criteria for FTD; 45(5.1%) fulfilled both clinical and neuropathological criteria for FTLD, and another 8 fulfilled only the clinical criteria. Forty percent of the cases were tauopathies. However, most FTLD cases were characterized by ubiquitin-positive, tau-negative inclusions. Co-existing hippocampal sclerosis and AD-type changes were observed in 12 and 10 cases, respectively. Although FTLD-MND-type is the most frequent FTLD in this prospectively assessed series, less common entities not included in the McKhann criteria, may also present clinically as FTD and should be considered as part of the neuropathologic spectrum of FTLDs that may be encountered in the dementia clinic. The better understanding of the cell death mechanisms related to those entities is likely to contribute for the development of a treatment for FTLD as well for a way of modulate brain aging. (AU)

FAPESP's process: 04/13838-3 - Pathological correlation of the association between dementia and behavioral changes in the brain during aging: a stereological study of autopsies
Grantee:Lea Tenenholz Grinberg
Support Opportunities: Scholarships in Brazil - Doctorate (Direct)