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01-09-2014 | Original Paper

Differential clinicopathologic and genetic features of late-onset amnestic dementias

Authors: Melissa E. Murray, Ashley Cannon, Neill R. Graff-Radford, Amanda M. Liesinger, Nicola J. Rutherford, Owen A. Ross, Ranjan Duara, Minerva M. Carrasquillo, Rosa Rademakers, Dennis W. Dickson

Published in: Acta Neuropathologica | Issue 3/2014

Abstract

Hippocampal sclerosis of the elderly (HpScl) and Alzheimer’s disease (AD), especially the limbic-predominant subtype (LP-AD), are amnestic syndromes that can be difficult to distinguish. To complicate matters, a subset has concomitant HpScl and AD (HpScl-AD). We examined a large cohort of autopsy-confirmed cases of HpScl, HpScl-AD, LP-AD, and typical AD to identify distinct clinical, genetic, and pathologic characteristics. HpScl cases were significantly older at death and had a substantially slower rate of cognitive decline than the AD subtypes. Genetic analysis revealed that the AD groups (AD, LP-AD, and HpScl–AD) were more likely to be APOE ε4 carriers. In contrast, the HpScl groups (HpScl and HpScl-AD) were more likely to exhibit genetic variants in GRN and TMEM106B that are associated with frontotemporal lobar degeneration. The HpScl groups had a high frequency of TDP-43 pathology that was most often Type A morphology and distribution, while typical AD and LP-AD had a significantly lower frequency of TDP-43 pathology that was most often Type B. These results suggest that HpScl and AD are pathologically and genetically distinct and non-synergistic neurodegenerative processes that present with amnestic dementia. Pure HpScl and HpScl with concomitant AD occur most often in elderly individuals.

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Title: Differential clinicopathologic and genetic features of late-onset amnestic dementias
Authors: Melissa E. Murray
Ashley Cannon
Neill R. Graff-Radford
Amanda M. Liesinger
Nicola J. Rutherford
Owen A. Ross
Ranjan Duara
Minerva M. Carrasquillo
Rosa Rademakers
Dennis W. Dickson
Publication date: 01-09-2014
Publisher: Springer Berlin Heidelberg
Published in: Acta Neuropathologica / Issue 3/2014
Print ISSN: 0001-6322
Electronic ISSN: 1432-0533
DOI: https://doi.org/10.1007/s00401-014-1302-2

2024 ASCO Annual Meeting

Springer Medicine

Differential clinicopathologic and genetic features of late-onset amnestic dementias

Abstract

2024 ASCO Annual Meeting

Springer Medicine

Abstract

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