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Title
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Combined Pathologies in FTLD-TDP Types A and C
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Author
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Abstract
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| This study investigated the presence of combined pathologies in a large cohort of autopsies that show a primary pathologic diagnosis of phosphorylated 43-kDa TAR DNA-binding protein (FTLD-TDP), the majority of which portrayed clinical phenotypes consistent with primary progressive aphasia or behavioral variant frontotemporal dementia (bvFTD). Thirty-eight cases with FTLD-TDP (30 type-A and 8 type-C) were identified to determine characteristic differences between cases with and without combined pathologies. Findings indicated that combined pathologies co-occur with FTLD-TDP type-A at a high frequency (50%)-greater than when compared to FTLD-TDP type-C cases (12.5%). Those with FTLD-TDP type-A and combined pathologies showed significantly longer lifespans (p<0.05), and longer disease durations (p<0.05), than those with only FTLD-TDP type-A. Cases with FTLD-TDP type-A and known genetic mutations tended not to show combined pathology. Those with the GRN mutation and FTLD-TDP type-A showed a significantly younger age of onset (p<0.05) and younger age at death (p<0.01) compared to noncarriers. In 1 bvFTD case, we highlight the rare presence of "triple" FTLD-TDP type-A, FTLD-tau, and Alzheimer pathology. The ante-and post-mortem features associated with combined pathologies in FTLD-related disorders are of useful consideration in the stratification of patients to drug trials, and in the development of therapeutic targets for FTLD. |
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Language
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English
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Source (journal)
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Journal of neuropathology and experimental neurology. - New York, N.Y.
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Publication
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New York, N.Y.
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2018
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ISSN
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0022-3069
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DOI
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10.1093/JNEN/NLY018
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Volume/pages
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77
:5
(2018)
, p. 405-412
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ISI
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000432259700007
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Full text (Publisher's DOI)
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