Right temporal variant frontotemporal dementia is pathologically heterogeneous: a case-series and a systematic review.

ABSTRACT: Although the right temporal variant frontotemporal dementia (rtvFTD) is characterised by distinct clinical and radiological features, its underlying histopathology remains elusive. Being considered a right-sided variant of semantic variant primary progressive aphasia (svPPA), TDP-43 type C pathology has been linked to the syndrome, but this has not been studied in detail in large cohorts. In this case report and systematic review, we report the autopsy results of five subjects diagnosed with rtvFTD from our cohort and 44 single rtvFTD subjects from the literature. Macroscopic pathological evaluation of the combined results revealed that rtvFTD demonstrated either a frontotemporal or temporal evolution, even if the degeneration started in the right temporal lobe initially. FTLD-TDP type C w

SUBMITTER: Ulugut H 

PROVIDER: S-EPMC8330072 | biostudies-literature | 2021 Aug

REPOSITORIES: biostudies-literature