The Right Temporal Variant of Frontotemporal Dementia Is Not Genetically Sporadic: A Case Series.

ABSTRACT:

Background

Right temporal variant frontotemporal dementia (rtvFTD) has been generally considered as a right sided variant of semantic variant primary progressive aphasia (svPPA), which is a genetically sporadic disorder. Recently, we have shown that rtvFTD has a unique clinical syndrome compared to svPPA and behavioral variant frontotemporal dementia.

Objective

We challenge the assumption that rtvFTD is a sporadic, non-familial variant of FTD by identifying potential autosomal dominant inheritance and related genes in rtvFTD.

Methods

We collected all subjects with a diagnosis of FTD or primary progressive aphasia who had undergone genetic screening (n = 284) and subsequently who had a genetic variant (n = 48) with a diagnosis of rtvFTD (n = 6) in 2 specialized memory

SUBMITTER: Ulugut Erkoyun H 

PROVIDER: S-EPMC7990443 | biostudies-literature | 2021

REPOSITORIES: biostudies-literature