Clinical and volumetric changes with increasing functional impairment in familial frontotemporal lobar degeneration.

ABSTRACT:

Introduction

The Advancing Research and Treatment in Frontotemporal Lobar Degeneration and Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects longitudinal studies were designed to describe the natural history of familial-frontotemporal lobar degeneration due to autosomal dominant mutations.

Methods

We examined cognitive performance, behavioral ratings, and brain volumes from the first time point in 320 MAPT, GRN, and C9orf72 family members, including 102 non-mutation carriers, 103 asymptomatic carriers, 43 mildly/questionably symptomatic carriers, and 72 carriers with dementia.

Results

Asymptomatic carriers showed similar scores on all clinical measures compared with noncarriers but reduced frontal and temporal volumes. Those with mild/questionable i

SUBMITTER: Olney NT 

PROVIDER: S-EPMC6988137 | biostudies-literature | 2020 Jan

REPOSITORIES: biostudies-literature