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Tracking disease progression in familial and sporadic frontotemporal lobar degeneration: Recent findings from ARTFL and LEFFTDS
Author(s) -
Rosen Howard J.,
Boeve Bradley F.,
Boxer Adam L.
Publication year - 2020
Publication title -
alzheimer's and dementia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 6.713
H-Index - 118
eISSN - 1552-5279
pISSN - 1552-5260
DOI - 10.1002/alz.12004
Subject(s) - frontotemporal lobar degeneration , natural history , frontotemporal dementia , disease , neurodegeneration , medicine , natural history study , asymptomatic , dementia , pathology , psychology
Abstract Introduction Familial frontotemporal lobar degeneration (f‐FTLD) due to autosomal dominant mutations is an important entity for developing treatments for FTLD. The Advancing Research and Treatment for Frontotemporal Lobar Degeneration (ARTFL) and Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects (LEFFTDS) longitudinal studies were designed to describe the natural history of f‐FTLD. Methods We summarized recent publications from the ARTFL and LEFFTDS studies, along with other recent publications describing the natural history of f‐FTLD. Results Published and emerging studies are producing data on all phases of f‐FTLD, including the asymptomatic and symptomatic phases of disease, as well as the transitional phase when symptoms are just beginning to develop. These data indicate that rates of change increase along with disease severity, which is consistent with commonly cited models of neurodegeneration, and that measurement of biomarkers may predict onset of symptoms. Discussion Data from large multisite studies are producing important data on the natural history of f‐FTLD that will be critical for planning intervention trials.