Tracking disease progression in familial and sporadic frontotemporal lobar degeneration: Recent findings from ARTFL and LEFFTDS

Howard J. Rosen, Bradley F. Boeve, Adam L. Boxer

Research output: Contribution to journalReview articlepeer-review

10 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)71-78
Number of pages8
JournalAlzheimer's and Dementia
Volume16
Issue number1
DOIs
StatePublished - Jan 1 2020

Keywords

  • C9orf72
  • GRN
  • MAPT
  • familial
  • frontotemporal lobar degeneration
  • genetic

ASJC Scopus subject areas

  • Epidemiology
  • Health Policy
  • Developmental Neuroscience
  • Clinical Neurology
  • Geriatrics and Gerontology
  • Psychiatry and Mental health
  • Cellular and Molecular Neuroscience

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