The Frontotemporal Dementia Prevention Initiative: Linking Together Genetic Frontotemporal Dementia Cohort Studies

Adv Exp Med Biol. 2021:1281:113-121. doi: 10.1007/978-3-030-51140-1_8.


Around one-third of frontotemporal dementia (FTD) is autosomal dominant with the major genetic causes being mutations in MAPT, GRN and C9orf72. Studying familial forms of FTD can provide a window into the earliest stages of the illness, many years before symptoms start. Large cohort studies have been set up in recent years to better understand this presymptomatic phase, including the Genetic FTD Initiative (GENFI) and the Advancing Research and Treatment for Frontotemporal Lobar Degeneration and Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects (ARTFL/LEFFTDS) studies. Whilst these studies have focused on the investigation of a variety of aspects of genetic FTD, from understanding the molecular pathogenesis to developing biomarkers, they also have a common goal: finding a way to prevent FTD. Researchers from these cohort studies have therefore come together to form the FTD Prevention Initiative (FPI), which has the overarching aim of promoting clinical trials of new therapies to prevent FTD through creating an international database of participants eligible for trials and uniform standards for conducting such trials. This chapter outlines the work of the FPI so far and its future goals over the next few years.

Keywords: C9orf72; Clinical trials; Frontotemporal dementia; Genetics; Progranulin; Tau.

MeSH terms

  • C9orf72 Protein / genetics
  • Cohort Studies
  • Frontotemporal Dementia* / genetics
  • Frontotemporal Dementia* / prevention & control
  • Humans
  • Mutation
  • Pick Disease of the Brain*
  • tau Proteins / genetics


  • C9orf72 Protein
  • C9orf72 protein, human
  • tau Proteins