Survival Estimates and Predictors in Genetic Frontotemporal Dementia: Insights from an International Retrospective Cohort Study

Highlights

  • Median survival in genetic frontotemporal dementia (FTD) averages approximately 7 years from symptom onset, with variation by genetic subtype (GRN, C9orf72, MAPT).
  • Survival heterogeneity is primarily driven by age at onset, clinical presentation—with motor symptoms portending poorer prognosis—and geographic factors rather than direct genetic group effects.
  • Development of an individual-level genetic FTD survival risk index offers potential for more accurate prognostication and patient stratification in clinical trials.
  • Motor impairment alongside cognitive and behavioural symptoms should be integrated into prognostic models to better capture disease trajectory in genetic FTD.

Background

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