The patterns of inheritance in early-onset dementia: Alzheimer's disease and frontotemporal dementia

Am J Alzheimers Dis Other Demen. 2015 May;30(3):299-306. doi: 10.1177/1533317514545825. Epub 2014 Aug 21.

Abstract

Aim: To investigate the patterns of inheritance and gene mutation status in early-onset dementia (EOD).

Methods: Data were collected on 202 consecutive patients presenting to an EOD clinic. Early-onset Alzheimer's disease (EOAD, n = 120) and early-onset frontotemporal dementia (EOFTD, n = 82) were studied.

Results: The majority of participants, 72.5% with EOAD and 74.4% with EOFTD, did not have a positive family history of dementia. An autosomal dominant pattern of inheritance was observed in 14.2% of patients with EOAD and 13.4% of patients with FTD. Of those with an autosomal dominant pattern of inheritance, 11.8% of EOAD and 45.5% of FTD probands had known pathogenic mutations. Only 1.6% of the total population of EOAD and 7.3% of EOFTD possessed known gene mutations.

Conclusion: Early-onset dementia does not appear to be a strongly inherited autosomal dominant condition. The majority of patients were sporadic. Known mutations were uncommon and do not explain the total autosomal dominant burden.

Keywords: Alzheimer’s disease; early onset dementia; family history; frontotemporal dementia; genes and dementia.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Age of Onset
  • Aged
  • Alzheimer Disease / genetics*
  • Cohort Studies
  • Female
  • Frontotemporal Dementia / genetics*
  • Genetic Predisposition to Disease
  • Humans
  • Male
  • Middle Aged