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. 2015 May;30(3):299-306.
doi: 10.1177/1533317514545825. Epub 2014 Aug 21.

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

Anna I Jarmolowicz  1 , Huei-Yang Chen  1 , Peter K Panegyres  2
Affiliations

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

Anna I Jarmolowicz et al. Am J Alzheimers Dis Other Demen. 2015 May.

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.

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Conflict of interest statement

The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Figures

Figure 1.
Figure 1.
Project design: Familial Dementia Study (FADS).
Figure 2.
Figure 2.
The patterns of inheritance in early-onset dementia.
See this image and copyright information in PMC

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