Differences in Sex Distribution between Genetic and Sporadic Frontotemporal Dementia

Sterre C. M. de Boer*, Lina Riedl, Sven J. van der Lee, Markus Otto, Sarah Anderl-Straub, Ramon Landin-Romero, Federica Sorrentino, Jay L. P. Fieldhouse, Lianne M. Reus, Blanca Vacaflor, Glenda Halliday, Daniela Galimberti, Janine Diehl-Schmid, Simon Ducharme, Olivier Piguet, Yolande A. L. Pijnenburg

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Background: Reported sex distributions differ between frontotemporal dementia (FTD) cohorts. Possible explanations are the evolving clinical criteria of FTD and its subtypes and the discovery of FTD causal genetic mutations that has resulted in varying demographics. Objective: Our aim was to determine the sex distribution of sporadic and genetic FTD cases and its subtypes in an international cohort. Methods: We included 910 patients with behavioral variant frontotemporal dementia (bvFTD; n = 654), non-fluent variant primary progressive aphasia (nfvPPA; n = 99), semantic variant primary progressive aphasia (svPPA; n = 117), and right temporal variant frontotemporal dementia (rtvFTD; n = 40). We compared sex distribution between genetic and sporadic FTD using χ2-tests. Results: The genetic FTD group consisted of 51.2% males, which did not differ from sporadic FTD (57.8% male, p = 0.08). In the sporadic bvFTD subgroup, males were predominant in contrast to genetic bvFTD (61.6% versus 52.9% males, p = 0.04). In the other clinical FTD subgroups, genetic cases were underrepresented and within the sporadic cases the sex distribution was somewhat equal. Conclusion: The higher male prevalence in sporadic bvFTD may provide important clues for its differential pathogenesis and warrants further research.
Original languageEnglish
Pages (from-to)1153-1161
Number of pages9
JournalJournal of Alzheimer's disease : JAD
Issue number3
Early online date4 Oct 2021
Publication statusPublished - 2021

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