Low Prevalence of Cancer in Patients withFrontotemporal Lobar Degeneration.

Kasper Katisko,Anne M Remes,Anne Koivisto,Päivi Hartikainen,Ville Korhonen,Seppo Helisalmi,Johanna Krüger,Annakaisa Haapasalo,Sanna-Kaisa Herukka,Eino Solje,Luisa Benussi

doi:10.3233/jad-170854

Abstract

Several studies have reported reduced risk of cancer in patients with Alzheimer's disease (AD) or Parkinson's disease. The relationship between cancer and frontotemporal lobar degeneration (FTLD) has not been previously reported. Here, our aim was to evaluate the occurrence of cancer in Finnish FTLD patients with a high proportion of C9ORF72 repeat expansion carriers in comparison to age- and sex-matched group of AD patients and control subjects classified as not cognitively impaired (NCI). The prevalence of cancer was 9.7% in FTLD, 18.7% in AD, and 17.4% in NCI (FTLD versus AD p = 0.012, FTLD versus NCI p = 0.029) groups. No differences were observed between C9ORF72 repeat expansion carriers and non-carriers inside the FTLD group. To our knowledge, this is the first study showing significantly lower prevalence of cancer in FTLD patients compared to patients with AD or NCI subjects. Our data suggest an inverse association between neurodegeneration and cancer and that FTLD-specific mechanisms may underlie the especially strong inverse association observed in this study.

Highlights

Frontotemporal lobar degeneration (FTLD) is a clinically and pathologically heterogeneous group of progressive neurodegenerative diseases that can be divided into four main clinical subtypes
No differences were observed within the FTLD group depending on the chromosome 9 open reading frame 72 (C9ORF72) repeat expansion status (p = 0.789) or between the Alzheimer’s disease (AD) and not cognitively impaired (NCI) groups (p = 0.790, Chi-square)
We found a significantly lower prevalence of cancer in FTLD patients as compared to AD patients or NCI subjects

Summary

Introduction

Frontotemporal lobar degeneration (FTLD) is a clinically and pathologically heterogeneous group of progressive neurodegenerative diseases that can be divided into four main clinical subtypes. These include behavioral variant of frontotemporal dementia (bvFTD) [1], nonfluent variant of primary progressive aphasia (nfvPPA), semantic variant of primary progressive aphasia (svPPA), and logopenic variant of primary progressive aphasia (lvPPA) [2]. In Finland, the C9ORF72 repeat expansion is the underlying cause for a great majority of familial cases [3], whereas other known mutations causing FTLD are extremely rare [4,5,6]. Specific risk factors for FTLD have not yet been identified

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