University of Oulu

Katisko, K., Solje, E., Korhonen, P. et al. Peripheral inflammatory markers and clinical correlations in patients with frontotemporal lobar degeneration with and without the C9orf72 repeat expansion. J Neurol 267, 76–86 (2020).

Peripheral inflammatory markers and clinical correlations in patients with frontotemporal lobar degeneration with and without the C9orf72 repeat expansion

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Author: Katisko, Kasper1; Solje, Eino1,2; Korhonen, Paula3;
Organizations: 1Institute of Clinical Medicine-Neurology, University of Eastern Finland, Kuopio, Finland
2Neuro Center, Neurology, Kuopio University Hospital, Kuopio, Finland
3A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Neulaniementie 2, 70211, Kuopio, Finland
4Neuro Center, Neurosurgery, Kuopio University Hospital, 70029, Kuopio, Finland
5MRC Oulu, Oulu University Hospital, Oulu, Finland
6Research Unit of Clinical Neuroscience, Neurology, University of Oulu, Oulu, Finland
Format: article
Version: published version
Access: open
Online Access: PDF Full Text (PDF, 2.4 MB)
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Language: English
Published: Springer Nature, 2020
Publish Date: 2020-04-27


In this study, our aim was to evaluate potential peripheral inflammatory changes in frontotemporal lobar degeneration (FTLD) patients carrying or not the C9orf72 repeat expansion. To this end, levels of several inflammatory markers (MCP-1, RANTES, IL-10, IL-17A, IL-12p, IFN-γ, IL-1β, IL-8, and hs-CRP) and blood cells counts in plasma and/or serum of FTLD patients (N = 98) with or without the C9orf72 repeat expansion were analyzed. In addition, we evaluated whether the analyzed peripheral inflammatory markers correlated with disease progression or distinct clinical phenotypes under the heterogenous FTLD spectrum. Elevated levels of pro-inflammatory RANTES or MCP-1 and decreased levels of anti-inflammatory IL-10 were found to associate with Parkinsonism and a more rapid disease progression, indicated by longitudinal measurements of either MMSE or ADCS-ADL decline. These findings were observed in the total cohort in general, whereas the C9orf72 repeat expansion carriers showed only slight differences in IL-10 and hemoglobin levels compared to non-carriers. Furthermore, these C9orf72 repeat expansion-associated differences were observed mostly in male subjects. The females in general showed elevated levels of several pro-inflammatory markers compared to males regardless of the C9orf72 genotype. Our study suggests that pro-inflammatory changes observed in the early symptomatic phase of FTLD are associated with distinct clinical profiles and a more rapid disease progression, and that the C9orf72 repeat expansion and gender may also affect the inflammatory profile in FTLD.

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Series: Journal of neurology
ISSN: 0340-5354
ISSN-E: 1432-1459
ISSN-L: 0340-5354
Volume: 267
Pages: 76 - 86
DOI: 10.1007/s00415-019-09552-1
Type of Publication: A1 Journal article – refereed
Field of Science: 3112 Neurosciences
Funding: Open access funding provided by University of Eastern Finland (UEF) including Kuopio University Hospital. This study was supported by the Academy of Finland (Grant nos. 315459, 315460), VTR Grant 5772816 of Kuopio University Hospital, Finnish Brain Foundation, Finnish Medical Foundation, Päivikki and Sakari Sohlberg Foundation, Finnish Alzheimer’s Disease Research Society, Maire Taponen Foundation, Finnish Cultural Foundation, OLVI-foundation, Maud Kuistila Memorial Foundation and Yrjö Jahnsson Foundation (Grant no. 20187070).
Academy of Finland Grant Number: 315460
Detailed Information: 315460 (Academy of Finland Funding decision)
Copyright information: © The Author(s) 2019. Open Access. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (, which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.