Overactive immune system, increased inflammation are seen in a subgroup of patients with frontotemporal dementia (FTD), according to the recent research from the University of Eastern Finland. Increased inflammation was associated with Parkinsonism symptoms and few autoimmune diseases may be more common among these patients. FTD is the second most common cause for early onset dementia. Currently, the precise mechanisms causing the disease are unknown, and there are no disease modifying or curative treatments. The most common genetic cause of FTD, the C9orf72 repeat expansion mutation, has been associated with immune system regulation.
‘Immune system alterations are responsible for pathogenesis and clinical features of FTD. This information could be used in designing future studies and for identifying new biomarkers or therapeutic strategies.’
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The association between systemic inflammation and clinical features of FTD was evaluated by analysing several inflammatory markers such as cytokines and C-reactive protein (CRP) from FTD patients’ blood samples.
The aim was to analyse whether the potential systemic inflammatory changes associate with specific features under the heterogeneous FTD spectrum, including psychotic symptoms, parkinsonism and disease progression.
Increased inflammation in blood, which was indicated by elevated levels of cytokines that promote inflammation (MCP-1 and RANTES) and decreased levels of cytokine that reduces inflammation (IL-10), were associated with parkinsonism symptoms and more rapid cognitive and functional decline. These findings were recently published in Journal of Neurology.
Cancer is rare among FTD patients
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Previous genetic and immunological studies have indicated that this inverse association may be explained by the opposite genetic activities of cancer and degeneration, and additionally by opposite immunological pathways.
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The prevalence of autoimmune diseases in general was not significantly higher in FTD compared to control groups. On the other hand, especially FTD patients with the C9orf72 repeat expansion mutation had potential association with an autoimmune skin disease bullous pemphigoid, which was indicated by elevated autoantibody levels in 12.5% of the patients.
Previous studies have indicated that autoimmune diseases are more common in FTD in general, but based on these results, the association likely differs between different autoimmune diseases and FTD genotypes.
"In all, this research provides novel insights into the potential contribution of immune system alterations to the pathogenesis and clinical features of FTD. This new information may be utilised in designing further studies and for identifying novel prognostic biomarkers or therapeutic strategies in FTD," says Kasper Katisko, B.M., from the University of Eastern Finland, who presented the findings in a doctoral thesis focusing on the role of inflammation and immune system function in frontotemporal dementia.
Source-Eurekalert