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While diagnostic criteria have been established and validated for most neurodegenerative diseases, the considerable overlap between individual nosological entities remains a significant diagnostic challenge. Increasing evidence suggests that neurodegeneration is often initiated by inflammation within the central nervous system. The identification of inflammation could serve as a first signal of the pathophysiological process. As such, validated biological markers ("biomarkers") of neuroinflammation are critically important. This study aimed to assess the presence and levels of inflammatory biomarkers in three neurodegenerative diseases: Lewy body diseases (LBD), multiple system atrophy (MSA), and 4-repeat tauopathies (4RT). A total of 83 LBD, 24 MSA, and 31 4RT patients were included, with 83 control subjects for comparison. Six immune-related proteins were analysed in cerebrospinal fluid (CSF) and blood serum (serum): C3 complement, C4 complement, haptoglobin, transferrin, orosomucoid, and β2 microglobulin (β2M). ANCOVA statistical analysis revealed significantly lower levels of several inflammatory biomarkers in LBD (CSF: transferrin, C3 complement, orosomucoid; Serum: orosomucoid, β2M) and MSA (CSF: transferrin, C3 complement, C4 complement, orosomucoid) compared to controls. Significant differences were also observed between the synucleinopathy patient groups (LBD and MSA) and 4RT in serum levels of C3 complement. Additionally, the CSF/serum quotients for transferrin (LBD and MSA) and C3 complement (LBD) were significantly lower in disease relative to controls. These findings suggest that inflammatory processes may play a role in the pathophysiology of neurodegenerative proteinopathies, warranting further research to confirm these associations. The identification of potential fluid biomarkers would then represent a promising step forward in the field.
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http://dx.doi.org/10.1007/s00702-025-02902-6 | DOI Listing |
bioRxiv
August 2025
Department of Microbiology, Immunology, & Pathology and Prion Research Center, Colorado State University, Fort Collins, CO, USA.
In synucleinopathies, the protein α-synuclein misfolds into Lewy bodies (LBs) in patients with Lewy body disease (LBD) or into glial cytoplasmic inclusions (GCIs) in patients with multiple system atrophy (MSA). The ability of a single misfolded protein to cause disparate diseases is explained by the prion strain hypothesis, which argues that protein conformation is a major determinant of disease. While structural, biochemical, and biological studies show that LBD and MSA patient samples contain distinct α-synuclein strains, we recently reported the unexpected finding of a novel α-synuclein strain in a Parkinson's disease with dementia patient sample containing GCI-like co-pathology along with widespread LB pathology.
View Article and Find Full Text PDFActa Neuropathol
August 2025
Neurodegenerative Diseases Research Section, National Institute of Neurological Disorders and Stroke, Bethesda, MD, 20892-3707, USA.
Mov Disord
August 2025
IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.
Background: Seed amplification assays (SAAs) for misfolded α-synuclein (syn) have shown inconsistent results in multiple system atrophy (MSA).
Objective: The objective of this study was to compare a novel syn SAA (synSAA) that distinguishes between Lewy body disease (LBD) and MSA syn-seeds (Amprion-SAA) with an LBD-specific synSAA (IRCCS Istituto delle Scienze Neurologiche di Bologna [ISNB]-SAA).
Methods: We applied both assays to cerebrospinal fluid samples from 114 patients with MSA, 49 patients with Parkinson disease (PD), 40 patients with progressive supranuclear palsy (PSP), and 46 controls.
Acta Neuropathol Commun
August 2025
Department of Microbiology, Immunology, and Pathology, Prion Research Center, Colorado State University, 300 West Lake Street, Fort Collins, CO, 80521, USA.
Multiple system atrophy (MSA) and the Lewy body diseases (LBDs) are caused by α-synuclein misfolding into distinct conformations, or strains, with unique biological properties. MSA patient samples readily transmit disease following intracranial (i.c.
View Article and Find Full Text PDFAnn Clin Transl Neurol
July 2025
Autonomic Unit, National Hospital for Neurology and Neurosurgery, London, UK.
Background: Pure autonomic failure (PAF) presents with autonomic failure without other neurological features. A third develop central neurological features, fulfilling criteria for multiple system atrophy (MSA) and Lewy body diseases (LBD), including Parkinson's disease and Dementia with Lewy bodies. We hypothesized multimodal autonomic biomarkers would identify differences between PAF, MSA, and LBD, and predict phenoconversion in patients presenting with PAF.
View Article and Find Full Text PDF