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. 2022;12(4):1125-1131.
doi: 10.3233/JPD-223161.

Cardiac Alpha-Synuclein Is Present in Alpha-Synucleinopathies

Keivan Javanshiri  1 , Tove Drakenberg  1 , Mattias Haglund  1 , Elisabet Englund  1
Affiliations

Cardiac Alpha-Synuclein Is Present in Alpha-Synucleinopathies

Keivan Javanshiri et al. J Parkinsons Dis. 2022.

Abstract

Background: Alpha-synucleinopathies (AS) are characterized by pathologic aggregations of alpha-synuclein (α-syn) in the central nervous system, and comprise dementia with Lewy bodies, Parkinson's disease, and multiple system atrophy. Previous studies on AS have reported findings of α-syn pathology in the peripheral nervous system of multiple organs, including the heart.

Objective: The aim of this study was to further investigate and confirm the presence of cardiac α-syn in AS compared to other major neurocognitive disorders in a neuropathologically confirmed cohort.

Methods: All deceased patients with performed autopsy and with neuropathologically confirmed AS at the Clinical Department of Pathology in Lund 2010-May 2021 were evaluated for inclusion. Cases with insufficiently sampled cardiac tissue or only limited neuropathological investigation were excluded. An age-matched group of individuals with other neurodegenerative diseases, having no α-syn in the CNS, served as controls. In total, 68 AS and 32 control cases were included in the study. Immunohistochemistry for detection of cardiac α-syn aggregates was performed.

Results: The AS group had a significantly higher prevalence of cardiac α-syn pathology (p≤0.001) than the control group, 82% and 0%, respectively.

Conclusion: This study confirms the association between AS and the presence of cardiac α-syn in a neuropathologically confirmed cohort. This motivates further research on potential pathophysiological effects on cardiac function in AS patients.

Keywords: Alpha-synuclein; Lewy body disease; Parkinson’s disease; cardiac nerve fibers; multiple system atrophy; synucleinopathies.

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Conflict of interest statement

MH is a former employee of Biogen and Eli Lilly but has no active financial interest in either company. The other authors have no conflict of interest to report.

Figures

Fig. 1
Fig. 1
Cardiac (epicardial) nerve strongly positive for alpha-synuclein pathology, with Lewy bodies present.
Fig. 2
Fig. 2
Cardiac nerve weakly positive for alpha-synuclein pathology.
Fig. 3
Fig. 3
Cardiac Lewy bodies, strong and focal positivity.
Fig. 4
Fig. 4
Cardiac nerve negative for alpha-synuclein pathology with questionable/minimal findings.
See this image and copyright information in PMC

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