Neuropathological evidence of body-first vs. brain-first Lewy body disease

Aggregation of alpha-synuclein into inclusion bodies, termed Lewy pathology, is a defining feature of Parkinson's disease (PD) and Dementia with Lewy bodies (DLB). In the majority of post mortem cases, the distribution of Lewy pathology seems to follow two overarching patterns: a caudo-rostral...

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Main Authors: Per Borghammer, Jacob Horsager, Katrine Andersen, Nathalie Van Den Berge, Anna Raunio, Shigeo Murayama, Laura Parkkinen, Liisa Myllykangas
Format: Article
Language:English
Published: Elsevier 2021-12-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996121003065
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author Per Borghammer
Jacob Horsager
Katrine Andersen
Nathalie Van Den Berge
Anna Raunio
Shigeo Murayama
Laura Parkkinen
Liisa Myllykangas
author_facet Per Borghammer
Jacob Horsager
Katrine Andersen
Nathalie Van Den Berge
Anna Raunio
Shigeo Murayama
Laura Parkkinen
Liisa Myllykangas
author_sort Per Borghammer
collection DOAJ
description Aggregation of alpha-synuclein into inclusion bodies, termed Lewy pathology, is a defining feature of Parkinson's disease (PD) and Dementia with Lewy bodies (DLB). In the majority of post mortem cases, the distribution of Lewy pathology seems to follow two overarching patterns: a caudo-rostral pattern with relatively more pathology in the brainstem than in the telencephalon, and an amygdala-centered pattern with the most abundant pathology in the “center of the brain”, including the amygdala, entorhinal cortex, and substantia nigra, and relatively less pathology in the lower brainstem and spinal autonomic nuclei. The recent body-first versus brain-first model of Lewy Body Disorders proposes that the initial pathogenic alpha-synuclein in some patients originates in the enteric nervous system with secondary spreading to the brain; and in other patients originates inside the CNS with secondary spreading to the lower brainstem and peripheral autonomic nervous system. Here, we use two existing post mortem datasets to explore the possibility that clinical body-first and brain-first subtypes are equivalent to the caudo-rostral and amygdala-centered patterns of Lewy pathology seen at post mortem.
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spelling doaj.art-e724fb4b6980453890175ae589978beb2022-12-21T23:11:52ZengElsevierNeurobiology of Disease1095-953X2021-12-01161105557Neuropathological evidence of body-first vs. brain-first Lewy body diseasePer Borghammer0Jacob Horsager1Katrine Andersen2Nathalie Van Den Berge3Anna Raunio4Shigeo Murayama5Laura Parkkinen6Liisa Myllykangas7Institute of Clinical Medicine, Aarhus University, Aarhus, Denmark; Corresponding author at: Department of Nuclear Medicine & PET, Institute of Clinical Medicine, Aarhus University Hospital, Aarhus, Denmark.Institute of Clinical Medicine, Aarhus University, Aarhus, DenmarkInstitute of Clinical Medicine, Aarhus University, Aarhus, DenmarkInstitute of Clinical Medicine, Aarhus University, Aarhus, DenmarkDepartment of Pathology, University of Helsinki, HUS Diagnostic Center, Helsinki University Hospital, Helsinki, FinlandBrain Bank for Aging Research, Tokyo Metropolitan Geriatric Hospital, Institute of Gerontology, Tokyo, JapanNuffield Department of Clinical Neurosciences, Oxford Parkinson's Disease Centre, University of Oxford, United KingdomDepartment of Pathology, University of Helsinki, HUS Diagnostic Center, Helsinki University Hospital, Helsinki, FinlandAggregation of alpha-synuclein into inclusion bodies, termed Lewy pathology, is a defining feature of Parkinson's disease (PD) and Dementia with Lewy bodies (DLB). In the majority of post mortem cases, the distribution of Lewy pathology seems to follow two overarching patterns: a caudo-rostral pattern with relatively more pathology in the brainstem than in the telencephalon, and an amygdala-centered pattern with the most abundant pathology in the “center of the brain”, including the amygdala, entorhinal cortex, and substantia nigra, and relatively less pathology in the lower brainstem and spinal autonomic nuclei. The recent body-first versus brain-first model of Lewy Body Disorders proposes that the initial pathogenic alpha-synuclein in some patients originates in the enteric nervous system with secondary spreading to the brain; and in other patients originates inside the CNS with secondary spreading to the lower brainstem and peripheral autonomic nervous system. Here, we use two existing post mortem datasets to explore the possibility that clinical body-first and brain-first subtypes are equivalent to the caudo-rostral and amygdala-centered patterns of Lewy pathology seen at post mortem.http://www.sciencedirect.com/science/article/pii/S0969996121003065Lewy bodyParkinson's diseaseAlpha-synucleinDementia with Lewy bodies
spellingShingle Per Borghammer
Jacob Horsager
Katrine Andersen
Nathalie Van Den Berge
Anna Raunio
Shigeo Murayama
Laura Parkkinen
Liisa Myllykangas
Neuropathological evidence of body-first vs. brain-first Lewy body disease
Neurobiology of Disease
Lewy body
Parkinson's disease
Alpha-synuclein
Dementia with Lewy bodies
title Neuropathological evidence of body-first vs. brain-first Lewy body disease
title_full Neuropathological evidence of body-first vs. brain-first Lewy body disease
title_fullStr Neuropathological evidence of body-first vs. brain-first Lewy body disease
title_full_unstemmed Neuropathological evidence of body-first vs. brain-first Lewy body disease
title_short Neuropathological evidence of body-first vs. brain-first Lewy body disease
title_sort neuropathological evidence of body first vs brain first lewy body disease
topic Lewy body
Parkinson's disease
Alpha-synuclein
Dementia with Lewy bodies
url http://www.sciencedirect.com/science/article/pii/S0969996121003065
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