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Pathological changes in the cerebellum of patients with Multiple System Atrophy and Parkinson's disease - a stereological study

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@article{d9e462d70e4147b3bd6259de62a51992,
title = "Pathological changes in the cerebellum of patients with Multiple System Atrophy and Parkinson's disease - a stereological study",
abstract = "Multiple System Atrophy (MSA) and Parkinson's disease (PD) are synucleinopathies characterized by aggregation of α-synuclein in brain cells. Recent studies have shown that morphological changes in terms of cerebral nerve cell loss and increase in glia cell numbers, the degree of brain atrophy and molecular and epidemiological findings are more severe in MSA than PD. In the present study, we performed a stereological comparison of cerebellar volumes, granule- and Purkinje cells in 13 patients diagnosed with MSA (8 MSA-P [striatonigral subtype] and 5 MSA-C [olivopontocerebellar subtype]), 12 PD patients and 15 age-matched control subjects. Only brains from MSA-C patients showed a reduction in the total number of Purkinje cells (anterior lobe) whereas both MSA-P and MSA-C patients had reduced Purkinje cell volumes (perikaryons and nuclei volume). The cerebellum of both diseases showed a reduction in the white matter volume compared to controls. The number of granule cells was unaffected in both diseases. Analyses of cell type-specific mRNA expression supported our structural data. This study of the cerebellum is in line with previous findings in the cerebrum and demonstrates that the degree of morphological changes is more pronounced in MSA-C than MSA-P and PD. Further, our results support an explicit involvement of cerebellar Purkinje cells and white matter connectivity in MSA-C>MSA-P and points to the potential importance of white matter alterations in PD pathology.",
author = "Rusholt, {Elisabeth H L} and Lisette Salvesen and Tomasz Brudek and Betel Tesfay and Bente Pakkenberg and Olesen, {Mikkel V}",
note = "{\circledC} 2019 International Society of Neuropathology.",
year = "2019",
month = "11",
day = "26",
doi = "10.1111/bpa.12806",
language = "English",
journal = "Brain Pathology",
issn = "1015-6305",
publisher = "Wiley-Blackwell Publishing, Inc",

}

RIS

TY - JOUR

T1 - Pathological changes in the cerebellum of patients with Multiple System Atrophy and Parkinson's disease - a stereological study

AU - Rusholt, Elisabeth H L

AU - Salvesen, Lisette

AU - Brudek, Tomasz

AU - Tesfay, Betel

AU - Pakkenberg, Bente

AU - Olesen, Mikkel V

N1 - © 2019 International Society of Neuropathology.

PY - 2019/11/26

Y1 - 2019/11/26

N2 - Multiple System Atrophy (MSA) and Parkinson's disease (PD) are synucleinopathies characterized by aggregation of α-synuclein in brain cells. Recent studies have shown that morphological changes in terms of cerebral nerve cell loss and increase in glia cell numbers, the degree of brain atrophy and molecular and epidemiological findings are more severe in MSA than PD. In the present study, we performed a stereological comparison of cerebellar volumes, granule- and Purkinje cells in 13 patients diagnosed with MSA (8 MSA-P [striatonigral subtype] and 5 MSA-C [olivopontocerebellar subtype]), 12 PD patients and 15 age-matched control subjects. Only brains from MSA-C patients showed a reduction in the total number of Purkinje cells (anterior lobe) whereas both MSA-P and MSA-C patients had reduced Purkinje cell volumes (perikaryons and nuclei volume). The cerebellum of both diseases showed a reduction in the white matter volume compared to controls. The number of granule cells was unaffected in both diseases. Analyses of cell type-specific mRNA expression supported our structural data. This study of the cerebellum is in line with previous findings in the cerebrum and demonstrates that the degree of morphological changes is more pronounced in MSA-C than MSA-P and PD. Further, our results support an explicit involvement of cerebellar Purkinje cells and white matter connectivity in MSA-C>MSA-P and points to the potential importance of white matter alterations in PD pathology.

AB - Multiple System Atrophy (MSA) and Parkinson's disease (PD) are synucleinopathies characterized by aggregation of α-synuclein in brain cells. Recent studies have shown that morphological changes in terms of cerebral nerve cell loss and increase in glia cell numbers, the degree of brain atrophy and molecular and epidemiological findings are more severe in MSA than PD. In the present study, we performed a stereological comparison of cerebellar volumes, granule- and Purkinje cells in 13 patients diagnosed with MSA (8 MSA-P [striatonigral subtype] and 5 MSA-C [olivopontocerebellar subtype]), 12 PD patients and 15 age-matched control subjects. Only brains from MSA-C patients showed a reduction in the total number of Purkinje cells (anterior lobe) whereas both MSA-P and MSA-C patients had reduced Purkinje cell volumes (perikaryons and nuclei volume). The cerebellum of both diseases showed a reduction in the white matter volume compared to controls. The number of granule cells was unaffected in both diseases. Analyses of cell type-specific mRNA expression supported our structural data. This study of the cerebellum is in line with previous findings in the cerebrum and demonstrates that the degree of morphological changes is more pronounced in MSA-C than MSA-P and PD. Further, our results support an explicit involvement of cerebellar Purkinje cells and white matter connectivity in MSA-C>MSA-P and points to the potential importance of white matter alterations in PD pathology.

U2 - 10.1111/bpa.12806

DO - 10.1111/bpa.12806

M3 - Journal article

JO - Brain Pathology

JF - Brain Pathology

SN - 1015-6305

ER -

ID: 58644691