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@ARTICLE{Deistung:902565,
author = {Deistung, A. and Jäschke, D. and Draganova, R. and
Pfaffenrot, V. and Hulst, T. and Steiner, K. M. and Thieme,
A. and Giordano, I. A. and Klockgether, T. and Tunc, S. and
Münchau, A. and Minnerop, Martina and Göricke, S. L. and
Reichenbach, J. R. and Timmann, D.},
title = {{Q}uantitative suspecptibility mapping reveals alterations
of denate nuclei in common types of ataxias},
journal = {Brain communications},
volume = {4},
number = {1},
issn = {2632-1297},
address = {[Großbritannien]},
publisher = {Guarantors of Brain},
reportid = {FZJ-2021-04364},
pages = {fcab306},
year = {2022},
abstract = {The cerebellar nuclei are a brain region with high iron
content. Surprisingly, little is known about iron content in
the cerebellar nuclei and its possible contribution to
pathology in cerebellar ataxias, with the only exception of
Friedreich’s ataxia. In the present exploratory
cross-sectional study, quantitative susceptibility mapping
was used to investigate volume, iron concentration and total
iron content of the dentate nuclei in common types of
hereditary and non-hereditary degenerative ataxias.
Seventy-nine patients with spinocerebellar ataxias of types
1, 2, 3 and 6; 15 patients with Friedreich’s ataxia; 18
patients with multiple system atrophy, cerebellar type and
111 healthy controls were also included. All underwent 3 T
MRI and clinical assessments. For each specific ataxia
subtype, voxel-based and volumes-of-interest-based group
analyses were performed in comparison with a corresponding
age- and sex-matched control group, both for volume,
magnetic susceptiblity (indicating iron concentration) and
susceptibility mass (indicating total iron content) of the
dentate nuclei. Spinocerebellar ataxia of type 1 and
multiple system atrophy, cerebellar type patients showed
higher susceptibilities in large parts of the dentate
nucleus but unaltered susceptibility masses compared with
controls. Friedreich’s ataxia patients and, only on a
trend level, spinocerebellar ataxia of type 2 patients
showed higher susceptibilities in more circumscribed parts
of the dentate. In contrast, spinocerebellar ataxia of type
6 patients revealed lower susceptibilities and
susceptibility masses compared with controls throughout the
dentate nucleus. Spinocerebellar ataxia of type 3 patients
showed no significant changes in susceptibility and
susceptibility mass. Lower volume of the dentate nuclei was
found to varying degrees in all ataxia types. It was most
pronounced in spinocerebellar ataxia of type 6 patients and
least prominent in spinocerebellar ataxia of type 3
patients. The findings show that alterations in
susceptibility revealed by quantitative susceptibility
mapping are common in the dentate nuclei in different types
of cerebellar ataxias. The most striking changes in
susceptibility were found in spinocerebellar ataxia of type
1, multiple system atrophy, cerebellar type and
spinocerebellar ataxia of type 6. Because iron content is
known to be high in glial cells but not in neurons of the
cerebellar nuclei, the higher susceptibility in
spinocerebellar ataxia of type 1 and multiple system
atrophy, cerebellar type may be explained by a reduction of
neurons (increase in iron concentration) and/or an increase
in iron-rich glial cells, e.g. microgliosis. Hypomyelination
also leads to higher susceptibility and could also
contribute. The lower susceptibility in SCA6 suggests a loss
of iron-rich glial cells. Quantitative susceptibility maps
warrant future studies of iron content and iron-rich cells
in ataxias to gain a more comprehensive understanding of the
pathogenesis of these diseases.},
cin = {INM-1},
ddc = {610},
cid = {I:(DE-Juel1)INM-1-20090406},
pnm = {5251 - Multilevel Brain Organization and Variability
(POF4-525)},
pid = {G:(DE-HGF)POF4-5251},
typ = {PUB:(DE-HGF)16},
pubmed = {35291442},
UT = {WOS:000804710200027},
doi = {10.1093/braincomms/fcab306},
url = {https://juser.fz-juelich.de/record/902565},
}