Brain iron accumulation in Wilson disease: a post mortem 7 Tesla MRI - histopathological study
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F00216208%3A11110%2F17%3A10363078" target="_blank" >RIV/00216208:11110/17:10363078 - isvavai.cz</a>
Alternative codes found
RIV/00064165:_____/17:10363078
Result on the web
<a href="http://dx.doi.org/10.1111/nan.12341" target="_blank" >http://dx.doi.org/10.1111/nan.12341</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1111/nan.12341" target="_blank" >10.1111/nan.12341</a>
Alternative languages
Result language
angličtina
Original language name
Brain iron accumulation in Wilson disease: a post mortem 7 Tesla MRI - histopathological study
Original language description
AimsIn Wilson disease (WD), T2/T2*-weighted (T2*w) MRI frequently shows hypointensity in the basal ganglia that is suggestive of paramagnetic deposits. It is currently unknown whether this hypointensity is related to copper or iron deposition. We examined the neuropathological correlates of this MRI pattern, particularly in relation to iron and copper concentrations. MethodsBrain slices from nine WD and six control cases were investigated using a 7T-MRI system. High-resolution T2*w images were acquired and R2* parametric maps were reconstructed using a multigradient recalled echo sequence. R2* was measured in the globus pallidus (GP) and the putamen. Corresponding histopathological sections containing the lentiform nucleus were examined using Turnbull iron staining, and double staining combining Turnbull with immunohistochemistry for macrophages or astrocytes. Quantitative densitometry of the iron staining as well as copper and iron concentrations were measured in the GP and putamen and correlated with R2* values. ResultsT2*w hypointensity in the GP and/or putamen was apparent in WD cases and R2* values correlated with quantitative densitometry of iron staining. In WD, iron and copper concentrations were increased in the putamen compared to controls. R2* was correlated with the iron concentration in the GP and putamen, whereas no correlation was observed for the copper concentration. Patients with more pronounced pathological severity in the putamen displayed increased iron concentration, which correlated with an elevated number of iron-containing macrophages. ConclusionsT2/T2*w hypointensity observed in vivo in the basal ganglia of WD patients is related to iron rather than copper deposits.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
30103 - Neurosciences (including psychophysiology)
Result continuities
Project
<a href="/en/project/NV15-25602A" target="_blank" >NV15-25602A: Biomarkers of progression and treatment response in neurodegenerative disorders</a><br>
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2017
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Neuropathology and Applied Neurobiology
ISSN
0305-1846
e-ISSN
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Volume of the periodical
43
Issue of the periodical within the volume
6
Country of publishing house
GB - UNITED KINGDOM
Number of pages
19
Pages from-to
514-532
UT code for WoS article
000410552900005
EID of the result in the Scopus database
2-s2.0-84994807311