MR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study

Aims: Non-invasive measures of brain iron content would be of great benefit in neurodegeneration with brain iron accumulation (NBIA) to serve as a biomarker for disease progression and evaluation of iron chelation therapy. Although magnetic resonance imaging (MRI) provides several quantitative measu...

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Autores principales:	Lena H.P. Vroegindeweij, Piotr A. Wielopolski, Agnita J.W. Boon, J.H. Paul Wilson, Rob M. Verdijk, Sipeng Zheng, Sylvestre Bonnet, Lucia Bossoni, Louise van der Weerd, Juan A. Hernandez-Tamames, Janneke G. Langendonk
Formato:	article
Lenguaje:	EN
Publicado:	Elsevier 2021
Materias:	Aceruloplasminemia Neurodegeneration with brain iron accumulation (NBIA) Postmortem MRI Iron Transverse relaxation rate (R2*) Susceptibility Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571
Acceso en línea:	https://doaj.org/article/25680c7f65bc474789a8738d5494e084
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spelling	oai:doaj.org-article:25680c7f65bc474789a8738d5494e0842021-11-28T04:29:13ZMR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study1095-957210.1016/j.neuroimage.2021.118752https://doaj.org/article/25680c7f65bc474789a8738d5494e0842021-12-01T00:00:00Zhttp://www.sciencedirect.com/science/article/pii/S1053811921010247https://doaj.org/toc/1095-9572Aims: Non-invasive measures of brain iron content would be of great benefit in neurodegeneration with brain iron accumulation (NBIA) to serve as a biomarker for disease progression and evaluation of iron chelation therapy. Although magnetic resonance imaging (MRI) provides several quantitative measures of brain iron content, none of these have been validated for patients with a severely increased cerebral iron burden. We aimed to validate R2* as a quantitative measure of brain iron content in aceruloplasminemia, the most severely iron-loaded NBIA phenotype. Methods: Tissue samples from 50 gray- and white matter regions of a postmortem aceruloplasminemia brain and control subject were scanned at 1.5 T to obtain R2, and biochemically analyzed with inductively coupled plasma mass spectrometry. For gray matter samples of the aceruloplasminemia brain, sample R2 values were compared with postmortem in situ MRI data that had been obtained from the same subject at 3 T – in situ R2. Relationships between R2 and tissue iron concentration were determined by linear regression analyses. Results: Median iron concentrations throughout the whole aceruloplasminemia brain were 10 to 15 times higher than in the control subject, and R2* was linearly associated with iron concentration. For gray matter samples of the aceruloplasminemia subject with an iron concentration up to 1000 mg/kg, 91% of variation in R2* could be explained by iron, and in situ R2* at 3 T and sample R2* at 1.5 T were highly correlated. For white matter regions of the aceruloplasminemia brain, 85% of variation in R2* could be explained by iron. Conclusions: R2* is highly sensitive to variations in iron concentration in the severely iron-loaded brain, and might be used as a non-invasive measure of brain iron content in aceruloplasminemia and potentially other NBIA disorders.Lena H.P. VroegindeweijPiotr A. WielopolskiAgnita J.W. BoonJ.H. Paul WilsonRob M. VerdijkSipeng ZhengSylvestre BonnetLucia BossoniLouise van der WeerdJuan A. Hernandez-TamamesJanneke G. LangendonkElsevierarticleAceruloplasminemiaNeurodegeneration with brain iron accumulation (NBIA)Postmortem MRIIronTransverse relaxation rate (R2*)SusceptibilityNeurosciences. Biological psychiatry. NeuropsychiatryRC321-571ENNeuroImage, Vol 245, Iss , Pp 118752- (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Aceruloplasminemia Neurodegeneration with brain iron accumulation (NBIA) Postmortem MRI Iron Transverse relaxation rate (R2*) Susceptibility Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571
spellingShingle	Aceruloplasminemia Neurodegeneration with brain iron accumulation (NBIA) Postmortem MRI Iron Transverse relaxation rate (R2*) Susceptibility Neurosciences. Biological psychiatry. Neuropsychiatry RC321-571 Lena H.P. Vroegindeweij Piotr A. Wielopolski Agnita J.W. Boon J.H. Paul Wilson Rob M. Verdijk Sipeng Zheng Sylvestre Bonnet Lucia Bossoni Louise van der Weerd Juan A. Hernandez-Tamames Janneke G. Langendonk MR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study
description	Aims: Non-invasive measures of brain iron content would be of great benefit in neurodegeneration with brain iron accumulation (NBIA) to serve as a biomarker for disease progression and evaluation of iron chelation therapy. Although magnetic resonance imaging (MRI) provides several quantitative measures of brain iron content, none of these have been validated for patients with a severely increased cerebral iron burden. We aimed to validate R2* as a quantitative measure of brain iron content in aceruloplasminemia, the most severely iron-loaded NBIA phenotype. Methods: Tissue samples from 50 gray- and white matter regions of a postmortem aceruloplasminemia brain and control subject were scanned at 1.5 T to obtain R2, and biochemically analyzed with inductively coupled plasma mass spectrometry. For gray matter samples of the aceruloplasminemia brain, sample R2 values were compared with postmortem in situ MRI data that had been obtained from the same subject at 3 T – in situ R2. Relationships between R2 and tissue iron concentration were determined by linear regression analyses. Results: Median iron concentrations throughout the whole aceruloplasminemia brain were 10 to 15 times higher than in the control subject, and R2* was linearly associated with iron concentration. For gray matter samples of the aceruloplasminemia subject with an iron concentration up to 1000 mg/kg, 91% of variation in R2* could be explained by iron, and in situ R2* at 3 T and sample R2* at 1.5 T were highly correlated. For white matter regions of the aceruloplasminemia brain, 85% of variation in R2* could be explained by iron. Conclusions: R2* is highly sensitive to variations in iron concentration in the severely iron-loaded brain, and might be used as a non-invasive measure of brain iron content in aceruloplasminemia and potentially other NBIA disorders.
format	article
author	Lena H.P. Vroegindeweij Piotr A. Wielopolski Agnita J.W. Boon J.H. Paul Wilson Rob M. Verdijk Sipeng Zheng Sylvestre Bonnet Lucia Bossoni Louise van der Weerd Juan A. Hernandez-Tamames Janneke G. Langendonk
author_facet	Lena H.P. Vroegindeweij Piotr A. Wielopolski Agnita J.W. Boon J.H. Paul Wilson Rob M. Verdijk Sipeng Zheng Sylvestre Bonnet Lucia Bossoni Louise van der Weerd Juan A. Hernandez-Tamames Janneke G. Langendonk
author_sort	Lena H.P. Vroegindeweij
title	MR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study
title_short	MR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study
title_full	MR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study
title_fullStr	MR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study
title_full_unstemmed	MR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study
title_sort	mr imaging for the quantitative assessment of brain iron in aceruloplasminemia: a postmortem validation study
publisher	Elsevier
publishDate	2021
url	https://doaj.org/article/25680c7f65bc474789a8738d5494e084
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MR imaging for the quantitative assessment of brain iron in aceruloplasminemia: A postmortem validation study

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