Cargando…

Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy

Therapeutic trials for Neurodegeneration with Brain Iron Accumulation have aimed at a reduction of cerebral iron content. A 13-year-old girl with mitochondrial membrane protein-associated neurodegeneration treated with an iron-chelating agent was monitored by R2 relaxometry, R2* relaxometry, and qua...

Descripción completa

Detalles Bibliográficos
Autores principales: Löbel, Ulrike, Schweser, Ferdinand, Nickel, Miriam, Deistung, Andreas, Grosse, Regine, Hagel, Christian, Fiehler, Jens, Schulz, Angela, Hartig, Monika, Reichenbach, Jürgen R, Kohlschütter, Alfried, Sedlacik, Jan
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Blackwell Publishing Ltd 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4284129/
https://www.ncbi.nlm.nih.gov/pubmed/25574478
http://dx.doi.org/10.1002/acn3.116
_version_ 1782351371022893056
author Löbel, Ulrike
Schweser, Ferdinand
Nickel, Miriam
Deistung, Andreas
Grosse, Regine
Hagel, Christian
Fiehler, Jens
Schulz, Angela
Hartig, Monika
Reichenbach, Jürgen R
Kohlschütter, Alfried
Sedlacik, Jan
author_facet Löbel, Ulrike
Schweser, Ferdinand
Nickel, Miriam
Deistung, Andreas
Grosse, Regine
Hagel, Christian
Fiehler, Jens
Schulz, Angela
Hartig, Monika
Reichenbach, Jürgen R
Kohlschütter, Alfried
Sedlacik, Jan
author_sort Löbel, Ulrike
collection PubMed
description Therapeutic trials for Neurodegeneration with Brain Iron Accumulation have aimed at a reduction of cerebral iron content. A 13-year-old girl with mitochondrial membrane protein-associated neurodegeneration treated with an iron-chelating agent was monitored by R2 relaxometry, R2* relaxometry, and quantitative susceptibility mapping to estimate the brain iron content. The highly increased brain iron content slowly decreased in the substantia nigra but remained stable for globus pallidus. The estimated iron content was higher by R2* compared to R2 and quantitative susceptibility mapping, a finding not previously observed in the brain of healthy volunteers. A hypothesis explaining this discrepancy is offered.
format Online
Article
Text
id pubmed-4284129
institution National Center for Biotechnology Information
language English
publishDate 2014
publisher Blackwell Publishing Ltd
record_format MEDLINE/PubMed
spelling pubmed-42841292015-01-08 Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy Löbel, Ulrike Schweser, Ferdinand Nickel, Miriam Deistung, Andreas Grosse, Regine Hagel, Christian Fiehler, Jens Schulz, Angela Hartig, Monika Reichenbach, Jürgen R Kohlschütter, Alfried Sedlacik, Jan Ann Clin Transl Neurol Brief Communications Therapeutic trials for Neurodegeneration with Brain Iron Accumulation have aimed at a reduction of cerebral iron content. A 13-year-old girl with mitochondrial membrane protein-associated neurodegeneration treated with an iron-chelating agent was monitored by R2 relaxometry, R2* relaxometry, and quantitative susceptibility mapping to estimate the brain iron content. The highly increased brain iron content slowly decreased in the substantia nigra but remained stable for globus pallidus. The estimated iron content was higher by R2* compared to R2 and quantitative susceptibility mapping, a finding not previously observed in the brain of healthy volunteers. A hypothesis explaining this discrepancy is offered. Blackwell Publishing Ltd 2014-12 2014-12-02 /pmc/articles/PMC4284129/ /pubmed/25574478 http://dx.doi.org/10.1002/acn3.116 Text en © 2014 The Authors. Annals of Clinical and Translational Neurology published by Wiley Periodicals, Inc on behalf of American Neurological Association. http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
spellingShingle Brief Communications
Löbel, Ulrike
Schweser, Ferdinand
Nickel, Miriam
Deistung, Andreas
Grosse, Regine
Hagel, Christian
Fiehler, Jens
Schulz, Angela
Hartig, Monika
Reichenbach, Jürgen R
Kohlschütter, Alfried
Sedlacik, Jan
Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy
title Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy
title_full Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy
title_fullStr Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy
title_full_unstemmed Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy
title_short Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy
title_sort brain iron quantification by mri in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy
topic Brief Communications
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4284129/
https://www.ncbi.nlm.nih.gov/pubmed/25574478
http://dx.doi.org/10.1002/acn3.116
work_keys_str_mv AT lobelulrike brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT schweserferdinand brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT nickelmiriam brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT deistungandreas brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT grosseregine brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT hagelchristian brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT fiehlerjens brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT schulzangela brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT hartigmonika brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT reichenbachjurgenr brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT kohlschutteralfried brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy
AT sedlacikjan brainironquantificationbymriinmitochondrialmembraneproteinassociatedneurodegenerationunderironchelatingtherapy