title: Brain iron quantification by MRI in mitochondrial membrane protein-associated neurodegeneration under iron-chelating therapy.
abstract: 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.
authors.affiliation: Department of Diagnostic and Interventional Neuroradiology, University Medical Center Hamburg-Eppendorf Hamburg, Germany.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Löbel
authors.affiliation: Buffalo Neuroimaging Analysis Center, Department of Neurology, School of Medicine and Biomedical Sciences, State University of New York at Buffalo Buffalo, New York, USA ; Buffalo Clinical and Translational Research Center (CTRC), Molecular and Translational Imaging Center, School of Medicine and Biomedical Science, State University of New York at Buffalo Buffalo, New York, USA.
authors.forename: Ferdinand
authors.initials: F
authors.lastname: Schweser
authors.affiliation: Clinic for Degenerative Brain Diseases, Department of Pediatrics, University Medical Center Hamburg-Eppendorf Hamburg, Germany.
authors.forename: Miriam
authors.initials: M
authors.lastname: Nickel
authors.affiliation: Medical Physics Group, Institute of Diagnostic and Interventional Radiology, Jena University Hospital, Friedrich Schiller University Jena, Germany.
authors.forename: Andreas
authors.initials: A
authors.lastname: Deistung
authors.affiliation: Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf Hamburg, Germany.
authors.forename: Regine
authors.initials: R
authors.lastname: Grosse
authors.affiliation: Institute of Neuropathology, University Medical Center Hamburg-Eppendorf Hamburg, Germany.
authors.forename: Christian
authors.initials: C
authors.lastname: Hagel
authors.affiliation: Department of Diagnostic and Interventional Neuroradiology, University Medical Center Hamburg-Eppendorf Hamburg, Germany.
authors.forename: Jens
authors.initials: J
authors.lastname: Fiehler
authors.affiliation: Clinic for Degenerative Brain Diseases, Department of Pediatrics, University Medical Center Hamburg-Eppendorf Hamburg, Germany.
authors.forename: Angela
authors.initials: A
authors.lastname: Schulz
authors.affiliation: Institute of Human Genetics, Technische Universität München Munich, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Hartig
authors.affiliation: Medical Physics Group, Institute of Diagnostic and Interventional Radiology, Jena University Hospital, Friedrich Schiller University Jena, Germany.
authors.forename: Jürgen R
authors.initials: JR
authors.lastname: Reichenbach
authors.affiliation: Clinic for Degenerative Brain Diseases, Department of Pediatrics, University Medical Center Hamburg-Eppendorf Hamburg, Germany.
authors.forename: Alfried
authors.initials: A
authors.lastname: Kohlschütter
authors.affiliation: Department of Diagnostic and Interventional Neuroradiology, University Medical Center Hamburg-Eppendorf Hamburg, Germany.
authors.forename: Jan
authors.initials: J
authors.lastname: Sedlacik