Open AccessBrain iron quantification by MRI in mitochondrial membrane protein‐associated neurodegeneration under iron‐chelating therapy
Author(s) -
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
Publication year - 2014
Publication title -
annals of clinical and translational neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.824
H-Index - 42
ISSN - 2328-9503
DOI - 10.1002/acn3.116
Subject(s) - neurodegeneration , substantia nigra , relaxometry , globus pallidus , medicine , pathology , magnetic resonance imaging , parkinson's disease , basal ganglia , disease , central nervous system , radiology , spin echo
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.