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

doi:10.1002/acn3.116

Cited by 25 publications

(18 citation statements)

References 19 publications

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“…The failure to detect significant R 2 * differences could be caused by the lower contrast‐to‐noise ratio in the R 2 * values. Alternatively, as R 2 * is sensitive to larger sized iron particles , it is also possible that the increased iron deposited in DN comprises smaller sized particles. These iron particles may be in the molecular form and not bound with proteins.…”

Section: Discussionmentioning

confidence: 99%

Dentate nucleus iron deposition is a potential biomarker for tremor‐dominant Parkinson's disease

Huang

Ling

et al. 2016

NMR in Biomedicine

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Parkinson disease (PD) is a heterogeneous neurodegenerative disorder with variable clinicopathologic phenotypes and underlying neuropathologic mechanisms. Each clinical phenotype has a unique set of motor symptoms. Tremor is the most frequent initial motor symptom of PD and is the most difficult symptom to treat. The dentate nucleus (DN) is a deep iron rich nucleus in the cerebellum and may be involved in PD tremor. In this study, we test the hypothesis that DN iron may be elevated in tremor dominant PD patients using quantitative susceptibility mapping. Forty-three patients with PD [19 tremor dominant (TD)/24 akinetic-rigid dominant (AR)] and 48 healthy gender- and age-matched controls were recruited. Multi-echo gradient echo data were collected for each subject on a 3.0 T MR system. Inter-group susceptibility differences in bilateral DN were investigated and correlations of clinical features with susceptibility were also examined. In contrast to the AR group, the TD group was found to have increased susceptibility in the bilateral DN, when compared to healthy controls. In addition, susceptibility was positively correlated with tremor score in drug naive PD patients. These findings indicate that iron load within DN may make an important contribution to motor phenotypes in PD. Moreover, our results suggest that TD and AR phenotypes of PD can be differentiated on the basis of the susceptibility of the DN at least on the group level.

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Section: Discussionmentioning

confidence: 99%

Dentate nucleus iron deposition is a potential biomarker for tremor‐dominant Parkinson's disease

Huang

Ling

et al. 2016

NMR in Biomedicine

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“…To date, QSM has shown strong potential for detecting cerebral microbleeds after traumatic brain injury and for characterizing glioblastomas—reviewed elsewhere . In addition, QSM is revealing novel information in aging, in chronic disorders of the nervous system such as multiple sclerosis, Alzheimer’s disease, Parkinson’s disease, Huntington’s disease, or motor neuron disease, and QSM has been used successfully for monitoring therapeutic intervention in neurodegeneration with brain iron accumulation …”

Section: Introductionmentioning

confidence: 99%

“…7,8 In addition, QSM is revealing novel information in aging, [9][10][11][12] in chronic disorders of the nervous system such as multiple sclerosis, 13,14 Alzheimer's disease, 15,16 Parkinson's disease, 17,18 Huntington's disease, 19 or motor neuron disease, 20 and QSM has been used successfully for monitoring therapeutic intervention in neurodegeneration with brain iron accumulation. 21 Such a range of applications stem from the para-and ferromagnetic properties of most transition metals and metalloids, and the diamagnetism of inorganic compounds and myelin lipids, all of which influence the local magnetic susceptibilities of biological tissue. Across brain parenchyma, iron is thought to be the primary contributor to QSM contrast differences, [22][23][24][25] although strong diamagnetic calcifications 26 and contributions of white matter microstructure 27 can modulate-sometimes dominate-local susceptibilities.…”

Section: Introductionmentioning

confidence: 99%

Prospective motion correction improves high‐resolution quantitative susceptibility mapping at 7T

Mattern

Sciarra

Lüsebrink

et al. 2018

Magnetic Resonance in Med

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Purpose Recent literature has shown the potential of high‐resolution quantitative susceptibility mapping (QSM) with ultra‐high field MRI for imaging the anatomy, the vasculature, and investigating their magnetostatic properties. Higher spatial resolutions, however, translate to longer scans resulting, therefore, in higher vulnerability to, and likelihood of, subject movement. We propose a gradient‐recalled echo sequence with prospective motion correction (PMC) to address such limitation. Methods Data from 4 subjects were acquired at 7T. The effect of small and large motion on QSM with and without PMC was assessed qualitatively and quantitatively. Full brain QSM and QSM‐based venograms with up to 0.33 mm isotropic voxel size were reconstructed. Results With PMC, motion artifacts in QSM and QSM‐based venograms were largely eliminated, enabling—in both large‐ and small‐amplitude motion regimes—accurate depiction of the cortex, vasculature, and other small anatomical structures that are often blurred as a result of head movement or indiscernible at lower image resolutions. Quantitative analyses demonstrated that uncorrected motion could bias regional susceptibility distributions, a trend that was greatly reduced with PMC. Conclusion Qualitatively, PMC prevented image degradation because of motion artifacts, providing highly detailed QSM images and venograms. Quantitatively, PMC increased the reproducibility of susceptibility measures.

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“…Its physiological function and the mechanism of iron accumulation located into GP and SN are not entirely understood [82]. A case study reported no change in clinical status after chelation therapy with 30 mg/kg/day deferiprone lasting two years [83].…”

Section: Other Nbia Disordersmentioning

confidence: 99%

Iron chelation in the treatment of neurodegenerative diseases

Dušek

Schneider

Aaseth

2016

Journal of Trace Elements in Medicine and Biology

106

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a b s t r a c tDisturbance of cerebral iron regulation is almost universal in neurodegenerative disorders. There is a growing body of evidence that increased iron deposits may contribute to degenerative changes. Thus, the effect of iron chelation therapy has been investigated in many neurological disorders including rare genetic syndromes with neurodegeneration with brain iron accumulation as well as common sporadic disorders such as Parkinson's disease, Alzheimer's disease, and multiple sclerosis. This review summarizes recent advances in understanding the role of iron in the etiology of neurodegeneration. Outcomes of studies investigating the effect of iron chelation therapy in neurodegenerative disorders are systematically presented in tables. Iron chelators, particularly the blood brain barrier-crossing compound deferiprone, are capable of decreasing cerebral iron in areas with abnormally high concentrations as documented by MRI. Yet, currently, there is no compelling evidence of the clinical effect of iron removal therapy on any neurological disorder. However, several studies indicate that it may prevent or slow down disease progression of several disorders such as aceruloplasminemia, pantothenate kinase-associated neurodegeneration or Parkinson's disease.

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

Cited by 25 publications

References 19 publications

Dentate nucleus iron deposition is a potential biomarker for tremor‐dominant Parkinson's disease

Dentate nucleus iron deposition is a potential biomarker for tremor‐dominant Parkinson's disease

Prospective motion correction improves high‐resolution quantitative susceptibility mapping at 7T

Iron chelation in the treatment of neurodegenerative diseases

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