Magnetic Susceptibility Changes in the Basal Ganglia and Brain Stem of Patients with Wilson’s Disease: Evaluation with Quantitative Susceptibility Mapping

Doğanay, Selim; Gümüş, Kazım; Koc, Gonca; Bayram, Ayşe Kaçar; Dogan, Mehmet Sait; Arslan, Duran; Gümüş, Hakan; Görkem, Süreyya Burcu; Ciraci, Saliha; Serin, Halil İbrahim; Çoşkun, Abdulhakim

doi:10.2463/mrms.mp.2016-0145

Cited by 18 publications

(17 citation statements)

References 34 publications

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“…Quantitative susceptibility mapping (QSM) is a recently developed MRI post-processing technique, which can quantitatively measure tissue susceptibility and reflect the concentrations of paramagnetic metal in vivo (Wang et al, 2017;Li et al, 2018). Using QSM, several recent studies found significantly increased susceptibility in the DGM of neurological WD patients, suggesting paramagnetic metal accumulation in the DGM is a character of neuropathology (Fritzsch et al, 2014;Doganay et al, 2018;Dezortova et al, 2019). In addition, QSM performed better than R2 * in distinguishing WD from healthy individuals and showed excellent diagnostic accuracy in the diagnosis of WD (Li et al, 2020).…”

Section: Introductionmentioning

confidence: 99%

Paramagnetic Metal Accumulation in the Deep Gray Matter Nuclei Is Associated With Neurodegeneration in Wilson’s Disease

Yuan

Chen

et al. 2020

Front. Neurosci.

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Background: Neuropathological studies have revealed copper and iron accumulation in the deep gray matter (DGM) nuclei of patients with Wilson's disease (WD). However, the association between metal accumulation and neurodegeneration in WD has not been well studied in vivo. The study was aimed to investigate whether metal accumulation in the DGM was associated with the structural and functional changes of DGM in neurological WD patients. Methods: Seventeen neurological WD patients and 20 healthy controls were recruited for the study. Mean bulk susceptibility values and volumes of DGM were obtained from quantitative susceptibility mapping (QSM). Regions of interest including the head of the caudate nucleus, globus pallidus, putamen, thalamus, substantia nigra, red nucleus, and dentate nucleus were manually segmented. The susceptibility values and volumes of DGM in different groups were compared using a linear regression model. Correlations between susceptibility values and volumes of DGM and Unified Wilson's Disease Rating Scale (UWDRS) neurological subscores were investigated. Results: The susceptibility values of all examined DGM in WD patients were higher than those in healthy controls (P < 0.05). Volume reductions were observed in the head of the caudate nucleus, globus pallidus, putamen, thalamus, and substantia nigra of WD patients (P < 0.001). Susceptibility values were negatively correlated with the volumes of the head of the caudate nucleus (r p = −0. 657, P = 0.037), putamen (r p = −0.667, P = 0.037), and thalamus (r p = −0. 613, P = 0.046) in WD patients. UWDRS neurological subscores were positively correlated with the susceptibility values of all examined DGM. The susceptibility values of putamen, head of the caudate nucleus, and dentate nucleus could well predict UWDRS neurological subscores. Conclusion: Our study provided in vivo evidence that paramagnetic metal accumulation in the DGM was associated with DGM atrophy and neurological impairment. The susceptibility of DGM could be used as a biomarker to assess the severity of neurodegeneration in WD.

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Section: Introductionmentioning

confidence: 99%

Paramagnetic Metal Accumulation in the Deep Gray Matter Nuclei Is Associated With Neurodegeneration in Wilson’s Disease

Yuan

Chen

et al. 2020

Front. Neurosci.

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“…Compared with QSM studies of differentiating the deposition of iron and copper in the lenticular nucleus in WD patients from HCs at 1.5T, 9,33 this QSM study on a clinical 3T MRI scanner indicated greater statistical power ( P values approximately reduced by a factor of 10), approaching that at 7T 32 . It is known that QSM at 3T is superior to QSM at 1.5T in terms of signal‐to‐noise ratio efficiency 27 .…”

Section: Discussionmentioning

confidence: 86%

Quantitative Measurement of Metal Accumulation in Brain of Patients With Wilson's Disease

Tong

et al. 2020

Movement Disorders

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A BS TRACT: Background: Currently, no study has evaluated metal accumulation in the brains of patients with Wilson's disease by using quantitative susceptibility mapping at 3T MRI. The objectives of this study were to qualitatively and quantitatively evaluate changes in magnetic susceptibility and R2* maps in deep gray matter nuclei to discriminate Wilson's disease patients from healthy controls and to evaluate their sensitivities in diagnosing Wilson's disease. Methods: Magnetic susceptibility and R2* maps and conventional T1-weighted, T2-weighted, and T2weighted fluid-attenuated inversion recovery images were obtained from 17 Wilson's disease patients and 14 age-matched healthy controls on a 3T MRI scanner. Differences between Wilson's disease and healthy control groups in susceptibility and R2* values in multiple deep nuclei were evaluated using a Mann-Whitney U test and receiver operating characteristic curves. The correlations of susceptibility and R2* values with Unified Wilson's Disease Rating Scale score were also performed. Results: Magnetic susceptibility and R2* can effectively distinguish different types of signal abnormalities. Magnetic susceptibility and R2* values in multiple deep nuclei of Wilson's disease patients were significantly higher than those in healthy controls. Magnetic susceptibility value in the substantia nigra had the highest area under the curve (0.888). There were positive correlations of the Unified Wilson's Disease Rating Scale score with susceptibility values in the caudate nucleus (r = 0.757, P = 0.011), putamen (r = 0.679, P = 0.031), and red nucleus (r = 0.638, P = 0.047), as well as R2* values in the caudate nucleus (r = 0.754, P = 0.012). Conclusions: Quantitative susceptibility mapping at 3T could be a useful tool to evaluate metal accumulation in deep gray matter nuclei of Wilson's disease patients.

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“…Major thalamic nuclei, especially inferior and lateral ones, can be identified with TSE contrast. The medial PLIC border (26) was easily discriminated from the adjacent lateral sensorimotor thalamic nuclei: from anterior to posterior: nucleus lateropolaris (32), nucleus ventrooralis (33), Vim (75), nucleus ventrocaudalis posterior (29), and nucleus ventrocaudalis anterior (28) ( Fig 6B).…”

Section: Thalamus and Dentatorubrothalamic Tractmentioning

confidence: 98%

3T MRI Whole-Brain Microscopy Discrimination of Subcortical Anatomy, Part 2: Basal Forebrain

Hoch

Bruno²,

Faustin³

et al. 2019

AJNR Am J Neuroradiol

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BACKGROUND AND PURPOSE: The basal forebrain contains multiple structures of great interest to emerging functional neurosurgery applications, yet many neuroradiologists are unfamiliar with this neuroanatomy because it is not resolved with current clinical MR imaging. MATERIALS AND METHODS: We applied an optimized TSE T2 sequence to washed whole postmortem brain samples (n ϭ 13) to demonstrate and characterize the detailed anatomy of the basal forebrain using a clinical 3T MR imaging scanner. We measured the size of selected internal myelinated pathways and measured subthalamic nucleus size, oblique orientation, and position relative to the intercommissural point. RESULTS: We identified most basal ganglia and diencephalon structures using serial axial, coronal, and sagittal planes relative to the intercommissural plane. Specific oblique image orientations demonstrated the positions and anatomic relationships for selected structures of interest to functional neurosurgery. We observed only 0.2-to 0.3-mm right-left differences in the anteroposterior and superoinferior length of the subthalamic nucleus (P ϭ .084 and .047, respectively). Individual variability for the subthalamic nucleus was greatest for angulation within the sagittal plane (range, 15°-37°), transverse dimension (range, 2-6.7 mm), and most inferior border (range, 4-7 mm below the intercommissural plane). CONCLUSIONS: Direct identification of basal forebrain structures in multiple planes using the TSE T2 sequence makes this challenging neuroanatomy more accessible to practicing neuroradiologists. This protocol can be used to better define individual variations relevant to functional neurosurgical targeting and validate/complement advanced MR imaging methods being developed for direct visualization of these structures in living patients. ABBREVIATIONS: DBS ϭ deep brain stimulation; DRT ϭ dentatorubrothalamic tract; PLIC ϭ posterior limb of the internal capsule; STN ϭ subthalamic nucleus; SUDC ϭ sudden unexplained death of childhood; Vim ϭ thalamic ventrointermedius nucleus; ZI ϭ zona incerta D eep to the cortical surface, the basal ganglia, thalamus, and subthalamus are vital basal forebrain structures involved in the regulation of autonomic, motor, sensory, limbic, and endocrine functions. 1,2 The metabolic demand of the basal forebrain exceeds the cerebral cortex in the resting state. 3 Focal pathologic functional or structural changes can have serious clinical consequences due to the compact organization of the basal forebrain. The thalamus is a complex hub receiving subcortical sensory and motor input that projects to both the cortex and striatum. 4 Thalamic infarction, demyelination, tumors, and other pathologies can cause chronic pain, 5 sensory loss in multiple modalities, amnesia, 6 dystonia, 7 and other disorders. 8,9 The subthalamus modulates basal ganglia output. 10 Ischemic and hyperglycemic injuries of the subthalamic nucleus can result in hemiballism. 11,12 The basal ganglia have complex connections to the cortical motor areas, including the...

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Magnetic Susceptibility Changes in the Basal Ganglia and Brain Stem of Patients with Wilson’s Disease: Evaluation with Quantitative Susceptibility Mapping

Cited by 18 publications

References 34 publications

Paramagnetic Metal Accumulation in the Deep Gray Matter Nuclei Is Associated With Neurodegeneration in Wilson’s Disease

Paramagnetic Metal Accumulation in the Deep Gray Matter Nuclei Is Associated With Neurodegeneration in Wilson’s Disease

Quantitative Measurement of Metal Accumulation in Brain of Patients With Wilson's Disease

3T MRI Whole-Brain Microscopy Discrimination of Subcortical Anatomy, Part 2: Basal Forebrain

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