Improvement of Diffusion Tensor Imaging (DTI) Parameters with Decoppering Treatment in Wilson’s Disease

Lawrence, Andrew David; Saini, Jitender; Sinha, Sanjib; Rao, Shivaji; Naggappa, M.; Bindu, Parayil Sankaran; Taly, AB

doi:10.1007/8904_2015_466

Cited by 15 publications

(9 citation statements)

References 22 publications

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“…DTI abnormalities including decreases in FA and increases in MD have previously been demonstrated in the white matter of newly diagnosed patients using region of interest analyses. 20 , 27 , 59–61 Associations between a neurological symptom score and increased MD in the anterior limb of the internal capsule and between a measure of disability and increased MD in frontal white matter have also been reported. 59 In one of the few previous prospective neuroimaging studies on Wilson’s disease, Lawrence et al .…”

Section: Discussionmentioning

confidence: 92%

Neuroimaging correlates of brain injury in Wilson’s disease: a multimodal, whole-brain MRI study

Shribman

Bocchetta

Sudre

et al. 2021

Brain

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Wilson’s disease is an autosomal-recessive disorder of copper metabolism with neurological and hepatic presentations. Chelation therapy is used to ‘de-copper’ patients but neurological outcomes remain unpredictable. A range of neuroimaging abnormalities have been described and may provide insights into disease mechanisms, in addition to prognostic and monitoring biomarkers. Previous quantitative MRI analyses have focussed on specific sequences or regions of interest, often stratifying chronically-treated patients according to persisting symptoms as opposed to initial presentation. In this cross-sectional study, we performed a combination of unbiased, whole-brain analyses on T1-weighted, fluid-attenuated inversion recovery, diffusion-weighted and susceptibility-weighted imaging data from 40 prospectively-recruited patients with Wilson’s disease (age range 16–68). We compared patients with neurological (n = 23) and hepatic (n = 17) presentations to determine the neuroradiological sequelae of the initial brain injury. We also subcategorized patients according to recent neurological status, classifying those with neurological presentations or deterioration in the preceding six months as having ‘active’ disease. This allowed us to compare patients with active (n = 5) and stable (n = 35) disease and identify imaging correlates for persistent neurological deficits and copper indices in chronically-treated, stable patients. Using a combination of voxel-based morphometry and region-of-interest volumetric analyses, we demonstrate that grey matter volumes are lower in the basal ganglia, thalamus, brainstem, cerebellum, anterior insula and orbitofrontal cortex when comparing patients with neurological and hepatic presentations. In chronically-treated, stable patients, the severity of neurological deficits correlated with grey matter volumes in similar, predominantly subcortical regions. In contrast, the severity of neurological deficits did not correlate with the volume of white matter hyperintensities, calculated using an automated lesion segmentation algorithm. Using tract-based spatial statistics, increasing neurological severity in chronically-treated patients was associated with decreasing axial diffusivity in white matter tracts whereas increasing serum non-caeruloplasmin-bound (‘free’) copper and active disease were associated with distinct patterns of increasing mean, axial and radial diffusivity. Whole-brain quantitative susceptibility mapping identified increased iron deposition in the putamen, cingulate and medial frontal cortices of patients with neurological presentations relative to those with hepatic presentations and neurological severity was associated with iron deposition in widespread cortical regions in chronically-treated patients. Our data indicate that composite measures of subcortical atrophy provide useful prognostic biomarkers, whereas abnormal mean, axial and radial diffusivity are promising monitoring biomarkers. Finally, deposition of brain iron in response to copper accumulation may directly contribute to neurodegeneration in Wilson’s disease.

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Section: Discussionmentioning

confidence: 92%

Neuroimaging correlates of brain injury in Wilson’s disease: a multimodal, whole-brain MRI study

Shribman

Bocchetta

Sudre

et al. 2021

Brain

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“…Jadav et al used the technique of DTI to evaluate white matter abnormalities in WD, finding MD significantly increased in the bilateral internal capsules and midbrain, and FA significantly decreased in the frontal and occipital white matter, bilateral internal capsules, midbrain, and pons, while the images were normal appearing on conventional MRI [ 15 ]. Li et al showed lower FA and higher MD in bilateral thalamus in patients, and Lawrence et al found higher FA in white matter in the initial phase of the patients [ 14 , 23 ]. Besides, some other methods were applied to diagnosis and follow-up in WD, either.…”

Section: Discussionmentioning

confidence: 99%

Study on Lesion Assessment of Cerebello-Thalamo-Cortical Network in Wilson’s Disease with Diffusion Tensor Imaging

Wang

Wu²,

et al. 2017

Neural Plasticity

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Wilson's disease (WD) is a genetic disorder of copper metabolism with pathological copper accumulation in the brain and any other tissues. This article aimed to assess lesions in cerebello-thalamo-cortical network with an advanced technique of diffusion tensor imaging (DTI) in WD. 35 WD patients and 30 age- and sex-matched healthy volunteers were recruited to accept diffusion-weighted images with 15 gradient vectors and conventional magnetic resonance imaging (MRI). The DTI parameters, including fractional anisotropy (FA) and mean diffusion (MD), were calculated by diffusion kurtosis estimator software. After registration, patient groups with FA mappings and MD mappings and normal groups were compared with 3dttest and receiver-operating characteristic (ROC) curve analysis, corrected with FDR simulations (p = 0.001, α = 0.05, cluster size = 326). We found that the degree of FA increased in the bilateral head of the caudate nucleus (HCN), lenticular nucleus (LN), ventral thalamus, substantia nigra (SN), red nucleus (RN), right dentate nucleus (DN), and decreased in the mediodorsal thalamus and extensive white matter. The value of MD increased in HCN, LN, SN, RN, and extensive white matter. The technique of DTI provides higher sensitivity and specificity than conventional MRI to detect Wilson's disease. Besides, lesions in the basal ganglia, thalamus, and cerebellum might disconnect the basal ganglia-thalamo-cortical circuits or dentato-rubro-thalamic (DRT) track and disrupt cerebello-thalamo-cortical network finally, which may cause clinical extrapyramidal symptoms.

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“…In this study, the values of MD, AxD, and RD were increased to mm 2 /s instead of 10 −3 μm 2 /s. This approach has been performed previously …”

Section: Methodsmentioning

confidence: 99%

Prospective Associations Between Diffusion Tensor Imaging Parameters and Frailty in Older Adults

Maltais

Barreto

Perus

et al. 2020

J American Geriatrics Society

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for the Multidomain Alzheimer Preventive Trial/Data Sharing Alzheimer (MAPT/DSA) Study Group BACKGROUND: Cross-sectional associations have been found between frail individuals and worse white matter (WM) integrity. However, the prospective association between WM integrity and frailty is still unclear. Our objectives were to measure associations between WM integrity using diffusion tensor imaging (DTI) and the 5-year worsening of frailty in community-dwelling older adults. DESIGN: Secondary analysis of the randomized controlled Multidomain Alzheimer Preventive Trial (MAPT). SETTING: Thirteen memory centers in France and Monaco between 2008 and 2011. PARTICIPANTS: Participants (mean age = 74.7 AE 3.9 years) with no dementia at baseline who had functional magnetic resonance imaging performed as part of the MAPT study (n = 227). MEASUREMENTS: Fractional anisotropy and mean diffusivity (MD), axial diffusivity (AxD), and radial diffusivity (RD) were acquired for 10 different brain regions. Frailty was assessed by the Fried frailty phenotype (score from 0 to 5, higher is worse) at up to seven time points for 5 years. Mixed effect ordinal logistic regression model was used to assess the prospective association between DTI parameters (independent variables) and frailty (dependent variable). All the analyses were adjusted for age, sex, baseline total intracranial volume, and the presence of one of the following cardiovascular risk factors (hypertension, diabetes, and/or hypercholesterolemia). RESULTS: A statistically significant association was found between the RD, AxD, and MD for different brain regions (anterior limb of internal capsule, external capsule, posterior corona radiata, posterior thalamic radiation, superior corona radiata, superior frontal occipital fasciculus, and superior longitudinal fasciculus) and worsening of frailty over 5 years after adjusting for multiple comparisons. CONCLUSIONS: This is the first study to show that WM integrity is associated with frailty in older adults. The mechanisms related to these results require further investigation.

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Improvement of Diffusion Tensor Imaging (DTI) Parameters with Decoppering Treatment in Wilson’s Disease

Cited by 15 publications

References 22 publications

Neuroimaging correlates of brain injury in Wilson’s disease: a multimodal, whole-brain MRI study

Neuroimaging correlates of brain injury in Wilson’s disease: a multimodal, whole-brain MRI study

Study on Lesion Assessment of Cerebello-Thalamo-Cortical Network in Wilson’s Disease with Diffusion Tensor Imaging

Prospective Associations Between Diffusion Tensor Imaging Parameters and Frailty in Older Adults

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