Neuropsychiatric Manifestations of Wilson Disease: Correlation with MRI and Glutamate Excitotoxicity

Kalita, Jayantee; Kumar, Vijay; Parashar, Vasudev; Misra, U. K.

doi:10.1007/s12035-021-02525-4

Cited by 9 publications

(8 citation statements)

References 71 publications

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“…the number of lesions (abnormal signs) shown on brain MRI in locations characteristic of WD; 1 point was scored for one lesion. As the number of lesions (occupied structures) visualised on brain MRI can correspond to the severity of neurological symptoms in the course of WD, positive correlations were found between brain MRI lesion load and the severity of neurological status, as well as neuropsychiatric inventory (NPI) (Kalita et al, 2021). However, due to physicians' superficial approach to radiological changes in the course of WD (different MRI sequences were scored the same) and due to discrepancies due to the different clinical significance associated with the observed changes in different brain MRI sequences (T1, T2 or T* gradient-echo sequences), this scale has not been more widely adopted for use.…”

Section: Neuroimaging -Brain Mrimentioning

confidence: 99%

“…W związku z tym, że liczba zmian (zajętych struktur) uwidocznionych w MRI mózgu może w przebiegu chW odpowiadać ciężkości objawów neurologicznych, stwierdzano dodatnie korelacje pomiędzy brain MRI lesion load a ciężkością stanu neurologicznego, a także objawów psychiatrycznych (ang. neuropsychiatric inventory, NPI), (Kalita et al, 2021). Jednak ze względu na powierzchowne podejście lekarzy do zmian radiologicznych w przebiegu chW (różne sekwencje badania MRI były punktowane tak samo) oraz ze względu na rozbieżności wynikające z różnego znaczenia klinicznego, jakie wiązano z obserwowanymi zmianami w różnych sekwencjach MRI mózgu (T1, T2 czy sekwencje gradientowe T*), skala ta nie została szerzej przyjęta do użytku.…”

Section: Neuroobrazowanie -Mri Mózguunclassified

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Biomarkers of the central nervous system injury in Wilson’s disease

Antos¹,

Litwin²,

Przybyłkowski³

et al. 2022

fpn

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symptoms of WD are mainly symptoms of liver damage (from a mild increase in the activity of liver enzymes to liver failure) and the central nervous system (CNS) (mainly a wide spectrum of involuntary movements). The article aims to review currently proposed biomarkers of CNS damage in WD: 1) clinical; 2) structural (neuroimaging) and 3) molecular, including a proposal of their future role (prognostic, diagnostic or monitoring of response to treatment). Literature review. WD is one of the few metabolic diseases that can be treated if treatment is started early and is properly administered. Treatment of WD is based on drugs causing a negative copper balance in the body (drugs that chelate copper or zinc salts). Early diagnosis of the disease, early initiation of anti-copper therapy, regular monitoring of copper metabolism, evaluation of liver function and damage of central nervous system are essential for the good prognosis. We conducted a targeted literature review of articles in English available in PubMed, searching for "Wilson's disease", "biomarkers", "neurofilaments". Below we present summary of collected data. Conclusions. Despite treatment, 10% of patients with WD experience early neurological deterioration, 50% of patients with neurological symptoms show no improvement. New therapies of treatment for WD, possibilities of faster diagnosis and better monitoring of the treatment

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Section: Neuroimaging -Brain Mrimentioning

confidence: 99%

Section: Neuroobrazowanie -Mri Mózguunclassified

Biomarkers of the central nervous system injury in Wilson’s disease

Antos¹,

Litwin²,

Przybyłkowski³

et al. 2022

fpn

View full text Add to dashboard Cite

show abstract

“…Both are important components of the basal ganglia thalamus cortex circuit and jointly regulate the motor function of the body [ 30 ]. Studies have found that the thalamic lesions are related to depression, and the caudate nucleus lesions are associated with anxiety [ 31 ]. Consistent with our study, several previous studies have confirmed that the volume atrophy of the bilateral caudate nucleus and thalamus in WDs is higher than that in HCs [ 10 , 32 , 33 ].…”

Section: Discussionmentioning

confidence: 99%

Machine learning for detecting Wilson's disease by amplitude of low-frequency fluctuation

Zhang

Peng

Chen

et al. 2023

Heliyon

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“…It was suggested that the neurological severity grade of WD correlated with urinary Cu. 24 As a motor pathway, the CST controls the movements of the limbs and trunk, originated from the motor regions and somatosensory, and terminates at motor neurons and spinal cord interneurons. 25 Previous studies about structural brain imaging have demonstrated that the integrity of the CST relates to residual motor outcomes.…”

Section: Discussionmentioning

confidence: 99%

Lesions in White Matter in Wilson’s Disease and Correlation with Clinical Characteristics

Wang

Wei

et al. 2022

Can. J. Neurol. Sci.

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Background: Neuroimaging studies in Wilson’s disease (WD) have identified various alterations in white matter (WM) microstructural organization. However, it remains unclear whether these alterations are localized to specific regions of fiber tracts, and what diagnostic value they might have. The purpose of this study is to explore the spatial profile of WM abnormalities along defined fiber tracts in WD and its clinical relevance. Methods: Ninety-nine patients with WD (62 men and 37 women) and 91 age- and sex-matched controls (59 men and 32 women) were recruited to take part in experiments of diffusion-weighted imaging with 64 gradient vectors. The data were calculated by FMRIB Software Library (FSL) software and Automated Fiber Quantification (AFQ) software. After registration, patient groups and normal groups were compared by Mann–Whitney U test analysis. Results: Compared with the controls, the patients with WD showed widespread fractional anisotropy reduction and mean diffusivity, radial diffusivity elevation of identified fiber tracts. Significant correlations between diffusion tensor imaging (DTI) parameters and the neurological Unified Wilson’s Disease Rating Scale (UWDRS-N), serum ceruloplasmin, and 24-h urinary copper excretion were found. Conclusions: The present study has provided evidence that the metrics of DTI could be utilized as a potential biomarker of neuropathological symptoms in WD. Damage to the microstructure of callosum forceps and corticospinal tract may be involved in the pathophysiological process of neurological symptoms in WD patients, such as gait and balance disturbances, involuntary movements, dysphagia, and autonomic dysfunction.

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Neuropsychiatric Manifestations of Wilson Disease: Correlation with MRI and Glutamate Excitotoxicity

Cited by 9 publications

References 71 publications

Biomarkers of the central nervous system injury in Wilson’s disease

Biomarkers of the central nervous system injury in Wilson’s disease

Machine learning for detecting Wilson's disease by amplitude of low-frequency fluctuation

Lesions in White Matter in Wilson’s Disease and Correlation with Clinical Characteristics

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