Six-year clinical and MRI quantitative susceptibility mapping (QSM) follow-up in neurological Wilson’s disease under zinc therapy: a case report

Zaino, Domenica; Chiarotti, Ivano; Battisti, Carla; Salvatore, Simona; Federico, Antonio; Cerase, Alfonso

doi:10.1007/s10072-018-3557-1

Cited by 7 publications

(4 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In presymptomatic patients, either a chelator or Zn can be used [88,89]. Neurological deterioration is rare in zinc salts treatment [67], thus in patients with neurological symptoms worsening after treatment with chelators, zinc therapy should be introduced [90].…”

Section: Zinc In the Therapy Of Wilson's Diseasementioning

confidence: 99%

Zinc as a Drug for Wilson’s Disease, Non-Alcoholic Liver Disease and COVID-19-Related Liver Injury

et al. 2021

View full text Add to dashboard Cite

Zinc is the second most abundant trace element in the human body, and it plays a fundamental role in human physiology, being an integral component of hundreds of enzymes and transcription factors. The discovery that zinc atoms may compete with copper for their absorption in the gastrointestinal tract let to introduce zinc in the therapy of Wilson’s disease, a congenital disorder of copper metabolism characterized by a systemic copper storage. Nowadays, zinc salts are considered one of the best therapeutic approach in patients affected by Wilson’s disease. On the basis of the similarities, at histological level, between Wilson’s disease and non-alcoholic liver disease, zinc has been successfully introduced in the therapy of non-alcoholic liver disease, with positive effects both on insulin resistance and oxidative stress. Recently, zinc deficiency has been indicated as a possible factor responsible for the susceptibility of elderly patients to undergo infection by SARS-CoV-2, the coronavirus responsible for the COVID-19 pandemic. Here, we present the data correlating zinc deficiency with the insurgence and progression of Covid-19 with low zinc levels associated with severe disease states. Finally, the relevance of zinc supplementation in aged people at risk for SARS-CoV-2 is underlined, with the aim that the zinc-based drug, classically used in the treatment of copper overload, might be recorded as one of the tools reducing the mortality of COVID-19, particularly in elderly people.

show abstract

Section: Zinc In the Therapy Of Wilson's Diseasementioning

confidence: 99%

Zinc as a Drug for Wilson’s Disease, Non-Alcoholic Liver Disease and COVID-19-Related Liver Injury

et al. 2021

View full text Add to dashboard Cite

show abstract

“…Several case reports reported that metal accumulation in the DGM changed with the progression or improvement of neurological symptoms (Dusek et al, 2018;Zaino et al, 2019). It is speculated that metal accumulation in the DGM is a surrogate indicator of oxidative stress and neuronal loss (Yan et al, 2018).…”

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.

View full text Add to dashboard Cite

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.

show abstract

“…Some experts suggested that all symptomatic WD patients should receive a chelator at the stage of initial treatment, and zinc salts may have a role in maintenance treatment [ 67 , 149 ]. For patients whose neurological symptoms continue to worsen after treatment with chelators, replacement with zinc may lead to improvement [ 168 ]. Zinc salts have few adverse effects, but the most common one is gastric irritation which can be influenced by the type of zinc salts.…”

Section: The Management Of Wdmentioning

confidence: 99%

Management Perspective of Wilson’s Disease: Early Diagnosis and Individualized Therapy

Yuan

Yang

Wang

2021

View full text Add to dashboard Cite

: Wilson’s disease (WD) is an inherited disease caused by mutations in ATP7B and is characterized by the pathological accumulation of copper in the liver and brain. Common clinical manifestations of WD include a wide range of liver disease and neurological symptoms. In some patients, psychiatric symptom may be the only manifestation at the time of diagnosis. The clinical features of WD are highly variable and can mimic any disease of the internal medicine. Therefore, for unexplained medical diseases, the possibility of WD should not be ignored. Early diagnosis and treatment can improve the prognosis of WD patients and reduce disability and early death. Gene sequencing is becoming a valuable method to diagnose WD, and if possible, all WD patients and their siblings should be genetically sequenced. Copper chelators including Dpenicillamine, trientine, and dimercaptosuccinic acid can significantly improve the liver injury and symptoms of WD patients, but may have limited effect on neurological symptoms. Zinc salts may be more appropriate for the treatment of asymptomatic patients or for the maintenance treatment of symptomatic patients. High-quality clinical trials for the drug treatment of WD are still lacking, therefore, individualized treatment options for patients are recommended. Individualized treatment can be determined based on the clinical features of the WD patients, efficacy and adverse effects of the drugs, and the experience of the physician. Liver transplantation is the only effective method to save patients with acute liver failure or with severe liver disease who fail drug treatment.

show abstract

Six-year clinical and MRI quantitative susceptibility mapping (QSM) follow-up in neurological Wilson’s disease under zinc therapy: a case report

Cited by 7 publications

References 5 publications

Zinc as a Drug for Wilson’s Disease, Non-Alcoholic Liver Disease and COVID-19-Related Liver Injury

Zinc as a Drug for Wilson’s Disease, Non-Alcoholic Liver Disease and COVID-19-Related Liver Injury

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

Management Perspective of Wilson’s Disease: Early Diagnosis and Individualized Therapy

Contact Info

Product

Resources

About