Adjunctive Antioxidant Therapy in Neurologic Wilson’s Disease Improves the Outcomes

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

doi:10.1007/s12031-019-01423-8

Cited by 17 publications

(7 citation statements)

References 47 publications

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“…Kalita et al (2019) showed that vitamin E and C improved clinical outcomes and oxidative stress for WD patients with neurological symptoms who used zinc with or without chelating therapy. It has been proven that vitamin E has antioxidant properties (Kalita et al, 2019). However, there are only a few studies on vitamin E as a treatment for WD.…”

Section: Discussionmentioning

confidence: 99%

Sepsis and multiple organ dysfunction syndrome as the first clinical presentation of Wilson's disease: case report

Zeltiņa

Dreimane

Paeglite

et al. 2022

Proceedings of the Latvian Academy of Sciences. Section B. Natural, Exact, and Applied Sciences.

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Wilson’s disease (WD) is a rare autosomal recessive disorder caused by a mutation in the ATP7B gene. The evolution of Wilson’s disease is the result of the accumulation of copper in affected tissues. In this study, we report on a 30-year-old patient with multiple organ dysfunction syndrome and sepsis. After the necessary investigations and genetic tests, the patient was diagnosed with WD. Unfortunately, the first symptoms appeared at least ten years ago with elevated liver transaminases and amenorrhea. It is possible that if any of the previous physicians had provided a detailed examination, the patient would have reached a diagnosis sooner. The patient received adequate penicillamine treatment and her condition improved. The patient manages to have a healthy child by slightly changing the treatment of Wilson's disease. This case demonstrates that proper examination and collection of anamnesis information can lead to a diagnosis of WD. In addition, we emphasise the importance of targeted testing for elevated transaminases, especially if the patient has amenorrhea at reproductive age. Wilson's disease is not a barrier to carrying a healthy child. Of course, more research is needed to develop the best treatment and disease management plans.

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Section: Discussionmentioning

confidence: 99%

Sepsis and multiple organ dysfunction syndrome as the first clinical presentation of Wilson's disease: case report

Zeltiņa

Dreimane

Paeglite

et al. 2022

Proceedings of the Latvian Academy of Sciences. Section B. Natural, Exact, and Applied Sciences.

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“…[331] Symptomatic improvement when vitamin E was added to the treatment regimen was occasionally reported, but data for hepatic WD are mainly anecdotal because no rigorous studies have been conducted. One study in which vitamin E and vitamin C were added to treatment with chelator and/or zinc showed clinical improvement in neurologic WD in the group receiving antioxidants, [332] with the caveat that the two groups were comparable but treatment was not randomized.…”

Section: Antioxidantsmentioning

confidence: 99%

A multidisciplinary approach to the diagnosis and management of Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases

et al. 2022

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This is the complete version of the American Association for the Study of Liver Diseases (AASLD) 2022 Guidance on Wilson disease (WD). It provides a contemporary approach to diagnosis and management of WD. Guidance documents, developed by a panel of experts, are formulated as recommendations to help clinicians implement the most current strategies for diagnosis and management. This Guidance was developed with the support and oversight of the AASLD Practice Guidelines Committee. The AASLD Practice Guidelines Committee chose to commission a guidance, rather than a guideline, because of the paucity of randomized controlled trials on this topic. AASLD Guidelines are supported by systematic reviews of the literature, formal ratings of evidence quality and strength of recommendations, and, if appropriate, meta-analysis of results using the Grading of Recommendations Assessment Development and Evaluation system. In contrast, this narrative review and its guidance statements are based on (1) formal review and analysis of the recently published international literature on WD, (2) guideline policies of the AASLD, and (3) the authors' experience. This practice Guidance is intended for use by physicians and other health professionals. As clinically appropriate, guidance statements should be tailored to the needs of individual patients. Specific guidance statements are evidence based whenever possible. Where such evidence is unavailable or inconsistent, guidance statements are based on expert consensus opinion. An executive summary providing a convenient overview is available (

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“…Kalita et al . [ 70 ] reported a reduction in oxidative stress and improve clinical status in neurological WD cases receiving vitamin C and E along with anti-copper therapy.…”

Section: T Reatmentmentioning

confidence: 99%

Wilson’s Disease Update

Thankappan

Bhattacharya

2022

Annals of Indian Academy of Neurology

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Wilson's disease (WD) is an autosomal recessive disorder due to ATP7B gene mutation, resulting in defective copper metabolism, with liver and brain being primarily affected. Being a treatable disorder, early diagnosis and proper management of WD may result in near complete recovery. It has received significant attention over the past 50 years, with several Indian contributions. This study collates published Indian studies on WD in Pubmed and Embase databases and puts them in perspective. Several Indian case series suggest that WD may be more prevalent than thought. Commonly detected ATP7B mutation in India is p.C271X. Although initial Indian series reported significant osseomuscular presentation, neuropsychiatric and hepatic manifestations dominated the later reports. A significant male predominance is observed in Indian series. Pure hepatic presentation starts earlier than neurological or osseomuscular WD. A positive family history may be seen in nearly 50% of Indian WD cases with a high rate of consanguinity. Up to two-third of Indian cases may be initially misdiagnosed, with a mean diagnostic delay of up to 2 years. Abnormalities in serum ceruloplasmin and 24-hour urinary copper has been reported in more than four-fifth cases. Brain MRI is abnormal in nearly all neurological WD cases. Copper chelation remains the mainstay of therapy, with D-penicillamine being the most widely used chelator in India. Global Assessment Scale for WD is a comprehensive tool for clinical monitoring. Hepatic presentation carries a five-time higher mortality risk than neurological, with up to 90% Indian neurological WD cases recovering to pre-morbid functionality with adequate therapy.

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Adjunctive Antioxidant Therapy in Neurologic Wilson’s Disease Improves the Outcomes

Cited by 17 publications

References 47 publications

Sepsis and multiple organ dysfunction syndrome as the first clinical presentation of Wilson's disease: case report

Sepsis and multiple organ dysfunction syndrome as the first clinical presentation of Wilson's disease: case report

A multidisciplinary approach to the diagnosis and management of Wilson disease: Executive summary of the 2022 Practice Guidance on Wilson disease from the American Association for the Study of Liver Diseases

Wilson’s Disease Update

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