Liver Transplantation in Neurological Wilson's Disease: Is There Indication? A Case Report

Mocchegiani, Federico; Gemini, S; Vincenzi, Paolo; Montalti, Roberto; Vecchi, Andrea; Nicolini, Daniele; Federici, Augusto B.; Coletta, M.; Pansini, Mihovil; Lanari, Jacopo; Svegliati‐Baroni, Gianluca; Risaliti, Andrea; Vivarelli, Marco

doi:10.1016/j.transproceed.2014.07.059

Cited by 12 publications

(4 citation statements)

References 26 publications

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“…We found assessment of the brain MRI changes in case and series reports for only 10 out of 32 patients [24,35,38,48,[55][56][57]61]. In nine cases, regression of disease was observed [24,35,38,48,[55][56][57], and in one, there was worsening that corresponded with clinical neurological deterioration [61]. The clear correlations between radiological changes and neurological status were adequately presented only in case reports.…”

Section: Resultsmentioning

confidence: 92%

“…The analysis of brain MRI in patients after LT retrieved from hepatological registries was very limited, apart from the study by Poujois et al [64], which documented a correlation between regression of MRI changes and clinical improvement (Table 1). We found assessment of the brain MRI changes in case and series reports for only 10 out of 32 patients [24,35,38,48,[55][56][57]61]. In nine cases, regression of disease was observed [24,35,38,48,[55][56][57], and in one, there was worsening that corresponded with clinical neurological deterioration [61].…”

Section: Resultsmentioning

confidence: 94%

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Liver transplantation as a treatment for Wilson’s disease with neurological presentation: a systematic literature review

et al. 2022

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Introduction Wilson’s disease (WD) is a potentially treatable, genetic disorder of copper metabolism, with survival similar to healthy populations if controlled. However, in almost 50% of WD patients, neurological symptoms persist despite treatment, and in up to 10% of patients, neurological deterioration is irreversible. International guidelines on WD treatment do not recommend liver transplantation (LT) as a treatment for neurological symptoms in WD. However, such treatment has been assessed in retrospective analyses, case and series reports. We aimed to systematically assess all available evidence on the effectiveness and safety of LT in WD patients with neurological presentation. Methods This systematic literature review was performed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies were identified by searching the PubMed database (up to 6 April 2021) and by screening reference lists. Results Based on the systematic literature review, 48 articles were identified, showing outcomes of LT in 302 WD patients with neurological symptoms. Of these patients, major improvement was found in 215 cases (71.2%), with no difference in neurological status before and after LT in 21 cases (6.9%). There were 29 deaths (9.6%), neurological worsening in 24 cases (7.9%), and 13 cases (4.3%) were lost to follow-up. Conclusions The results suggest that LT is a promising method of WD management in patients with severe, neurological symptoms, particularly if the patient has not responded to pharmacological de-coppering treatment. Further studies of LT in these patients are warranted.

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Section: Resultsmentioning

confidence: 92%

Section: Resultsmentioning

confidence: 94%

Liver transplantation as a treatment for Wilson’s disease with neurological presentation: a systematic literature review

et al. 2022

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“…The authors speculate that T2 changes observed in these two cases (patients 4 and 6) probably reflected edema rather than necrosis or cystic degeneration. Mocchegiani et al, 33 Litwin et al, 34 Wu et al, 35 and Stracciari et al 36 reported cases of MRI changes that resolved after hepatic transplantation, and more recently, Dusek et al 18 also claimed that T2 hyperintensities should be reversible.…”

Section: Discussionmentioning

confidence: 99%

BrainMRIin the Decision for Liver Transplantation in Pediatric Neurological Wilson's Disease

Pinto

Malaquias

Miranda

et al. 2022

Movement Disord Clin Pract

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Background Neurological Wilson's disease (WD) presentation in the pediatric population is rare, and liver transplantation (LT) in these patients remains controversial. The aim of the present study was to assess the role of brain magnetic resonance imaging (MRI) in predicting reversion of brain lesions and neurological outcomes in pediatric WD patients after LT. Methods Patients with confirmed WD (Leipzig score ≥4), disease onset in pediatric age (<18 years), neurological involvement, and submitted to LT were selected. Clinical records and pre‐ and post‐LT brain MRI were evaluated. Results Six patients met the pre‐defined inclusion criteria, one of whom died shortly after LT and was excluded. The indication for LT was end‐stage liver disease in two patients and neurological worsening despite optimized treatment in three patients. After LT, the neurological picture progressively improved in all patients. Pre‐LT brain MRI showed T1‐weighted hyperintensities in four patients, which quickly resolved afterward. T2‐weighted hyperintensities were observed in four patients before LT, completely resolving in one patient, stabilizing in two, and improving in one after LT. A direct correlation could not be found between clinical and neuroradiological improvement. Progressive clinical improvement was observed even in patients with irreversible brain MRI changes. Conversely, some patients with normal MRI had only slight neurological improvement. Conclusions The pattern of T2‐weighted hyperintensities after LT was unpredictable and did not correlate with neurological outcomes, suggesting that these changes may not entail irreversible clinical damage. Therefore, brain MRI does not seem to have prognostic value for assessing clinical response to LT.

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“…This not only restores copper homeostasis in the liver but also extrahepatic copper metabolism in other tissues as well, most notably in the central nervous system. That is why liver transplant has been also suggested in patients with deterioration neurologic symptoms without any signs of liver failure (61)(62)(63)(64)(65)(66). However, the role of liver transplantation as a treatment of neurologic deficits remains controversial.…”

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confidence: 99%

Clinical management of Wilson disease

Hedera¹

2019

Ann. Transl. Med

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The availability of effective therapies distinguishes Wilson disease (WD) from other inherited neurometabolic diseases. The cause of hepatic, neurologic or psychiatric symptoms is copper overload and subsequent copper toxicity. Diagnosed WD patients require life-long pharmacologic therapy that is focused on reversal of copper overload with maintenance of a long-term negative copper balance. This is associated with the rapid control of free or non-ceruloplasmin bound copper that is mostly responsible for acute cytotoxic effects. Currently available therapies can be divided into chelators and zinc salts. They have different mechanisms of action and the onset of efficacy that influences their selection in acute and chronic stages of therapy. We review the use of D-penicillamine and trientine for chelation therapies, including the required monitoring of therapy for its efficacy and possible overtreatment with iatrogenic copper deficiency.Additionally, the use of zinc salts is also discussed, including a possibility of its use for the initial therapy in an acute stage of the disease. Supportive and symptomatic therapies for liver failure and neuropsychiatric symptoms are also reviewed.

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Liver Transplantation in Neurological Wilson's Disease: Is There Indication? A Case Report

Cited by 12 publications

References 26 publications

Liver transplantation as a treatment for Wilson’s disease with neurological presentation: a systematic literature review

Liver transplantation as a treatment for Wilson’s disease with neurological presentation: a systematic literature review

BrainMRIin the Decision for Liver Transplantation in Pediatric Neurological Wilson's Disease

Clinical management of Wilson disease

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