Wilson’s disease — hepatocerebral dystrophy

The article presents a detailed review of modern ideas on Wilson’s disease – hepatocerebral dystrophy. The definition, terminology, history of the study of the disease are stated. Special attention is paid to the analysis of the pathogenesis of hepatocerebral dystrophy, including the genetic basis of its development, the disturbance of copper metabolism. The clinical picture is thoroughly described, taking into account the characteristics of liver lesion and extrahepatic manifestations, diagnostics, classification. Particular attention is paid to the means of pathogenetic and symptomatic treatment.

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Resolved Psychosis after Liver Transplantation in a Patient with Wilson’s Disease

Clinical Practice and Epidemiology in Mental Health ◽

10.2174/1745017901107010182 ◽

2011 ◽

Vol 7 (1) ◽

pp. 182-184 ◽

Cited By ~ 16

Author(s):

Orazio Sorbello ◽

Daniela Riccio ◽

Margherita Sini ◽

Mauro Carta ◽

Luigi Demelia

Keyword(s):

Liver Transplantation ◽

Liver Function ◽

Wilson’S Disease ◽

Psychiatric Symptoms ◽

Copper Metabolism ◽

Wilson's Disease ◽

Hepatic Disease ◽

Behavioral Symptoms ◽

Psychiatric Disease ◽

History Of

A psychiatric involvement is frequently present in Wilson’s disease. Psychiatric symptoms are sometimes the first and only manifestation of Wilson’s disease. More often a psychiatric involvement is present beside a neurologic or hepatic disease. We describe the case of a 18 years-old male patient who shows a clinic and laboratoristic pattern of cirrhosis and an history of subchronic hallucinatory psychosis, behavioral symptoms and mood disturbances with depressed mood. He hadn’t familiar history of liver or psychiatric disease. Laboratory and imaging tests confirmed the diagnosis of Wilson’s disease with psichiatric involvement. After liver transplantation copper metabolism and liver function normalised and we noticed no recurrency of the psichiatric illness. Very few cases of psychiatric improvement after orthotopic liver transplantation (OLT) has been described until now.

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The Need for Consensus About Liver Transplantation For Patients With Neuropsychiatric Wilson’s Disease

Progress in Transplantation ◽

10.1177/15269248211002806 ◽

2021 ◽

pp. 152692482110028

Author(s):

Alberto Ferrarese ◽

Patrizia Burra

Keyword(s):

Liver Transplantation ◽

Patient Outcomes ◽

Wilson’S Disease ◽

Therapeutic Option ◽

Copper Metabolism ◽

Wilson's Disease ◽

Published Data ◽

Neurological Improvement ◽

Effective Therapeutic Option

Liver transplantation is considered an effective therapeutic option for Wilson’s disease (WD) patients with hepatic phenotype, since it removes the inherited defects of copper metabolism, and is associated with excellent graft and patient outcomes. The role of liver transplantation in WD patients with mixed hepatic and neuropsychiatric phenotype has remained controversial over time, mainly because of high post-operative complications, reduced survival and a variable, unpredictable rate of neurological improvement. This article critically discusses the recently published data in this field, focussing in more detail on isolated neuropsychiatric phenotype as a potential indication for liver transplantation in WD patients.

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Effective Treatment of Neurological Symptoms with Normal Doses of Botulinum Neurotoxin in Wilson’s Disease: Six Cases and Literature Review

Toxins ◽

10.3390/toxins13040241 ◽

2021 ◽

Vol 13 (4) ◽

pp. 241

Author(s):

Harald Hefter ◽

Sara Samadzadeh

Keyword(s):

Botulinum Neurotoxin ◽

Wilson’S Disease ◽

Animal Experiments ◽

Symptomatic Treatment ◽

Wilson's Disease ◽

University Hospital ◽

Laboratory Findings ◽

Dose Information ◽

High Doses ◽

The University

Background: Recent cell-based and animal experiments have demonstrated an effective reduction in botulinum neurotoxin A (BoNT/A) by copper. Aim: We aimed to analyze whether the successful symptomatic BoNT/A treatment of patients with Wilson’s disease (WD) corresponds with unusually high doses per session. Methods: Among the 156 WD patients regularly seen at the outpatient department of the university hospital in Düsseldorf (Germany), only 6 patients had been treated with BoNT/A during the past 5 years. The laboratory findings, indications for BoNT treatment, preparations, and doses per session were extracted retrospectively from the charts. These parameters were compared with those of 13 other patients described in the literature. Results: BoNT/A injection therapy is a rare (<4%) symptomatic treatment in WD, only necessary in exceptional cases, and is often applied only transiently. In those cases for which dose information was available, the dose per session and indication appear to be within usual limits. Conclusion: Despite the evidence that copper can interfere with the botulinum toxin in preclinical models, patients with WD do not require higher doses of the toxin than other patients with dystonia.

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