Liver Transplantation for Neuropsychiatric Wilson Disease

Abstract

Although neuropsychiatric manifestations are prominent in some patients with Wilson disease, there is little published information regarding the efficacy of liver transplantation for these patients. A 22-year-old male with advanced neurological impairment and prominent psychiatric manifestations due to Wilson disease who underwent liver transplantation is presented. After transplantation, the ceruloplasmin and copper studies normalized and eventually the Kayser-Fleischer rings disappeared. Neurological recovery was very slow and incomplete, and his behavioural and personality disorder was entirely unaffected. He committed suicide 43 months post-transplantation. A review of the small number of related published cases in the English language literature shows variable neurological recovery post-transplantation, but the course of psychiatric manifestations is virtually never described. This case suggests that one must be cautious regarding liver transplantation for Wilson disease in patients with prior psychiatric manifestations. Aggressive medical management is likely to be preferable in most cases.

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

“…It has been described both the curative effect of the transplant on neuropsichiatric symptoms [26] and the severe worsening of psychiatric illness [27]. In conclusion from our study emerge some prospectives of resolution also of psychiatric disease accompained by late-postoperative persistent improvement, especially in case of early treatment.…”

Update on the clinical management of Wilson's disease

“…Improvement or even a complete resolution after liver transplantation has been reported in some patients with severe and progressive neurologic deficits who did not respond to conventional chelation therapies and they did not require liver transplant because of failing liver functions 91,94–98. These positive neurologic outcomes are not universal, and no improvements or further progression have been also observed 99,100. That is why liver transplantation to reverse neurologic deficits needs to be indicated with considerable caution and should be reserved for patients who progress in spite of best medical management and their neuroimaging does not show severe structural changes that would make meaningful recovery unlikely.…”

Use of the molecular adsorbents recirculating system as a treatment for acute decompensated wilson disease

“…In patients with only a short history of presentation, stabilizing patients with MARS will allow a more thorough assessment to determine their eligibility for liver transplantation, particularly if there is a concern of preexisting neuropsychiatric involvement that could hinder the outcome. [17][18][19] In our second patient, we believe that the main benefit of MARS was improvement of the patient's hepatic encephalopathy. Given that only 1 treatment was performed, the amount of copper removed would not have been significant.…”