Different neurological outcome of liver transplantation for Wilson's disease in two homozygotic twins

Abstract Wilson's disease is a genetic disorder characterized by accumulation of copper in many organs and tissues. Phenotypic manifestations are wide-ranging from neuropsychiatric disorders, to severe liver disease requiring liver transplantation. Clinical presentation is not often related to...

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Bibliographic Details
Published in:Clinical neurology and neurosurgery 2007-01, Vol.109 (1), p.71-75
Main Authors: Senzolo, Marco, Loreno, Massimiliano, Fagiuoli, Stefano, Zanus, Giacomo, Canova, Daniele, Masier, Annalisa, Russo, Francesco Paolo, Sturniolo, Giacomo Carlo, Burra, Patrizia
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Copper
Disease
Diseases in Twins
Fatal Outcome
Hepatolenticular Degeneration - surgery
Histology
Homozygotic twins
Humans
Liver cirrhosis
Liver disease
Liver Transplantation
Medical imaging
Medical sciences
Middle Aged
Middle schools
Mutation
Neurological disease
Neurology
Neurosurgery
Neurotoxicity
Siblings
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Transplants & implants
Treatment Outcome
Twins, Monozygotic
Wilson's disease
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Summary:Abstract Wilson's disease is a genetic disorder characterized by accumulation of copper in many organs and tissues. Phenotypic manifestations are wide-ranging from neuropsychiatric disorders, to severe liver disease requiring liver transplantation. Clinical presentation is not often related to the genetic defect and siblings may have different type of disease. Liver transplantation is indicated for all patients with Wilson's disease and decompensated liver cirrhosis unresponsive to medical therapy, but its efficacy in resolving the neurological symptoms is still controversial, because as far now, very different outcomes have been reported. We describe here on the exceptional case of two homozygotic twins, both with liver cirrhosis due to Wilson's disease, one of them with severe neuropsychiatric involvement, who both underwent liver transplantation and subsequently had very different outcome despite same genetic background. The presence of neurological clinical manifestations in Wilson's disease should recommend caution indicating liver transplantation, because irreversible brain damage may exist.
ISSN:0303-8467
1872-6968
DOI:10.1016/j.clineuro.2006.01.008