Skip to main content Accessibility help

We use cookies to distinguish you from other users and to provide you with a better experience on our websites. Close this message to accept cookies or find out how to manage your cookie settings.

Close cookie message
×
Hostname: page-component-586b7cd67f-vdxz6 Total loading time: 0 Render date: 2024-11-27T20:33:05.194Z Has data issue: false hasContentIssue false

Chapter 14 - The Liver–Brain Relationship in Wilson Disease

Published online by Cambridge University Press:  27 July 2023

Robert Laureno
Robert Laureno
Affiliation:
Georgetown University, Washington DC
Get access

Summary

The neuropathology of Wilson disease resembles that of chronic nonfamilial hepatocerebral disease. The neurological features of the hereditary disease also resemble those of the nonfamilial disease. Liver transplantation improves both the lesions on MRI scans and the neurologic disorder of Wilson disease. For these reasons and others there is no reason to invoke copper toxicity in the pathogenesis of Wilsonian hepatocerebral disease.

Keywords

Wilson diseaseHepatocerebral degenerationCopper neurotoxicity
Type
Chapter
Information
Insights into Clinical Neurology , pp. 72 - 74
Publisher: Cambridge University Press
Print publication year: 2023

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

References

Schiff, E. R., Sorrell, M. F., Maddrey, W. C.. Schiff’s Diseases of the Liver. Chichester, John Wiley & Sons, 2012.Google Scholar
Victor, M., Adams, R. D., Cole, M.. The acquired (non-Wilsonian) type of chronic hepatocerebral degeneration. Medicine (Baltimore) 1965; 44: 345–396.CrossRefGoogle ScholarPubMed
Waggoner, R. W., Malamud, N.. Wilson’s disease in the light of cerebral changes following ordinary acquired liver disorders. J Nerv Ment Dis 1942; 96: 410–423.Google Scholar
Victor, M.. Neurologic changes in liver disease. Res Publ Assoc Res Nerv Ment Dis 1974; 53: 1–12.Google ScholarPubMed
Dusek, P., Litwin, T., Czlonkowska, A.. Neurologic impairment in Wilson disease. Ann Transl Med 2019; 7: S64.CrossRefGoogle ScholarPubMed
Poujois, A., Sobesky, R., Meissner, W. G. et al. Liver transplantation as a rescue therapy for severe neurologic forms of Wilson disease. Neurology 2020; 94: e2189–e2202.CrossRefGoogle ScholarPubMed
Compston, A.. Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver, by Kinnier, S. A. Wilson, (from the National Hospital, and the Laboratory of the National Hospital, Queen Square, London) Brain 1912: 34; 295–509. Brain 2009; 132: 1997–2001.Google Scholar
Shulman, L. M., Minagar, A., Weiner, W. J.. Reversal of parkinsonism following liver transplantation. Neurology 2003; 60: 519.CrossRefGoogle ScholarPubMed
Naegele, T., Grodd, W., Viebahn, R. et al. MR imaging and (1)H spectroscopy of brain metabolites in hepatic encephalopathy: time-course of renormalization after liver transplantation. Radiology 2000; 216: 683–691.CrossRefGoogle ScholarPubMed
Tanner, M. S.. Indian childhood cirrhosis and Tyrolean childhood cirrhosis. Disorders of a copper transport gene? Adv Exp Med Biol 1999; 448: 127–137.CrossRefGoogle ScholarPubMed
Fuentealba, I. C., Aburto, E. M.. Animal models of copper-associated liver disease. Comp Hepatol 2003; 2: 5.CrossRefGoogle ScholarPubMed

Save book to Kindle

To save this book to your Kindle, first ensure [email protected] is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×