Acquired (non-Wilsonian) hepatocerebral degeneration: complex management decisions.
Portal systemic encephalopathy, in its many guises, can be reversible after medical management or liver transplantation. It is much less certain whether patients with a longstanding neurodegenerative syndrome (known in the medical vernacular as acquired hepatocerebral degeneration) can improve. Impressive neuroimaging abnormalities have been reported in this entity.(1) However, the combination of a severe disabling neurologic deficit and widespread magnetic resonance abnormalities tempers the enthusiasm of transplant surgeons to proceed with liver transplantation. In our liver transplantation program, we were recently confronted with such a case, and present herein not only the characteristic magnetic resonance imaging findings but also some of the dilemmas of management.
Wijdicks
Eelco F m
EF
Department of Neurology and Medicine, Mayo Clinic, Rochester, MN 55905, USA. wijde@mayo.edu
Wiesner
Russell H
RH
D001921:Brain / Q000473:pathology*; D005260:Female; D006527:Hepatolenticular Degeneration / Q000473:pathology* / Q000601:surgery*; D006801:Humans; D016031:Liver Transplantation; D008279:Magnetic Resonance Imaging; D008875:Middle Aged