
Get answers to your Neurosurgery questions by
e-mailing Dr. Schnee.
Dr. Schnee’s Bio | Q&A Archive
Chronic acquired (Non-Wilsonian) hepatocerebral degeneration
Hepatocerebral degeneration is a brain disorder caused by liver damage. This "Non-Wilsonian" type means that the liver damage is not caused by the deposition of copper in the liver, which is a key feature of Wilson's disease. This longstanding (chronic) condition may lead to irreversible neurological (nervous system) symptoms.
Liver damage can lead to the accumulation of ammonia and other toxic materials in the body. This happens when the liver doesn't work properly to break down and release these chemicals The toxic materials can destroy brain tissue, leading to coma, other neurological problems, and potentially death. Specific brain areas, such as the basal ganglia, appear to be more susceptible to injury caused by liver failure. Since the basal ganglia are involved in the regulation of movement, people affected by this disorder may develop tremors, twitching, involuntary movements, and other symptoms.
This type of chronic acquired hepatocerebral degeneration, and the neurological syndrome it causes, may occur in any case of acquired liver failure including hepatitis.
Wijdicks EF, Wiesner RH. Acquired (non-Wilsonian) hepatocerebral degeneration: complex management decisions. Liver Transpl. 2003 Sep;9(9):993-4.
Burkhard PR, Delavelle J, Du Pasquier R, Spahr L. Chronic parkinsonism associated with cirrhosis: a distinct subset of acquired hepatocerebral degeneration. Arch Neurol. 2003 Apr;60(4):521-8.