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Hepatocerebral degeneration - Overview

Alternative Names

Chronic acquired (Non-Wilsonian) hepatocerebral degeneration

Definition of Hepatocerebral degeneration:

Hepatocerebral degeneration is a brain disorder that occurs in people with liver damage.

Causes, incidence, and risk factors:

This condition may occur in any case of acquired liver failure, including hepatitis.

Liver damage can lead to the build-up 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.

Specific areas of the brain, such as the basal ganglia, are more likely to be injured from liver failure. The basal ganglia help regulate movement.

This condition is the "Non-Wilsonian" type. This means that the liver damage is not caused by copper deposits in the liver, which is a key feature of Wilson's disease.

Liver anatomy

Reviewed last on: 7/15/2008
Luc Jasmin, MD, PhD, Department of Neurosurgery and Gene Therapeutics Research Institute, Cedars-Sinai Medical Center, Los Angeles, CA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

References

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.

Ferrante MA. Endogenous Metabolic Disorders. In: Goetz CG. Goetz: Textbook of Clinical Neurology. 3rd ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 38.

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