Liver damage can lead to the accumulation of ammonia and other toxic materials when the organ doesn't function properly to metabolize and excrete them. These chemicals can destroy brain tissue, leading to coma 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 neurologic symptoms.

Hepatocerebral degeneration and the neurological syndrome it causes may occur in any case of acquired liver failure including hepatitis.

Symptoms may include:

• Tremor (shaking of arms, head)
• Ataxia (unsteady walking)
• Difficulty walking
• Chorea (uncontrolled body movements)
• Myoclonus (spasm of muscles)
• Impairment of intellectual function
• Coma
• Jaundice
• Rigidity
• Gastrointestinal bleeding from esophageal varices
• Ascites (fluid in the abdomen, causing swelling)

A thorough neurological examination may reveal signs of dementia, involuntary movements, and gait instability.�Laboratory studies may show an elevated ammonia level and abnormal liver function tests.

Imaging studies of the head may not show any abnormalities or may show non-specific changes in the basal ganglia. An EEG test may show diffuse slowing consistent with an abnormal metabolic process.

Treatment is aimed at reducing the metabolic by-products that accumulate due to liver failure. This may include laxatives such as lactulose that lower the level of ammonia in the blood. There is no specific treatment of the neurologic syndrome, because it is due to the liver disease. A liver transplant may cure the liver disease, but the neurological symptoms may or may not be reversible.

The patient may continue to get worse and even die�if a liver transplant is not performed.�If a transplant occurs early in the course of the disease, the neurological syndrome may be reversible.

Complications include hepatic coma and severe brain damage.

Call your provider if you have any signs or symptoms of liver disease.

It is not possible to prevent all forms of liver disease, but alcoholic and viral hepatitis are potentially preventable. Not drinking, drinking only in moderation, and avoidance of risky behavior such as IV drug use (particularly sharing needles and other injection paraphernalia) and unprotected sexual encounters may significantly decrease the risk of acquiring alcoholic or viral 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.