Hepatocerebral degeneration

Definition

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

Alternative Names

Chronic acquired (Non-Wilsonian) hepatocerebral degeneration

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.

Symptoms

Symptoms may include:

• Difficulty walking
• Impaired intellectual function
• Jaundice
• Muscle spasm (myoclonus)
• Rigidity
• Shaking of arms, head (tremor)
• Twitching
• Uncontrolled body movements (chorea)
• Unsteady walking (ataxia)

Signs and tests

Signs include:

• Coma
• Fluid in the abdomen that causes swelling (ascites)
• Gastrointestinal bleeding from enlarged veins in the food pipe (esophageal varices)

A nervous system (neurological) examination may show signs of:

• Dementia
• Involuntary movements
• Walking instability

Laboratory tests may show a high ammonia level in the bloodstream and abnormal liver function.

Other tests may include:

• MRI of the head
• EEG (may show general slowing of brain waves)
• CT scan of the head

Treatment

Treatment helps reduce the toxic chemicals that build up from liver failure. It may include laxatives such as lactulose, which lower the level of ammonia in the blood.

A treatment called branched-chain amino acid therapy has been reported in a few patients to improve symptoms and reverse the brain damage from this condition.

There is no specific treatment for the neurologic syndrome, because it is caused by irreversible liver damage. A liver transplant may cure the liver disease. However, this operation may not reverse the symptoms of brain damage.

Expectations (prognosis)

This is a long-term (chronic) condition that may lead to irreversible nervous system (neurological) symptoms.

The patient may continue to get worse and may die without a liver transplant. If a transplant is done early in the course of the disease, the neurological syndrome may be reversible.

Complications

Complications include:

• Hepatic coma
• Severe brain damage

Calling your health care provider

Call your health care provider if you have any symptoms of liver disease.

Prevention

It is not possible to prevent all forms of liver disease. However, alcoholic and viral hepatitis may be prevented.

To reduce your risk of getting alcoholic or viral hepatitis:

• Avoid risky behaviors, such as IV drug use or unprotected sex
• Don't drink, or drink only in moderation

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.