Chronic acquired (Non-Wilsonian) hepatocerebral degeneration

Definition

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.

Alternative Names

Causes

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

Symptoms

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 (enlarged veins in the food pipe)
• Ascites (fluid in the abdomen, causing swelling)

Exams and Tests

A thorough neurological examination may show signs of dementia, involuntary movements, and walking instability. Laboratory tests may show a high ammonia level in the bloodstream and abnormal liver function test results.

A head CT scan or a cranial MRI may not show any abnormalities, or may show non-specific changes in the basal ganglia region of the brain. An EEG test may show general slowing of brain waves, which is consistent with an abnormal metabolic (chemical breakdown) process.

Treatment

Treatment is aimed at reducing the toxic chemicals that build-up due to liver failure. This may include laxatives such as lactulose, which lower the level of ammonia in the blood.

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

Outlook (Prognosis)

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.

Possible Complications

Complications include hepatic coma and severe brain damage.

When to Contact a Medical Professional

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

Prevention

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 avoiding risky behavior (such as IV drug use or unprotected sex) 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.