Chronic acquired (non-Wilsonian) hepatocerebral degeneration

Alternative names
Hepatocerebral degeneration

Definition
Hepatocerebral degeneration is a brain disorder caused by liver damage. It is usually a chronic condition that may lead to irreversible neurological symptoms.

Causes, incidence, and risk factors

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 alcoholic hepatitis, autoimmune hepatitis, viral hepatitis, and others.

Symptoms

Symptoms may include:

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

Signs and tests

A thorough neurological examination may reveal signs of dementia, involuntary movements, and gait instability. Other physical signs of liver disease such as ascites (distended abdomen filled with fluid) and jaundice (yellow color to the skin and whites of eyes) may be evident. 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. Diagnosis is based on these neurological findings in seen in the setting of known liver impairment.

Treatment
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 be curative of the liver disease, but the neurological symptoms may or may not be reversible.

Expectations (prognosis)
The natural history of this illness is progressive worsening with eventual death if the liver is not replaced by transplant. If a transplant occurs early in the course of the disease, the neurological syndrome may be reversible.

Complications
Complications include hepatic coma and severe brain damage.

Calling your health care provider
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 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.

Johns Hopkins patient information

Last revised: December 7, 2012
by Sharon M. Smith, M.D.

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