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    Hepatocerebral degeneration

    Chronic acquired (Non-Wilsonian) hepatocerebral degeneration

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

    Causes

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

    Liver damage can lead to the buildup of ammonia and other toxic materials in the body. This happens when the liver doesn't work properly to break down and eliminate these chemicals. The toxic materials can damage 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 control 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)

    Exams 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 antibiotics or a medication such as lactulose, which lowers the level of ammonia in the blood.

    A treatment called branched-chain amino acid therapy may also improve symptoms and reverse 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.

    Outlook (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.

    Possible Complications

    Complications include:

    • Hepatic coma
    • Severe brain damage

    When to Contact a Medical Professional

    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

    Garcia-Tiso G. Cirrhosis and its sequellae. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 157.

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    • Liver anatomy

      illustration

      • Liver anatomy

        illustration

      Tests for Hepatocerebral degeneration

        Review Date: 7/7/2010

        Reviewed By: David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, California. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

        The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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