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Hepatic Encephalopathy(Encephalopathy, Hepatic; Portal-Systemic Encephalopathy; Encephalopathy, Portal-Systemic)

Pronounced: MET-a-bol-ik En-SEF-a-lo-PATH-ee

Definition

Hepatic encephalopathy is a problem with the brain that is caused by liver disease. The problem may be temporary or permanent. People with a liver disease called cirrhosis are most commonly affected.

Oxygen and Blood Flow to the Brain

oxygen brain lungs

If the liver is not working properly, toxins can build up in the blood. The toxic blood travels to the brain, affecting the brain's ability to function.

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Causes

A liver with disease cannot filter the harmful items in blood. These toxins build up in the blood, which reaches the brain and affects the brain’s ability to work properly.

Risk Factors

Factors that increase your chances of developing hepatic encephalopathy include:

  • Certain conditions that affect the levels of fluids and electrolytes such as hyponatremia and hyperkalemia
  • Kidney failure
  • Infections
  • Gastrointestinal bleeding
  • Certain medicines such as sedatives and anti-epileptics
  • Constipation
  • Cirrhosis
  • Hepatitis—infectious or autoimmune
Symptoms

In the early stages, you may not notice any symptoms. As the condition progresses, symptoms may include:

  • Changes in behavior and personality
  • Shortened attention span
  • Depression or anxiety
  • Insomnia
  • Fatigue
  • Forgetfulness
  • Disorientation
  • Slurred speech
  • Tremor , particularly a flapping tremor of the hands
  • Confusion
  • Loss of consciousness

If you have liver problems and any of the above symptoms, call your doctor right away.

These problems can develop quickly. They may resolve when the underlying condition is reversed, but immediate treatment is needed.

Diagnosis

This condition can be serious. It can quickly become an emergency. You may need to be hospitalized. Doctors will do an exam to assess the condition of your nervous system.

To assess your liver and kidneys your doctor may order:

  • Liver function tests
  • Kidney function tests
  • Imaging tests of the liver

Your doctor may also order tests to assess your brain and nervous system such as:

  • Images of the brain with MRI scan or CT scan
  • EEG to look at brain waves for evidence of encephalopathy and/or seizures
  • Lumbar puncture to look for other causes of the encephalopathy
Treatment
Hospitalization and Emergency Care

The initial treatment will focus on treating and trying to reverse the underlying problems. If possible, toxins in your blood will be removed or neutralized.

Medications

Medications may be used to:

  • Neutralize toxins in the intestine such as ammonia
  • Prevent intestinal bacteria from creating ammonia
  • Remove blood from the intestines
  • Reduce ammonia production by intestinal bacteria
  • Treat the condition that started the encephalopathy
  • Reduce recurrence
Dietary Restrictions

Your doctor may recommend changes in your diet. Tube feeding may be needed to supply nutrients, especially in the case of coma.

Transplantation

If this condition is due to liver failure, you may need a liver transplant.

Prevention

To help reduce your chance of getting this condition, take the following steps:

  • Get early treatment for liver problems.
  • If you have a disease such as cirrhosis, see your doctor regularly.
  • Follow your doctor's instructions regarding medication. Avoid overdosing.
  • Avoid being exposed to poisons or toxins.
  • Avoid excessive alcohol.
  • Do not use IV drugs.

RESOURCES:

National Digestive Diseases Information Clearinghouse
http://digestive.niddk.nih.gov

National Institute of Neurological Disorders and Stroke
http://www.ninds.nih.gov

CANADIAN RESOURCES:

Canadian Liver Foundation
http://www.liver.ca

Health Canada
http://www.hc-sc.gc.ca

References:

Bernuau J. Acute liver failure: avoidance of deleterious co-factors and early specific medical therapy for the liver are better than late intensive care for the brain (review). J Hepatol. 2004;41:152-155.

Butterworth RF. Role of circulating neurotoxins in the pathogenesis of hepatic encephalopathy: potential for improvement following their removal by liver assist devices. Liver Int. 2003;23(suppl 3):5-9. Review.

Hepatic encephalopathy. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated August 7, 2012. Accessed May 20, 2013.

Highleyman L. Hepatitis C. HCV Advocate website. Available at: http://www.hcvadvocate.org/hepatitis/hepC/hepatic_encephalopathy.html. Published 2002. Accessed May 20, 2013.

Juretschke L. Kernicterus: still a concern. Neonatal Netw. 2005;24:7-9.

Lizardi-Cervera J, Almeda P, Guevara L, et al. Hepatic encephalopathy: a review. Ann Hepatol. 2003;2:122-130. Review.

Metabolic encephalopathy. EBSCO DynaMed website. Available at: https://dynamed.ebscohost.com/about/about-us. Updated August 11, 2009. Accessed May 20, 2013.

Siegal GJ, Agranoff BW, Albers RW, Uhler MD. Metabolic encephalopathies. In: Basic Neurochemistry: Molecular, Cellular, and Medical Aspects. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1999.

Toftengi F, Larsen F. Management of patients with fulminant hepatic failure and brain edema. Metab Brain Dis. 2004;19:207-214.

4/2/2010 DynaMed's Systematic Literature Surveillance https://dynamed.ebscohost.com/about/about-us: FDA approves new use of Xifaxan for patients with liver disease. US Food and Drug Administration website. Available at: http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm206104.htm. Published March 24, 2010. Accessed April 2, 2010.



Last reviewed May 2013 by Rimas Lukas, MD; Michael Woods, MD

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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