Reviewed By: Pramod Kerkar, MD, FFARCSI

Hepatic encephalopathy which is also known as portosystemic encephalopathy, is a decline in brain function that occurs in patients with severe liver disease such as liver cirrhosis, liver failure and portal hypertension. Since the liver is not able to function properly and remove toxins from systemic blood, the brain is exposed to excessive toxins such as ammonia and manganese. It specifically refers to encephalopathy that occurs in patients with acute liver failure.

Is Hepatic Encephalopathy Fatal?

Acute type of hepatic encephalopathy can be fatal, while chronic hepatic encephalopathy is a slow process. On occasions, even with intense treatment, encephalopathy caused by acute (fulminant) hepatitis is fatal in around 80% patients and individuals suffering from chronic liver failure die in hepatic coma. Therefore, early identification and treatment of underlying precipitating cause can lead to resolution of hepatic encephalopathy in about 80% of the patients.

Treatment is aimed at reduction and/or cessation of the underlying cause such as avoiding sedatives that depress central nervous system, correcting hypovolemia, gastrointestinal bleeding, metabolic disturbances, infection, constipation, hypoxia, hypokalemia or hyponatremia. Antibiotics (rifaxamin, metronidazole or neomycin) may be given to clear out any infection. Since hyperammonemia is considered the most common cause of hepatic encephalopathy, measures should be taken to reduce ammonia accumulation in the brain; this is achieved with zinc therapy. Lactulose is used to reduce nitrogenous load from the intestines. Dietary protein is limited or restricted in patients with hepatic encephalopathy. Liver transplantation is done in cases of liver failure.

What Are The Different Types Of Hepatic Encephalopathy?

Hepatic encephalopathy is sub-classified according to severity and pattern of the underlying disease and can be acute (short term) or chronic (long term). It can even be recurrent and persistent in patients with chronic liver disease.

Acute hepatic encephalopathy can be a result of severe liver disease and occurs in individuals with acute fulminant viral hepatitis (severe viral hepatitis, the onset of which is sudden), toxic hepatitis (this might be caused by an exposure to alcohol, drugs, chemicals or other supplements) and Reye’s syndrome (this condition is rare and causes sudden inflammation of brain and liver; is seen only in children). Acute hepatic encephalopathy may also indicate terminal liver failure.

Chronic hepatic encephalopathy with recurrence is mostly seen in patients with severe liver cirrhosis. Chronic hepatic encephalopathy can also be permanent, which is rare and seen in patients with seizure disorder and spinal cord injury.

Clinical Manifestation Of Hepatic Encephalopathy

The clinical manifestation of hepatic encephalopathy depends on the severity of the underlying disease and may include poor coordination, difficulty thinking, personality and behavior changes, confusion, forgetfulness, poor judgment, difficulty with motor skills such as writing and driving, musty breath, lethargy, drowsiness, anxiety, fatigue, sleep disturbance, confused and slurred speech, tremors, slow movements and if not treated on time then it might even lead to coma and death.

In most of the patients, hepatic encephalopathy may be precipitated or hepatic function may be worsened due to various causes including an infection, gastrointestinal bleeding, excess dietary intake of proteins, excessive intestinal load of nitrogen along with reduced nitrogen excretion, constipation, renal failure, hypoxia, recent surgery, trauma, immunosuppressants, hyponatremia, hypokalemia, diuretics, sedatives such as benzodiazepines and/or barbiturates.

Diagnosis Of Hepatic Encephalopathy

Several tests and imaging are carried out for accurate diagnosis of hepatic encephalopathy. A complete blood test is done to check levels of red blood cells, white blood cells and platelets. Decreased red blood cells will indicate loss of blood and lack of oxygen (hypoxia). Blood tests also indicate levels of sodium, potassium and ammonia and impaired levels indicate liver dysfunction. Liver function tests are also carried out and if liver enzymes are elevated then it is a mark of liver damage and compromised liver function.

MRI scans and CT scans are used to check for brain bleeding, edema and hyperammonemia in relation to hepatic encephalopathy.

The differential diagnosis of hepatic encephalopathy include other causes of hyperammonemia such as valproate induced hyperammonemia, adult onset citrullinemia and late onset of ornithine transcarbamylas deficiency.

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Pramod Kerkar

Written, Edited or Reviewed By:

, MD,FFARCSI

Pain Assist Inc.

Last Modified On: August 11, 2018

This article does not provide medical advice. See disclaimer

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