Hepatic encephalopathy (portosystemic encephalopathy, liver encephalopathy, or hepatic coma) is deterioration of brain function that occurs because toxic substances normally removed by the liver build up in the blood and reach the brain.

• Hepatic encephalopathy may be triggered by bleeding in the digestive tract, an infection, failure to take drugs as prescribed, or another stress in people who have a long-standing (chronic) liver disorder.
• People become confused, disoriented, and drowsy, with changes in personality, behavior, and mood.
• Doctors base the diagnosis on symptoms, results of the examination, and response to treatment.
• Eliminating the trigger, eating less meat, and taking lactulose (a laxative) and rifaximin (an antibiotic) may help symptoms resolve.

Substances absorbed into the bloodstream from the intestine pass through the liver, where toxins are normally removed. Many of these toxins (such as ammonia) are normal breakdown products of the digestion of protein. In hepatic encephalopathy, toxins are not removed because liver function is impaired. Also, some toxins may completely bypass the liver through abnormal connections (called collateral vessels) that form between the portal venous system (which supplies blood to the liver) and the general circulation. These vessels form as a result of liver disease and portal hypertension (see Manifestations of Liver Disease: Portal Hypertension). A procedure used to treat portal hypertension (called portosystemic shunting) may also enable toxins to bypass the liver. Whatever the cause, the outcome is the same: Toxins can reach the brain and affect its function. Exactly which substances are toxic to the brain is not known. However, high levels of protein breakdown products in the blood, such as ammonia, appear to play a role.

In people with a long-standing (chronic) liver disorder, encephalopathy is usually triggered by an event such as

• Having an infection
• Not taking drugs as prescribed
• Bleeding in the digestive tract, such as bleeding from enlarged, twisted (varicose) veins in the esophagus (esophageal varices)
• Eating too much animal protein, particularly in red meat
• Being dehydrated
• Having an electrolyte imbalance
• Taking certain drugs, especially alcohol, some sedatives, pain relievers (analgesics), or diuretics

Symptoms

Symptoms are those of impaired brain function, especially reduced alertness and confusion. In the earliest stages, subtle changes appear in logical thinking, personality, and behavior. The person's mood may change, and judgment may be impaired. Normal sleep patterns may be disturbed. At any stage of encephalopathy, the person's breath may have a musty sweet odor.

As the disorder progresses, people cannot hold their hands steady when they stretch out their arms, resulting in a crude flapping motion of the hands (asterixis). Also, people usually become drowsy and confused, and movements and speech become sluggish. Disorientation is common. Less often, people with encephalopathy become agitated and excited or have seizures. Eventually, they may lose consciousness and lapse into a coma.

Diagnosis

Diagnosis is based mainly on symptoms, results of the examination, and response to treatment. Doctors ask about possible triggers for the encephalopathy (such as an infection or a drug) to identify possible causes. They do blood tests to identify triggers, particularly disorders that can be treated (such as infections), and to confirm the diagnosis. The ammonia level is also measured. The level is usually abnormally high (indicating liver malfunction), but measuring the level is not always a reliable way to diagnose encephalopathy.

In older people, hepatic encephalopathy may be more difficult to recognize in its early stages because its initial symptoms (such as disturbed sleep patterns and mild confusion) may be attributed to dementia or erroneously labeled as delirium (see Delirium and Dementia: Overview of Delirium and Dementia).

Treatment

Doctors try to eliminate any triggers for the encephalopathy, such as an infection or a drug.

Doctors also try to eliminate toxic substances from the intestine because these substances can contribute to encephalopathy. They may use one or more of the following measures:

• Diet: Usually, doctors advise people to limit how much meat and other animal protein they eat. Toxins (such as ammonia) are formed during the digestion of animal protein, particularly in red meat but also in fish, cheese, and eggs. To make sure people get enough protein, doctors advise them to eat more foods that contain vegetable protein, such as soy protein.
• Lactulose: Lactulose, a synthetic sugar taken by mouth, acts as a laxative, speeding up the passage of food. Because of this and other effects, it decreases the amount of ammonia that is absorbed by the body.
• Enemas: Cleansing enemas may be given to speed up the passage of food and eliminate toxins.
• Antibiotics: Doctors may prescribe antibiotics (such as rifaximin) that are taken by mouth but are not absorbed from the intestine. These antibiotics remain in the intestine, where they can reduce the number of bacteria that form toxins during digestion.

With treatment, hepatic encephalopathy is frequently reversible. In fact, complete recovery is possible, especially if the encephalopathy was triggered by a reversible cause. However, people with a chronic liver disorder are susceptible to future episodes of encephalopathy.

Last full review/revision August 2012 by Steven K. Herrine, MD