Hepatic Encephalopathy

What is hepatic encephalopathy?

Hepatic encephalopathy is brain dysfunction caused by liver dysfunction. “Encephalopathy” is brain dysfunction, and “hepatic” means liver-related.

Encephalopathy affects your central nervous system and how you think, feel and act. Symptoms can range from confusion and disorientation to erratic behavior and personality changes. It can get better with treatment, but it can be life-threatening without.

Hepatic encephalopathy is caused by neurotoxins in your blood — substances that are toxic to your brain and nervous system. Normally, your liver filters these toxins from your blood. But if your liver isn’t performing this function as usual, the neurotoxins continue to circulate. Eventually, they enter and affect your brain. This usually happens in advanced liver disease, when liver functions begin to fail.

What are the symptoms of hepatic encephalopathy?

Hepatic encephalopathy can show up in a wide variety of ways. Not everyone will have every symptom. It can affect your:

• Mood and personality.
• Behavior and impulse control.
• Memory, concentration and thinking.
• Consciousness, lucidity and sleep patterns.
• Coordination and motor functions.
• Autonomy and ability to care for yourself.

Symptoms may occur suddenly or come on gradually, and they may be steady and lasting or come and go in episodes. They may get better or worse, depending on your overall health condition. Hepatic encephalopathy can be mild to severe, and symptoms may be barely noticeable to striking. Healthcare providers sometimes group symptoms by their severity as a way of grading, or staging, the disease.

Grading system

The West Haven Criteria is the most common grading system healthcare providers use for hepatic encephalopathy. It grades symptoms on a scale of 0 to 4. Stages 0 to 1 are sometimes called minimal hepatic encephalopathy (MHE) or covert HE. Symptoms in these stages may not be outwardly noticeable. Stages 2 to 4, also called overt hepatic encephalopathy, feature more obvious symptoms.

Symptoms by grade

Possible symptoms by grade include:

Grade 0

• Subtle changes in short-term memory, concentration and reaction time, only recognizable perhaps to you or those closest to you. They might show up on standard neuropsychological tests.

Grade 1

• Mild confusion or forgetfulness.
• Mood swings, like euphoria or anxiety.
• Difficulties adding and subtracting.
• Difficulties with fine motor skills, like writing.
• Sleeping during the day and waking at night.

Grade 2

• Clear personality changes.
• Inappropriate or out-of-character behaviors.
• Lethargy and apathy.
• Disorientation in time (what day or year is it?).
• Slurred speech (dysarthria).

Grade 3

• Slow thinking and sluggish movements.
• Disorientation in space (Where am I?).
• Drowsiness and loss of general awareness.
• Severe confusion, delirium or amnesia.
• Involuntary twitching, tremors or asterixis.

Grade 4

• Total loss of consciousness (coma).

What are the most serious signs to look out for?

Any symptoms of overt hepatic encephalopathy are serious. It’s important to treat the condition as soon as possible to prevent it from worsening or causing permanent damage. Severe hepatic encephalopathy can advance to coma or even death. If you see Grade 3 symptoms, like severe drowsiness, confusion or amnesia, disorientation in space and time, or involuntary movements, treatment is especially urgent.

What causes hepatic encephalopathy?

Hepatic encephalopathy happens when your liver doesn’t filter neurotoxins out of your blood as it normally would. This might be because your liver has temporarily or permanently lost some of its function (liver failure). It might also be because the blood that normally passes through your liver for filtering isn’t passing through it anymore (portosystemic shunt). Sometimes, both things are happening.

The blood your liver filters comes directly from your digestive system, via your portal vein (portal circulation). This blood carries waste products left over from the digestive process, including ammonia and others. Your liver filters them out before sending the blood back out to your body (systemic circulation). If it doesn’t, they build up in your blood and eventually damage your brain tissues. 

Types of hepatic encephalopathy

Healthcare providers define different types of hepatic encephalopathy based on the original cause.

Type A

Type A is caused by acute liver failure. This is a sudden loss of liver function due to a sudden, severe liver injury. Viral infections, autoimmune liver disease and acetaminophen poisoning are common causes. Cerebral edema, swelling in your brain, is a frequent side effect that may contribute to the effects of hepatic encephalopathy. Acute liver failure is an emergency and some cases may require a liver transplant.

Type B

Type B is caused by a portosystemic shunt. A shunt is when blood flow bypasses its normal route through a new, abnormal passageway. A portosystemic shunt is when your portal circulation bypasses your liver on its way to your systemic circulation. Your body may create a shunt if there's a blockage in the normal pathway. Sometimes, a healthcare provider creates one for you for the same reason (transjugular intrahepatic portosystemic shunt, or TIPS). Hepatic encephalopathy is a possible complication in both cases.

Type C

Type C is caused by chronic liver failure. Chronic liver failure is the gradual decline of liver function from chronic liver disease. It’s not reversible. Chronic hepatitis C, chronic alcohol use disorder and metabolic dysfunction-associated steatotic liver disease (MASLD) are common causes. At this stage, extensive scarring in your liver (cirrhosis) may prevent it from working right. Scarring may also compress your portal vein (portal hypertension,) triggering a portosystemic shunt. (This would still be Type C.)

Triggering events

Chronic liver disease works slowly, and you can live with it for a long time before it really begins to affect you. Your body compensates for it until it can’t, which is when liver failure sets in. Healthcare providers have noticed that it’s often a secondary health problem or stress factor that tips the scales enough to trigger hepatic encephalopathy in people with chronic liver disease. These triggers might include:

• Acute gastrointestinal bleeding.
• Acute kidney failure.
• Infection.
• Surgery.
• Constipation.
• Dehydration.
• Electrolyte imbalance.
• Taking the wrong medication.
• An alcohol binge.

How is hepatic encephalopathy diagnosed?

A hepatologist or gastroenterologist usually diagnoses hepatic encephalopathy. Diagnosis involves:

• Evaluating your symptoms. They'll check your vital signs, ask about your symptoms and then try to confirm them with observation and testing.
• Assessing your underlying condition. They’ll need to confirm if you have liver disease (acute or chronic) or a portosystemic shunt, or both.
• Ruling out other causes. Based on their evaluations, they might need to investigate other possible causes of your symptoms.

What labs or tests are used to diagnose hepatic encephalopathy?

You might have:

• Blood tests to check your liver function or ammonia levels.
• A Doppler ultrasound to look at the blood flow through your liver.
• Imaging tests to look at your brain, like a CT scan or MRI.
• An EEG test to measure your brain activity.

What is the treatment for hepatic encephalopathy?

Treatment for hepatic encephalopathy includes:

• Managing any urgent conditions that may have triggered it.
• Treating the underlying condition causing it (liver disease or shunt).
• Reducing the levels of toxins circulating in your blood.

Treatment for the first two will vary, depending on the nature of the conditions affecting you.

For liver failure, whether acute or chronic, there’s often no direct treatment. Healthcare providers try to reduce any stress factors affecting your liver and offer supportive care to address symptoms and complications. If they deem it necessary, they’ll put you on a waiting list for a liver transplant. For a portosystemic shunt, providers may use interventional radiology techniques to redirect your blood flow.

Reducing neurotoxins in your blood

Healthcare providers target the neurotoxins in your blood at their source, which is in your gut. As many of these toxins are byproducts of certain bacteria in your gut, providers may prescribe antibiotics to reduce the number of these bacteria. They combine these with an osmotic laxative to speed up the passage of food waste through your gut and reduce how much it can absorb into your bloodstream.

Medications

The most common medications healthcare providers prescribe are:

• Rifaximin. This antibiotic is nonabsorbable by your intestines. Neomycin is an alternative.
• Lactulose. This synthetic laxative draws water into your bowel. MiraLAX® is an alternative.

Supplemental medications include:

• Branched-chain amino acids. Branched-chain amino acid supplements can help build muscle and clear ammonia from your blood through your muscle tissue.
• Probiotics. Probiotics can encourage the more helpful types of gut bacteria to compete with the less helpful types that produce neurotoxins.

Hemodialysis

In the case of acute liver failure, which is an emergency, hemodialysis may be helpful for treating Type A hepatic encephalopathy. This is a way of filtering toxins from your blood, using a dialysis machine.

Can your brain recover from hepatic encephalopathy?

With timely treatment, recovery is possible, but how much your brain recovers can vary. In general, if the causes affecting you are brief, and if they’re relatively mild, you’re more likely to recover fully. If you have a permanent condition, you’ll need ongoing therapy to manage your neurotoxin levels. Even with treatment, chronic liver failure will continue, and hepatic encephalopathy could relapse or recur.

What is life expectancy with hepatic encephalopathy?

Hepatic encephalopathy is one factor affecting your life expectancy. The conditions causing it are others. Healthcare providers can’t predict life expectancy in every scenario. For example, if you have hepatic encephalopathy with acute liver failure, this is an emergency that you may or may not survive. Having hepatic encephalopathy makes things more serious. But if you do survive it, your life expectancy afterward might be normal.

On the other hand, if you have chronic liver failure, this is eventually fatal regardless of hepatic encephalopathy. Having hepatic encephalopathy makes things more serious. At this stage, most models predict a life expectancy of up to two years. Roughly half of people with both conditions live a full year after diagnosis, and half of those live another full year. But this changes if you have a liver transplant.

How do I take care of myself while living with chronic hepatic encephalopathy?

If you have persistent or recurrent hepatic encephalopathy, taking care of yourself is more important than ever. You might also need more help than ever to do it. You’ll do better in the long run if you can:

• Stay in touch. Your healthcare team will want to keep a close eye on your symptoms and your response to treatment. For some people with hepatic encephalopathy, keeping track of symptoms and appointments might be difficult. It’s a good idea to keep a friend, family member or caretaker in the loop to support you. They can take notes and help you remember things.
• Take care of the basics. Take care of your general health by making sure you get adequate nutrition and exercise. A registered dietitian can assess your nutritional needs and help design a personalized eating plan that works for you. Physical activity can also help preserve your brain health. If you’re having difficulties coordinating movements, a physical therapist can help.
• Accept help. Hepatic encephalopathy can become a disability. You might need to accept a little help with the practical parts of life — or even a lot. In addition to your personal support network, you might need to consult with a social worker or home care nurse to help take care of these needs. You should also stop driving, even if your condition is minimal or goes away at times.
• Find a living liver donor. The competition for donor livers is steep, and not everyone gets one when they need it. But you can skip the waiting list if you’re able to find a living volunteer to donate part of their liver. It only takes a portion of a healthy liver from a living donor to grow into a full-size liver within your body. Your donor’s liver will also grow back to full size for them.