Diagnosing Hepatic Encephalopathy

Hepatic Encephalopathy, sometimes referred to as portosystemic encephalopathy or PSE, is a condition that causes temporary worsening of brain function in people with advanced liver disease. When your liver is damaged it can no longer remove toxic substances from your blood. These toxins build up and can travel through your body until they reach your brain, causing mental and physical symptoms of HE.

Hepatic Encephalopathy often starts slowly, and at first you may not be aware you have it. The stages of HE span from mild to severe and symptoms vary depending on how bad your liver disease is. It’s important for you and your family to become familiar with the signs of Hepatic Encephalopathy so you can tell your doctor right away if you think you may have it. Prompt identification and treatment of HE is essential to stopping the progression of this condition. Progression may not stop despite best efforts.

Read on to learn more about HE, its symptoms, stages and treatment. Being a fully-informed participant in your medical care is an important factor in staying as healthy as possible. The American Liver Foundation is here to answer all your questions about HE, one step at a time.

You only have one liver and it’s one of the largest and most important organs in your body. Your liver performs many jobs to keep you healthy including filtering everything that enters your body, such as food, drink and medicine.

After your intestines break down things that you eat or drink into their component parts, your liver is responsible for separating the good stuff from the bad. It sends the good things – such as vitamins and nutrients – into your bloodstream for your body to use and changes the bad or toxic things, making them harmless.

HE is most often seen in people with chronic liver disease. Anything that damages your liver over many years – such as long-term alcohol abuse or chronic hepatitis – can cause it to form scar tissue. As hard scar tissue replaces soft, healthy tissue two things begin to happen:

• The scarred tissue cannot carry out the process of changing toxins into harmless substances like a healthy liver normally would.
• The scarred tissue can block the flow of blood through the liver causing high blood pressure in the veins in and around your liver (called the portal venous system). This condition is known as portal hypertension.

When your liver can’t filter toxins from your blood or when blood flow through your liver is blocked, toxins build up in your bloodstream and can get into your brain.

Ammonia, which is produced by your body when proteins are digested, is one of the toxins that’s normally made harmless by your liver. But when ammonia, or a range of other toxic substances, build up in your body when your liver isn’t working well, it may affect your brain and cause HE.

Liver failure occurs if the liver has lost all of its function due to cirrhosis caused by different liver diseases.

What are the common causes of cirrhosis?

• Hepatitis B & C
• Alcohol-related Liver Disease
• Non-alcoholic Fatty Liver Disease (NAFLD) & Non-alcoholic Steatohepatitis (NASH)
• Autoimmune Hepatitis
• Bile duct disease such as Primary Biliary Cirrhosis (PBC) and Primary Sclerosing Cholangitis (PSC)
• Metabolic diseases such as Hemochromatosis, Wilson disease and Alpha-1 antitrypsin deficiency

Liver failure is a life-threatening condition that requires hospitalization. Many people do not have any liver damage symptoms until serious liver problems have already developed slowly and silently.

What are the symptoms and signs of liver failure caused by cirrhosis?
For some people, cirrhosis is diagnosed unintentionally. Cirrhosis often does not have any specific signs and symptoms in the early stage. The non-specific symptoms may be:

• Nausea
• Loss of appetite
• Fatigue
• Diarrhea
• Jaundice (yellowing of the skin and whites of the eyes)

As cirrhosis progresses, symptoms and complications can appear that make it apparent that the liver is not doing well. These could be the symptoms of Hepatic Encephalopathy (HE) and other complications due to cirrhosis. In addition to Hepatic Encephalopathy (HE), following complications are signs of liver damage or cirrhosis:

• Fluid build up and painful swelling of the legs (edema) and abdomen (ascites)
• Bruising and bleeding easily
• Enlarged veins in the lower esophagus (esophageal varices) and stomach (gastropathy)
• Enlarged spleen (splenomegaly)
• Stone-like particles in gallbladder and bile duct (gallstones)
• Liver cancer (hepatocellular carcinoma)

Chronic liver failure indicates that the liver has been failing gradually, possibly for years.

If the liver is failing, a liver transplant may be needed in some cases.

What is a liver transplant?
A liver transplant is the process of replacing a sick liver with a donated, healthy liver. Liver transplants require that the blood type and body size of the donor match the person receiving the transplant. Donated livers come from living and non-living donors. Liver transplant surgery usually takes between four and twelve hours. Most patients stay in the hospital for up to three weeks after surgery. There are currently over 17,000 patients waiting for a liver transplant here in the United States. Historically between 5,000 to 6,000 liver transplants happen annually.
In the U.S., there are more people who need a liver transplant than there are donated livers. The major reason for liver transplants here in the U.S. is hepatitis C.

The process to be eligible for a liver transplant is:

• Person’s doctor refers him or her to be seen at a transplant center;
• At the transplant center, the transplant team evaluates the person’s overall physical and mental health, plan to pay for transplant related medical expenses, and emotional support family and friends will provide;
• Based on the findings, the team decides if the person is eligible for a liver transplant;
• If the person is eligible, the center will add him or her to the transplant waiting list.

The waiting list is prioritized so the sickest people are at the top of the list. The time a person spends on the waiting list depends on:

• Blood type
• Body size
• Stage of liver disease
• Overall health
• Availability of a matching liver

Most patients return to a regular lifestyle six months to a year after a successful liver transplant. In some patients, the liver disease they had before the transplant comes back and they may need treatment or another transplant.

For more information about cirrhosis of the liver and liver transplants, call our helpline at 1-800-465-4837 or consult your healthcare provider.

For more information about Hepatic Encephalopathy stages, consult your healthcare provider.

Here are some facts about who gets HE:

• Most often seen in people with cirrhosis
• Can occur in people of any age who have acute or chronic liver disease
• Affects men and women equally
• More likely to occur in people that have had a TIPS procedure or surgical shunts

About 7 out of 10 people with cirrhosis develop minimal HE (Grade 0); the exact number isn’t known because symptoms are subtle at this stage, making it difficult to diagnose. What is known is that you are at least three times more likely to progress from minimal to obvious, or overt, symptoms of HE if it’s not diagnosed early. So communicate with your doctor right away if you suspect you may have HE so that treatment can be started as soon as possible.

People with chronic liver disease are at greater risk of developing a more chronic form of the disorder where symptoms get worse or continue to come back, known as “HE recurrence.” It isn’t known why some people experience HE recurrences and others do not, but there are several possible triggers.

HE encompasses a range of symptoms depending on the severity of the condition, so symptoms can vary widely from one person to another. Symptoms may begin slowly and gradually get worse, or they may occur suddenly and be severe from the start.

Mild to moderate symptoms of HE may include the following mental and physical changes:

• Mental
  • Mild confusion
  • Short attention span
  • Forgetfulness
  • Mood swings
  • Personality changes
  • Inappropriate behavior
  • Difficulty doing basic math
• Physical
  • Change in sleep patterns (like sleeping during the day and staying up at night)
  • Difficulty writing or doing other small hand movements
  • Breath that smells musty or sweet
  • Slurred speech

More severe symptoms of HE may include these mental and physical changes:

• Mental
  • Marked confusion
  • Severe anxiety or fearfulness
  • Disorientation regarding time and place
  • Inability to perform mental tasks such as doing basic math
• Physical
  • Extreme sleepiness
  • Slowed or sluggish movement
  • Shaking of hands or arms (called “flapping”)
  • Jumbled, slurred speech that can’t be understood

In the most severe form of HE, people can become unresponsive, unconscious and enter a coma.

The severity of HE is judged according to your symptoms. The most commonly used staging scale of Hepatic Encephalopathy is called the West Haven Grading System:

• Grade 0: Minimal HE
  This stage is very hard to detect as changes in your memory, concentration and intellectual functioning are so minimal that they may not be outwardly noticeable, even to you. Coordination can be affected and although subtle, may impact your ability to drive a car. If you recently had poorer performance at work or have committed a number of traffic violations while driving, it would be worth bringing this to the attention of your healthcare provider. You may be referred for special testing, called neuropsychiatric testing, to evaluate your thinking abilities by doing a number of specifically designed tasks with a trained examiner. If your test reveals some deficits, your healthcare provider will likely schedule frequent follow-up visits to closely follow your condition. There are currently no medications approved by the FDA to treat minimal HE.
• Grade 1: Mild HE
  You may have a short attention span, notice mood changes like depression or irritability, and have sleep problems.
• Grade 2: Moderate HE
  You may keep forgetting things, have no energy and exhibit inappropriate behavior. Your speech may be slurred and you can have trouble doing mental tasks such as basic math. Your hands might shake and you can have difficulty writing.
• Grade 3: Severe HE
  You may be confused as to where you are or what day it is and be extremely sleepy, but can still be woken up. You may be unable to do basic mental tasks, feel extremely anxious and act strangely.
• Grade 4: Coma
  The last stage of HE is when the person becomes unconscious and slips into a coma.

An episode of HE may be triggered by any of the following things:

• Infections
• Constipation
• Dehydration: This happens when you don’t get enough water or other fluids.
• Bleeding from your intestines, stomach or esophagus (the tube that connects your mouth to your stomach). This is referred to as gastrointestinal, or GI, bleeding.
• Medications that affect your nervous system, such as sleeping pills, antidepressants or tranquilizers.
• Kidney problems
• An alcohol binge
• Surgery
• Having a portosystemic shunt: This is a tube that’s placed in your liver, sometimes called a TIPS, or a surgical procedure to reroute blood flow and relieve high blood pressure in the veins in and around your liver, a condition called portal hypertension.

There is no specific test used to diagnose HE. A Hepatic Encephalopathy diagnosis is based on a combination of three things:

• Your medical history;
• Your symptoms;
• A thorough clinical exam by your healthcare provider.

Blood tests can identify abnormalities associated with liver and kidney dysfunction, infections, bleeding and other conditions that may contribute to HE. However, these tests are not specific to HE and simply aid in making the Hepatic Encephalopathy diagnosis which is based on your history and symptoms. Ammonia levels are sometimes used, but these values alone cannot diagnose HE.

Because many of the symptoms of HE also occur in people with other types of brain disease or damage – such as stroke, brain tumor, or bleeding inside the skull – your healthcare provider may order specialized pictures of your brain to rule these out.

These imaging tests, as they’re called, are obtained by using various types of equipment and will likely include MRI (magnetic resonance imaging) and CT (computerized tomography) scans. In addition, your doctor may order an EEG (electroencephalogram), a test that measures the electrical activity of your brain, to look for brain wave changes associated with HE.

Since there is no specific “HE test” the diagnosis of HE is often referred to as a diagnosis of exclusion. This means that it’s important for your doctor to exclude – or rule out – other possible causes for your symptoms in order to correctly diagnose you with HE.