The 5 Stages of Huntington’s Disease

Huntington's disease (HD) is a neurological disorder caused by a genetic mutation passed down through families. The disease destroys cells in different parts of the brain, causing symptoms that get worse over time.

More than 30,000 Americans have HD. The disease can develop in people of any sex, gender, or ethnic background. However, recent studies have hinted at more frequent—and more severe—HD in people who are female.

People from European backgrounds are most likely to pass on the gene mutation that causes HD, while people of Japanese, Chinese, and African descent have lower risks.

The genetic mutation that causes Huntington's disease is present at birth, but symptoms of the disease do not appear right away. Instead, neurological damage can happen slowly and without symptoms for decades during the preclinical stage of the disease.

Noticeable symptoms of HD usually show up between the ages of 30 and 50. The early symptoms can include subtle behavior and cognitive changes. Over time, difficulties with movement will become more noticeable. Damage to the part of the brain that controls movement eventually causes severe issues with coordination, speech, and swallowing.

Preclinical Stage

The preclinical stage of a disease is a period when the disease can be detected through screening or biological testing, but there are no obvious physical symptoms yet. During this time, damage can occur at the cellular level, but there is not enough damage yet for a person to notice it in their daily life.

The preclinical (or prodromal) stage of Huntington's disease can last for 20 years or longer. While you may not have physical symptoms in this stage, you may already have a diagnosis because genetic testing for Huntington's disease has come a long way since the gene responsible for causing the disease was identified in 1993.

Symptoms

In the 10 to 15 years before the physical symptoms of HD start, you might have cognitive, emotional, or behavioral changes, such as:

• Depression
• Difficulty learning new things
• Irritability
• Trouble with decision-making
• Poor coordination

It can be difficult to link the emotional and cognitive symptoms to HD at first. You might not make the connection until later when the physical symptoms of the disease appear.

This overview will take a closer look at how Huntington's disease develops, and what to expect at different stages of the disease.

Early Stage

During the preclinical stage of HD, neurologic damage occurs but you will not have any noticeable physical signs. As cells are gradually destroyed, the symptoms slowly begin to appear in your 30s to 50s.

This stage—called stage I—is when physical symptoms first start to appear. This stage can last for up to eight years. You may have already experienced slight emotional, cognitive, or behavioral symptoms by this stage without recognizing them as being related to HD. You are considered to be in the active disease stage of HD once the physical symptoms first appear.

Symptoms

Symptoms that you might experience in stage I include:

• Loss of coordination
• Trouble performing complex movements
• Involuntary twitches in the fingers, toes, or face (chorea)
• Depression
• Difficulty with concentration or problem solving
• Irritability
• Loss of inhibitions

Functionally, most people in stage I can still carry on with their usual activities in daily life. Things like bathing, eating, and driving may not present physical challenges at this stage, but behavior and cognition issues can make working and relationships more challenging.

People in stage I may still be able to work but might perform at a lower level or need to reduce their work hours.

Early Intermediate Stage

In the early intermediate stage (stage II) of HD, the physical signs of the disease are more noticeable and may begin to affect your daily life.

Stage II is when the physical symptoms progress, and work or family relationships can become strained due to the behavioral and emotional symptoms of the disease. This stage can last for three to 13 years from when the symptoms start.

While individual symptoms are similar to those in stage I, they will increase in severity during stage II. Complex movements might become more difficult, and involuntary movements and twitching will become more obvious to those around you.

Treatment

People in all stages of HD are treated with medications to ease their symptoms. These include:

• Tetrabenazine and deuterabenazine for involuntary movements (chorea)
• Medications that treat depression and mood disorders
• Antipsychotics to control hallucinations or outbursts

There is no set treatment regiment for HD. Instead, each person is treated for the symptoms that cause them the most trouble.

It's important in the early stages of the disease, however, to make plans for the future. This may include conversations or decisions about things like:

• The kind of care that you want as the disease progresses
• Workplace plans
• Establishing a healthcare team
• Identifying support systems
• Making plans for long-term care
• Participation in clinical trials
• Legal and financial planning
  

Late Intermediate Stage

In the late intermediate stage of HD, the disease's effect on your life becomes more pronounced. Sometimes referred to as stage III, many people at this stage of the disease can no longer work and struggle to complete basic daily tasks.

Symptoms

In stage III HD, your symptoms may include:

• Severe chorea, or involuntary movements
• Difficulty walking
• Trouble performing motor tasks
• Frequent falls
• Difficulty swallowing
• Weight loss
• Concentration issues and difficulty organizing thoughts
• Disinterest in previous hobbies or activities
• Memory problems
• Depression
• Mood swings

People in this stage usually need someone to help them with everyday tasks and more complicated needs like managing their housing and finances. The late intermediate stage of HD can last from five to 16 years from the start of symptoms.

Treatment

Treating HD does not change much as the disease progresses. Medications are prescribed based on the symptoms affecting a person's life, with the main focus being on helping people manage their basic needs. Complications easily arise from falls or infections, which is why skilled nursing care is an important part of a treatment plan for people at this stage of HD.

People with HD will want to start thinking about these needs at earlier stages of the disease, which may include completing advanced directives to dictate how they will want to be cared for as they lose motor and cognitive independence.

Early Advanced Stage

The early advanced stage of HD usually begins about a decade after the onset of the disease but can range from nine to 21 years after symptoms start.

Sometimes known as stage IV, this is when some people with HD start to require more advanced care. The need for major assistance with finances, household tasks, and even personal care means that it might be more difficult for someone at this stage to live at home.

Symptoms

The symptoms of advanced HD are severe and can include:

• Extreme difficulty with voluntary movements
• Rigidity
• Severe involuntary movements (dystonia)
• Abnormally slow movements (bradykinesia)
• Severe cognitive loss
• Depression
• Apathy about the disease
• Psychosis
  

Advanced Stage

At the most advanced stage of HD (stage V), many people with the disease require around-the-clock skilled nursing care. Their movement is severely limited, and it can be difficult to complete any basic motor function.

Swallowing can be hard if not impossible, and feeding assistance is often required (including a temporary or permanent feeding tube).

Symptoms

Other symptoms of advanced HD include:

• Immobility/bedridden
• Complete dependence on others for personal tasks
• Nonverbal, unable to communicate
• Unable to swallow, eat independently

Many people at the most advanced stage of HD depend on others for all of their personal and healthcare needs. At this point, infections and falls are common complications. In fact, infections like pneumonia or injuries related to falls are the leading causes of death in people at this stage of HD.

Summary

Huntington's disease is a neurodegenerative disease that causes emotional, behavioral, cognitive, and physical problems.

Early in the disease, damage to nerve cells might not be noticeable. In the preclinical phase, damage can occur with no symptoms, and you are considered to be in the active disease state when motor symptoms start. These symptoms, like rigidity and involuntary twitches, can affect your daily life and make it difficult to complete tasks.

By the later stages of the disease, you may become immobile and require full-time skilled nursing care. HD is usually fatal 10 to 30 years after symptoms begin, many times from complications like infections, falls, or suicide. Early diagnosis can help you and your family make preparations for your care.

A Word From Verywell

Huntington's disease (HD) is a genetic neurodegenerative disease that develops without symptoms for the first few decades. Once the symptoms start to affect your daily life, you will be diagnosed with active disease. The disease is staged based on your motor function and ability to complete everyday tasks.

Typically, HD progresses for 10 to 30 years. Most people with HD die from complications related to the disease. There is no cure for HD, and current medications can only relieve its symptoms, not slow or delay the progression.

If Huntington's disease runs in your family, you may want to have genetic testing. If you find out that you have it, you may consider participating in clinical trials of new medications.