Huntington’s disease is a hereditary condition that gradually breaks down your brain’s nerve cells, affecting your physical movements, emotions, and cognitive abilities.

While it’s possible to manage the symptoms with treatment, the condition is ultimately fatal. Most people with Huntington’s disease live for up to two decades after symptoms begin.

Huntington’s disease is much more common in people with European ancestry, affecting about 3-7 out of every 100,000 people of European descent.

There are two types of Huntington’s disease: adult-onset and early onset.

Adult onset

Adult onset is the most common type of Huntington’s disease. Symptoms usually begin when people are in their 30s or 40s, though there is some research suggesting that some people can develop it as young adults ages 20-29.

Initial signs often include:

  • depression
  • irritability
  • hallucinations
  • psychosis
  • minor involuntary movements
  • poor coordination
  • difficulty understanding new information
  • trouble making decisions

Symptoms that may occur as the disease progresses include:

  • uncontrolled twitching movements called chorea
  • difficulty walking
  • trouble swallowing and speaking
  • confusion
  • memory loss
  • personality changes
  • speech changes
  • decline in cognitive abilities

Early onset

Early onset or juvenile Huntington’s disease is less common. Symptoms usually start to appear in childhood or adolescence. Early onset Huntington’s disease causes mental, emotional, and physical changes, like:

  • drooling
  • clumsiness
  • slurred speech
  • slow movements
  • frequent falling
  • rigid muscles
  • seizures
  • sudden decline in school performance

When to see a doctor?

The earliest symptoms of Huntington’s include focus and memory problems, depression and mood swings, and unusual clumsiness.

That said, these symptoms have many other far more common causes, so unless you have a family history of the disease, you are not likely to have Huntington’s based on these signs alone.

Nevertheless, seeing a doctor if you’re concerned can help you get some peace of mind.

A defect in a single gene, the huntingtin (HTT) gene, causes Huntington’s disease. That said, the genetic mutation is different from many other mutations. There isn’t a substitution or a missing section in the gene. Instead, there’s a copying error.

Specifically, a section of the genetic code within this gene called the cytosine-adenine-guanine (CAG) trinucleotide is copied too many times. The number of repeated copies tends to increase with each generation.

Huntington’s is considered an autosomal dominant disorder. This means that one copy of the abnormal gene is enough to cause the disease. If one of your parents has this genetic defect, you have a 50% chance of inheriting it. You can also pass it on to your children.

In general, symptoms of Huntington’s disease show up earlier in people with a larger number of repeats. The disease also progresses faster as more repeats build up.

What are the risk factors for Huntington’s disease?

Your genetics determines whether or not you will develop Huntington’s. However, some factors can influence the time of onset and how the condition progresses.

  • the number of times CAG repeats in your HTT
  • if your CAG is unstable
  • the effect of other genes on your HTT (genetic modifiers)

Research also suggests that environmental factors like stress, your level of physical activity, toxins you’re exposed to, and your diet may speed up or slow the progression of the disease.

Medications

Medications can provide relief from some of your physical and psychiatric symptoms. The types and amounts of drugs needed will change as your condition progresses.

  • Involuntary movements may be treated with tetrabenazine, deutetrabenazine, and antipsychotic drugs.
  • Muscle rigidity and involuntary muscle contractions can be treated with diazepam.
  • Depression and other psychiatric symptoms can be treated with antidepressants and mood-stabilizing drugs.

Therapy

Physical therapy can help improve your coordination, balance, and flexibility. With this training, your mobility is improved, and falls may be prevented.

Occupational therapy can be used to evaluate your daily activities and recommend devices that help with:

  • movement
  • eating and drinking
  • bathing
  • getting dressed

Speech therapy may be able to help you speak clearly. If you can’t speak, you’ll be taught other types of communication. Speech therapists can also help with swallowing and eating problems.

Psychotherapy can help you work through emotional and mental problems. It can also help you develop coping skills.

Family history plays a major role in the diagnosis of Huntington’s disease. However, a variety of clinical and laboratory testing can be done to assist in diagnosing the problem.

Neurological tests

A neurologist will do tests to check your:

  • reflexes
  • coordination
  • balance
  • muscle tone
  • strength
  • sense of touch
  • hearing
  • vision

Brain function and imaging tests

If you’ve had seizures, you may need an electroencephalogram (EEG). This test measures the electrical activity in your brain.

Brain-imaging tests can also be used to detect physical changes in your brain.

  • MRI scans use magnetic fields to record brain images with a high level of detail.
  • CT scans combine several X-rays to produce a cross-sectional image of your brain.

Psychiatric tests

Your doctor might ask you to undergo a psychiatric evaluation. This evaluation checks your coping skills, emotional state, and behavioral patterns. A psychiatrist will also look for signs of impaired thinking.

You may be tested for substance misuse to see if drugs might explain your symptoms.

Genetic testing

Genetic testing for Huntington’s disease has been available since 1993. If you have a family history of the condition, you can find out whether you carry the mutation.

In cases of pregnancy where one partner carries the mutation, it’s possible to conceive with in-vitro fertilization (IVF) and test the embryo for the mutation. Alternatively, the fetus can be tested during the pregnancy via tissue from the placenta.

There’s no way to stop this disease from progressing. The rate of progression differs for each person and depends on the number of genetic repeats present in your genes. A lower number usually means that the disease will progress more slowly.

People with the adult-onset form of Huntington’s disease usually live for 15-20 years after symptoms begin to appear. The early onset form generally progresses at a faster rate. People may live for 10-15 years after the onset of symptoms.

Causes of death among people with Huntington’s disease include:

  • infections, like pneumonia
  • injuries from falling
  • complications from being unable to swallow

Because of the nature of this disease, people living with Huntington’s disease have a greater chance of taking their own life.

Help is out there

If you or someone you know is in crisis and considering suicide or self-harm, please seek support:

If you’re calling on behalf of someone else, stay with them until help arrives. You may remove weapons or substances that can cause harm if you can do so safely.

If you are not in the same household, stay on the phone with them until help arrives.

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Learn more: The life expectancy of Huntington’s disease.

If you’re having trouble coping with your condition, consider joining a support group. It can help to meet other people with Huntington’s disease and share your concerns.

You can find support and other resources at the Huntington’s Disease Society of America or the Huntington’s Disease Association in the United Kingdom. Other countries, or your local state, may have their own organizations.

If you need help performing daily tasks or getting around, contact the health and social services agencies in your area. They might be able to set up daytime care.

Talk with your doctor for information on the type of care you might begin to need as your condition progresses. You might have to move into an assisted living facility or set up in-home nursing care.