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Huntington's disease

Huntington's disease is an inherited disorder that causes progressive damage to your brain cells. This leads to a gradual loss of your mental ability, problems with controlling your movements and changes in your personality.

About Huntington's disease

Huntington's disease causes progressive damage to cells in areas of your brain called the basal ganglia and cerebral cortex. These areas are involved in your control of movement, ability to make plans, how motivated you are and your personality.

Huntington's disease usually develops between the ages of 30 and 50, but it can start at any age. Symptoms, which differ from person to person, get gradually worse, sometimes over a period of up to 20 years. In Europe, it’s estimated that between four and eight people in 100,000 have Huntington’s disease, although the true number may be higher because not everyone will seek treatment. It affects men and women equally.

Huntington's disease was previously called Huntington's chorea. Chorea means jerky, involuntary movements – a main symptom of the condition.

Juvenile Huntington's disease

Juvenile Huntington's disease is a type of Huntington's disease that develops before you're 20 and is usually more severe. It affects around five to 10 in 100 people with Huntington's disease. Most people with juvenile Huntington’s disease have a father with the condition.

Symptoms of Huntington's disease

Symptoms of Huntington’s disease change as the condition progresses. You may not notice the early symptoms of Huntington's disease. This is because they are mild at first and people without the disease can have the same symptoms for other reasons.

Early symptoms include:

  • a mild tremor
  • clumsiness
  • lack of concentration
  • irritability
  • short-term memory problems
  • mood changes
  • depression

These symptoms may be caused by problems other than Huntington’s disease, but if you have any of these symptoms, see your GP for advice.

Later symptoms fall into three categories – physical, emotional and behavioural. Over time, these symptoms get worse and you’re likely to need more care from other people.

Physical symptoms

You may have some of the following symptoms.

  • Involuntary fidgeting movements of your limbs, face and body called chorea. This may start in your face, hands and feet and gradually spread over time to the rest of your body.
  • Difficulty co-ordinating your movements. This will get gradually worse as the disease progresses.
  • Difficulty gripping objects. You may become clumsy and drop things.
  • Difficulty walking. You may become unsteady and fall over more often.
  • Speech problems. You may sound like you’re slurring.
  • Difficulty swallowing. This can lead to food or drink going down your windpipe, causing you to choke.
  • Weight loss. This can happen because you're having difficulty eating and drinking, and because you're burning more calories due to the continuous movements of chorea.

Emotional symptoms

Emotional symptoms include depression, not only because of the difficulties you may face by having a progressive disease but also as a direct result of the damage to certain brain cells. Other symptoms include:

  • frustration at being unable to work or do simple tasks
  • behaving stubbornly
  • having mood swings
  • becoming more irritable and antisocial
  • having fewer inhibitions
  • difficulty controlling your anger
  • becoming more aggressive

Behavioural symptoms

The behavioural symptoms of Huntington's disease are often the most distressing for you, your family and your carers. Your personality can become gradually more self-centred and unmotivated, putting a strain on personal relationships. You may appear lazy or uninterested in life, spending days doing little or neglecting your personal hygiene.

There are a number of other behavioural symptoms, which include:

  • a loss of drive and initiative
  • more difficulty with problem-solving, judgement, attention and concentration
  • losing the ability to organise yourself and to do more than one task at a time
  • memory loss
  • finding it more difficult to understand speech

Symptoms of juvenile Huntington's disease

If you have juvenile Huntington’s disease, your symptoms will be similar to those of Huntington's disease, but you’re more likely to have muscular rigidity (stiff muscles) and less likely to have involuntary movements (chorea). Early symptoms of juvenile Huntington’s disease may be similar to those of Parkinson’s disease.

If you have juvenile Huntington's disease, you will usually first develop symptoms in your teens. You may be irritable and aggressive, or start to act out of character, possibly displaying inappropriate social or sexual behaviour.

These symptoms may be caused by problems other than juvenile Huntington’s disease. If you or your child has any of these symptoms, see your GP for advice. Confirming a diagnosis of juvenile Huntington’s disease may be difficult and take a long time because the disease is very rare.

Complications of Huntington’s disease

As your later symptoms get worse, you may have complications. These include:

  • poor muscle control and weakness
  • difficulty with eating and drinking
  • problems with speaking and talking to others
  • changes in your usual emotions, behaviour and personality, for example, being more aggressive or having memory problems

You may need more care provided by either your family, friends or your team at a Huntington’s disease multidisciplinary clinic. This is a specialty clinic that provides comprehensive co-ordinated care for people with Huntington’s disease. Changing and increasing the level of care you receive may help you to maintain more independence and help your family to cope as your disease progresses.

It’s possible that having Huntington’s disease may lead to you having suicidal thoughts, but you may find that taking antidepressant medicines helps prevent this.

Causes of Huntington's disease

Huntington's disease is caused by a faulty gene that runs in families. In people with Huntington’s disease, this gene is longer in size than in people without the disease, which is what makes it faulty. The longer the faulty gene, the more chance you have of developing the disease and passing it on to your children. The gene produces a protein called Huntingtin. Scientists are still researching how this faulty gene causes the disease.

Genes are present in two copies: one from your mother and one from your father. The faulty gene that causes Huntington's disease is dominant. This means that if you inherit a copy of this gene from either parent, you will go on to develop the disease at some point in your life.

If you have one parent with Huntington's disease, you have a one in two chance of inheriting the faulty gene. The risk is one in two for each child. 

How Huntington's disease is inherited.

Around three in 100 people with Huntington's disease have no family history of the condition. This may happen when relatives from previous generations weren't diagnosed with Huntington's disease because reliable testing wasn’t available at the time. However, genetic testing is far more reliable now, making it easier to get a diagnosis.

Diagnosis of Huntington's disease

Your GP will ask you about your symptoms and examine you. He or she will usually ask you about your medical history and that of your family. Your GP may ask you to carry out some simple tasks to assess any chorea that you may have.

You may have symptoms of Huntington's disease for a long time before you’re diagnosed. Depending on your symptoms and family medical history, your GP may refer you to a genetics clinic, a Huntington’s disease multidisciplinary clinic, or a neurologist or psychiatrist to confirm your diagnosis. The only way to be sure that your symptoms are caused by Huntington’s disease is to have a genetic test.

The genetic tests fall into three categories: diagnostic, pre-symptomatic predictive and prenatal. Whichever test you’re offered, you will be offered genetic counselling before you have the test because there are many complex issues to discuss and consider. See our frequently asked questions for more information.

Diagnostic tests

These tests are carried out if you have symptoms of Huntington's disease. You will have two blood tests. The results of these tests will take between four and six weeks to come back.

Pre-symptomatic predictive tests

These tests are carried out if you have no symptoms of Huntington's disease, but you have family members who are affected by it. The tests will show whether or not you have inherited the Huntington’s disease gene, but not when you may begin to show symptoms.

The decision to have these tests is a serious one and you're advised not to rush into making a decision. A positive result can be devastating as it means that one day you will most likely become seriously ill. Many people prefer not to find out and instead aim to live their lives to the full. There are also issues surrounding testing if your parents haven't been tested. This is because if you have a positive result, it means that one of your parents also has the faulty gene, even if they don’t yet have any symptoms.

In the UK, you can't have pre-symptomatic tests if you're under 18.

Prenatal tests

These tests can be carried out on your unborn baby if your family is affected by Huntington's disease. Prenatal tests are done between week 11 and 13 of pregnancy. For more information about what a prenatal test involves, please see our frequently asked questions.

Treatment of Huntington's disease

At the moment, there is no cure for Huntington's disease, but there are ways to manage your symptoms. You will be referred to a Huntington’s disease multidisciplinary clinic, where a number of different health professionals will work together as a team to help you and your family.

Your neurologist may prescribe you medicines to reduce your involuntary movements and to alleviate depression. Mood stabilisers and antipsychotic medicines can also help with some of the emotional disturbances that you may have.

At the multidisciplinary clinic, the members of the team who may become involved in your care include the following.

  • Speech and language therapists can help improve your communication and help you to swallow.
  • Dietitians can advise you on adequate calorie and nutrient intake to stop weight loss.
  • Occupational therapists can provide you with equipment to help you eat, such as non-slip mats and straws, and help to make your home safer by adapting it to your needs.
  • Physiotherapists can help with any balance or musculoskeletal problems.

Counselling and support groups can be helpful, both for you and your family.

Special considerations

Having a baby

If you know that you carry the gene for Huntington's disease, you can choose to have in vitro fertilisation (IVF), where embryos are tested for the Huntington's disease gene and only those that don't carry the faulty gene are placed in your womb. This is called pre-implantation genetic diagnosis.

In the UK, pre-implantation genetic diagnosis is only available to parents who already know that they carry the gene for Huntington's disease. This is only available at certain clinics licensed by the Human Fertilisation and Embryology Authority.

Future promise

Scientists are investigating several medicines that could slow the progression of Huntington's disease. Some of these have shown promise and are being tested in clinical trials. Examples include the nutritional supplement, coenzyme Q10, and memantine, which is already used to treat people with severe Alzheimer’s disease.

Some clinical studies have used stem cells (the most basic form of cells from which others develop) to grow cells that can be transplanted into the brain of people with Huntington's disease to improve their symptoms. However, research is still in the very early stages.

Scientists are also hopeful that research into RNA interference (RNAi), which can potentially shut off any gene in the body, may lead to an effective treatment in the future by stopping the production of the Huntingtin protein.

 

Produced by Louise Abbott, Bupa Health Information Team, May 2012.

For answers to frequently asked questions on this topic, see FAQs.

For sources and links to further information, see Resources.

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  • This information was published by Bupa's Health Information Team and is based on reputable sources of medical evidence. It has been reviewed by appropriate medical or clinical professionals. Photos are only for illustrative purposes and do not reflect every presentation of a condition. The content is intended only for general information and does not replace the need for personal advice from a qualified health professional. For more details on how we produce our content and its sources, visit the about our health information page.

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