Huntington's disease

Expert Reviewer Dr Jeremy Rees, Consultant Neurologist
Next review due February 2023

Huntington’s disease is an inherited disease that causes progressive damage to your brain cells. It causes extra movements that look like fidgeting (called chorea) and changes your personality, mood and ability to think clearly.

Anxious woman

About Huntington’s disease

Huntington’s disease causes progressive damage to parts of your brain that help to control movement and the way you think, understand and remember. It’s linked to changes in a specific gene. The disease affects between one in 10,000 and one in 20,000 people in the UK. It affects men and women equally.

If the faulty gene that causes Huntington’s disease is passed on from one parent, you will get the disease (a pattern of inheritance known as ‘autosomal dominant’). So, the chance of passing the disease on from an affected parent to a child is 50%.

Huntington’s disease can start at any age, but the symptoms usually first appear between ages 30 and 50. The symptoms gradually get worse, usually over around 10 to 25 years until the person dies.

Juvenile Huntington's disease is a type of Huntington's disease that starts before you’re 20. It’s usually worse than the adult form and starts more like Parkinson’s disease. It’s rare, affecting only around six out of every 100 people with Huntington’s disease.

Symptoms of Huntington’s disease

The symptoms of Huntington’s disease vary from person to person. In the early stages of the disease, the symptoms may be very mild. The symptoms will eventually become worse, but the rate at which they progress can vary from person to person.

Huntington’s disease can affect your:

  • movements, causing jerky movements, twitching and uncontrollable fidgeting (called chorea)
  • mood, making you irritable, aggressive, anxious or depressed
  • thinking patterns, affecting your concentration and memory
  • behaviour and personality, causing changes in the way you interact with others, mood changes and a loss of interest in things

These symptoms can be caused by problems other than Huntington’s disease. But if you have a combination of these, you should see your GP. If you know that Huntington’s disease runs in your family, it’s important to discuss this with your doctor, even if you are not displaying any of the symptoms.

As Huntington’s disease progresses, it can affect your:

  • speech and language skills, so you find it harder to talk, remember words and communicate
  • walking and coordination, making you more likely to trip or fall over
  • swallowing, so you find it harder to eat or drink without choking – this can lead to weight loss

Huntington’s disease can affect your ability to work and do simple tasks. It may affect your social activities and relationships. You may also develop depression and dementia. Eventually, you’re likely to need more care from other people.

The early symptoms of juvenile Huntington’s disease (which starts in childhood or teenage years) may be different from Huntington’s disease that starts in adults. Generally, children and younger people are more likely to have stiffness and clumsiness, behavioural changes and epilepsy, and less likely to have uncontrollable fidgeting and twitching movements. They may find it harder to concentrate at school and they may have behavioural problems.

Diagnosis of Huntington’s disease

If you have symptoms of Huntington’s disease, your GP will refer you to a specialist genetics clinic or a neurologist.

The doctor will check you for possible symptoms of the disease, such as involuntary movements. They may be able to tell that you probably have Huntington’s disease from your symptoms and your family history. But the only way to be sure that you have Huntington’s disease is to have a genetic test.

Genetic testing

  • Two types of blood test can find out if you’ve got the abnormal Huntington’s disease gene. Diagnostic testing: this will confirm the diagnosis if you already have symptoms of Huntington’s disease.
  • Predictive testing: the test will show if you’ve got the Huntington’s disease gene. But it can’t show when you’ll begin to show symptoms, how bad these will be or how quickly the disease will progress.

You need to be at least 18 years old to have the test. Your doctor will refer you for genetic counselling in a regional genetics clinic before you decide to have any genetic tests. You’ll be offered information on the disease and at least three counselling sessions to help you decide whether to have the test. This will give you the chance to talk everything through. If you have the Huntington’s disease gene, this will affect other members of your family, who may be unaware that they have the gene too. The decision to have predictive testing is a serious one – don’t be rushed or pressured by anyone else into deciding to have it or not.

Your doctor or counsellor can direct you to a Huntington’s disease support group or other resources, such as the Huntington’s Disease Association – see Other helpful websites.

Prenatal testing

You’ll be offered prenatal testing if you’re pregnant and you or your partner has a family history of Huntington’s disease or has been diagnosed with it. You’ll be offered counselling before and after the test, and will need to give your informed written consent.

Two main types of test are part of your antenatal care:

  • Chorionic villus sampling (CVS): this can be carried out between 11 and 13 weeks of your pregnancy. It involves taking cells from inside your womb (uterus).
  • Amniocentesis: this is normally carried out when you’re 15 to 18 weeks pregnant. It takes a sample of amniotic fluid for testing.

Doctors will check the cells to see if your unborn baby has the faulty gene that causes Huntington’s disease. Both tests have their pros and cons so you’ll need to discuss these with a doctor or midwife first.

Preimplantation genetic diagnosis (PGD)

If you or your partner has the Huntington’s gene, you can have a procedure called preimplantation genetic diagnosis (PGD) to prevent you passing the disease on to your unborn child. Embryos are created by in vitro fertilisation (IVF) and tested for the Huntington’s gene. Only embryos that don’t have the Huntington’s gene are implanted in your womb (uterus). This means your baby doesn’t have the faulty gene and won’t develop Huntington’s disease.

This procedure carries the same risks as IVF. Your specialist can discuss this with you to help you make an informed decision. You and your partner will also be offered genetic counselling. In the UK, preimplantation genetic diagnosis is only available at certain clinics licensed by the Human Fertilisation and Embryology Authority.

Treatment of Huntington’s disease

Huntington’s disease can’t be cured or treated. But there are ways to help manage your symptoms. Lots of different healthcare professionals will work together as a team to help you and your family in a specialist Huntington’s disease clinic.

You may be prescribed medicines to help with:

  • involuntary movements (chorea) and stiff muscles
  • other symptoms, such as sleeping problems
  • emotional and behavioural symptoms, such as obsessive-compulsive behaviour, aggression or irritability
  • depression and anxiety

You may also be offered:

  • speech and language therapy to help your communication and swallowing
  • advice from a dietitian to stop you losing weight and make eating more comfortable
  • occupational therapy to make your daily living easier and safer
  • physiotherapy and exercises to do at home to help your balance, muscle or joint problems
  • social care and psychiatry services for physical and emotional support
  • clinical nurse specialists for advice and referrals to other services (such as a dentist to help you look after your teeth properly)

You and your family can be offered counselling, especially if you have depression or are prone to risky behaviour. Cognitive behavioural therapy (CBT) may be useful if you’ve developed obsessive or compulsive behaviour. Support groups can help your family and friends too.

You’ll have a check-up with your doctor every six to 12 months, or more often if you have any problems. They’ll want to check how you and your family are coping, physically and emotionally, and if you need any extra support or treatments.

Causes of Huntington’s disease

Huntington's disease is caused by a faulty gene that makes a protein called huntingtin. This gene is longer in people with Huntington’s disease than in people without the disease. The longer the faulty gene, the earlier the symptoms may start. Scientists are still researching how this faulty gene actually causes the disease.

If you inherit this gene from either parent, you’ll develop the disease at some point in your life. Most people with juvenile Huntington’s disease inherit it from their dad. You can find out if you have the faulty gene by having a blood test. You’ll be offered genetic counselling first.

Some people with Huntington's disease seem to have no known family history of the disease. This may be explained by the early death of an affected parent before their symptoms appeared, by adoption or even by mistaken paternity.

Living with Huntington’s disease

If you have Huntington’s disease, you need to take good care of your general health, especially as your symptoms get worse. Living with Huntington’s disease can be a challenge, so it’s important to make sure you have the right support in place.

Charities such as the Huntington’s Disease Association can provide you, your family and your carers with support and advice. The Huntington’s Disease Association may also be able to put you in touch with a Specialist Huntington’s Disease Adviser, who can provide practical and emotional support, and help you organise appointments.

As Huntington’s disease gets worse, you’ll be more likely to catch infections, especially if you’ve lost weight or find it hard to swallow. You should have flu and pneumonia vaccinations, which will also protect against meningitis and septicaemia (blood poisoning).

It’s important to stay physically active at all stages of the disease. Not only will this help your balance and keep you moving, but it can also help with other symptoms, such as breathing problems or extreme tiredness (fatigue). A physiotherapist can give you advice about staying as active as you can.

As the disease progresses, you may find it harder to do everyday tasks, such as doing up buttons on your clothes. As your balance and walking become affected, you may also be more likely to fall over. An occupational therapist can help you continue to do activities that keep you active and mentally alert for as long as possible.

If you’ve been diagnosed with Huntington’s disease and have a driving license, you must tell the Driver and Vehicle Licensing Authority (DVLA). You could be fined £1,000 if you don’t. The DVLA may contact your doctor for more details, arrange for you to be examined or ask you to take a driving assessment.

Coping with late stages of Huntington’s disease

Huntington’s disease affects everyone differently, especially in the later stages. But it’s likely that you’ll have a period, perhaps of some years, where you need care from others. As your symptoms get worse, you’ll probably find it harder to carry out normal daily functions, such as speaking, eating, walking and caring for yourself physically.

Your care may be provided by your family and friends or by your team at a specialist Huntington’s disease clinic. Adapting your care as your disease progresses may help you to stay as independent as possible, and also help your family to cope. Most people with Huntington’s disease need nursing care of some sort towards the end of their life.

Research into Huntington’s disease cure

There’s a lot of research into Huntington’s disease. Doctors around the world are trying to understand the disease better, to help control the symptoms and, eventually, to find a cure. One promising recent example is a trial, published in May 2019 in the New England Journal of Medicine, which found that a treatment designed to inhibit the production of huntingtin was safe and led to reduced levels of the protein.

If you have Huntington’s disease or test positive for the faulty gene, you may be able to take part in some research studies. More information on current research is available from the Huntington’s Disease Association’s website.

Frequently asked questions

  • If you’ve inherited the faulty gene, it’s certain that you’ll have Huntington’s disease at some point in your life. But you can live for years with the faulty gene and without any symptoms of the disease.

    Genes are bits of code inside each of your cells that tell them what to do. In Huntington’s disease, the faulty gene makes a harmful version of a protein called huntingtin, which then damages specific brain cells. This gene is longer in people with Huntington’s disease than in people without the disease.

    You can have a genetic test to see if you have the faulty gene. But the test won’t tell you when the disease will start.

  • There’s no right time to tell your child that you have the gene for Huntington's disease and that they have a chance of carrying it. But your child will learn about it at some point and may be angry that you didn’t tell them sooner.

    Your child may already have noticed changes in you because of your symptoms. Your condition may have caused tensions at home that they’ve picked up on. They could think it’s their fault that you’re angry, irritable or anxious. Being honest and open with your child can address their fears and help them cope with your illness.

    How you go about telling your child depends on their age, and whether or not you already have any symptoms. Start with the basics and let them ask questions. You could research and write down answers to things they may want to know beforehand, so you’re well prepared.

    If you’d like some support to help you when talking to your child, contact the Huntington's Disease Association. The charity can put you in touch with other families dealing with a similar situation and has a team of specialist youth workers.

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Related information

    • Huntington’s disease. BMJ Best Practice., last reviewed October 2019
    • Huntington’s disease. PatientPlus. www., last edited May 2016
    • Huntington disease. Medscape., updated February 2019
    • Hamilton A, Ferm U, Heemskerk A, et al. Management of speech, language and communication difficulties in Huntington’s disease. European Huntington’s Disease Network. 2012., accessed December 2019
    • Neurology. Other neurological syndromes. Oxford Handbook of General Practice. 4th ed. Oxford Medicine Online., published online May 2016
    • Benefits and risks of genetic testing. Genetic Alliance UK., last reviewed July 2016
    • Making the decision to have predictive testing. Genetic Alliance UK., last reviewed July 2016
    • Amniocentesis. PatientPlus., last edited December 2016
    • Chorionic villus sampling. PatientPlus., last edited December 2016
    • Preimplantation genetic diagnosis. Medscape., updated August 2018
    • Pre-implantation genetic diagnosis (PGD). How can I get PGD treatment? Human Fertilisation & Embryology Authority., accessed December 2019
    • Brotherton A, Campos L, Rowell A, et al. Nutritional management of individuals with Huntington’s disease: nutritional guidance. European Huntington’s Disease Network. Neurogen Dis Manage 2012; 2(1):33–43., accessed December 2019
    • Cook C, Page K, Wagstaff A, et al. Development of guidelines for occupational therapy in Huntington’s disease. European Huntington’s Disease Network. Neurogen Dis Manage 2012; 2(1):79–87., accessed December 2019
    • Quinn L, Busse M. Development of physiotherapy guidance and treatment-based classifications for people with Huntington’s disease. European Huntington’s Disease Network. Neurogen Dis Manage 2012; 2(1):21–31., accessed December 2019
    • Manley G, Lane H, Carlsson A, et al. Guideline for oral healthcare of adults with Huntington’s disease. European Huntington’s Disease Network. Neurogen Dis Manage 2012; 2(1):55–65., accessed December 2019
    • Getting the care you need. Huntington’s Disease Association., accessed December 2019
    • Other diseases causing dementia. Practical Dementia Care. 3rd ed. Oxford Medicine Online., published online May 2016
    • Huntington’s disease and driving. Driver and Vehicle Licensing Authority., accessed November 2019
    • What happens after you tell DVLA. Driver and Vehicle Licensing Authority., accessed November 2019
    • Later stages of Huntington’s: what to expect. Huntington’s Disease Association., accessed December 2019
    • Thinking about the end of life. Huntington’s Disease Association., accessed November 2019
    • Get involved in research. Huntington’s Disease Association., accessed December 2019
    • Other research studies and resources. Huntington’s Disease Association., accessed December 2019
    • DNA, genes, chromosomes and mutations. Genetic Alliance UK., last reviewed May 2016
    • Huntington’s disease: Talking to children about Huntington’s disease. Huntington’s Disease Association., accessed December 2019
    • For children and young people. Huntington’s Disease Association., accessed November 2019
    • Tabrizi SJ, Leavitt BR, Landwehrmeyer GB, et al. Huntingtin expression in patients with Huntington’s Disease. N Engl J Med 2019; 380(24):2307–316. DOI: 10.1056/NEJMoa1900907
  • Reviewed by Victoria Goldman, Freelance Health Editor, and Graham Pembrey, Lead Editor, Bupa Health Content Team, February 2020
    Expert reviewer Dr Jeremy Rees, Consultant Neurologist
    Next review due February 2023