Huntington's Disease and Juvenile Huntington's Disease

Also known as

  • Huntington's Chorea

Huntington's Disease is a neurodegenerative hereditary condition that damages healthy brain cells and erodes a person's ability to speak, walk, talk, swallow and think clearly. In adults, it typically begins between ages 30-50. A juvenile version affects 5-10% of those with HD and begins before age 20.

Condition details

Huntington's Disease is caused by a faulty gene. This fault leads to damage to healthy brain cells, causing gradual erosion of an affected person's mental, emotional and physical capabilities. In adults, it typically begins to show between ages 30-50. A juvenile version (JHD) also exists, which affects children and young people aged under 20. Roughly 5-10% of all those with HD have this version. HD in adults is often characterised by jerky, uncontrolled movements. In juvenile cases, children more often show muscle rigidity instead. At present, there is no cure, although treatments and therapies can help to manage symptoms as the disease progresses. Every person who has the affected gene will develop the disease at some stage of life, and has a 50% chance of passing it on to their children. Genetic testing is available to show if a person or embryo has the faulty gene, although it can't predict the age at which it will affect them if the person is indeed gene positive.

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Photos of affected individuals

Click images to enlarge.

  • No baby imageBaby
  • No toddler imageToddler
  • No child 4-10 imageChild 4-10
  • No child 10-18 imageChild 10-18
  • No adult imageAdult

Typical age range of first manifestation

  • Does not manifest in Baby
  • Does not manifest in Toddler
  • Does not manifest in Child 4-10
  • Does not manifest in Child 10-18
  • Can manifest in Adult

Symptoms

Toddler
Lay terms Clinical terms
  • Leg stiffness Clumsiness Decline in cognitive function Difficulty with speech Behavioural changes Seizures (25-30% of those affected by JHD experience seizures)
Child 4-10
Lay terms Clinical terms
  • Leg stiffness Clumsiness Decline in cognitive function Difficulty with speech Behavioural changes Seizures (25-30% of those affected by JHD experience seizures)
Child 10-18
Lay terms Clinical terms
  • Leg stiffness Clumsiness Decline in cognitive function Difficulty with speech Behavioural changes Seizures (25-30% of those affected by JHD experience seizures)
Adult
Lay terms Clinical terms
  • Uncontrollable
  • jerky movement Lack of coordination and balance Lack of concentration Apathy Depression Short-term memory problems Lack of inhibitions Changes in mood
  • which can include aggression or antisocial behaviour Difficulty with speech Weight loss through additional movements and/or inability to feed oneself properly Decline in cognitive function Difficulty swallowing

Affected genes identified to date

  • HTT gene / IT15 on chromosome 4

Inheritance patterns

Is Autosomal dominant

Are carriers affected?

Yes, in 100% of cases.

How many are affected?

Around 8,000 in the UK are estimated to be affected, although the true number may be much higher.

Support groups and organisations

Huntington's Disease Association
Suite 24, Liverpool Science Park
Innovation Centre 1
131 Mount Pleasant
Liverpool
L3 5TF
Tel 0151 331 5444
Facebook
Scottish Huntington's Association
St James Business Centre
Linwood Road
Paisley
PA3 3AT
Tel 0141 848 0308
Facebook
Huntington's Disease Association Northern Ireland
53 Andersonstown Road
Belfast
BT11 9AG
Tel 028 2177 1812
Facebook

Known experts

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