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Huntington’s Disease (Huntington’s Chorea)

  • - Huntington's Disease
  • Jun 25, 2011
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Tags: | dementia | huntington's chorea | huntington's disease | inheritable dominant disease |

What is Huntington’s Disease?

Huntington’s diseaseHuntington’s disease is a progressive disease that results in the slow loss of affected brain cells. It is an inherited condition that begins in adulthood. At present, typical Huntington’s disease is a lethal condition. Huntington’s disease is characterised by involuntary movements, dementia and psychological disturbances that worsen as the disease progresses.

Statistics on Huntington’s Disease

Huntington’s disease runs in families. It is an inheritable dominant disease, which means that receiving an abnormal gene from one parent will cause the disease, even if the matching gene from the other parent is normal. So, an affected parent has a 50% chance of passing the disease gene onto their children.

In some cases, a parent that carries the mutant gene but does not develop the disease can pass on the disease gene to a child and the child will develop Huntington’s disease, this is more common if passed down by the father.

Huntington’s disease occurs in 4 to 8 people per 100,000. Most of these people will have a previous family history of the disease. However, about 10% of people who develop the condition will not have it in their family history. De novo (meaning “new”) mutations can arise by chance in a person in which there is no previous family history. These “new” cases then also have a 50% chance of passing the disease gene onto their children. 

Huntington’s disease is about 10 times more prevalent in Western European populations compared to African and Asian populations.  Huntington’s occurs about twice as often in Tasmania compared to the rest of Australia due to an individual with Huntington’s settling in Tasmania many years ago and passing the mutation on. This local effect in Tasmania is known as a founder effect.

Risk Factors for Huntington’s Disease

Mutations in the huntingtin gene cause Huntington’s disease. These mutations in the gene result in the huntingtin protein being longer in length. This longer protein then accumulates abnormally in the brain cells and eventually leads to brain cell death. The age the disease begins in a person is heavily dependent on the length of the mutant protein, i.e. the longer the protein, the earlier the person will develop the disease. 

In cases where a parent has the diseased huntingtin gene but does not develop Huntington’s disease, the length of the mutant protein in the parent is only slightly longer than normal. However, in their child who will get passed the diseased gene, the mutant protein is longer and the child will then develop the disease as an adult, a concept known as anticipation.

Progression of Huntington’s Disease

Huntington’s diseaseHuntington’s disease is a lethal disorder. Death usually occurs within 15 - 20 years of showing signs of the disease.

Due to the loss of brain cells involved with motor function, individuals have difficulty controlling their movement, swallowing and clearing their lungs. The most common cause of death is pneumonia due to the individual not being able to clear their lungs properly and having problems with swallowing, which results in food and liquid entering their lungs. The second most common cause of death is heart failure, followed by suicide.   

Symptoms of Huntington’s Disease

Initially with Huntington’s disease, especially prior to diagnosis, family members often report that the individual becomes very difficult to live with, being aggressive and “out of sorts”. This can be very frustrating for the affected person and their family, and often subsequent diagnosis gives the family some much-needed relief since they now understand the reasons behind the personality and behavioural changes of their loved-one.

There are three different types of functions that are affected with Huntington’s disease, these include: motor function (movement), cognition (thought) and neuropsychiatric behavior (mental disease issues). 

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