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What is Huntington's Disease?

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  • Written By: Garry Crystal
  • Edited By: Niki Foster
  • Last Modified Date: 16 November 2016
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Huntington's disease is an inherited disorder that causes the deterioration of brain cells. The disease affects both the brain and the body. As the deterioration progresses, cells within specific areas of the brain die. Although Huntington's disease can begin in childhood, the symptoms do not usually show themselves until around the age of 30.

Huntington's disease was named after doctor George Huntington in 1872. The disorder now affects around one in every 10,000 people. The major factor in Huntington’s disease is genetics. A child who has one parent afflicted with the disease has a 50% chance of developing the disease himself. Although it is usually an inherited disease, around 3% of sufferers have no trace of it within their family history.

The first symptoms of Huntington's disease usually appear around the age of 30 to 50. The disease affects the sufferer's mobility, as well as the way they think and communicate. Huntington's disease does not only have an impact on the person who is affected by it. It is a condition that touches the lives of everyone who is close to the sufferer. Ultimately, the sufferer will become completely dependent on those around her for her complete care.

As the neurons in the brain begin to die, the symptoms will gradually worsen. The sufferer may exhibit uncontrollable jerking movements. The face and body may also be prone to uncontrollable spasms. Other possible symptoms include the inability to walk and major disorientation. Communication will become difficult, and speech will be slurred.

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As Huntington's disease sets in, the mood and personality of the sufferer will change. Depression is normal. and mood swings are also to be expected. Mental ability will rapidly deteriorate as dementia sets in. This results in memory loss and difficulty with the simplest of problems.

The severity of the symptoms of Huntington’s disease is different from person to person. Inherited DNA factors play a large role in the severity of the symptoms. For some people, the symptoms can be very mild, but for others, they continue to grow until death and can last for around 20 years. Suicide is very common in sufferers of Huntington's disease.

There are medications that can be prescribed to help the sufferer cope with the symptoms of Huntington's disease, but there is no way, at the moment, to prevent it. Research and testing is an ongoing concern. There are many organizations and help groups that deal with Huntington’s disease. They provide information and support to those affected by this profoundly debilitating disease.

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robbie21
Post 4

Another famous, albeit fictional, Huntington's sufferer is the character Thirteen from the TV show House. Her character follows a trajectory where she doesn't know her situation, finds out she *will* get the disease, begins to develop some symptoms, engages in self-destructive behavior, and ultimately gets a grip on her condition and comes to terms with it.

She also serves time in prison for helping her brother to die once he got really sick. Her greatest fear is that no one will do that for her, but House promises to be the one (although given how the series ended, one wonders how he could keep that promise).

Kat919
Post 3

@anon752 - What's particularly tragic about Huntington's genetic transmission is that it is dominant. What that means is that if one parent has the disease, all their children have a 50/50 chance of getting it; the other parent does not have to be a carrier.

And because it doesn't show up until adulthood, people have plenty of time to procreate before being diagnosed. The folk singer Woody Guthrie ("This Land is Your Land") is a famous case. He was quite ill -- I think he was even in a mental hospital -- for some years before he was finally diagnosed.

His son, Arlo Guthrie, is also a folk singer but seems to have escaped the disease; it would have shown up

by now. There are a couple of scenes in the movie Alice's Restaurant that deal with his father's illness; I remember one where Pete Seeger and some singers are visiting Woody Guthrie in the hospital and another in which Arlo tries to get out of the draft by explaining that he could develop Huntington's.
anon260162
Post 2

There is no method of transmission. It is genetic.

anon752
Post 1

How is a diagnosis made for Huntington's, and is there a method of transmission?

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