Huntingtons+Disease

=__Huntington's Disease__=

__What is Huntington's Disease?__
Huntington's disease is an inherited disorder, caused by the deterioration of cells in areas of the brain responsible for movement and coordination degeneration and can cause "deterioration and gradual loss of function of areas of the brain". This causes a disorder known as chorea, or constant, jerky movements that the individual can not control. It was first outlined by American physician George Huntington, in 1872. It is one of the few genetic point mutations that can be accurately tested for.

**__Symptoms__**
The symptoms of Huntington's disease do not appear suddenly, but rather, slowly as the disease progresses on. This makes Huntington's more difficult to diagnose, because some of the symptoms can be associated to other diseases or disorders. The first symptoms to appear are usually physical. It occurs, in different geographical regions, from five to eight people out of 100,000. Around ten percent of all HD cases occur in people aged under 20. A child who inherits the Huntington's gene will eventually develop the illness, although onset typically does not occur until ages 35-50 or later.

The most obvious physical symptom is the exhibition of involuntary jerky movements, known as //chorea//. This gets worse as the disease progresses, causing lack of coordination, inability to make appropriate facial expressions, and speech, among others. Later, these become virtually impossible, and the patient needs assistance in such things as speaking, eating, and moving. Symptoms- -Uncontrollable facial expressions -Inability to do basic movements, such as standing and sitting -Difficulty with speech -Difficulty with chewing and swallowing -Lack of coordination
 * Physical**

Not all cognitive abilities are affected by Huntington's disease. Functions //not// affected are memory, language skills, and in some cases learning new skills. Symptoms- -Inability to stop oneself's innappropriate actions -Decline in perceptual skills -Impaired psychomotor function (slowing of thought process to control muscles)
 * Cognitive**

These symptoms vary between subjects. Psychiatric symptoms of Huntington's disease are commonly negative changes in behavioral attitude. Symptoms- -Anxiety -Depression -Egocentrism -Aggression -(Increased) compulsivity
 * Psychiatric**

**__Causes__**
The gene that causes Huntington's disease has been named the HD gene, which is located on the shorter arm of chromosome 4. It has a sequence of C-A-G, or Cytosine-Adenine-Guanine. This is repeated over and over. This codes for the amino acid //glutamate//. CAG, when repeated 40 times or less, is the equivalent to a protein called //huntingtin (Htt)//. When this sequence is repeated too many times, Htt mutates. This mutation causes cell death in selective areas in the brain, causing the symptoms of Huntington's disease.

ImageSource: http://www.path.sunysb.edu/neuropath/images/misc/miscbrain2.jpg

__Inheritance__
If both of the parents have the disease, than the inheritance odds is 75%. However, usually if it's only one parent, the odds are 50% from mother --> daughter, mother --> son, father --> daughter, and father --> son.

**__Diagnosis__**
There is a fifty percent chance of a child whose parent has HD to inherit the disease. Symptoms usually do not start out physically, but emotionally and mentally. Therefore in the early stages, it is important to have psychological tests done, not just physical. Testing for HD with a blood test tests for the number of repetitions of CAG. An abnormal amount of repetitions could mean that the patient has HD.

**__Management__**
There is currently no proven cure Huntington's disease. For this reason, when children are going to be tested for the disease, counselling sessions usually precede the actual diagnosis. Today, there are treatments available to alleviate (temporarily) the chorea that is associated with HD. However, the medications used cause other symptoms to flare. HD patients need two to three times the nutrition (mostly of caloric value) than an average person to maintain body weight. None of this will truly stop Huntington's disease. Therefore the most common "treatment" for HD patients is counseling and therapy.

**__Interesting Facts and Statistics__**

 * An estimate of around 30,000 people are diagnosed with Huntington's Disease in the United States
 * Although more like to occur to adults, Huntington's Disease for juveniles account for about one-sixth of all cases
 * Huntington's Disease, sometimes also known as Huntington's chorea, is named from the Greek term for choreography, or dance.
 * In the United States, an estimated number of 150,000-200,000 people are in risk of inheriting Huntington's from one or both of their parents.
 * Contrary to popular belief, Huntington's Disease is not fully genetically passed on; if a child does not inherit the Huntington's gene, he/she will have no chance of developing Huntington's Disease or of passing it on to offsprings, since Huntington's does not "skip" generations, and will not be inherited in such way.
 * Diets and supplements, as well as exercise are recommended for patients diagnosed with Huntingtong's Disease. Foods such as cantaloupe, black currants, blueberries, sweet potatoes, asparagus, kale are known to be able to help nourish the brain, aid memory and support immunity for Huntington's patients.
 * Men and women have equal chances of being affected.
 * An estimate of 6,500 to 8,000 people are diagnosed in the UK. Twice as many people may have inherited the disease but have not yet discovered it due to undeveloped symptoms. This group of unaware victims, may have had children and passed on the disorder.


 * The Huntington's Disease is usually inherited from the father rather than the mother.
 * Some patients, due to psychiatric effects, lose more than 25% of their brain cells before they die.

=__Images and Diagrams__=



**__Video of Huntingtons Disease__**
media type="youtube" key="65xf1olEpQM"

**__Bibliography__**
-[|www.wrongdiagnosis.com/h/huntingtons_disease/inherit.htm] -[|www.hdsa.org] -[|www.huntingtonproject.org] -[|www.euro-hd.net] -[|www.highhqfoundation.org] -[|www.hda.org.uk/charity/research.html] -[|www.sirna.com/wt/page/neurology] -http://www.youtube.com/watch?v=65xf1olEpQM http://www.helpguide.org/elder/huntingtons_disease.htm http://www.nhsdirect.nhs.uk/articles/article.aspx?printPage=1&articleId=199