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Huntington's Disease

Huntington’s disease is a hereditary disorder that destroys certain areas of the brain involved in intellect, emotion, and movement development. It is characterized by the loss of mental abilities; developing of emotional problems; and uncontrollable movement of the limbs and face.

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Is huntingtons painful?

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It can be, especially for the family.

Can you die from huntingtons disease?

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Yes. Most individuals afflicted with Huntington's Disease die prematurely, due to the progression of the disease.

A person can be tested for the allele that causes huntingtons disease because of that allele is different from that of a normal allele?

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Because Huntington's is a genetic disorder and it is known what sequence in what region causes the disorder.

Is Huntingtons disease non disjunction?

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No. Hemophilia is the result of any of a number of mutations such as reversals, translations, deletions, etc. These mutations need to have occurred in a very specific segment of the coding found on the X chromosome. The replication of that X chromosome however is unaffected with the exception that the mutation on it is replicated as well.

How do you know if you have got huntingtons disease?

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First, since this is a genetic disease, that is transmitted only by inheritance, a determination of family history of HD is usually made. There are also characteristic symptoms that become noticeable in later life, most commonly chorea- or uncontrolled movements of the body. In recent years, a genetic test has been developed that permits screening the the chromosome that causes HD- if you do not have that chromosome, you do not have, and will not develop HD. If you DO have that chromosome, at some point the symptoms of HD will develop. The exact age varies from one person to another. There is additional information at the website of the Huntington's Disease Society of America.

Is Huntington disease more common in some parts of the world than others?

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Yep- people who had a parent with HD. Since it is a genetic disease, that is the only way to get it- inherit it from a parent. Since it is inherited, there will be areas that have more cases- notably England, Australia, and Venezuela.

What results in either too much or too little movement as exemplified by Huntington's disease and Parkinson's disease?

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Disorders of the _________result in either too much or too little movement, as exemplified by Huntington's disease and Parkinson's disease

Do genes effect your health?

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Yes. For example if cancer if very common in your family, you likely have genes that are more susceptible to it. That's why people say "its hereditary." It means its caused by your genes. If this is hereditary for you, you are more likely to die from cancer.

How can you inherit huntingtons disease?

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Huntington's is a genetic disease meaning that it is inherited to offspring of effected parents. If one of your biological parents have it you are at risk. I would ask my doctor for genetic testing to see if I had it.

What is the perfect time for testing for huntington's disease?

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A very, VERY personal decision. A person that has a parent that has Huntingtons, and is now an adult, might decide to be tested for HD prior to having children, or to help plan what they want to do in life. At present, there is no cure and limited treatment for Huntingtons. Some people may not WANT to know that they have a disease that is ultimately fatal, and has no cure. My wife has HD, and we have 4 adult children. Three of them decided to be tested- one decided not to.

Huntington's disease is an example of a genetic disorder caused by?

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It is a familial disorder that is genetic, and causes degeneration of some of the nerve cells in the brain. You inherit it from your parents.

The defect is in the HD gene (an inherited unit which contains a code for a protein), which is located on the short arm of chromosome 4.

When does huntington's disease appear?

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The age at which the SYMPTOMS of HD appear varies. For MOST people, it is during their 30s-40s. With some people, it is older. There are a very few cases of juvenile HD, where the symptoms appear with children a few years old- but that is extremely rare.

How does huntington's disease affect the nervous system?

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Yes, Huntington's disease is a central nervous system disorder.

Why is huntingtons disease more common than other serious dominant genetic disorders?

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Huntingtons is a genetically inherited disease from a parent. when chromosomes are passed onto a child, the child wont always receive the chromosome containing the gene. But, if they do inherit the gene they will have the disease no matter what, because the disease is dominant. If it was recessive, the child could inherit the disease but not necessarily get the disease.

What is the neurotransmitter that is deficient in huntington's disease?

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The neurotransmitter that has been thought to be deficient is enkephalin. The striatal neurons in the caudate and putamen degenerate, causing decrease release of enkaphalin, leading to decreased activity of the indirect pathway of the basal ganglia. There is decreased inhibition on the globus pallidus externus, leading to increased inhibition of the subthalamic nulceus. This inactivates the indirect pathway, leading to hyperkinetic movements.

What is the percentage of people in the world have Huntington's disease?

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Becasue HD is carried on a dominant gene. Unlike recessive gene diseases, where you would have to inherit the gene from BOTH parents, with a dominant gene disorder- well, you will inherit one of two genes from the parent with HD. One carries HD, the other does not. Odds are 1 out of two, or 50%. For each child born to that parent.

Is the allele causing huntington's disease dominant or recessive?

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Huntington's disease is caused by a dominant allele

What is everyday life like with Huntingtons disease?

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Depends on what stage of the disease has been reached. Up until symptoms appear, life is the same as for anyone else. As the symptoms increase, the person will have difficulty with movement, appear to be clumsy, spill or drop things. Their walk will become unsteady, and speech slurred- they may appear to be intoxicated- a problem when dealing with law enforcement officers. Problems with swallowing and choking are common, as are falls. Persons will also have problems with memory, reasoning, and be subject to irrational angry outbursts.

Is Huntingtons Disease a recessive gene?

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Unfortunately, HD is carried on a dominant gene. If one parent has the gene for HD, and the other does not, the offspring has a 50/50 chance of developing HD. However, if you do NOT inherit the gene for HD, you cannot be a "carrier" of HD. The Huntingtons Disease Society of America has more information at their website. You may also contact me through my message board if you need additional information.

Are there any cures for huntingtons disease?

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Various drugs may be used to treat Wolff-Parkinson-White syndrome, as well as other cardiac arrhythmias. The purpose of these drugs is to slow the electrical signals and excitation of heart muscles.

How does huntington's disease progress?

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until complete heart failure and biological death!

addition:

Tay-Sachs disease is a fatal genetic lipid storage disorder in which harmful quantities of a fatty substance called ganglioside GM2 build up in tissues and nerve cells in the brain.

Presently there is no treatment for Tay-Sachs disease. Anticonvulsant medicine may initially control seizures. Other supportive treatment includes proper nutrition and hydration and techniques to keep the airway open. Children may eventually need a feeding tube.

Even with the best of care, children with Tay-Sachs disease usually die by age 4, from recurring infection.

At the present time, the prognosis for a baby born with Tay-Sachs disease is certain death. Nothing can be done to keep the baby alive.

What is your life expectancy if you have Huntington's disease?

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No real way to answer, since there is no way to know how long ANY one person will live. HD DOES shorten your life expectancy, by it varies person to person. For a very few people that have juvenile HD, symptoms appear when they are a few years old, and their lives may be very short. Adults may typically see onset of symptoms in the 30's to 50's, and live about 20 yrs beyond that. My wife has HD, and is 69 years old. No one person, no one answer. See the website for the HD Society of America for more information.

How does Huntingtons disease affect physical features?

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Huntington's disease is an incurable hereditary disease that affect the motor neruons in the brain, causing misfiring and eventually nerve and cell death. Its sufferers beginto get jerky, parkinsons like movement, which in late stages becomes detremental to physical health. Although Huntington's won't directly kill you, sufferers are killed by secondary infections such as pneumonia or get heart attacks as a result of hypertension. Huntington's also causes changes in personality, including depression and dementia. See the link below for information on HD.