What is Huntingtons disease?

Answer 1

Huntington's disease (know as HD) is a fatal neurological disease. It results from genetically programmed degeneration of brain cells in certain areas of the brain.

It is a brain disorder usually appearing in mid-life but it can also strike in teenage years. HD results in emotional disturbance, uncontrolled movements, and loss of intellectual ability. A faulty gene causes this disease. The faulty gene runs in families. The faulty gene is passed from parent to child through the mutation of a normal gene. Whether one child inherits the gene will have no bearing on whether other children in the family will inherit the gene. If you have one parent with Huntington's disease, you have a 1 in 2 chance of inheriting the faulty gene. A person who has HD usually first develops symptoms that seem out of character.

Early symptoms include:

• Mood swings
• Short-term memory problems
• Mood changes
• Mild tremor
• Clumsiness
• Depression
• Lack of concentration
• Irritability
• Anti-social behaviour
• Trouble driving a car
• Increasing difficulty learning new things

Concentrating or focusing on intellectual tasks becomes more difficult as the disease progresses; symptoms of the disease worsen over time. Eventually a person with HD may have difficulty feeding and swallowing. Scientists are still researching how the faulty gene causes the disease. At this time (2011-03-20), there is no known way to reverse or stop the course of HD. However, research continues and in recent years scientists have made a number of breakthroughs in this area. At this time, HD is generally fatal within 20 years of symptoms first appearing, but I think that future research will bring a more positive prognosis.

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Answer 2

Dr. George Huntington of Long Island, New York first described this disease in 1872. Huntington's Chorea is inherited, degenerative disorder of the Central Nervous System caused by a dominant gene. If one person's parents has developed this disease there is a 50% chance their children will inherit it. The symptoms generally start around 35 years of age, but can onset later. This disease causes dysfunctional movements (often referred too as 'clumsiness' in it's earlier stages), but as the disease progresses the movements of the person become uncontrollable. The movements and body postures appear bizarre. Other symptoms include: Forgetfulness; irritability and as the disease progresses dementia, memory loss and severe anger outbursts as well as lack of reasoning. Patients suffering from Huntington's Chorea show degenerative changes in the basal ganglia structures, which ultimately result in a severely shrunken brain and enlarged ventricles. The symptoms are caused by a significant reduction in volume and activity of two principle neurotransmitters (naturally occurring chemicals in the brain) called Acetylcholine and GABA and, in turn affecting the activity of the neurotransmitter Dopamine which becomes hyperactive. Medications can ease the symptoms (including the use of dopamine antagonists or neuroleptics) but, there may be severe side effect. There is no cure for Huntington's Chorea. However, new techniques involving neural grafting (implanting healthy fetal brain cells into the damaged area) may offer hope for the sufferer in the near future. This disease may continue relentlessly for 10 to 20 years and cause of death is commonly Heart disease after a severe illness or due to falls or choking. There is hope! Scientists are getting closer to a cure for this disease and in the near future they will be advanced treatments and medications.

Answer 3

Huntington's chorea is a disease that is a progressive, degenerative neurological disorder that slowly takes away a persons ability to walk, talk, and reason.

In the cases I've seen the onset hits earlier in women than in men. Two examples are my aunt and my uncle. (These are my mothers older siblings.)

My aunt started showing signs in her mid-30s, just like her mother, and also just like her mother, she passed away within 10 years of the official diagnoses.

My uncle started showing signs in his late-30s or early-40s and lasted close to 15 years after his official diagnoses.

There are things that can slow down the progression of the disease, but there is no cure.

This disease has a 50/50 chance of being spread to the children of a couple where only one has the disease. But in the almost unheard of instance where 2 carriers have a child, it is 100%, that child will also have the gene. And then there is the chance that this child's signs will start to show in the late teens and early 20s.

My mom and her younger brother were tested almost immediately after the onset and official diagnoses of their older siblings. (The latter were diagnosed within months of each other.) Niether are carriers. So I am not a carrier. While this is a good thing for my family and the families they will have, I worry everyday about the my cousins. My aunts daughter, who is my oldest cousin, was diagnosed at 35 and now at 39 lives in a nursing home. Her symptoms started to show a lot earlier than my aunts and I know we may only have 7 or 8 years left with her. It makes me sad for her daughter. And incredibly worried for her too.

Answer 4

Huntington's disease, chorea, or disorder(HD), is aneurodegenerative genetic disorder that affects muscle coordination and leads to cognitive decline and dementia.

Answer 5

Huntington' s disease, chorea , or disorder ( HD) , is a neurodegenerative genetic disorder that affects muscle coordination and leads to cognitive decline and dementia. It typically becomes noticeable in middle age . HD is the most common genetic cause of abnormal involuntary writhing movements called chorea and is much more common in people of Western European descent than in those from Asia or Africa. The disease is caused by an autosomal dominant mutation on either of an individual's two copies of a gene called Huntingtin , which means any child of an affected parent has a 50 % risk of inheriting the disease. In rare situations where both parents have an affected gene, or either parent has two affected copies, this risk is greatly increased. Physical symptoms of Huntington's disease can begin at any age from infancy to old age , but usually begin between 35 and 44 years of age . About 6% of cases start before the age of 21 years with an akinetic-rigid syndrome ; they progress faster and vary slightly . The variant is classified as juvenile, akinetic -rigid or Westphal variant HD .

Answer 6

According to the Mayo Clinic "Huntington's diseases is caused by an inherited defect in a single gene".