o How many Australians have Huntington's disease? The best available published evidence of relevance to Australia suggests a prevalence rate of 8.4 per 100,000 people. This indicates that with a current population of 25.7 million people in Australia1, there is currently around 2,160 people with a diagnosis of HD.
HD is rare, affecting about 30,000 Americans.
Huntington's disease is currently found in many different countries and ethnic groups around the world. The highest frequencies of HD are found in Europe and countries of European origin, such as the United States and Australia. The lowest documented frequencies of HD are found in Africa, China, Japan, and Finland.
Huntington's disease (also known as Huntington's chorea) is a rare genetic disorder that usually affects middle-aged people. It destroys cells in the brain, affecting the ability to move, think, and behave.
Frequency. Huntington disease affects an estimated 3 to 7 per 100,000 people of European ancestry. The disorder appears to be less common in some other populations, including people of Japanese, Chinese, and African descent.
Huntington's disease is caused by a faulty gene. Cells in parts of the brain are very sensitive to the effects of the faulty gene. This makes them function poorly and eventually die. A parent with the Huntington's disease gene has one good copy of the gene and one faulty copy.
There have been reports that juvenile onset Huntington's chorea is almost always inherited from the father, and that late-onset Huntington's chorea is inherited more often from the mother than from the father.
When to see a doctor. See your health care provider if you notice changes in your movements, emotional state or mental ability. The signs and symptoms of Huntington's disease can be caused by a number of different conditions. Therefore, it's important to get a prompt, thorough diagnosis.
Lots of people at risk of Huntington's disease decide they'd rather not know until any symptoms appear. If you do want to know, ask your GP for a referral to a genetic counsellor. You'll have several appointments with the counsellor.
The overall presentation of HD was considered to be initially mild by most families in this study, but progression in motor and/or behavior symptoms eventually occurred in all 30 subjects evaluated more than once.
In the United States, Huntington's disease occurs in about one of every 10,000 to 20,000 people. It affects males and females equally and crosses all ethnic and racial boundaries. Typically, symptoms begin between age 30 and 55.
Huntington's disease does not skip generations. Each child of a parent with Huntington's has a 50% chance of inheriting the defective gene. If a child does not inherit the gene, he or she cannot pass it on. If the child does inherit the gene, he or she can pass it on and will develop the disease.
The future of Huntington's disease management
“The goal is to slow down the course of Huntington's disease, preferably preventing its progression entirely.” Younes indicated there might even be an opportunity to proactively start treatment for people who carry the genetic mutation that causes Huntington's disease.
The symptoms usually start at 30 to 50 years of age, but can begin earlier than this (juvenile Huntington's disease) or much later. Once they start, the symptoms usually get gradually worse.
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that usually starts in mid-adult life. The clinical disease progresses to death over an average of 20 years.
If a parent has Huntington disease, the child has a 50% chance of developing it. If the child doesn't develop the disease, he or she won't pass it along to his or her children. For 1% to 3% of people with Huntington disease, no family history of the disorder is ever identified.
The survival of Huntington's disease (HD) patients is reported to be 15–20 years. However, most studies on the survival of HD have been conducted in patients without genetic confirmation with the possible inclusion of non-HD patients, and all studies have been conducted in Western countries.
The most painful conditions in Huntington's, said Achterberg, are limb pain, headache, abdominal pain and fractures. “I think we should do more work in validation of pain tools, including observational pain instruments,” he said. “And for clinicians, I suggest you be especially aware of abdominal pain.”
Conclusion The most primary cause of death in HD is aspiration pneumonia.
They may lack inhibition, and do or say things that one would normally find embarrassing. People with Huntington's may also be less able to control their emotions, possibly leading to outbursts of screaming, swearing, slamming doors, hitting walls, or the like. Patterns of behavior can change as the disease progresses.
Both men and women can get it. If a parent has the Huntington's disease gene, there's a: 1 in 2 (50%) chance of each of their children developing the condition – affected children are also able to pass the gene to any children they have.
In early stage HD, individuals are largely functional and may continue to work, drive, handle money, and live independently. Symptoms may include minor involuntary movements, subtle loss of coordination, difficulty thinking through complex problems, and perhaps some depression, irritability, or disinhibition.
As I mentioned before, it is possible to get HD even if your parents don't have it. If your parent passes away before showing symptoms of HD, you might not know whether they had a broken HD gene.