Background: Although the typical
Huntington's disease symptoms can develop at any time, but they often first appear when people are in their 30s or 40s. If the condition develops before age 20, it's called juvenile Huntington's disease. When Huntington's develops early, symptoms are somewhat different and the disease may progress faster.
People can start to show the symptoms of Huntington's disease at almost any age. Most will develop problems between the ages of 30 and 50. The condition gradually gets worse for around 10-25 years, until the person dies.
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. It's only done once all the benefits and risks have been explained.
Huntington's disease can affect someone physically, their thinking and their behaviour. Most people start experiencing symptoms as young adults or in middle age, though some people develop a rapidly progressive form of the disease before the age of 20.
Huntington's disease (HD) is a progressive brain disorder caused by a defective gene. This disease causes changes in the central area of the brain, which affect movement, mood and thinking skills.
HD is caused by a mutation in the gene for a protein called huntingtin. The defect causes the building blocks of DNA called cytosine, adenine, and guanine (CAG) to repeat many more times than they normally do. Most people have fewer than 27 CAG repeats in their HD gene, so they are not at risk for the disease.
Early signs and symptoms can include irritability, depression, small involuntary movements, poor coordination, and trouble learning new information or making decisions. Many people with Huntington disease develop involuntary jerking or twitching movements known as chorea.
The Age of Persons Experiencing the Onset of HD Can Vary
About 10% have onset of motor symptoms after age 60 and 10% have Juvenile onset HD, where symptoms manifest before age 20.
You're usually only at risk of developing it if one of your parents has or had it. 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.
Symptoms typically emerge from age 30 to 50, but also can develop in children and older adults. Late-onset Huntington's, characterized by some as emerging after age 5o and others after age 60, is thought to be less severe than earlier onset Huntington's.
During late-stage Huntington's disease, or stage V, individuals experience speech difficulty, weight loss, and loss of bowel and bladder control. They will have dementia and severe limitations in voluntary movement and will be fully dependent on assistance.
There's currently no cure for Huntington's disease or any way to stop it getting worse.
Although stress does not play a direct role in the onset and development of Huntington´s disease (HD) itself, it does have an influence on the course of the disease.
Medical imaging techniques, such as computerized tomography (CT) and magnetic resonance imaging (MRI) can reveal atrophy of the caudate nuclei, which is observed in the early stages of Huntington's disease.
In this first Asian study on survival in HD patients, the median survival from onset was 14.5 years. Although a direct comparison is not possible, it appears that the mean survival in our study is shorter that that reported by Rinaldi et al. [14] (20 years, 95% CI: 18.3–21.7). In a study by Pekmezovic et al.
HD is a rare, adult-onset, autosomal dominant, progressive neurodegenerative disease. George Huntington (Figure 1) was the first person to provide a comprehensive description of adult-onset HD in 1872; he was only 22 years old at the time.
In Australia it is estimated that over 1,800 people have Huntington's Disease and approximately 9,000 are at risk (Huntington's NSW ACT website, 2019) with a reported prevalence rate in Australia which ranges from 4.5 per 100,000 to 6.5 per 100,000 (Pringsheim et al., 2012).
Huntington's disease impairs the functioning of the brain, which can result in apathy, trouble organizing, impulsivity, irritability and anger, unawareness, disinhibition, preservation, and other psychiatric symptoms. These emotional and behavioral symptoms can further complicate the caregiver's role.
HD is a genetic disorder caused by expanded CAG trinucleotide repeats in the huntingtin (HTT) gene [1]. Conversely, the cause of MS is unknown, and possibly a result of an environ- mental trigger in a genetically susceptible host [2].