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).
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 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.
1 Extrapolating across Australia's population of 23.7 million2, the number of people with HD is about 1,600. Given the hereditary nature of HD, this means another 8,000 people are at risk.
Affected populations
About 30,000 people in the United States have Huntington's disease and another 200,000 are at risk of developing the condition. Symptoms commonly develop between ages 30 and 50. The disease progresses slowly and a person may live for another 15-20 years after the onset of symptoms.
There was evidence of an increasing prevalence of between 15 and 20% per decade in studies from Australia, North America and Western Europe. Conclusions: The prevalence of HD varies more than tenfold between different geographical regions.
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.
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.
Very occasionally, it's possible to develop Huntington's disease without having a history of it in your family. But this is usually just because one of your parents was never diagnosed with it.
Summary. Although no disease-modifying therapies currently exist to slow or halt the progression of Huntington's disease, many new types of treatment are under investigation that may offer hope for the future.
Huntington's Disease (HD) is not fatal in itself. People with HD have a shorter life expectancy and die of other life-threatening complications related to this disease. Pneumonia and heart disease are the two leading causes of death for people with HD.
If you have Huntington disease, it is important to closely monitor you condition, and contact your healthcare provider if you notice any of the following symptoms: Increasing difficulty with motor tasks such as walking. Trouble swallowing. Mood changes, such as increasing symptoms of depression or feelings of suicide.
Myth 1: HD is a male disease. Fact: Both men and women can be born with the HD gene.
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.
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.
Symptoms of Huntington's disease usually develop between ages 30 and 50, but they can appear as early as age 2 or as late as 80. The hallmark symptom of Huntington's disease is uncontrolled movement of the arms, legs, head, face and upper body.
Huntington's disease can cause a wide range of symptoms, including problems with mental health, behaviour, movement and communication. 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 the symptoms start to affect your daily life, you will be diagnosed with active disease. The disease is staged based on your motor function and ability to complete everyday tasks. Typically, HD progresses for 10 to 30 years.
Symptoms of Huntington's Disease
These symptoms may include uncontrolled movements, impaired coordination, changes in behavior, emotion, judgment, cognition, and more. In addition, because it is a progressive disorder, symptoms become worse and additional symptoms will develop over time.
Typical age at onset of symptoms is in the 40s1. The cause is a gene mutation in chromosome 4 with a CAG trinucleotide repeat of 35 or greater2. Repeat size can expand or contract with the next generation.
While the cause of the disease is known — a single mutated gene — there is no cure. “Our plan is to conduct human clinical trials that deliver stem cells to replace damaged brain cells, reducing levels of harmful proteins that build up in the brains of Huntington's disease patients.”
On average, individuals with Huntington's disease live for 10 to 30 years after the onset of symptoms. However, it is essential to note that this is an average estimate, and some individuals may live longer or shorter than this range.