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Huntington's disease (HD) is a neurodegenerative condition that involves different combinations of movement disorders, dementia, and behavioural or psychiatric manifestations. It is estimated that around 2,160 Australians have an HD diagnosis, with approximately 1,200 Australians living with the disease and 6,000 people at risk. The prevalence of HD varies across Australia, with Tasmania having the highest prevalence of 12.1 cases per 100,000 people. In most Western countries, the predicted prevalence of HD is 5 to 7 cases per 100,000 individuals, which is more in line with the rest of the Australian continent.
| Characteristics | Values |
|---|---|
| Number of people with Huntington's Disease in Australia | 1,200 (approximately) |
| Number of people at risk | 6,000 |
| Prevalence in Tasmania | 12.1 per 100,000 people |
| Prevalence in Australia | 5 to 7 per 100,000 people |
| Prevalence in Caucasian populations | 5.7 per 100,000 |
| Prevalence in Asian populations | 0.40 per 100,000 |
| Prevalence based on a 2010 meta-analysis | 2.71 per 100,000 persons |
| Prevalence based on a meta-analysis of studies from 1985 to 2022 | Increasing modestly over time |
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What You'll Learn
Prevalence in Tasmania
Tasmania, an island located off the southern coast of Australia, has the highest prevalence of Huntington's disease (HD) in the country, with 12.1 cases per 100,000 people. This is significantly higher than the predicted prevalence of 5 to 7 cases per 100,000 individuals in most Western countries. The unusually high prevalence in Tasmania has attracted the attention of researchers, who have conducted various studies on the region's HD population, making it a well-studied area in the global context of the disease.
The origins of HD in Tasmania can be traced back to 1842 when an English woman named Mary, who was born in Long Sutton, Somerset, England, in 1806, migrated to Launceston, Tasmania, with her husband, Charles, and their seven children. Mary went on to have seven more children in Tasmania, and out of her 14 children, nine developed HD. These nine children later had children of their own, perpetuating the presence of the disease in the region. As of 1990, seven of the nine lineages descended from Mary still had living descendants, with six branches containing descendants who were either at risk of developing HD or already affected by it.
The high prevalence of HD in Tasmania has resulted in the establishment of support systems and associations to assist those impacted by the disease. The Huntington's Disease Association provides financial and moral support, special equipment, food supplements, and information resources to Tasmanians affected by HD. The association also facilitates connections between individuals and families affected by HD, fostering a sense of community and support.
In addition to the Huntington's Disease Association, other initiatives have been undertaken to support and improve the lives of those impacted by HD in Tasmania. The Map-HD Registry, led by Professor Julie Stout at Monash University's Turner Institute for Brain and Mental Health, aims to map the locations of individuals with HD across Australia, including Tasmania, to identify obstacles in accessing clinical care and community services. This registry helps increase the efficiency of HD research, connects people with research and clinical trial opportunities, and informs the development of new treatments.
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Prevalence in other states
Huntington's disease (HD) is a neurodegenerative condition with wide-ranging neuropsychiatric implications. It is a rare disease with a predicted prevalence of 5 to 7 cases per 100,000 individuals in most Western countries. However, the prevalence in Australia varies, with some areas reaching as high as 12.1 cases per 100,000 people. This variation in prevalence across the continent has prompted researchers to conduct various studies to understand the disease better.
Tasmania, an island state off the southern coast of Australia, has the highest prevalence of HD in the country, with 12.1 cases per 100,000 people. The unusually high prevalence in Tasmania can be traced back to 1842 when an English woman named "Mary" from Somerset, England, brought the disease to the region. Mary had 13 children, 11 of whom carried the HD gene. Nine of her 14 children developed HD, and as of 1990, seven of these nine lineages still had living descendants affected by or at risk of HD. This unique situation has made Tasmania a well-studied and important area in the global context of HD research.
The Australian state of Victoria, particularly Melbourne, has also contributed significantly to HD research. The first HD clinic in Australia was established at the Royal Melbourne Hospital in 1972 by Professor Edmond Yu-Kien Chiu. Melbourne-based researchers, such as Dr Danny Hatters from the University of Melbourne's Bio21 Institute, are actively studying the role of huntingtin (Htt) clumps in disease pathogenesis. Additionally, the Map-HD Registry, an initiative of the Huntington's Disease Network of Australia (HDNA), is based at Monash University's Turner Institute for Brain and Mental Health in Melbourne. This registry aims to map the locations of individuals with HD, identify obstacles in accessing clinical care and community services, and understand their experiences with disability insurance entitlements.
While Tasmania and Victoria have been prominent in HD research and community support, other states in Australia are also making important contributions. For example, Clement Loy and Elizabeth McCusker in New South Wales and Peter Panegyres in Western Australia are involved in significant HD research projects. Furthermore, collaborative projects are taking place in Adelaide, South Australia, utilising a sheep model of HD and conducting other clinical research. These efforts demonstrate a nationwide commitment to understanding and supporting individuals affected by HD in Australia.
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Risk factors
It is estimated that around 1,200 to 2,160 Australians have an HD diagnosis, and 6,000 people are at risk. The prevalence of Huntington's disease varies throughout the continent, reaching as high as 12.1 per 100,000 in some areas, such as Tasmania. This is an unusually high prevalence compared to other Western countries, where the predicted prevalence is 5 to 7 cases per 100,000 individuals.
Huntington's disease (HD) is a neurodegenerative condition with a wide range of neuropsychiatric manifestations. It is caused by a CAG trinucleotide repeat expansion in the huntingtin gene (HTT) on chromosome 4. The risk of developing HD is influenced by several factors:
Genetic Factors
HD is inherited in an autosomal dominant pattern. Individuals with 40 or more CAG repeats in the HTT gene will likely express the condition. The number of CAG repeats is inversely correlated with the age of onset, meaning that a higher number of repeats may lead to an earlier onset of the disease.
Family History
A positive family history of HD is a significant risk factor. In Australia, there have been cases of large families with multiple generations affected by HD, contributing to the high prevalence in certain regions, such as Tasmania.
Ethnicity
The prevalence of HD varies among different ethnic groups. Caucasian populations have a higher prevalence, with 5.7 per 100,000 cases, compared to 0.40 per 100,000 in Asian populations.
Brain Atrophy
Structural MRI studies have found that atrophy of certain brain regions, such as the caudate and putamen, is correlated with decreased functional capacity in individuals with manifest HD. However, further studies are needed to fully understand the relationship between brain atrophy and mortality in HD patients.
Psychiatric Conditions
There is a link between HD and psychiatric conditions. People with a family history of psychiatric disorders or dementia may have an increased risk of developing HD.
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Support and services
It is estimated that around 2,160 Australians have been diagnosed with Huntington's disease (HD). However, many are not receiving the care and services they need. To address this, Australia has developed a large and supportive HD community.
Huntington's Australia (HA) is the peak national body dedicated to supporting individuals and families impacted by HD across the country. It was established in October 2023 through the unification of five state and territory associations. HA provides a wide range of services, including training and education for caregivers, disability support providers, allied health professionals, and other stakeholders. They also offer comprehensive support for individuals at every stage of their HD journey, from those at risk or newly diagnosed to those experiencing advanced symptoms.
The Huntington's Disease Association provides support specifically for Tasmanians affected by HD, including those living with HD, families, caregivers, and anyone at-risk. The association can offer financial and moral support, special equipment, food supplements, and information booklets.
Additionally, the Map-HD Registry, an initiative of the Huntington's Disease Network of Australia (HDNA), is Australia's first nationwide registry to map people living with HD. The registry aims to identify the locations of individuals with HD, obstacles in accessing HD clinical care and community services, and experiences with Commonwealth-supported disability insurance entitlements. This information will help improve care and services for those with HD and increase the efficiency of research and the development of new treatments.
The Huntington's Community Connect is an online peer forum for community members directly impacted by HD. It is a safe space for individuals to connect with others, share stories, and exchange advice and guidance. The forum is actively monitored and regularly updated to ensure it meets the needs of the HD community.
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Research and treatments
Huntington's disease (HD) is a debilitating neurodegenerative disease that is currently incurable. It is a rare genetic brain disease caused by a defective gene that produces too much of a protein called huntingtin. The build-up of this protein damages cells in the brain, affecting the nervous system. The classic signs of HD include emotional, cognitive, and motor disturbances, including jerky involuntary movements (chorea), rigidity, changes in using the limbs (apraxia), loss of control of bodily functions, and dementia. The median age of onset is 41 years old, and the median survival is 18.8 years.
Huntington's disease is a rare and debilitating condition that affects a small percentage of the population in Australia and worldwide. While there is currently no cure for HD, several research initiatives and treatments are underway to better understand the disease and improve the quality of life for those affected. Here is an overview of some key research and treatment efforts:
- Australia's first nationwide registry, Map-HD Registry: Led by Professor Julie Stout at Monash University's Turner Institute for Brain and Mental Health, this initiative aims to map the locations and needs of people living with HD across Australia. By collecting vital information, the registry will help identify areas where HD support and clinical services are needed, improve the efficiency of HD research, connect people with research and clinical trial opportunities, and inform the development of new treatments.
- Structural magnetic resonance imaging (MRI) studies: These studies have found atrophy of specific brain structures, such as the caudate and putamen, to be correlated with decreased functional capacity in individuals with manifest HD. While clinical measurements such as reduced frontal horn width to intercaudate distance ratio (FH:CC) or increased intercaudate distance to inner table width ratio (CC:IT) have been used to quantify atrophy, more research is needed to understand the relationship between brain atrophy and mortality in HD.
- Preclinical therapeutic approaches: Researchers at The Florey have successfully tested new preclinical therapeutic approaches, which will be taken into new clinical trials. One such discovery is a drug that has beneficial effects on mitochondria and synapses that use the neurotransmitter glutamate. This has led to a new clinical trial, in collaboration with Australian colleagues, aimed at delaying the onset of Huntington's disease in those with the gene mutation.
- Gene-silencing agents and experimental drugs: Researchers are optimistic about the potential of gene-silencing agents to slow down or even stop the progression of HD. One such experimental drug, RG6042, has shown promising results in early trials, with significant reductions of mHTT (the faulty huntingtin protein) in participants. However, larger trials are needed to confirm the drug's efficacy and safety before it can become widely available.
- Australian Huntington's Disease Association Tasmania Inc.: This association provides comprehensive support for Tasmanians affected by HD, including financial and moral support, special equipment, food supplements, information booklets, and connections to others affected by the disease. The association also aims to educate families and help them cope with and understand HD, empowering them to develop a strong unified voice.
These research and treatment efforts provide hope for individuals and families impacted by Huntington's disease in Australia. While there is currently no cure, the ongoing dedication to advancing diagnoses, treatments, and patient outcomes offers the potential for improved quality of life and, ultimately, a transformative breakthrough in the understanding and management of this debilitating disease.
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Frequently asked questions
It is estimated that around 1,200 Australians have Huntington's disease (HD).
The prevalence of HD in Australia is estimated to be 5 to 7 cases per 100,000 individuals. However, in Tasmania, the prevalence is higher, with 12.1 cases per 100,000 people.
Approximately 6,000 people in Australia are at risk of developing HD.
Australia has developed a large and supportive HD community. Organisations such as Huntington's Australia (HA) and the Huntington's Disease Association provide support, education, and resources for individuals and families impacted by HD. The country's first nationwide registry, Map-HD, aims to map the locations of people with HD and identify areas where HD support and clinical services are needed.
