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</html>";s:4:"text";s:6618:" Huntington's disease is an inherited brain disorder that is caused when specific cells in the brain die. Symptoms typically start in middle age between 30-50 years, although they can also develop in younger and older people and become worse as the disease progresses until death occurs. Huntington's disease is often described as a family disease. While it is not a common disease, its impact is far-reaching. The complexity of this decision is likely why only 10% of people living at-risk in the USA decide to pursue predictive testing. A mutation is a change in a gene that causes a disease. The specific region of the brain impacted by Huntington’s disease is the basal ganglia, a region deep in the brain that is also known to be responsible for Parkinson’s disease. The outcome of the disease and the lack of treatment are among the reasons why people who have known Huntington’s disease in the family seek out genetic testing. Huntington’s disease is a relatively uncommon condition, affecting approximately 1 out of every 10,000-20,000 people worldwide, with a slightly higher prevalence among people of European descent. This decision is extremely complex, with many factors weighing in as pros and cons. 2015 Apr 23;1:15005. doi:10.1038/nrdp.2015.5, Richard H. Myers. HD is caused by a mutation in a gene that all humans have. Huntington's disease affects one in every 10,000 persons or approximately 30,000 people in the United States. It's estimated that for every person who has Huntington's disease, the disorder impacts another 20 individuals including friends, caregivers, and family members who may be at risk for developing the disease themselves. 2004 Apr; 1(2): 255–262. The reason this disease is characterized as dominant is that having only one defective gene is enough to cause the condition, even if a person has another normal chromosome. Huntington’s disease is a hereditary condition that is marked by a severe movement disorder and progressive dementia. NeuroRx. The effectiveness of these drugs varies from patient to patient and they tend to become less effective over the course of the illness. Gene coding is made of a sequence of nucleic acids, which are molecules on our DNA that code for the proteins that our bodies need for normal function. Like Parkinson’s disease, Huntington’s disease is characterized by movement problems, but Huntington’s disease is more rapidly progressive, is fatal, and dementia is the most prominent symptom of the disease. If you or someone you know has Huntington’s disease or has been told that the disease will emerge in upcoming years, it is helpful to connect with support groups as a way to understand about how to cope and to lean on those who have similar experiences. The discovery of the genetic mutation causing Huntington's disease made possible the use of predictive testing to identify currently unaffected carriers. The decision whether to have predictive testing is extremely personal and it's important to take your time and make an informed choice. What Does Genetic Counseling for Pregnancy Involve? A genetic counselor will also help you identify other issues, such as the impact testing may have on your relationship with your spouse, children and extended family members, your career, and your insurance and finances. Another way to prevent getting this page in the future is to use Privacy Pass. Read our, Medically reviewed by Diana Apetauerova, MD, Medically reviewed by Shaheen Lakhan, MD, PhD, Medically reviewed by Nicholas R. Metrus, MD, Verywell Health uses cookies to provide you with a great user experience. This is described as a CAG repeat. Huntington's disease is an autosomal dominant hereditary condition for which there is genetic testing. Although, individuals with 36-39 CAG repeats often have a later age of onset than what is typical observed. Unfortunately, Huntington’s disease is currently incurable, and those who are diagnosed with the disease have no treatment options except for supportive care, which is focused on injury prevention and avoidance of complications, such as malnutrition and infections. Persons with 26 CAG repeats or fewer are mutation-negative and will never develop the disease. The gene abnormality, an increase in CAG repeats, is found on chromosome 4. They will either inherit the gene with the mutation or they will get the normal gene from their parent. A neurological exam is also performed to look for early symptoms. Your IP: 185.2.4.44 By using Verywell Health, you accept our, Huntington's Disease Genetic Test Logistics, How the HD Gene Causes Huntington’s Disease, Dopamine: What It Does for You and Related Conditions, The Rare Genetic Disorder That Affects Muscle Control and Coordination, Explore the Treatment Options for Juvenile Huntington's Disease, Some Babies Are Born Weak Due to a Genetic Mutation — Here's Why, Why Neurofibromatosis Type 2 Causes Tumor Growth, Hereditary Risk Factors of Alzheimer’s Disease, Tardive Dyskinesia Is a Movement Disorder Due to Neuroleptic Use, How Genetics Testing Can Help Identify Parkinson's Disease, Medications and Non-Drug Approaches to Treat Huntington's Disease. Anyone considering predictive testing should meet with a genetic counselor. Individuals who receive a mutation-positive result may initially experience feelings of depression, anger, fear or despair. Huma Sheikh, MD, is board-certified in neurology and specializes in migraine and stroke. Additionally, while there is no treatment at this time, research is advancing, and you might consider participating in a research study as a way to learn about your options and to gain access to the latest developing and emerging therapeutic options. Heidi Moawad is a neurologist and expert in the field of brain health and neurological disorders. It is believed that this elongation or the resulting fragmentation may be toxic to nerve cells in the brain. For example, results from predictive testing could make it difficult to purchase life, disability or long-term care insurance. Genetic testing for Huntington’s disease is a huge advancement that can help with decision-making. This means that if a person inherits only one defective gene for Huntington’s disease, then that person is very likely to develop the disease. • If you would like more information on Huntington's disease, please visit the Huntington's Disease Society of America's website at http://www.hdsa.org . 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