Updated: Jan 8
Huntington’s Disease (HD) is an inherited, yet rare, neurodegenerative disease. It has a broad impact on a person’s physical, mental, and emotional capabilities and is terminal. Symptoms generally begin during adulthood and include depression, irritability, and poor coordination. Individuals may also experience changes in personality. People with HD live between 10-20 years after signs and symptoms begin.
This article provides insight into the etiology, epidemiology and management of Huntington's Disease.
What Is Huntington’s Disease (HD)?
Huntington’s Disease is a genetic and progressive brain disorder. It causes the breakdown of nerve cells in the brain. It is known as a “family disease” because the child of any HD patient has a 50% chance of developing the fatal disease. A person who develops HD before the age of 20 is categorized as having juvenile or jHD. This specific type can have different symptoms and the disease may progress faster.
Currently, there are about 41,000 symptomatic American patients. Furthermore, there are over 200,000 individuals at risk of inheriting the disease. HD affects approximately 3 to 7 people per 100,000 of European ancestry. However, the disorder is less common in people of Japanese, Chinese, or African descent.
What Causes HD?
Huntington’s Disease is caused by a mutation in the HTT gene, which is responsible for a protein called huntingtin. It plays an important role in the neurons in the brain. A person needs only one copy of the defective gene to develop the disorder, which categorizes HD as an autosomal dominant disorder.
How Does HD Affect People?
Huntington’s Disease causes movement, cognitive, and psychiatric disorders. Symptoms vary from person to person, and some appear more dominant or have a greater effect on a person’s function. However, this can change throughout the progression of the disease.
Movement disorders can affect voluntary or involuntary actions. For example, involuntary jerking or writhing is called chorea. There can also be troubles with muscle rigidity or contracture (dystopia). Slow or abnormal eye movements, as well as difficulty with speech or swelling, can be voluntary movement symptoms. Impairments in voluntary movements have a greater effect on a person’s ability to remain independent.
Cognitive disorders include difficulty organizing, prioritizing, or focusing. Patients may lack flexibility or impulse control, sometimes leading to sexual promiscuity. Furthermore, a lack of self-awareness and slowness in processing can occur.
The most common psychiatric disorder associated with HD is depression due to changes in brain function. Patients may experience irritability, sadness, apathy, social withdrawal, insomnia. fatigue, and suicidal thoughts. Furthermore, obsessive-compulsive disorder (OCD), mania, and bipolar disorder (BD) are also common.
Furthermore, weight loss is common as the disease progresses.
In juvenile Huntington’s disease, symptoms vary slightly from adults. They can include difficulty focusing, rapid drop in school performance, and behavioral issues. Frequent falls, clumsiness, seizure, and tremors are also common.
How Can Technology Affect HD Patients?
Rockefeller University (US) has developed a technology, using neuroloids (miniature 3D tissue cultures) to research cell groupings from human embryonic stem cells. The continued research and development of this tech can help screen for future Huntington’s treatments.
Furthermore, a team of researchers from the UCL Huntington’s Disease Centre in London conducted a study on how wearable devices may aid in the diagnosis of neurological disorders, specifically Huntington’s. Although the technology needs improvement, researchers hope that earlier detection of the disease can lead to being able to stop its progression.
How Can Nootropics Aid Patients?
Huntington’s disease causes mitochondrial dysfunction, which results in a reduction in cellular energy. In theory, a therapy that raises cellular energy levels would slow down the neurodegenerative process caused by HD. Creatine, a nootropic, is critical in maintaining cell energy and maybe among many erogens that provide a safe and readily available therapeutic strategy to help delay the progression of HD.
Huntington’s disease is a fatal neurodegenerative disorder. Caused by a genetic mutation with a 50% chance of inheritance, it results in movement-related, cognitive, and psychiatric changes. There is no cure or proven method of slowing the progression of the disease, but technology is being developed to help in earlier detection of the disease, as well as future cures.
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A friendly reminder: We've done our research, but you should too! Check our sources against your own and always exercise sound judgment.
https://www.mayoclinic.org/diseases-conditions/huntingtons-disease/symptoms-causes/syc-20356117 https://hdsa.org/what-is-hd/overview-of-huntingtons-disease/ https://medlineplus.gov/genetics/condition/huntington-disease/#causes https://www.drugtargetreview.com/news/49580/new-technology-developed-to-screen-for-huntingtons-treatments/ https://huntingtonsdiseasenews.com/2021/03/02/wearable-devices-may-aid-huntingtons-diagnosis-but-evaluation-studies-need-improvement-review-finds/ https://pubmed.ncbi.nlm.nih.gov/16055197/ Image credits: https://unsplash.com/ by Hal Gatewood and CDC
About the Author
Project Intern for The Hope Project (Texas, USA)
Davinia Ibizugbe is a Nigerian writer. She has planned her education around the hopes of becoming a future Neurosurgeon. Her interest in neurology developed early in her life. Davina has a passion for learning about neurological diseases and methods to combat them.