Question - Which of the following is a genetic disease that causes neurons in the brain to waste away and die?

Answered by: Karen Hill  |  Category: General  |  Last Updated: 22-06-2022  |  Views: 1477  |  Total Questions: 14

Huntington's disease (HD) is an inherited disease that causes certain nerve cells in the brain to waste away. People are born with the defective gene, but symptoms usually don't appear until middle age. Early symptoms of HD may include uncontrolled movements, clumsiness, and balance problems. Huntington's disease is caused by an inherited defect in a single gene. Huntington's disease is an autosomal dominant disorder, which means that a person needs only one copy of the defective gene to develop the disorder. The part of the brain most affected by HD is a group of nerve cells at the base of the brain known collectively as the basal ganglia. In Huntington's disease, traffic jams in the cell's control center kill brain cells. Huntington's disease is a relatively rare fatal inherited condition that gradually kills off healthy nerve cells in the brain, leading to loss of language, thinking and reasoning abilities, memory, coordination and movement. Degenerative nerve diseases affect many of your body's activities, such as balance, movement, talking, breathing, and heart function. Sometimes the cause is a medical condition such as alcoholism, a tumor, or a stroke. Other causes may include toxins, chemicals, and viruses.

https://www.alz.org/alzheimers-dementia/what-is-dementia/types-of-dementia/huntington-s-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.

https://www.sciencedaily.com/releases/2010/02/100223101428.htm

HD sufferers are born with the disease although they don't show symptoms until late in life. In a new study, researchers identified a protective pathway in the brain that may explain why symptoms take so long to appear. The symptoms of Huntington's disease are caused by cell death in specific regions of the brain.

https://medschool.ucsd.edu/som/neurosciences/centers/huntingtons-disease/professionals/Pages/stages-

Stages of Disease Progression (Shoulson, 1981) and Alternate Stages of Huntington's Disease (Pollard & Best, 1996) Stage I: (0 to 8 years from illness onset) Alternate Stage I: Defiance. Alternate Stage II: Perseverance. Stage III: (5 – 16 years from illness onset) Alternate Stage III: Compassion.

https://www.huntingtonsnsw.org.au/information/hd-facts/what-are-chances

The defective gene may be passed from parent to child at conception. If a person does not inherit the defective gene from the affected parent they can't pass it on to their own children. Huntington's Disease does not appear in one generation, skip the next, then reappear in a third or subsequent generation.

https://www.nhs.uk/conditions/huntingtons-disease/symptoms/

The first symptoms of Huntington's disease often include: difficulty concentrating. memory lapses. depression – including low mood, a lack of interest in things, and feelings of hopelessness.

https://www.healthline.com/health-news/link-between-alzheimers-parkinsons-and-huntingtons

In Alzheimer's, it's tau. In Parkinson's, it's alpha-synuclein. In Huntington's, it's huntingtin. The Loyola researchers concluded that these different proteins behave in the same way when they enter brain cells.

https://mosaicscience.com/story/how-close-are-we-cure-huntingtons

There is no cure, and symptoms on average begin in the mid-40s (it then usually takes around 15 years to kill). Indeed, for more than 100 years after the disease was characterised, those at 50:50 risk of inheriting it had no way of ending the uncertainty until the symptoms started.

https://patient.info/brain-nerves/huntingtons-disease-leaflet

Huntington's disease is an inherited (genetic) condition that affects the brain and nervous system. It is a slowly progressive condition that interferes with the movements of your body, can affect your awareness, thinking and judgement and can lead to a change in your behaviour.

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4072816/

HD manifests with chorea, cognitive and psychiatric symptoms. MRI and PET are able to detect changes in the brains of HD gene carriers years ahead of the manifestation of the disease and have also proved to be powerful in assessing disease progression.

https://genetics.thetech.org/ask-a-geneticist/risk-huntingtons-disease-hd

As I mentioned, HD is a dominantly inherited disease. This means that if one of your parents has the disease, you have a 50% chance of getting it from them. And if your parents don't have the disease, you probably won't get it.

https://huntingtonsdiseasenews.com/2019/04/18/pain-prevalent-huntingtons-patients-burden-lower/

Huntington's disease leads to a devastating loss of nerve cells in a brain region called the striatum, one of the areas involved in the perception of pain. Overall, 41. 3% of the patients felt pain. Depending on the study, the prevalence of pain could range from 10% to 75%.

https://hdsa.org/what-is-hd/overview-of-huntingtons-disease/

Huntington's disease (HD) is a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain. It deteriorates a person's physical and mental abilities usually during their prime working years and has no cure. Every person who inherits the expanded HD gene will eventually develop the disease.

https://www.aoporphan.com/global_en/rare-diseases/neurology-metabolic-disorders/huntingtons-disease

Huntington's disease is a genetic disorder affecting the central nervous system and which causes the progressive degeneration of brain cells. This leads to the degeneration of motor skills and cognitive abilities, as well as behavioral difficulties.