What Exactly is Huntington Disease?

I get asked this quite often. So, I’m providing information from the HDSA link below.Please always feel free to ask. I get more offended when people don’t.

HD affects the whole brain, but certain areas are more vulnerable than others. Pictured above are the basal ganglia — a group of nerves cell clusters, called nuclei. These nuclei play a key role in movement and behavior control and are the parts of the brain most prominently affected in early HD

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. HD is known as the quintessential family disease because every child of a parent with HD has a 50/50 chance of inheriting the faulty gene. Today, there are approximately 30,000 symptomatic Americans and more than 200,000 at-risk of inheriting the disease.The symptoms of HD are described as having ALS, Parkinson’s and Alzheimer’s — simultaneously.Symptoms usually appear between the ages of 30 to 50, and worsen over a 10 to 25-year period. Ultimately, the weakened individual succumbs to pneumonia, heart failure or other complications. Everyone has the gene that causes HD, but only those that inherit the expansion of the gene will develop HD and perhaps pass it on to each of their children. Every person who inherits the expanded HD gene will eventually develop the disease. Over time, HD affects the individual’s ability to reason, walk and speak.Symptoms Include:

• Personality changes, mood swings & depression
• Forgetfulness & impaired judgment
• Unsteady gait & involuntary movements (chorea)
• Slurred speech, difficulty in swallowing & significant weight loss

The Huntingtin Gene

Huntington’s Disease manifests as a triad of motor, cognitive, and psychiatric symptoms which begin insidiously and progress over many years, until the death of the individual. The average length of survival after clinical diagnosis is typically 10–20 years, but some people have lived thirty or forty years. Late stage HD may last up to a decade or more.
https://hdsa.org/what-is-hd/overview-of-huntingtons-disease/