What is Huntington disease?
Huntington's (or Huntington) disease (HD) is a genetic condition that causes deterioration or death of cells, called neurons, in the brain. These are located in several areas of the brain including those that control movement, thinking, and behavior. The first symptoms of HD may be personality and behavioral changes, psychiatric problems, cognitive decline (affecting mostly processing, judgment, and logic). This may lead to a long delay in getting diagnosed.
HD also involves choreatic movements, which are rapid uncontrolled muscle movements (tics or jerks). These movements can occur all over the body and are not in someone's control.
HD has a range of other symptoms as well, such as dystonia or unusual positioning of the body, significant weight loss, difficulty swallowing, and a loss of coordination. HD is progressive, which means that the disease gets worse with time, and eventually leads to death. Because this disease is progressive, it is referred to as a neurodegenerative disorder.
HD is a genetic disorder, which means it is inherited or passed down in families. It is inherited in an autosomal dominant manner. This means that individuals with HD have a 50% chance of passing on HD to each child they have. Neither the gender of the child nor the gene status of other children has any bearing on whether a specific child will inherit HD.
The onset of symptoms is often in adulthood, between the ages of 30 and 50. The symptoms become progressively worse over the next 10-25 years before the individual eventually passes away from complications of the disease like pneumonia or heart failure. More than 30,000 Americans are currently affected by this condition.
SOURCE: Emory University - Department of Human Genetics in collaboration with ThinkGenetic • https://www.thinkgenetic.com/diseases/huntington-disease/overview/1355 • DATE UPDATED: 2017-10-31
NINDS Huntington's Disease Information Page. National Institute of Health. Updated January 2016. Accessed from http://www.ninds.nih.gov/disorders/huntington/huntington.htm
"Huntington's Disease" National Organization for Rare Disorders. Updated 2007. Accessed from http://rarediseases.org/rare-diseases/huntingtons-disease/
"What is Huntington's Disease?" Huntington's Disease Society of America. Accessed from http://hdsa.org/what-is-hd/