What Is Huntington's Disease?
Huntington’s Disease (HD) is a fatal neurodegenerative disease that causes the progressive deterioration of nerve cells in the brain. This genetic disorder weakens a person’s physical and mental abilities during the prime of his or her life. Over a 10- to 30-year period, this inherited disease will cause a person to lose his/her ability to talk, walk, and eat.
HD, also known as Huntington’s chorea, is a mixture of motor, cognitive, and behavioral features that lead to the increasing need for assistance from skilled health care professionals.
Symptoms of Huntington’s Disease, which usually appear between the ages of 30 and 50, include:
- Chorea (involuntary movements) and unsteady gait
- Personality changes, mood swings, depression
- Impaired judgement, forgetfulness
- Slurred speech
- Difficulty swallowing
- Significant weight loss
Symptoms of the disease eventually cause complications such as pneumonia, heart failure and poor nutrition. There is no cure for Huntington’s Disease and no treatment that can slow its progression, although some medications may alleviate certain symptoms.
The Stages of Huntington’s Disease
Each person diagnosed with Huntington’s Disease can present unique symptoms; however, the common symptoms experienced can be segmented into three progressive stages.
Early Stage of HD
- Subtle changes in coordination
- Possible chorea
- Trouble problem solving
- Depressed/irritable mood
Middle Stage of HD
- Increased involuntary movements
- Difficulty swallowing
- Slurred speech
Late Stage of HD
- Loss of ability to walk and speak
- Dependent on others for care
- Choking becomes a concern
- Retains awareness and can comprehend language
It is a common misconception that Huntington’s Disease is an autoimmune disease, but HD is a hereditary disease. Genetic disorders such as Huntington’s Disease can only be inherited from a parent. Each child of a parent with Huntington’s Disease has a 50 percent chance of inheriting the gene that causes HD.**
Juvenile Huntington’s Disease
While a majority of individuals show symptoms of Huntington’s Disease during the prime of their lives, a fraction are diagnosed with Juvenile Huntington’s Disease (JHD), which causes Huntington’s Disease to develop before the age of 20.
Juvenile Huntington’s Disease is rare and only prevalent in 10% of HD diagnoses, affecting children and adolescents. Typically, Juvenile Huntington’s Disease is present when the inherited gene comes from the father. Unlike adults, the earlier the onset, the faster the HD progression.
The symptoms of Juvenile Huntington’s Disease are slightly different from those presented during adult onset HD.**
Juvenile Huntington’s Disease Symptoms Include:
- Stiffness of legs
- Clumsiness of arms and legs
- Decline in cognitive function
- Changes in behavior
- Seizures
- Changes in oral motor function
- Chorea (in adolescents)
Woody Guthrie
Woody Guthrie, an American folk musician most famous for his song, “This Land is Your Land,” died in the late 1960s after being diagnosed with Huntington’s Disease, which he inherited from his mother.
To date, Woody Guthrie is the only known celebrity to have been diagnosed with Huntington’s Disease.
**Facts and information sourced from http://hdsa.org/.
