The progression of Huntington's Disease (HD) and accompanying cognitive loss takes between 10 to 20 years to complete, with complications resulting from the loss resulting in death. According to the Huntington Disease Society of America, typical onset occurs between the ages of 20 and 50 years old with extremely rare cases of juvenile HD beginning from the age of 2. There are no existing cures for HD, yet quality of life may be prolonged via exercise, proper nutrition and superior care giving supports.
Huntington's Chorea: Motor Skill Degeneration
Researchers for the HOPE program at Stanford University suggest that Huntington's chorea is the definitive term used to describe the rapid motor skill degeneration taking place in the individual with mild to moderate HD. Daily activities are impaired by the inability to control movements and can result in serious injury.
Behavioral and Personality Changes
Behavioral and personality changes may become problematic as the individual experiences both cognitive decline and motor skill degeneration paired with an increasing knowledge of dependency on others. The Department of Neurology at the Kansas University Medical Center lists numerous behavioral and personality challenges to include: anxiety, depression, mania, obsessive-compulsive behaviors, agitation, hostile outbursts, hallucinations and delusional thinking, sleep disturbances and withdrawal from social supports. Further alterations in personality (which often prove devastating to familial care providers) can include increasing irritability, the tendency to find fault in others, constant complaining, suspicion, impulsive or crude attitudes and behaviors towards loved ones, loss of interest in previously enjoyable activities and increasing difficulty making or maintaining decisions, plans or solving problems on their own.
Cognitive Decline
Cognitive (thought-processes) decline as the disease begins to deteriorate several functions. The ability to make decisions, plan for the future and pay attention to tasks at hand pair with the increasing inability to concentrate. The Huntington Disease Society of America posits that loss of cognitive function can begin slowly with inability to recall events or names of items. Eventually, other symptoms associated with completing tasks become problematic including coping with stressors, orientation and navigation, language and speech impediments, impaired judgments, inappropriate behaviors, depression and increasing psychosis. Cognitive decline adds to aggravation due to the awareness of fidgeting and odd movements of the limbs and trunk, poor coordination of movements, added clumsiness and forgetfulness.
Disease Progression
As HD progresses, so too does the likelihood of difficulty experienced with daily activity (i.e. dressing, eating, basic hygiene). The ability to eat normally and swallow without choking produces a tremendous deal of stress and danger to living alone or without extra care provisions. According to the National Institute of Neurological Disorders and Stroke, the ability to operate machinery or vehicles becomes increasingly more impaired as the disease progresses rendering such tasks impossible as cognitive decline and the degeneration processes continue. The individual will become dependent on others for care. Most individuals with HD experience falls due to accidents with broken bones, traumatic brain injuries, infection and heart failure, which can ultimately result in death.
References
- Huntington's Disease Society of America: What is Huntington's Diseases [HD]
- National Institute of Neurological Disorders and Stroke: Huntington's Disease, What is the Prognosis?
- Stanford University: HOPES, Huntington's Outreach Project for Education at Stanford
- Department of Neurology, Kansas University Medical Center: Behavioral Problems in Huntington's Disease
