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By: Dennise Evans
Huntington disease and or Huntington Chorea
Huntington's disease is caused by a genetic defect on chromosome
The defect causes a part of DNA, called a CAG repeat, to occur many more times than it is supposed to
. Normally, this section of DNA is repeated 10 to 28 times. But in persons with Huntington's disease, it is repeated 36 to 120 times.
As the gene is passed down through families, the number of repeats tends to get larger.
The larger the number of repeats, the greater your chance of developing symptoms at an earlier age.
Therefore, as the disease is passed along in families, symptoms develop at younger and younger ages.
There are two forms of Huntington's disease. The most common is adult-onset Huntington's
disease. Persons with this form usually develop symptoms in their mid-30s and 40s.
An early-onset form of Huntington's disease accounts for a small number of cases and begins in childhood or adolescence
early-onset form of Huntington's disease adult-onset Huntington's disease
effect the motor neurons in the brain, jerky, parkinsonism like movement, which in
late stages becomes detrimental to physical health.
sufferers are killed by secondary infections
pneumonia or get heart attacks as a result of hypertension
Huntington's also causes changes in personality, including depression and dementia.
Behavior changes may occur before movement problems, and can include:
Behavioral disturbances
HallucinationsIrritabilityMoodinessRestlessness or
fidgetingParanoiaPsychosis
Abnormal and unusual movements includeFacial movements, including grimacesHead turning to shift eye positionQuick, sudden, sometimes wild jerking movements of the arms, legs, face, and other body partsSlow, uncontrolled movementsUnsteady gaitDementia that slowly gets worse, including:Disorientation or confusionLoss of judgmentLoss of memoryPersonality changesSpeech changes
Huntington's disease causes disability that gets worse over time. Persons with this disease usually die within 15 to 20 years. The cause of death is often infection, although suicide is also common.
It is important to realize that the disease affects everyone differently. The number of CAG repeats may determine the severity of symptoms.
Persons with few repeats may have mild abnormal movements later in life and slow disease progression, while those with a large number of repeats may be severely affected at a young age.
There is no cure for Huntington's disease, and there is no known way to stop the disease from getting worse.
Dopamine blockers may help reduce abnormal behaviors and movements.
There has been some evidence to suggest that co-enzyme Q10 may also help slow down the course of the disease, but it is not conclusive
was a storyteller through songHis work ranged from social commentaries
about the working conditions of migrant workers and the urban poor to ballads and children’s songs.
“very man, woman and child has within them
the power to make a
difference.”
In 1967, Woody Guthrie, lost his battle with HDHe was just 55 years oldAt the time, little was known about the disease. Marjorie Guthrie convinced, President Jimmy
Carter to form a Commission to study neurological diseases, and HD.
Since that time, research has progressed rapidly In 2004, HD formed a pipeline for drug discovery
that begins in the laboratory with basic science.
Woody Guthrie
The Society is a National, voluntary health organization
dedicated to improving the lives of people with Huntington's disease and their families.
Promote support research, and medical efforts to eradicate Huntington's disease.
Educate the public and health professionals about Huntington's disease.
"Woody Guthrie." Woody Guthrie. Woody Guthrie Publications, n.d. Web. <http://www.woodyguthrie.org/thislandconcert/hdsa.htm>.
"Â ." Huntington's Disease Society of America. N.p., n.d. Web. 05 Mar. 2013. <http://www.hdsa.org/>.