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 What is Huntington's Disease?

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  Huntington's Disease is a neurodegenerative disorder. Its symptoms are
brought on by a loss of nerve cells in the part of the brain known as the
corpus triatum. Initial onset of the disease usually occurs around midlife,
between the ages of 35 to 50. From then on, it gradually progresses. HD has
been recognized by the Ministry of Health and Welfare in Japan (See also
'Information on Health and Human Services in Japan'), and the number of
registered patients at the end of 1998 was 526, making HD a relatively rare
illness among Japanese: 0.5 to 0.6 cases per one million of the population.
The number of sufferers in other countries, especially among white people,
is greater, and a figure of 4 to 10 cases per one hundred thousand has been
reported.
              
  The name Huntington's Disease, or Huntington's Chorea, came into usage
after Dr. George Huntington, who lived in Long Island, USA, first described
the illness as 'hereditary chorea' in his report "On Chorea" published in 1872.
The word 'chorea' is derived from the ancient Greek word for dance,'chorein'
which indicates involuntary movement, one of the symptoms of this disease.
Symptoms can be divided in three parts: 1) cognitive impairment (difficulties
in the thinking process, decision making and remembering); 2) loss of muscle
control (involuntary movements, difficulty in swallowing); and 3) difficulty
in emotional control (depression, emotional outbursts, irritability, etc.)
              
  The extent and appearance of the development of the disease differ vastly
from person to person. In the advanced stage, difficulty in swallowing food
becomes a big problem, as well as difficulties in communication and walking.
In spite of severe symptoms, HD patient is still able to recognize his/her
surrounding environment, family members and others, and has likes and
dislikes. On average, the disease lasts for about 15 to 20 years from
the initial onset.
             
  The other characteristic of HD is that it is a hereditary disease that is
passed on from one generation to the next. Because the gene responsible
is passed on as a so-called 'dominant inheritance', the possibility of
a child inheriting the gene from an affected parent is 50 percent. How to
explain about HD to a child who is 'at risk' status presents a difficult
issue, but as a preferred approach, it is recommended that the information
be gradually shared by all the family members, taking it one step at a time.
               
         The last 20 years have seen considerable advances in the body of knowledge
concerning the cause of HD and how to treat it. In 1993, as a result of 10
years of international effort, researchers were able to discover the gene
responsible for HD. This discovery served to promote intense debate on the
problem, leading to the establishment of guidelines for dealing with the
ethical, legal and social issues. These guidelines now
serve as a model and frame of reference for genetic diagnosis in all other
hereditary diseases as well. (See also 'Guidelines provided by the WFN/IHA
on genetic counseling and predictive testing')
                
  In most cases, during the early years after initial onset, the patient
can maintain an independent lifestyle, and counseling from specialists will
provide the necessary aid and treatment. Professionals will do all in their
power to help the patient and their family and to prolong the sufferer's
quality of life as an independent person. It is reassuring to know that
there is a team of medical and social experts near at hand: home helpers,
nurses, health advisers, social workers, kinesitherapists, speech
therapists, physiotherapists, nutritionists, psychiatrists etc. All of them
are there to help. Let us all work together to help and support each other.

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