Scottish
Huntingtons Association Illustrated Pages
Information Resources
All About Huntingtons Disease
What is Huntington's Disease?
Around 1850, US
family doctor George Huntington went out riding, joining his father (also a
doctor) on his
rounds. They came across 'two women, mother and daughter,
both tall, thin, almost cadaverous, both bowing,
twisting, grimacing...'. they said. Huntington was amazed and almost fearful, and
the encounter triggered his
lifelong interest in the disease now named after him. First
called Huntington's chorea (a term that describes the
strange dancing movements that accompany the illness), it's
now known as Huntington's Disease.
Huntingtons Disease is a neurological disorder, a disease of
the brain. Cells in the central part of the brainknown as the basal ganglia
die. Since so much of the
brain’s activity passes through this area, the death of these cells affects
virtually everything about a person. Including movements, moods, and thinking
processes.
But because the damage caused by HD is only inside the brain, the person with
HD may look relatively able bodied until the later stages of the disease.
Caregivers
often mistakenly assume that changes in the person are due to lack of
motivation, laziness or worse, and not to the disease itself.
HD is a genetic disease that you get by inheriting a
defective gene for HD from one parent. Every child of a parent with the
defective gene for HD has a 50/50 chance
of inheriting the gene, no matter how many children that parent has. If you do
not inherit the HD gene, you cannot get the disease and you cannot pass it on
to your
children or their descendants. If you do get the HD gene, you will eventually
get the disease. HD always manifests itself if you live long enough. It never
skips a
generation. As a genetic disease, HD is referred to as “a disease of families.”
In many families touched by HD, more than one family member may have HD at the
same
time. Many relatives are at risk of later developing the disease.
Although people can first exhibit signs of HD at any age,
most people first show them when they are in their 30s and 40s. Thus, HD is
described as an
“adult-onset disease”. As a progressive disease, HD begins very subtly and only
the person with HD, close friends or relatives, and the trained eye of a
physician
can detect its earliest signs. It progresses in stages, slowly advancing for
many years. It usually takes at least 15 years for the disease to run its
course, sometimes
longer. Younger people with MND may find their illness progresses at a faster
rate.
Scottish Huntingtons
Association,
Thistle House, 61
Main Road,
Elderslie, PA5 9BA,
Tel; 01505 322245,
email: sha-admin@hdscotland.org.uk
The Scottish Huntington's Association is
a Registered Charity: No 121496.