Huntingdon's Disease - What is It?

Huntingdon's disease affects roughly 7 in ever 100,000The gene for Huntingdon's disease is dominant, which
people. It is an inherited disease which starts to exhibitmeans you only need one parent with the disease to
symptoms between the age of 25 and 55. From theinherit it yourself. There is a 50 percent chance of a
first symptoms, it takes between 10 and 25 years toparent passing on Huntingdon's disease to their child.
completely degenerate. At present, it has no cure.This makes the late onset of the disease doubly tragic.
It is a cruel disease which gradually robs the person ofOften, the person will have started a family before
their intellectual abilities. This includes their long and shortthey experience the early symptoms. They then have
term memory, their thoughts and their ability tothe combined fear of waiting for confirmation that they
communicate. It also affects the body and ishave the disease, and the knowledge that their children
characterized by violent jerks and twitches.may be at great risk of Huntingdon's disease
The gene which causes Huntindon's disease wasthemselves.
discovered in 1993. In a specific part of the geneticBy far the greatest incidence of Huntingdon's disease
code it repeats the information needlessly. The geneis in Barranquitas, a small village in Venezuela. Nearly
then passes this faulty information onto the huntingtin,50 percent of the population are carriers of the gene.
the protein produced by the gene. This repeatedThat amounts to around 25,000 people.
information makes the protein too long to functionThere is a genetic test available which can confirm
properly. This extra long protein lacks the protection awhether a person has the disease. There is specialist
normal, shorter protein gives to the brain, allowingcounselling on offer for people who have a family
deterioration in the frontal lobes and basal ganglia.history of Huntingdon's disease, who may live in
These are the regions of the brain responsible forconstant anxiety that they or their children have
higher functioning and movement.inherited the faulty gene.