Huntington's Disease Association of Ireland

The ten most commonly asked questions about HD

01 What is Huntington’s Disease?
Huntington’s Disease (HD) – also called Huntington’s Chorea – is an inherited disorder of the central nervous system.  It causes progressive deterioration with varying symptoms which may include involuntary movements, speech impairment, and intellectual and emotional changes. Symptoms usually appear between the ages of 30 and 45, although they may appear earlier or later.
02 How does the disease get its name?
The disease is named after Dr. George Huntington, an American physician, who was the first to publish a precise description of the symptoms and course of the disease in an article written in 1872.
03 How is the disease inherited?
This is a genetic disease.  Each child of an affected parent has a one in two chance or 50% likelihood of inheriting the gene.  People with the abnormal gene will always develop the disease, unless they die of other causes prior to developing signs and symptoms..  People who do not inherit the HD gene will not develop the disease, neither will their children, or the children’s children.  The disease does not “skip a generation”.
04 How is it caused?
It is caused by a dominant defective gene which causes certain brain cells to deteriorate.
05 How many people in Ireland are affected?
It is estimated that *400 people have the illness in the Republic of Ireland with a further 2000 individuals at risk of developing the condition.  On average for every individual with the disease there are 22 close family members indirectly affected.  Therefore over 9000 family members may require support and information.
06 How is Huntington’s Disease diagnosed?
The gene for HD was found in 1993.  Details on diagnostic testing are available from the National Genetics Centre in Dublin.  Please phone (01) 4558111.
07 Is there any test to predict if a person will get the disease?
Details on the predictive test are available from the National Genetics Centre in Dublin.  Please phone (01) 4558111.
08 Can Huntington’s Disease strike without a family history of it?
Spontaneous mutation of the genes can occur.  Contact HDAI office for information.
09 What treatments are there for the disease?
There are no treatments that will halt or reverse the disease process at present.  It is possible to treat some of the effects, such as depression, and gross involuntary movements, with various drugs.  Improvements in general health, such as adequate nutrition, will bring about improvements in the status of the person, and in their enjoyment of life.

Immense strides are being made in medical research.  Huge sums of money are being put into research.  Phone HDAI for details.

10 Is there a special diet required for sufferers?
There can be rapid weight loss, and it is estimated that HD patients need something in the order of 5000 kilo calories a day to sustain them – twice that of the average adult intake.  It has been shown that many patients who manage to gain weight also find that their clinical state improves.

*Based on a survey carried out in Northern Ireland in 1992.

   

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