Huntington’s Disease

George Huntington 1850 – 1916

Huntington’s disease is an inherited progressive dementia that affects the individual’s cognition, behavior and movement.  Huntington’s disease does not skip generations.  Each child of a parent with Huntington’s has a 50/50 chance of inheriting the defective gene.  If a child does not inherit the gene, he or she cannot pass it on. If the child does inherit the gene, he or she can pass it on and will develop the disease.

The cognitive and behavioral symptoms of dementia due to Huntington’s include memory problems, impaired judgment, problems with short-term memory, organizing, coping, concentrating, mood swings, depression and speech problems (especially slurred speech). Delusions and hallucinations may occur. In addition, the individual may experience fidgety behavior, lack of coordination, difficulty ambulating, and uncontrollable jerking movements of the face and body.  Symptoms that may also occur are irritability, anxiety, aggressive outbursts and social withdrawal.

The average lifespan after onset is 10-25 years and the younger the age of onset, the more rapid the progression of the disease.  Symptoms generally appear between the ages of 30 and 50, but can strike children and young adults.

The gene discovery has made possible a predictive test for Huntington’s from a blood sample allowing those at risk to find out whether or not they will develop the disease.  Pre-and post-test counseling is critical.


 

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