Huntington Disease
Huntington Disease (HD) is an inherited brain disorder that causes cells in specific parts of the brain to die. Huntington disease is a genetic disorder. About one in every 10,000 Canadians has HD, but one in every 1,000 are touched by HD whether at risk, as a caregiver, family member or friend. The HD gene is dominant, which means that each child born to a parent with Huntington disease has a 50% chance of sharing the same fate.
The symptoms of HD include:
• Emotional turmoil (depression, apathy, obsessive behaviour)
• Mental loss (inability to focus, think and recall, make decisions)
• Physical deterioration (weight loss, involuntary movements, diminished coordination, inability to walk, talk, swallow)
From the Huntington Society of Canada, 2010, "What is Huntington disease?"
Juvenile HD is characterized by a movement disorder which differs from that of affected adults.
The earlier the disease onset, the more likely it is that the child will be very rigid, and the less likely it is that he or she will have the chorea (involuntary movements) see in most adults. In 25-30% of cases, there is a tendency to epileptic seizures – something almost never seen in adults with HD. Juvenile HD also involves changes in behaviour and mental function.
Like its adult counterpart, juvenile HD remains incurable, and there are no treatments which can stop or slow the course of the disease. Dramatic advances in research have given rise to tremendous optimism that new forms of therapy will soon be in sight.
From "Juvenile Huntington Disease: A Resource for Families, Health Professionals and Caregivers, 2008"
Information for caregivers :