A Devastating Inheritance: Huntington's Disease
Huntington’s disease (HD) is a progressive, degenerative brain disorder that causes certain nerve cells in the brain to waste away. Huntington’s is inherited, and if one of your parents has Huntington’s disease, you would have a 50% chance of getting the gene for the disease. Everyone who carries the gene will develop the disease. Since the HD gene was isolated in 1993, one can be tested to see if they are a carrier, however because there is no cure for HD, some people choose not to be tested.
About 1 in 10,000 Canadians has HD.
There are two types of HD: i) adult-onset, the most common form, with symptom onset in the mid 30s and 40s; and ii) early onset, which accounts for about 16% of all HD cases, with symptoms developing in childhood or adolescence.
Huntington’s disease is associated with three types of symptoms:
· Movement symptoms, referred to as chorea, which consist of jerking, involuntary movements (‘tics’) of the limbs, trunk or face;
· Cognitive symptoms including a gradual impairment of concentration, memory, judgment, reasoning, decision-making and learning. This cognitive decline starts in a very subtle fashion, but eventually results in dementia; and
· Psychiatric symptoms may include depression, and psychotic behaviours such as delusions, hallucinations, paranoia and inappropriate outbursts.
HD usually runs its course in about 10 to 30 years, with a strong correlation between an earlier onset and a more rapid progression of the disease.
Jennifer Hartman, Guest Blogger
