About Huntington's Disease

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Huntington's Disease Information

The information in this website was acquired from sources in the United Kingdom and the United States and is presented in accordance with the available data. There are currently relatively few South African statistics accessible, however links to that material are provided on our research and resource page. Note: This website's material and links are offered for informative purposes only and should be discussed with a competent qualified and registered medical professional practitioner.

What is Huntington's Disease?

Huntington's disease is a rare, inherited disease that causes the progressive breakdown (degeneration) of nerve cells in the brain. Huntington's disease has a wide impact on a person's functional abilities and usually results in movement, thinking (cognitive) and psychiatric disorders.

Who does it affect?

Huntington's disease (HD) is a rare inherited neurological ailment that affects approximately 8,000 people in the UK, with an additional 32,000 people at risk of getting it. There is a substantial frequency of HD in the White and mixed-race populations of South Africa. Most of the families with HD have Dutch or British ancestry, which suggests that they inherited the European HD allele. A 1987 study revealed the HD prevalence among South African Blacks to be very low – around 0.6 cases per million people. Huntington’s disease is believed to have first arrived in South Africa over 300 years ago when Dutch colonists settled into the Western Cape in the 1600’s. Dr. Michael Hayden, a South African researcher, traced back the origins of the disease in this country.

There is no cure for this complex disease, and only a few therapy choices are available to assist to control symptoms. Huntington's disease attacks nerve cells in the brain, resulting in a slew of symptoms that worsen over time.

What are the symptoms?

Symptoms can emerge at any age, but they are most common between the ages of 30 and 50. Approximately 5-10% of patients with Huntington's Disease develop symptoms before the age of 20 (known as Juvenile Huntington's disease), while 10% develop symptoms beyond the age of 60. The average survival time after the beginning of symptoms is 15 to 20 years. Every child born naturally to parents carrying the defective Huntington gene has a 50% risk of acquiring it. A genetic test can be used to determine whether or not a person possesses the defective gene.

What are the stages of this disease?

Huntington's disease is a hereditary neurological condition that runs in families. Those who are naturally born to a parent who has Huntington's disease have a 50:50 chance of receiving the gene. Most persons with Huntington's disease exhibit symptoms in their forties and fifties, however modest alterations can occur much earlier. About 5-10% of patients get symptoms before the age of 20 (Juvenile Huntington's disease), and 10% develop symptoms after the age of 60.

1. Motor (movement): This disorder is distinguished by involuntary movements (chorea) and impairment of voluntary motions.

2. Cognitive (learning and thinking): disorders begin with a lack of mental quickness and flexibility. Executive functions such as organization, regulation, and perception are the most commonly impaired.

3. Psychiatric (mental illness/health): Depression and anxiety are the most frequent psychiatric disorders, as are changes in personality and mood, such as irritability, apathy, or disinhibition.