What is Huntington’s Disease?

Huntington’s disease is a genetic neurodegenerative condition that results in brain cell death, caused by a faulty version of the ‘huntingtin’ gene that we all have.

The regions of the brain affected are the frontal lobe and basal ganglia. These regions are responsible for motor movement and control and coordination, cognition, personality and emotions.

Deterioration in these regions of the brain leads to significant impairments in one’s ability to think feel and move. As a result people will experience the symptoms of Huntington’s disease

Whilst people are born with a faulty version of the gene, they are not born with Huntington’s disease, instead onset predominantly occurs in young-middle aged adulthood. There is a juvenile form of the disease that presents before the age of 20 years and although rare, it is more rapidly progressive.

Currently there is no cure for Huntington’s disease or effective treatment.

If you would like to learn more about Huntington’s disease symptoms, Juvenile HD or the genetics of HD, please click the links below to find out more.

Symptoms of HD

There is no specific order in which symptoms may present and they can also vary in their severity. Symptoms may appear as Physical, Cognitive or Psychological changes.

Juvenile HD

Juvenile Huntington’s disease (JHD) occurs when a child or adolescent under the age of 20 years develops the symptoms of Huntington’s disease.

Genetics of HD

Huntington’s disease is caused by a fault on one of these genes- the huntingtin gene which is on chromosome 4, a chromosome we all posses.

Testing for HD

Making a decision to undergo a predictive testing is often the most challenging decision a person at risk will need to make.

What Areas of the Brain Are Affected?

Frontal Lobe

The Frontal Lobe

The Frontal lobe is located at the front of the brain and important for our ability to communicate, primary motor function (i.e. our ability to consciously move our muscles), emotional expression, speech, problem solving, memory, language, judgment and social interactions (making social decisions, knowing when to do things, suppressing impulses)

The Basal Ganglia

The basal ganglia are located deep in the brain and control voluntary movements, affects spatial awareness, balance and coordination, and our ability to move on from a thought or task as well as prioritising tasks. Memory formation and storage as well as complex emotional responses are also controlled from this region. The basal ganglia have strong connections to the frontal lobes and damage to the basal ganglia can also lead to impaired functioning of the frontal lobes.