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What is Huntington disease?

Huntington disease is a rare, progressive brain disorder. It gradually kills nerve cells in the brain. This slowly deteriorates a person’s physical and mental abilities. The disease is genetic, which means it is inherited from your parents. There is no cure, and it is fatal.

People are born with the defective gene that causes the disease. But symptoms usually don’t appear until middle age. Most cases of Huntington disease are diagnosed between the ages of 35 and 55. There is also an early-onset form of the disease called juvenile Huntington disease. It affects people under age 20.

Symptoms of Huntington disease

There are many symptoms of Huntington disease. Early signs include:

• Irritability
• Depression
• Poor coordination
• Small involuntary body movements
• Trouble making decisions
• Trouble learning new information

As the disease progresses and gets worse, you may experience:

• Jerking or twitching movements you can’t control (called chorea)
• Trouble walking, speaking, and swallowing
• Changes in personality
• Decline in thinking and reasoning abilities
• Significant weight loss

In young people, symptoms include being unsteady on your feet, rigid muscles, slurred speech, and seizures. As thinking and reasoning abilities get worse, school performance also gets worse. Juvenile Huntington disease tends to progress faster than the adult-onset form of the disease.

What causes Huntington disease?

A single gene is to blame for Huntington disease. It is passed on from parent to child at the time the child is conceived. If one parent carries the defective gene, the child has a 50% chance of inheriting the gene and developing the disease. If the child does not inherit the defective gene, they will not develop the disease.

How is Huntington disease diagnosed?

Healthcare providers use a variety of tools to diagnose Huntington disease. They will start with a physical exam to observe your symptoms. They will ask questions about symptoms and your family medical history. A brain imaging test, such as CT or MRI, can show abnormalities in the brain. A lab can detect the Huntington disease gene with a simple blood test.

Can Huntington disease be prevented or avoided?

Huntington disease is a genetic disorder. It cannot be prevented or avoided.

Huntington disease treatment

There is no cure for Huntington disease. Nothing can stop or reverse the disease’s course. The goal of treatment is to help relieve symptoms. This will help you function for as long as possible.

There is no single treatment for the disorder. Medicines can be used to treat some symptoms. For example, some “antipsychotic” medicines can help with uncontrollable body movements.

Medicines used for treating uncontrolled body movement generally have side effects. They may make you nauseated, sleepy, or restless. Movement disorder medicines also may worsen other symptoms associated with Huntington disease, such as rigid muscles.

Adapting your everyday life to the symptoms of the disorder is necessary. Different therapies can help. You may require:

• Physical therapy, such as walking.
• Occupational therapy (activities using your hands).
• Speech therapies (to help with slurred speech or trouble swallowing).

You also may require assistance at home for activities of daily living. These could include cooking, cleaning, and everyday chores. As the disorder progresses, you may need help in dressing, eating, and toileting. There may come a time that you need 24-hour care.

Living with Huntington disease

Huntington disease is progressive. That means it gets worse over time. Living with the disorder means preparing for the symptoms to worsen. Eventually you will need help with everyday activities. These include getting around your home, hygiene, eating, and decision-making. A trusted advisor can help with important decisions and in monitoring changes in your behavior.

People with Huntington disease usually die within 15 to 20 years of their diagnosis. The most common causes of death are infections (such as pneumonia) and injuries related to falls

FOR MORE INFORMATION

Huntington Society of Canada
https://www.huntingtonsociety.ca/