Huntington's disease (chorea)

What is Huntington's disease?

Huntington's disease causes certain nerve cells in the brain to stop working properly. It leads to mental deterioration and loss of control over major muscle movements. Typically, the symptoms of the illness begin between ages 35 and 50, although they can start as early as childhood or later in life.

Regardless of at what age it starts, Huntington's disease worsens over time. It is an inherited (genetic) disease. Each child of a parent with Huntington's disease has a 50% chance of inheriting the illness.

Huntington's disease is relatively uncommon. It affects people from all ethnic groups. The disease affects males and females equally.

Symptoms of Huntington's disease

Huntington's disease can affect:

• Intellectual ability
  • Memory loss
  • Inattention
  • Dementia
• Uncontrollable movements
  • Twitching
  • Clumsiness
  • Dance-like movements
  • Slurred speech
  • Difficulty walking
  • Difficulty swallowing
• Uncontrollable emotions
  • Personality changes
  • Depression
  • Irritability
  • Anxiety or lack of emotions (apathy).

Symptoms vary from person to person. They also can change over time. They usually start as subtle differences and progress to profound disability.

Huntington's disease is rare in children. In children, behavioral problems and mental deterioration are prominent. Rigidity and seizures are common. Huntington's disease progresses more rapidly in children than in adults.

Diagnosing Huntington's disease

The symptoms of Huntington's disease begin subtly. As a result, diagnosis may not be made until the disease has started to worsen.

Someone with a family history of Huntington's disease who has symptoms often is diagnosed based on a physical and neurological exam.

A blood test can detect the gene that causes Huntington's disease. Everyone who carries the Huntington's gene eventually will develop the disease. The test cannot predict when symptoms will begin, or how severe they will be.

Genetic testing carries enormous emotional and practical consequences. You should discuss these issues with your doctor. Some people choose to be tested so they can make informed decisions about the future. Other people choose not to be tested, knowing that they cannot do anything to affect whether they have inherited the defective gene.

Many experts recommend that children under 18 should not get a genetic test. At 18, they can decide for themselves if they want to get tested.

Some hospitals offer prenatal testing. If you have a family history of Huntington's disease, you can determine your risk of passing the gene to your children.

Expected duration of Huntington's disease

The gene that causes Huntington's disease is present from birth. Symptoms typically begin in midlife. They last until the end of life.

Preventing Huntington's disease

There is no way to prevent this disease in people who have inherited the genetic abnormality.

If you have a family history of Huntington's disease, you may choose to have a genetic test. This can help you to determine your chances of passing the gene on to your children.

Treating Huntington's disease

There is no treatment to cure Huntington's disease or slow its progression. Several treatments are being tested to see if they can at least slow the progression of the disease.

Therapies can lessen the severity of symptoms. These include speech therapy and physical therapy.

Medications can help to control mood and involuntary movements.

When to call a professional

Discuss a family history of Huntington's disease with your doctor. Consider genetic testing to determine whether you carry the Huntington's gene. You also may want to understand the risks involved before having children.

Call your doctor if you experience any problems with emotional control, intellectual ability or movement. Keep in mind that these symptoms are not specific to Huntington's disease.

Prognosis

There is no treatment to halt the progression of Huntington's disease.

The course of the disease varies from person to person. Some people have mild symptoms that progress slowly; others have severe symptoms at a young age.

People who exercise and remain active tend to have milder symptoms. Their disease may also progress more slowly than those who do not remain active.

However, everyone with Huntington's disease ultimately becomes severely debilitated. They have difficulty eating and swallowing. They become susceptible to infection. These complications usually cause death.

For adults, the time from the first symptoms until death is usually 10 to 20 years. In juvenile Huntington's disease, the disease progresses more rapidly. Death usually occurs within eight to 10 years of disease onset.

Additional info

Huntington's Disease Society of America (HDSA)
https://www.hdsa.org/