Huntington’s Disease (HD)

Huntington’s Disease Latest News

Huntington’s disease (HD) has long been impossible to cure, but new research is finally giving fresh hope.

About Huntington’s Disease

• It is a rare hereditary disorder in which brain cells, or neurons, in certain areas of your brain start to break down. 
  • The destruction of nerve cells happens in the basal ganglia, or the region of your brain that regulates your body’s movements.
  • It also affects the brain cortex (surface of your brain) that regulates your thinking, decision-making, and memory.
• As the neurons degenerate, the disease can lead to emotional disturbances, loss of intellectual abilities, and uncontrolled movements.
• What causes HD?
  • A genetic mutation of the HTT gene causes HD. If one of your parents has HD, you have a 50% chance of also developing it.
  • The HTT gene makes a protein called huntingtin. This protein helps your nerve cells (neurons) function.
  • If you have HD, your DNA doesn’t have all the information needed to make the huntingtin protein.
  • As a result, these proteins grow in an abnormal shape and destroy (instead of help) your neurons.
  • Your neurons die because of this genetic mutation.
• How common is HD?
  • HD affects an estimated 3 to 7 out of every 100,000 people, most often people of European ancestry (biological family comes from European descent).
  • There are two main types of HD:
  • Adult-onset: This is the most common type. Symptoms usually start after age 30.
  • Early-onset (juvenile Huntington’s disease): This rare type affects children and teenagers. Symptoms appear before age 20.
• Symptoms:
  • Common symptoms include uncontrollable dance-like movements (chorea) and abnormal body postures, as well as problems with behavior, emotion, thinking, and personality.
  • Other symptoms may include tremor (unintentional back-and-forth movement in the person’s muscles) and unusual eye movements. The eye movements can happen early in the disease.
  • These symptoms get worse over time.
  • As HD progresses, you will need constant assistance and supervision because of the debilitating nature of the disease.
  • People usually die from the disease within 15 to 20 years of developing symptoms.
• Treatment: There is no treatment that can stop or reverse HD, but health care providers can offer medications to help with certain symptoms.

Source: SA

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