Saturday 2 February 2019

What you need to know about Huntington's disease


Huntington's disease is an incurable, hereditary brain disorder. It is a devastating disease that causes damage to brain cells, or neurons.
It happens when a faulty gene causes toxic proteins to collect in the brain.
Huntington's disease (HD) affects one person in every 10,000, or around 30,000 people in the United States. Another 150,000 or more people are at risk of developing the condition.
The first signs normally appear between the ages of 30 and 50 years.
Fast facts about Huntington's disease
·     Huntington's disease (HD) is an inherited disease that attacks nerve cells gradually over time.
·     The disease happens when a faulty gene makes an abnormal version of the huntingtin protein.
·     Early symptoms may include mood swings, clumsiness, and unusual behavior.
·     During the later stages of the disease, choking becomes a major concern.
·     There is currently no cure, but medications may help relieve symptoms.

What is Huntington's?

Huntington's disease (HD) is a neurological condition. It is an inherited disease that happens due to faulty genes. Toxic proteins collect in the brain and cause damage, leading to neurological symptoms.
As parts of the brain deteriorate, this affects movement, behavior, and cognition. It becomes harder to walk, think, reason, swallow, and talk. Eventually, the person will need full-time care. The complications are usually fatal.
There is currently no cure, but treatment can help with symptoms.

Symptoms

Signs and symptoms are most likely to appear between the ages of 30 and 50 years, but they can occur at any age. They tend to worsen over 10 to 20 years.
Eventually the Huntington's disease or its complications are fatal.
According to the Huntington's Disease Society of America (HDSA), the symptoms of HD can feel like having amyotrophic lateral sclerosis (ALS), Parkinson's and Alzheimer's all in one.
The key symptoms include:
·         personality changes, mood swings, and depression
·         problems with memory and judgment
·         unsteady walk and uncontrollable movements
·         difficulty speaking and swallowing, and weight loss
How signs and symptoms evolve can vary between individuals. In some people, depression occurs before motor skills are affected. Mood swings and unusual behavior are common early signs.

Early signs and symptoms

Early symptoms may not be recognized if HD has not previously occurred in the family. It can take a long time to reach a diagnosis.
Initial signs and symptoms include:
·         slight uncontrollable movements
·         small changes in coordination and clumsiness
·         stumbling
·         slight signs of mood and emotional change
·         lack of focus, slight concentration problems, and difficulty functioning, for example, at work
·         lapses in short-term memory
·         depression
·         irritability
The person may lose motivation and focus, and appear lethargic and lacking in initiative.
Other possible signs of HD may include stumbling, dropping things, and forgetting people's names. However, most people do these from time to time.

The middle and later stages

In time, symptoms become more severe.
These include physical changes, loss of motion control, and emotional and cognitive changes.

Physical changes

The person may experience:
·         difficulty speaking, including looking for words and slurring
·         weight loss, leading to weakness
·         difficulty eating and swallowing, as the muscles in the mouth and diaphragm may not work properly
·         risk of choking, especially in the later stages
·         uncontrollable movements
There may be uncontrollable body movements, including:
·         uncontrollable movements of the face
·         jerking of parts of the face and the head
·         flicking or fidgety movements of the arms, legs, and body
·         lurching and stumbling
As HD progresses, the uncontrollable movements occur more often and with usually with more intensity. Eventually they may become slower as the muscles become more rigid.


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