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.
Huntington’s usually starts between the ages of 30 and 50 years. Changes in mood and thinking can be an early sign.
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
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
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.
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
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.
These may alternate rather than occurring consistently.
lack of emotion becomes more apparent
There may be:
a loss of initiative
a loss of organizational skills
problems with multitasking
The later stage
Eventually, the person will no longer be able to walk or talk, and they will need full nursing care.
However, they will usually understand most of what is being said and will be aware of friends and family members.
What to know about Parkinson’s dementia
Find out about Parkinson’s dementia, another neurologial condition
The inability to do things that used to be easy can lead to frustration and depression.
Weight loss can make the symptoms worse and weaken the patient’s immune system, making them more vulnerable to infections and other complications.
HD itself is not usually fatal, but choking, pneumonia or another infection can be.
Throughout all stages, it is important to adjust the patient’s diet to ensure adequate food intake.
HD is caused by a faulty gene (mhTT) on chromosome number 4.
A normal copy of the gene produces huntingtin, a protein. The faulty gene is larger than it should be. This leads to excessive production of cytosine, adenine, and guanine (CAG), the building blocks of DNA. Normally, CAG repeats between 10 and 35 times, but in HD, it repeats from 36 to 120 times. If it repeats 40 times or more, symptoms are likely.