Huntington's disease
Find a neuro specialistHuntington’s disease damages nerves in the brain and causes problems with involuntary movements and thinking processes that get worse over time. You may notice unintended movements and struggle with mental tasks.
If believe that you or a loved one may have Huntington’s disease, see your Aurora Health Care provider to learn more and get treatment as quickly as possible.
What is Huntington's disease?
Huntington’s disease is a progressive neurological disorder caused by mutation of the HTT gene, also known as the huntingtin gene. When a person has Huntington’s disease their cells make abnormal versions of huntingtin protein which disrupts the normal function of nerve cells in the brain. The abnormal proteins cause specific damage to nerves in the brain and the beginning of Huntington’s disease.
Huntington’s is often inherited from parents. When it develops in an individual with no family history, it’s called sporadic Huntington’s disease.
Other names for Huntington’s disease
Other names for Huntington’s disease are Huntington’s chorea and Huntington chronic progressive hereditary chorea.
At what age does Huntington’s disease appear?
Huntington’s disease usually appears in adults in their 30s or 40s. Huntington’s disease happens more rarely in children and adolescents. When Huntington’s disease starts at a younger age, symptoms become serious more quickly than in adults.
The chances of Huntington’s disease occurring in a child are low. Huntington’s disease is very rare and about 6% of the total number of Huntington’s diagnoses are in children and adolescents.
Huntington’s disease life expectancy
If you have Huntington’s disease your life expectancy is decreased. Adults with Huntington’s usually live 15 to 20 years after Huntington’s disease symptoms begin. Children usually live 10 to 15 years after symptoms begin.
Huntington’s disease symptoms
The damage to nerves in the brain alters messages that travel from the brain to other parts of the body. Those altered messages cause movement disorders (dyskinesias) and other symptoms of Huntington’s disease.
Involuntary jerking movements (chorea) are the symptoms most often associated with Huntington’s disease. For some people, Huntington’s causes rigidity of the muscles (akinesia) or unusual unchanging postures (dystonia). Another symptom of Huntington’s disease, hyperreflexia, creates unexpected stretch reflexes in the muscles. Arms or legs may flail out suddenly.
Different combinations of these symptoms happen for each person and get worse over time which makes both big and small movements difficult.
Other potential Huntington’s disease symptoms include:
- Tremors
- Unusual eye movements
- Slurred speech
- Insomnia
- Loss of energy
- Fatigue
- Seizures
You may also have problems with swallowing and eating, which can result in losing too much weight. If choking becomes a problem, it can lead to infections in the lungs.
Mental & emotional symptoms of Huntington’s disease
Along with these physical symptoms, Huntington’s disease often causes mental disorders leading to loss of decision-making skills, ability to learn new things or ability to put thoughts into words. Due to the gradually debilitating nature of Huntington’s disease symptoms, people sometimes withdraw from social situations.
Changes in mental and physical abilities get worse over time and lead to being unable to work, drive or perform self-care tasks. Some people have serious changes in behavior, including mood swings, irritability, depression, angry outbursts and losing touch with reality. However, many people with Huntington’s disease retain enough mental capacity to express themselves and stay aware of their surroundings.
How is Huntington's disease diagnosed?
To diagnose Huntington’s disease, your doctor will perform a physical and ask about your family and personal medical history. They’ll test your reflexes, balance, muscle tone, hearing, walking, movements and mental abilities.
Brain imaging tests such as MRI will evaluate whether there have been changes in your brain that point to Huntington’s or other conditions. Blood tests may help rule out other conditions. Your doctor may also consult with other specialists such as psychiatrists, neurologists or speech pathologists to help manage your diagnosis or treatment.
Genetic testing may be ordered to confirm or reject the possibility of Huntington’s disease. Prenatal testing is available for people who are concerned about passing the disease to a child.
Huntington's disease treatments
Treatments for Huntington’s disease focus on managing symptoms because there is no cure and no way to prevent the disease.
Medications for Huntington’s disease treatments are available to manage hallucinations, delusions, violent outbursts, depression, anxiety and Huntington’s chorea.
It’s important to watch for medication side effects like fatigue, restlessness and feeling overly excited. Your doctor can help you make decisions about what medications are best for you.
Clinical trials are underway to look for new Huntington’s disease treatments. Along with other subjects, researchers are exploring the cellular basis of Huntington’s disease. They’re looking at things such as:
- How other changes to the Huntington gene affect the disease
- Chemical signaling between cells in the brain
- Optogenetics – where light beams are used to treat neurons in animals
- Biomarkers (parts of cells) that may predict, diagnose or monitor Huntington’s disease
- Stem cell study of cells donated by people with Huntington’s disease
Let your doctor know if you’re interested in participating in clinical trials.
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