Huntington's Disease

What is Huntington's Disease?

Huntington's disease (HD) is a hereditary and deadly disorder that causes nerve cells in the brain tobreak down. This causes physical and mental abilities to weaken, and they get worse over time. There is no cure. If it starts early in life, it’s called juvenile Huntington’s disease.

A diagnosis of Huntington's disease may come as quite a shock. There's a lot to take in.

But tapping into a support system, such as a social worker, therapist, or support group, can make the journey a bit less daunting. With the help of a healthcare team, people with Huntington's can live independently for many years.

Symptoms of Huntington's Disease

Although symptoms may first show up in midlife, Huntington's can strike anyone from childhood to advanced age. Symptoms often first appear when people are in their 30s or 40s. Over 10 to 25 years, the disease gradually kills nerve cells in the brain. This affects the body, mind, and emotions. If symptoms begin before age 20, it’s called juvenile Huntington’s Disease and it may get worse faster.

Symptoms can vary greatly from person to person. And stress or excitement can worsen symptoms.

Some symptoms are easier to spot than others. Abnormal movements may be the first thing you notice. Weight loss can be a concern at all stages.

Symptoms of Huntington's disease tend to develop in stages.

Early stage symptoms

Changes may be quite subtle in early stages, making it possible to keep driving and working. You may just require a little extra help.

Common early symptoms include:

Trouble learning new things

Trouble making decisions

Memory lapses

Mood swings

Clumsiness

Slow or abnormal eye movements

Muscle problems (dystonia)

Trouble sleeping (insomnia)

Loss of energy and fatigue

Middle stage symptoms

With time, symptoms begin to interfere more with your day-to-day life. For example, you might start to drop things or to fall. Or you may have trouble speaking or swallowing.

Staying organized may be difficult. And emotional changes may put pressure on relationships.

Common middle stage symptoms include:

Uncontrolled twitching movements (chorea)

Trouble walking

Confusion

Memory loss

Personality changes

Speech changes

Decline in thinking abilities

Swallowing problems

Breathing problems

Thoughts of death, dying, or suicide

Weight loss

Development of obsessive-compulsive disorder (OCD), bipolar disorder, or mania

Late stage symptoms

In this stage, people with Huntington's must depend on others for their care. Walking and speaking are not possible Most likely you will still be aware of loved ones around you. Fidgety movements may become severe, or may subside.

Juvenile Huntington’s disease symptoms

In children or teens, Huntington's may progress more quickly and cause symptoms like:

Stiff or awkward walking

Increased clumsiness

Changes in speech

Trouble paying attention

Sudden drop in school performance

Behavior problems

Tremors

Seizures

Diagnosing Huntington's Disease

Family history plays a major role in a diagnosis. Your doctor will ask you questions about your medical background and give you a physical exam. If you and your doctor suspect Huntington’s disease, a neurologist will conduct more tests.

A neurologist may test:

Reflexes

Muscle strength

Balance

Sense of touch

Vision

Hearing

Mood and mental status

Memory

Reasoning

Thinking skills

Speech

Treatment and Home Remedies for Huntington's Disease

For now, treating Huntington's involves managing symptoms:

Medications can help control fidgety movements. Your doctor can work closely with you to manage any side effects and to change medications, if needed.

Some antipsychotic drugs have a side effect that controls movement and have been helpful for some people.

Antidepressants can also help with obsessive-compulsive disorder.

Mood-stabilizing drugs can ease symptoms of mood disorders, but may cause other side effects.

Speech or language therapy may be helpful for any problems with speech or swallowing.

Occupational or physical therapy may help you learn how to better control movements. And assistive devices such as handrails can help you manage your changing physical abilities.

Nutritional support ranges from using special utensils to focusing on nutrient- dense foods to supplementing with tube feeding in later stages.

Exercise may be very helpful. People with Huntington's who stay as fit and active as they can seem to do better than those who do not.

Psychotherapy can teach you ways to manage changes in your emotions and how you think. Strategies like breaking tasks into simpler steps may go a long way toward making these changes a bit easier for you and your family.

Family members can help by making some changes at home:

Serve extra meals and add high-calorie supplements to help you stay at a healthy weight.

Limit distractions during meal times.

Choose foods that are easier to chew and swallow.

Use forks and other utensils made for people with limited motor skills.

Use covered cups with straws or drinking spouts.

Keep a regular routine.

Use phone or computer reminders for tasks.

Keep life as calm, simple, and low-stress as possible.

For children, work with the school counselor to make an education plan.

See friends and maintain social interactions as much as possible.

Add wheelchair ramps and elevators to the home if possible.

Add safety bars in bathrooms, next to the bed, and at stairs.

Use voice-controlled lights and other “smart” home features.

Use electronic speech programs or picture charts to aid communication.

Causes of Huntington's Disease

In 1993, researchers found the gene that causes Huntington's. Everyone has the HD gene, but in some families an abnormal copy of the gene gets passed from parent to child. If you have a parent with Huntington's disease, you have a 50% chance of having the gene and developing the disease.

Also:

Men and women are equally likely to inherit the abnormal gene.

If you don't have the abnormal gene, you can't get Huntington's or pass it on to your children.

The disease doesn't skip generations.

If you or members of your family plan to be tested for Huntington's, it's a good idea to get professional genetic counseling first. Counselors can help explain what to expect from the test results.

With knowledge about the HD gene, scientists have been able to learn a great deal about how the disease affects the brain. More importantly, this discovery may help pave the way for future treatment.

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