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Huntington's disease is a progressive, degenerative disease that causes certain nerve cells in your brain to waste away. As a result, you may experience uncontrolled movements, emotional disturbances and mental deterioration. Huntington's disease is an inherited disease. Signs and symptoms usually develop in middle age. Younger people with Huntington's disease often have a more severe case, and their symptoms may progress more quickly. Rarely, children may develop Huntington's disease. Medications are available to help manage the signs and symptoms of Huntington's disease, but treatments can't prevent the physical and mental decline associated with the condition.
Complications of Huntington’s disease
After onset of the disease, signs and symptoms continue until death. Though the signs and symptoms vary from person to person, vital functions, such as swallowing, eating, speaking and walking, usually degenerate over time. Depression is common in Huntington's disease, and some people are at risk of suicide. However, death generally occurs as a result of complications of the disease, such as a fall or an infection like pneumonia.
Causes of Huntington’s disease
Signs & Symptoms of Huntington’s disease
The signs and symptoms of Huntington's disease can vary significantly from person to person. Huntington's disease usually develops slowly, and the severity of signs and symptoms is related to the degree of nerve cell loss. Death occurs about 10 to 30 years after signs and symptoms first appear. The disease progression may occur faster in younger people.
Early signs and symptoms of Huntington's disease often include:
Your family and friends may notice these changes before you become aware of them.
Later signs and symptoms of Huntington's disease can include:
Young people who develop Huntington's disease may have signs and symptoms that mimic Parkinson's disease:
Seizures may also occur in those with early-onset Huntington's disease.
Diagnosis of Huntington’s disease
During an initial evaluation, your doctor will:
If your doctor suspects Huntington's disease, you'll likely:
Your doctor may also suggest:
These imaging tests can help your doctor identify any changes to your brain's structure and rule out other disorders.
Treatments of Huntington’s disease
No satisfactory treatment is available to stop or reverse Huntington's disease. Some approaches can control signs and symptoms, but Huntington's disease eventually causes physical and mental disability. As the disease progresses, long-term nursing home care may be necessary.
Tetrabenazine (Xenazine) is the first medication to be specifically approved by the Food and Drug Administration for the treatment of the signs or symptoms of Huntington's. It helps reduce the jerky, involuntary movements of Huntington's disease by increasing the amount of dopamine available in the brain. Possible side effects include insomnia, drowsiness, nausea and restlessness. This medication isn't recommended for use in anyone with depression, especially anyone with suicidal thoughts. Tranquilizers such as clonazepam (Klonopin) and antipsychotic drugs such as haloperidol and clozapine (Clozaril) can help control movements, violent outbursts and hallucinations. While these medications can be helpful, a common side effect is sedation, and in some cases, these medications may cause additional stiffness and rigidity. Various medications, including fluoxetine (Prozac, Sarafem), sertraline (Zoloft) and nortriptyline (Pamelor), can help control depression and the obsessive-compulsive rituals that some people with Huntington's disease develop. Medications such as lithium (Eskalith, Lithobid) can help control extreme emotions and mood swings. Side effects from many of the drugs used to treat the symptoms of Huntington's disease may include hyperexcitability, fatigue and restlessness.
Huntington's disease can impair your speech, affecting your ability to express complex thoughts. You may find that speech therapy helps. Remind friends, family members and caregivers that if you don't speak, it doesn't necessarily mean that you don't understand what's going on. Ask people to continue talking to you and keep your environment as normal as possible.
Physical and occupational therapy
Physical therapy can help keep muscles stronger and more flexible, which helps maintain balance and may lessen the risk of falling. Occupational therapy can help make your home safer and give you strategies for coping with memory and concentration problems. Later in the disease, occupational therapy can assist you with eating, dressing and hygiene challenges.
Experimental treatments and new research
Scientists are working to try to come up with new treatments to slow the course of Huntington's disease. One area of research is the combination of certain cancer and AIDS drugs. This combination has halted the progress of Huntington's in fruit flies. The use of stem cells is another avenue for research. Transplanted in the brain, stem cells might help reduce some of the damage that's been done to the neurons in the brain. Animal studies have shown promising results, but much more research must occur. In October 2008, researchers began recruiting for a randomized, placebo-controlled clinical trial of a new drug currently known as ACR16. This medication stabilizes levels of dopamine, which may improve motor, cognitive and psychiatric functioning.
Prevention of Huntington’s disease
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