By Interactive Metronome - January 2, 2014

Chorea? Didn’t we fight a war there once?

George Huntington was the first to write about “chorea” in 1872, although the disease appears to have been plaguing mankind for hundreds, if not thousands, of years. Chorea comes from the Greek word for dance, and was used to describe how those affected will twist and writhe uncontrollably in the later stages of the disease. Today, the Huntington’s disease affects nearly 30,000 people in the US, with as many as 150,000 at high risk of developing symptoms. It is estimated that Huntington’s affects 1 per 10,000; meaning over 700,000 people worldwide may be living with the condition.


Huntington’s disease is a hereditary condition with no known cure. The disease is caused by a defect in a single gene, now called Huntingtin; since it is an autosomal dominant disorder, a person only needs one affected copy to inherit Huntington’s. Therefore, any children of a Huntington’s carrier will have a 50 percent chance of developing the disorder. A normal gene will have what is known as a CAG repeat up to 28 times; however, affected genes may repeat this sequence as many as 120 times, with more repeats leading to worse symptoms that will develop at a younger age.


Symptoms usually appear between 30 and 50 years of age, but juvenile Huntington’s is common in families with a long history of the disease. The disease will progress for up to 30 years before individuals succumb to infections, falls or, unfortunately, suicide. As with most degenerative genetic conditions, Huntington’s will ultimately have an affect on all areas of the body. Although Huntington’s usually affects a person’s cognitive abilities and behavior, the physical signs are generally easier to identify in the early stages.


Involuntary twitching, jerking and muscle contractions are common. Individuals will ultimately lose fine motor control, which affects everything from handwriting to saccadic eye movement. Trouble with voluntary movement leads to difficulty keeping a job, driving, performing activities of daily living (ADLs), and maintaining independence. Generally, earlier symptoms are a sign of more severe cases with more rapid degeneration; that can make it especially difficult for families with multiple generations of sufferers. In time, individuals will require 24-hour assistance, a daunting task for one parent who may be left to care for an ailing spouse and newly diagnosed child.


Huntington’s takes more than an individual’s physical independence; the cognitive and behavioral changes may deprive sufferers of a quality life during their final years. The disease may lead to difficulty with focus, impulse control, mental flexibility, memory, spatial awareness, planning and sequencing, conversation and organization. Comorbid conditions include obsessive-compulsive disorder, bipolar disorder and epilepsy. Additionally, individuals may suffer from anxiety, insomnia, antisocial behavior, fatigue, changes in appetite, reduced or hyperactive sex drive, and depression.


This depression comes from the physical changes in the brain, but is also related to the feelings of helplessness many sufferers experience as the disease progresses. Sadly, the loss of function and quality of life leads many Huntington’s patients to take their own lives. Fortunately, new research is leading to treatments that can drastically increase the quality of life for those affected by Huntington’s. While there is no cure, drugs such as Xenazine and the anti-anxiety drug Valium have shown promise suppressing involuntary movements. Antipsychotic drugs like Haloperidol and Clozapine will not only help with anger, irritability and moodiness, but also help control twitching and jerking movements. Other drugs used to treat Huntington’s symptoms include Lexapro, Klonopin, Prozac, Zoloft, Lithium and Depakote; however, all of these drugs focus on specific symptoms and must be used under the direct supervision of a licensed caregiver.


But what if drugs are not a suitable treatment? What if additional training could help maintain/regain independence without the use of drugs? Huntington’s patients often seek assistance from occupational, physical and speech therapists. These professionals work with sufferers to help maintain independence and quality of life. However, it is all too common for these therapies to be administered individually, possibly years apart. That is where Interactive Metronome® (IM) can fill in the void and take therapy to the next level.


Research shows that engaging whole body movements in combination with cognitive tasks leads to overall better outcomes. IM is a patented and unique training tool that challenges thinking and movement simultaneously, providing real-time millisecond feedback to help synchronize the body’s internal clock. Restoring the critical timing and rhythm to functional brain networks helps to improve neural efficiency and communication. Improving this functionality and communication helps keep your brain active and can prevent the rapid degeneration of neurons. IM has been shown to improve many of the areas that are affected by Huntington’s, including: focus, memory, planning and sequencing, balance, coordination, spatial awareness, impulse control, fine motor skills and fluency.


Check out the research on mental timing and IM here to find out how Interactive Metronome® can change your life. You might not even have to leave your house! The new IM-Home allows individuals to enjoy all of the benefits of IM training without having to visit a clinic three times a week. One of our favorite “brain docs” is Dr. Kevin McGrew, a leader in the field of psychometrics and brain timing. Check out his blog, where he talks about the connection between timing and Huntington’s in laboratory studies. If you still have questions, ask your healthcare provider how IM training could help restore the function Huntington’s has taken.

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