Scientists Discover What Causes Huntington’s Disease, Opening a Potential New Path for Alzheimer’s and Parkinson’s Research 

Julie Andrews, 87, star of “The Sound of Music,” “My Fair Lady,” and “Mary Poppins,” has been a stalwart advocate in the fight against Huntington’s disease for over 30 years. She has been a spokesperson and supporter of the Hereditary Disease Foundation (HDF), which is committed to finding treatments and cures for Huntington’s and other hereditary illnesses.  

Huntington’s disease (also known as Huntington’s chorea), is a rare neurological disorder. The symptoms can include: 

    • Difficulty concentrating and memory lapses; 
    • Depression; 
    • Stumbling and clumsiness; 
    • Involuntary jerking or fidgety movements of the limbs and body; 
    • Mood swings and personality changes; 
    • Problems swallowing, speaking, and breathing;  
    • Difficulty moving;
    • Cognitive decline.  

There is no treatment yet to halt the progression of Huntington’s, which leads to death after 10 to 25 years. But one could be on the way due to a recent discovery that I will describe later in this article!

“This is a devastating inherited condition that often waits until mid-life to strike,” Andrews says. “Huntington’s is also a ‘marker disease,’ which means that if a cure is found, researchers will gain insight on how to cure hereditary forms of Alzheimer’s disease, Parkinson’s disease, Lou Gehrig’s disease, other genetic diseases — even cancer.” 

Because it is what’s known as an “autosomal-dominant disorder,” each child of a parent with Huntington’s disease has a 50 percent risk of inheriting the illness. In the United States, the prevalence of the disease is approximately 10 cases per 100,000 people—about 30,000 people in all. However, there are another 150,000 individuals at risk. 

Is Huntington’s Disease Always Inherited? 

Because HD is a genetic disease, the condition is always inherited — individuals are only at risk of developing the condition if one or both of their parents have had it. Occasionally, it is possible to develop Huntington’s disease without having a known family history, but this is usually just because one of your parents had it but was never diagnosed with it. 

Symptoms of Huntington’s disease typically start between the age of 30 and 50, but some individuals experience them much earlier.  

A New Breakthrough Could Have Implications for Huntington’s and Other Degenerative Brain Diseases, including Alzheimer’s 

Scientists at the Stowers Institute for Medical Research in Kansas City have identified what is responsible for causing Huntington’s disease and are proposing a potential treatment approach.  

Similar to other neurodegenerative diseases, Huntington’s occurs when certain proteins in one area of the brain begin to form toxic clumps. Then, the process starts spreading. According to Randal Halfmann of the Stowers Institute, these individuals begin to lose control of their body movements and have mental impediments over time. He describes how Huntington’s has symptoms that overlap those of diseases like Alzheimer’s and Parkinson’s. 

Halfmann says his team has developed a tool that allowed them to conduct a series of experiments inside individual cells, using cells as “test tubes.” According to Halfmann, “In Huntington’s disease, the segment of a protein that becomes abnormal is called polyQ. So the team began creating and testing lots of different versions of polyQ inside living cells. The approach worked. The team found the match that seems to ignite Huntington’s disease. In the end, what starts this little forest fire in the brain or in a neuron is a single molecule of polyQ.” 

Once the team had identified that molecule, they were able to find a way to prevent it from spreading — at least in the lab — by flooding the cell with a specific type of protein. Halfmann says the next step will be to develop a drug that can do something similar in people. He believes that the study, which appears in the journal eLife, could help researchers studying other diseases, including Alzheimer’s, Parkinson’s, and ALS (also known as Lou Gehrig’s disease). 

How the Huntington’s Study Can Help with Alzheimer’s Research 

Studies like this show that scientists are finally closing in on strategies that will slow or halt diseases including Parkinson’s and Alzheimer’s, according to Corinne Lasmézas, who studies neurodegenerative diseases at the Wertheim UF Scripps Institute in Jupiter, Florida, who was not involved in the Huntington’s study. 

Early Alzheimer’s drugs targeted the large amyloid plaques found in the brains of people with the disease. But these drugs didn’t work. According to Lasmézas, newer Alzheimer’s drugs, such as lecanemab, still remove large clumps of amyloid, “but they also recognize the ones that are smaller and that are more toxic. And this is why they block more efficiently, the neuronal toxicity.” These smaller clumps form before plaques appear, and are closer to the event that touches off Alzheimer’s in the first place, Lasmézas says. 

“For a long time, we didn’t know much about the mechanism of neurodegenerative diseases,” she says. “Within the last, let’s say, 15 years, there’s been literally an explosion of knowledge.” 

Do You Have a Loved One with Huntington’s Disease or Another Neurodegenerative Disorder? 

Carlos Briceño is a local journalist residing in Maryland who writes about his wife’s and daughter’s battles with Huntington’s disease. In 2018, his wife, Jill, found out she was gene-positive for Huntington’s disease at the age of 41, while his daughter found out she was gene-positive for HD in 2019 when she was 22. Jill and Carlos write about their day-to-day struggles and triumphs to share their knowledge and to let others know they are not alone. You can read their articles about caregiving, love and marriage, cognitive effects, and what happens when life doesn’t go as planned here. 

For additional resources, please visit the Huntington’s Disease Society of America website here.  

If You Have a Loved One with a Neurodegenerative Disorder, Be Sure to Plan Ahead! 

Do you or a loved one have Alzheimer’s, Parkinson’s, Huntington’s, or another neurodegenerative disease? If you have not done Incapacity Planning, Long-Term Care Planning, or Estate Planning (or had your Planning documents reviewed in the past three to five years), now is a good time to plan and get prepared! Among other services, we offer peace of mind through our four levels of lifetime protection planning:       

Level 1 — Incapacity Planning is about protecting your assets from lifetime probate.       

Level 2 — Revocable Living Trust Estate Planning is about protecting your assets from lifetime probate and after-death probate.       

Level 3 — Living Trust Plus® Asset Protection Planning provides protection from probate, lawsuits, home care, and assisted living expenses by allowing access to Veterans Aid and Attendance benefits, and nursing home expenses by allowing access to Medicaid.   

Level 4 – Life Care Planning, Medicaid Asset Protection, and Veterans Asset Protection provides comprehensive planning and filing services, often at times of crisis, though this type of planning can be done anytime someone is beyond the first step of the Elder Care Continuum aka Aging Continuum.        

Please contact us whenever you are ready to ensure that you have the appropriate level of planning: 

Northern Virginia Elder Law Attorney: 703-691-1888              
Fredericksburg, VA Elder Law Attorney: 540-479-1435              
Rockville, MD Elder Law Attorney: 301-519-8041              
Annapolis, MD Elder Law Attorney: 410-216-0703    

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About Evan H Farr, CELA, CAP

Evan H. Farr is a 4-time Best-Selling author in the field of Elder Law and Estate Planning. In addition to being one of approximately 500 Certified Elder Law Attorneys in the Country, Evan is one of approximately 100 members of the Council of Advanced Practitioners of the National Academy of Elder Law Attorneys and is a Charter Member of the Academy of Special Needs Planners.

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