The effect of multidisciplinary rehabilitation on brain structure and cognition in Huntington's disease

Read Full Text: The effect of multidisciplinary rehabilitation on brain structure and cognition in Huntington’s disease: An exploratory study (Free to access)
Journal: Brain and Behavior (Impact Factor: 2.072)
Year Published: 2015
Ranked 50th on our 2014-2019 list of the 50 most influential articles

Huntington’s Disease is a genetic disorder that causes a progressive breakdown of nerve cells in the brain. Symptoms often appear in patients’ 30s or 40s.

The time from the emergence of symptoms to the patient’s death is typically between 10-30 years.

There is currently no cure for Huntington’s Disease and medications, at best, help manage symptoms, as opposed to actually altering the course of the disease.

While we never want to give our patients false hope, and we can’t predict the future, and there are new potential treatments on the horizon for Huntington’s Disease.

The neuroplasticity of the brain continues to surprise researchers, even in cases where we once thought the degeneration of the brain was fixed. In this very early, very exploratory research, participants in multidisciplinary rehabilitation demonstrated increased gray matter volume in specific areas of the brain. They also demonstrated significant improvement in verbal learning and memory.

Let’s dive into what this means for our patients and the therapy we provide.

Important reminder about the symptoms of Huntington’s Disease

Like other neurodegenerative diseases, such as Parkinson’s Disease and Alzheimer’s Disease, Huntington’s disease affects a person’s functional abilities over time. Both physical and mental abilities are affected.

The presentation of symptoms varies from patient to patient, and I encourage you to look at the full range of symptoms laid out by the Mayo Clinic.

Most relevant to this study are the cognitive deficits, which have been shown to correlate to loss of gray matter in the brain.

What multidisciplinary therapy was provided in this study?

15 patients undertook multidisciplinary therapy for 9 months. The therapy consisted of:

  • 1x/week supervised clinical exercise
  • 3x/week self-directed home-based exercise
  • Occupational therapy every 2 weeks

The team that designed the program consisted of:

  • Physical therapists
  • Exercise physiologists
  • Occupational therapists
  • Strength and conditioning specialists

What details were given about the OT that was provided?

Hidden in the article is a Word document with the details of all of the therapy provided! I’m attaching it here for ease of access:

Supplementary Appendix attachment (22.6 KB)

The short version is that OT consisted of the following exercises, designed to enhance cognition and executive function:

  • Verbal planning
  • Memory
  • Problem solving

What assessments were used?

MRI images of the brain were an important outcome measure used in this study.

Cognitive and executive function measures (that OTs can administer) included:

What were the results—and why do the researchers believe that the therapy appeared to have some positive benefits?

The imaging showed that, as expected, there was gray matter loss in many areas of the patients’ brains. However, contrary to expectations, two specific regions actually showed brain matter increases. These regions were the right caudate and dorsolateral prefrontal cortex (bilaterally in this region).

Also, on the cognitive assessments, the researchers saw a steady decline on the majority of the tests—except there was a significant improvement in verbal learning and memory on the HVLT-R!

The researchers believe the improvements in gray matter and learning in those particular areas were correlated, as the dorsolateral prefrontal cortex has been shown to be a primary driver of memory retrieval and recognition.

This is exciting because it suggests that neuroplasticity may still be present in patients with HD—and that the plasticity seems amenable to therapeutic interventions!

This complements previous research, like we have seen in Parkinson’s Disease, which suggests that exercise can increase neuroplasticity. This also suggests that patients with Huntington’s Disease might find that lifestyle factors can play a role in the clinical presentation of the disease.

Takeaways for OT practitioners

(These are my personal takeaways, and were not mentioned in the article.)

1. These patients should be on our caseload.

Since Huntington’s Disease is rare, it might be intimidating when a new patient with the disease is scheduled to your caseload. But, on our caseloads is exactly where these patients need to be.

Even though we still have much to learn about this disease, we need to remember that there simply aren’t many answers for these patients out there–and, at this point, the most helpful thing we can do is to listen carefully to the individual in front of us. After all, we can be valuable partners and guides as they work to maintain their quality of life in the areas that matter most to them.

2. Just like in Parkinson’s Disease, exercise seems to hold promise in activating neuroplasticity. And, at the very least, exercise helps maintain physical health for as long as possible.

We’ve discussed in the Club how exercise can induce neuroplasticity in Parkinson’s Disease—and how that, in part, helps the brain combat the degenerative effects of the disease.

This current article suggests that the same may be true in Huntington’s Disease. But, overall, the evidence does not seem as robust in HD as in PD. The most recent article I could find specifically on the effects of exercise in HD found that the effects of exercise on cognition were “incongruent.”

So, even though this current article appears to hold promise, it’s our responsibility to keep tabs on the research for the latest developments. And, in the meantime, if I had a family member with Huntington’s Disease, I would certainly continue to recommend regular exercise. Not only would I optimistically hope that exercise might combat the effects of the disease—if nothing else, exercise can have a dramatic positive impact on mood, physical health, and overall quality of life.

(Possibly) Earn CEUs/PDUs for reading this article

Many of you can receive continuing education credits for reading this article. Here’s a form to help you do it, along with information to help you understand who qualifies.

And, here’s the full APA citation you might need:

Cruickshank, T. M., Thompson, J. A., D, J. F. D., Reyes, A. P., Bynevelt, M., Georgiou-Karistianis, N., … Ziman, M. R. (2015). The effect of multidisciplinary rehabilitation on brain structure and cognition in Huntingtons disease: an exploratory study. Brain and Behavior , 5 (2). doi: 10.1002/brb3.312

What questions/thoughts does this article raise for you?

As an OT, I worked in a SNF that was a center for HD. I acquired a great deal of experience treating this population. It was also sad to watch these patients suffer as the disease progressed. I wrote two articles on HD for ADVANCE several years ago, and when I was a travel therapist in a facility with HD patients, I had some expertise with this population that I could share.
Whether we can improve the brain function of those with HD as this study implies, therapy can offer patients a longer period of maintaining their independence before they rapidly decline. Staff, caregiver education and equipment recommendations are also an important part of treatment. Aspiration is a risk for this population, therefore, OT and ST need to be involved in addressing dysphagia.
HD effects an individual in a global way; physical, cognitive, emotional, and psychological. I had one patient who was not diagnosed until his mid 50’s, and his wife divorced him because she suspected him of becoming an alcoholic due to his ataxic gait, and changes in behavior. Offspring have a 50% chance of inheriting the disease, and only through screening can they decide whether it is wise to start their own families.
In the future, I hope more resources will go into finding a cure for this debilitating disease.

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It is so interesting to me the implications of exercising in both disease and wellness. As a long time health enthusiast and occupational therapist , I can definitely see a connection. With the aging of the Baby Boomers, OTs have a wonderful opportunity to consult in senior living environments as clients transition from independence to assisted levels of care… and not just on physical limitations to to also address cognitive and social skills as well.

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This is a great topic; I am a huge believer in the many positive effects of exercise. I recently read a great book that gave me some insight into Huntington’s. It is called Inside the O’Brien’s by Lisa Genova. She is an author that often takes a disease and writes an insightful book, giving some understanding of life with that disorder.

Here is the synopsis for anyone interested in the book:

"Joe O’Brien is a forty-four-year-old police officer from the Irish Catholic neighborhood of Charlestown, Massachusetts. A devoted husband, proud father of four children in their twenties, and respected officer, Joe begins experiencing bouts of disorganized thinking, uncharacteristic temper outbursts, and strange, involuntary movements. He initially attributes these episodes to the stress of his job, but as these symptoms worsen, he agrees to see a neurologist and is handed a diagnosis that will change his and his family’s lives forever: Huntington’s Disease.

Huntington’s is a lethal neurodegenerative disease with no treatment and no cure. Each of Joe’s four children has a 50 percent chance of inheriting their father’s disease, and a simple blood test can reveal their genetic fate. While watching her potential future in her father’s escalating symptoms, twenty-one-year-old daughter Katie struggles with the questions this test imposes on her young adult life. Does she want to know? What if she’s gene positive? Can she live with the constant anxiety of not knowing?

As Joe’s symptoms worsen and he’s eventually stripped of his badge and more, Joe struggles to maintain hope and a sense of purpose, while Katie and her siblings must find the courage to either live a life “at risk” or learn their fate."

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Sarah,
Thank you for placing the information from the supplementary table which included details related to the ‘exercises used in occupational therapy sessions.’ The plan included all the evidence based pieces of exercise: warm up, aerobic activity, resistant exercise, and cool down as well as fine motor and gross motor related to balance and core. Finally, functional activities related to daily activities with cognitive activities to help with planning/organization, memory, and problem solving where included.
Unlike some articles we have read this appears to be a well rounded OT approach to the client. The problem solving activities caught my attention. Sudoku, cross word puzzles, and word scrambles are cognitive activities I have encouraged clients to do daily to keep their brain active. In the old days, these activities came daily to clients in the form of a daily paper with comics. There are electronic apps to complete some of these types of activities now but I wonder if the keyboard allows for the same grey matter formation as the traditional pencil paper tasks.
In children the answer in research is no. Grey matter is changing secondary to screen time and keyboard use and lack of pencil paper tasks. The study did not distinguish methods of completing the activities.
James and Everhardt did some of the first studies which showed writing letters and tracing created brain cells and typing did not in children. (James, K. & Everhardt, L. (2012). The effects of handwriting experience on functional brain development in pre-literate children. Trends in Neuroscience and Education, 1(1), 32–42. https://doi.org/10.1016/j.2012.08.001). More recently Hutton, Dudley, and group found decreased white matter in children who were exposed to more screen time than recommended by the AMA. (Hutton, J. S., Dudley, J., Horowitz-Kraus, T., DeWitt, T., & Holland, S. K. (2019). Associations Between Screen-Based Media Use and Brain White Matter Integrity in Preschool-Aged Children. JAMA Pediatrics, e193869–e193869. https://doi.org/10.1001/jamapediatrics.2019.3869)
There is also research on student learning more when they take notes by hand vs typing see Cindy May’s article at https://www.scientificamerican.com/article/a-learning-secret-don-t-take-notes-with-a-laptop/.
My point is that once again the brain develops and demonstrates plasticity; this knowledge allows occupational therapy practitioners across the lifespan to consider how to use this knowledge for the benefit of both young and old clients.

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Thank you for all these helpful links from this article!
I think this study is great to look into more neurological diseases, specifically ones that can tend to be seen less often, but still relevant to practice. I really enjoyed the tables breaking down specific exercises performed throughout the study. I also liked seeing some more function based tasks specific to OT. This really helps me realize it has to be a team effort. Exercise alone is great, functional activities alone are great, but putting them together could mean more outcomes for our clients. I would be curious to see if/when a larger study is completed for this research to continue to support the use of multiple disciplines and approaches.
I think that neurological disorders are becoming more prevalent in our caseloads and populations across all ages and practice settings, so I enjoy reading and learning more about what research is available and saying about our role in neurological conditions.

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These are some great points Sherry! I think this is some great connections to grey matter with use of technology versus fine motor to complete word searches or puzzles. I will definitely keep this in mind when working with my patients! And thanks for the article links as well! I know these will be helpful to look at!

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I have had few patients with HD. This article will certainly make me an advocate for future HD patients to get multi-disciplinary treatment in my home health setting. I have found that patients with some compromised cognitive issues have responded well to non-traditional physical and creative activities like batting a balloon with a pool noodle to keep it up in the air and stacking cones and balancing a ball on the top. I have been surprise by the patiens tenacity and problem solving given their limitations. There is no right answer and thus no sense of failure to meeting these challenge. Sometimes offering patients something out of the ordinary engages them more than a typical task. It would be interesting if a further study could include more out of the box challenges to see if there is further increase in brain matter.

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Wow, @sherry, this is really fascinating. I had never seen any research related to white matter and using paper and pencil (versus screen time.) I think we all have a basic understanding that less screen time is better for all of us, but this really takes it to a new level.

And, I agree that this research has impacts across the life-span!

A concept I keep seeing in research about cognitive decline is the importance of building our “cognitive reserve” as this may have a neuroprotective effect. And so if building cognitive reserve is important for our patients, it seems we definitely need to be pointing them away from screens!

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Hear! Hear! I totally agree with you! The way I’ve begun to think about this is that the research seems to be suggesting that exercise not only has a neuroprotective effect, but it almost seems to prime the brain for new learning. I bet we can all relate to this in our own lives- that when we exercise we feel more ready to learn later in the day. So, that intuitively makes sense to me that this would be true for our patients as well!

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Lauren and Sarah,
I can not agree more with this statement ‘exercise alone is not enough.’ First this does not speak to the uniqueness of the profession of occupational therapy and second it is less client-centered unless the client is an athlete or ‘workout buff’.
Recently my father-in-law was admitted to SNF and I worked hard to not be ‘that family member’ but the outcome of ‘therapy’ was that my father-in-law could walk with minimal help but not dress self, manage clothes, or toilet despite my multiple voiced concerns. We moved my father-in-law to assisted living and were offered home health therapy and I was blunt with the individual organizing therapy. I stated I wanted a self-care OT not a biomechanical OT because increasing my father-in-laws ability to bath, dress, and toilet would decrease cost of his care monthly. OT practitioner’s need to embrace the impact we can have on client’s by understanding client’s needs and costs. Increasing my father-in-law’s self care abilities could save up to 1000.00 per month in his care at an assisted living facility. These type of studies which balance exercise, self care, and cognitive approaches can demonstrate are worth in dollars

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Thank you for sharing your Wealth of knowledge on HD. I have a sincere appreciation for the perspective you offered in viewing this through the lens of Life. By the use of therapeutic self in working with clients and bringing to life the impacts of the disease, I hope discussions like this bring about more awareness for us as professionals to share as it pertains to HD, for instance the impacts and interruptions to life and routine, as in the client thought to be struggling with alcoholism when he was actually dealing with this condition.

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Hello!
I just joined, and sorry for the late response. A very exciting event occurred two years ago in the world of Hd. In the UK an RNA suppression drug was trialed with 15 patients to see if it would harm them. It was a O trial so, it wasn’t intended to show anything, just hoping not to kill tgem. It required the medicine to be delivered via a spinal tap. After 15 months, all patients were reverted to the meds, because they found that not only did it not have any side effects that weren’t related to a spinal tap; these patients showed a decrease in the replication of the Hd Proetin. The replications instead of getting worse, slowed down. This was the biggest news in 2017. A possible treatment for hd. In this, the company who has the drug got it accepted as orphan status, the researchers fast tracked the IRB and because it was so successful it went straight to a 2-3 trial! This trail included US participants and a larger sample. 206 Hd patients across the county were recruited last spring and the study began this fall. It will be a two year study. It has three levels, sadly one has to be a placebo. They receive the drug via spinal tap every other month. The patients in the 0 trials are still receiving the drug and many have (antidotally) said their motor skills have improved. My guess is this improvement is function. This is the biggest news in Hd since we found the gene. The same drug system is being used to treat SMA infants. I know Jama had an article on the 0 trials that was published last summer. I can try and find it in my database if interested. Otherwise, if you look up Ionis Hd drug treatment trial or go to the Hd web site you’ll find info there!
I have a friend in the trail. After preparing for his death for 20 years, he is now wondering if he may live. This is the most advancement we’ve ever had with Hd. While it isn’t a cure —-it isn’t DNA editing—something to stop or slow the replication of the Hd protein is huge!

Teri Rupp.

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Oh wow!! I kept reading that promising trials were underway, but it is so nice to have someone actually break one down for us. Here’s an easy to read update on the trials that I found:

I always say that we never want to offer false hope to patients. But, seeing that new pharmaceutical treatment options may be coming to market in the next few decades, makes our work of helping patients maintain quality of life through exercise and building cognitive reserve even more important.

Yes, I agree. I actually knew this was coming up so I insisted that my friend continue to do all his own ADLs and do private yoga while waiting for the trial. He and I both believe that this contributing to him being selected. He had an OT do a battery of tests prior to the study in order to do them again in a year, then year two. This isn’t part of the study. He doesn’t know if he is in a treatment category but, it may help ease his worry.

This is the best news for Hd in years, and it will contribute to our treatment knowledge of other types of dementia. Of all the trials in Hd, this is the one that shows the mist promise. It showed enough that it went from clinical trial 0 to 2/3 and they did it in less than 18 months from the results of that first trial.
Teri

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I thoroughly enjoyed reading and learning from this article as an OTA student! I am loving getting to know all the areas in which we can make a difference in the lives of these individuals. It is so amazing to see that if we simply take time to provide these people with a well-rounded POC, that they can actually fight against Huntington’s Disease and regain some function. It is so promising! I also found it very beneficial to see a table of the specific intervention plan that was used.

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