Interviewing an HD Expert, Part 1: ADHD, Stimulants, and Huntington’s Disease
First in a two-part series.
Something astounding happened recently.
My wife, Jill, found someone who knows more about Huntington’s disease (HD) than she does. Because her father was gene-positive for HD, Jill saw firsthand what the disease does to a person. Many people on his side of the family have also had it, and both Jill and our daughter, Alexus, are gene-positive, so Jill has learned a lot through personal observation and study.
She and I recently interviewed Herwig Lange, MD, a German neurologist who has more than 40 years of experience with HD and juvenile HD. As Huntington’s Disease News’ Larry Luxner reported in 2020, Lange has been researching the disease since 1969 and founded his country’s first HD clinic in Düsseldorf in 1982.
Herwig Lange, MD, is a prominent German neurologist and expert on Huntington’s disease. (Courtesy of Herwig Lange)
During the course of our 40-minute conversation over Zoom, Jill found Lange to be thought-provoking, and she singled out two important points for me to share. This week, I’ll address what she learned about attention-deficit hyperactivity disorder (ADHD) from him, and next week, I’ll talk about the criteria for diagnosing HD, which Lange thinks should be updated.
ADHD and Huntington’s
As I’ve previously written, Jill has ADHD. As a result, she has been prescribed stimulants, which is common for patients who have trouble focusing.
However, through clinical observations, basic knowledge of the brain, and scientific literature, Lange shared that HD often causes attention deficits, and that these deficits have a different biological basis than the attention issues associated with ADHD.
How stimulants can affect Huntington’s patients
Put simply, stimulants help those with ADHD pay better attention and stay alert by increasing dopamine levels.
The cruel irony is that dopamine levels are already relatively high in people with HD in relation to the damaged dopamine receptors in their striatum. The striatum is the part of the brain that deals with motor functions, cognitive executive functions, and movement control. In people with HD, it’s “much less effective,” Lange said, “with less and less functioning cells.” The relatively high dopamine levels cause the less-effective striatum to become overstimulated, which can lead to an increase in involuntary and hyperkinetic movements, such as chorea and psychotic symptoms. This is why medications that block or reduce dopamine are effective in reducing hyperkinesia and psychosis, he explained.
As a result, Lange said, stimulants can be detrimental to someone with HD by worsening these involuntary movements and causing them to manifest sooner. In other words, it’s like giving caffeine pills to someone who’s already had a gallon of coffee.
This was news to Jill.
Because she’d noticed that her stimulant medication wasn’t helping with her ADHD (and after talking to Lange, she now understands why), Jill and her doctor came to the conclusion that she should stop taking the stimulants.
In sharing this information, Jill and I recognize that what we think is the right decision for us may not be the right decision for someone else, which is why I have to end this column with a few important caveats.
If you think you have ADHD, it’s important to be careful about self-diagnosing. Any concerns about this serious mental health issue, any HD-related symptoms, or taking medication should be discussed with a medical professional.
Note: Huntington’s Disease News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website. The opinions expressed in this column are not those of Huntington’s Disease News or its parent company, Bionews, and are intended to spark discussion about issues pertaining to Huntington’s disease.
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Comments
Bonnie Cordova
I so appreciate your column. My daughter has HD and it touches my heart that you're sharing your process. It's so isolating, this brand of grief.
Carlos Briceño
Thank you Bonnie for sharing. I am sorry your family is also suffering under the pressure and stress of HD. I am glad you are finding comfort and support by reading about our journey. HD can be very isolating and that’s what motivates Jill to keep sharing. She wants people to feel like they are not alone in their battle with HD. If you or your daughter feel isolated or that you can’t talk about what is going on in your lives, remember you can always reach out to the social worker from your daughters doctors office. They are always willing to help patients and their families. It’s important to not allow HD to rob you of community.
Lauren Holder
Great post, Carlos! This is a topic I've really wanted to address. My personal experience in the past year has been that the ADHD medication I take really helps me. I can't have other stimulants with it, of course - no caffeine or anything because then I do feel a bit twitchy, but otherwise it's been a tremendous help to me. I believe there needs to be a lot more research in this regard, especially in the pre-symptomatic and prodromal stage of HD.
Carlos Briceño
Hi Lauren,
Thank you for always being so active with HD awareness. I am happy that the medication is working for you. Our journey is a never ending learning experience and we try to share all of our experiences and what we learn because of them along the way. Hopefully soon there will be a cure for HD and you and Jill will only have to worry about your ADHD like me.
Mrs Corey L Ross
I dont have Huntingtons Disease,but some of my classlmates at a school for students with varous neurological disorders had the choriform HD symptoms that was linket to their ADHD ,can a child born with developmental disabilities have such a devastating neurodegenerative disorder on top of their organic brain impairment
Carlos Briceño
Hello Mrs. Ross,
Thank you for reaching out with your question. I am sorry to say that I don't know enough about this to render an answer to your very thoughtful question. If they are seeing a neurologist they could ask the doctor what their medical advice would be. I hope your friends find answers.
Chana
Thanks for posting this information! As someone with pre-manifest HD, I've been looking for information about how to treat early stage executive dysfunction for a while now.
I haven't had any luck finding information on the topic, except seeing that a few people seem to use Vyvanse (which, it sounds like, would be the wrong? thing to take as an HD patient), until seeing this post just now. So I'm really glad you posted this! Thank you.
My follow up question to this would be - what *do* we take instead? If not something like Adderall or Vyvanse?
And if executive dysfunction is caused by something else entirely in people with HD, are the symptoms still exactly the same as for people with ADHD? Or are there any noticeable differences in symptoms between early HD and ADHD?
So far, it sounds like they're really similar -- mood dysregulation, difficulty with time management and organization, difficulty making decisions, etc.
Anyway, thanks again! Will have to check out more of your posts now that I've stumbled across your site!
Carlos Briceño
Thank you for reading and reaching out. I hope our past and future columns help you in some way. Your questions are great questions. Jill and I have been trying to find experts in their field to help answer difficult questions like yours. I will continue to share what we learn, but I am not a doctor, so I don't know and cannot give advice on the medical side of HD.
We were so grateful for Dr. Lange and his advice, but the questions you have would be something you could discuss with your neurologist.
Lillya
3rd year medical student here, just getting off my psychiatry rotation. There are non-stimulant medications that can help for ADHD! Stimulants are considered first line and the other options may not be as effective but they can still provide some benefit! Talk to a psychiatrist about medications like Strattera, guanfacine, Qelbree, clonidine, etc. All meds have their own risks associated so you should discuss the safety profiles of those meds in conjunction with HD with your doctor but the point being there are options!
Good luck!