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International Parkinson and Movement Disorder Society
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Current and future approaches to treatment of dystonia

March 13, 2023
Series:Dystonia Special Series
In part 5 of the Dystonia Special Series, Dr. Francesca Morgante interviews Dr. Jill Ostrem about current and future approaches to treatment of dystonia.

[00:00:00] Dr. Francesca Morgante: Welcome to the MDS Podcast, the official podcast of the International Parkinson and Movement Disorder Society. My name is Francesca Morgante from St. George's University Hospital, London. And this is the special series on dystonia. It's my pleasure to introduce our guest, Dr. Jill Ostrem from UCSF. Welcome Jill.

View complete transcript

[00:00:27] Dr. Jill Ostrem: Thank you. It's a pleasure to be here.

[00:00:31] Dr. Francesca Morgante: Today's podcast is about current and future approaches to the treatment of dystonia. Jill, can you list for us, which are the main treatment for dystonia?

[00:00:44] Dr. Jill Ostrem: Absolutely. I mean, dystonia is a complicated movement disorder and there's many different types of dystonia that patients and children and adults can suffer from. But the main approaches are to use medications. Some oral medications [00:01:00] can be helpful for some forms of dystonia. We also have the ability to use neurotoxins.

Such as Botox that could be injected into some of the muscles that are overactive in dystonia, that can be very useful. We also have surgical treatments for more severe cases like deep brain stimulation, a therapy that uses a brain like pacemaker to stimulate specific areas in the brain that can really help the movements to be better.

And then there's augmented therapies like physical therapy and occupational therapy that can also really benefit patients.

[00:01:32] Dr. Francesca Morgante: When you mentioned the medication for dystonia, among this medication which are the most important to treat dystonia, especially in children.

[00:01:40] Dr. Jill Ostrem: Well, there is a rare form of dystonia known as dopa responsive dystonia, which can be really well treated if it can be identified with levodopa. So replacing dopamine, that's the main problem in the brain leading to the dystonia, but it's very rare. So most of the time that is not gonna help people [00:02:00] with dystonia or children.

Children also though, can respond quite well to anticholinergic medications like Artane. For some reason that really can be pretty well tolerated in children and even at high doses they can see quite a bit of benefit. Adults usually have more side effects from that medication and get less benefit.

[00:02:18] Dr. Francesca Morgante: So the first line medication for adults with dystonia is botulinum toxin. Can you describe the mechanism of action of botulinum toxin and how it is able to improve the symptom of dystonia?

[00:02:30] Dr. Jill Ostrem: Yes, it is first line therapy, I think for adults with certain types of dystonia. So if they have a focal dystonia, so not really dystonia affecting the whole body, but an area of the body that is afflicted by the dystonia. So the most common would be a cervical dystonia affecting the neck area and the botulinum toxin works by blocking the release of acetylcholine that comes from the nerve and leaves the nerve terminal and goes to the muscle and helps muscles to contract. So by introducing this [00:03:00] neurotoxin and the specific muscle that's overactive, the muscle becomes weaker, the activation of that muscle is reduced and it can relieve the spasm of dystonia and it works for about three months.

And then the nerve terminals kind of grow back and you need to then introduce that neurotoxin again to get the similar benefit

[00:03:19] Dr. Francesca Morgante: How are the muscles that we need to inject with botulinum toxin are selected? How do we make that choice in clinic? 

[00:03:27] Dr. Jill Ostrem: Yeah, that's a really good question. It's part of the art of medicine and a lot of thought is given to that. We look at patients positioning, the posture of the head the range of motion. Sometimes if there's an involuntary tremor associated with it that can influence muscle selection. And we have an appreciation for which muscles are contributing to the vector in which the head is overly active and being pulled.

And those are the muscles that we can then target. And of course, it's an art, like I said, so, sometimes we don't get it right the first time and we need to adjust the [00:04:00] muscles or the dose that we're putting into each muscle on future cycles of injections. So, over time though, usually you can achieve a pretty good improvement for most people with this approach.

[00:04:10] Dr. Francesca Morgante: Is there any room for having the injection guided by some techniques like electromyography of ultrasound?

[00:04:19] Dr. Jill Ostrem: Yeah, a lot of people now will do the injections with some kind of an assistive Approach where we're using EMG or electromyography like you said. And this allows us to hear the muscle activity through the same needle that the neurotoxin is being injected through.

And this can be very useful, cause you can confirm that you're in the correct muscle. You can hear when the muscle is very active and contracting, and that can help give you confirmation. You're in a very dystonic muscle. Also, you're right, ultrasound can also be very useful, especially for deeper muscles. Or maybe muscles that are harder to identify with EMG for some reason. You can also [00:05:00] use ultrasound, which is commonly available in some clinics now to help visualize exactly where you want to position the needle to get into the appropriate muscle.

[00:05:08] Dr. Francesca Morgante: The treatment with botulinum toxin has allowed to treat successfully many cases with focal dystonia, as you mentioned. But there are a group of patients that have more severe dystonia affecting several body parts. In this case, in children or adults with generalized dystonia or severe segmental dystonia, which is the treatment to consider.

[00:05:32] Dr. Jill Ostrem: Yes. That's right. So these are, again, rare conditions, but when they occur can be very challenging and very disabling. So generalized dysonia, like you said. This can occur in, children and progress into adulthood. Or more severe forms of segmental dystonia are often seen in adults. So we have surgical treatments that can help really get at maybe the main source of the problem in the brain. So directly influencing the motor circuit in the brain to help [00:06:00] relieve dystonia. So this involves placing an electrode into the brain in a therapy known as deep brain stimulation. The electrode is then connected to an extension wire that goes into the chest wall.

It connects to a battery or a neurostimulator that's under the skin. And then we can adjust the stimulation settings to help improve patient's symptoms. So it's a very tailored therapy. Sometimes it can take a little bit of time to find the best settings for someone to have the most benefit from this therapy.

But in the right hands we see really profound improvements in people with especially isolated dystonia who haven't had symptoms long enough to develop contractures of various areas of the body. If everything is still moving well with good range of motion and not a lot of secondary issues from having dystonia long time, we can see really good improvement in the outcomes of patients who are treated this way.

[00:06:52] Dr. Francesca Morgante: And thus the treatment with deep brain stimulation has a long-lasting effect?

[00:06:56] Dr. Jill Ostrem: Yes, it does seem to. We have only had this [00:07:00] therapy available for the last 20, maybe 30 years. But really, been mostly utilized, I'd say aggressively in the last 15 years. So our outcomes aren't really, very, very long. But, for the most part, yes, the therapy seems to help patients for at least as long as we've been using this kind of therapy and there can be some progression of the disease in some patients who still, deal with, some reemergence of symptoms despite having DBS. But, overall, we do think that the benefit of this therapy is pretty sustained.

[00:07:32] Dr. Francesca Morgante: You are one of the major experts in the field of deep brain stimulation for dystonia. What are in your opinion the best candidates for this therapy?

[00:07:43] Dr. Jill Ostrem: Yeah, that's such a good question. Definitely the young children who are developing primary or isolated generalized dystonia who have DYT1 known mutations who are having significant disability, especially gait issues, it is likely to progress. So we really would like to [00:08:00] identify these children early and treat them as soon as we can.

So that they can live out a more normal life with less disability and can really be a powerful therapy for them. It can be life changing. And then I would also say for adults who have isolated or segmental cervical dystonia who are not getting enough benefit from botulinum toxin injections for whatever reason, maybe they have a complex muscle pattern or they just don't tolerate the injections. It is a very powerful therapy for cervical dystonia as well. So people who do less well with DBS are patients who have a secondary form of dystonia, and there's some caveats to that. But in general, if there's been brain injury, we can still see meaningful benefit in dystonia itself.

But maybe the outcomes are not quite as dramatic as we see in our isolated or primary dystonia patients.

[00:08:52] Dr. Francesca Morgante: So how do you see the field of neuromodulation for dystonia in the future? Are there any development ahead of us [00:09:00] that might improve the therapy?

[00:09:01] Dr. Jill Ostrem: Yeah, so we have a pretty good way of helping people with the current use of deep brain stimulation. I'm hoping that we'll have greater awareness and there will be more people who will be referred to DBS centers and consider this kind of therapy, which can really be quite beneficial.

It's become much safer and outcomes are getting better and better. With that said, there are still some limitations about how in fact are we actually having this improvement in patients dystonia symptoms. What is the core mechanism behind how DBS works? And so we're starting to be able to answer some of those questions.

We have newer deep brain stimulation systems now commercially available that also will allow for us not only to stimulate the brain, but also to record some of the brain activity around the DBS electrode, and that's fascinating. We may be able to get to a point where we can use some of that information about electrically what's going on in this area of the brain to help modify [00:10:00] how we deliver the stimulation and it may result in even better outcomes, we hope.

[00:10:05] Dr. Francesca Morgante: Thanks Dr. Ostrem, for this extensive review on the treatment of dystonia and having provided to us also a look to the future. Thank you.

[00:10:15] Dr. Jill Ostrem: My pleasure. Thank you so much. 

Special thank you to:

Dr. Jill Ostrem
Medical Director and Division Chief, Neurologist, and Deep brain stimulation specialist
UCSF Movement Disorders and Neuromodulation Center

Dr. Francesca Morgante, MD, PhD 

St George's, University of London

London, England

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