Melanie Cole, MS (Host): Welcome to Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole, and we have three Northwestern Medicine Marianjoy Rehabilitation Hospital physicians with us today, highlighting the significance of muscle tone in young pediatric patients. Joining me is Dr. Larissa Pavone, she's a Pediatric Physiatrist and Program Director of the Physical Medicine and Rehabilitation Residency Program; Dr. Mary Keen, she's a Pediatric Physical Medicine and Rehabilitation Specialist; and Dr. Anton Dietzen, he's a Pediatric Physical Medicine and Rehabilitation Specialist. And they're all at Northwestern Medicine Marianjoy Rehabilitation Hospital.

Doctors, thank you so much for joining us today. And Dr. Keen, I'd like to start with you. Can you explain the significance of muscle tone in young pediatric patients and its impact on their development?

Dr. Mary Keen: Well, physicians are always concerned when there's a tone difference in a very young child. Either high tone or low tone can lead to abnormal development because it adversely affects development. And the underlying reason that affects development is that it usually is reflecting a problem with the brain. With high tone or low tone, the concern is that there's something impacting development. With low tone, it can be a brain problem or it could be a problem with the more peripheral nervous system. With high tone, it's almost always a brain problem. So, it's a warning sign. Any tone abnormality is a warning sign that there may be something wrong. And every physician is concerned to find out what that is and treat it if we can to alleviate any impact on development.

Melanie Cole, MS: Thank you so much, Dr. Keen. So Dr. Dietzen, let's first talk about high tone or hypertonia. For other providers, what are the primary causes of high muscle tone in young pediatric patients and how does it manifest? So, what would primary care or other rehabilitation specialists really be looking for in early signs?

Dr. Anton Dietzen: As Dr. Keen mentioned, with high tone, it's usually coming from either the brain or the spinal cord. And so, tone just in general exists on a spectrum. And so, no single child has the perfect amount of tone. There's a spectrum of what's normal, both from low tone to high tone. And so, you can have a child that has a little bit higher than typical tone and have it not necessarily be a problem. But typically, warning signs, I think, are things to kind of watch more closely. If a child has really significant reflux, sometimes that can be from tone. If they're having milestone delays, maybe they're not rolling over, or maybe they're rolling over too early, but it's a really abnormal-looking roll, like with arching of the back or cervical extension, and some of those things can kind of be clues that we maybe need to watch this child's tone more closely or get a really thorough history and make sure there's not anything that would make us consider some further diagnostic workup or imaging or something like that.

Dr. Larissa Pavone: I think one of the things that you mentioned and that we see early on in some infants is, I think, it's always a little disconcerting. Another thing you might see is when there's just overall stiffness. I feel like, on occasion, I'll have infants come in who the parents or the pediatrician or even sometimes a family member just feels like the baby's stiff. And that's always a little bit challenging.

Dr. Anton Dietzen: Yeah, that's a good point. And sometimes even something as simple as like it's more difficult than my other children to get them into the car seat because they're stiff.

Melanie Cole, MS: These are all great points. And Dr. Pavone, can you discuss some of the challenges of managing spasticity, dystonia, and rigidity in these young pediatric patients? As Dr. Dietzen was just saying, even putting them in a car seat can be challenging. Speak about some of the challenges in treatment and managing.

Dr. Larissa Pavone: Yeah, this can be very challenging. And I think Dr. Dietzen and Dr. Keen and I have all partnered together, sometimes on different cases when it's really challenging. I think the biggest to start is defining what type of high tone the child might have, or the hypertonia, because there's different types, and you listed them, spasticity, dystonia, rigidity. They all fall under that spectrum of hypertonia. But for each type of hypertonia, there's a little bit of variation in the medications that you would choose, or sometimes the medications are similar, but you might preferentially choose one first and try it. And I think some of the challenges that come from this is that even though we know a lot about the brain, and as we stated before, hypertonia often can come from something that's abnormal in the brain, we don't know everything, and don't fully understand the pathology that's causing these tone abnormalities. And so, we do have a handful of medications that we'll use to treat each one of these conditions, but it's a lot of trial and error unfortunately. There's a good amount of science behind it, but there's still a lot of unknown and a lot of trial and error.

Dr. Mary Keen: And at the same time, while we're trying medicines, we have to remember that sometimes hypertonia just resolves. Sometimes it will just go away, especially in the youngest children under two.

Dr. Anton Dietzen: Sometimes the first thing that we do is kind of look for potential triggers for high tone. Is there constipation? Is there excessive drooling that they're having a hard time managing? Is there some kind of discomfort? And then, I think as physiatrists, we always do a good job of trying to look at the child holistically and manage those potential triggers first. But then if the tone is interfering with their development, that's when we think about adding kind of other interventions on top of therapy or bracing or things like that, like medicines. But that's a really, sometimes a tough decision to make because a lot of the medicines Dr. Pavone mentioned, we have limited data on how they affect a child's long-term cognitive outcomes. There's some evidence that they might inhibit learning new motor skills and things like that. So, I think sometimes the hardest part is really trying to figure out when they're using the tone and when the tone is really kind of getting in the way of their development, and knowing that that's when we have to make some type of intervention.

Melanie Cole, MS: Dr. Keen, why don't you expand for just a minute. You mentioned that sometimes it can go away on its own, but I imagine parents get really scared about these kinds of things. What do you tell them when you're talking about pharmacologic and non-pharmacologic strategies and treatments that you would use at Marianjoy?

Dr. Mary Keen: Well, in the background, there is always a concern about what is causing the tone. And parents will be fairly persistent about it, and we always have the team looking at it. A neurologist would almost always be involved in the care of our patients. But for example, if a child is just one year old and have stiffness just in their legs, there is a possibility of something like cerebral palsy. When parents are persistent asking what's going on, I will explain that that's a possibility, but we need to wait and see if the tone goes away, because when there's tone just in the legs, most of the time it will just go away. If it doesn't, if it persists and, as Dr. Dietzen explained, if it starts to interfere with function, I always try to look at the medication that has the least side effects, but I will mention three or four medicines with the patient's parent and talk about each medication, the pros and cons, and then tell them which one I recommend we use first.

Dr. Larissa Pavone: I think for some of the infants, we're talking about some of the medications that we use and how we proceed cautiously with the medications. A lot of times, we'll use non-pharmacologic approaches at first if it's not impairing their development too much. So, I always say, physical therapy, occupational therapy, those are some of the first things that we might prescribe for an infant that we're noticing increased tone on. And those therapists are really great at helping with the development, helping work out the tone. And so, usually, I think all of us, probably our first step, if it's not too significant, would be to start with therapists or through early intervention.

Melanie Cole, MS: Dr. Dietzen, I'd like to discuss hypotonia now, or low tone. What are some of the common etiologies of low muscle tone in pediatric patients? How does it affect their motor development? And what are the key indicators, as you told us, for hypertonia? What should physiatrists, primary care, and other physical rehabilitation specialists be looking for during assessments?

Dr. Anton Dietzen: I think like Dr. Keen mentioned at the beginning, again, we're often looking, is there something kind of going on at the level of the brain or the level of the peripheral nervous system, sometimes even at the muscle dysfunction within the muscle itself. And again, kind of recognizing that tone occurs on a spectrum. Sometimes you have kids who are lower tone, but it's still not necessarily a problem and they're going to develop through it. But other times, if it's really on the more severe side of the spectrum or impacting them more negatively, then we want to be aggressive in looking for the cause. The low tone with regards to cerebral palsy is the least common type of cerebral palsy and often has a genetic underpinning. So, sometimes we'll lean on Neurology or Genetics to kind of help come to an answer there in terms of that.

I think, often, it's just a child whose motor milestones are delayed. Maybe they're rolling late or they're not rolling at all. And so, they develop some flatness in the head or some torticollis from kind of being stuck in one position. Again, like reflux sometimes from low tone can be an issue or when you're moving the child, they have difficulty controlling their head as sufficiently for their age.

Dr. Mary Keen: So, a child who is very low tone may have a hard time holding up their head. If they're lying on the floor, they may not lift their limbs as much as a child typically would. If you put them on their tummy, they might have a hard time lifting their head. But in general, a way to try to treat hypotonia is to put them in positions that gives them an opportunity and encourages them to use the muscle. For example, if they're low tone and have a hard time lifting their head, I typically would recommend parents put the kiddo on their tummy so the child has some practice lifting up their head and neck and pushing up on their arms.

Dr. Anton Dietzen: That's so important, because I think a lot of times those children are the hardest to work on tummy time with, because they really don't like it because it just feels really difficult for them and really challenging for them. And so parents, obviously, the worst sound in the world is listening to your child cry. And so, you feel like you're doing something wrong by, putting them in this challenging positioning, that your concern is making them uncomfortable. So, a lot of times I think it's just kind of giving permission to put them on their tummy and that it's okay, or maybe educating on doing it over a towel roll or on an incline or on your chest to make it a little easier for the child to participate and a little more enjoyable for them.

Dr. Mary Keen: Absolutely, you're right, Dr. Dietzen.

Dr. Larissa Pavone: A little baby personal training.

Dr. Anton Dietzen: Yes.

Melanie Cole, MS: Baby personal training. I love it. Now, Dr. Pavone, speak about some of the best practices for treating this and how they differ from the treatments of hypertonia, because as we're talking about both of these, kind of contrast and compare those treatments.

Dr. Larissa Pavone: So, hypertonia is challenging because there are limited treatments for it, and I think we've all had this challenge when a parent comes in and really wants to do everything they possibly can for the child. So, the first thing to do is to either through genetics or partnering with our neurology colleagues, if there's a significant hypotonia present, is looking if there's something diagnostically contributing to the hypotonia, just to see if there's any medications or interventions based on a disease process that could be treated. But I would say a majority of the time with hypotonia, there isn't a medicine that is going to help. And it's really, I always say, good old-fashioned therapy, lots of physical therapy, occupational therapy, speech therapy if needed, to really work on strengthening. Sometimes we will use different bracing or orthotic options to help progress the child along. I know I'm definitely missing some treatments, so I'll see if Dr. Keen or Dr. Dietzen have something else to add.

Dr. Mary Keen: One of my favorite things for hypotonia is pool therapy. Marianjoy has a beautiful pool. And it's a great place for a very little child and even older children who have weakness or low tone, because the water provides buoyancy and helps them hold themselves up. And at the same time, the water provides gentle resistance for strengthening. So, it's one of my favorite things.

Melanie Cole, MS: Dr. Keen, how do you approach the interdisciplinary management of hypertonia and hypotonia in these young pediatric patients? I'd like you to use this time now to tell us about your team. You mentioned neurologists involved and we've got PT and OT. Talk about your team and all of the people involved for these young pediatric patients.

Dr. Mary Keen: The best thing about PMNR is that we typically work as a team, and we have expert pediatric physical therapists, expert pediatric occupational therapists, and expert speech therapists who are dedicated to helping children grow and develop. Our whole team is passionate about kids and their families, and I think that's what it takes to have a really good team. We have a team that's passionate about working together for the benefit of the child and the family.

Dr. Larissa Pavone: I also want to bring up, I think, one important team member that we haven't mentioned yet, but is oftentimes the captain, the quarterback, is the pediatrician. The pediatrician, the child's general pediatrician plays such an essential role in screening for muscle tone and really guiding parents through the process and kind of deciding next steps for the child and being the home base. So, a partnership with the child's pediatrician is essential as well.

Melanie Cole, MS: That's a great point, Dr. Pavone. Dr. Dietzen, I'm going to give you the last word today. In what unique ways is Marianjoy able to treat children with abnormal muscle tone? I'd like you to just summarize this very important discussion that we're having today.

Dr. Anton Dietzen: Well, I think, Dr. Keen kind of mentioned our pediatric department's vision statement is passion for kids, compassion for families. And so, I think, we have just an incredible group of people here who are all passionate about taking care of families and children with special needs and willing to kind of do the extra work to do it well. And so, I think, that's really the key, whether it's excessive muscle tone or not enough muscle tone. Ultimately, the foundation is just being followed really closely, working really closely with your therapist, having good communication between your PCP, your physiatrist, and your therapist to kind of know when it's time to intervene, whether that's with bracing or with medications or with further diagnostics and being able to have that conversation with families in a way that's sensitive to the fact that that's a really big next step and big decision and comes with a lot of different associations for every family.

Dr. Mary Keen: And brainstorming together. Brainstorming together can be so useful.

Dr. Anton Dietzen: Yes, the three of us definitely do that a lot, which is wonderful.

Dr. Larissa Pavone: I also think an important thing to think about, because as we mentioned earlier on, there are a lot of things that are still unknown when we're dealing with hypertonia, hypotonia, and tone abnormalities. So, always thinking about research opportunities for the children as well. And so, all three of us stay abreast on those research activities that are going on, and try and partner to connect families, if there is an appropriate research study going on, connect them with the appropriate resource.

Melanie Cole, MS: Thank you all so much for this enlightening and lively conversation today. You're all so great to have as guests. And thank you for sharing your incredible expertise with us today. And to refer your patient or for more information, please visit our website at breakthroughsforphysicians.nm.org/rehabilitation to get connected with one of our providers.

That concludes this episode of Better Edge, a Northwestern Medicine podcast for physicians. I'm Melanie Cole. Thanks so much for joining us today.