Hi everyone, thanks for joining us. I'm Mary Dubon, Peds Rehab Doc here in Boston and Vice Chair of Education for our Peds Rehab Medicine community through AAPMNR. And through AAPMNR, we're doing our web-based lecture series for Pediatric Rehabilitation Medicine. And so we're very excited today to have Dr. Kelly Pham here with us, who's gonna be going through Tone Management Jeopardy with us, which will be a fun, interactive game here, but also a really great review for everybody too with some good pearls in here. So how we're going to do it today for those of you who are on live is I don't want anyone to feel stressed about it at all. It's totally like a low pressure game, but what we're gonna do just to make it fun is to kind of, I have everyone's name up, who's the attendees, and so I'll call on someone and then Dr. Pham will put up the board, the Jeopardy board for you guys, and you guys can pick whatever the category is and however much money. It's not real money, unfortunately, I'm sorry to say, but this Jeopardy board here, and so you can pick and then she'll pull up the question and you can unmute yourself to answer. So I'll have everyone unmute, like each person unmute themselves when I call on them. And then if you are not sure and you wanna phone a friend, then anyone can unmute themselves and kind of jump in or put in the chat to answer. And if people all don't know, then that's why we're here to learn anyway, right? So it should all work out. This is being recorded so people can listen and learn from it later on too. And then if you guys want to claim CME credits, you just log into AAPMNR later through MyEducation. And I know Megan has sent those details out through FizzForum, but if there's any questions, you can always message me or her directly as well. So without further ado, thank you so much for Dr. Pham from Seattle Children's Hospital for joining us today to talk about Tone Management. So I'll pass it along to her to say hi and then we'll start the game. Thanks, Mary. Hi everyone. So as Mary said, I'm a Peace Rehab Doc at Seattle Children's. I'm also the co-director of our Surgical Tone Management program. And so Tone Management is my passion. And so I'm hoping to make this a fun, interactive lecture because I think that's a better way to learn and to be engaged in learning. And the more engaged you are in learning, I think the more likely you are to remember things. So don't hesitate on just putting a guess out there. If it's not right, no one will judge you. It doesn't matter. This is a very friendly game of Jumper Game. So we're just gonna start right off. So if Mary, you wanna pick someone to start, that would be excellent. All right, I need to unmute myself there. FYI, Megan, I just sent you a message. It looks like some people are having a hard time logging on. So if you're able to somehow- I did it. I took, I did it. Yeah, I apologize to interrupt, but I know three people who are unable to log on right now. Oh no. I just, I approved some people, so they should be joining us, I hope. Okay, perfect. I think, yeah, if that's not working or something like that, if you can share like the ID and password or something like that for people to manually put it in, that would be helpful as well. So sorry for a little technical interruption there. But yeah, so why don't we start with Daniel? So if you wanna unmute yourself and then you can pick your category and give it a try. And if you can't unmute for some reason and you wanna go in the text in the chat box, you can do it that way too. No worries that you can't unmute. Do you wanna just type into the chat box? Yep, okay. Baclofen Pump 100. Baclofen Pumps for 100. Excellent, Daniel. Oh, and this is even a double jeopardy. Hooray. So, okay. This prevents air from entering the system during a pump refill. And this is a very, very important part of your pump refill. You never wanna miss this step and it happens multiple times throughout your refill. Any thoughts from the group? And I wonder if people wanna just unmute themselves since we don't have a huge group here. Do people, if you can, wanna just unmute and... Like the, I don't know what it's called, the filter thing, the little disc filter. Close, close, close, close. Anyone else have any thoughts? Anyone, anyone, anyone? Okay, wait, someone put something in the chat. Hold on. Oh, okay. Being sure to empty air from syringe prior to tapping the pump. Okay, okay. That's another good thought. Any other thoughts? The type of clamp. Ah, okay, we're getting closer. Keeping the needle in for both, keeping the same needle in for both extraction of the backfill and refill. That is definitely helpful. Okay, so good. We have a couple of ideas. This is great. The answer is, the best answer is the extension tubing and the clamping of the extension tubing during the procedure is extremely important. Extremely important. So if you don't clamp the extension tubing when you're taking your syringe on and off, you have a higher likelihood of introducing air into the pump. The reason that's not good is because if you have enough air in the pump, that actually locks up the mechanism of the pump and it stops working. So you don't want that to happen. So clamping is very, very important. Oops, sorry. Oh, hold on. I'm gonna just go back. Don't look. Sorry, the mouse is a little bit funny on this Zoom. Pay no attention to the man behind the curtain, you guys. Exactly. Okay. Megan, just so you know, if you can actually type in there what the username and password is, I think a lot of people are still having a lot of trouble getting on. And so if you guys look in the picture, this is the extension tubing here. This little guy, the filter, it's actually, it's kind of interesting. It's a little bit more historical that we use the filter. The filter is actually meant for back in the day when we used to use those little ampules that had baclofen and you had to draw it up. And it's theoretically to block any glass from getting through into the reservoir of the pump. We still use them. I think the likelihood of actually getting glass into the pump during a refill is probably so low at this point that I don't know how necessary that is, but I don't know that anyone's willing to not do it. Any questions about that one? Feel free to unmute or put things in the chat. If not, whoever said that about the clamp, the type of clamp, you get to choose the next one. Peter. Peter, what would you like to do next? Peter would like to go baclofen pump for 200. Excellent, you're on a baclofen pump train. So when changing the concentration of the drug during a refill, this programming step is necessary to ensure that the patient gets the proper dose of medication. So you're changing the concentration. What do you need to do when you program the pump? So Laura says catheter bolus. Any other ideas? Priming bolus. Priming bolus, okay, any other ideas? Okay, so this is actually a bridge bolus. So a priming bolus is the bolus that fills up the catheter when the catheter is in the pump. So it's a bridge bolus. So it's a bridge bolus that fills up the catheter when the catheter is empty, right? So that is one type of bolus that we use. When you're changing the concentration, you actually use what is called a bridge bolus. And what that does is it actually bridges the two concentrations. So when you're changing the concentration, you have one concentration in the reservoir of the pump that you've just filled, and you have one concentration, a different concentration in the catheter. So the bridge bolus basically tells the pump there are two different concentrations of medication, where they are, and so that it actually gives the medication at a different volume per amount of time for the medication that's in the catheter compared to the medication that is in the pump. And so that's called a bridge bolus. Okay, next, anyone, anyone, anyone, anyone? Why don't we have Emily, because I know Emily had commented on something earlier before. Please, Emily, what would you like to do next? Let's say terminology for 100. Ooh, terminology, I love terminology. Okay, perfect. This is defined as passive, or as resistance to passive range of motion. This is one of my favorite things to teach residents and fellows. What do you think? Hypertonia. Ah, anyone else have thoughts? Anyone? Anything in the chat? Nothing in the chat. Can I give a clue? Because I've worked through this. Please. Yeah, Emily, you were close, but look closely as to, does she comment on if the resistance is high or low in this? There you go. So what is tone? So I feel like as providers, we talk about tone a lot. Oh my gosh, the patient has tone. There's so much tone. But what does that mean? Tone really doesn't describe very much. You have to quantify it. You have to qualify it. Is it hypertonicity? Is it hypotonicity? Tell me more about it. Just saying they've got tone means nothing. I have tone. You have tone. We all have some amount of tone. So Emily, since you mentioned hypertonia, can you further explain what hypertonicity is and what spasticity is? Yes, which is what I thought you were getting at. So I went in confident. You did. But you know it, which is good. So spasticity is dependent on velocity. So it's a velocity-dependent resistance. So with more, with higher velocity, we have more resistance. Exactly. And so you're exactly right. Spasticity is velocity-dependent tone or velocity-dependent resistance to passive range of motion, which is the definition of tone. So hypertonicity just means increased resistance to passive range of motion. And it includes spasticity, but it also includes other things, which is important to consider. Okay, next. Who would like to go next? All right, I'm gonna find someone. Hold on. Okay. The name. Perfect. Maximal Medications 400. There you go, Becky. Thank you. Enteral Medications? Was that Enteral Medications, Becky? I'm sorry. No, Injectable. Oh, Injectable. Thank you for clarifying. Okay. Injectable Medications for 400. So this medication has less protein and so may work better in children who have developed antibodies to onobotulinum toxin A. So this is 400. A little bit of a tougher question. Any ideas, anyone? Mary? What does onobotulinum toxin B? Or is it Dysport or Xeomin? Excellent. So Xeomin, which is incobotulinum toxin. So some people will move to Xeomin if they find that a child is no longer responding, or they have developed antibodies to onobotulinum toxin. So there are a couple of different reasons why we use different botulinum toxins. As a provider, clinically, I really just use onobotulinum toxin and my use of onobotulinum toxin in the last five years has reduced significantly with increasing research on repeated use of Botox, especially in children who have CP. Some people feel like Xeomin is more effective once you see onobotulinum toxin starting to wear off. But it's not something that I clinically use in my practice. Does anyone here use it? Or do they have more experience with it? I'll take that as a no. Okay. Jeremy, excellent job. What would you like to choose next? Let's go Enteral Medications for $500. Ooh, he's going for it. Thank you, Jeremy. This is awesome. Okay, this medication is a jack of all trades as it can be used to treat seizures, pain, headaches, and even dystonia. Oh, we have a taker. So, Nicholas says, what is gabapentin? Excellent, Nicholas. Beautiful. Yes, what is gabapentin? So, there's not a ton of research on gabapentin and its use in dystonia, but I will say that there is some, and I have a handful of patients who I have started on gabapentin for treatment of dystonia, and it is very effective. Oftentimes, I think it's more limiting than some of the other medications that we use, like baclofen and artane for dystonia, because I think it causes a little more sedation in many kids, and it can cause urinary retention, which is sort of interesting, but something to watch out for. But, yeah, it can be a really great medication. I find that it's most effective for kids whose families complain that they have more sensitivity. I hear it a lot more in the lower extremities, like when we're trying to put their braces on. They're just very, very sensitive. They get very irritable, very fussy when we're trying to put their braces on, and they just don't want you touching their legs. Those are the kids who I've had a lot of good success with gabapentin in treating their dystonia. So, really nice. All right, Nicholas, you're up. What would you like to do next? I also would like to point out, I appreciate that Nicholas answered in the form of a question, so bonus points to Nicholas. Extra points, Nicholas. Good job. All right, he said rhizotomy for 500. Oh, he just goes for it. Okay, Nicholas. This surgery can be used for the treatment of dystonia. Any thoughts from anyone? Anything in the chat, Mary? No, my husband is sitting here trying to figure out the answer. He's not in medicine. Oh, is he? But deep brain stimulation. That's an excellent thought. Sorry, was there another one? No, we have two folks that said deep brain stimulation and then we had another... Nicholas says anterior or ventral rhizotomy. That was Nicholas who said that? That was Nicholas again, yeah. Nicholas, okay. So, Nicholas must be at a center where they do this. So, ventral rhizotomy or ventral dorsal rhizotomy. And so, this is a surgery that's similar to selective dorsal rhizotomy. It's where the neurosurgeon goes in and they isolate, in this case, either the ventral roots or both the ventral and the dorsal roots, and they cut a portion of them. There is very little research to support ventral rhizotomy or ventral dorsal rhizotomy. I think it was the AAPMNR Journal Club a month or two ago, I actually presented one of the studies of children with CP who were GMFCS levels 3 and 4 who had ventral dorsal rhizotomies, which is kind of crazy, and I don't think anyone in this country is doing that surgery on kids who are ambulatory. This surgery is typically done for children who are typically GMFCS level 5, some children who are GMFCS level 4 who maybe are just power wheelchair users, but who don't necessarily have any ability to ambulate or bear weight through their legs. Because when you're cutting the ventral roots, you have a significant decrease in motor output, of course, which helps to treat dystonia, but it also results in you being unable or less able to use your muscles in your legs. Any questions about that? I know it's not something that's done at a lot of centers around the country. What centers do you know of that do do it? So we do it at Seattle Children's. Gillette actually does a lot of them. There's a neurosurgeon down in L.A. who I think is not associated with an academic program who does them. Indiana, I know one of the neurosurgeons there who's doing them. I think that T.S. Park at Wash U does some of them. So there are certainly people across the country who are doing them. It's not nearly as common, of course, as the selective dorsal rhizotomy, but as someone who does a lot of surgical tone management, I'm really excited about this surgery because I think that it has a possibility for some patients of replacing the need for a baclofen pump. And as many of you may know, baclofen pumps are very high risk. They have a very high risk of complications, and they require a lot of management. And for us in Seattle, we have a huge catchment area. As Mary knows, we serve Washington, Wyoming, Alaska, Montana, Idaho. And so if we have a kid who's in rural Alaska, I can't give them a baclofen pump because there's no one to manage the pump. If something happens to the pump, they're going to go through withdrawal or overdose, and there's nothing you can do because they're in rural Alaska where they don't have running water. So I think that it started out as this surgery that was really more of a last-ditch effort, like there's nothing else we can do for you, let's try this. But it's becoming more common, and it's becoming a little more mainstream. I'm hopeful that we'll see some research coming out of Gillette in the coming years because they've done quite a few of them and seen very good results. Thanks, Kelly. And there was a question in the chat here, too. Is it only affecting lower extremity dystonia? That's an excellent question. So theoretically, it should only be treating lower extremity dystonia because those are the nerves that are being cut, right? They're lumbosacral nerves that are being cut. But what they have noticed, what we have noticed around the country of providers who are doing this, is that dystonia reduces in the rest of the body as well. And so I think that stems from having sort of less input from the lower extremities, less dystonia in the lower extremities, sort of leading to more generalized dystonia. But most institutions have seen great improvement not only in the upper extremities, but trunk, head, and neck as well with this surgery. There is such a thing as a cervical ventral rhizotomy. There are people who are doing those. There's a neurosurgeon in Indiana that I've talked to who is doing them. It's certainly not as common, and I think the risks are, of course, much higher. They go below C5, of course, to preserve the diaphragm. But I think that just from a bony standpoint, you're more likely to have complications in the cervical spine. Any other questions on that one? Okay. Nicholas, I think you got that one. What would you like to do next, Nicholas? All right. Rhizotomy for 400. This explains the presence of spasticity in the ankle plantar flexors one year after SDR. Any thoughts from anyone? There are a couple different reasons for this. One is probably the more common reason that you would think of, and one, I think, is a little less common. Anyone, anyone, anyone? I think we're missing the Jeopardy music. You know what? I actually took it out because I thought it was really obnoxious. Yeah, no, it probably is. I think everyone's probably thanking us for not having it there. Yeah. Nothing in the chat so far, so. All right, well, let's move on for time's sake. So oftentimes with selective dorsal rhizotomy, the neurosurgeon will be more conservative when it comes to the sacral roots, right? So S1 and S2, they tend to cut a decreased percentage of those nerves and a decreased number of those nerves, if that makes sense. So when they cut a nerve, they don't just cut the entire thing. They usually cut a certain percentage of each nerve. So once they get to the sacral roots, because you worry about bowel and bladder control, they cut fewer nerves and a lower percentage of the nerves that are cut. So there's a higher likelihood of kids having sort of remnant spasticity in their plantar flexors. So another reason, though, might be because we want to keep that. And a lot of orthopedic surgeons will say, hey, leave a little bit of spasticity in the gastrocs, please. Because if you get rid of all of the spasticity, then some of those kids are using it to stand upright. And so for kids who are maybe a little bit lower functioning, a little bit weaker, as they get bigger, their bodies get taller, they weigh more, and you need more strength to keep your body upright. So if they don't have that spasticity in their plantar flexors, they're maybe more likely to crouch. And so some people in the CP world and in the tone management and SDR world would say, hey, leave a little bit of that spasticity in the plantar flexors just to keep them upright as they get older. So that's another reason why. Any questions on that? I did not. OK. Great. I'm just thinking. Let's go with Elizabeth next. You can either unmute yourself or you can put it in the chat. Oh, there we go. Entral Meds for 100. OK. Entral Meds for 100. This medication prevents the release of calcium from the sarcoplasmic reticulum. Anyone? Please feel free. Sorry, I didn't hear you. What was it? What is Dantrolene? Thank you. Is that you, Jeremy? That is correct. Awesome, Jeremy. What is Dantrolene? So Dantrolene works at the level of the muscle, which is one of the reasons we like it. So theoretically, it is less sedating. But you know what one of the side effects is still? Sedation. So though we like it for that, it's not perfect. It's still sedating. One of the reasons we tend to not use it as first line is because of the risk of hepatotoxicity. It requires monitoring of LFTs over time. That being said, I have seen Dantrolene be extremely effective. And Mary, you might remember this. One of your patients who had baclofen pump explant at about 700 mics a day. Pump had to be explanted because of spine fusion, infection, pump infection. Everything was infected. Pump came out at about 700 mics a day. Dantrolene was one of the medications that I think was extremely helpful in preventing withdrawal and treating this child's spasticity and dystonia. And so Dantrolene is a really great medication to keep in our back pocket. All right, Jeremy, what do you think next? Let's stick with enteral medications for 200. Awesome. Okay, common side effects of this medication include sedation, constipation, weakness, and dry mouth. And oftentimes, we like this side effect of dry mouth. What is baclofen? We have in the chat, we do have Maya is the first one to say, what is artane? Yes, artane. So, trihexyphenidyl or artane. So, a lot of those side effects, sedation, constipation, every single medication that we give for tone management is going to cause those things. As an anticholinergic, artane also has the risk of causing dry mouth and of causing urinary retention. The urinary retention risk is pretty low, but it does happen, so we always let families know. Dry mouth is certainly one to think about, and it can be really, really helpful for a lot of our kids who have salaria. All right. Maya, what would you like to choose next? Enteral meds for 300. Okay, excellent. This medication is an alpha agonist and is used to treat spasticity. I would say this is a less commonly used medication. Wow, we have like several answers, but tizanidine is the first one, and we also have a clonidine in there, too. Awesome. You guys got both of them. So, tizanidine and clonidine are the two alpha agonists that we sometimes use. And I would say, again, these are more second-line agents. Tizanidine I tend to use more in kids who are older, kids who have spinal cord injury, who have primarily spasticity. It can be extremely sedating, so even in older kids, just be cautious with that. And then clonidine is a really helpful medication. We use it oftentimes in kids who have paroxysmal sympathetic hyperactivity after an acute brain injury. It's helpful not only for hypertension, but also for the posturing dystonia spasticity that comes along with that. But we also use it in kids who have baseline hypertonicity. And it also can be sedating. It's great because you can use it as a patch or an enteral medication. It's also really helpful for sleep. And so a lot of people will use it just at nighttime as well. So lots of different options for using clonidine. All right, so whoever answered tizanidine first, would you like to choose the next one? I was going to say it's Nicholas, but I want to give Laura a chance because Laura was the first to say clonidine. Laura. Yep. All right, let's finish out enteral meds for four. Okay, awesome. All right. Ooh, double jeopardy again. So the typical starting dose of this medication is 0.025 mgs per kick per dose every six to eight hours. If you ever do consults in the hospital, this is a very important thing to know. Anything in the chat, Mary? We have Maya with what is diazepam? Excellent. What is diazepam? Diazepam is a really commonly used medication for hypertonicity. I tend to use this more in kids who are really little, so two years old and younger. There's very little research on the safety and efficacy of baclofen in kids who are less than two to two and a half. That's where we tend to use diazepam. Diazepam is also very easy to get in a liquid form. When kids are really little, we put them on teeny tiny doses so it's easier to dose. We also use diazepam as needed medication for kids who are in the hospital. For kids who are here for acute tone management issues or they had a fracture and their dystonia is higher, diazepam is often a good go-to medication for PRN. Next. It looks like Maya was the first one to answer what is diazepam, so why don't we give Maya another shot? All right, Maya. Injectables for 100. Injectables for 100. This medication binds to the SNAP-25 protein of the SNARE complex, preventing the release of acetylcholine into the neuromuscular junction. Maya says, what is botulinum toxin? Botulinum toxin. Which one? Maya says A and Becky says Botox. Anything else? We got a bunch of A's. A bunch of A's. And I only have a B. Good, okay. So hopefully this is a known picture for you. So the SNAP-25, you can see right here, this is part of the SNARE complex, which includes all of these things. Basically this complex helps the synaptic vesicle to bind to the synaptic cleft and to release acetylcholine into that neuromuscular junction. And so SNAP-25 is on a botulinum toxin A. You could also say C or E. Those would be correct answers as well. I think common things being common, a lot of people use on a botulinum toxin A. All right, so whoever said A, you get to choose what's next. It looks like Maya's on a roll, but Anton actually also said A, so I want to give Anton a shot. Anton, what do you think, Anton? Okay, injectables for 200. Injectables for 200. This injection cannot be done with ultrasound guidance alone. This is a little bit of a tricky question. So Maya says, what is phenol, as does Anton and Emily and Becky. Oh, okay, awesome. Okay, so let's go with that. So it's actually a motor end plate phenol injection, specifically. So if you are doing chemoneuralysis of a motor end plate, so if you are doing chemoneuralysis of the nerve directly, so if you're doing something like obturator nerve, for example, you are right at the nerve. You could theoretically use just ultrasound for that. When you are doing a motor end plate injection, you're looking for where the nerve inserts on the muscle, you have to have stim. You have to have stim because you need to know that you are at the level of the nerve and that you're not just stimming the muscle. And you can't see the motor end plate with ultrasound. So that is the one injection that you really need to have stim and you can't do ultrasound alone. And this is just a picture of that. So usually we have our syringe full of phenol. We have extension tubing because it's a very, for those of you who have done it, it's a very finicky thing to find the motor end plate and find the nerve. So it's nice to have just a little more flexibility with just using a needle when you're doing this injection. And then there's this beautiful old school stim machine that we use to stimulate the nerve. Okay. So Laura was the first one to say phenol, so why don't we go with Laura? All right, Laura. All right, let's go back to pumps for three. Pumps for 300, excellent. Okay. This volume is not included in the post dye study priming bolus. So when you do a dye study, you do a priming bolus, but this volume is not included in that. All right, we have, Elizabeth says, the pump tubing. Excellent. So this is a lovely picture from our new tablets. And so you see the reservoir volume here in blue. And then you have the internal tubing. So that's the tubing that is within the pump. And then you have the catheter access port chamber here. And then you have your catheter that is external. And so when you do a catheter access port study, you access it here. You draw back everything in the catheter. So this part still has drug in it. So the internal tubing does not need to be used in that calculation. And in the old school, like 1980s blue things that we used to talk to the pumps, you had to look at all of the calculations and the numbers and all of that and make sure it was the right volume of the tubing in order to fill the catheter. And now they have this really beautiful picture that just shows you where the drug is based on what you have done. And so that internal pump tubing still has drug. All right, that was, was that Laura? That was Elizabeth. Oh, it was Elizabeth. Thank you. Elizabeth, what would you like to choose? Backup and pumps for 500. Oh, excellent. Okay. Okay, this is the minimum programmable flow rate in a day. And this is a challenging one. So if no one gets this one, do not feel bad. Oh, but someone answered. Three micrograms a day and then we have a 96. Okay, so it's actually 0.048 milliliters per day. So it depends on the concentration of the drug as to the actual amount of drug. But the volume of drug at its minimum is 0.048 milliliters. Clinically, I don't think that's a super important number. Basically, if you want to go as low as you possibly can on a pump, you put it on minimally infusing. And you have to know that minimally infusing at 2,000 mics per ml is very different from minimally infusing at 500 mics per ml. Those are going to be pretty different doses just because of the volume that the pump can put out at its slowest. Okay, next. Anyone? Sorry, I was totally muted. I'm just going down the list to see who hasn't gone yet. Connie hasn't gone yet. Connie, do you want to try? Sure. Injectable medications for 300, please. All right. Side effects of this medication include cardiac arrhythmia. And there's one study from the 1990s by Dennis Matthews from Colorado in which they looked at the dosage of this medication at which you start to see cardiac arrhythmias. And they basically just escalated the dose and escalated the dose and then said, okay, we're seeing arrhythmias now. This is it. Don't go higher than that. And that's really all we have to go on is that one study. So Elizabeth says phenol. That is correct. Phenol. So you want to be careful not to inject it intravascularly, and you still want to be conscious of not going over the max dose for phenol. Excellent. Looks like Elizabeth is on a roll, so do you want to pick the next one, too? We'll go for injectable medications for 500. Excellent. Okay, so this is the follow-up to that question. In a 30-kilogram child, this is a safe maximum dose of 5% phenol to inject. This one takes a little bit of calculating. Okay, are people calculating or are people like, I have no idea? Just tell us the answer. I'm getting blanks, so I feel like I'm not sure what that would be. Okay. And so this is important when you're thinking about preventing cardiac arrhythmia, right? You want to know what that max dose is. So the answer is 18 mL, and this is the calculation. So 30 mg per kg is approximately your maximum dose. And, again, this comes from that study from the 1990s from Dennis Matthews that has never been recreated, and it's the only thing we have to go by as to the maximum dose of phenol, at which point you run the risk of having cardiac arrhythmias. So 5% phenol is 30 mg, I'm sorry, 50 mg per mL. And so when you do the calculations, that's 900 mg, which for 50 mg per mL is 18 mL. So when you're thinking about going to do phenol for a child, a child who's 30 kilos, you don't want to go above 18 mL. It's a pretty safe dose to avoid arrhythmia. All right. Next. I am just looking down the list. Mary Lynch, do you want to try? Let's do terminology 500. Oh, excellent. Okay. Oh, I love this one. This is the close association of an unwanted movement with an intended movement. We have a synergy. Oh, okay. Anyone else? Synergy, I think, could be an answer. I think the better answer is what we would call overflow, and there's a lot of discrepancy about what overflow is and what does that mean. Some people say that overflow is dystonia. Some people say that it's overflow. I think we sort of don't really know, and sometimes it's hard to tell whether it's overflow or dystonia in a child who has dystonia or in a child who doesn't have it. I think terminology can be really important, but I think that once you start getting into the terminology, it really just shows that there are things that we don't yet know or understand, especially as it pertains to movement disorder. Great. So... How about, I don't know if I'm saying it right, Jiao-Fang? You can type into the box or unmute. Okay, we'll go back to the top of the list, but let me try Amy. All right, Amy says back lip and pump for 400. Excellent. Okay. These are both ways to determine if a back lip and pump has flipped in the pocket. And I remember the first time I saw this, and it was one of those like, oh my gosh, that is what it looks like. And it was really kind of exciting. So when you get to see it in real life, it's not great for your patient, of course, but kind of exciting as a provider when you see it the first time. We have a lot of action on this one. So we have ultrasound and x-ray. What do you see on ultrasound and x-ray, though? What are you looking for? We also have palpation, and we have follow your finger along the side to determine if the catheter is going in the correct direction. You don't see the reservoir when you're doing ultrasound is what Elizabeth says. Great. Okay, lots of good answers. I love this. So I will preface this with, we do not do ultrasound here, and so we do not have an ultrasound machine unless I were to say to radiology, hey, can we use your ultrasound machine and take a look at this or have them take a look at it? And so I think for a lot of people, it's much more accessible to use x-ray because it's easy. So one of the things that you can see is the connector of the catheter. I think this was what someone was mentioning about sort of feeling that. You can feel it in some patients who are thinner. In other patients, you might not be able to feel that. So the picture on the top here, this is normal. So this connector for the catheter comes off going clockwise. And so if the pump is flipped, you will see it coming off going counterclockwise. So you'll see it pointing in that direction. And so that's one sign that your pump is flipped. The other thing you can do is verify on a lateral view where the reservoir is. So this is abnormal. So this is the patient's belly, and you can see the reservoir is right here. It's a little sort of accordion thing, and all of the mechanics of the pump are on the inside right now. And the mechanics of the pump should be superficial. So this is another indication that the pump is flipped. You can also sort of see that the connector here is maybe pointing in the opposite direction. So great. I'm glad people knew about that. That's awesome. Okay. So who's up next? Let's see. I'm just looking. Let's go with Emily again because, you know, she said ultrasound and x-ray. Perfect. Let's do terminology for 200. Okay. Terminology for 200. This involves involuntary sustained or intermittent muscle contractions that cause twisting and repetitive movements, abnormal postures, or both. And this is one of my favorite definitions, and most of the definitions in this section come from Peter Sanger, who's a movement disorder neurologist in LA. He has some really amazing articles on movement disorder, on hypertonicity. I would definitely recommend checking them out. We got lots of dystonias in the comments here. All right. So what is dystonia? So follow-up to that, is dystonia considered hypertonicity? Why don't we give that to Emily since Emily had the other hypertonicity and was the first one to answer this too. Perfect. I feel like I normally consider it, yeah, I feel like it's high tone that causes the twisting and posturing. It is considered hypertonicity, right? By definition, hypertonicity is basically an increase in tone. So an increase in resistance to passive range of motion. So spasticity fits in that, dystonia fits in that, rigidity fits in that. Those are all types of hypertonicity. The difference is that dystonia is also a type of movement disorder. Spasticity is not, right? So that's where dystonia fits into two different categories. But you're exactly right. All right. Why don't we give Emily that next one too. All right. Terminology 300. Excellent. Okay. This is a slow continuous involuntary writhing movement that prevents the maintenance of stable posture. Okay. Amy says apoptosis. Very nice. What is apoptosis? And this is one of those things that if you Google it, you will find nothing on the internet, no good videos on YouTube. But when you see it in person, it's another one of those like, oh my gosh, there it is. It's apoptosis. And it's pretty impressive when you see it for the first time. So great job. All right. Let's give the next one to Amy then. Amy says terminology for 400. Excellent. Okay. This describes a sequence of repeated, often non-rhythmic, brief shock-like jerks due to sudden involuntary contraction or relaxation of one or more muscles. Got a couple myoclonus. Very good. Excellent. So yes, that's myoclonus. And I think one of the big things that I wanted to, the reason why I wanted to have this be myoclonus is sort of to trick you a little bit, because it sounds very much like the definition of chorea. But it's not. And they're different. And so chorea is actually more complex. It's slower. Whereas myoclonus is more rhythmic. It's more repeated. It's more repetitive. And so that's one of the biggest differences that you'll see between myoclonus and chorea. But I think that they're sometimes a little bit challenging to differentiate. All right. Great. Emily, let's go back to you then. Rhizotomy 100. Excellent. Easiest question. These are the most common functional levels for which SDR is successful. All right, Becky says GMFCS level 1 to 3 or maybe 1 to 2, and then we also have an L2 to S1. The answer is actually GMFCS levels 2 and 3. We do SDR in patients who are level 1. But interestingly, the gains that you see in a child who's a GMFCS level 1 are actually less because they're so high-functioning to start. You typically see more gains in someone who's a GMFCS level 2 or 3. Bonus, what else are we looking for in the ideal candidate for SDR? I think it was Becky, did you answer that one? I don't think so, but something else you're looking for is like the ability to participate in physical therapy following procedure. Yeah, that's a huge one. Participating in therapy is because the post-operative rehab with SDR is extremely important to promote a good outcome. Other things that we look for are strength. We want to make sure that the child is strong enough because you're getting rid of all of their spasticity. If they're using their spasticity in any way functionally, you don't want to make it such that the child is unable to get back to walking even after considerable rehab. We want to look for good isolated motor control. It's been shown in research that children with better motor control have better outcomes with SDR. We're looking for a specific age range, about 3-8. But actually, I just read a research study that just came out this month that showed actually that kids who are in the 10-18 year age group, actually do pretty well too in terms of their functional outcomes on the GMFM, as well as self-reported self-care skills and quality of life after SDR. I think that there may be a little bit of a shift in that. There's certainly different providers across the country who will do SDR in patients who are a little bit older. Dr. Fahm, what article was that? You know what? I can send it to you. At the end of this, you'll have my e-mail address because it's so new. I literally just read it this morning, but I'm happy to send it to you. It's from a group in the UK. They also looked at dystonia. They didn't quantify dystonia, which I'm disappointed by, but they looked at kids who had dystonia and kids who did not have dystonia, and they didn't see a difference in the kids who had it versus didn't have it. But we don't know how much dystonia they had because they didn't report their Barry Albright dystonia scale scores, which is what they used to measure it. But yes, e-mail me. I'm happy to send it to you. We have two more. Becky, do you want to pick the next one? Rhizotomy for 300. Excellent. This procedure is done in the immediate post-operative period to improve range of motion. Emily says serocasting. Excellent. Thank you, Emily. Beautiful. Last, before our final jeopardy, this is a double jeopardy. This is used in the OR to determine whether a spinal root is dorsal or ventral and how abnormal it is. Maya says EMG. Excellent. Neuromonitoring, which includes EMG. There are surface EMG electrodes that are placed in specific muscle groups that allow you to, when the roots are stimulated, they allow you to see what muscles are being activated. That is part of what makes the rhizotomy selective, because you're looking for specific nerve roots. You're also selective for dorsal and not ventral in this case. I don't need you guys to tell me about SDR, because we've been through a lot of SDR questions and I want to make sure we get to our final jeopardy question. Let's just put this out there. This is a 10-year-old child with an ITV pump in place for three years with loss of efficacy. Family reports increasing hypertonicity. Your workup thus far has included an X-ray to evaluate the catheter, which appears to be intact. You did a catheter access port and a dye study that showed free-flowing CSF and free-flowing contrast. Then what test, this test can be used to evaluate for micro tears in the catheter. This is for you, Mary. If people want to put their answers in the chat. That was lovely, Kelly. We're starting to get some answers in the chat. Maybe we'll give people a few more seconds here to- Perfect. Yeah. If everyone can enter in something, I would love that. So far, the only tests we have listed is an MRI. I've never done an MRI to look at a catheter. No, I don't think I ever have. Does anyone else have other thoughts? Does anyone want to say anything about it? We do have a comment from Jeremy. Did we check for infection, constipation, and retention? We absolutely did, Jeremy. That was the first thing that we did. Because you always want to make sure that there's not something else causing their hypertonicity to be high. And that's a really important point, right? In the acute phase, if you have a child who has a pump who presents with an increase in their hypertonicity, you always want to look for something else. But you still want to evaluate the pump. Because even though we say oftentimes, it's not the pump, it's not the pump. Sometimes it's the pump. And so we do need to evaluate the pump. For this case, when I created this, I was thinking of more of the, like, slowly losing efficacy. Like, oh, it's not really working very well. We tried going up. When we go up, we don't really see a big difference. More of that sort of case rather than an acute, their hypertonicity is increased. But that's an excellent point. Anything else in the chat? Some people are thinking about bolus trials. Oh, that's a very good idea. So the thing you have to consider when you're talking about a micro tear, and this is why you can't see a micro tear, excuse me, on dye study, is because you're injecting too fast, right? So with a cap study, basically, you draw out all of the medication and some CSF through the catheter. You empty the catheter. And then once you're able to do that, as long as you're able to do that and empty the catheter, then you insert your dye. You inject your dye. You watch under fluoro to see, is the dye going through the catheter? Is it getting stuck? Is it flowing freely through the CSF? But when you're pushing that dye, you're pushing it fast. So the same theoretically is true when you're pushing a bolus. So when you're pushing a bolus, theoretically, the medicine is going faster through the pump. And so you might not see a difference. You might see effect from the bolus, but it doesn't really tell you about micro tears. And so the answer to this is actually something called an indium dye study. And this is a really interesting study that I think at some institutions, people do a lot of, and at other institutions, they never do. And so I am at an institution that never does this. Part of the reason why is that it takes a really long time. It's really hard to get indium dye. And so it's not something that we carry at our institution. And so it's not a study that we do. But there are other studies or other centers across the country that do a ton of these. So basically, what you do is you put this tiny little bit of indium dye into the reservoir of the pump. And then you watch the dye. You watch the dye as the baclofen is pumped through the catheter over the course of a couple of days. And so you can see in pictures A and C, this is normal at four hours. So basically, the only place you can see the indium is in the pump. You can't really see it very well because it hasn't gone into the catheter. This is just the med in the catheter, right? Because you're waiting for the teeny tiny little bit of medication that's being pumped through the catheter depending on the patient's dose. And so in B and D here, this is at 24 hours. So you can see that there's still indium in the reservoir. But now you can see that the indium is going up into the catheter. Now, with indium, if there's a micro tear, because it's going so slow, it's going as slow as the pump is infusing medication, you can see the indium leaking out. And that's not something that you can see in a catheter access port of dye study. The other thing that you can see, you can see if the catheter is kinked. You can see if the catheter is disconnected because the indium never leaves the reservoir. Or you see it just stopping as it's tracking up through the catheter. So this is actually, it's kind of an interesting approach to evaluating the catheter. I would say what more people do instead of doing this indium dye study is they do every single other bit of workup that they can do, and then they just replace the catheter rather than doing an indium dye study. So I think that's more sort of typical practice around the country. But some people do a lot of it, like I said. And you might end up at one of those places where they do a lot of it. So I'm going to get out of this. And I'm going to pull up my last slide here so that you guys have my email address. Does anyone have any questions about that? You got a few people thanking you and saying this was great. Oh, good. Thanks. I'm so glad that people enjoyed it and that it was interactive. Thank you for participating. It's always better with participation. So all right. Feel free to email me if you guys have any questions or if you want that article. Let me know. I'm happy to send it out. Perfect. Thank you so much, Dr. Plum. Yeah, you're getting great comments in the chat box. So I'll just give a few seconds for you guys to copy down that email address for Dr. Pham if you wanted to. And then just a reminder again that you can claim CME credits for this if you'd like through the AAPMNR website. And then we are going to be doing our next webinar next month. And we're going to do it on the topic of difficult conversations that you have for patients when you're trying to get you have for patients when you're taking care of patients with spinal cord injury. And Dr. Rashad is going to be giving that. So look for more information on that. It should be the end of March. And then we'll hope to see you guys there. All right. Take care, everyone. Thanks again. Thank you. Thank you so much.

Dr. Kelly Pham

Seattle Children's Hospital

tone management

pediatric rehabilitation medicine

Jeopardy-style game

baclofen pumps

injectable medications

hypertonia

movement disorders

selective dorsal rhizotomy