We're going to get started. Welcome to the Neuromuscular and Electrodiagnostic Medicine Community Meeting for Member May. I'm Colin Franz. I am, amongst other things, I am the Community Chair for Neuromuscular and EDX, and I am beyond delighted to have some wonderful speakers organized tonight for this event. And I'm going to quickly introduce the speakers so I don't eat up too much of their time and the time for everyone to potentially ask questions, interact with our panel today. But it's going to be Drs. Peter Grant, Adenike Adeweye, and Teh Cheung. And briefly, we'll go in order here. Dr. Grant, one of the most distinguished figures in the field of PM&R and electrodiagnostic medicine, easily, and I can tell you he's a past president of the AANEM, the American Association of Neuromuscular and Electrodiagnostic Medicine. He has an academic affiliation as a professor at Ohio State University, and he's had a career, I think, that's touched a lot of people, and has certainly been a strong proponent and advocate for the role of physiatrists and PM&R in electrodiagnostic and neuromuscular medicine. He's done things to uplift the standards of practice within the field of electrodiagnostic medicine, which has been important and increasingly important now as there's pressures to change the electrodiagnostic training, potentially for PM&R residents. And his leadership roles have also included being the founding chairman of the Electrodiagnostic Laboratory Accreditation Committee and his ongoing work and advocacy for the field with the AANEM really highlights his commitment to our field and just the excellence and integrity in electrodiagnostic practice, which I think we'll come through with his presentation tonight. Dr. Attaway is Assistant Professor of Physical Medicine and Rehab in Neurology at Northwestern University and at the Shirley Ryan Ability Lab, formerly the Rehabilitation Institute of Chicago. She's been here so long, actually, between an MD and a PhD, that she's worked at both places. And as, you know, I think a lot of stories that she could probably, more stories that she could share about the old Rehabilitation Institute of Chicago. Despite her having been around for a long time, she's also like a rising star in her field. She completed her Neuromuscular Medicine Fellowship just last year at Northwestern in the Department of Neurology. She's been working to bridge her PhD work on neuromotor control and bioelectric signal processing and machine learning to her clinical directed research now on motor unit dysfunction after spinal cord injury and plus her clinical electrodiagnostic work. And that's gonna really tie in nicely when you see the work she's been doing about personalizing treatments using electrophysiology for patients with spinal cord injury who present to a nerve transfer clinic. And finally, Dr. Chung, who's an Associate Professor of PM&R and Neurology at Johns Hopkins University is also the Director of the Johns Hopkins POTS program. He's bringing a wealth of expertise in neuromuscular disorders. He having completed his Neuromuscular Medicine Fellowship at Hopkins after he completed his PM&R residency there as well. And he'll be sharing his insights on POTS, dysautonomia and drawing from both his research experience in this area, as well as his leadership as Director of the clinic at Johns Hopkins. So with that said, we'll have Dr. Grant kick things off with his presentation. He can put up your slides and we will try to fit in some questions between talks and as well as have some discussion at the end. Great, thanks, Colin. I appreciate the introduction. You know, this was the first title that I had for this talk, Electrodiagnostic Medicine, Performance, Qualifications, Competency, Quality and Ethics. That's pretty boring. So I wanted something with a little more of a hook. So I decided to call it EDX Medicine, Our Physiatric Heritage, because that's one of the biggest points that I want to get across tonight. When we talk about electrodiagnostic medicine, who should and shouldn't be doing EDX testing, we talk about ethics and fraud and abuse, which I'll get to in the end. I think it's important that everybody here as physiatrists understands that this is part of our heritage. I think Colin mentioned that I'm an assistant professor, adjunct at Ohio State, but actually I'm in private practice on the other side of the country in Medford, Oregon. I've been here for 39 years. I'm fortunate enough to have two sons who also trained at Ohio State who are with me in practice now. So overview of what we're going to talk about is my part, to perform or not to perform EDX studies. You know, that's going to be the question with that part of the discussion. What qualifications should a person have to be able to perform them with good quality? And then about promoting quality in electrodiagnostic medicine, because as I will show you, quality has actually gone down over the last 10 to 20 years in the studies that are being produced across the country. And then the last part will be what I call the rest of the story. And that's where I get into some of the fraud and abuse, which is really pretty amazing. So to perform or not to perform. You know, there's a lot of people that come out, a lot of residents come out of training and feel like they don't have a lot of confidence. They haven't, they may mainly barely got their 200 number of EDX studies and just not feeling like it's something that they may want to incorporate into their practices, whether academic or private or otherwise. And then there's also people that have been doing this for a while, but maybe aren't doing a lot of it and starting to feel like maybe they should or should not be doing it anymore. So we'll look into that just a little bit. But as I said, I really want to hammer home that this is in our blood. This EDX testing and EDX medicine has defined our specialty for over 75 years. For, in fact, my father, who was a past president of AAPMNR, I was born in 1955 when he was in his residency training. He did EDX throughout his career. It sets us apart from other specialties because other specialties know us and know the excellent electrodiagnostic testing that we provide for them and for their patients and how much it helps them. You may not have known this, but most of the original public studies, the studies that gave us the normative tables, they told us how far to measure and where to stimulate and which muscles for different levels in EMG. Those studies were done by physiatrists, not neurologists. And it brings to mind a number of the greats, Ernie Johnson and Joe Goodgold and Randy Bradham and others, including one of my mentors, Sue Hubbell, who's on this call. Hey, Sue, shout out to you. Thank you. But there's a lot of the work that was done in setting out EDX testing was done by physiatrists and not so much by neurologists. I'm gonna say that we're the best physicians to do these studies. And I have actually had a number of my neurologic colleagues who I'm close with agree with me on that. In fact, some of them have told me that without me saying it. Now, it's because of the foundational training that we get in our residencies. Anatomy, functional anatomy, kinesiology, neuropathology. We learn a lot about musculoskeletal disorders and neuromuscular diseases, non-operative orthopedics. I mean, these are the things that help us to develop a very good differential when we are in the EMG lab and we're trying to figure out what's going on with certain patients. So we truly are, by our own training, the best physicians to be doing these tests. We're the best physicians by that training, by that same training that I mentioned, to perform the HMP and then develop an inclusive differential diagnosis to then do the testing and more accurately and reliably diagnose these patients. I think I'm speaking to the choir here, preaching to the choir, but understand that EDS testing is a physiologic test. I've been around long enough that when CT scans first came on the scene, a lot of people said, oh, we're not gonna need you guys anymore. We've got CT scans. That didn't really happen, did it? Same thing with MRIs, and they got higher resolution. We're not gonna need you guys. They still need us. It's because all of those tests are anatomic. We are physiologic. Together, they're great together, okay? So EDX testing has an undisputed role, not only in the diagnosis, but also the management of a lot of the things that we see, radiculopathies, plexopathies. And so it has an importance that goes beyond just making the diagnosis. And this is something that really needs to be in your PMNR toolbox. I'm gonna make a case for the argument to perform EDX studies. If you don't feel confident, there's ways that you can become more confident. If you don't feel like you have enough training, there's ways to get more. But this is something that most every PMNR physician should have in their toolbox because it complements everything else, including the anatomic studies that are out there. It also complements neuromuscular and musculoskeletal ultrasound, one of the greatest ultrasonographers I know, Jeff Stokowski, he will tell you that he continues to do EDX regularly because it is so important. Just like spinal cord rehab, stroke rehab is foundational in PMNR, so should electrodiagnostic testing be. All of the procedures, these ancillary procedures we do, ultrasound injections, Botox, all of these other things are benefited by having a good knowledge of electrodiagnostic medicine. Now, there were some big cuts back in 2013 in the reimbursement for what we do in electrodiagnostic medicine, but that has slowly actually come back. And it's still something that is, can definitely help from the financial side of your practice whether you're in private practice or academia. You know, I can't talk specific numbers because they are a little different where we are, but that's also, it's illegal for me to talk specific numbers and put it to CPT codes. But I can say that in my area, and my area is not vastly different from any other region in the United States, an EDX study pays about the same as six or seven full consults. So it is people that say, there were cuts in reimbursement, it's not worth it anymore, need to relook at that part. Doing, incorporating this into your practice will make you a better physiatrist, not only by the studies that you do, but also by the other things that you do and how understanding the neurophysiology that underscores those problems will make you a better physician and be able to help your patients more. The other thing that I have to mention is, and this has already started to happen, is that if we don't have enough PM&R physicians that are performing good quality studies, the door is open. And when the door is open, others come through the door. And what ends up happening is we end up having poor quality studies that are done by PTs, chiropractors, sometimes done by MDs, DOs that just don't have good training. And what ends up happening is that the patients are the ones that suffer. In the last 25 years plus, I've worked with the FBI, OIG, I have ongoing cases with them, with private insurers, with different states and U.S. attorneys. And I have seen the fraudsters move in when these doors open up. So that's just one more reason why we need to be the ones that are doing these tests. This constriction that I talk about of the availability to have well-trained physicians doing this really opens up this door and the opportunity becomes available. If we are doing these tests, the opportunity won't be there. So in summary of this part, to perform or not to perform EDX testing portion, the summary is EDX testing is our heritage. It's what we're known for. It's informative and it gives great clinical information. It pays well. It's physiologic. It's complimentary to anatomics testing. And it's medically necessary and appropriate in many of the patients that we see and treat. And as I said before, it's an important tool that we as physiatrists should have in our toolbox. So let's talk about qualifications to perform EDX testing. You know, in our residency training, we get to 200. We talk about 200 EDX studies. Now, unfortunately, as Colin mentioned, there's some talk about lowering that number and that's a whole nother discussion and I could go off on an hour talk on that. But I wanted to point out something that a lot of people don't know is that neurology residency training, ACGME does not require any studies. Now, most of the time neurologists, and this is not a knock. I have many friends that are neurologists. I'm not gonna tell you anything that they wouldn't tell you. But I will say that the neurologists that end up doing EMGs either had a training program that did have EMG or they do a fellowship afterwards to get that. non-EMG experience in PM&R sets us up, as I said before, to being good EMGers. And then we have a strong network of teachers and mentors in residency and beyond. And then when we talk post-residency, there's lots of learning opportunities, you know, through the AANEM and conferences there, in addition to conferences at AAPM&R. AANEM has position papers and learning materials, as does the AAPM&R. There's board certification through the ABEM and there's lab accreditation through AANEM. Now I'll mention in a minute a little more, but AAPM&R, in fact, I think it's in my next slide, so maybe I'll hold off. Well, promoting quality in EDX medicine. I'll come to what I was going to say there in just a second. So to promote quality, good training is important. PM&R residency is huge. Post-residency, ongoing learning is important. You know, maybe becoming ABEM certified would be good, having your lab accredited. And calling out the fraud and abuse you see. The things that I will talk about later, the types of fraud that I see in mobile diagnostic labs and some inappropriate equipment that people use, that is in every community in the United States, I can assure you. I've had people say, nope, it's not mine. And I'm saying, you've got your head buried in the sand. It's there. And so calling out the bad studies and the fraud and abuse that's in your community and in your area is important for promoting quality across the board. Advocacy is important. I was just talking today, two days ago, I was in Washington, D.C., and I was talking again today with a good friend of mine who is a congressman, has been for 27 years, and this House Bill 2639, the Electrodiagnostic Medicine Patient Protection and Fraud Elimination Act is something that hopefully later this year will be passed. And it's going to be something that's going to be huge and knocking out a lot of the fraud that's out there. But again, I have to emphasize that if good EMGs aren't performing these tests, then somebody else is going to be doing them and they usually are going to be of lesser quality. So here's the rest of the story. This is the EDX fraud and abuse that I was mentioning. There's a lot more poor quality in the EDX studies that are being done across the nation. And some of the reasons for this trend include that there's less EDX offerings at American Academy of Neurology and AAPMNR. Now, AAPMNR is actually turning the corner a little on this. Dr. Franz, who introduced us, and I, and some others are doing a half-day pre-course at the upcoming meeting in San Diego called EMG Reboot. And people that are either just out of residency or have been in practice, anybody that wants to sort of get back into it and feel confident in their skills again can go to that class. We're also going to have more offerings hopefully in the coming years at AAPMNR. There's less people that are taking the ABEM, the board exam for EDX medicine. We'd like to see that turn around a bit. And then the training in EDX in residencies, both neuro and AAPMNR, there's just more of a challenge to get those 200. It doesn't mean that we should make them go away, but compared to when I trained, there's ultrasound, there's injections, there's Botox, there's all kinds of other things that weren't there, but we shouldn't let ADX slide. We need to continue to do that as well. Sometimes people that don't have any or less involvement with AANEM, that's sort of the organization that really is out there for EDX medicine. If you really want to hone your skills and learn more, that's a good place to go. And then sometimes people that have been out in practice for a while but aren't doing much start to fall off. And then of course EDX fraud and abuse itself is something that fights against good quality because most of those are horrible quality. I already talked about when there's less trained physicians, others are going to come in. There's a declining interest in residents. You know, some of the time people, residents come out and they just don't feel confident. They don't feel prepared. And so they don't end up incorporating it into their practice, whether they're private practice, group practice, you know, whatever, academic, whatever. And some physicians in practice end up not doing enough studies to feel confident and end up just dropping off altogether. So there's lots of reasons that we can have poor quality. Now, as I mentioned before, unfortunately there's a lot of others that are starting to come into the game and starting to do these tests. And I'm going to even talk about, and often it's driven by finances and not, you know, wanting good quality medicine. So what other providers are there that do EDX testing? This was a question that I'll have you read real quick, but you know, who can be paid for nerve conduction and EMG studies by Medicare? Not private, but by Medicare. Neurologists and PMR physicians? Well, I hope so. Physical therapists? Hmm. Orthopedic surgeons? Well, the answer is actually D. And that's because Medicare is not in the business of getting into scope of practice issues. I could actually go and perform cardiovascular surgery. I wouldn't want you to be my patient, but they don't differentiate. If I have an MD or DO, I can do any of those things that MDs and DOs do. With regards to PTs, they actually gave PTs the right to do and bill for EDX testing back in 1997. It was put into the LCDs in 2001. Now you have to have what's called an ECF certification through the APTA, and there's only 204 that are doing it now, but that's by Medicare standards. In private payment systems, there's a lot more that do that across the country. And in different states, there's more or less. Now, who can interpret the studies? In some states, that's a whole different question. But what ends up happening is, let's say you're in a state that doesn't allow PTs or chiropractors to interpret. They can perform the testing like a technician, but they're not allowed to interpret. Well, they get around that. If it's a carpal tunnel, they'll say studies were consistent with carpal tunnel or consistent with cervical radiculopathy, and thereby get away from being actually saying a diagnosis. Although between you and me and the LAMPOs, that's essentially what they're doing, of course. The problem is, if you look, the LCDs, Medicare's guidelines say that they are not allowed to do that, but Medicare doesn't have any enforcement mechanisms. So they continue to do that. This data was from 2019. Probably in the next year or so, we're going to get some more updated, but it's not going to be much different. It's been like this for years. These are the numbers of nerve conductions and EMGs that are done by different specialties. Neurology, two-thirds of it. About a third, the big orange piece of pie is PM&R. The little 1% gray is pain medicine. Those are probably going to be physiatrists anyway, but 2% are physical therapists and 3% are other MDs or DOs. Now, if we do the math on that, if you go down to the bottom there, I say there's 5 to 6 million EDX studies done per year across the country, all insurances. That means that there's 100 to 120,000 EDX studies done by PTs and 150, 180 done by non-PM&R, non-neurodocs. My question is, are you okay with those numbers? I don't think I am. These are some of the docs that are actually submitting EDX claims for reimbursement and being paid by Medicare. The list is crazy. You want to say, why is a nephrologist doing that? Why is a cardiologist doing that? One of the things I mentioned with regards to declining EDX is the fraud and abuse. I'm going to be real quick with this because I'm getting near the end of my time. I'm going to go ahead just a bit because I want to talk about some of the main types of EDX fraud include something called a mobile diagnostic lab. There's some inappropriate equipment that's used, including handhelds. Then there's some really crazy, very creative and sophisticated fraud schemes that are out there. Mobile diagnostic labs basically are a tech that's being sent into a family physician's office. I've seen them in cardiologists, pulmonologists, podiatrists, but anyway, a tech, unsupervised, sent into an office, does a barrage, what we call a shotgun, because they don't know which test, which nerves to do and not do. When you and I might do seven, they are going to do 25 or 30. Typically, very poor waveforms, uninterpretable waveforms are obtained. There's some MD or DO that will see these results of these tests weeks or months later, maybe thousands of miles away, and then they'll get them to bill. It's a very inaccurate way of doing this testing. Usually, there's no EMG performed at all, though they are diagnosing radiculopathies with this. My first case was in 1997. It was a gentleman, 47-year-old guy that was told that he had a perineal neuropathy. As it turns out, three weeks later, I did the study and he had ALS. He was charged $7,500. This is back in 1997. My current high charge is $35,000 for one nerve conduction study on one patient. Now, you say, what insurance company would ever, in their right mind, ever pay for that? It was a fraud case that I did with the FBI in Houston on that one. Basically, the FBI healthcare fraud field agent told me, well, we looked at it and they only were paying one of every 23 claims. But when you're charging $35,000, one of every 23, you're still making out like a bandit. This is a very interesting one because this gentleman, I'm not going to go through all of this, but 22 nerves on every patient. Every patient was diagnosed with generalized neuropathy and told they needed to be seen in six months to have a repeat study, charging $5,500 per exam. The gentleman that did this, PM&R physician, I'm sorry to say, down in Louisiana, was sitting at home on his couch, reading these waveforms after his regular day of physiatric practice at work. When he would do that, he was making $1.5 million per year. It's a lot of money in fraud. There's a lot of, I'm not telling you to go into fraud by saying that, but there's a lot of money in fraud. Lastly, I want to mention very quickly and an appropriate piece of equipment, and you may have heard of it, called pain fiber nerve conduction or PFNCS. This equipment is basically a type of quantitative sensory testing. They'll put a little stimulator on a dermatome and they'll slowly turn it up and say, tell me when you feel it. The patient has to respond by saying, yes, no. Immediately, you have a subjective test. It's not objective at all. When I am dealing with this case in a court of law as an expert witness, I will turn to the jury and I will say, I want to tell you the difference between this and what we do. Those of us that do this appropriately, we do a test that's objective. What I mean by that is I can do my test in the ICU on a patient that's comatose and have accurate results. This test, they have to be awake and they have to tell me yes or no. Now, that's fine if you believe the patient, but if it's a worker's comp claim, personal injury claim, or something else, maybe the patient won't always want to tell you the exact truth. They also, the company that does this says that their testing is 97% sensitive in diagnosing a radiculopathy while what we do is only 37% sensitive. Very last thing, handheld devices. There's a company that makes them. They did it for upper extremity for years. They still do. Now, they do it for lower extremity. It's something to watch out for. It's highly inaccurate. It's used. It's usually put in the hands. The company will come in and teach the orthopedist or family physicians, medical assistant or receptionist, I've seen both, to do these tests for them. Just very quickly, the last slide I'll show you is, these are some of the waveforms that I get in some of my fraud cases. Tell me that you can read accurately those sensory responses when there is 60 cycle interference across the whole page. You can't, nobody can, and yet they're making diagnoses. They're having treatments, medications, injections, and surgeries based on diagnoses made on these horrible testing. I'm going to leave it right there, Colin. Thank you everybody for being here tonight, and we're going to have some questions, I think, at the end, Colin, or did you want to have a few questions now and then also at the end? Yeah, if there's any immediate questions, I'm happy to take one or two, and then we'll save some time in the end for some broader discussion. I have seen some of those waveforms in the past from your prior presentations, and sometimes I even reference them when I teaching residents about, it's like, we don't want to end up on one of Dr. Grant's future presentations. We got to, you got to do better than that. Well, thanks everybody. Yeah, so feel free to type some questions in the chat. We're going to start switching over. Dr. Attaway, why don't you put up your slides, and we can, if there's a quick question, I think people have the ability to unmute, but I'd say mute it if you're not going to ask an immediate question or raise your hand, and otherwise, I'll watch the questions, and we'll pull them for the Dr. Grant at the end if something comes up. Thanks, everyone. All right, and then just make sure you are, you're unmuted so you're ready to take off there, Dr. Attaway. Can you see my screen? We can. Excellent. All right, well, for that great talk, we're going to shift gears a little bit. And so for this talk, I wanted to focus on looking at changes to the lower motor neuron after spinal cord injury, specifically those of cervical spinal cord injury and the implications that that has for reconstructive surgical options. So nothing to disclose. And hopefully by the end, you are able to sort of summarize the benefits of timely EDX studies after cervical spinal cord injury and describe how these lower motor neuron changes affect the reconstructive options after SCI. Okay. All right, so just so we're all on the same page, and hopefully we've all been trained in SCI and I think traditionally when we think about spinal cord injury, we're all taught, you know, here's an example of a C6, C7 dislocation. At the level of the injury, in paralegional, you have injury to your descending tracts or your upper motor neuron and injury to the cell bodies. So your lower motor neuron, and then above the injury, the segments are normal, the individual has voluntary control of those nerve muscle groups and below the level of the injury, the cell bodies are intact, but because there is no descending input, they're unable to activate those muscles. And so traditionally, that is what we're taught. And just to hammer that point home, at the level of the injury, I'd say you have damage to that lower motor neuron over time, and that results in degeneration atrophy. And we know that lower motor neuron death can lead to irreversible denervation atrophy, which is to be differentiated from disuse atrophy, which is typically irreversible. So going back to that figure, so what if it's the case that below the lesion, the cell bodies are not necessarily intact? And so we've had increasing evidence that suggests that below the level of the injury, that that lower motor neuron actually may be damaged. So not just at that level, but also below the level. And as physiatrists, when we see individuals with spinal cord injury, and we do our ASIA exam, we do our international classification of hand function and tetraplegia exam, these examinations look at weakness. So they don't distinguish between upper and lower motor neuron lesions. And so we're not, and because we're not doing this, this has implications for a lot of things. And so things like neuromodulation, functional electrical stimulation, certainly things like candidacy for diaphragmatic pacing, and also surgical options, which we'll delve into a little bit more. And so these are a couple of surgical options that are available after a spinal cord injury. On the left, you've got tendon transfers, and those have been around for a very long time, which is, within spinal cord injury, the idea is that you're taking a muscle that is innervated by your nerve above the level of the injury, and you are then attaching it to a muscle below the level of the injury. And so in this figure on the left, that's the brachioradialis, that is above the level of the injury, that then they're attaching to the flexor pulse as long as to restore thumb function. On the right side, you have a nerve transfer, which is a similar idea, but now you're taking a nerve that's going to a muscle that is, again, above level of the injury, and you are then re-innervating a nerve muscle group below the level of the injury. And so that's what's that's depicting here on the left. And on the right, this is sort of similar to that initial diagram I showed you, right, where the donor nerve is taking above the injury at the level of the injury, so in the lesional area in the spinal cord. You have both injury upper and lower. And, you know, traditionally, again, in this journal article published in 2021, right, we know that denervation atrophy is irreversible, and so it's time-sensitive to evaluate these nerve muscle groups, and the thought is that below the level of the injury, it's not as time-sensitive, they don't get referred to surgery. But if it's the case that this is not necessarily true, right, that means that now infralesionally is also time-sensitive. And so we wanted, and so to give you a little bit more background into the surgical options that are available, commonly, for someone who, you know, you want to restore elbow extension, one can take the axillary branch of the posterior deltoid and use that to re-innervate the triceps. So the recipient would be the radial branch going to your triceps. For something like restoration of wrist and finger extension, you could take the axillary branch going to the teres minor, or commonly the radial branch going to your supinator, and using that to re-innovate the posterior interosseous nerve to restore wrist and finger extension. And for wrist and thumb flexion, a common transfer is a musculopectaneous branch to the brachialis as the donor, and the recipient would be your anterior interosseous nerve to then restore finger and wrist flexion. And here's a video of a brachialis to that anterior interosseous nerve transfer courtesy of Dr. Ida Fox, I think that should be playing here, so here's an individual pre-operatively trying to complete this PEG task, and obviously that's difficult to do with his level of weakness, and here he is after the procedure, and if you can see now his FPL, so you can see that thumb flexion, that finger flexion that's allowing him to grasp that PEG. And so what you're really doing with these procedures is you're restoring specific, you're evaluating the patient's needs, right, and then using the information that you gather to determine which specific functions you want to restore, which determines what procedures they undergo. And so we wanted to delve into that question of what is happening below the level of the injury. And so in this study, we looked retrospectively at individuals with cervical spinal cord injury, specifically subacutely, because previous studies had mostly been done in people with chronic spinal cord injury, and again, and here we're looking at these denervation potentials, so your fibrillation potentials, your positive shockwaves, right, and this was a binary yes or no. Is this present in these muscles? And these were mainly males, around 44 years of age, and again, subacutely, so on average they were three, three and a half months from their injury, and also as low as three weeks from their injury. So we're looking at all levels, A through D, and, you know, C1 to C7 neurologic levels, mainly C4, C5, and some C6. And so I want to sort of direct you, so on the right is just a pictorial representation of what you're seeing on the left, so the, if you look at these, let me put on my pointer here, laser pointer, excellent. So you know, each individual has their own specific neurologic level, and so if we normalize the presence of these denervation potentials relative to a neurologic injury, we're seeing a presence of that, you know, one level above and below, which is not surprising, but we're also seeing it two, three, four, and even five segments below the level of their injury in, you know, 47 to almost 80% of muscles, of muscles that were tested. And so this is, you know, we show that we're seeing a high frequency of these denervation potentials in these segments beneath the lesion epicenter, but the issue with denervation potentials is that these are, you know, nonspecific, they're descriptive markers, and certainly something like a compound muscle action potential gives you a little bit more quantitative information, but we do need more robust quantitative ways of evaluating low motor neuron function in this population of individuals that do not have voluntary, do not necessarily have voluntary activation of their motor units. And I think there are questions in the chat, but I guess we will take them at the end unless they are. And so, so we turn to more quantitative measures. So talk a little bit about motor unit number estimation, and for those of you who are unfamiliar with it, again, you know, this has been around for a while, there are different methods that have been developed over time, and it gives us a method of non-invasively measuring the number of motor units specifically, it's more of an index of the motor unit number as opposed to an actual quantification of all the units in the muscle. And so in this example for your extensor indices, you would, you know, set up the way you would typically do for nerve conduction studies, and then obtain your compound muscle action potential, you know, eight centimeters proximal to your, to your electrodes. And then the next step, which would be different, is you would provide these small currents, very small currents, that in order to elicit single motor unit potentials from the surface, and you would repeat that procedure all across that length of that nerve in order to get, you know, different units with different morphologies, with the assumption that each unit that is repeatable is a different single motor unit potential, and then get an, you know, an estimate, this index of the motor unit number by just, you know, simple, simply dividing that CMAP with your average single motor unit potentials. And so for this protocol, we had 15 total individuals, mainly male, significant, so 10 out of 15 were subacute, so within three or so months from their, from their injury, most of them were traumatic, 14 out of 15 were traumatic. In this population, we, as you can see here, most of them were between C1 to C5, and some C6 neurologic level of injuries, right, because we wanted to sort of focus on the muscle below the level of injury, we focused on the extensor indices, and the anconia sort of as a substitute for your triceps muting. And what you can see here on the left side is your compound muscle action potential, and on the right are the average number of motor units, and so you can see pictorially a difference between the blue, which is the number of units in our control subjects, and the number of units in our individuals with spinal cord injury. And so why is this important? When we think about the implications this has on options for nerve transfer and think about timing of that, it's important to figure out, you know, does this person have a mixed picture, right, how severe is that lower motor neuron injury, because when we think about if it's just an upper motor neuron and we think about timing, we have a lot more time, you know, 12 to 18 months, we don't think there's denervation, the person has a lot more leeway in terms of considering their surgical options, but if they have significant burden of lower motor neuron damage, it's important to catch these individuals early to give them the option for a nerve transfer. And then I like this figure, you see, it gives you sort of, you know, gives you a framework of what could be happening, right, so do they have, you know, no lower motor neuron generation, mild or severe, and sort of think about it as you're approaching your electrodiagnostic studies and how that's going to sort of influence your conversations with your surgical colleagues. And when you think about that framework for electrodiagnostics, perhaps these individuals, right, who, you know, who don't have a lot of injury, those certainly seem like people that would be great candidates for nerve transfer, right, who don't have that denervation atrophy. And if you've seen someone who's two years out, you've got severe burden, perhaps they're a better candidate for tendon transfer. The question is if they have mild or moderate injury, it's not so clear what to do, where to go with that, and certainly the decision between these particular transfers is not just based on electrodiagnostics, there are other factors that play into that patient preference and presence of spasticity, but we're focusing on what we're seeing electrodiagnostically and how that could sort of inform what decision you make. Given that, you know, there are different pathways, I think that sort of lends itself to, it's sort of important for us now to find these patients early. And I think that as physiatrists, we're sort of in a unique position where we're treating people with spinal cord injury earlier on, we're seeing them in acute and patient rehab setting, and we should start thinking about doing these studies earlier, right, seeing them, you know, two months, three months out of their injury and doing these studies so that you can actually have these conversations with them early on. And, you know, a lot of times we get patients who are, you know, 12 months out and who would, who perhaps would have been candidates for nerve transfers who no longer are. And I wanted to close us on these couple of slides. There was a study where, that looked at the rate of reconstruction in patients with tetraplegia between 2012 and 2017 in over 400,000 encounters, only about 0.4% of people underwent upper extremity reconstruction during this time period. And in a subsequent study where they surveyed physiatrists with interest in spinal cord injury and injury medicine and hand surgeons, they saw that, highlighted there, of, there are about 39% of physiatrists in the past 12 months had referred individuals for surgical reconstruction and only 24% of surgeons have, had done these procedures. But then when you ask the physiatrists or the surgeons, if they would have wanted the procedures for themselves, if they had tetraplegia, that number went, is, you know, 63% compared to 96% within surgeons. And so that was, you know, an interesting, interesting finding. And the thought that the major factor that determined whether these procedures are performed is that presence of a relationship between the surgeon and a physiatrist. And so, and I thought that was, you know, a pretty interesting, pretty interesting finding. And that kind of underscores our importance as a physiatrist and our role as physiatrists in, in, in identifying who could benefit from, from these transfers. And so just some take up, take home points that if, you know, you fall asleep during this, that hopefully you'll, you'll take away is that low motor neurone degeneration has implications for patient prognosis. Surgical rehabilitation and timely EDX studies is key for distinguishing the patterns of injury and dictating the best treatment option. And so with that, I'd like to thank everyone, and I guess we can take some questions. Great. Thank you so much, Dr. Attaway. This was a fascinating talk. We have a couple general questions, and I think, well, why don't we switch over, let Dr. Chung share his slide, and then I'm going to direct the first question to you and Dr. Grant in that order from Dr. Ramos. Will ultrasound, this is not specific to the tetraplegia stuff, but will ultrasound supplant or complement workup for carpal tunnel syndrome or ulnar neuropathy or any other entrapment syndrome that standard of care diagnosis is made by EDX testing? And for some context, they seem to have trained when ultrasound wasn't being readily available in residency training, so probably won't ever have that skill in their arsenal. So what do you think, Dr. Attaway? So I think from the perspective of transfers, I think they would definitely complement our EDX. So I think a great example is when you have someone who has a lot of spasticity or whose positioning is not ideal, it's important that you be in the right muscle. And so, for example, if the surgeon wants to take the nerve to your extensor carpi radialis or brevis, right, that's hard to sort of differentiate when you're doing your EDX, but that ultrasound is actually quite helpful in localizing and making sure you're in the right spot, right, because you're trying to determine, is this muscle, nerve muscle group, a good donor or not? So definitely very complimentary. Dr. Grant, do you have anything to add? Yeah, well, actually, I would say the short answer is no, it's not going to surplan it. I think Dr. Stokowski, who again, you know, has written four textbooks on ultrasound, he would say that it wouldn't either. And it's something, as has been mentioned, is complimentary. It also is an anatomic test. It's a dynamic anatomic test. And so it can give even more information, but it still is not a physiologic test of the function of the nerve, which is what, it becomes really important when you're talking CTS or ulnar neuropathy or something. I am like Dr. Ramos, I'm not going to ever be doing it. Luckily, I have two sons in my practice that do it. And I do have certain patients that I will have them see after I see them, post-trauma, post-op, things like that, where adding that anatomic component to know more exactly anatomically what's going on, where I'm finding the problem can compliment and show some really good. And then the last thing I'll mention is I'm on a worldwide task force currently, that's looking to standardize the grading or severity scale for carpal tunnel syndrome. And one of the first things we did was we said, we're going to throw out ultrasound. We don't want that in our standard grading for EDX. Not that it isn't important, it is important, but I think that EDX stands on its own, and it's going to be able to give us a good answer in most all the cases. Occasionally, certain cases, adding ultrasound could give us more detailed answers, but it's not going to supplant it, no. Great. Well, I think that was a really good discussion, and Dr. Chung has his slides up. We're really excited to have you with us and finish on this topic. We're on track probably to have around 10 minutes left at the end for some discussion, so don't worry if I didn't get to your question. I'll also add a great talk, by the way, to Dr. Attaway that I see there, and we'll get back to the question about the norms in a second. All right, Dr. Chung, the floor is yours. Hi, well, thanks for the invitation. So my name is Tae Chung. I run POTS Clinic at Johns Hopkins. I'm neuromuscular, so I'm switching gears a little bit from more EDX discussion to more like broad neuromuscular. I'm also neuromuscular trained, so I'm going to be talking about POTS. I titled this as a final frontier for PMNR, so hopefully the next short 15-minute presentation, I'm hoping some of you guys, I convinced some of you guys that POTS is really the new frontier or new territory for PMNR doctors. All right, so now, and one of the reasons is actually probably you already see POTS patients, whether you have the diagnosis of POTS or something else, such as chronic fatigue syndrome, Ehlers-Danlos syndrome, which is hypermobile joint syndrome, chronic pain, whatever you name it as a physiatry, especially physical medicine, rehabilitation doctor, there's really no way you haven't seen this patient. These are really typical patients you might've seen today or last week, typical young female mostly female presenting with a very severe fatigue, brain fogginess, headache, chronic pain, everywhere. And if you're poking everywhere, they have all the pain. However, interestingly, all the known tests, we do MRI of brain and all entire spine, EDX everywhere, everything comes back as normal. And it's very frustrating because patients are very debilitated. These are usually somebody who used to be very active and healthy and typically some kind of infection. These days, more COVID infection, they develop this debilitating condition. Then nothing comes out and you start thinking maybe patients are crazy. Some of you already sent the patients to psychiatrists. Now so these are very common problems. I'm pretty sure you remember those patients in your clinic and POTS, first of all, is a syndromic diagnosis. I'm pretty sure most of these patients have POTS and it's not one single thing, the syndromic diagnosis, which means it's not a single diagnosis, probably it's a heterogeneous ideology there. But it's at least a diagnosis, not a symptom or a sign, it's a diagnosis. So I'm not going to get into too much technical details about this diagnosis code, but whatever that is, it's also a syndrome, but it's very characteristic, although each symptom can be very nonspecific, such as chronic fatigue and headache and brain fogginess, it's not each symptom doesn't tell this is actually POTS. It's a combination of symptoms that makes it very characteristic. So it's also important to rule out some other mimicking conditions, such as some cardiac arrhythmia and other things too. And I think a lot of you probably heard more about POTS after COVID pandemic, because there's a sharp rise in POTS, especially after COVID pandemic, and there's more increasing awareness. Even before COVID pandemic, POTS is one of the probably most common chronic neurological illness out there. Now what is POTS then? I'm going to get to the point. I see POTS as a more paradoxical sympathetic basal motor dysfunction. In fact, this is likely, again, this is a clinical syndrome, so it's not one thing, but likely this is peripheral autonomic nervous system problem. And in fact, you can see both low and high sympathetic tones, which is why I say it's paradoxical sympathetic dysfunction, such as low sympathetic tone, such as low basal motor tone. At the same time, you have a high adrenaline, so sympathetic tones there. Now what is happening there? I think there's pretty substantial evidence suggesting that underlying there's a basal motor dysfunction. And as you may remember, blood vessel basal motor tone is largely determined by sympathetic nervous system. And as you further remember from neuroanatomy class, this comes out of this short segment of the spine. It's called sympathetic chain ganglia. It sends out long axon. Part of it goes to blood vessel basal motor innervation. The other part goes to sweat gland. And from this short segment, it innervates to entire blood vessels in our human body, including brain and all the way to the limbs. And basically, there's, I mean, some evidence suggesting that the sympathetic ganglia level or axonal level, there's a damage there. So it's basically basal motor pump, basal motor dysfunction. What is happening in, again, in typical cases, either COVID infection or a lot of cases is after EBD virus, which is mononucleosis. And usually people get rid of the virus, but through molecular mimicry, such as Guillain-Barre syndrome, people probably antibody, you know, well, against the COVID virus, but some of the antibody may recognize their own body tissue, in this case, sympathetic nervous system, maybe their molecular structures are similar. They inflame the sympathetic nervous system as an autoimmune inflammation and causes POTS. So, and as a result, as a result of basal motor innervation and inflammation, you have a brain fogginess, a decreased cerebral blood flow. They have a, not only just brain fogginess, they have a really short memory, short attention and migraine headache as well. And also they have a severe exercise intolerance. They cannot tolerate, they cannot increase blood flow to their muscle, especially with the endurance type or cardiovascular type of training. Now, more fundamentally in cardiopulmonary physiology, there's a reduced cardiac preload because in the periphery, they cannot return the blood back to the heart at long. So they have a severe fatigue and exertion of dyspnea or air hunger. Now, at the same time, this is a more peripheral nervous system problem. There's in the central nervous system, there are various reflex arc is intact. And in fact, sometimes it's even exaggerated. So the brain is trying to compensate for this low basal motor tone by increasing adrenaline or increasing sympathetic tone at the central nervous system level. So that results in pretty much increased adrenaline and fight or flight symptoms and basically results in tachycardia or palpitation. Now, tachycardia in POTS is actually a compensatory mechanism that compensates for reduced cardiac output. So because of tachycardia, most patients, they feel a little bit easier, lightheaded, but they don't usually pass out. In fact, most POTS patients after a few years, they don't even really experience dizziness or orthostatic intolerance. They just feel very fatigued. Although on the tilt table test or standing test, their vital signs go crazy. Now, actually, tachycardia is usually not a problem in POTS patients. It's actually the adrenaline surge that causes anxiety and sleep disturbance. They wake them up at night and hold GI disturbance because it slows down GI motility. And that causes severe symptoms, make them very uncomfortable. So in summary, a lot of people, these people have a very multitude of nonspecific symptoms, but it can come down to low sympathetic tone causing brain fatigue and exercise intolerance. At the same time, there's a hyper-sympathetic or overcompensation of sympathetic system that's causing palpitation, anxiety, lack of sleeping and or GI problems. So therefore, that's why it causes paradoxical sympathetic problem. And that is actually posing pretty unique challenges for treating these patients. Well, first of all, of course, for lower sympathetic tone, we approach, you know, with a little more medications, sympathetic pneumatic medication that causes vasoconstriction such as midodrine or some stimulants such as Adderall, Vyvanse or methipenidate. It can increase adrenaline tone. At the same time, there are medications you can use for high sympathetic tone such as beta blockers, anxiolytics, antidepressants and other things at the same time too. But if you think about it, this is a paradoxical problem. A lot of times you use a beta blocker to tone down sympathetic overcompensation that could potentially worsen the basal motor symptom and vice versa. You use stimulants to perk them up and help with the fatigue and brain fogginess, but it can actually worsen their sleep pattern and GI problems at the same time. So that's actually why when you have POTS, there's no one medication that can address all the problems. So there are just a couple of things to consider when treating POTS is that this balance between hypo-hypersympathetic tone can be very challenging. And that's actually why I try to avoid polypharmacy. And oftentimes you'll see that people use medication that both brings down sympathetic tone or increase sympathetic tone at the same time. That's actually really not that because doctors really don't know what they're doing, but this is the nature of their problem and symptoms. Exercise, diet, rehab is a very powerful, important intervention, but at the end of the day, this is not a magical cure. The exercise probably helps because over long-term exercise treatment, cardiovascular training, it increases blood volume by up to 35%, which is huge, but because of their exercise intolerance, it's very difficult to get there. So this is exercise treatment for POTS patients. It's not, you know, like typical exercise treatment for other conditions. So to be careful about that. And as you can imagine, it affects a lot of different systems, GI or neurological, musculoskeletal system. So long-term, frequent multidisciplinary team is necessary to take care of these patients. Another interesting thing about this POTS is that any stress, both physical and mental, can flare up their symptoms. I mean, even the emotional stress likely through adrenaline reaction, whatever, it can actually trigger their flares and managing those, you know, stress is also a very important part of POTS treatment. And obviously, over-exercise, over-exertion is always one of the most frequent, common causes of flares. We're going to talk a little bit about IVIG. So like I said, not all POTS is autoimmune, but probably we suspect that a large portion of POTS is autoimmune. Basically, POTS, as I say, is a Guillain-Barre or CIDP on the autonomic nervous system. It's just that autonomic nervous system is difficult to investigate or, you know, take a look at it, which is why it's under appreciated. But sometimes immunomodulating therapy, such as IVIG, can provide significant benefits that other exercises and other things cannot provide. So now, why do I think Pulmona is an ideal field for POTS care? First of all, multidisciplinary approach. We are very, very familiar with this setting. Most POTS desiratories have this multidisciplinary setting. They work with the physical occupational therapists, oftentimes nutritionists as well. And like I said, briefly mentioned, physical intervention, such as therapeutic exercise, probably provides the most significant amount of volume expansion, significant benefits, and also lifestyle modification. I don't like the term lifestyle modification. It doesn't sound like actual medical intervention, but in POTS case, such as stress management, other things, it's essential for POTS care. And I think desiratories are probably the best, you know, group of doctors who can provide this type of care too. And also, first of all, I feel like PIM&R, I personally feel like we need to expand our territory. And at this point, no other, you know, specialties are claiming POTS as their own field. I mean, most of the POTS patients are currently seen by cardiologists and neurologists, but you can, I can guarantee I work a lot with these people. Nobody likes POTS patients, but at the same time, you're already seeing them. I'm pretty sure I, you know, you can diagnose POTS patients in your own country, in many cases. So we started a POTS clinic program in 2017, officially named as a POTS clinic program, based on PIM&R department. We grew quickly. We started as a one-man clinic, and now we have two full-time nurse practitioners, three attending doctors. We have a rheumatologist and even ophthalmologist in our clinic and collaborate with the neurology and cardiology and even psychiatry and GI doctors as well. In our own clinic based on PIM&R, we see more than 1,500 new patients every year and complete, you know, 5,000, 4,500 visits, which is probably, we are probably likely the largest POTS clinic programs in the country. And I think one of our successes is probably because we are based on PIM&R. Most other POTS or autonomic clinics, they are, you know, neurology based and they usually just diagnose and then keep them out of their clinic. They just don't have this concept or model where they can only tune in and follow up their patients with this multidisciplinary team. So, and also last, again, some updates, advancements is that, especially after COVID pandemic, there have been actually some clinical trials going on. Actually, prior to COVID, there is actually absolutely no sponsored trial for any POTS. Now, after COVID, there are actually three actively ongoing trials. And again, like I'm actually the lead PI for this first phase two study that's actually just completed. The F-cortisone one is actually a new immune modulating drug. Basically, it works similar to IVIG, normal IGG level. We just completed this patient recruitment. You know, in the next couple of months, we're going to have some preliminary data, unblinded data. Also, NIH just launched a very big clinical trial. It says recover auto, but it's post-COVID POTS trial. I mean, the eligibility criteria, you have to meet the POTS criteria. I'm also one of the full lead PIs of this one. So, there are two major ones and there's another third phase two study and also targeting post-COVID POTS. There are actually a few more coming. I'm talking to some other industries that are very interested. Just wanted to tell you that actually, I've actually got very involved in this clinical trials at this point. And I'm hearing from a lot of industry pharmaceutical companies that are seeing some opportunities behind this POTS. They are seeing money behind it. I have a pretty good feeling that there will be a lot more clinical trial targeting POTS. So, whether you're in academia or outside, this is a great opportunity. I can see that this POTS, you know, practice and field will bloom in the next few to several years. And I really think PMA doctors should really be the main people who are leading this disease, who really need this care. And patients are really suffering. So, this is the last slide that I have. This is very complicated. Look at this. Other than physiatrists, who else can do this better? So, this is my last slide. Thank you very much. Thank you. Yeah, you know, we have some immediate questions for you, Dr. Chung. So, maybe we'll, even though there's a few that are up in the queue, just since it's right after your talk. I believe the first question was referring to like an off-labeled treatment for POTS. Any thoughts on, I believe, low-dose naltrexone for POTS? That would be from Dr. Alajin. So, the first question about the off-label use of, are you talking about LDN specifically or in general? I'm just making that point. I think we have to, since this is for CME, we just need to point out that it's an off-label use, I think, before you, yeah. But just, I think they're just curious about your thought about that approach. Yeah, yeah, absolutely. LDN is one of the commonly used medications. Now, I actually had not been using LDN for a long time because I'm still very, I consider myself as clinician scientist and the rationale doesn't make sense. I try not to use it and their rationale has been, well, this is the low-dose naltrexone may have some anti-inflammatory effects and because it's potentially autoimmune disease, maybe it can lower the inflammation. I still don't believe that's the case. However, in my experience, people have been actually having some benefits in chronic pain. So, one of the things, a lot of POTS patients have a little, at least low-grade pain, constant low-grade pain. When they get LDN, it's a little bit of heroines. Not everybody gets benefits. Some people usually get their low, low-grade pain is like four out of 10, maybe downwards to three to two out of 10 kind of stuff. But at the same time, it's a really self-therapeutic dose, very low side effect profile, very safe drug. I understand this is off-label use, but because of the low side effect profile and little bit of potential benefit, I started using. So, if they don't really work, I stopped prescribing it, but almost nothing wouldn't hurt with this LDN. Great. I see there's been some really good chat too around neuromuscular ultrasound, which I think adds to the discussion we already had earlier. And also, one of my heroes, Dr. Howard, has provided an excellent answer too about that AANEM normative data, which I fully agree with. So, just for anyone's curious, just open up the chat. You can read through that. There's another question for you, Dr. Chung, about amplified musculoskeletal pain syndrome. I had to double-check that. Did I get that abbreviation correctly? Sorry. I don't see that one. Is there any association between POTS and AMS? And we see a lot of POTS in our pain clinic. Well, huge association, actually. I'm glad that Dr. Hulk asked about this. So, I think AMS is, my understanding about AMS is that it was kind of diagnosis more preferred or used by a lot of physical therapists. And I don't think a lot of physiologists or neurologists use that diagnosis. So, given that, I'm not even sure if there's an ICD-10 code for AMS per se, but I really think this is a great name and labeling that really accurately describes what POTS patients are experiencing. So, like I said, there's probably sympathetic nerve dysfunction. I theorize that POTS patients have actually a lot of increased pain perception and adrenaline and sympathetic nervous system has a role in modulating pain perception. And when people have sympathetic denervation or in general, autonomic nerve dysfunction, they do actually have some amplified pain perception, which can be quite debilitating as well. So, I think there's a huge overlap between POTS and AMS syndrome. And then one more question would, does POTS go away? This is from Dr. Jamieson. So, unfortunately, not likely. Currently, it also depends on the POTS definition too. Some people, I have actually observed several cases where they get the COVID infection and then likely the damage is the sympathetic nervous system they may have. But for the nerve, it may be just one time thing, but for the nerve to regrow, it's going to take several months. So, it may eventually go away. Rather than those rare cases, POTS doesn't seem to go away. I know like in my 1990s, a lot of pediatric studies have shown that one third of these POTS patients that goes away, about the same, gets worse. But that turns out to be probably not true. A lot of pediatric doctors, they don't see patients after they get out of the hospital. So, I think it's a little bit after they get out of the pediatric clinic, but it's usually waxing and reining. After they get out of the pediatric clinic, they might get better, but then they have a lot of flares, they come back. So, it's more accepted that most POTS cases, they don't really go away. Well, we're running out of time on the webinar. We had a really fantastic series of talks. It's a lot of great discussion. I see that there's requests for some contact information. It's in the chat. We can continue this discussion also through the community, where you can put questions or items up for discussion. I'm just like blown away by the talks tonight. Thanks everyone for their time. Turned out like well over 50 people today. Plus, it'll be available, I believe, as a recording online. And we have options for, I think, maybe doing something in addition to another virtual event as a community, which keep an eye out for. Plus, in person at the next Academy meeting, we'll have a meet up there, which will get scheduled when that becomes available. So, thanks everyone. And especially thanks to the audience for turning out tonight. There's a lot to do after work for you guys. So, really appreciate you spending it with us. And thanks to our speakers.

Neuromuscular

Electrodiagnostic Medicine

Community Meeting

Dr. Grant

Dr. Adeweye

Dr. Chung

Physiatry

Lower Motor Neuron

Spinal Cord Injury

Postural Orthostatic Tachycardia Syndrome

POTS