Well, maybe I'll get started so we can try to do all the cases in the time and we can keep this. I'm at Ginsburg, by the way. Here it is. I'm a pediatric neurologist at Akron Children's Hospital. I don't have any relevant disclosures for this talk. And as we get into the case, we can keep it informal. But if people have specific comments or questions later or, you know, more of a formal question, just maybe raise your hand and I can bring the microphone just so that if people are listening online or even just in the audience itself, everyone can hear you. Okay. So let's get into it. This is a case of a 17-year-old right-handed male coming in with dizzy spells and left-sided hand weakness. His history progressed over about two years. It was both sides, but left more than right. And he also had tremors, which were one of the main things that were bothering him when he originally came to attention. But he had also noticed he was having more trouble playing the guitar. He couldn't grip a 30-pound dumbbell, so he could do the curl, but he just couldn't hold on to it. He also, on review of systems, had migraineous headaches, some orthostatic intolerance. That seemed to be the dizziness part of things. He had had some tics in the past that had resolved. And he had some occasional forearm pain, but not any clear sensory symptoms, true paresthesias or numbness or anesthesia anywhere. He was, he previously had actually come to neurology attention like five years prior because of alternating hand weakness. His exams at that time were reportedly normal, and it was thought to be functional. And he also had a history of Tourette's, anxiety, and headaches. But again, the tics had resolved mostly. And there was no significant family history. So on exam for this patient, he certainly didn't have any functional components this time around. He had atrophy that was fairly substantial in the left more than the hand, more than the right hand intrinsics. There weren't any obvious vesiculations. He had normal tone, and he had an asymmetric kinetic tremor with some polyminimiclonus. And then his reflexes were sort of threes throughout. He had a Hoffman on the left. His plant responses were downgoing, and there were no pathologic jaw jerks, facial, or pectoral reflexes. And he had a normal sensory exam. Here's his strength. And to sort of summarize it, he had weakness that was worse on the left side than on the right side. And it was predominantly in the forearm and hand intrinsics. There was some trace arm extension weakness on the left side. And really, all other muscle groups, everything in the legs, all the other proximal upper extremity muscles, were normal strength. So at this point, I don't know if anybody wants to jump in with a differential diagnosis or what you would do. I met this patient in the EMG lab. If you don't want an EMG next, then you're probably at the wrong conference, but feel free to share that opinion. But maybe people want to see the EMG first, so I'll do that. OK. I know everybody uses different software and tables, so—oh, no, this isn't actually showing up. But at least for the people in the room. So these are the sensory amplitudes, and they were essentially unremarkable. So he had no sensory findings. And then here is motor nerve conduction studies. And so the left and right APB amplitudes were within our lab's normal range. There weren't any signs of demyelination there. And then for the ulnar, they were really, like, right at the edge. Probably a little low, but right at the edge. The FDI was actually a little bit lower, so the left FDI CMAP was only 4.4. And the right side was, again, just under reference range. His F waves were, at best, impersistent. They just weren't easy to obtain. And then here's his needle exam. And again, to sort of summarize, so really in the C8 to T1 muscles predominantly, he had chronic—pretty chronic—neurogenic changes and reduced recruitment. And he had just really trace spontaneous activity, a very rare fib, some fasciculations in a couple muscles, again, very infrequent. There were some changes in the triceps as well on the left side. But his C5-C6 muscles were basically normal. He had some chronic changes, I thought, in the cervical paraspinals. And then we just took one muscle, distal muscle, on the right side of the FDI, and that was overall similar, maybe slightly better recruitment than on the left side, which was the more symptomatic side. So the next question is, what would you do next, and if there's anybody who has some thoughts for the differential. It's not meant to be like a super weird narwhal-unicorn-zebra case. So if you don't feel like I'm really trying to trick you up, it's not meant to be super—yes? So, um, Hiriyama Monomulic emyotrophy? Yep. So I'm going to actually just repeat it out. I think that'll be easier. So vote for Hiriyama Monomulic emyotrophy. Yeah, and just to repeat for anybody who can hear, because of the restriction to mainly the C8T1, a little bit of C7 motor neuron findings, and now I already forgot the last thing that you said, so we'll get to it. Any other thoughts or concerns that anybody else had? Yes? Okay, so we're hearing concerns that maybe there should be more spontaneous activity for a motor neuron disorder. Does anybody have a thought about if there's anything else that they would do next? Any other diagnostic testing? C-spine imaging. Okay, so that's actually what we have next, so I'm just going to put that up. So on the left-hand side, we have a sagittal T2-weighted imaging of the cervical spine in a neutral position, and this part was actually read as normal. I would argue, and I know it's going to be difficult because the lights in the room are up, but I think there's a little bit of atrophy of the core. There's thinning of the normal thickening in the cervical area. On a good computer, you may be able to look and maybe see, is there any signal changes? Not definitely. The middle image and the right image are T1 post-contrast images, again, also of the cervical spine, but the middle image is in sort of neutral neck extension, and the rightmost image is with full neck flexion. Now, hopefully you can appreciate that there is this contrast that emerges posterior to the dura in the cervical spine right here, corresponding to the epidural venous plexus just filling with the contrast agent. So we took this to be confirmatory of monomialic amyotrophy or Hirayama disease. That was our final diagnosis, and then this patient, he didn't want to do any interventions at all, really. We just monitored for actually kind of a year and a half or so. We did follow with some hand dynamometry that remained stable. He did some occupational therapies. I guess I shouldn't say he didn't do anything, but he felt like the hand exercises improved his strength a little bit, actually. And then we did some Unix, which is motor unit number index, to just sort of estimate if there was any ongoing subclinical changes to his motor unit numbers. And you know what? They kind of stayed stable. There's some jumping around a little bit that I think is just a technical part of the study or maybe my lack of skill. But we followed him for a year and a half or so after that, and then he kind of like started a food truck and then gave up on that and graduated and moved on to adult land and I lost track of him at that point, but he was pretty much stable. So just a quick review of monomialic amyotrophy, aka Hirayama disease. Clinically, again, the classic features, it's an asymmetric chronic progressive weakness predominantly affecting those hand and forearm muscles because of the myotomes that it affects. And it's most common in males under the age of 20. The other features that you can see are, there are reports of patients with intervals of stability and then later progression, even years out. Some people will have paresthesias, colparesis, fasciculations, and tremors. And there are, actually now I remember what you had mentioned before, yes, there are sometimes some kind of like soft upper motor neurons signs or increased reflexes, I would say. The diagnosis is usually going to be based on the EMG showing that intraspinal canal process, but just really limited to those myotomes, C7 to T1, and again, predominantly the C8-T1 muscles and dynamic MRI with the dural displacement on contrast that I showed. And I don't know how it is for other folks at other institutions, but like, you really have to ask very specifically for this on your MRI order or talk to the radiologist because they will not do this by default and they won't necessarily know what you're looking for unless you're very explicit, but yes. He actually had one first where they didn't do it and I had to send him back. Anyway, treatment is, you know, all of this is a rare disease. There's not like controlled trials. A lot of it is very, you know, just opinion. But some people will do a soft cervical collar to limit neck flexion for a period of months. And for folks who may be showing severe progression or ongoing progression, there have been surgeons who have operated on this and either done an anterior fusion or posterior duroplasty. Again, I'd say the reports and literature are that these are effective, but the natural history of this condition is to stabilize at a certain point, so it's not always clear if it's the intervention or the natural history. The differential diagnosis usually involves things like juvenile ALS or other structural myelopathies. I'd say to go back to the question from before, you know, because it's so slow, I think it doesn't always have to have a lot of active FIBs or active denervation findings because it's just going on over years. And then in terms of the pathophysiology, again, it's a lot of hand waving, guessing, but it's thought to maybe reflect disproportionate growth of the cervical spine and the vertebral column when you're going through that big growth spurt. And then there's this anterior displacement of the posterior dora that maybe leads to chronic local ischemia or compression. And if other people have other hypotheses, I'm also interested in hearing those. So monitoring is usually part of it because the question is, do you do something more aggressive and interventional if things are worsening? And one guideline to just doing something like MUNI or consider doing EMG, handheld dynamometry makes sense as a possible option for me because those are some of the weakest areas. And so I'm also curious if other people have opinions on that. This is one study of MUNI in Hirayama disease that basically showed that the weaker muscles or the more effective muscles kind of stayed stable and the ones that were a little bit higher immunity values tended to just go towards that lower portion and just plateau, which seems kind of consistent with what you see clinically. And to summarize, so monomyelic amyotrophy or Hirayama disease, slowly progressive, plateauing lower motor neuron disorder. You can see, again, maybe some brisk reflexes, but it is really predominantly a lower motor neuron disease. Differential usually includes things like juvenile ALS that you might want to exclude and structural myelopathies. And then EMG and MRI with deflection, extension, post-contrast, sagittal images is what's critical for the diagnosis. And then you might consider using some of these tools like MUNI-MUNIX, dynamometry and clinical exams to monitor for the need for additional interventions. So thanks very much for listening. Does anybody have any comments or questions or other things that they've done? This is just now personal curiosity. I can also bring the microphone out, hold on. Yeah, it's purportedly more common in males. I think the thought process is that they go through that very rapid height growth spurt and maybe in those individuals that might structurally... Hmm, yeah. Did it work? Sorry, just to repeat for folks in the audience, Dr. Abdul-Hamid has had a few patients who are weightlifters, so she's asked some of them not to weightlift after they've presented. No, the patients I've seen have not been Asian, but I think we have a smaller Asian population demographically where we practice. Like what we learned is like it would be young people that practice. Yeah, but I wonder if that's just where it was one of those discovery things. So just again to repeat it, in Pakistan they have a very large population of these patients, 12 plus a year, okay. And do you do any other monitoring for these patients at the clinical exams or do you repeat EMGs or anything? Yeah, my last patient, who I saw with this, religiously followed the cervical collar for six months, progressed during that time, took it off, and then stabilized. Okay, that's probably it. Okay, thanks so much. I'm going to get on to the next presentation for Dr. Bonner. Alright, good morning. I'm Alex Bonner. I'm a pediatric neuromuscular neurologist just getting started at Cleveland Clinic, and I am grateful to be up here with these wonderful individuals today. So I'm going to talk about a case that I was introduced to in fellowship that I titled Foot Drop Don't Stop. I don't have any financial disclosures. So this case is about a four-year-old female. This was kind of around Thanksgiving leading up to the holidays. She had injured her left great toe and was limping around, but then it was not a major injury. I don't even have a lot of details about the injury because it was pretty insignificant. The toe was doing fine, but she continued to have this change in her ambulation after it healed. So she saw orthopedics, and ortho noticed that she was not able to actively dorsiflex at the ankle to a complete 90 degrees. But otherwise, her exam looked great. And they did notice a little bit of a funky toddler walk, but they just thought that that decrease in dorsiflexion was odd. So they said, all right, let's get an AFO, do a little PT. She did not do PT. She did get the AFO, came back, and the strength was actually zero out of five at this follow-up visit about six weeks later in her dorsiflexion and eversion. But plantar flexion, inversion, toe flexion, all of that is looking pretty good. She's four. She said she could feel the top of her foot. Take that for what you will. Nothing notable at the fibular head, and her straight leg raise was negative. Reflexes are all intact and symmetric. And otherwise, this kid's doing beautifully. So there's a pretty good thorough review of systems at all of these visits that I'm not necessarily going to talk through, but that I was looking through. And everybody was checking in on spinal symptoms, sensory, is there anything else going on, any other weakness anywhere, dysphagia. I'll let you know. They did a pretty thorough job, and this kid's doing great. Otherwise. So you're thinking, all right, this kid's got a foot drop. She's four. This is an interesting case conference. Where is it going to get interesting? Where are the details? What more do you have to tell us? So is there a weird family history, something else going on? And I don't actually have that much for you. So she was adopted after birth, so we don't have a lot of family history to work with. Maybe some prenatal exposure to marijuana, a little bit of early speech delay, a little bit of fine motor delay, but she has never had gross motor delays and is all caught up and doing beautifully now. She's described as sassy and typical, which is my favorite kind of toddler. But family is concerned that things are worsening during this period as she's having this ongoing evaluation. So she presents to one of our wonderful PM&R colleagues about 10 weeks after the onset of symptoms. And at this point, she's falling several times per day. She's happy-go-lucky about it, but she's having these frequent falls. She's pain-free. Again, otherwise, we're checking in on all these other symptoms, and she's doing great. No bowel-bladder problems, no other swallowing difficulties, extraocular movements, nothing else that we can kind of pick up on. But on a very thorough exam, noting that there is a little bit of weakness now in that plantar flexion on that side compared to the other side, and a slight asymmetry of the patellar reflexes as well. So we said, all right, well, let's take a picture of this lower extremity, see if we can take a look kind of at the sciatic, if maybe there's some progression of involvement, something slowly progressive in this age group. So we take a picture, and what you can see in this picture is on... I don't look at a lot of lower extremity bones, so I will direct your eyes to... Is this the laser pointer? This one? Okay. So for those who are not looking at my laser pointer, kind of the bottom right of the screen, it's the distal fibula. There's a bit of marrow enhancement there, and like every good radiology report, they described this could be normal, or maybe it's cancer. So we said, cool, right. So they were really saying that the etiology of this fibular finding is unclear, that you can see this in the setting of altered gait mechanics, maybe a stress injury. We know this kiddo has had altered gait mechanics, so perhaps it's all that, but you better make sure it's not a lymphoproliferative disorder. All righty. So then this kiddo was sent to get an EMG to see if we could localize a little bit further. So the EMG findings here, I did not highlight them very well for you on the slide, so I'll just talk through it, but on the sensory side, the last two nerves that we looked at are the left and right superficial perineal, and you can see that they're both really qualifying as normal, but that the amplitude is about half on the left side of what it is on the right. So we were saying, oh, it looks like maybe there's some sensory involvement of this process affecting the superficial perineal. And then we are seeing some amplitude loss on that left motor perineal involvement as well. No signs of demyelinating, nothing else particularly notable here. I put some waves in here just for fun. And then on the needle examination, again, we're seeing both active, a decent amount of active change, actually, in the tibialis anterior with some chronic changes in tibialis anterior and EDB with this little hint of chronic change in the left gastrocnemius as well. So again, the thought had been, what's going on with this sciatic nerve? There's a lot of this kind of perineal involvement, but then there seems to be almost this extension into things involving the medial gastroc. But she came to see one of our wonderful neuromuscular docs and, again, followed up with our wonderful PM&R team. And she was doing better with PT. Again, otherwise completely asymptomatic, starting to improve with PT. But our astute clinicians said, you know, I think we need to take a look at that spine because in up to 10 to, even in some reports, up to 40% of cases, there can be some sensory involvement, sensory electrodiagnostic involvement in an L5 root process. So we better take a look. So we took a look at the spine, and there's a little something funky there. And so then you look a little more. I know, even an interesting case conference, I bet you didn't see that coming. So this patient actually has this extensive, large, septated syringomyelia with an associated Chiari. So if you keep looking all the way up, this kiddo has a pretty dense kind of tonsillar herniation. And so she was sent over to our neurosurgery colleagues for a suboccipital craniectomy, a C1 laminectomy, and duroplasty. And again, I encountered this case in my fellowships. I haven't gotten a follow-up all that terribly closely. But from what I hear, she's doing pretty well. So take-home points. I think in kids, we want it to make sense, right, that this kid had no bowel bladder symptoms. She was having no headaches. She was otherwise this beautifully healthy kid. But we couldn't come up with an answer for why this had happened. So I think, you know, it makes sense to keep looking. Foot drop, we get kind of bored with on the adult side. But this four-year-old kiddo, she's happy-go-lucky. But we needed to figure out what was going on. And I'm glad that we did before something more serious began to develop. And then don't forget your anatomic variants. Oh, and this has been described in the past as well. It's quite infrequent. But I was finding some case reports in the literature of pediatric patients with Chiari malformation and syringomyelia who presented with this abrupt onset unilateral foot drop. They had some more clear searing-like symptoms. So the diagnostic process was a little faster for these kiddos. But it's been reported. All right. That's all I got for you guys. Thanks for joining. Yeah, that is always very tricky. I tend to ask, if a kid's not potty trained, there's only so much you can do. But if they're potty trained, I try to just get a sense of if there have been any changes. Because potty training, I have a toddler, potty training is not going well. And so it doesn't happen overnight. So just trying to get a sense of was there a change, wherever they're kind of at, their current stable level of potty training. Has there been a change in that? More accidents than usual? Can we attribute it to a stressor? I think it's tricky. And maybe that was one of the reasons this, maybe that would have been more helpful information if this kid were not this age. Up next, we have the lovely Dr. Hoda Abdul-Hameed from Pittsburgh. Hello everyone and thank you so much for being here and I want to thank Dr. Mosher and wishing her a speedy recovery for inviting me for this too. So today we're gonna talk about a case of a pediatric EMG and I titled it Creepy Crawly and Leg Weakness. I work at UPMC Children's Hospital which is part of the University of Pittsburgh. I serve on an advisory board for Sarepta Catalyst Pfizer but has nothing to do with this presentation. So my patient summary, I think you guys know where Pittsburgh is located. So there's a large Amish community close to us and this patient came to us from Northern West Virginia. She's a five-year-old previously healthy girl who presented for further evaluation of weakness. She presented to the emergency room. The parents give us the story that three days prior she had difficulty walking and she was dragging her feet on the floor and also noticed that she was not able to use her scissors for her arts and crafts and then one day prior to coming to our emergency room her weakness continued and by that evening she was refusing to walk. They did take her a couple of days earlier to the family doctor who gave her a shot and some pills but we don't have any exact information about it per her father and she continued to progress and get weak. Since she was not any better she was taken to an outside hospital for an evaluation and then transferred to us. In our hospital in the emergency room they noted that she had some diffused target-like lesions with distal and proximal weakness and diminished reflexes and so probably they called our fellow who came to the emergency room and they requested an MRI of the spine and that showed ventral root enhancement which in most cases again a lot of times even before seeing the rest of the story of the patient but many times we get at the end of the comment of the MRI this is consistent with GBS and this is exactly what this MRI report said which is sometimes like yeah everyone sees it and then they say oh this is GBS which you cannot have consistent GBS on MRI I'm sorry. So of course we proceeded since there was no structural lesions we proceeded with a lumbar puncture and that showed only one white blood cell, zero RBCs, glucose was normal at 85 and protein of 39 so it was not elevated and they even sent Lyme titers and that was negative and you may or may not know but Pittsburgh now is leading the country in Lyme disease as an endemic area. So this was presumed Lyme even though it was negative and the patient was admitted to the hospital to receive IVIG at a dose of two gram per kg over four days. Would you like any other investigations? EMG, thank you, well they did consult ID instead of me so they consulted ID and ID said this is quotations as she's a cute five-year-old who presents with three days of ascending weakness hyperreflexia and spinal root enhancement on MRI with normal CSF which is a red flag right and evidence of multiple erythema migraines rashes on exam. Her presentation and story are most consistent with GBS associated with early disseminated Lyme disease. This is a rare association however it's been described. Her rash is virtually diagnostic of Lyme disease. So of course IV seftraxone for Lyme was prescribed and then they sent CSF and serum Lyme serology. That was still negative. So the patient actually continued to progress and in the hospital she actually became even weaker and we had to admit her to the ICU. She got intubated and it was very difficult to extubate her even like 10 days later. That's when they called me and said hold on you know I think we should see this patient. She has GBS we give IVIG should we do plex now and I said no wait and then let's do the EMG first. So her EMG showed diffused motor axonal neuropathy with very low amplitudes. There was like 0.1 very low and then her sensory nerve conductions were normal. So it makes you think you know maybe this is a variant of GBS but her needle exam was very interesting. So she had increasing social activity with positive sharp waves and fibrillation potentials and she had short brief motor unit potentials and mostly normal recruitment but she wasn't really moving much but whatever we could get. There were occasional large motor unit potentials but it was hard to assess but definitely there was increased insertion activity. At that time I said okay this EMG is concerning for something else. It's not really typical of Guillain-Barre syndrome and I said you know we should look for something and this could be a presynaptic defect such as neuromuscular junction blockade. I did not do RepStim, Dr. Ginsberg, I should have, but it would have been a stimulated RepStim. Yeah, and I did not. They do produce honey, but she was a five-year-old. Yes, they have of course home cans. She's Amish, but she didn't eat of it. She didn't eat any of that. She had ptosis and some weakness, but it was mostly the lower extremity. That's how she presented. It was not bad, but that's a great thought, but that's a great thought, but the EMG should show maybe more higher amplitudes and motor unit potentials consistent with more neurogenic. Excellent, yeah. Right, so yes, that's a great thought. So that's in the differential. It's very rare in children, and it doesn't progress as fast. So this happened over three days. That's a clue here, and also the geographical distribution and the name of my presentation. Say that again. Yes, okay. So our fellow went back because of the EMG findings and worsening of her symptoms and the geographic distribution, and he said, Eureka, I found it, and they did find the tick. So this was a huge tick. That actually, I mean, no it's not, but it's not the actual picture, but actually our radiologist at the time, he swears that he saw it on the MRI because we ended up imaging her. I couldn't find that picture. But yeah, the patients, I mean luckily we found the tick. It was in the back of her neck, and so it was a tick paralysis, which is pretty rare, but that was actually our second case in the hospital. But tick paralysis is very rare. There's like probably a few cases reported in the literature in the U.S. It's more common in Australia, but it's related to a larger tick, so we can easily see it and find it. These are the name of the different ticks. Dermacentor, there's different names for them, different kinds. Rocky Mountain Wood tick and the American dog tick. In Australia, it's a scrub tick, which is Ixodus. It's the saliva of the tick while it's feeding, but also because it's also excreting at the same time, so it's the neurotoxin, and it's an engorged female tick during feeding, which also with the Lyme disease, it's still an engorged female tick. Then it causes a presynaptic blockage to the neuromuscular junction, and the clinical presentation is usually fatigue, progressive weakness, ataxia, so the differential diagnosis, I mean, essentially is what the ID and us thought initially, which is Guillain-Barré syndrome, botulism, just like you mentioned. Of course, acute flaccid myelitis or polio, but we don't have polio in the U.S. anymore, luckily. And then myasthenia gravis, less likely, and spinal cord lesions. So interestingly, this patient, I didn't want to confuse you more, but she did have an arachnoid cyst in her lumbar spine, so that also can be a red herring. So the treatment is actually, you have to find the tick, because the earlier you remove it, the earlier they will actually improve. So you, if you remove it within 24 hours, they can improve right away, but if it takes longer and they progressed as much weakness as our patient, it may take a week or 10 days for them to improve, but they eventually will improve. And you have to remove it carefully, so you have to get the mouth of it out, so usually use a small tweezer or, in the past, they used matchsticks, but I don't think we should do that, but, and make sure that to pull it slowly. And then you give it to the ID doctor and say, you know, can you identify it? And then supportive care, of course, for respiratory muscle weakness. And the improvement usually can occur as early as within 24 hours, but depends on the length of weakness and how long the tick was attached. And then you recommend, of course, protective clothing and remover of any ticks as soon as they come in from outside. If you have a dog, then you have to inspect it as well. So this is my presentation and I'm happy to take any questions. I have a question. Do you think that the ventral root enhancement was real or not real and just an artifact? That's a great question. So sometimes the MRI is performed after the lumbar puncture, and I do believe sometimes that can give you some enhancement. It probably was not real, probably was very faint, and it was not real, but that's, we see this often. It depends on who's the reader and whether or not it was done after the lumbar puncture. So I cannot explain it all the time. Can you just summarize your thought process as to why it wasn't the axonal variant of GBS versus a presynaptic or a neuromuscular junction? Right. So actually the thought came to us that this is, you know, AMN and that is an axonal variant of motor axonal neuropathy. It's the EMG findings because you do see increase in social activity. The motor units are actually quite small and it shows they look almost myopathic. So you have a neuropathy, but with myopathic motor unit potentials, that makes you think of a presynaptic neuromuscular junction blockade and instability and the, and if you can do single fiber, you will see the significant deterrent, but yeah. That's a great question. Actually, I'm not sure I know the exact answer, but it's the location. I don't know if you develop immunity, probably not. It's another type of, I mean this tick paralysis is presynaptic and she will probably develop new synaptic connections and junctions. But because of the location, yes, it can happen again. So we did warn them about that. Thank you so much. Okay, so I'm the next presenter, and you'll excuse me if I don't go on stage tonight. I found it to be a little dangerous up there. I am Catherine Mosier, I'm a pediatric physiatrist from Akron Children's, I work with Matt. And before I give my presentation, so I don't forget, I also just wanna mention that I am the co-chair of the Pediatric Neuromuscular Task Force for the AANEM. I'm so grateful to you all for being here today and being a part of our programming, and I would like to formally invite you to please make recommendations or pitch ideas for next year's meeting. They are due by November 15th, believe it or not. So it comes up pretty quick, so while you're here, be thinking about things that you would like to see for next year, and please log into AANEM and pitch those ideas. Also, this is our second year doing this session, and I think it's been really well-received. So if you like this, please volunteer to be a case presenter in the future, okay? All right, so my case is somewhat simple. It's just a case that kinda threw me for a loop because I assumed I knew what was happening, and I wasn't, and part of what helped me was neuromuscular ultrasound, so thought I'd throw that in there as demonstrating how it can be useful clinically as an extension of our study. I do not have any relevant financial disclosures. So the history is this is a 16-year-old male who presented with numbness and weakness in his left hand that occurred after he had fallen asleep about eight months previous to this visit, and he had been, he really was passed out for a minimum of six hours. This is a young man with a drug history, and from the best that he could tell me, he had been leaning on his elbow in a chair and fell asleep. And then when he woke up, he had significant numbness of the whole forearm and the hand that lasted for about a month, but then he had persistent numbness in digit five and weakness in the pinky finger with grasp. So I think you all can imagine why I thought, oh, this will be easy slam dunk, right? He also had this history of some leg and calf pain that happened around the same time, maybe before, not a very good historian for probably obvious reasons, which seemed like it was unrelated, but I just noted it and did a little bit of study there to make sure he didn't have something more widespread. And his past medical history was significant for this substance use disorder and anxiety. So on his exam, his strength was really five out of five throughout his upper arm and wrist. He had intact finger abduction on the right, on the left, his finger abduction was a little bit weaker, and bilaterally, he had a little bit of thumb weakness. He had numbness to light touch in the left digit five and middle half of digit four. So your classic ulnar distribution and otherwise no numbness in the remainder of his upper extremity. And then his lower extremity was pretty much intact, so. Next slide. So anybody wanna weigh in currently on the differential? Everybody, I assume, would pretty much be thinking this was a ulnar nerve lesion at the elbow, correct? Yeah, so. So this is what we found. He did not have a drop at the elbow. Sorry, I'm having a hard time seeing from my position. Sorry, I thought I got rid of those. Yeah, so there's the key thing. So at the wrist, below elbow and above elbow, there's no significant drop. So we had to go higher. We went into the axilla and you see there's about a 50% drop in amplitude. So, and then the EMG, there were some minimal changes and a little bit of polyphysicity and increased, or and reduced recruitment pattern. And so here's the tracings. And so if you look at the second from the, yeah, right there, thanks, Matt. You can see you have this big drop between, you know, the axilla and the above elbow site. And then this is the ultrasound findings. So, and Matt maybe can also point out. So there was increased diameter and in the short axis. And if you look at the long axis, you can see there's kind of some swelling in the middle there that you can appreciate. And then this should be a video. So that's a short axis video across the area of swelling. And then the next slide should be a long axis video. So this is between, it's in the upper arm right here, yeah. So I just thought that was, you know, kind of a unique and cool finding to share. I have no idea then how he was laying for this six hours or more, but obviously he was not putting most of the pressure on the elbow. So, any questions? So if you watch this video, you can see, can you sort of point out where the nerve is, Matt, so that... Yeah, I'm just trying to highlight it over here. Here's the ulnar nerve in cross-section, and just scrolling more proximally, I think, on this one. Yes. It just starts to, it starts to thicken here. Yeah. Can you see the girth starts to increase? And maybe even decrease in echogenicity. Yes. So the fascicles become not as obvious. Well, and I, so I'm glad you brought that up. I did put that in the report because he had the thumb weakness, which was not explainable on this basis. So I did mention that if he had, you know, similar episodes that it would be a consideration to look at, you know. Well, and he had no follow-up, he just disappeared off the face of the earth after this, so I don't even know what happened. Do you want to show the longitudinal? I'll show the longitudinal, just for anybody who didn't hear the audience comment, just that maybe 40% or so of HIV patients may be asymptomatic or have asymptomatic lesions. Okay. Wait, sorry, long, hold on. sausages out and then gets thinner again. I thought it was kind of interesting, too, the duration for that conduction block, and I think there was a paper and a podcast recently about the natural history of conduction block and ulnar neuropathy as the elbow out of the Dutch group. And I want to say that they found that some of them really lasted like a year or some very long time. Somebody else will correct me and will remember it better than I am, but that some kind of lost a really long time. Yeah, I mean it is really interesting because he wasn't very weak for the degree of conduction. Okay, so this is the last one. I always get to go last because I always turn in my stuff last. So I didn't even bother playing coy with what this was. If you look in the program it says what it is. So I'm very grateful that there's someone that's an expert in Lyme disease in here because I am not. I am a pediatric physiatrist out at Texas Children's. So I named it here back pain neuropathy and an unexpected diagnosis, but it's really not unexpected if you've looked through the program. And I kind of spell it out everywhere. But to overview, we'll go through the case, the EMG findings, and then a discussion as well. So this was really unusual for me because I'm from South Texas. We saw an 18-year-old female from the Rio Grande Valley. If you guys don't know where that is, it's kind of down by the Texas border or the Mexico border who was previously in good health until February 2023, give or take, when she developed a rash attributed to insect bites. Improved over time, but she was hiking with her family out in Mexico over the summer. Afterward, she developed headaches and congestion. She was treated for kind of a sinus infection with antibiotics. She said she thought it got a little bit better, but symptoms kind of progressed as far as pain to severe low back pain. And they thought it was kind of due to a musculoskeletal cause. She was treated with steroids, didn't really improve. Kind of a background on her, she was a dance major at an Ivy League school before this. So MSK-related pain wasn't completely out of the picture. At her outside hospital in the Rio Grande Valley, she continued to have worsening severe pain. Pain was just kind of the big predominating symptom when she met us and kind of from the beginning. So much so that I think people thought she was FND adverse. She was also reporting kind of subjective numbness in her feet and hands. And then she was having some kind of vague cranial nerve abnormality. She said her eyes felt weak. She was saying she had dysphagia, but again some of this was all pretty hard to see on physical exam. There's a lot of fluctuations with her. So at the outside hospital, they weren't sure what to do with it. She was requiring opioids and for her pain. So they then just kind of decided to to go to IVIG just to see if there was some sort of improvement, which she said she did see a little bit, but it didn't last. So this is what prompted her admission to us at the end of May for further workup. And again, I'm not a neurologist, so I will not try to go into this too much, but some of the findings that we did get when she was admitted to us, she had a normal brain and spine MRI. She had positive Lyme titers both in her serum and in her CSF. She had elevated protein and albumin in her CSF, kind of marginally elevated white blood cell count and 84% lymphocytes. It seemed pretty clear to us at the time that she was diagnosed with Lyme disease in the neuroborreliosis that comes with it. And ID started her with her appropriate therapy with IV recephin and doxycycline. So kind of at the beginning, we're all like, oh, this is pretty straightforward neuroborreliosis or some neurologic manifestations of Lyme disease. We thought maybe the pain was related to Lyme arthritis and Lyme radiculoneuritis. All of our residents that wanted to do pain were really excited to see her. And they thought maybe, you know, the cranial nerve findings were just typical cranial neuritis and the numbness could have been kind of the axonal sensory motor polyneuropathy that you can see with Lyme disease. However, she continued to get worse. When I was involved, she was telling me she couldn't move things. She was telling me her pain was really severe. But oddly enough, when our resident did his strength exam, he found her five out of five. She was reporting a lot of numbness and paresthesias and we could not get reflexes on her for anything. But she kept telling us it's getting worse. My pain is terrible. I cannot move. And just to clarify, my resident was a college baseball player and he was huge. So it wasn't like me going up there and being like, oh, I think she's strong. This was a much larger person than me. So yeah, so despite therapy, appropriate treatment, she continued to get worse. The pain just continued to spread. It was to a point where she couldn't eat. She was losing a lot of weight. People were kind of actually worried about whether this was FND with some like eating disorder overlay with her ballet history. So it was kind of unclear for us at the time. Eventually, she also did develop urinary retention. I think that's what kind of raised a little bit more alarm bells. She did have to be straight cathed. So at that point, they ordered an EMG. I think the tracings kind of tell a pretty good story. She had no sensory responses. We were able to be obtained. And you can see on her motor findings here, there's some pretty clear conduction block, we thought, as well as some temporal dispersion. All of her latencies were pretty delayed and her velocities were pretty slow as well, as well as some small amplitude findings relatively. Her F waves were prolonged. The only one we were able to obtain was in the right median. And so then we're like, maybe there is some axonal findings. But when we moved on to the EMG, there was no denervation seen. It was all pretty reduced recruitment. We weren't able to do too much needle exam just because she did tolerate it so poorly. Pain, again, was just a huge barrier for her to get testing done. So my colleague was the one who did this study. She felt the findings are most consistent with a severe sensory motor demyelinating polyneuropathy affecting both upper and lower extremities. She felt just based on the degree of slowing that this was probably more of a primarily demyelinating process. And all of the findings did seem to support Albert's criteria for AIDP. So then I think things kind of got interesting for me as someone that does not see Lyme disease and does not see a lot of Guillain-Barre mimics in the pediatric population. I don't know if it's more popular for adults, but for me it was kind of new and novel. And our neurologist and I had kind of some fun talking it through. But we were wondering if this was, is this neuroborreliosis presenting in an unusual way? You know, if you did a lit search, it looks like it could present as a demyelinating neuropathy, even though classically it's more a sensory motor axonal neuropathy. Or is this kind of Lyme disease triggering Guillain-Barre, which apparently can also do as well, as opposed to like campylobacter jejuni, maybe Lyme disease triggered this immune reaction that then set off Guillain-Barre. So, so we kind of went back and forth, kind of more of an esoteric discussion because in the end we ended up treating her with everything. She continued to get her Lyme, her Lyme treatment as well as IVIG that had to escalate to plex. However, kind of as far as summarizing the findings for the EMG, I think because we felt she didn't get better with just the treatment for Lyme, that there was probably a AIDP Guillain-Barre component as well. And so just to summarize for me, since this was again kind of new and novel for me as someone from South Texas that does not see Lyme disease, that Lyme disease can present with this neuroborreliosis in 10 to 15 percent of cases, and that there are peripheral nervous system manifestations. I felt they could be pretty challenging because it can mimic other diseases such as Guillain-Barre, which I did not know. And I wouldn't say it's poorly described, but I just couldn't find a lot of literature that described what the neuropathy looked like. A lot of it was just talking to other people and them giving me a little bit more information. Also, we were lucky that she could give us a history of a tick exposure or rash, but it took actually a while to elicit this from her. And honestly, at this point she was so pan-positive and everything in her review of systems that it was kind of hard to pinpoint that. So I just thought it was interesting to include Lyme disease and differential diagnosis of Guillain-Barre in appropriate settings, such as if you are from an area of exposure, mostly the Northeast, not where I'm from, or if symptoms are refractory to appropriate treatment. Additionally, pain being a large component of her presentation, I think also kind of pointed them more in that direction. I do also, since we did also talk a lot about Lyme disease previously, I would also raise the point that I thought it was interesting that I think EMG really helped her in the sense that everyone really did think she was FND. And I think EMG's role in FND is actually really interesting because it's been a big thing in our institution. So had she not had those findings, I think people really would have gone more down the FND route, maybe not have taken her symptoms quite as seriously. So I thought it was nice to have that EMG support for her also. So in the end, what ended up happening, she was really slow to recover. She ended up going to TIR, which is our adult counterpart of the rehab facility. Years later, I guess it's been about a year at this point, she's still not quite walking. For some reason, it's just been really refractory for her to get better. But we haven't been able to see her in person since she is at the adult facility now. So thank you. No, she actually went back home to the Rio Grande Valley. So besides the adult PM&R docs telling me how she was doing, we didn't get to see. But yeah, good question. And I think there's one back there too. What were her conduction velocities? I'm sorry, I can see back here. And then did y'all consider repeating? Yeah, good question. She didn't improve. Yeah, good question. So her nerve conduction velocities were as low as 19. And I think the fastest we got was 31. We did consider repeating just because she was so slow to recover. But because I think it was just so poorly tolerated. At this point, she was on a pain pump. We thought we might have to sedate her. We didn't end up repeating it. Yeah, no, very good question. Yeah, we thought about it. But I think just because of the pain, we ended up deferring. Also, I think some people did feel like she was getting better. I think from a rehab standpoint, we noticed her trunk was super weak. So we kind of argued that point. But on MMT, she did okay. So it was really equivocal whether she was getting better or not. Just functionally, she couldn't even sit by herself. So we kind of are we kind of disagreed over whether or not she was or wasn't getting better. Yeah, good questions. Okay, well, thank you guys. I was used to being like running over time. So nice to have extra time. All right, well thank you everyone so much for attending, appreciate your participation. Thanks to all our presenters today and please feel free to reach out to me if you have any questions or suggestions for future meetings.

pediatric neuromuscular disorders

peripheral nerve diseases

Hirayama disease

dynamic cervical MRI

syringomyelia

Chiari malformation

tick paralysis

neuroborreliosis

EMG

neuromuscular ultrasound

electromyography