Hello, everyone. Good to see you all, at least virtually. Hopefully soon we'll be able to see each other live. My name's Mike Salino. I'm a physiatrist at Moss Rehab and an associate professor at the Sidney Kimmel College of Medicine. I would like to welcome you to the horses and zebra session in the Spasticity Zoo. I have assembled some of the nation's experts in spasticity management. I am lucky to call each of them friends. If there is a difficult case that I'm having a problem with, these are the folks who I call upon to help sort through the difficulties. Each one of the faculty members tonight is gonna compare and contrast a series of cases. The horses will be relatively straightforward cases in which the answer may be obvious, and the zebra case may be something that looks normal but has an unusual twist and unusual presentation. This will be used for educational purposes to understand both the typical and atypical presentations that we encounter in a spasticity management clinic. Again, I'd like to thank you for joining us here. I'm now gonna turn the floor over to individual faculty members who will introduce themselves and their affiliations. First, my colleague at Moss, Dr. Dan Moon. Hi, my name's Dan Moon at Moss Rehab. I work in the GATE Lab, the Motor Control Analysis Laboratory, and I do intrathecal back-lipid pump management. It's nice to see all you guys. Here are my disclosures. So I sort of miss the instructions, and I'm probably talking more about fruit bats and penguins than I am about horses and zebras, but I do see a wide variety of cases, and this case sort of struck me as interesting and worthy of presenting for this session. Here is a 35-year-old male who was found down with HUNTESC grade five subarachnoid hemorrhage and an intraventricular hemorrhage resulting in a disorder of consciousness, seizures, central storming pneumonia, and he received a left craniectomy, MCA clipping, tracheostomy, PEG-2, and required multiple teeth extractions. I was consulted to see him because he presented with repetitive biting of the lips, leading to skin breakdown and bleeding inside the lip, and his team was wondering if he would benefit from chemodermabrasion of the jaw muscles. However, if you look at him, this is sort of a brief physical exam, you can see that he has some skin breakdown, primarily on the inner surface of his right lower lip. So therefore, I'll be talking about not necessarily horses and zebras, but I guess more like penguins and fruit bats, orofacial movement disorders. These are a group of conditions that affect the motor aspect of the trigeminal, facial, and hypoglossal cranial nerves. There are three major ones, bruxism, which is probably more of your horse, which is the most common, but there are also oromandibular dystonias and orofacial dyskinesias. Bruxism is primarily a cranial nerve five disorder. There are two types, sleep bruxism, which obviously is associated with sleep, typically grinding, and awake bruxism, which is a subconscious stereotypic mandibular activity during wakefulness. And the individual can actually voluntary control and terminate it when they're aware of it. EMG findings will show rhythmic masticatory muscle activity with a frequency of about one Hertz. There are a lot of risk factors associations with this, such as stress, anxiety, maladaptive coping skills and personality traits. Some drugs are associated with this condition, as well as obstructive sleep apnea and GERD. The goal is to prevent tooth destruction, alleviating pain or headaches. Unfortunately, oral appliances, which we typically see used, provide only short-term relief, and underlying causes such as CPAP, such as sleep apnea should be addressed with a CPAP or a proton pump inhibitors to address the GERD. There's some evidence for clonazepam, clonidine, and gabapentin, but again, this is limited. There is some evidence as well for botulinum toxin injections, but this only reduces intensity, but not completely eliminating it. EMG-based feedback and stress reduction are also helpful. Another horse or zebra, as you can call it, is oromandibular dystonia. This consists of involuntary repetitive sustained muscle contractions, resulting in abnormal posturing. There are many different types of this in the face. Some result in jaw opening, closing, or deviating. Others are associated with paraoral movements, as well as lingual movements. These are commonly triggered by talking, yawning, chewing, or swallowing, and usually increase in intensity during stress and throughout the day, as well with emotionally upset situations or fatigue. They usually disappear during sleep and are milder in the morning. Oftentimes, these are in combination with blepharospasms, and there are many different causes. Primary or idiopathic usually don't have an underlying cause identified, whereas secondary, you know, the most common one is tardive dystonia, which is associated with neuroleptic use, but they're also perfectly induced, post-anoxic, neurodegenerative, and head injury-associated oromandibular dystonias. Botulinum toxin injections are considered first-line, but again, there's limited evidence. And finally, you have orofacial dyskinesias, which are involuntary, rhythmic, repetitive, stereotypic movements of the face, lip, and or tongue. These can range from being barely noticeable to resulting in complete social or functional impairment. Causes can be primary or spontaneous, which there is no underlying cause identified, but these are common in elderly, people with intellectual disabilities, dementia, autism, and schizophrenia. They're also perfectly induced dyskinesias associated with edentulism, otherwise known as missing teeth, poorly fitting dentures, and oral pain. If it's acute drug-induced, this usually occurs within 24 hours to five days of introduction of the agent. Tardive dyskinesias, which is what we're most familiar with, are due to prolonged continuous exposure to a dopamine receptor-blocking agent, and they persist and sometimes even worsen if the agent is removed. Treatment includes optimizing oral health using agents such as tetrabenazine, imantadine, or clonazepam, and botulinum toxin injections, but again, these all have limited evidence. Going back to this patient, he has what I assume to be a primary or spontaneous oral mandibular dyskinesia. This is based on sort of the pattern, but also the fact that he doesn't have any other underlying conditions other than he has missing teeth or edentulism. We reviewed his medications, and nothing really comes out as being a cause of this, and he's already on imantadine and gabapentin for reasons other than dyskinesia, and this should help lessen it, but it continues. And in addition to that, the area of breakdown already had a tooth nearby. Therefore, we suggested using botulinum toxin injections to the orbicularis oris, and only over one site where the skin breakdown was occurring. Reviewing this muscle, it actually has two parts. The orbicularis oris upper pars or peripheralis region or the marginalis region. Figures on your right side of the screen show the activation of these two portions of the orbicularis oris muscle. Pars peripheralis results in a puckering type of action, whereas activation of the pars marginalis results in inversion of the lips, which is similar to what's seen in this case. Here's a cross-section of these muscles shown on your left, and you can see the pars marginalis is right inside the lip itself, underneath the vermilion. And on your right is the injection protocol, and for this case, we only used one site using a very small amount, which is convenient. Here's another case of a oromandibular dystonia. This is a lady with a history of anoxic brain injury. What's not shown is she also suffers from blepharospasms. In addition to improving cosmesis, the botulinum toxin injections also improved her speech comprehensibility. For this patient, fortunately, this case was self-limited. He did not require repeat injections, and this is probably because the oral pain improved and therefore he did not require repeat treatment. However, orophacial movement disorders can be associated with central nervous system disorders, medications, as well as peripheral nerve disorders. It can affect speech, eating, swallowing, hygiene, and self-esteem, and therefore it's important to identify and address these early to prevent further complications. Thank you. Here are my references, and I'd like to acknowledge Dr. Siegel and Dr. Marino for their thoughtful discussion in sending me these patients who are very interesting. Thank you. Our next presenter for the presentation is my colleague, Dr. Rez Farid. Thanks, Mike. I'm Dr. Rez Farid, University of Missouri in Columbia, where again, shockingly, it's San Diego-like here, 72 degrees and sunny, Chamber of Commerce Day. So I have about 130 or so patients who have pumps. I take care of spasticity. I do nerve blocks and botulinum toxin in pumps in kids and adults. So today, my case is gonna be kind of a combination. It's a combination case, really. Okay, so the whole zebra thing and the horse thing, sometimes it's a mix. So I think this is a mix and it bugged me. So I wanted to get some help from some experts too. And this is my case. This is my disclosure. I used to do some talking for, there hasn't been much of that going on, but we'll still list it. So this is a 57-year-old lady who had MS. She had MS, I don't know, seven to 10 years, if I remember correctly, and she was ultimately referred to me. She had severe hip flexion and adductor spasticity and kind of, in CP terms, a little windswept over to the right side. Hips at about 70 degrees, knees at 90 degrees, and I couldn't move her. Any type of extension of the hips or trying to spread the legs apart was met with tremendous resistance. And it would take several minutes for that just to calm down, to get her back to where she started. I'm gonna add here, she had a previous MRI that showed MS plaques up and down her thoracic spine. She had tried oral medicines, baclofen, tizanidine, ciproheptidine, and some botulinum toxin, et cetera. And she's just the lady you look at, and you know how you do this long enough, you see a patient, you're like, wow, you need a pump. You're just a pump candidate. You just have to have one immediately. Here we do screening doses. Some would argue you don't have to, but I do it, and I'm glad I did it in this case. So I set her up for a 50-microgram screening dose, and this is what I got. I took this lady who was stuck as stuck could be, and turned her into this. And I'm happy as a clam. I'm like, my gosh, look at this. I can spread her legs apart. I can move them up and down. Boy, I can't wait, and neither could she to have a pump. Now, she still had some end-of-range soft tissue contractures, but I thought to myself, this is the day you go home and you say, this must be what surgery feels like. You take somebody who's miserable, you get this huge ego trip about how wonderful you are, and you're like, wow, my goodness, look at me. So she gets a pump, and as I sometimes do, I say, you've gone through this trouble having a pump. I wanna get it titrated as fast as possible, and here in Missouri, we're able to do that. We don't get a whole bunch of insurance resistance. So I brought her into inpatient rehab and titrated her up 20% every day, just hoping to get there. Next thing you know, I'm at 300 micrograms, and I keep turning her up. I change her to flex dosing, and I have her on 500 micrograms within two weeks after implant. I'm like, wow, I usually don't turn people up this fast, but every day I go in and see her, and she just looks exactly the same as she did prior to the trial, and of course, worst case scenario, she looks at me. She says, I have a lot of faith in you, doc. I believe in you. You're gonna take good care of me, and I'm sitting there walking out of a room thinking, ma'am, I am taking lousy care of you. I don't understand why you're not getting any better. So I turned to the neuromodulation publication by Dr. M. Salino that says, how do you work up a troubleshooting baclofen pump? And so I checked the reservoir contents. They filled it in the OR. I checked the logs, all good. I program a 50-microgram bolus. The first time, I think I programmed 100-microgram bolus after that, no response, and I do a catheter access port aspiration. I stick the needle in. I'm able to draw up some fluid. Excuse me, it takes me about two minutes to get my three CCs, but, you know, hey, I got fluid. I'm feeling pretty, you know, like, huh, things look like they're connected. I'm feeling okay, but I'm also suspicious, especially in the clinical response, and I want her so badly to do well. So I do a contrasted CT study. That's kind of my go-to thing. I take her to the CT scan. I put a needle in her cap. I aspirate fluid again, sure enough, and I put in about three CCs of OmniPaque, and I get that CT scan right after that, and this is what I see. So here is where the contrast is. Normally, it should be down here in a meniscal-dependent position, like a puddle here at the bottom of the intrathecal space, and it's not there. It's up here. The radiologist reports, I think, said something. The initial radiology report said, you know, yeah, contrast in the intrathecal space or something like that, and I had to call them up, and I said, you know, could you just rewrite that report because there's no contrast in the intrathecal space. That's intradural, and so they changed that report, and then with that report and the clinical lack of efficacy, I go back to the surgeon, and I say, hey, surgeon, you know, I think I've got a problem with this catheter because he had this great trial, et cetera, and he does a catheter access port, and he says, no, Ferret, it's fine. Go away. Just keep turning up the pump, and, you know, we're all stubborn, right? We're all kind of done this a long time kind of thing, so I was like, well, okay, you're not helping me, but maybe you're right. I'll give you some extra consideration, so I go to the next part of the troubleshooting, which is repeat the intrathecal Backlund trial, so I take her back to do an intrathecal Backlund trial, and again, I get the exact same response, beautiful looseness in her legs, nothing to it. I'm like, okay, she responds to the medicine. She has a failure in the delivery system. This should be a no-brainer. If only I were young and had learned to implant and manage pumps and catheters myself surgically, I'd be in a great situation, but instead, I'm at the mercy of neurosurgery, so I call a second neurosurgeon, who I don't send as much business to, but who I consider my good friend, and I say, hey, why don't you take a look at this lady, and so he goes to her. She goes to his office, and he does a cap access, and he's like, hey, Rez, I've never seen one look better, and so we got a little more of a verbal jousting, so to speak, and I'm whining and moaning about things, and I finally say, well, look, you might as well just take it out then, because it's not doing her a lick of good. If you're not going to change the catheter, just take it out, and he kind of feels guilty about it, because he, at least in this case, he's able to admit that he's a neurosurgeon and not a physiatrist and doesn't know as much about pumps, so he decides to take her to the operating room. I'm like, thank you, thank you so much, and in the operating room, I get a phone call, and he says, hey, this is what I did. I went in, and I injected some contrast through the catheter, and I did a myelogram, and this is what I got, and I had suggested to him that maybe if you didn't want to change the whole catheter, could you at least yank it back down a couple inches, and so that's what he did. He opened up the spine. He pulled the catheter tip down a couple levels. First, he took this image, and then he got this image, which shows intrathecal contrast spreading up and down the spinal axis, and so he called me, and it's like, yeah, hey, you know, it made a difference at least in terms of flow. Now, this is when I would have liked to have said, yes, can you just replace the whole catheter? I think you guys would agree that that's the right thing to do when you have a catheter that's bad is not to generally reposition it, but maybe just to redo the whole thing, but he didn't do that, so he sent her back to me with this adjustment of her catheter level, and so I've titrated her up now, and she's much better. She was much better at 300. She's much better at 500. I got greedy when I saw her a couple weeks ago, and I've now eventually turned her up to 750 micrograms using flex dosing with 100-microgram boluses. She's still not where she was with that trial, even at 750 micrograms a day, but at least she can spread her legs out. She can move her legs. She's still, if I stretch her left leg more, she'll have a hip flexion spasm, but she's at least much better, so I'm not saying that moving a catheter tip is the most viable of options, but in this case, it was the only option I had. I couldn't negotiate for any more. I couldn't get somebody to replace the catheter. I don't replace catheters myself, but that left me wanting, right? I thought, well, why is it that the cap, I usually feel pretty good about it when I do my cap access. I place the needle. I wait to see if spinal fluid comes my way, and then if it does, I aspirate, and if it doesn't, I aspirate again, but I really don't know for sure what really makes me happy when I do a cap access. Is it three cc's in one minute? Because I have taken a catheter and put it in a water bucket and tried to aspirate from that catheter. I can get about three cc's in 45 seconds. That's my record, so asking for three cc's in one minute would have to be pretty pristine flow. Maybe two minutes is fine. I don't know, but I certainly, and I have seen with my toy catheter on the desk that if I puncture a hole in it, I can still aspirate from it. Medicine, I mean, when I push forward, it kind of leaks out. So if catheters have small tears, I'm not sure that catheter access ports are as wonderful as we might think. That was a cap. What is it good for? Absolutely nothing. No, maybe not absolutely nothing, but maybe not as good as we think. So I pulled up a couple articles about catheter studies. Andrew Skalsky just published this one last year. This second one talks about, was the one that gave me the idea that the importance of putting a needle in and having no negative pressure on the catheter itself, just letting, put the needle in and letting it see if it comes back, assuming that it's working right. So I just think there's some work to do with catheters and I wanted to see what anybody else thought. Is this a horse? Is this the way it just works for everybody? You see a beautiful 50 microgram trial and you can't really mimic it. And unless maybe I get to 1200 micrograms a day, I don't know. Or is it a zebra that's an integral catheter tip? And maybe it's still, there are MS plaques that are blocking flow or causing inflammation around the catheter tip that I can't see. But that's what I wanted to talk about. And if anybody has any comments or suggestions, I know it's not a live forum, but I've got four other experts on the phone, maybe. So on the line, if you guys have anything to share, I'd certainly appreciate it. Thank you, Reza. In the interest of time, we're going to continue with the presentations and then exchange some discussion at the end. Next up on the list is my colleague at Jefferson, Dr. Kimberly Heckert. Hello. Thank you for joining. I'm Kimberly Heckert. I'm a director of a spasticity management fellowship at Thomas Jefferson University. I do chemo denervation. I have some pumps. I do a little bit of everything. I have a spasticity clinic there, and this is a case from my clinic. Some cases have elements of horses and zebras in them. And I was delighted to find out there really is something that is a zebra and horse hybrid. It's called a zorse. So I present to you a zorse from my clinic. I am on the speaker's bureau for Allergan and have served on some advisory boards for Ibsen. My case involves a 54-year-old gentleman with history of spastic diplegia who presented to establish care with me. No one could seem to get his pump settings right. The background was he had an intrathecal bacillus pump implanted two years prior to meeting me, and this occurred after having had two trials. One was with a 50-microgram bolus, and he was perceived to be too weak with that. So he had a second trial of 25 micrograms, and the result was perceived to be good, and so he proceeded to implantation. He describes spasticity in both of his lower limbs, causing stiffness and scissoring and an inability to elevate his legs at rest. This bothered him because he needed to elevate his legs for chronic edema, and he noted that following his pump placement, he could elevate his feet for edema management, but what he said was he experienced worsening toe flexion spasms at that point. From there, he tried various dosing regimens with his other physician, including simple continuous, bolus only, and flex dosing. He described that dose increases would relieve his proximal spasms, but he would get more distal spasms, and that further increases made it harder for him to ambulate. Ultimately, he became so frustrated that he demanded his pump be shut off, and his physician had decreased his pump to minimal flow settings. So before electing to have his pump removed, he decided to get another opinion with me. His past medical history included chronic edema of the legs treated with furosemide, 40 to 80 milligrams daily, and a surgical history of ankle fusions bilaterally. His review of systems was negative, including questions related to bowel and bladder function. On examination, he had scissoring gait. His knees remained extended throughout the gait cycle. It was a step-through, or rather more like a swing-through gait pattern, where his initial contact was with foot flat, and he used forearm crutches, no orthoses. Significant spasticity was observed in the hip abductors, both the knee flexors and extensors, and the ankle plantar flexors within his available range. It did appear that his knee extension spasticity helped his ability to stand. He had cavus feet with pronated valgus ankles and limited passive range of motion at the ankles because he had the history of the fusions. While he was seated on the examination table, his toes were not in flexion, but when he went to prop his feet up, positioning his hips in flexion with his knees in extension, we observed a sort of tenodesis phenomenon, where his toes were brought passively into flexion. He had less than anti-gravity hip flexion while seated, and it was difficult for him to isolate major muscle groups for manual muscle testing, but there was weakness in both legs. His pump was interrogated, confirming minimal flow settings, three micrograms of baclofen a day. The logs were reviewed with no concerning events. My initial plan of care included obtaining x-rays, which revealed no obvious catheter My initial plan of care included obtaining x-rays, which revealed no obvious catheter disruption. His catheter access port was accessed in my office, and I withdrew three milliliters of fluid and confirmed CSF by sending for beta 2 transferrin. Because the patient was willing to try again, I reprogrammed his pump to deliver 25 micrograms a day, simple continuous dosing, feeling that this was relatively safe since he had obviously tolerated a 25 microgram bolus dose. Once I had all of that information, we collectively decided that we would pursue an inpatient rehab admission to allow for frequent dose adjustments and monitoring of the impact of those adjustments on his gait in particular and other functional tasks. This would have been very difficult for me to do with him as an outpatient because he came three hours to see me. So before bringing him into inpatient rehab, I decided to rule out any other subtle catheter problems. We also had discussed the use of neurotoxin for treatment of his ankle plantar flexors and long toe flexors and coordinating those injections about two weeks prior to his rehab admission so that he would be at peak effect when he came in. So before organizing the inpatient rehab admission, I brought him into the radiology suite for a CT myelogram. But on the day that he came in for this procedure, he was noted with significantly worsened spasticity despite the fact that I had just restarted his pump at 25 micrograms a day. He had abdominal guarding and increased spasticity that brought his hips into flexion and this made the side port access a little more technically challenging than it had just been in my office a week or so prior. These are actual images from his CT study. On the scalp view, you can see his baclofen pump. And here is a view demonstrating the spinal cord and dye all around the CSF. And here is a video of that study where again we can see his cord, lots of dye. And as we make our way down, we will soon see the level at which his catheter enters the spine. Here it is. My cursor has, there's the catheter. There is no extravasation of dye. I'm going to follow it with my cursor. Here it comes all the way around traversing to the abdomen where it's going to meet up with his pump that we see now coming into this image. Here is the little spot of dye that is the catheter and finally connecting to his baclofen pump. So this was a normal study in terms of the dye and the catheter. In contrast, this next image is a CT myelogram where there is dye extravasation. This is not the patient that I'm discussing. This is just what we might expect to see in the event of a catheter disruption. So here we can see the catheter inside, some dye in there. We don't see that dye in the intrathecal space. And when we come to the level where the catheter enters the spine, there is extravasation in the back. So that would be the difference. Back to my patient, this is just another coronal view showing the catheter tip. We'll dye there. So why was his spasticity so much worse on that day? Well, I got my first glance from a sagittal view here. We have a very large bladder as big as a fetus in there. Marked bladder distension, arguably the largest bladder I may have ever seen with grade two spondylolisthesis of L5 on S1. Upon further questioning, the patient continued to deny any difficulty with voiding, with the exception that he had voided less on that particular day owing to not taking his furosemide so that he didn't have to stop to urinate on the long trip from his home to my hospital. Regrettably, he was unsuccessful voiding in the radiology suite and continued to experience spasms. And so we directed him to the emergency department where he was subsequently straight catheterized for more than 1600 milliliters of urine. To this day, he has not disclosed this to me, but he did disclose to the ED physician that he had actually been to a different ED the night prior and had received anesthesia for a removal of a foreign body and had not voided well since that time. Additional and more specific questioning revealed many more symptoms of very longstanding urinary retention, intermittent constipation, and sexual dysfunction, even though all of these things had been denied during prior visits. What happened next? Well, I referred this gentleman to urology and after a period of intermittent catheterization, he now voids well on low-dose sildenafil. He did proceed with the inpatient admission, inpatient rehab, and was able to be titrated and discharged on a new flex dosing regimen that gave him a total daily dose of 69 micrograms. That is all it took for this gentleman to walk well. He was then referred to neurosurgery for his spondylolisthesis. At a number of different occasions, I have had to continue to provide education and counseling to this gentleman because there is still, or at least was for quite some time, a disconnect with his understanding in what he was experiencing in terms of spasms and what the causality was for these symptoms. So I did spend a great deal of time discussing with him common things that can increase spasticity such as pain, urinary retention, and constipation. I would frequently get calls from him where he would tell me that his pump was causing him more spasms. So what are the take-home points of this case? I think we should always ask about pain in addition to spasticity. They often go very closely hand-in-hand. And asking about bowel and bladder is so important, and of course I did this, but I think specifically to have asked about what was normal for this patient may have led me to an understanding in advance that he was severely retaining. So I think to ask a patient what they perceive as normal is an important question. And then the other take-home point that I have brought from this case is to ask specifically about sexual function and recognize that patients may not feel comfortable disclosing this information on their own. So with this, I'd like to thank you for your attention, and I will turn it over to the next presenter. Thank you, Dr. Heckert. We are now going to return to the Midwest and ask my colleague Dr. Ketchum to present his case. Thank you, Dr. Cellino. My name is Nick Ketchum. I am a physiatrist at the Medical College of Wisconsin in Milwaukee, Wisconsin. I'm one of the associate residency directors here, and I do spasticity management, stroke rehab, a lot of neurotoxin, alcohol, and intrathecal baclofen management. And today I'm going to talk about horses and zebras in the spasticity adjacent zoo. And this right here, I have a quagga. A quagga is a cousin of a zebra, and it actually went extinct in the late 1800s. And it's, if you can see here, it's half, the front half actually has stripes, the back half does not. And so I'm going to talk about something that's a cousin of spasticity, in my opinion, in terms of motor activity stuff, and so we'll go through the case. So my disclosures, and here we go. So my patient is a 65-year-old female. She has a history of muscle stiffness and pain in her left chest and trunk. And so way back, backing up, she has a history of breast cancer, status post-surgery, chemo, radiation in 2006. She had invasive ductal carcinoma with some positive nodes and receptor positive. She had a partial mastectomy with an axillary node dissection. She underwent adjuvant chemotherapy and radiation therapy way back, you know, 10, 15 years ago, and was on tamoxifen from 2011 to 2006 to 2011. When I saw her, you know, she talked about having this left trunk and shoulder pain, you know, really underneath the axilla, just this unremitting pain and stiffness and spasms that started after her breast cancer treatment, you know, about 10 years ago. This pain was so severe at points where it's resulted in ED visits, cardiac workups, and throughout this time and still ongoing, she has been followed by her oncologist and there's been repeated workups and she's not had any recurrence of disease, thankfully. And functionally, she feels the active range of motion of her left shoulder is restricted, and this really limits her day-to-day activities, you know, in addition to the pain. And so really using that upper extremity has been very difficult for her over the years, and this has, you know, resulted in numerous things and even some social isolation just because she really can't do the things that she wants to do actively and she's in so much pain. And in terms of what she's tried, she was actually for a long time placed on opiate medications for this, arguably inappropriately. She tried NSAIDs, Tramadol, Flexeril, lidocaine ointments, steroid injections, trigger point injections. She'd been on gabapentin, she did physical therapy, she did yoga, none of which gave her any meaningful relief. You know, the trigger point injections to her upper trapezius area gave her a few hours of improvement with the lidocaine that she had injected, but really nothing meaningful in terms of relief over this, you know, about 10-year period of pain. And she'd seen numerous providers who had, you know, recommended this treatment in our system. She had seen by her primary care, obviously her oncologist throughout this, but specific for these symptoms, primary care. She had actually seen somebody in the general PM&R department. She was referred to pain management who prescribed the opiates. She had done, like I said, physical therapy. She had done massage therapy. She'd actually been seen in our spine clinic in case this was something cervicogenic, which that was ruled out. She was actually then seen by our PM&R sports physicians looking at, you know, is this something intrinsic shoulder causing her symptoms. Prior diagnosis that she's had for these symptoms included adhesive capsulitis, lymphedema, which she had management for this and did not improve her symptoms, fibromyalgia, but she did not have widespread symptoms by any means. She was labeled as just simply chronic pain, glenohumeral arthritis, and that's kind of, you know, the gamut of what she'd been labeled as up to me. When I saw her, in general, there was no lymphadenopathy. Surgical scars were present, well healed. She did have, you know, some restricted range of motion of her neck to the right and a resting left neck tilt. Extremities had some trace left upper extremity edema, but nothing significant. MSK exam, she was hypertonic to the left pec major, upper trapezius, and serratus anterior, and these were areas where she was reporting her symptoms had been this whole time, and just comparing left to right, she had a slightly hypertrophic left upper trapezius. Neurologically, she had a decreased light touch sensation to the left medial forearm and the ulnar hand, and I have my motor exam up here as well, which noticed maybe some trace weakness in wrist extensors, the EIP, and the hand intrinsics. We actually did an EMG on her, given her history, and just to summarize, she did have mild abnormal findings in terms of some axon loss in the left medial entrobacocutaneous nerve, the ulnar snap to the little finger, the ulnar C-MAP, and these were side-to-side changes, and there were old inactive abnormalities in the opponent's pollicis, the first dorsal neurasius, and the extensor indices with no active or ongoing denervation, just large motor units with neuropathic recruitment, and we did actually, we stuck a needle in, we examined the areas where she was having stiffness and pain and all her symptoms, and what we found is that she couldn't turn off these muscles. They were constantly firing with motor units in the pec major, the trapezius, and the serratus anterior. And that was certainly an interesting finding, but nothing, no classic findings of radiation plexopathy with myotomic discharges or anything. But we were thinking, what is going on? And so these symptoms had persisted despite multiple years and many diagnoses, many treatments, but nothing had really given her any benefits. And the pain was muscular, not neuropathic. And so even though she had maybe some changes, suggestive of an old brachial plexopathy, possibly related to her prior cancer treatment, this pain was really muscular in nature and not neuropathic. And again, this did seem to be ruled out. They ruled out any spine findings or cervicogenic radicular issues could be causing that. And the EMG kind of confirmed that. And then we had these subtle exam findings in terms of the neck tilt and the hypertrophic trapezius. And I didn't mention her passive range of motion was full and unrestricted. She just had pain with range of motion that limited it actively. And so given all these findings, given these treatments that have tried and haven't led to any benefit, and the constellation of things that we saw on exam, our question was, is this a secondary dystonia in the setting of the surgery, chemotherapy, radiation, and is this a radiation plexopathy? And so, again, looking at these muscles, typical normal muscles should be electrically silent at rest if somebody's trying to, in a supine position, trying to relax, but these muscles could not turn off. And there were these constant involuntary motor units in these muscles. And so we said, hey, look, let's treat this as a focal dystonia. And we treated her with IncoBotulinum toxin A, 150 units divided into these areas where she was having these symptoms. And we found those involuntarily active motor units. So a total of 150 units into these symptomatic and areas with these motor unit findings. And so we saw her for follow-up. And she came in and she said she had 100% relief. She was crying by two sentences into the visit because nothing else had really given any meaningful impact for days, let alone the time that she got relief from this. She said she was thrilled with the results. The big thing was in her house, she couldn't lift the heavy water pitcher, she couldn't reach to the top shelf. She could do her ADLs pain-free. And the biggest thing was the fact that she could get back to doing these things that were so restricted for so long. And she had no side effects. Obviously, we wanna make sure that the treatment that we're providing is effective, but also safe first and foremost. And she had symptomatic improvement for three and a half months. And I have seen her in follow-up since then. And she has mild recurrence of symptoms when we have her follow-up at about four months from the prior injections. And we injected in the same pattern. And again, she reports this tremendous relief of her symptoms. And so again, maybe not a spasticity case, but a spasticity adjacent case. And takeaways that I had with this and just wanted to share and discuss maybe, clearly not all shoulder and neck and trunk pain needs botulinum toxin, but some might. And this did seem to be more of a muscular, like a pec trap and trunk issue. And so kind of in that general region, but not a classic cervical dystonia by any means. In refractory cases of muscular pain, and especially given this history, this zebra cousin might be on the differential. And zebras need many zookeepers. I certainly wasn't the only one looking at this patient. And I have to give thanks to one of my colleagues in sports medicine to really take a step back and think about this instead of saying shoulder pain, neck pain, muscular pain, calling her fibromyalgia or chronic pain, really dig into the history and say, what really could be going on or could be causing this? And so certainly not a common case, but something that I think was met with some really good results thinking a little bit outside of the box. And so with that, I'll kick it back to Dr. Salino. Thank you, Dr. Ketchum, very well done. I will back clean up in this time of playoff baseball and give you my cases. Okay, my name's Mike Salino. I'm a physiatrist at Moss Rehab with Dr. Moon as well as sharing an academic appointment with Dr. Heckert at Thomas Jefferson, the Sidney Kimmel College of Medicine involved deeply with the spasticity fellowship that Dr. Heckert so ably runs. Manage a lot of intrathecal delivery systems as well as doing oral and intramuscular management of spasticity. These are my disclosures. I work with many of the device and pharmaceutical companies that are involved in neuromodulation. There might be some minor touch points on off-label issues. I'll do my best to bring those out. And I will only discuss branded products where it is absolutely necessary for educational purposes. So my objectives for the little bit is compare and contrast the typical and atypical spasticity issue specifically related to intrathecal drug delivery. The cases have been de-identified for security purposes. So this is what we were all taught in medical school that if you hear hoof beats, you might need to consider both the usual and the unusual issues. In this case, I'm going to talk about a horse, a zebra, and perhaps the even more rare zebraic unicorn. This is not a real animal, obviously, as opposed to what Dr. Heckert and Dr. Ketchum brought up. I will use those. Said another way, uncommon manifestations of common diseases are more common than common manifestations of uncommon diseases. This was actually said to be my first internal medicine attending when I was a third-year medical student, kind of not knowing which end of the stethoscope to put in my ears. And he really guided me an awful lot in those early formative years of medical training. So case number one, a 35-year-old female with spastic paraparesis due to multiple sclerosis come with intrathecal therapy, has used therapy for two years without issue. She's on a stable dose of 200. She reports her spasticity is very well-controlled, no intervening issues, including stable neurogenic bowel and bladder, no changes there. Because she was so thin, her pump was implanted in a subfascial pocket. She comes in for her routine refill. Her alarm date is in about 15 days. Attempt to obtain telemetry from the system was unsuccessful. So next steps in management on this straightforward case. Important to recognize that currently there are a couple of different intrathecal drug delivery systems that are programmable that are available for commercial use in the United States. The Synchromed 2 system, the Medstream system, and the Prometra system all had FDA approval for Baclofen. Medstream, which was by Johnson & Johnson, is out of commercial use at this point. I believe all the patients that were implanted are now replaced with other systems. But there are two systems that are in active use, the Synchromed 2 system and the Prometra system. So it is now pertinent to recognize that you have to use the right programmer for the right system. There is no crosstalk between the two major companies. And you have to identify which system the patient has. And always make sure that the programmers are up to date. This is on the right-hand side of the slide shows an example where the software had not been updated. At first, I panicked a little bit, thinking that there was a pump malfunction. But this was really that I had failed to update the software on the programmer. And once it was, everything was fine. Make sure that all the batteries are fresh and that the programmers are fully charged. So this is the most typical cause for failure to obtain telemetry. You know, it's something electronic or something mechanical that is relatively easily fixed. So in this particular scenario, the clinician was using the wrong programmer. I will take the blame that I was using the wrong programmer. And upon conversion to the correct programmer, interrogation, refill, and reprogramming commenced in an uneventful fashion. Case presentation two. Again, presenting exactly the same way with exactly the same characteristics. Again, presenting for refill 15 days before alarm date. Failure to obtain telemetry. Going back, we made sure that the correct programmer was being used, it was fully charged, and update with the fresh batteries, not un-indated, updated batteries. Sorry for my spelling there. We actually confirmed this with a second programmer and obtained absolutely similar results. So what are the next steps in management in this particular case? Well, one of the things that we have learned to have at our disposal is a bedside ultrasound. I will acknowledge at this point that the utilization of ultrasound for the management of intrathecal delivery systems is an off-label presentation. Not because the ultrasound harms the systems in any way, it's just that it has not been formally tested. This is the sonographic appearance of the Synchromed II system. You could see the bright white line is the top surface of the pump, and then this dark well represents the reservoir access port. If you go to the perimeter of the pump, you see this little step-off and this triangular-shaped area that represents the catheter access port. So it has a very unique signature. This is the ultrasound view of the Prometra system, and instead of seeing a level area for the reservoir access port, you actually see a little bump out, almost feels like a little button above it that you can palpate with your hands. And similarly, the catheter access port looks very similar to the reservoir port in the Synchromed system. So there's a distinct ultrasound appearance of these systems. So we took our patient and put the bedside ultrasound on it, and this is what we saw. We scanned back and forth over the pump and saw neither the reservoir access port or the catheter access port demonstrating a flipped pump. What was unique about this case is that this was a Prometra II system, and we learned that the antenna of the Prometra system is unidirectional, which means it can only be interrogated when the pump is right-side up. This is in distinction to the Synchromed II system in which telemetry can be obtained, but obviously you're not going to be able to do a refill because the reservoir access port is actually facing towards the abdomen. Unbeknownst to me, when I first saw my first flipped pump a few years ago, I didn't realize that this had a formal diagnosis, and the diagnosis is actually something called Twiddler syndrome. It actually comes from the cardiology literature in which individuals were playing with their pacemakers by just turning it over and over. In this particular case, because the pump was inverted or upside down, the patient was sent for urgent surgical revision, and we were able to obtain telemetry easily and accomplish the refill after surgery. Case three, again, looking absolutely identical to the other cases. Again, everything's looking good. Presents to clinic. Again, we are unable to get telemetry. Again, using the horse analogy, verified that we were using the correct batteries and the fully charged programmer. Again, used a second programmer, got similar results. While we could palpate it, palpate the edges of the pump, we were unable to see the pump clearly on ultrasound. We weren't able to see any distinguishing features. So at this point, we then got a CT scan. Let me get that up. So this is not a dye injection. You can still see the catheter, maybe not as brightly as you would with a CT myelogram, but this is a non-contrast CT. Again, you could see the catheter coming down. Eventually you're gonna see the catheter begin to come out. It's not as easy to see in the unenhanced study, but you can see that the pump has migrated into the abdomen. This is not an unreported complication with subfascial insertion. So it actually herniated through the abdominal musculature. The abdominal musculature and was now in the peritoneum. Obviously this didn't happen in an instant. Let me go back to my screen share. So the non-contrast CT demonstrated migration into the abdominal cavity. Went for urgent surgical revision. Both neurosurgery and general surgery went in because there was concern that the pump was actually adherent to bowel. And in this case, it actually was. So the patient did require a small bowel resection. The abdominal muscle and fascial deficit was closed. The pump was actually rerouted to the opposite side and the patient continues with intrathecal baclofen therapy to this day without issues. So that is certainly the zebraic unicorn. It's the only time I've ever seen that in my career. So again, horses and zebras, if you hear the hoof beats, consider both the usual and the unusual causes. Let us now open it up to the entire panel that we can ask questions of each other. I'm actually gonna ask a question to my colleague, Dr. Moon. Dan, when you decided to do the injections, is that just based on anatomic landmark or can you do some electric diagnostic evaluation to determine which muscle to inject? It's primarily based on anatomical landmarks and the clinical presentation, but you could hook it up to EMG machine and listen for the active units as well. Rez, I'll make a comment about your case. I think that's the earliest subdural intradural case that I've ever seen that it's happened that quickly. I think the natural history is usually a few years into the implant. My concern when I see those situations is I worry that there's sort of an encapsulated pocket of fluid that is baclofen enriched, sort of like a water balloon that could burst open at any time. And there have been a handful of cases where patients had that poor response to therapy and then all of a sudden for no reason presented with overdose. And then they would go through this sequence of withdraw overdose, withdraw overdose. So I always worry when I see those intradural or subdural encapsulations. I agree with you completely. I think a catheter revision is an order. I would make that plea. I would wean the patient down first because you don't know how much they are getting, go for a complete catheter revision and then start them up from scratch. Perhaps going a little bit lower with a catheter tip, because you did get a great response with the lumbar injection. Maybe you don't need to be as high in the thoracic spine. I agree, thanks. Other questions or comments by my distinguished colleagues to one another? I'll add one for Dan too. When he was relaying his case, I had a lady who came in and some of these do have those emotional, psychological instabilities, but she was dislocating her jaw laterally. And we ended up injecting her lateral pterygoid, which back in the day, I wasn't confident I could find. This was before we were doing ultrasound. Maybe you could find it with ultrasound. But we were using CT guidance to put a needle in her jaw to get her lateral pterygoid, just injecting 15 units at a time. And that kept her from dislocating, but it wasn't long-term sustainable. But thanks for bringing back the memories of her, as that was an emotional time to be a doc around here. No problem. Thank you for sharing. Well, I certainly want to thank the audience for their attention in this virtual world. Please give any comments and feedbacks in your evaluation of the session. We are always looking to improve upon these sessions. Most importantly, I want to thank my esteemed colleagues who took time out of their schedule to put these presentations together and took time out of their evening to present tonight. Let us all hope that the world begins to correct itself and that we could do this live again next year. Thank you so much. Thank you.

spasticity management

Horses and Zebras

orofacial movement disorders

botulinum toxin injections

intrathecal baclofen

hip flexion

adductor spasticity

chronic chest and trunk pain

breast cancer treatment

secondary dystonia