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The trigeminal autonomic cephalalgias are a group of headache disorders that appear similar to each other and other headache disorders but have important differences. Proper diagnosis is crucial for proper treatment.  In this episode, Gordon Smith, MD, FAAN, speaks with Mark Burish, MD, PhD author of the article “Cluster Headache, SUNCT, and SUNA,” in the Continuum April 2024 Headache issue. Dr. Smith is a Continuum Audio interviewer and professor and chair of neurology at Kenneth and Dianne Wright Distinguished Chair in Clinical and Translational Research at Virginia Commonwealth University in Richmond, Virginia. Dr. Burish is an associate professor at UT Health Houston in Houston, Texas. Additional Resources Read the article: Cluster Headache, SUNCT, and SUNA Subscribe to Continuum: continpub.com/Spring024 Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @gordonsmithMD Transcript Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the Show Notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the Show Notes. AAN members: stay tuned after the episode to hear how you can get CME for listening.   Dr Smith: This is Dr Gordon Smith. Today, I'm interviewing Dr. Mark Burish on cluster headache, which is part of the April 2024 Continuum issue on headache. Dr Burish is an Associate Professor of Neurology at the University of Texas Health Science Center at Houston, which is located in Houston, Texas. Mark, thanks so much for joining me today on Continuum Audio. I was really excited to be asked to talk with you about this article. When I recertified from my boards the last time (and actually, it will be the last time I have to take the exam), I did the AAN course on all of neurology. And I'm a neuromuscular guy, right, and so I was actually kind of worried about the headache part because I thought, “How interesting could that be?” And I was blown away at how fascinating headache has become, and in particular, your topic (cluster, SUNCT, SUNA, the trigeminal autonomic cephalalgias) - such a great topic. But before we start talking about them, I'd love to just hear more about how you got interested in this area - both headache, this topic in particular. What's your story, Mark? Dr Burish: Well, thank you very much for having me. I’m honored to be part of this. I got into headache probably the way many people do; is, in residency, you figure out what you like, and your residency clinic tends to start collecting patients that you like (not that you're trading them with other residents, but you see certain patients). And mine (by the end of residency) had a lot of headache and pain patients into it. Then, I was very fortunate and had the opportunity to do some research as part of my career. I'm an MD-PhD, and I spend about half my time now doing research on cluster headaches, so I'm very fascinated by these types of diseases. Dr Smith: Can you tell us really briefly what you're working on in your research? Dr Burish: Cluster headache is such a poorly researched area. There's not a lot of people in it, so we do a little bit of everything: we have a clinical trial going; we do some basic science on the circadian mechanisms (cluster gets this very weird timing to it, where the headaches happen same time every day); and we do a little bit of starting to wade into the genetics. Dr Smith: Well, super exciting. I was actually blown away by the statistics on cluster (as common as multiple sclerosis), and the severity of pain I was amazed to learn is above that of childbirth (it was, like, between nine and ten out of ten, which is really crazy). And I'm worried that I missed these patients in my neuromuscular clinic. So, maybe we can begin by - just tell us what you think our listeners need to know. If they have to drop off right now, what message do they need to remember from our conversation? Dr Burish: I think there's two things. First of all, the first-line treatments for these headaches have not changed recently. For cluster headache, you still treat it with oxygen, the triptans (the faster triptans; not the oral ones, but the injectables and nasals), and you prevent them with verapamil. For SUNCT and SUNA, you use lamotrigine. So, those have not changed over time. There are some new treatments, which we'll talk about later. Then the second point is, there are four different types of headaches in this family and they all look very, very similar (one-sided pain, autonomic features, ipsilateral lacrimation, rhinorrhea - that type of thing). They differ in the treatments and how long they last. If you get them wrong (if you misdiagnose them), you're probably not going to give them correct treatment. Indomethacin works very well for two of them (the ones with hemicrania in the name, so not the ones we're going to discuss today). And then SUNCT, SUNA, and cluster headache - indomethacin does not work very well. So, it's important to distinguish them and get them right. Dr Smith: Maybe we can start there, Mark. I mean, I was kind of appalled to learn that the average delay in diagnosis is four to nine years in your article, and given the severity of pain and the impact it has on these patients, that's clearly a challenge. What's so hard about this? And do you have pearls on how we can recognize these patients? And how do you sort this out practically in clinic? Dr Burish: For cluster headache patients especially, it is a lot more common than we would think it is, but it still goes misdiagnosed, partly because most cluster headache patients are episodic. So, there's an episodic version where you get them every day for a few weeks and then they might go away for a year. So, I think what happens is that patients start to get into a cycle and they either get confused for sinusitis (because it happens in the spring), or they schedule a visit with a neurologist or somebody else, but the headaches are over by the time they see them, and they cancel the visit. So, I think they get misdiagnosed partly because it's either confused or they don't see doctors fast enough. I think a little bit more awareness of what this disease is and then, somehow, a mechanism to get these patients in a little bit more urgently is probably what's necessary. Dr Smith: Well, Mark, access is a real issue in neurology more broadly, and I'd love to talk to you about that in a moment, but I wonder if we could go back. You talked about how similar these are to one another, yet the treatments are different. How do you sort out the diagnosis when you're seeing a patient? Let's say you have someone who comes in who has episodic, unilateral, very severe pain and some of these autonomic features. What are the pearls for differentiating cluster, SUNCT, and SUNA from each other? Dr Burish: The big difference between all these different headaches is the timing. As a general rule, SUNCT and SUNA attacks last seconds (they're very similar to trigeminal neuralgia); paroxysmal hemicrania (that's one of the hemicrania ones, where indomethacin helps) - those attacks last minutes; cluster headache attacks last about an hour; and the hemicrania continua is constant (that's the other hemicrania one where indomethacin works). The other part is how often they happen. Again, SUNCT and SUNA - very similar to trigeminal neuralgia, may happen hundreds of times a day; paroxysmal hemicrania - dozens of times a day; cluster headache - maybe a handful of times; and then, hemicrania is constant. Based on how long the attacks are and how frequent the attacks are, you can generally separate them out. And if you're not sure, just try indomethacin. And then if it doesn't work, you're trying to distinguish between SUNCT and SUNA, which lasts seconds, and cluster headache, which lasts an hour, so fairly easy to distinguish those. Dr Smith: How long does it take to medicine to work in a patient with hemicrania continua or paroxysmal hemicrania? I’ll remind our listeners - there's a separate article in the same issue of Continuum on that topic - but for our purposes, let's say you try that; how long do you need to try it? Dr Burish: Yeah, there's a great, another article about how much to give and how it works. It is generally pretty quick. I have noticed with most patients that the onset is twenty-four to forty-eight hours. And then, if you stop the medicine, the same thing - offset is kind of twenty-four to forty-eight hours. So, patients know pretty quick whether it's going to work. Dr Smith: Wow - that's awesome. One of the things I was interested in was so-called “secondary cluster.” So, you've seen your patient and let's say you've diagnosed them with cluster (primary cluster). Do you do additional testing? Do they need imaging or other laboratory workup? Dr Burish: Yeah. The differential for cluster (and cluster is the one that we know the most about; it is the most common of all the trigeminal autonomic cephalalgias) - it's a fascinating differential. If you don't know much about them, migraine is probably the most common. If y

Most patients with migraine require acute treatment for at least some attacks. There is no one-size-fits-all acute treatment and multiple treatment trials are sometimes necessary to determine the optimal regimen for patients. In this episode, Teshamae Monteith, MD, FAAN, speaks with Rebecca Burch, MD, FAHS author of the article “Acute Treatment of Migraine,” in the Continuum April 2024 Headache issue. Dr. Monteith is the associate editor of Continuum® Audio and an associate professor of clinical neurology at the University of Miami Miller School of Medicine in Miami, Florida. Dr. Burch is an assistant professor in the Department of Neurological Sciences at Larner College of Medicine, University of Vermont, Burlington, Vermont.  Additional Resources Read the article: Acute Treatment of Migraine Subscribe to Continuum: continpub.com/Spring2024 Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @headacheMD Guest: @RebeccaCBurch Transcript Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the Show Notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the Show Notes. AAN members, stay turned after the episode to get CME for listening. Dr Monteith: This is Dr Teshamae Monteith, Associate Editor of Continuum Audio. Today I'm interviewing Dr Rebecca Burch on acute treatment of migraine, which is part of the April 2024 Continuum issue on headache. Dr Burch is an Assistant Professor at Larner College of Medicine at the University of Vermont in Burlington, Vermont. Well, hi, Rebecca - thank you so much for being on our podcast. Dr Burch: Thank you so much for having me. It's always such a pleasure to talk with you. Dr Monteith: You wrote a really excellent article on acute management of migraine - really detailed. Dr Burch: Thanks so much. I'm glad you enjoyed it. I had a lot of fun writing it. Dr Monteith: Why don't you tell our listeners, what did you set out to do in writing this article? Dr Burch: Whenever I write a review article on a topic, I aim for two things, and these were the same things that I was aiming for here with this one. One is practicality and just for it to be really applicable to clinical practice and every day what we do - the ins and outs - and that was the case here as well. I really love a good table in a paper like this. I spend a lot of time on tables. I want people to be able to print them out, use them as reference, bookmark them. So, that was one thing that I aimed for - was just for this to be really useful. The other thing is, I really wanted to instill a sense of confidence in people after reading this article. I think the management of migraine can be very overwhelming for people taking care of people with migraine. And there are so many acute treatment options, so I wanted to give a framework for how to think about acute treatment (how to approach it), and then within that framework, to really go into the nuances of all the various options, and how to choose between them, and what to do in specific circumstances. And I also really wanted to cover what to do when the first couple of options don't work. Because I think most neurologists, PCPs, are comfortable prescribing sumatriptan, and then the question is, what happens when that doesn't work or the patient doesn't tolerate it? What do you do for rescue therapy? What do you do for your fifth-line treatment? And I think that was an area that I really wanted to cover as well. Dr Monteith: Yeah, you got a lot done, for sure. So, I agree - there's been so many options, new options, even over the past five or definitely ten years. One of the things that excited me about going into headache medicine were all the options, thinking of migraine and other headache disorders as a treatable disorder. What made you interested in headache medicine? Dr Burch: Like so many other people who ended up going into headache medicine, I had a fantastic mentor in residency who was really great at treating headache patients - as Brian McGeeney at Boston Medical Center (he's now at Brigham and Women's). He was really passionate about headache medicine, and seeing patients with him was always such a delight because he always had something to try. And many other situations, it would be, like, “Well, this person, we've tried something; we don't know what else to do.” But when you work with a headache specialist as a mentor or as a preceptor, they have so many things they can do, and people largely get better. And they're so grateful - it changes people's lives to be able to treat their migraine, their other headaches effectively. So that was really inspiring. And then when I started doing headache rotations and sort of thinking about whether this was the right subspecialty for me, I quickly realized two things about headache medicine that ended up being what I really love about it to this day. One is the longitudinal relationships that we have with patients - we take care of people for a long time. And it doesn't always have to be that we're seeing people every three months and making tweaks - sometimes it's once a year. But we do get to know people. You know, I have two children. Many of my patients saw me through both of those pregnancies and ask about my kids, and it's just lovely to have that sort of personal relationship over time. And then the other aspect that I really love is that we can't see patients in isolation just as their migraine disorder or headache disorder; we really have to think about who they are as a whole person. What's going on in your life? What are your stressors? How's your job, how's your family? How are you sleeping? How's your mood? Are you exercising? What's your diet like? All of these things impact how someone's migraine disorder is going. And I like to joke, “I'm half life coach, you know, and half pharmacologist,” and I love that. I love that I bring my whole self every time I see a patient and see their whole self, too. Dr Monteith: I can just imagine how well you do that. You mentioned the power of mentorship, and that seems to be a theme when interviewing authors (that mentors are super important). And I know you've been an incredible mentor. Why don't you tell us a little bit about your academic journey? I mean, I see you in the halls at these major conferences, but I've never pulled you aside and said, “Hey, what's your journey - your academic journey – like, other than your great editorial work for neurology, of course?” Dr Burch: I did my fellowship at Brigham and Women's and then stayed on there as an attending, and ultimately took over as fellowship director before I took a break, which I'll talk about in a minute. In that time, I was doing clinical care and I had a research program and I was doing education - doing a lot of teaching for CME work, and teaching primary care and subspecialists about migraine - and I really love that piece of things - and precepting fellows. And then, I also had my editorial work on top of that. I have been a medical journal editor as long as I have been a headache specialist. We were talking about mentors, and I want to talk, at some point, about my fantastic mentor, Elizabeth Loder, who is also a research editor, in addition to being an outstanding headache medicine clinician and researcher and educator. But she got me started as an Assistant Editor for Headache in my fellowship year - the journal Headache - and I continued as an Associate Editor there. I worked as a Research Editor for the British Medical Journal for a while and then joined the journal Neurology, where I am one of the eight Associate Editors. I cover the general neurology portfolio, which includes a lot of things - includes headache medicine, includes traumatic brain injury, pain, spine, neuro-oncology, neuro-otology - there's a whole bunch of different things that I have learned a lot about since starting as an editor. So, I have always had a lot of different parts to my job, which keeps me interested. It's also a lot, and I do always talk about the fact that I ended up taking a year off because I think it's important to be real about the lives that we lead and our jobs as academic neurologist. So I ended up having a bunch of family health issues that came up in 2021, and combined with all of the other things that we're doing, I just couldn't keep it all going. And I ended up getting sort of burned out a little bit and was having trouble balancing all of that and the family health issues that were going on. And I ended up taking about a year off from clinical work. I continued with my editorial work and kind of got everything sorted out with my family, and then just started my current position in January. I'd just like to bring that up to show that – you know, not everyone's going to be able to take a year off - I recognize that. But I think it's important to normalize that just being “pedal to the metal” all the time is not feasible for anyone. And we need to recognize that it's okay to take breaks periodically. So, I'm kind of an evangelist for the “taking-a

Headache medicine relies heavily on the patient’s history, perhaps more than any other field in neurology. A systematic approach to history taking is critical in evaluating patients with headache. In this episode, Katie Grouse, MD, FAAN, speaks with Deborah Friedman, MD, MPH, FAAN author of the article “Approach to the Patient With Headache,” in the Continuum April 2024 Headache issue. Dr. Grouse is Dr. Grouse is a Continuum® Audio interviewer and a clinical assistant professor at the University of California San Francisco in San Francisco, California. Dr. Friedman is a neuro-Ophthalmologist and headache specialist in Dallas, Texas. Additional Resources Read the article: Approach to the Patient with Headache Subscribe to Continuum: continpub.com/Spring2024 Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Full transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the Show Notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the Show Notes. AAN members: Stay tuned after the episode to hear how you can get CME for listening. Dr Grouse: This is Dr. Katie Grouse. Today, I'm interviewing Dr Deborah Friedman on approach to the clinic patient with headache, which is part of an issue on headache. Dr. Friedman is a neuro-ophthalmologist and headache specialist in Dallas, Texas. Deborah, I'd love if we could just start by you telling us more about you. How did you become interested in the diagnosis and treatment of headache? Dr Friedman: I guess one of the lessons in life that I have learned regarding this question is, “never say never.” I started as a neuro-ophthalmologist - that's what I did my fellowship in. My very first job was in Syracuse, New York, at Upstate Medical University, and there was no headache specialist in Syracuse at the time. And I started seeing neuro-ophthalmology patients and specifically told the person who did my scheduling for me, “Do not schedule headache patients. I am not a headache doctor; I'm a neuro-ophthalmologist.” Well, these people just snuck in the door. They got referred in for their visual disturbances, right - we know what that was - or for their, you know, transient loss of vision or some type of visual manifestation of migraine or eye pain, right? So, I started seeing the patients and I figure, “Well, I did a neurology residency; I can treat headache as well as anybody else.” And so I started treating their headaches. and they would come back to see me in follow-up and say, “You gave me my life back,” and I was pretty blown away by that. This was a few decades ago, and we didn't give very many people “their lives back” at the time in neurology, so I decided I should go learn more about headache medicine. And I started attending national meetings of what is now the American Headache Society. I found that I really, really loved treating headache, and it has a natural marriage with neuro-ophthalmology. As my career progressed, I ended up doing more headache medicine and less neuro-ophthalmology, but I still love both. Dr Grouse: Yeah, absolutely. I think the treatment of headache can be so satisfying and I'm so happy to hear that you were able to discover that love of treating headache in your own career. Why do you think it's important for neurology clinicians to read your article? Dr Friedman: Well, headache is the most common disorder seen in general neurology. It is actually the most common neurological disorder overall, by a factor of ten. And it is one of the most common causes of neurologic disability worldwide - like it's in (routinely in) the top five. So, it's an important problem, and patients are going to come see us, and we need to know how to effectively interview them so we can effectively manage them. I think, in a nutshell, that's why. Dr Grouse: You mentioned in your article the importance of making time to discuss the headache - so much so that, actually, you said that if they mentioned it offhand at the end of the visit that they have a headache, you really should be scheduling time for them to come back, to prepare and organize the information, and to have the time to really talk with them. I find this is such an important point and, in my mind, really gets to the heart of what you're trying to tell us in your article - that the way you take the history can make or break your ability to diagnose and treat the problem. Can you talk more about that? Dr Friedman: Sure. The history is absolutely the most important part of the office visit with headache medicine. I mean, they always say, “In medicine and in neurology, ninety percent of the diagnosis is made by history.” And that is more than true in headache medicine. So, you have to really get a good history. And it's a skill, but there's also kind of an art to it. So, there are certain questions you want to have answers to, but there's also this art of how to relate to the patient and how to really get them to tell you what you need to know, right? When I wrote the article, I really tried to convey that, because I think a lot of it can be learned. But there are a lot of nuances to taking a headache history, and I think that, for many people, it's helpful to have a guide to do that. Dr Grouse: Following up on what you just said - you mentioned, of course, the art of taking the good history for headache, which I completely agree is absolutely true. However, in your article, you also mentioned that things like various questionnaire tools, AI, can also be really helpful for diagnosis, which seems to be the opposite of the art of medicine. Tell me more about how you can incorporate that into taking your history. Dr Friedman: I find that questionnaires are incredibly helpful. I devised my own - it is one of the questionnaires that's available in the article (there's a link for it). It's not that I just read the questionnaire and I walk in the room knowing exactly what's going on - sometimes that's true - but at least I have a good idea of what I'm going to be facing when I walk into the room and start talking to the patient. The other reason (perhaps more importantly) that I think it's so helpful is because it gets the patient thinking about the details of their headaches and the details of their life and, you know, like, what medications they've taken in the past. And it really prepares the patient for the interview. In a lot of ways, I think that's more important than the information it gives me. But I do look at all the questionnaires, and I'll say, “Well, you know, you checked off this, and what did you mean by that? And you said this or that on your questionnaire.” And I kind of refer to it so they at least know that I looked at it - there's nothing more irritating than filling out a long questionnaire and then nobody ever looks at it - so, I do look at it and I do acknowledge in front of them that I have looked at it and am looking at it. But I think that they help in many ways. There are programs in AI that the patient will just enter information into online and the program will just spit out a narrative, as well as a diagnosis or a differential diagnosis. For clinicians that are really under a lot of time constraints, I think these can help considerably as well. Dr Grouse: That's really interesting, and that actually brings me to the next question I wanted to ask, which was - do you have any tips for the many busy neurologists out there (many listening to this podcast right now) who really want to do a good job gathering information and taking a careful history but are really limited on time to be able to do this? What other tools out there would you recommend for them, or tips? Dr Friedman: Yeah, I think that probably the questionnaires and the AI-based programs are very helpful. There is - I have no financial relationship with this company; I just happen to know about it and I know the people that developed it - but it's called BonTriage (as opposed to bon voyage), and it was developed by headache specialists. And I've seen the product and I've seen the output that can be used, and I think that one is incredibly helpful. It was really made for primary care, so that people could do this thing online and then just walk in with a piece of paper, hand it to their primary care doctor, and they'd have the whole history and the differential diagnosis. But it's equally as useful for neurologists. Dr Grouse: How about in history taking - any tricks to get the history you need and let the patient feel heard without necessarily taking lots of time going down the wrong pathway? Dr Friedman: Yeah, that can be really hard, and sometimes patients just want to bring you down what you would consider the wrong pathway (obviously, they consider it the right pathway). People have different styles of interviewing and people have different styles of answering the question. I find that it's often very obvious early on whether the patient is going to do better by asking closed-ended questions or asking open-ended questions. I always start with open-ende

Headache is among the most common neurologic disorders worldwide. The differential diagnosis for primary and secondary headache disorders is broad and making an accurate diagnosis is essential for effective management. In this episode, Lyell K. Jones Jr, MD, FAAN, speaks with Amy Gelfand, MD, who served as the guest editor of the Continuum® April 2024 Headache issue. They provide a preview of the issue, which publishes on April 3, 2024. Dr. Jones is the editor-in-chief of Continuum: Lifelong Learning in Neurology® and is a professor of neurology at Mayo Clinic in Rochester, Minnesota. Dr. Gelfand is an associate professor at Benioff Children’s Hospitals, University of California San Francisco in San Francisco, California. Additional Resources Continuum website: ContinuumJournal.com Subscribe to Continuum and save 15%: continpub.com/Spring2024 More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @LyellJ Guest: @aagelfand Full transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by clicking on the link in the Show Notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the Show Notes.   Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum Lifelong Learning in Neurology. Today, I'm interviewing Dr Amy Gelfand, who recently served as Continuum's guest editor for our latest issue on headache disorders. Dr. Gelfand is a child neurologist at the University of California, San Francisco, where she is an associate professor of neurology, and she also happens to be Editor-in-Chief of the journal Headache. Dr Gelfand, welcome, and thank you for joining us today. Dr Gelfand: Thank you so much for having me. Dr Jones: Dr Gelfand, this issue is full of extremely helpful clinical descriptions and treatment strategies for headache disorders. With your perspective as the editor for this issue - and you've just read all these wonderful articles and edited these articles - what were you most surprised to learn? Dr Gelfand: I would say that the medication overuse headache article I think is where you'll find the most surprising content. This is an area in headache medicine that has been controversial. I think what we've got is new data - relatively new data, published in Neurology (in the Green Journal) in 2022 - the MOTS trial, showing that what we all thought was not necessarily true. In headache medicine, there was this mantra that, if somebody is overusing (too frequently using) a certain kind of headache acute medication, you've got to stop them; you've got to have them stop it completely before you can get them on a preventive treatment if you expect it to work. Turns out, in this trial, that's not the case. People were randomized to either stopping the overused acute medicine and starting a preventive versus continuing it and starting a new preventive, and they did equally well. I think that's really taught us that that dogma was not based in evidence (was not true), and what really matters is getting a patient started on an effective migraine preventive treatment. Dr Jones: Wow, that is really – that is kind of ground shaking, isn't it? That's going to change a lot of practices for a lot of neurologists out there. Do you think that's going to be well received, or has it been well received so far? Dr Gelfand: I think it has. I want it to get out there further, so I hope everybody will read in that chapter and really pick up on that piece. I think it's helpful for patients, too - that we don't necessarily need to disrupt what makes them feel like they're getting some acute, in-the-moment relief. We just need to make sure we're getting a good-quality migraine preventive therapy started. That's the most important thing. We don't necessarily need to ask them to change something about their acute treatment. Dr Jones: That's fantastic, and it certainly could make things a little more straightforward, I think for people who are helping patients manage this. To be honest with you, the term, “medication overuse” almost sounds like it's putting the onus on the patient a little bit. Dr Gelfand: It very much does sound that way. It is a very challenging term for a lot of reasons. And I agree with you that that's a problematic part of this whole terminology. Dr Jones: Well, just three minutes into the interview here and, Dr Gelfand, you've already changed people's practice. I think that's wonderful, and we'll look forward to reading that specific article in the issue. Again, from your view as a headache specialist and a leader in the field, what do you think the biggest debate or controversy is in headache medicine right now? Dr Gelfand: I think where we're really a little bit stuck in trying to figure out how to move forward is how to take care of patients who have continuous headache. It's not even really a fully defined term, but if you imagine a person who - they wake up, headache is present; it continues to be present throughout the entire day; they go to bed- it's still present; if they happen to wake up in the middle of the night to go to the bathroom, it's there then - it's just there all the time. It can be hard to imagine that situation is real - that somebody could have a headache that is continuously present for weeks, months - but this is true of some of our patients who have chronic migraine, our patients who have new, daily, persistent headache, certain other headache disorders. This entire group of patients who have continuous headache have historically been excluded from treatment trials, so our existing data don't necessarily generalize to how to treat their condition. And we need to change that, because this is a group that is arguably most in need of research, most in need of effective therapies. The question is how? Who exactly should be included in the inclusion criteria? And then, what are your outcome measures? Historically, in migraine treatment trials, we use headache days per month or migraine days per month. Days of headache per month may or may not be the right primary outcome measure for somebody who's starting from a point of continuous headache. Maybe more appropriate is, how many severe headache days you're having in a month, or how much disability you have from your headache disease. It's an area that's evolving and really does need to evolve, because this is a patient population that has been underserved in research thus far. Dr Jones: I learned that, I think, in reading one of the articles talking about continuous headache at onset – so, the headaches that are continuous from day one, which is, as I understand it, pretty uncommon. But really very little of the clinical trial data speak to how to care for those patients - is that right? Dr Gelfand: That is exactly right. And, epidemiologically, maybe not as common. But in a headache clinic, we certainly see patients who have had these headache disorders where it starts on one particular day, it becomes continuous within twenty-four hours of onset and has now been going for at least three months, and we would call that new, daily, persistent headache. Or equally commonly, people with chronic migraine where it ramped up over maybe a short to medium-long period to daily and continuous. And now they have been experiencing continuous headache for some number of months, if not longer. Dr Jones: This question may be a little bit of an unfair question. One of the challenges with headache is that, unlike some other areas of a diverse specialty of neurology, there aren't as many biomarkers as you might have for dealing with patients who have cerebral ischemia or neuromuscular disease. Do you find that that leads to more differences of opinion or more variability in diagnosis and management than you might see in other areas? Dr Gelfand: I'm so glad you asked that question. What I find that leads to is more stigma. Many of our patients are not believed, including by medical professionals who they've met before. People might think they are faking their symptoms, or that there's some sort of secondary gain, or this is something related to - they just don't know how to manage stress. This is a real problem for patients with migraine to be encountering so much stigma. As a headache medicine clinician, when I'm meeting a patient, oftentimes I need to make sure to acknowledge that, almost certainly, they've encountered that before. I need to reassure them that they're not going to be experiencing that in our headache clinic, and really try to undo some of that harm to be able to build trust that we're going to have a collaborative relationship moving forward - we're going to be a team; we're going to be determining the next steps in treatment together - and that I 100% believe them that the symptoms they are experiencing are real, are very challenging. Because migraine and other primary headache disorders are real neurologic diseases that can be quite severe. But because we have a paucity of biomarkers, it's hard for some people outside the field to recognize that. And that, I think, has been really difficult for patients historically. Dr Jones: So, a challenge for clinicians has become really more of a burden for patients. Dr Gelfand: Yes - well said. Dr Jones: Yeah. That's too bad

Regardless of the underlying cause of spinal cord disease, we have many tools at our disposal to improve symptoms and function in these patients. Even better, technology in this area is advancing rapidly. In this episode, Lyell Jones, MD, FAAN, speaks with Kathy Chuang, MD, author of the article “Symptomatic Treatment of Myelopathy,” in the Continuum February 2024 Spinal Cord Disorders issue. Dr. Jones is the editor-in-chief of Continuum: Lifelong Learning in Neurology® and is a professor of neurology at Mayo Clinic in Rochester, Minnesota. Dr. Chuang is an instructor in neurology at Harvard Medical School and assistant in neurology co-director at Paralysis Center, Massachusetts General Hospital and Spaulding Rehabilitation Hospital in Boston, Massachusetts. Additional Resources Read the article: Symptomatic Treatment of Myelopathy Subscribe to Continuum: shop.lww.com/Continuum Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @LyellJ Transcript  Full transcript available on Libsyn   Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the show notes. Subscribers also have access exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the show notes. AAN members, stay tuned after the episode to hear how you can get CME for listening. Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum Lifelong Learning in Neurology. Today, I'm interviewing Dr Kathy Chuang, who has recently authored an article on symptomatic management of myelopathy in the latest issue of Continuum, on spinal cord disorders. Dr. Chuang is a neurologist and physical medicine and rehabilitation specialist at Mass General, where she serves as Co-Director of the MGH Paralysis Program and Chief of the Neuromuscular Rehabilitation Program. Dr Chuang, welcome, and thank you for joining us today. Would you introduce yourself to our listeners? Dr Chuang: Hi, my name is Kathy Chuang. As you said, I'm a neurologist at Mass General Hospital specializing in neuromuscular medicine, also physiatry, physical medicine, and rehab. And I'm glad to be here. Dr Jones: Thank you for joining us. Basically, if we want to know more about managing spinal cord disorders, we have come to the right person, right? Dr Chuang: I try to do my best with all patients - yep. Dr Jones: For our listeners who are new to Continuum, Continuum is a journal dedicated to helping clinicians deliver the highest quality neurologic care to their patients, and we do this with high-quality and current clinical reviews. For our long-time Continuum Audio listeners, you'll notice a few different things with our latest issue and series of author interviews. For many years, Continuum Audio has been a great way to learn about our Continuum articles. Starting with our issue on spinal cord disorders (this issue), I'm happy to announce that our Continuum Audio interviews will now be available to all on your favorite open podcast platform, with some exciting new content in our interviews. Dr. Chuang, your article is absolutely full of extremely helpful and clinically relevant recommendations for the treatment of myelopathy, regardless of the cause. If there were one single most important practice-changing recommendation that you'd like our listeners to take away, what would that be? Dr Chuang: I think the most important thing to take away is that spinal cord injury of any type spans so many organ systems, it is good to get people - or multidisciplinary care - involved early on. There's eighteen model systems for spinal cord injuries scattered across the US. Those can be great avenues of resources for patients and for practitioners, for people around. Physical medicine and rehab specialists (our physiatrists or spinal cord injury specialists) can be very useful. And then, also for each individual organ system, there are specialists involved. And so, having that multidisciplinary care is probably the most important thing for a patient that's suffering from myelopathy because every patient is different and coordinating that care is so important to them. Dr Jones: So, teamwork is probably the most important thing, and I think most of our listeners who have taken care of patients with spinal cord disorders realize that that's really key. Your article - it leads off with such a great review of one of the big problems with myelopathy, which is spasticity management. From a medication perspective, I think many of us struggle with the balance between controlling the spasticity and some of the side effects of those medications, like sedation. How do you walk that fine line, Dr. Chuang? Dr Chuang: Spasticity management, like everything else, is patient directed. It depends on what the patient is most complaining of. If a patient has spasticity but they're not actually having any complaints from it, we don't need to treat, because of fear of side effects. I tend to try to use focal procedures (like botulinum toxin injections) earlier on, in order to try and spare side effects of antispasticity medications. Use of other conservative therapies, like bracing, stretching, is very essential. Another thing to consider is that dantrolene doesn't usually have side effects - cognitive side effects, at least - and actually can be monitored pretty closely for hepatotoxicity, which is its major side effect. Other possibilities are the baclofen pumps, which can be very useful in patients with spinal cord injury because their spasticity is often more in their lower limbs than in their upper limbs. By using multimodality approaches, we can definitely limit the amount of cognitive side effects of medications. Dr Jones: That's fantastic. Do you start with that multimodal at the beginning, or do you step into it with one, then the other, then the other? Dr Chuang: I usually start off with a low-dose baclofen because they usually have generalized tone - first, in order to see if they have cognitive side effects with it and if so, at what dose. Also, so that insurers have a trial of some medication before we proceed to something as expensive as botulinum toxin injection. But yes, if there's significant focal spasticity, especially, I try to bring in botulinum toxin injections as early as possible, just because of the possibility of minimizing the effect. Dr Jones: That's a great point - that you can start these from multiple angles and start them early. And great point about dantrolene - I think the hepatotoxicity makes many of us nervous. But it’s a key point there - that it can spare some of the cognitive side effects. Dr Chuang: Yes, and actually, it can be monitored pretty closely. As long as a patient has access to labs, we can check liver function tests weekly or every two weeks until you're on a stable dose, and after that, only at intervals. And it can be weaned off just as quickly. Dr Jones: Fantastic. Another issue that you cover really nicely in the article, that I think is an underrecognized complication of spinal cord diseases - neuropathic pain. What's your approach to that problem, Dr. Chuang? Dr Chuang: Neuropathic pain is very, very tough to treat a lot of times. I usually give the chance of gabapentin, pregabalin, and duloxetine early, just to see if we can start managing their pain early and to try to prevent potentiation of pain. But I also tend to try to get pain management specialists on early, and also keep in mind that there can be other causes of pain other than just the actual spinal cord injury itself. Because of deafferentation and reafferentation, patients may think of neuropathic pain, and it could be something as simple as appendicitis. If there's a change in pain, there always needs to be a workup for acute causes. Again, multidisciplinary treatment, especially with pain specialists, can be really helpful. Dr Jones: Great point about thinking of other causes, including appendicitis or the musculoskeletal things that I'm sure can be pain generators in this pain population, right? Dr Chuang: Yeah, it's very common. Patients can often fracture themselves just with a simple transfer and that can cause a huge flare-up of pain. So, not all pain should be just dismissed as being neuropathic or just from the spinal cord injury itself. Dr Jones: Great point - thank you. Another topic that you cover - that I think is mystifying to many of us - is the neurogenic bladder problems that occur in patients with myelopathy. You talk about the different types - how do you tell them apart? Dr Chuang: It's hard to tell them apart from a patient perspective because a patient will just say that they have difficulty with urination. With a spastic bladder or detrusor sphincter dyssynergia, oftentimes, patients will complain of a short stream and having to force things out. And with an atonic bladder or flaccid bladder, they have difficulty initiating a stream. What can be useful are postvoid residuals - where, if a patient is in the hospital, or if you have access to an ultrasound, or if they see a urologist - after they void, you measure the amount of urine left in their bladder.

This bonus episode of Continuum Audio features Continuum Aloud with Dr. Michael Kentris narrating the Selected Topics in Neurology Practice article from the February 2024 issue on Spinal Cord Disorders. Dr. Michael Kentris is a Neurologist at Bon Secours Mercy Health in Youngstown, Ohio and Continuum Aloud program lead. Continuum Aloud is verbatim, audiobook-style recordings of each Continuum article. It is a Continuum subscriber-only benefit, and audio files are available at ContinuumJournal.com at the article level or on the AAN’s Online Learning Center at continpub.com/Aloud. Additional Resources Read the article for free: Continuum 2024 and Beyond Listen to all of the Continuum Aloud articles: continpub.com/Aloud Subscribe to Continuum: shop.lww.com/continuum Social Media: @ContinuumAAN @DrKentris @LyellJ

Too much, or not enough? A wide range of nutritional deficiencies and toxic exposures may cause spinal cord dysfunction. To make matters even more confusing, the clinical presentations for these disorders may overlap. In this episode, Teshamae Monteith, MD, FAAN, speaks with Kathryn Holroyd, MD, an author of the article “Metabolic and Toxic Myelopathies,” in the Continuum February 2024 Spinal Cord Disorders issue. Dr. Monteith is the associate editor of Continuum® Audio and an associate professor of clinical neurology at the University of Miami Miller School of Medicine in Miami, Florida. Dr. Holroyd is an instructor in the Department of Neurology at Yale School of Medicine in New Haven, Connecticut. Additional Resources Read the article: Metabolic and Toxic Myelopathies Subscribe to Continuum: shop.lww.com/Continuum Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @headacheMD Transcript  Full transcript available on Libsyn Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the show notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you’re not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the show notes. AAN members, stay tuned after the episode to hear how you can get CME for listening. Dr Monteith: This is Dr Teshamae Monteith, Associate Editor of Continuum Audio. Today, I'm interviewing Dr Kathryn Holroyd on toxic metabolic myelopathies, which is part of the February Continuum issue on spinal cord disorders. Dr. Holroyd is an instructor in the Department of Neurology at Yale School of Medicine in New Haven, Connecticut. Katie, thank you so much for being with us on the podcast, and thank you so much for your excellent article. It was filled with a lot of really great tips. Dr Holroyd: Thank you - happy to be here. Dr. Monteith: I want to start off with knowing, how did you gain expertise in spinal cord diseases? Dr Holroyd: Yeah, I have a fairly diverse clinical background. My primary work now is as a neurohospitalist. But after residency training, I did two one-year fellowships: one in neuroimmunology and one in neuroinfectious diseases. I think, with those things together – you know, a lot of these, especially acute-onset myelopathies, tend to present inpatient for diagnosis – so, we see a lot of those in my hospital practice. Then, I think, specifically for toxic metabolic myelopathies - to identify these, you often have to know what it's not. So, my experience with some of the other autoimmune and infectious disorders really comes into play. Then finally, I kind of focused on global health work, which is why I primarily do neurohospitalist work - to allow for travel. I spent the past year working at a neuro HIV research site in Thailand, and I've done some work (mainly with education) in Zambia. But I've seen that, kind of, all how people's environments and local areas can really affect what disorders are more common, and I think it's really important to take that into account with especially this topic, as well. Dr Monteith: Well, your work in global health could be a whole other area, a whole other podcast that I would really want to record with you. But let's start with, what did you seek to accomplish when writing your article? Dr Holroyd: I think, when I was writing the article along with Dr. Berkowitz, the co-author, we really wanted to focus on things that would be clinically relevant, not just for neurologists, but for clinicians all over who may not have access to a subspecialist neurologist. We tried to focus less on metabolic pathways or disturbances and focus more on clinical pearls. I tried to think, “When I see these patients, what are the questions that I have that are not easily answerable from Google or UpToDate or a textbook? And how can we really use primary evidence to answer some of those questions? For example, what percent of patients with B12 deficiency actually have an abnormal MRI? Those are the things we were asking ourselves and, hopefully, that we were able to answer through the article. We focused on three main categories of toxic metabolic myelopathies, as you can see from the work. Dr Monteith: So, specifically, you've been writing about nutritional deficiencies, environmental and dietary toxins, drug abuse, medical illnesses, and oncological treatments. When you wrote your article and, comparing it to even, like, five or ten years ago, what has changed? Dr Holroyd: It's a great question because, I think, even when I started writing the article, it's easy to feel like not much has changed in these particular disorders. But if you go deeper, I think that's not the case. The main ways in which things have changed, I think, on the nutritional front, is there’s been an increase in weight loss and weight-loss surgery, which is one of the main contributors to all nutritional deficiencies. The second main category is - in some of these toxic myelopathies - is the increasing rates of drug use, particularly heroin, which we talk about in detail in the article. Additionally, along those lines, with climate change - we often don't think about the way that climate change can really affect disorders that are related to nutrition or the way that certain foods are prepared, especially with increasing rates of drought, and that really relates to konzo. Finally, there's been great advances in the treatment of all sorts of cancers, particularly with immunologic therapy. The one immunologic complication we talk about is with immune checkpoint inhibitors, and I think there's been a huge increase in clinicians seeing these as complications of checkpoint inhibition. So, those are the three main ways that I think these have evolved in the past decade. Dr Monteith: Great. You spoke about your interest in clinical pearls - can you describe some essential points that you wanted readers to take away with when diagnosing and managing patients that are presenting with myelopathies thought to be due to toxic or metabolic etiologies? Dr Holroyd: Yeah, and a lot of these are so different it's hard to find overarching themes, but I think there are a few that come through in the article. The first is that a lot - not all, but a lot - of these are reversible. Diagnosing them early is important and can really make a difference in patient outcomes. The second is a real clinical principle of all neurology that I learned from Dr Berkowitz, my co-author - is that neurology really is time course and localization. Amongst these, I think it's important to look at the time course, whether it's acute or subacute, and the location in the cord, whether it's a subacute combined degeneration or a more dorsal-column-only-predominant myelopathy - that can help you narrow the differential. A couple other small things is that, overall, these toxic myelopathies tend to be more thoracic cord-predominant and affect the legs more than the arms. In the majority of cases, the MRI will actually be normal, which is a big difference from a lot of the other autoimmune or infectious myelopathies. I think those are some main takeaways. And finally, you really have to be careful when you're interpreting the lab tests and make sure that the clinical picture fits with the lab tests that you're measuring - for example, the vitamin or other cause - and make sure that you really are correlating the diagnosis with that test. Then, I think the cause of the deficiency will affect your treatment choice; whether you're dosing supplements orally or IV, and what dose you choose - those are the major things to take into account. Dr Monteith: I really like what you say because, I think, as neurologists, we are always thinking about localization, localization, localization, but that time course also matters for a number of diseases. Dr Holroyd: And to that point, I think the clinical diagnosis is particularly important in resource-limited settings, where advanced diagnostics, such as MRI or lumbar puncture, may not be available. For example, konzo - the WHO has very clear clinical criteria of how to define this disorder, given that in most of the regions where cassava root is primarily eaten, there are not these diagnostics. I think we can apply that globally or even in our own practice in areas of the US or other places - to really rely on your clinical judgment and the time course and the localization of the biolopathy[IG1] . Dr Monteith: Yeah. What was that like when you were practicing in Zambia? Dr Holroyd: I worked primarily with Dr Deanna Saylor, who is there funding and working with neurology residents, and we would see a wide variety of clinical cases but have very little real-time information. So, I really admire the residents who train and work in Zambia and have to make clinical decisions with very little information. In those settings, the history – so, asking people about recent ingestions, any drugs, diet at home, any exposures that might cause increased risk of these conditions - is very important. And sometimes you have to rely on empiric treatments, such as vitamin B12, in cases where you may not be able to send for those tests - e

The explosion in diagnostic tools to identify immune-mediated myelopathies has led to much more precise diagnosis and treatment of these patients, but also created gaps in knowledge. In this episode, Kait Nevel, MD speaks with Michael Levy, MD, PhD, FAAN author of the article “Immune-Mediated Myelopathies,” in the Continuum February 2024 Spinal Cord Disorders issue. Dr. Nevel is a Continuum® Audio interviewer and a neurologist and neuro-oncologist at Indiana University School of Medicine in Indianapolis, Indiana. Dr. Levy is an associate professor at Massachusetts General Hospital and Harvard Medical School in Boston, Massachusetts.  Additional Resources Read the article: Immune-Mediated Myelopathies Subscribe to Continuum: shop.lww.com/Continuum Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @IUneurodocmom Guest: @mlevy18 Transcript  Full transcript available on Libsyn Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the show notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the episode notes. AAN members, stay tuned after the episode to hear how you can get CME for listening. Dr Nevel: This is Dr Kait Nevel. Today, I'm interviewing Dr Michael Levy on immune-mediated myelopathies, which is part of the February 2024 Continuum issue on spinal cord disorders. Dr Levy is an Associate Professor at Massachusetts General Hospital in Harvard Medical School in Boston, Massachusetts. Welcome to the podcast. Thank you so much for being here today and chatting with me about your article. I really enjoyed reading your article. I read through it, and it felt like, you know, just really enjoyable. I had no concept of time when I was reading it. I encourage all of the listeners to read the article, too. I'll start with just a really broad question, with - what is the most important, clinically relevant thing from your article that you'd like the neurologist listening today to know? Dr Levy: I would say that the group of conditions in which the immune system can attack the spinal cord are growing. We're getting better at identifying the specific antigens, like in the case of NMO neuromyelitis optica and in MOG antibody disease. We're getting better at identifying targets for treatment, like with neurosarcoidosis - identifying those biologics that seem to help. And then, we're even beginning to characterize some of the idiopathic forms, some of which follow covid, or vaccines, or other conditions. I think the message is that we're getting a lot better out there, and if you have a case of inflammation in the spinal cord, then this is something that has a good workup now (and people should be paying attention to articles like this that give them an idea for how to work this up), and then the most appropriate treatment. Dr Nevel: Right. And not to get too much “in the weeds,” but in your article, you really outlined very nicely an algorithm or stepwise approach in evaluating patients with suspected immune-mediated myelopathies. Could you just briefly go through the general principles of that evaluation and stepwise approach, and what you would consider really necessary tests to order for these patients? Dr Levy: Sure. I would say that the first thing that you want to do is to make sure that it's inflammatory. And to do that, we have – the blood tests are few and far in between. If you're dealing with inflammation in the spinal cord, the few ways that we have to convince ourselves that there's truly inflammation - there are MRI and spinal fluid - and those objective tests need to be considered in the appropriate clinical context. The order of events is: patient comes in, reports certain neurological functions that localize to the spinal cord - that's step one, Step two, neurological exam that confirms that there's a neurological problem that localizes to the spinal cord. And then, numbers three, four, five are objective workups, including MRI of the spinal cord and other parts of the neuraxis, CSF testing, and blood testing, all of which then support your differential diagnosis. For each diagnosis, that order is the same, and it should always result in an answer for you, which ultimately may all be negative (and then we have a plan for that, too). If all your workup is negative, you don't know what caused it - at least a plan to deal with that as well. Dr Nevel: Building off of that - in your article, you mentioned that there are shared features between the different immune-mediated myelopathies. We have some tests that can help us differentiate, but what are some of the limitations or strengths of our currently available diagnostic evaluations - our clinical clues to help us differentiate between the different types? Dr Levy: The biggest limitation, of course, is that it's hard to access the spinal cord. We're not going to biopsy almost any patient unless we really have to rule out cancer. Otherwise, we don't want to take a punch out of a very small spinal cord that's carrying a bunch of fibers going in and out of the brain. So, that is our biggest limitation. We can't physically see it under the microscope, so we have to infer what's going on with MRIs and spinal fluid. And of course, spinal fluid isn't necessarily directly in touch with the inflammation - it could just be around it and bathing it. But we're hoping that there are clues from the spinal cord that shed into the spinal fluid that we can detect by lumbar puncture. I do think that we're getting better and also we're identifying things in the bloodstream that could also impact the spinal cord. And of course, blood tests are much easier to do, and some of these blood tests look for antibodies, which we know last for months and months. So, even if a person is having trouble getting their workup done on time, these antibody tests are still useful, even months after onset. Dr Nevel: Yeah. In your opinion, what have been some of the bigger breakthroughs? And I know there's been a lot in immune-mediated myelopathies over, let's say, the past five to ten years. That's a long timeframe, and I know a lot of things have happened during that timeframe – but what do you think has made the biggest impact in either evaluation and/or treatment for these patients? Dr Levy: When I was training, everything in the spinal cord was always MS. It was just - everything was multiple sclerosis in this big bucket of MS that we thought was heterogeneous. Now we're identifying the biomarkers that actually are distinguishing these patients from MS. We know what the immune system is targeting now in many of these conditions. Then, based on that immunological pathway, there are drug targets that have been developed. So, for even a very small disease, with 20,000 people in the US (one in 100,000) who have neuromyelitis optica, we now have three FDA-approved drugs because the science is so well worked out. And now there are two trials in MOG antibody disease, for example. As we identify new biomarkers based on the antigen specificity of the disease, I think we're going to have more and more specific therapies for each of these conditions, even if they're rare diseases. Dr Nevel: Yeah, that's great. Thanks for mentioning those, and I urge the listeners to check out the article to read a little bit more about some of those treatments for NMO spectrum disorder and MOG antibody disease that are in trials. What's the most common mistake that clinicians can make when evaluating or treating patients with immune-mediated myelopathies. What should we watch out for or to try to avoid doing? Dr Levy: I would say, at the beginning, there might be an urge to overtreat because we know that “time is spinal cord” - we don't want to waste time; we don't want to lose time. Some clinicians might just be inclined to give high doses of steroids, even in cases that they're not sure are inflammatory. The big overlap here is especially in older people who might have vascular myelopathy, where steroids might make things worse and it might delay their care. So that's the first problem – is, when physicians rush to judgment. Then the other big problem is when they take their time, and they say, “Well, this is just multiple sclerosis, probably. And we know that, in the end, MS patients do the same whether they're treated or not treated, and so we can take our time with this.” Whereas if we know that this is actually NMOSD, time is spinal cord and destruction is ongoing and potentially irreversible. I would say that there's problems on both sides of the time window. My approach is to be aggressive very early on and try to identify whether or not it's inflammatory. And then if it's not, then you can take a step back and go to the other chapters in this continuum - try to figure out what this is – and if it is inflammatory, then you definitely want to get on top of the treatment. Dr Nevel: Yeah, finding that sweet spot; making sure that you're not waiting too long but that you're not treating inappropriately or the wrong thing. So, what do you think - let's s

While collectively uncommon, the clinical presentation of genetically-mediated spinal cord disorders frequently overlaps with other neurologic conditions. Our understanding of these disorders has grown considerably. In this episode, Kait Nevel, MD, speaks with Kara Stavros, MD, FAAN, author of the article “Genetic Myelopathies,” in the Continuum February 2024 Spinal Cord Disorders issue. Dr. Nevel is a Continuum® Audio interviewer and a neurologist and neuro-oncologist at Indiana University School of Medicine in Indianapolis, Indiana. Dr. Stavros is an associate professor of neurology and clinician educator at Warren Alpert Medical School of Brown University in Providence, Rhode Island. Additional Resources Read the article: Genetic Myelopathies Subscribe to Continuum: shop.lww.com/Continuum Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @IUneurodocmom Guest: @StavrosKara Transcript  Full transcript available on Libsyn Dr Jones: This is Dr. Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal, from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast of the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by clicking on the link in the show notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you’re not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the episode notes. AAN members, stay tuned after the episode to hear how you can get CME for listening. Dr Nevel: This is Dr Kait Nevel. Today, I'm interviewing Dr Kara Stavros on genetic myelopathies, which is part of the February 2024 Continuum issue on spinal cord disorders. Dr Stavros is an Associate Professor of Neurology and Clinician Educator at Warren Alpert Medical School at Brown University in Providence, Rhode Island. Welcome to the podcast. What is the biggest takeaway from your article that you'd like the neurologists listening to this to know? Dr Stavros: I would have to say that there's maybe two big takeaways that I would want to highlight. One would be that, generally speaking, in a nutshell, the genetic myelopathies can present with chronic and progressive symptoms, oftentimes (but not always) a family history of similar symptoms, and involvement of other structures outside of the spinal cord. Exclusion of the more treatable causes of myelopathy is a really key and important step in the diagnostic process. And because there are many different causes of genetic myelopathies, in some cases, the symptoms can overlap. I think this really underscores the utility of doing genetic testing to really confirm the precise underlying neurologic condition. The second takeaway that I would want to highlight is that, while treatment for most of these conditions is typically supportive, there have been a number of recent therapeutic breakthroughs for treatments in ALS, spinal muscular atrophy, adrenal myeloneuropathy, and Friedreich ataxia. While these aren't cures, it's really exciting and gratifying to see new therapeutics emerge via different mechanisms for patients with conditions that we've had very little treatment options for in the past. Dr Nevel: Yeah, I really enjoyed reading that in your article - about these treatments that have been coming out over the past several years. The one with Friedreich’s ataxia, too - that looked like it was really just recently approved this year. Dr Stavros: Yes. Dr Nevel: And so, kind of jumping off of that topic - there have been these exciting treatments that have been coming through. What do you think is going to be the next big thing? Or what do you think is the next thing that might come through? Or what's going on in research in genetic myelopathies that might help our patients? Dr Stavros: That's a really great question. I think that, as far as the future in this area, genetic testing has definitely grown in terms of being able to identify more genes now that are implicated in these disorders than ever before. But this is still an area where our knowledge is continuing to evolve. So, I think the future holds further advancements in our ability to successfully diagnose patients who have these conditions and provide them with the sense of closure that having a definitive diagnosis brings, as well as opening the door to potentially targeted treatment options once a specific diagnosis is made. Another thing I think the future holds is continued development of expanded treatment options for patients with these conditions, both in terms of advancing our supportive care capabilities and then also providing more disease-modifying therapies. Again, as I mentioned, in recent years, new disease-modifying treatments have actually become available for several of these conditions. And I think that's just the beginning. There's going to be more to come, for sure. Dr. Nevel: Yeah, that would be great. Going back to the genetic testing and how things are - we're finding more and more and more genes. When you decide that genetic testing is indicated, how do you counsel your patients about genetic testing and walk them through that process? Dr Stavros: Okay - I would say that it usually starts with having a conversation with the patient about whether they want to pursue genetic testing or not for the particular condition or conditions that are suspected. Genetic testing is really helpful to, again, confirm the diagnosis once the initial diagnostic workup perhaps has given you some clues as to what the underlying condition might be. Again, because sometimes the clinical symptoms can overlap in different genetic myelopathies in particular, the genetic diagnosis can be really important as far as getting a definitive, final diagnosis. Usually testing is pretty carefully considered and the risks versus the benefits are explored with the patient. Oftentimes, this is done in conjunction with a genetic counselor or with genetics clinic. So, there's a lot of teamwork there in working with the genetics department, at least in my experience. There's a lot of options that might include testing a panel of genes for the suspected condition, to up to whole-exome sequencing. Again, this is really like an evolving landscape. So, we have a current understanding of the genes that are implicated in some of the genetic myelopathies, but there's still so much that we don't know. So, a lot of times, testing can result inconclusive or may be falsely negative, and it can be tough because a negative test doesn't necessarily exclude a potential genetic etiology. It becomes a very nuanced, I think, conversation and journey with the patient. Dr Nevel: Yeah, and in your article you mentioned some of the health care disparities that exist around genetic testing and access to genetic testing, specifically. How do we, as clinicians, try to mitigate inequities in regard to access, or in regards to being able to offer our patients genetic testing - is there anything that we can do? Dr Stavros: I do think there are some resources available, where free or sponsored testing can be utilized from nonprofit organizations or pharmaceutical companies. But you're right that this is a real area for potential health care disparities. And making sure that we have equitable access to genetic testing is really important. Some of the issues that come up are: limited access due to location; due to socioeconomic factors; a lack of awareness on the part of the patient or sometimes the provider about testing that's available; cost, of course, being a big issue, oftentimes; and sometimes, distrust of how the medical information, the genetic information, might be used or protected. Dr Nevel: What do you think is one of the most challenging things about managing patients with genetic myelopathies? Dr Stavros: I think one of the more challenging aspects of the care is the diagnostic journey. I think that some of these conditions - most of them are not terribly common – and they may not always be at the top of our differential diagnosis in the course of a workup for myelopathy. The first step, I think, is really continuing to be aware of these conditions and not letting them become a “blind spot” when we're formulating a differential diagnosis for a patient with myelopathic symptoms. I think it can really take some time to reach the ultimate diagnosis for most of these conditions. Another challenging aspect, which I alluded to earlier, is sometimes when genetic testing might come back inconclusive or nonrevealing, and there remains some diagnostic uncertainty despite best efforts and a thorough workup -that can be frustrating as well, sometimes. Again, our knowledge of these genetics and the genetic mutations underlying these disorders is still really evolving. But on the flip side, there's a lot of rewarding aspects as well. I think one of the most rewarding aspects is trying to help patients identify interventions that improve their quality of life, and working with the patients and their families (who oftentimes become very expert in their own rare conditions in their own right), and working amongst the interdisciplinary teams. So many of these conditions are associated with extraneurologic manifestations, and so patients need coordination of care with other specialists. Hereditary spastic paraplegia is a great example, as well as Fr

Tumors affecting the spine are fortunately uncommon, and may arise within the spine or metastasize from malignancies elsewhere. Effective treatment is determined by tumor type, location, and urgency. In this episode, Allison Weathers, MD, FAAN, speaks with J. Ricardo McFaline-Figueroa, MD, PhD, author of the article “Spinal Cord Neoplasms,” in the Continuum February 2024 Spinal Cord Disorders issue. Dr. Weathers is a Continuum® Audio interviewer and the associate chief medical information officer at Cleveland Clinic in Cleveland, Ohio. Dr. McFaline-Figueroa is a physician at Dana-Farber Cancer Institute and instructor in neurology at Brigham and Women’s Hospital and Harvard Medical School in Boston, Massachusetts. Additional Resources Read the article: Spinal Cord Neoplasms Subscribe to Continuum: shop.lww.com/Continuum Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud More about the American Academy of Neurology: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Transcript  Full transcript available on Libsyn Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the show notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you’re not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the show notes. AAN members, stay tuned after the episode to hear how you can get CME for listening. Dr Weathers: This is Dr Allison Weathers. Today I'm interviewing Dr Riccardo McFaline-Figueroa on spinal cord neoplasms, which is part of the February Continuum issue on spinal cord disorders. Dr McFaline-Figueroa is a physician at Dana Farber Cancer Institute in Boston, Massachusetts, an instructor in neurology at Brigham and Women's Hospital and Harvard Medical School in Boston, Massachusetts. Welcome to the podcast. You do a really fantastic job in the article providing a comprehensive overview. But if you had to come up with the most important clinical takeaway from the article that you want our listeners to walk away with, what would that be? Dr McFaline-Figueroa: I think the most important thing to remember about tumors of the spinal cord is, one, that there is no specific diagnostic feature on imaging that can be used to determine what a neoplasm of the spinal cord is or even if it is a neoplasm - I think, going in broadly, when you're looking at a mass lesion of the spinal cord, is very important. Then the second is just to know that there's just such a wide range of cancer possibilities that can do this, that getting an appropriate diagnosis becomes very important. Dr Weathers: I think two really salient points right there. I want to explore something that you said a little bit more. You talk about the concept that there's no one diagnostic feature of the MRI, and actually, I was thinking about this as I was reading your article. What struck me, too, is that that holds true, actually, for the patient’s presenting signs and symptoms, right? As a neurohospitalist, I'm constantly struggling with always how to balance not missing a diagnosis such as this (a diagnosis of spinal cord tumor), versus putting a patient through what can be an often unnecessary and very costly workup, right? So, this is such a rare diagnosis, but the presenting symptoms and signs, just back radicular pain and weakness, are shared by so many common conditions. What's your approach to distinguishing between them, and what are some of the red flags you look for to know when further workup really is indicated? Dr McFaline-Figueroa: Certainly. I think there's not one way to decide whether you need to go down the route of exploring a neoplasm of the spinal cord, but there are certainly things that would clue someone into this needing to be the case. I think, one, as opposed to a lot of the monophasic illnesses that we see in neurology, certainly it's something that is progressive; is certainly something that increases the certainty, and it's different from ischemia of the spinal cord or an acute demyelinating event. I think another important feature is also just the context. I think, even though these are rare, when you're dealing with a patient with a known history of cancer, that's when “the rare” becomes common, and that's when you have to really start thinking about it being a neoplasm of the spinal cord. It's still not perfect; it covers some of the side effects of treatment that can look a little bit like a spinal cord neoplasm. But certainly, that should increase the level of suspicion for something going on in that compartment that's neoplastic. Dr Weathers: I think that's such a great take-home quote for our listeners to think about - when the rare becomes common. You actually hit on the point that I wanted to ask you about next. In neurology, we always talk about how important the history and the exam are - it's kind of our core of what we do. But it feels especially true when talking about neoplasms of the spinal cord. You mentioned, obviously - the big one is that if they have a history of cancer, especially in active history, that's a pretty big clue that something more serious could be going on. But what else is key in the history? Why are the history and the exam so especially important when you're concerned about or dealing with neoplasms of the spinal cord? Dr McFaline-Figueroa: When we're dealing with the spinal cord, we're dealing with a lot of different compartments. I think, to your question, the one where thinking about history and physical becomes the most important is when you're thinking about the possibility of leptomeningeal involvement, right? The leptomeningeal space is not easily imaged, right? We can't really see much what's going on in the CSF. And so, we rely on - imaging-wise - on there being deposition of cancer cells along the dura or along the direct surface of the cord. But oftentimes, that's not the case. That's when knowing exactly what someone's cancer history is, what their stage in the natural history is, whether they're progressing or not progressing, have some knowledge of what the oncologic medications that they're on are (because brain penetration is different for several of them), and then really hearing for those signs and symptoms that are connected to that compartment - signs of increased intracranial pressure, signs of cranial neuropathy that may or may not be evidence on imaging, radiculopathies. So those are the things that are very important in all investigations of spinal cord tumors. But certainly for leptomeninges, it's often the case that, really, history and physical are all you have to try to get the diagnosis right. Dr Weathers: You make, actually, a really great point in the article that I think it bears mentioning here. Because I was embarrassed when I read it, because I said, “I have been guilty of that” - that the history of, kind of, these very generic histories of cancer; you know, “Oh, they had lung cancer” - is probably not sufficient, right? That there's value in getting really specific. Why is that? Dr McFaline-Figueroa: That's certainly why we all specialize in different things, right? For a neuro-oncology standpoint, it sounds very different to me to hear the same history in a patient with melanoma versus the patient with bladder cancer. You think of melanoma from a neuro-oncologic standpoint, you're thinking of a cancer that is incredibly trophic for the brain and spinal cord, probably because it's derived also from ectoderm (so it's kind of the same origin of the cells), and it just makes your level of suspicion go so much higher when you are in that mind space. Thinking of a melanoma patient versus someone with a tumor, that very rarely (if it all) goes to the central nervous system. I think that's something that's really important. And those are two big extremes. But even - like I mentioned - even in lung cancer, certainly, small cell versus non-small cell are very different in terms of when and how they can affect the spinal cord or any part of the central nervous system. So, that one is a little bit more nuanced and, being a neuro-oncologist - but still, it’s specific as you can be when you're discussing with your neuro-oncology colleagues or medical oncology colleagues, and the better for trying to figure these things out. Dr Weathers: An excellent pro tip right there. You were very gracious about it - about that we all have different specialties. I was reflecting on that, too - this is such an important yet definitely pretty specialized topic; how did you become interested and develop your expertise in it? Dr McFaline-Figueroa: My clinical work is on all sorts of tumors of the central nervous system. Actually, in neuro-oncology, we also do a little bit of peripheral nervous system tumors, depending on how they present. And it's all a continuum. Not to use that - well, we just happen to be on Continuum. But it's all a continuum: brain, spinal cord - it's all one big compartment. And it forces you to be really familiar with all of those. And I think it's an interesting topic - we don't talk about it as much as we do for some of the other – you know, cancers of the brain, for example. In terms of becoming an expert, for me, I mean a lot of it is just, at this point, experience. A

Compressive myelopathy caused by degenerative spine disease is common, but the pathophysiology is surprisingly complex and there are potential surprises in the evaluation of these patients. In this episode, Katie Grouse, MD, FAAN, speaks with Ligia Onofrei, MD, author of the article “Structural Myelopathies,” in the Continuum February 2024 Spinal Cord Disorders issue. Dr. Grouse is a Continuum® Audio interviewer and a clinical assistant professor at the University of California San Francisco in San Francisco, California. Dr. Onofrei is an associate professor of neurology and neuromuscular medicine at the University of Utah in Salt Lake City, Utah. Additional Resources Read the article: Structural Myelopathies Subscribe to Continuum: shop.lww.com/Continuum Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud American Academy of Neurology website: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Guest: @Ligia_OnofreiMD Transcript  Full transcript available on Libsyn Dr Jones: This is Dr. Lyell Jones, editor-in-chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast of the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the show notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the show notes. AAN members, stay tuned after the episode to hear how you can get CME for listening. Dr Grouse: This is Dr. Katie Grouse. Today, I'm interviewing Dr. Ligia Onofrei about our article on structural myelopathies in the February 2024 Continuum issue on spinal cord disorders. Dr Onofrei is an Associate Professor of Neurology in neuromuscular medicine at the University of Utah, in Salt Lake City, Utah. Welcome to the podcast. Just to kind of get started, I wanted to ask you, the topic of your Continuum article is cervical and thoracic structural myelopathies - what are these and how common are they? Dr Onofrei: So actually, structural myelopathies are the most common myelopathies that we encounter clinically. I know in neurology we tend to focus on things like MS or NMO or transverse myelitis as the myelopathies that we talk about most commonly, but we actually see them a fair bit. As you will see in my article, it's really hard to actually give you a precise number as to how common they are. We know they're common because we encounter them a lot, but there are also a lot of patients out there who have them who are undiagnosed. Structural myelopathies really refer to both the symptoms of myelopathy but also having compression of the spinal cord. That's what you have to have in order to have a structural myelopathy. Dr Grouse: How did you become interested in this area of neurology? Dr. Onofrei: It’s a bit of a different kind of story in neurology than the usual career trajectory. Actually, when I was a resident, there was a patient at the VA who had Parkinson's disease and myelopathy, and he went undiagnosed for months because people kept blaming his dexterity issues and day changes on his Parkinson's. But, in fact, he really had a cervical myopathy that was actually quite severe. When we got him diagnosed. I remember thinking to myself, “I really want to learn more about it.” And I was asking around and what I saw, even though my attendance at the time were super smart and very well versed in neurological issues, they just weren't comfortable with degenerative disorders of the spine. I wanted to learn more. I read what was available and I actually went to the AAN Spine Course, which at the time was a full day. I met Dr JD Bartleson, who was my mentor - who became my mentor, I should say. He gave me some really terrific advice about how to learn more. When I finished my residency at the University of Utah, I went on to do a neuromuscular fellowship, also at the University of Utah. But during that fellowship, I actually had two months to spend as additional training time outside of neurology, and I chose to spend it with the spine and musculoskeletal physical medicine and rehabilitation specialists at the University of Utah. They taught me a lot about degenerative spine issues, musculoskeletal issues, and I felt I really, for the first time, had a really good grasp of the diagnosis, and also the interplay between degenerative spine issues and neurological disorders. And then after that, I did something even less typical for neurologists. After I graduated fellowship, I actually went on to have a clinic embedded within the neurosurgery department at our institution. I evaluated patients – like, a day a week - patients who had spine issues and were referred for surgical evaluation. I would evaluate the patients in conjunction with one of the neurosurgeons, and then we would decide together if they needed surgery. It was a really great education to understand the interplay between degenerative spine issues and neurological disorders. Dr Grouse: That sounds like a circuitous but very interesting path, and very fruitful in the end. You mentioned that even very adept clinicians can miss this important and actually common diagnosis. What are some early signs that are easily missed? Dr. Onofrei: I think, with myelopathy, the most important part is actually just thinking about it as a diagnostic possibility. If you think about it, then you will essentially ask the questions that are really important diagnosis. I think it can be especially difficult if it's a patient who has a preexisting neurological disorder because we get stuck in asking the kind of things we usually ask our patients with MS or Parkinson's, or whatever else they may have. But it's really important to understand the trajectory of symptoms always. If they're having dexterity changes, “Did that happen all of a sudden? Was there something else happening?” Asking about dexterity changes to start with is a super helpful, important part of the diagnosis. And then also asking about gait changes. Again, if they have a preexisting neurological diagnosis, asking them if they've had a big change, a rapid progression, if something else happened in their disease - that's the beginning step. It's actually very, very basic information, but asking about these changes is super important. Then, once people have identified those changes, then you can delve into the more specific questions that are really unique to myelopathy, like manipulating small objects, manipulating utensils - for example, zippers or buttons. That's a really sensitive way to ask for dexterity changes for myelopathy. For gait abnormalities, it’s a little bit less unique to myelopathy. A lot of the symptoms overlap phenotypically with, like, peripheral neuropathies. For example, having difficulty on uneven ground or getting your toes caught on something. But identifying a shift in your gait is usually that key initial diagnostic clue. Dr Grouse: Really, really helpful. And, I think, always a great reminder with almost anything - you don't think of it, you won't diagnose it. Sounds like for myelopathies - structural myopathies – this could be especially true. Thinking about this article, what do you think would come as the biggest surprise to our listeners who read the article? Dr. Onofrei: It’s a really great question. I think there can be a lot of different surprises in each little section. But, to me, the thing that stands out is how complex the pathophysiology of myelopathy actually really is. There's so much more than just direct compression of the spinal cord. When you have compression of the spinal cord, you are stretching the spinal cord; you are inducing changes to the gray matter, the white matter. But you're also changing the actual biology of the cells. When you're causing compression of the spinal cord, you're inducing hypoxic or ischemic injury, and that triggers a neuroinflammatory cascade and it causes apoptosis of the neurons and the oligodendroglia. I think what was really interesting to learn is that, when you're decompressing the spinal cord with surgery, that reduces that cascade of neuroinflammation but it doesn't eliminate it. You will still have some residual apoptosis of the cells even after decompression. This actually is probably one of the pieces of information that supports the idea that we really should be intervening at an earlier stage for these patients. Dr Grouse: Does this mean that, even after decompression, patients can continue to deteriorate or do worse as a result of that apoptosis and those changes? Dr Onofrei: I think that the way I would interpret that, more in practice, is that those patients might not improve. They might not have any improvement post surgery. In fact, any surgeon who is an ethical surgeon will tell you that they cannot promise improvement with decompressive surgery, but we do notice improvement in a significant proportion of patients. While you can never promise that there's actual hope for these patients, it's just that some patients may not improve and we don't have a great way to predict who will improve and who will not improve. Dr Grouse: I was also curious, when you mentioned about what chronic compression looks like, why does chronic compression look so different from acute compression of the cord, both how it presents and how the patients can look? Dr Onofrei: That's a really fantastic question. I thi

Traumatic spinal cord injury is a potentially devastating disorder. Best practices in clinical care for these patients has evolved, with implications for long term outcomes. In this episode, Aaron Berkowitz, MD, PhD, FAAN, speaks with Saef Izzy, MD, author of the article “Traumatic Spinal Cord Injury,” in the Continuum February 2024 Spinal Cord Disorders issue. Dr. Berkowitz is a Continuum® Audio interviewer and professor of neurology at the University of California San Francisco, Department of Neurology, a neurohospitalist, general neurologist, and clinician educator at the San Francisco VA Medical Center and San Francisco General Hospital in San Francisco, California. Dr. Izzy is an assistant professor of neurology at Harvard Medical School and an associate neurologist in the Department of Neurology, Divisions of Neurocritical Care and Cerebrovascular Diseases at Brigham and Women’s Hospital in Boston, Massachusetts. Additional Resources Read the article: Traumatic Spinal Cord Injury Subscribe to Continuum: shop.lww.com/Continuum Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud American Academy of Neurology website: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @AaronLBerkowitz Guest: @SaefIzzy Full transcript available here Dr Jones: This is Dr. Lyell Jones, editor-in-chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast of the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by clicking on the link in the show notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the episode notes. AAN members, stay tuned after the episode to hear how you can get CME for listening. Dr Berkowitz: This is Dr. Aaron Berkowitz. Today, I'm interviewing Dr. Saef Izzy about his article on traumatic spinal cord disorders from the February 2024 Continuum issue on spinal cord disorders. Dr. Izzy is an Assistant Professor of Neurology at Harvard Medical School and an associate neurologist at Brigham and Women's Hospital in Boston, Massachusetts. Welcome to the podcast, Dr. Izzy. So, let's say a patient comes to the emergency room with an acute spinal cord injury due to a car accident. Walk us through your approach. What's going through your mind when you hear this pager go off and you're walking down to the emergency room; what are you thinking? Dr Izzy: Yeah, great question. So, one of the first question is, what's the medical status of the patient? And, starting from, “How sick is the patient? (looking at the ABCD - basically, airway, breathing, circulation), make sure the patient is stable from that perspective, with the specific focus then going to be the injury level and the injury severity. And with that, once the patient is clinically stable, we try to pay very close attention to that aspect, especially since we know the patient is coming with a spinal cord injury from the prefield assessment. So, having a very close assessment to the spinal cord using a standardized tool (such as the ASIA, which is the American Spinal Injury Association Impairment Scale) will be very helpful to communicate the level of injury to the rest of the team, which usually is going to be a multidisciplinary team approach from the emergency room into neurosurgery, neurology and other disciplines where we'll be involved. So, having a standardized tool will be a key. ASIA, as a scale - it starts with a letter “A” - and to be A when there is a complete injury, with loss of motor and sensory, and E is basically normal exam (a neuro exam with normal motor and sensory examination). And between B to D, they have some preserved voluntary anal contraction and some of the reflexes, such as the bulbocavernosus reflexes, with a various degree of motor and sensory. Having an early introduction into this scale will be super helpful to communicate with other services. Then will be the decision about who I should image, and also, whether I should clear the C-collar or not. And this is also another sort of decision making, comes into the patient mental status, the injury severity, the level of injury, as well as ability to perform a reliable neurological examination - all plays a role into this decision. In this article, we have elaborated on the clinical decision making - an approach in the acute setting - and we provided, in Figure 1, a comprehensive approach about patient we should think about imaging and what modality of imaging, as well. Dr Berkowitz: Perfect, that's so helpful. So, you're thinking through the ABCDs, as you mentioned. And then a detailed neurologic exam to get a sense of the degree of injury. And then you mentioned the decisions about imaging. Tell us a little bit about how you think about who to image, what to image, how to image, after you've done your neurologic exam. Dr Izzy: So, the imaging in general, as we know, that previously used to be an x-ray. But the recent literature really focus on utilizing the utility of high-quality CAT scans as it's provide more comprehensive characterization of vertebral fractures. And that will be very helpful to identify the level and severity of radiographic injury. However, MRI will always be superior, as it also provides the extent of the cord compression, signs of cord injury, as well as could help us rule out ligamentous injury within, especially, the first 48 hours post the event. In addition to that, we have to pay attention to a patient at risk of having vascular injuries, as many spinal cord, especially the cervical and skull-based injuries, can associate with blunt cerebrovascular injuries, which often missed in the emergency room, and even in the acute stay, as no one would have thought about that aspect. That's why, in this article, we have highlighted the role of Memphis criteria, which is a very valuable tool to identify patients at risk to be scanned. And also the Biffl Scale, which used to be known at the Denver, and modified Denver Scale, to assist classifying the level of vascular injury. Dr Berkowitz: Great. So, I want to pick up on a number of the things you mentioned there. So, let's talk about injury to the bony structures that could result in impingement on neurologic structures, such as the nerve roots or cord or cauda equina. So, Often, neurology and neurosurgery are consulted together in these patients, right? And both arrive at the bedside in the emergency room. Tell us a little bit about working with our neurosurgical colleagues to figure out who should go for surgery, what type of surgery they should go for, and the neurologist’s role in helping in that decision making. Dr Izzy: I believe the neurologists have a significant role in the acute setting, especially with performing a very thorough, refined neurological examination when it comes into assessing the cranial nerve, because often traumatic spinal cord injury could associate with traumatic head injury. In addition to that, perform a very thorough motor and sensory exam, with a specific look into reflexes, as well as anal reflexes. And documenting that, in conjuncture with the neurosurgery colleague, will be super helpful. We have to know that doing neurological assessment, or also relying on them, the ASIA scale is a key, but also could be confounded in the acute setting with other multisystemic events, including respiratory failure, using some pain medication, traumatic brain injury, hypotension, which all could confound the initial exam. That's why having a repeated exam for this patient throughout the hospital stay will be a key, especially when we are using some of these examination in the acute setting to guide our prognostication. Also, when it comes into the neurological assessment, looking into, not only the level of injury, but paying attention into the levels below. And documenting this exam is also a very critical aspect of assessment. One of the early decisions we share - many times when we, as neurologists, get consulted on these patients who should go to surgery, and that's a whole topic by itself discussed thoroughly in this article about the literature on a patient who should basically pursue surgery. And, one of the main highlight of the literature that, pursuing surgery in less than 24 hours has been associated with improved outcomes. Yet the literature on that still need further evaluation, especially now the most common practice that patients with worsening exam, mass effect, and epidural mass takes priority. But further studies on this area definitely require further exploration. Dr Berkowitz: Another aspect you mentioned is blunt cerebrovascular injury - so, injury to the carotid or vertebral arteries in the neck or in the skull base. So, are CT angiograms part of the standard neuroimaging now for patients with spine injury, or on a case-by-case basis, or perhaps should they be? Dr Izzy: Great question. It's not. That's why paying specific attention for a patient at risk, and that's where the Memphis screening protocol takes place. And we encourage our colleagues from neurology and neurosurgery, as well as emergency department, to try to keep this sort of screening, helpful protocol handy when approach traumatic spinal cord injury. More specifically patients, who have basilar skull fracture with involvement of the carotid canal; the one with a basilar fracture

The spinal cord is a fragile network containing hundreds of millions of neurons, all passing through a conduit about the size of a dime. A consistent, organized approach to the diagnosis of spinal cord disease is necessary to give patients the best possible care. In this episode, Teshamae Monteith, MD, FAAN, speaks with Carlos Pardo, MD, author of the article “Clinical Approach to Myelopathy Diagnosis,” in the Continuum February 2024 Spinal Cord Disorders issue. Dr. Monteith is the associate editor of Continuum® Audio and an associate professor of clinical neurology at the University of Miami Miller School of Medicine in Miami, Florida. Dr. Pardo is a professor of neurology and pathology at Johns Hopkins University School of Medicine and director of the Johns Hopkins Myelitis and Myelopathy Center in Baltimore, Maryland. Additional Resources Read the article: Clinical Approach to Myelopathy Diagnosis Subscribe to Continuum: shop.lww.com/Continuum Earn CME (available only to AAN members): continpub.com/AudioCME Continuum® Aloud (verbatim audio-book style recordings of articles available only to Continuum® subscribers): continpub.com/Aloud American Academy of Neurology website: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @headacheMD Transcript Full Transcript Available Here Dr Jones: This is Dr. Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal, from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast of the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the show notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the show notes. AAN members, stay tuned after the episode to hear how you can get CME for listening. Dr Monteith: This is Dr Teshamae Monteith, Associate Editor of Continuum Audio. Today, I'm interviewing Dr Carlos Pardo about his article on an Integrative Clinical Approach to Myelopathy Diagnosis, which is found in the February 2024 Continuum issue on spinal cord disorders. Dr Pardo is a professor of neurology and pathology at Johns Hopkins University School of Medicine in Baltimore, Maryland. Welcome to the podcast. Carlos, thank you so much for this wonderful article. I think it was great! Dr Pardo: Thank you very much for the invitation and, particularly, to continue to write about myelitis and myelopathy - that is one of my passions in my activities as a clinical neurologist. And I think that this is basically one of the areas in which I thought, after finishing my residency training here, to focus, because there was absolutely no good understanding of the biology, clinical profile – particularly, understanding of the pathophysiology of myelitis and myelopathies, and what was called (at that time) transverse myelitis. So, that is what I have spent the past 25 years is try to understand that concept and apply what I was trained, as a neurologist and neuropathologist, to be translated in the clinical practice. Dr Monteith: Great. Well, I definitely want to know - how did you get into this area? Dr Pardo: That's a very nice question. Dr Monteith: I'm going to give you an easy one. Dr Pardo: I was trained as a clinical neurologist, but at the same time was trained as a clinical and experimental neuropathologist. When I finished my residency training, along with some of my co-residents and colleagues in my residency training, we took the challenge to take a neurological disorder that was called at that time transverse myelitis, to investigate diagnosis, clinical neurology of those patients, and investigate the etiological factors contributing to that. Very soon, we discovered that that group of patients that we call transverse myelitis was a very heterogeneous group of patients. And that basically put us in the situation to expand our approach to investigate what were those etiological factors contributing to those pathologies that we call, at that time, transverse myelitis. Since then, we have been focused on that. We have been focusing on characterizing patients with inflammatory myelopathies, with vascular myelopathies, with patients with infection disorders associated myelopathies. That is one of the main messages of the paper, and is - we need to think in a very etiological approach, because the variety of etiological factors that may contribute to spinal cord disorders is quite broad - it's very extensive. We need to be extremely careful when we approach those patients. There are very common myelopathies, there are very rare myelopathies. So, obviously, we always look for the commonalities and common pathologies, but we shouldn't basically forget about those myelopathies that may be rare but are present. I will say, frequently, we ignore the possibility of metabolic-associated myelopathies because we don't see those too much. But after we do an analysis of that equation - the clinical profile, temporal evolution, lesion topography, and biomarkers in imaging, blood, spinal fluid - and we don't find a clear explanation, we need to stop a little bit and think more about other things that we are missing. And frequently, metabolical disorders of the spinal cord are missed, or other type of pathology. That the reason the clinician need to have open mind and, occasionally, need to think out of the box, particularly when there is no clear answer to the search for etiological factors. Dr Monteith: I mean, when we think about spinal cord lesions, they can obviously be devastating because they affect patient's ability to ambulate. Why don't you tell us the most important takeaways from your article? Dr Pardo: Yeah, so this is a very important aspect of the article. The first thing is, if we are going to treat the patient, if we are going to focus in the management of a clinical problem, we need to understand first, what is the clinical diagnosis? What is the cause of the problem? Importantly, what is the etiology or the etiological factors contributing to that problem? The first thing that I always emphasize is, we are not able to treat a patient with a neurological condition if we don't have a very precise diagnosis, regardless what we are investigating in that patient. Specifically, for spinal cord disorders, there is a multitude of etiologies and pathogenic factors and other causes of the disease that may be involved. For that reason, the clinician, the health care provider, need to be aware about how to approach that question; is, we need to answer first the cause and the profile of the spinal cord disorder. And when we need to answer the cause, we need to focus first in evaluating clearly what has been the evolution of the symptoms, how is the neurological exam, how the evolution of the symptoms are going to help us to identify those etiological factors that we are looking for. For that reason, in the approach that I am suggesting to take in patient with the spinal cord disorder, the first critical element of that approach is to sit down and talk very well with the patient about what is going on - what are the main symptoms that are present, what has been the temporal evolution of those symptoms, and what has been basically the pattern of progression of those symptoms - because those are the clinical elements that will facilitate the clinician a much better understanding for the clinical diagnosis. Evaluating the clinical profile of symptoms and evaluating the temporal profile of symptoms is probably the first step for solving that critical equation about the diagnosis of spinal cord disorders. The main target is to establish a diagnosis. Dr Monteith: And that's really the bread and butter of neurology, because we have a global audience and we have some neurologists that practice in areas with very limited resources. But you do speak of some very cool things that I want to also touch on, such as precision medicine, the advances in biomarker development and neuroimaging, as well as investigating different viral etiologies in the pathology of spinal cord disease. So, can you just speak to some of that? You've been in this field now - you said, 25 years - how that evolution has helped you better treat patients. Dr Pardo: That's a very important question, because in 25 years we have learned tons about myelopathies, myelitis, and noninflammatory myelopathies - and it's quite amazing. I think that one of the most important aspects of spinal cord disorders is that, in the past 25 years, we have learned about mechanism of the disease in spinal cord disorders. Back in the 20th century we used the term transverse myelitis, and one of the main messages that I have for the clinicians who are reading the article is, please stop using that terminology. We have now capability to establish a more precise diagnosis, a more etiologically oriented diagnosis. If you can take a look at what happened in the past 25 years, understanding spinal cord disorders is quite amazing. We have a better understanding of the immunological factors that contribute to myelopathies. We are able to diagnose myelopathies associated with aquaporin 4 disorders, or MOG-associated disorders, or demyelinating diseases, or infectious disorders. So, in the past 25 years, with a combination of different tools in laboratory studies, studies of spinal fluid analysis, studies of the blood, we have basically able to identify biological markers that may guide us to treat more precisely those patients that are suffering from immune-related disorders. In

Spinal cord disorders are common and frequently disabling. Despite advances in our ability to diagnose and treat patients with spinal cord disease, many are underserved by their health care systems due to gaps in knowledge and care. In this episode, Lyell K. Jones Jr, MD, FAAN, speaks with Shamik Bhattacharyya, MD, FAAN, who served as the guest editor of the Continuum® February 2024 Spinal Cord Disorders issue. They provide a preview of the issue, which publishes on February 8, 2024. Dr. Jones is the editor-in-chief of Continuum: Lifelong Learning in Neurology® and is a professor of neurology at Mayo Clinic in Rochester, Minnesota. Dr. Bhattacharyya is the Anne M. Finucane Distinguished Chair in Neurology and chief of the division of spinal cord disorders at Brigham Women's Hospital and an assistant professor of neurology at Harvard Medical School in Boston, Massachusetts. Additional Resources Continuum website: ContinuumJournal.com Subscribe to Continuum: shop.lww.com/Continuum American Academy of Neurology website: aan.com Social Media facebook.com/continuumcme @ContinuumAAN Host: @LyellJ Guest: @shamik_b Full transcript available here Transcript  Dr Jones: This is Dr. Lyell Jones, Editor-in-Chief of Continuum, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. Thank you for joining us on Continuum Audio, a companion podcast to the journal. Continuum Audio features conversations with the guest editors and authors of Continuum, who are the leading experts in their fields. Subscribers to the Continuum journal can read the full article or listen to verbatim recordings of the article by visiting the link in the show notes. Subscribers also have access to exclusive audio content not featured on the podcast. As an ad-free journal entirely supported by subscriptions, if you're not already a subscriber, we encourage you to become one. For more information on subscribing, please visit the link in the show notes. Dr Jones: This is Dr. Lyell Jones, Editor-in-Chief of Continuum: Lifelong Learning in Neurology. Today I'm interviewing Dr. Shamik Bhattacharyya, who recently served as Continuum’s Guest Editor for our latest issue, on spinal cord disorders. Dr. Bhattacharyya is a neurologist at Brigham and Women's Hospital, where he serves as Chief of the Division of Spinal Cord Disorders and as an Assistant Professor of Neurology at Harvard Medical School, in Boston, Massachusetts. Dr.  Bhattacharyya, it's great to see you - welcome. Thank you for joining us today. Dr Bhattacharyya: Good to see you, Dr. Jones. I look forward to speaking. Dr Jones: So, for our listeners who are new to Continuum, Continuum is a journal dedicated to helping clinicians deliver the highest neurologic care to their patients. We do so with high-quality clinical reviews and content in our journal and in our audio format. For our long-time listeners to Continuum Audio, you'll notice a few different things with our latest issue and our latest author interviews. For many years, Continuum Audio has been a great way to learn about Continuum articles. Starting with this issue on spinal cord disorders, I'm happy to announce that our Continuum Audio interviews will now be available to all on your favorite open podcast platforms. We'll hear some exciting new content in our interviews, and we're also going to introduce interviews with our guest editors, like Dr Bhattacharyya, who are really indispensable in putting these issues together. In this issue, specifically, Dr. Bhattacharyya is full of extremely helpful clinical descriptions and treatment strategies for patients with spinal cord disorders. As the editor, you got really a broad view of the whole range of spinal cord disease. What was the most surprising thing when you were reviewing these articles? Dr Bhattacharyya: I think as a field, neurology - the knowledge base in neurology - grows bigger and bigger and bigger each day and in fields hard to keep up and how to integrate all of it together, right? I think all of us deal with it. And that's the hope of Continuum, is that you can provide these periodic refreshers. I got refreshed myself! Even though I see the patients day in and day out, when you actually read about the advances, for example, in hereditary spastic paraplegias, or the nuances of how neoplasms in the spinal cord are now classified- you say “wow”, I didn't actually know that. The knowledge spreads and grows, and I think that's the beauty of being an editor of some of these issues - is that you get to learn yourself and maybe perhaps even apply them in the clinical situation. Dr Jones: You and I are both educators. And that's, I think, one of the secret joys of teaching is that you end up learning a lot, sometimes from the people you're teaching, right? I guess maybe that's not a surprise - that you learn something by reading it. I guess it was probably pretty nice, huh? Dr Bhattacharyya: It was very good. I think the authors all come from different geographic backgrounds, even from different training backgrounds. In spinal cord disorders, there are trials in some aspects, but in other aspects it's really opinion-based practice, right? So, it was good to also see how other institutions do it. And I imagine it's the same for readers when they see how they do it at their institution and also get a viewpoint of how it's done at other places. That's the valuable perspective piece for putting together a different of authors and see how people do it at different places. Dr Jones: Always nice to learn from others. And speaking of learning - for our clinicians who are listening to our interview today, Shamik, tell us a little bit about the basics of how spinal cord disorders present. I know as an educator, sometimes for, especially junior learners, it's a little mysterious and I'm not really sure why that is, but what are some of the basic clinical tenets of how spinal cord disorders present? Dr Bhattacharyya: I'm glad you brought this up, because in some ways, spinal cord is the orphan child of neurology, right? I think for most neurology trainees, the nervous system stops at the brainstem and then progresses again at the nerves. The spinal cord is really just viewed as this conduit of tracts up and down, and that's all it does is a big set of wires, which is not true, right? A lot of primary neurological processing happens at the level of the spinal cord, and it really is a continuation of the central nervous system. And I hope, with this issue, people get a sense of that. For spinal cord disorders (also called myelopathy; the name goes, synonymously, hand in hand), I think one of the principal functions of the spinal cord is balance. A lot of the program - the neural programming of balance on postural reflexes are hard wired into the spinal cord. I think one of the key aspects of spinal cord disorders is imbalance. I think that people should think of this as a core feature of myelopathy. If you take an example for cervical spondylotic disease, people think, is it going to be off your hands? Well, I think most patients with cervical spondylotic myelopathy actually complain of gait imbalance as one of the early features of the disease. So, imbalance, bilateral weakness, and/or bilateral numbness, tingling, paresthesia - those aspects are suggestive of spinal cord disorder. Bowel and bladder dysfunction can be, but it's not universally true. Now, there’s some specific symptoms that I think are especially suggestive of spinal cord disorders I think that are kind of fun to ask about, and if true, can help you localize. One is the Lhermitte sign; you ask people to flex their neck and say, like, “Do you feel sharp, shooting thing, like, down your hands or your back?” In your legs? If true, you have something, right? That's a spinal cord disorder. The other sign that I think is clinically helpful is weakness on one leg and numbness on the other, like Brown-Séquard syndrome or hemicord syndrome. If you find that to be true - and you often see that with multiple sclerosis lesions or other traumatic lesions - that is a spinal cord disorder. I think those clues can come out in history and on exam, and can help you localize it better. Dr Jones: It's nice to know those specific features - in other words, those things that, when you do see or hear them, really should make us think about spinal cord disorders, right? Again, they might not be the most common way they present, but it's good to have those in your pocket, right? Dr Bhattacharyya: Right. Dr Jones: You mentioned this - spinal cord pathology occupies kind of an interesting place in the neurological world, right? There really aren't “myelopathists,” but you direct a division on spinal cord disorders, which is - I think is pretty uncommon. Tell us a little about that. How does that work at your institution? Dr Bhattacharyya: Maybe I can start with the history of this, right - of how this actually came about. I was graduating as a fellow and entering as a faculty in our neurology department. Initially, my interest was in autoimmune neurological disorder - it still is in autoimmune neurological disorders. And yet, when they saw patients who came in for myelitis and turned out they didn't have an inflammatory myelopathy, there really was no home for them, right? - it's a strange space. And that includes even for garden-variety, cervical spondylotic disease that's causing myelopathy - there is no good neurology home for those patients. After the first year of seeing patients, I felt that we need to do better for that. That's why we ended up opening the spinal cord disorders clinic, which was actually the only neurology-based one in our system. There are plenty run by physiatry, surgery, pain management, and other services. But the only neurology one in our system focused specifically on neurologic management of patients with any type of spinal cord pathology. Dr Jon

Continuum Audio features conversations with the guest editors and authors of Continuum: Lifelong Learning in Neurology, the premier topic-based neurology clinical review and CME journal from the American Academy of Neurology. AAN members can earn CME for listening to interviews for review articles and completing the evaluation on the AAN’s Online Learning Center. Read the articles: ContinuumJournal.com Subscribe to Continuum: shop.lww.com/Continuum More about the American Academy of Neurology: aan.com