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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.

2 DAYS AGO

Neuropalliative Care in Severe Acute Brain Injury and Stroke With Dr. Claire Creutzfeldt

Severe acute brain injury presents acute and longitudinal challenges. Addressing total pain involves managing physical symptoms and providing emotional, social, and spiritual support to enhance quality of life for patients and their families. In this episode, Kait Nevel, MD, speaks with Claire J. Creutzfeldt, MD, author of the article "Neuropalliative Care in Severe Acute Brain Injury and Stroke" in the Continuum® December 2025 Neuropalliative Care issue. Dr. Nevel is a Continuum® Audio interviewer and a neurologist and neuro-oncologist at Indiana University School of Medicine in Indianapolis, Indiana. Dr. Liewluck is a professor in the department of neurology at the University of Washington in Seattle, Washington. Additional Resources Read the article: Neuropalliative Care in Severe Acute Brain Injury and Stroke With Dr. Claire Creutzfeldt 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: @cj_creutzfeldt Full episode transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum. Thank you for listening to Continuum Audio. Be sure to visit the links in the episode notes for information about earning CME, subscribing to the journal, and exclusive access to interviews not featured on the podcast. Dr Nevel: Hello, this is Dr Kait Nevel. Today I'm interviewing Dr Claire Creutzfeldt about her article on neuropalliative care in severe acute brain injury and stroke, which appears in the December 2025 Continuum issue on neuropalliative care. Claire, welcome to the podcast, and please introduce yourself to the audience. Dr Creutzfeldt: Thanks, thanks for having me. Yeah, I'm an associate professor of neurology at the University of Washington. I'm a stroke neurologist and palliative care researcher and really have focused my career on how we can best integrate palliative care principles into the care of patients with severe stroke and other neurocritical illness. Dr Nevel: Wonderful. Well, I'm looking forward to talking to you today about your excellent article that I really enjoyed reading. To get us started, can you tell us what you feel is the most important takeaway from your article for the practicing neurologist? Dr Creutzfeldt: Yeah. You know, I think one is always a little biased by what one is working on currently. And I think what I'm most excited about or feel more strongly about is this idea that stroke and severe acute brain injury are not an event, but really a chronic illness that people are left with usually for the rest of their lives, that change their life radically. And I think that education, research funding, also the clinical setting, current healthcare models aren't set up for that. And this idea that severe acute brain injury, you know, should be viewed as a lifelong condition that requires support across all ranges of goals of care. So curative, restorative, palliative and end-of-life care. Dr Nevel: Yeah, I love that part of your article, how you really highlighted that concept. And I think obviously that's something that we see in neurology and learn, especially as we transition out of our residency stages. But I think especially for the trainees listening, can sometimes be hospital inpatient-heavy, if you will, that kind of you can lose sight of that, that these acute strokes, severe acute brain injury, it turns into a chronic illness or condition that patients are dealing with lifelong. Dr Creutzfeldt: Often what we do in a very acute setting is like, is really cool and sexy and like, we can cure people from their stroke if they come, you know, at the right time with the right kind of stroke to the right hospital. And often the symptoms that people come in with much later on are harder to treat and address, partly because the focus in education, clinical and research just hasn't been as much on that time. Dr Nevel: Yeah, absolutely. So, can you talk to us about this concept of total pain? What does it mean, and how do we incorporate this concept into the way that we view our approach, our patient care? Dr Creutzfeldt: Total pain is a very old word, but it's sort of coming back into fashion in the palliative care world because it really describes all those sources of suffering or sources of distress, like, beyond what we sort of really think of as sort of the physical symptoms in recovery of stroke. As many of you know, palliative care often thinks in this multidimensional way of the physical distress, physical pain, but also psychological, emotional, social and spiritual, existential. And both- we sort of created sort of a figure that incorporates all of them and also includes both patients and their family members. They share some of these sources of distress, but they also have distinct ones that need to be addressed. And at the core of that total pain is what we need to provide, is sort of optimal communication and goals-of-care prognosis. Dr Nevel: Yeah, I'm thinking about all of those aspects and not just focusing on one. How does the disease trajectory of severe acute brain injury and stroke play a role in the palliative care approach? And how should we kind of going back to that original point of this idea of severe acute brain injury being an acute event and then oftentimes turning into kind of a chronic condition? How does that play a role in how we address palliative care with our patients, or kind of the stages of palliative care with our patients? Dr Creutzfeldt: Yeah, I think several things, especially for neurologists, is the more traditional palliative care illnesses, like cancer or congestive heart failure, illnesses where people are diagnosed when they're still functioning at a relatively high level and tend to have time to consider their prognosis and their goals of care in the end of life wishes and to meet with palliative care and to consider their personhood. Who am I? What's most important for me? And stroke, people with stroke, they not only present at their worst, they meet us at their worst, at a time when the patient themselves usually can't speak for themselves, when their personhood has been stripped from them. And then as providers, we, you know, we often really just get that one opportunity to get the conversation right and to guide people towards, you know, what we would call optimal and goal-concordant care. So, the challenges are many. I do think that the burden of these early conversations is on neurologists and really requires the neurologists to show compassion, to learn communication skills, think really hard about how you want to communicate prognosis and goals of care early on, because it's going to color people's experiences and decisions longitudinally. You asked about, sort of, this trajectory. And I do think it's important to think about, you know, what really happens even after the thrombectomy or even after we discharge people, especially from the ICU. Because for us, often after sort of day five or six, you know, we're sort of done. We're thinking about secondary stroke prevention. And, you know, how do I get the patient to rehab or out of the hospital? For the patients and families, this is when it really all just starts. You know, this is when they- when they're first memories are usually, you know, they hardly remember that acute setting. And so, when they are medically stable, we're done with the acute blood pressure treatment where we've removed the Foley, we've made a decision about nutrition. For us that tends to be a time where we let go a little; for patients and families that tends to actually be the time when they have to think about how am I going to live with this and what are the next several months or years going to look like? And so being there for them is important. Dr Nevel: That's such a, I think, important point, that when we have our plan in place, we know medically what the plan is for that patient and we're starting to step back, think about rehab or discharge. That's when oftentimes more quote-unquote "reality" steps in for patients and families about what their future is going to look like. Dr Creutzfeldt: And medical stability is not even close to neurological stability. And so, they are still in the middle of real prognostic uncertainty, and often waxing and waning symptoms or new symptoms coming up for them. Like pain, you know, post thalamic pain syndrome, just as an example, tends to be something that doesn't develop until later. Dr Nevel: Right, right. Absolutely. And since you touched on this concept of prognostic uncertainty, and, you know, that's something that's so challenging in severe acute brain injury, especially the early days when you talk about this, you know, that things tend to become a little bit more certain as more time passes. But these are really hard conversations because a lot of times feel like big decisions that need to be made early on, you know? Dr Creutzfeldt: Huge! Dr Nevel: Sometimes things like trach and PEG and things like that. How do you approach that conversation? I know you talk about that a little bit in your article. You touch on that, some of the, kind of, strategies or concepts that we use in palliative care to approach this prognostic uncertainty with patients. Dr Creutzfeldt: Yeah, I think the challenge is to balance this acknowledging uncertainty with still being able to guide the families and allow them to trust you. So, there are a few things that I have said in the past, and I have taught in the past, and I don't use anymore. They include sentences like I don't have a crystal ball, for example. Nobody was asking you for one. The other one that I want us to

22 min
17 DEC

End-of-Life Care and Hospice With Dr. Claudia Chou

In the hospital setting, neurologists may be responsible for managing common end-of-life symptoms. Comprehensive end-of-life care integrates knowledge of the biomedical aspects of disease with patients' values and preferences for care; psychosocial, cultural, and spiritual needs; and support for patients and their families. In this episode, Teshamae Monteith, MD, FAAN, speaks with Claudia Z. Chou, MD, author of the article "End-of-Life Care and Hospice" in the Continuum® December 2025 Neuropalliative Care 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. Knox is an assistant professor of neurology and a consultant in the Division of Community Internal Medicine, Geriatrics and Palliative Care at Mayo Clinic in Rochester, Minnesota. Additional Resources Read the article: End-of-Life Care and Hospice 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 Full episode transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum. Thank you for listening to Continuum Audio. Be sure to visit the links in the episode notes for information about earning CME, subscribing to the journal, and exclusive access to interviews not featured on the podcast. Dr Monteith: This is Dr Teshamae Monteith. Today I'm interviewing Dr Claudia Chou about her article on end-of-life care and hospice, which is found in the December 2025 Continuum issue on neuropalliative care. Welcome to our podcast. How are you? Dr Chou: I'm doing well. Thank you for having me. This is really exciting to be here. Dr Monteith: Absolutely. So, why don't you introduce yourself to our audience? Dr Chou: Sure. My name is Claudia Chou. I am a full time hospice and palliative medicine physician at Mayo Clinic in Rochester. I'm trained in neurology, movement disorders, and hospice and palliative medicine. I'm also passionate about education, and I'm the program director for the Hospice and Palliative Medicine fellowship here. Dr Monteith: Cool. So just learning about your training, I kind of have an idea of how you got into this work, but why don't you tell me what inspired you to get into this area? Dr Chou: It was chance, actually. And really just good luck, being in the right place at the right time. I was in my residency and felt like I was missing something in my training. I was seeing these patients who were suffering strokes and had acute decline in functional status. We were seeing patients with new diagnosis of glioblastoma and knowing what that future looked like for them. And while I went into neurology because of a love of neuroscience, localizing the lesion, all of those things that we all love about neurology, I still felt like I didn't have the skill set to serve patients where they perhaps needed me the most in those difficult times where they were dealing with serious illness and functional decline. And so, the serendipitous thing was that I saw a grand rounds presentation by someone who works in neurology and palliative care for people with Parkinson's disease. And truly, it's not an exaggeration to say that by the end of that lecture, I said, I need to do palliative care, I need to rotate in this, I need to learn more. I think this is what I've been missing. And I had plans to practice both movement disorders neurology and palliative care, but I finished training in 2020… and that was not a long time ago. We can think of all the things that were going on, all the different global forces that were influencing our day-to-day decisions. And the way things worked out, staying in palliative care was really what my family and I needed. Dr Monteith: Wow, so that's really interesting. Must have been a great lecturer. Dr Chou: Yes, like one of the best. Dr Monteith: So why don't you tell me about the objectives of your article? Dr Chou: The objectives may be to fill in some of the gaps in knowledge that may be present for the general neurologist. We learn so much in neurology training, so much about how to diagnose and treat diseases, and I think I would argue that this really is part and parcel of all we should be doing. We are the experts in these diseases, and just because we're shifting to end-of-life or transitioning to a different type of care doesn't mean that we back out of someone's care entirely or transition over to a hospice or palliative care expert. It is part of our job to be there and guide patients and their care partners through this next phase. You know, I'm not saying we all need to be hospice and palliative care experts, but we need to be able to take those first steps with patients and their care partners. And so, I think objectives are really to focus in on, what are those core pieces of knowledge for end-of-life care and understanding hospice so we can take those first steps with patients and their care partners? Dr Monteith: So, why don't you give us some of those essential points in your article? Dr Chou: Yeah. In one section of the article, I talk about common symptoms that someone might experience at the end of life and how we might manage those. These days, a lot of hospitals have order sets that talk us through those symptoms. We can check things off of a drop-down menu. And yet I think there's a little bit more nuance to that. There may be situations in which we would choose one medication over another. There may be medications that we've never really thought of in terms of symptom management before. Something that I learned in my hospice and palliative medicine fellowship was that haloperidol can be helpful for nausea. I know that's usually not one of our go-tos in neurology for any number of reasons. So, I think that extra knowledge can take us pretty far when we're managing end of life symptoms, particularly in the hospital setting. And then I think the other component is the hospice component. A lot of us may have not had experience talking about hospice, talking about what hospice can provide, and again, knowing how to take those first steps with patients. We may be referring to social work or palliative medicine to start those conversations. But again, I think this is something that's definitely learnable and something that should be part of our skill set in neurology. Dr Monteith: Great. And so, when you speak about symptom management and being more comfortable with the tools that we have, how can we be more efficient and more effective at that? Dr Chou: Think about what the common symptoms are at end of life. We may know this kind of intuitively, but what we commonly see are things like pain, nausea, dyspnea, anxiety, delirium or agitation. And so, I think having a little bit of a checklist in mind can be helpful. You know, how can I systematically think through a differential, almost, for why my patient might be uncomfortable? Why they might be restless? Have I thought through these different symptoms? Can I try a medication from my tool kit? See if that works, and if it does, we can continue on. If not, what's the next thing that I can pivot to? So, I think these are common skills for a little bit of a differential diagnosis, if you will, and how to work through these problems just with the end-of-life lens on it. Dr Monteith: So, are there any, like, validated tools or checklists that are freely available? Dr Chou: I don't think there's been anything particularly validated for end-of-life care in neurologic disease. And so, a lot of our treatments and our approaches are empiric, but I don't think there's been anything validated, per se. Dr Monteith: Great. So, why don't we talk a little bit about the approach to discussions on hospice? We all, as you kind of alluded to, want to be effective neurologists, care for our patients, but we sometimes deal with very debilitating diseases. And so, when we think that or suspect that our patient is kind of terminally ill, how do we approach that to our patients? Of course, our patients come from different backgrounds, different experiences. So, what is your approach? Dr Chou: So, when we talk about hospice and when a patient may be appropriate for hospice, we have to acknowledge that we think that they may be in the last six months of their disease. We as the neurologist are the experts in their disease and the best ones to weigh in on that prognosis. The patient and their care partners then have to accept that the type of care that hospice provides is what makes sense for them. Hospice focuses on comfort and treating a patient's comfort as the primary goal. Hospice is not as interested in treating cancer, say, to prolong life. Hospice is not as interested in life-prolonging measures and treatments that are not focused at comfort and quality of life. And so, when we have that alignment between our understanding of a patient's disease and their prognosis and the patient care partner's goal is to focus on comfort and quality of life above all else, that's when we have a patient who might be appropriate for hospice and ready to hear more about what that actually entails. Dr Monteith: And what are some, maybe, myths that neurologist healthcare professionals may have about hospice that you really want us to kind of have some clarity on? Dr Chou: That's a great question. What we often tell patients is that hospice's goal is to help patients live as well as possible in the time that they have left. Again, our primary objective is not life prolongation, but quality of life. Hospice's goal is also not to speed up or slow down the natural

20 min
10 DEC

The Approach to Serious-Illness Conversations With Dr. Jessica Besbris

Neurologists are privileged to act as guides for patients as they navigate the complex course of serious neurologic illnesses. Because of the impact on quality of life, personhood, and prognosis, neurologists must be able to conduct serious-illness conversations to improve rapport, reduce patient anxiety and depression, and increase the likelihood that treatment choices agree with patient goals and values. In this episode, Teshamae Monteith, MD, FAAN speaks with Jessica M. Besbris, MD, author of the article "The Approach to Serious-Illness Conversations" in the Continuum® December 2025 Neuropalliative Care 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. Besbris is an assistant professor of neurology and internal medicine, and the director of the neuropalliative care, at Cedars-Sinai Medical Center in Los Angeles, California. Additional Resources Read the article: The Approach to Serious-Illness Conversations 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 Guest: @JessBesbris Full episode transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum. Thank you for listening to Continuum Audio. Be sure to visit the links in the episode notes for information about earning CME, subscribing to the journal, and exclusive access to interviews not featured on the podcast. Dr Monteith: Hi, this is Dr Teshamae Monteith. Today I'm interviewing Dr Jessica Besbris about her article on the approach to serious illness conversation, which is found in the December 2025 Continuum issue on neuropalliative care. How are you? Dr Besbris: I'm doing great. Thank you so much for having me here today. Dr Monteith: Well, thank you for being on our podcast. Dr Besbris: My pleasure. Dr Monteith: Why don't we start off with you introducing yourself? Dr Besbris: Sure. So, my name is Jessica Besbris. I am a neurologist with fellowship training in palliative care, and I am currently at Cedars Sinai Medical Center in Los Angeles, where I am the director of our neuropalliative care program. Dr Monteith: Excellent. So, how did you get involved in that? Dr Besbris: Like, I think, many neurologists, I always knew I wanted to be a neurologist---or, I should say, from the moment I decided to be a doctor I knew that that was the type of doctor I wanted to be, a neurologist. So, I went into medical school with the aim of becoming a neurologist. And very quickly, when I started my clinical years, I was exposed to patients who were living with very serious illnesses. And I found myself really drawn to opportunities to help, opportunities to make people feel better, opportunities to improve quality of life in situations that on the face of it seemed really challenging, where maybe it seemed like our usual treatments were not necessarily the answer or were not the only answer. And so, I pretty quickly recognized that taking care of patients with serious illness was going to be a big part of my life as a neurologist and that palliative care was the way I wanted to help these patients and families. Dr Monteith: And you mentioned you're leading the group. So, how many colleagues do you have in the program? Dr Besbris: We have a very large palliative care group, but within neuropalliative care, it's myself and one other physician, a nurse practitioner, and a social worker. Dr Monteith: Okay, well, I know you guys are busy. Dr Besbris: Yes, we are very happy to be busy. Dr Monteith: Yes. So, let's talk about the objectives of your article. Dr Besbris: Sure. So, the goal of this article is to impress upon neurologists that it really is all of our jobs as neurologists to be having these conversations with our patients who are affected with serious illness. And then, in most areas of neurology, these conversations will come up. Whether it's giving a life changing diagnosis, or talking about treatment choices, or treatment not going the way that we had hoped, or even sometimes progression of disease or end-of-life care. These topics will come up for most of us in neurology, and really, we're hoping that this article not only makes the case that neurologists can and should be having these conversations, but that there are skills that we can teach in this article and with other resources to improve the skill level and sense of confidence that neurologists have when they enter into these conversations. Dr Monteith: Great. I read that there are some developments in the field, on organizational levels, about really making these skills part of standard of care in terms of education. So, can you speak to that? Dr Besbris: Yes. So, there have been a couple of really landmark papers and changes in the educational landscape that I think have really brought neuropalliative care in general, and serious illness conversation in particular, to the forefront. So, there were the position statements released by the American Academy of Neurology in 1996 and 2022, both of which really said, hey, all neurologists should be doing this and receive training on how to have these conversations and provide this care. And the ACGME, the Accreditation Council for Graduate Medical Education, also requires neurology residency programs to learn how to communicate with patients and families, assess goals, and talk about end-of-life care. So, there's a real structural imperative now for neurologists to learn early on how to have serious illness conversations with their patients. Dr Monteith: Great. If there's anything for our listeners to get out of this conversation, what are the essential points? Dr Besbris: If you only take away one or two things from this conversation, I hope that they're that this is an awesome responsibility to be in a moment with a patient going through something challenging, to meet them in that moment with thoughtful, honest, empathic conversations about who they are and what's important to them. And that, just like any other procedure, these are skills that can be taught so that you can feel really confident and comfortable being in these moments. Dr Monteith: Excellent. Wow. Okay, I feel your energy and your empathy already. And so, why don't we just talk about skills? What is the best way to deliver tough news? I read this wonderful chart on SPIKES protocol. Dr Besbris: Yeah, the SPIKES protocol is one really well-known way to deliver serious news. And what's nice about SPIKES is it gives a mnemonic. And as neurology learners, we all love a good mnemonic to help you really center yourself when you're entering into these conversations so that you have a structured format to follow, just like with any procedure. So, the SPIKES protocol stands for Setting: so, making sure you have the right environment; Perception, or assessing what your patient or surrogate decision maker knows already so that you know where to begin; receiving an Invitation to deliver serious news. And then K stands for Knowledge, delivering in a clear and concise way the information that you want to make sure the family or patient walk away with. E for exploring Emotion; and S for really Summarizing what's been discussed and Strategizing on next steps. I think that having these kinds of conversations, it's just like being expert in anything. When you first start learning, it's helpful to have a set of very concrete steps you can follow. And you might even think through the mnemonic as you get ready to walk into that room. And as you become more expert, the flow becomes more natural. And maybe what you do before walking in to prepare is just honing what is that headline? What is that concise statement that I'm really going to give? And the rest may start to feel more natural and less protocolized. Dr Monteith: And there are a few other mnemonics. There's the NURSE mnemonic, which I like. You know, there's a balance between saying things and sounding kind of… you know, sometimes they're like, well, how could you understand what I'm going through? Have you been through something like this? And people shy away, and they're afraid to kind of be a part of these conversations. So how do we approach that with this, a NURSE mnemonic in a way that's kind of sincere? Dr Besbris: Absolutely. So, the NURSE mnemonic, unlike SPIKES, is not a step-by-step protocol. So, NURSE is a mnemonic, but you don't go through each letter and sort of give a naming statement and then an understanding statement and then a respecting statement and so on. Nurse is really a toolkit of different types of statements that we can give in response to emotions so that when you find yourself in a situation where a patient or family member is tearful, is scared, is angry, is expressing feelings, you have some phrases ready that feel authentic to you and that you feel are going to meet the moment and allow you to empathically respond to those emotions. Because until we do that, we really can't move further in this conversation with our patients and families feeling heard and respected. So, that NURSE mnemonic, those Naming, Understanding, Respecting, Supporting and Exploring statements, are really examples of statements that we can use to meet that moment with empathy and understanding and without implying that we have walked in their shoes. We want to avoid being presumptuous and really focus on just being present and empathic. Dr Monteith: So, let's just kind of run through, I think it's really important. Let's run through some of these examples. Maybe if someone's crying hysterically

22 min
3 DEC

December 2025 Neuropalliative Care Issue With Dr. Maisha T. Robinson

In this episode, Lyell K. Jones Jr, MD, FAAN, speaks with Maisha T. Robinson, MD, MSHPM, FAAN, FAAHPM, who served as the guest editor of the December 2025 Neuropalliative Care issue. They provide a preview of the issue, which publishes on December 2, 2025. 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. Robinson is the Chair of the Division of Palliative Medicine and an assistant professor of neurology at Mayo Clinic in Jacksonville, Florida. Additional Resources Read the issue: continuum.aan.com Subscribe to Continuum®: shop.lww.com/Continuum 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 Guest: @neuropalldoc Full episode transcript available here Dr Jones: Most of us who see patients with chronic progressive neurologic disease are aware of the value of palliative care. The focus on symptom management and quality of life is a key aspect of helping these patients. But how many of us are comfortable starting the conversation about palliative care or care at the end of life? Today we have the opportunity to speak with a leading expert on neuropalliative care, Dr Maisha Robinson, about how we can better integrate neuropalliative care into our practices. Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum. Thank you for listening to Continuum Audio. Be sure to visit the links in the episode notes for information about subscribing to the journal, listening to verbatim recordings of the articles, and exclusive access to interviews not featured on the podcast. Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum: Lifelong Learning in Neurology. Today I'm interviewing Dr Maisha Robinson, who is Continuum's Guest Editor for our latest issue of Continuum on neuropalliative care, and our first-ever issue fully dedicated to this topic. Dr Robinson is an assistant professor of neurology at Mayo Clinic in Florida, where she is Chair of the Division of Palliative Medicine, and she also serves on the AAN Board of Directors as Chair of the Member Engagement Committee. Dr Robinson, welcome. Thank you for joining us today. Why don't you introduce yourself to our listeners? Dr Robinson: Well, Dr Jones, thank you for having me. Really a pleasure to be here. I'm Maisha Robinson at the Mayo Clinic in Jacksonville, Florida. I spent my time as a neurohospitalist, a general palliative care physician, and a neuropalliative care physician. Dr Jones: So, this is a topic that at Continuum, we have heard about from subscribers for a long time requesting a fully dedicated issue to palliative care. And we've titled this neuropalliative Care. So, we want to respond to our subscribers and bring them content that they're interested in. I also think that palliative medicine is a big education gap in our specialty of neurology and something that we have room to improve on. So, let's start with the basics, Dr Robinson. Palliative medicine has been around for a long time, but this concept of "neuropalliative care" feels relatively new. What is neuropalliative care? Dr Robinson: That's a great question. Generally, what I would say is palliative care, first of all, is really just a specialty that focuses on trying to improve quality of life for people that have a serious or advanced medical condition. And neuropalliative care is really palliative care for people with neurologic conditions. And you'll see a number of neurologists doing neuropalliative care, but also there are internists as well, and people from other specialties, who focus on patients with neurologic disease and really trying to improve their quality of life. Dr Jones: Got it. And so, it's really the principles of palliative medicine in a specialty-specific context, which I think is important for us given the prevalence of chronic disease in our specialty. And I was obviously reading through these articles in this issue, and in the really wonderful articles, there are some themes that came up multiple times in various different articles. And one of them was obviously the importance of communication with patients and families. I think, and I'm speaking a little bit from personal experience here, many physicians feel uncomfortable bringing up the discussion of palliative care. And I'm sure that is something that reflects on your practice, too. How often do you have a patient who shows up to clinic and they ask you, why am I here? Dr Robinson: It happens all the time, because colleagues who are referring patients are nervous to tell them that they're sending them to palliative care. But we try to tell people it's really just to normalize it, to say that the palliative care team is going to see you, they're going to help with some symptoms, they're going to help you think about big picture, and they're going to be sort of an added layer of support to your team. And I think if people approach it from that standpoint, then patients and family members will say, that sounds great, I need a little extra support. Dr Jones: So, I think most neurologists have a threshold at which they would feel more comfortable having specialty support, having a palliative medicine specialist to help them in symptom management with the patient. For the palliative care that they provide themselves---and we want our subscribers to read this issue and feel more comfortable with delivering some palliative care on their own---how would you encourage them to begin that conversation? How should they initiate that conversation with a patient about working more toward palliative management of symptoms? Dr Robinson: So, one of the things we recommend is really introducing an approach to palliative care very early in the disease process. So, discussions about big picture and goals of care, discussions about who might help make medical decisions if the person can't make them for themselves. Those kinds of things can be discussed very early on. And in fact, that's palliative care. And then they can talk to patients more about the fact that as the disease progresses, there may be an additional team that can help walk along alongside the neurologist in helping you prepare for what's to come. You know, I think it's very important for patients and family members who feel like you're not abandoning them, but you're adding additional resources. And so, I like the way that we often will suggest to people to say partner or collaborate or bring in extra resources with the palliative care team. I think patients and family members will respond to that. Dr Jones: Yeah. So, by talking about it early, you kind of, at least, help to avoid that problem of the patient perceiving the introduction of palliative care as the quote-unquote "giving-up problem." Is that right? Dr Robinson: Correct. Because we also don't want to see people who are just being referred to us for end-of-life care. Palliative care is about much more than that. But if patients will Google palliative care, they may see hospice come up. And so, introducing the concept early and discussing some palliative topics early will allow the patient and family members to think that, okay, this isn't because I'm at the end of life. This is just because my clinician wants to make sure that I have all the bases covered. Dr Jones: This was also mentioned in several of the articles, the studies that have shown how frequently palliative care is initiated very near the end of life, which is usually, I think, perceived as a missed opportunity, right? To not wait so long to take advantage of what palliative care has to offer. Dr Robinson: That's correct. And the benefit of palliative care is that oftentimes we work alongside an interdisciplinary team, a team that could be quite helpful to patients and their support systems throughout the course of the disease. So, we have chaplains, we have nurses, we often have other clinicians, advanced practice providers as well, who work with us. We have spiritual advisors as well. And the patients and family members could benefit from some of those resources throughout the course of the disease. Who they might need to meet with may vary depending on what the disease is and how they're doing. But there's definitely some benefit to having a longitudinal relationship with the palliative care team and not just seeking them out at the end of life. Dr Jones: So- that's very helpful. So, it'll obviously vary according to an individual provider's level of comfort, right, where they're comfortable providing certain palliative management care versus when they need to have some assistance from a specialist. Are there types of care or are there certain thresholds that you say, wow, this patient really should go see a specialist in palliative medicine or neuropalliative care? Dr Robinson: So, I think that if there are, for instance, refractory symptoms, where the neurologist has been working with a patient for a while trying to manage certain symptoms and they're having some challenges, that person may benefit from being referred to palliative care. If patients are being hospitalized multiple times and frequently, that may suggest that a good serious-illness conversation may be necessary. If there are concerns about long-term artificial nutrition, hydration, or functional and cognitive decline, then some of those patients have benefited from palliative care. Not only the patient, but also the caregiver, because our team really focuses on trying to make sure that we're walking through the course of disease with these patients to ensure that all of the needs are managed both for the patient and the family member. Dr Jones: Got it. And that's very helpful. And I know that we

22 min
26 NOV

Dystrophinopathies With Dr. Divya Jayaraman

Dystrophinopathies are heritable muscle disorders caused by pathogenic variants in the DMD gene, leading to progressive muscle breakdown, proximal weakness, cardiomyopathy, and respiratory failure. Diagnosis and management are evolving areas of neuromuscular neurology. In this episode, Kait Nevel, MD, speaks with Divya Jayaraman, MD, PhD, an author of the article "Dystrophinopathies" in the Continuum® October 2025 Muscle and Neuromuscular Junction 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. Jayaraman is an assistant professor of neurology and pediatrics in the division of child neurology at the Columbia University Irving Medical Center in New York, New York. Additional Resources Read the article: Dystrophinopathies 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 Full episode transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum. Thank you for listening to Continuum Audio. Be sure to visit the links in the episode notes for information about earning CME subscribing to the journal, and exclusive access to interviews not featured on the podcast. Dr Nevel: Hello, this is Dr Kate Nevel. Today I'm interviewing Dr Divya Jayaraman about her article on dystrophinopathies, which she wrote with Dr Partha Ghosh. This article appears in the October 2025 Continuum issue on muscle and neuromuscular junction disorders. Divya, welcome to the podcast, and please introduce yourself to the audience. Dr Jayaraman: Thank you so much, Dr Nevel. My name is Divya, and I am an assistant professor of Neurology and Pediatrics at Columbia University Irving Medical Center, and also an attending physician in the Pediatric Neuromuscular program there. In that capacity, I see patients with pediatric neuromuscular disorders and also some general pediatric neurology patients and also do research, primarily clinical research and clinical trials on pediatric neuromuscular disorders. Dr Nevel: Wonderful. Thank you for sharing that background with us. To set us on the same page for our discussion, before we get into some more details of the article, perhaps, could you start with some definitions? What comprises the dystrophinopathies? What are some of the core features? Dr Jayaraman: So, the dystrophinopathies, I like that term because it is a smaller subset from the muscular dystrophies. The dystrophinopathies are a spectrum of clinical phenotypes that are all associated with mutations in the DMD gene on chromosome X. So, that includes DMD---or, Duchenne muscular dystrophy---, Becker muscular dystrophy, intermediate muscular dystrophy (which falls in between the two), dilated cardiomyopathy, asymptomatic hyperCKemia, and manifesting female carriers. In terms of the core features of these conditions, so, there's some variability, weakness being prominent in Duchenne and also Becker. The asymptomatic hyperCKemia, on the other hand, may have minimal symptoms and might be found incidentally by just having a high CK on their labs. They all will have some degree of elevated CK. The dilated cardiomyopathy patients, and also the Becker patients to a lesser degree, will have cardiac involvement out of proportion to skeletal muscle involvement, and then the manifesting carriers likewise can have elevated CK and prominent cardiac involvement as well as some milder weakness. Dr Nevel: Now that we have some definitions, for the practicing neurologists out there, what do you think is the most important takeaway from your article about the dystrophinopathies? Dr Jayaraman: I like this question because it suggests that there's something that, really, any neurologist could do to help us pick up these patients sooner. And the big takeaway I want everyone to get from this is to check the CK, or creatine kinase, level. It's a simple, cheap, easy test that anyone can order, and it really helps us a lot in terms of setting the patient on the diagnostic odyssey. And in terms of whom you should be thinking about checking a CK in, obviously patients who present with some of the classic clinical features of Duchenne muscular dystrophy. This would include young boys who have toe walking, as they're presenting, sign; or motor delayed, delayed walking. They may have calf hypertrophy, which is what we say nowadays. You might have seen calf pseudohypertrophy in your neurology textbooks, but we just say calf hypertrophy now. Or patients can often have a Gowers sign or Gowers maneuver, which is named after a person called Gowers who described this phenomenon where the child will basically turn over and use their hands on the floor to stand up, usually with a wide-based gait, and then they'll sort of march their hands up their legs. That's the sort of classic Gowers maneuver. There are modified versions of that as well. So, if anyone presents with this classic presentation, for sure the best first step is to check a CK. But I would also think about checking a CK for some atypical cases. For example, any boy with any kind of motor or speech delay for whom you might not necessarily be thinking about a muscle disorder, it's always good practice to check a CK. Even a boy with autism for whom you may not get a good clinical exam. This patient might present to a general pediatric neurology clinic. I always check a CK in those patients, and you'll pick up a lot of cases that way. For the adult folks in particular, the adult neurologist, a female patient could show up in your clinic with asymptomatic hyperCKemia. And I think it's an important differential to think about for them because this could have implications not just for their own cardiac risks, but also for their family planning. Dr Nevel: So, tell us a little bit more about the timing of diagnosis. Biggest takeaway: check a CK if this is anywhere on your radar, even if somewhat of an atypical case. Why is it so important to get kiddos started on that diagnostic odyssey, as you called it, early? Dr Jayaraman: This is especially important for kids because if they especially get a Duchenne muscular dystrophy diagnosis, you might be making them eligible for treatments that we've had for some time, and also treatments that were not available earlier that hinge on making that diagnosis. So, for example, people may be skeptical about steroids, but there's population data to suggest that initiation and implementation of steroids could delay the onset of loss of ambulation as much as three years. So, you don't want to deprive patients of the chance to get that. And then all the newer emerging therapies---which we'll be talking about later, I'm sure---require a Duchenne muscular dystrophy diagnosis. So, that's why it's so important to check a CK, have this on your radar, and then get them to a good specialist. Dr Nevel: I know that you alluded already, or shared a few of the kind of exam paroles or findings among patients with dystrophinopathy. But could you share with us a little bit more how you approach these patients in the clinic who are presenting with muscle weakness, perhaps? And how do you approach this or think about this in terms of ways to potentially differentiate between a dystrophinopathy versus another cause of motor weakness or delay? Dr Jayaraman: It's helpful to think through the neuraxis and what kinds of disorders can present along that neuraxis. A major differential that I'm always thinking about when I'm seeing a child with proximal weakness is spinal muscular atrophy, which is a genetic anterior horn cell disorder that can also present in this age group. And some of the key differences there would be things like reflexes. So, you should have dropped reflexes in spinal muscular atrophy. In DMD, surprisingly, they might have preserved Achilles reflexes even if their patellar reflexes are lost. It may only be much later that they go on to lose their Achilles reflex. So, if you can get an Achilles reflex, that's quite reassuring, and if you cannot, then you need to be thinking about spinal muscular atrophy. They can both have low muscle tone and can present quite similarly, including with proximal weakness, and can even have neck flexion weakness. So, this is an important distinction to make. The reason for that is, obviously there are treatments for both conditions, but for spinal muscular atrophy, timing is very, very important. Time is motor neurons, so the sooner you make that diagnosis the better. Other considerations would be the congenital muscular dystrophies. So, for those that they tend to present a lot younger, like in infancy or very early on, and they can have much, much higher CKS in that age range than a comparable Duchenne or Becker muscular dystrophy patient. They can also have other involvement of the central nervous system that you wouldn't see in the dystrophinopathies, for example. My mnemonic for the congenital muscular dystrophies is muscle-eye-brain disease, which is one of the subtypes. So, you think about muscle involvement, eye involvement, and brain involvement. So, they need an ophthalmology valve. They can have brain malformations, which you typically don't see in the dystrophinopathies. I think those are some of the major considerations that I have. Obviously, it's always good to think about the rest of the neuraxis as well. Like, could this be a central nervous system process? Do they have upper motor neuron signs? But that's just using all of your exam tools as a neurologist. Dr Nevel: Yeah, absolutely. So, let's say you have a patient in clinic a

25 min
19 NOV

Myotonic Dystrophy With Dr. Paloma Gonzalez Perez

Myotonic dystrophies (DM), in addition to muscle weakness and myotonia, are associated with broad and variable multiorgan involvement. Neurologists need to recognize DM to ensure prompt diagnosis, effective symptom management, and prevention of life-threatening events. In this episode, Casey Albin, MD, speaks with Paloma Gonzalez Perez, MD, PhD, author of the article "Myotonic Dystrophy" in the Continuum® October 2025 Muscle and Neuromuscular Junction Disorders issue. Dr. Albin is a Continuum® Audio interviewer, associate editor of media engagement, and an assistant professor of neurology and neurosurgery at Emory University School of Medicine in Atlanta, Georgia. Dr. Gonzalez Perez is an assistant professor at Harvard Medical School in Boston, Massachusetts. Additional Resources Read the article: Myotonic Dystrophy 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: @caseyalbin Full episode transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum. Thank you for listening to Continuum Audio. Be sure to visit the links in the episode notes for information about earning CME, subscribing to the journal, and exclusive access to interviews not featured on the podcast. Dr Albin: Hello everyone, this is Dr Casey Albin. Today I'm interviewing Dr Paloma Gonzalez-Perez about her article on myotonic dystrophy, which appears in the October 2025 Continuum issue on muscle and neuromuscular junction disorders. Welcome to the podcast, Dr Gonzalez-Perez. I'd love for you to introduce yourself to our listeners. Dr Gonzalez-Perez: Thank you very much for the invitation. My name is Paloma Gonzalez-Perez. I'm a neuromuscular neurologist at Massachusetts General Hospital in Boston since 2018. And I'm originally from Spain. I did residency there and also here in Iowa City. And then I did the neuromuscular fellowship here at Mass General Brigham, and then I stayed here as a faculty. So, my focus is myopathies, and more specifically muscular dystrophies, and more particularly myotonic dystrophy, which is what we are going to talk today. Dr Albin: Wonderful. And this is a really fantastic tour de force article about myotonic dystrophy. And in reading your article, it really did stand out to me that these myotonic dystrophies are probably under-recognized. And so, I was hoping that, just to start, you could tell us a little bit about, what is a myotonic dystrophy, and how should we sort of situate that within the larger context of all muscular dystrophies? Dr Gonzalez-Perez: Yes, so muscular dystrophies, we have many of them, right? And mostly affecting the skeletal muscle. And basically, the definition of muscular dystrophy is a genetic or inherited muscle disease that causes a progressive muscle weakness. And also, in the muscle biopsies of patients with muscular dystrophies, we see some fractures that are characteristic of this category of muscle diseases, such as, for example, the nuclei of the muscle fibers are in the center---that's what we call internal nuclei---or maybe fat infiltration or increased connective tissue or a variability in the size of the muscle fibers. So, now in the last few years, the genetic testing is more accessible to us. So, we don't need muscle biopsies all the time to diagnose patients with muscular dystrophy. So many times, we go directly to genetic testing. And this is basically the category of muscular dystrophies. Myotonic dystrophy is very fascinating muscular dystrophy in the sense that many times not only affect the skeletal muscle, but other organs can be affected. And it is true that other muscular dystrophies can affect other organs such as, for example, the brain and the heart, which is something that we always have in mind as a clinician to make sure this muscular dystrophy affect the heart or affect the brain, because it is important for patient care. But myotonic dystrophy actually can affect any organ in the body. I think it is one of these muscular dystrophies in which there is a multisystem involvement of the body. So, the immune, immunological system can be affected and the endocrine system can be affected, the GI system can be affected. In addition to, obviously, to the brain, to the heart, to the skeletal muscle. And sometimes that is why it is under-recognized because of course, if there is a very severe phenotype, maybe the patient comes very easily to a neurologist who is very familiar with myotonic dystrophy. But if the phenotype is a little bit milder, and maybe it doesn't affect much the skeletal muscle. So, these patients probably are in the care of other specialists, such as, for example cardiology or GI doctors, and obviously these specialists are not really aware of this muscular dystrophy. So, I think it is a complex disease because it is very variable in phenotype, can affect many organs and can be also mild. Dr Albin: That is fantastic. That is just a wonderful overview of, really, muscular dystrophy. One of the things I was really curious about: the name includes myotonia. Is myotonia, like, always present, or is that a little bit misleading? Dr Gonzalez-Perez: Yeah. I would say that it is a little bit misleading---maybe not too much in myotonic dystrophy type one, because it is true that in adults with myotonic dystrophy type one, many times they have the myotonia, but not many times they complain about the myotonia. This is the thing. So, it is a diagnostic clue that we have at bedside when we ask the patient, for example, to squeeze the hands and then release and we see the myotonia there. And then, obviously, this can actually give you the diagnosis at bedside, but the patients usually don't come to the clinic complaining of this myotonia, which is delaying the relaxation of the muscles. Sometimes they don't- they are not bothered by that. They don't need treatment for that. But it is a very important clue at bedside. I have to say, adults, myotonic dystrophy type one, because the congenital myotonic dystrophy type one you don't see myotonia, clinical myotonia. These babies, right, are born with severe muscle weakness and we don't see myotonia. And then myotonic dystrophy type two, many patients don't have clinical myotonia. And then, you know, the absence of myotonia, the absence of this delay in the muscle relaxation doesn't rule out a myotonic dystrophy, and especially doesn't rule out a myotonic dystrophy type two. Dr Albin: Fantastic. So probably is going to be a feature of the adult-onset type one. May or may not be present in type two. And then the congenital forum where children are presenting as infants, they're not going to tell you that, oh, I have delayed relaxation. That's not going to be part of that. Dr Gonzalez-Perez: Exactly. Dr Albin: This is one of those things that I think, unless you're in neuromuscular clinic, you might not think to ask people about. Maybe the patient isn't actually saying, oh, I have this delayed reaction. How do you get them to give you that history? Like, what are the questions that you ask? Dr Gonzalez-Perez: Sometimes I will say, do your hands get locked? You know, this could be the first question that they noticed something there, and then they can give you maybe the clue. But actually, it's the exam more than the question. I will say it's more do the exam and, you know, intentionally test for myotonia. And you test for spontaneous myotonia and percussion myotonia. So spontaneous myotonia, we tell the patient to squeeze the hands very strongly and then open the hands quickly. And then if they cannot open the hands quickly, this is a delay in muscle relaxation. We call it grip myotonia, spontaneous grip myotonia. Or sometimes close your eyes very, very, very strongly and then open the eyes quickly. And if they have this delay in the eye opening, we call it eyelid myotonia. This eye is spontaneous myotonia, you don't touch the patient and you don't use your hammer yet. And then if we don't find anything, we go to the hammer. We use our reflex hammer, and then we try to test for percussion myotonia. And sometimes we with the reflect hammer, we tap the thinner eminence of the hand, and we can see that you tap, there is a contraction, and then the thumb goes up and then takes a while to go down again. It is a delay in the relaxation of the thinner eminence muscles. Or sometimes in the posterior aspect of the forearm, if we tap the extensor digitorum communis muscle. Again, so, there is a contraction of that muscle, the fingers go up and then take a while to go down. It is also a perfusion myotonia of the extensor digitorum communis muscle. Sometimes people do it even in the tongue. I don't do that because could be very painful. But you can, you know, use a tongue depressor and put it in the tongue, and you tap the tongue depressor and sometimes there is contraction of the tongue, which can be very painful. I don't do it. So- but this is the perfusion myotonia, that can give you also a clue. Dr Albin: That's fantastic. I think this is one of the most memorable things that I saw in pediatric neurology. I remember very distinctly a kid coming in, and then us also examining the mother and having that delayed relaxation. And just one of those really great neurologic exams, those little findings to tuck away to really make a diagnosis, recognizing that not all patients with muscular dystrophy or myotonic dystrophy will have that finding. But so beautiful. And I think that's a really great explanation. And I will also direct our listeners, if you are a Continuum subscriber, she has some really wonderful videos in her article from the EMG sound

23 min
12 NOV

Muscle Channelopathies and Rhabdomyolysis with Dr. Hani Kushlaf

Genetic variants that underlie skeletal muscle channelopathies and rhabdomyolysis can also cause persistent and progressive muscle weakness. The availability and expanded use of genetic testing allows for the identification of new genes causing periodic paralysis and rhabdomyolysis. In this episode, Teshamae Monteith, MD, FAAN speaks with Hani Kushlaf, MD, MS, FAAN, author of the article "Muscle Channelopathies and Rhabdomyolysis" in the Continuum® October 2025 Muscle and Neuromuscular Junction 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. Kushlaf is a professor of neurology and pathology as well as the director of the Neuromuscular Division, director of Neuromuscular Research, and director of the Neuromuscular Medicine Fellowship in the Department of Neurology and Rehabilitation Medicine and the Department of Pathology and Laboratory Medicine at the University of Cincinnati College of Medicine in Cincinnati, Ohio. Additional Resources Read the article: Muscle Channelopathies and Rhabdomyolysis 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 Guest: @HaniKushlaf Full episode transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum. Thank you for listening to Continuum Audio. Be sure to visit the links in the episode notes for information about earning CME, subscribing to the journal, and exclusive access to interviews not featured on the podcast. Dr Monteith: This is Dr Teshamae Monteith. Today I'm interviewing Dr Hani Kushlaf about his article on muscle channelopathies and rhabdomyolysis, which appears in the October 2025 Continuum issue on muscle and neuromuscular junction disorders. Hi, Hani. How are you? Dr Kushlaf: Good. How are you doing? Thank you for having me. Dr Monteith: Well, thank you for coming on our podcast. So why don't you introduce yourself? Dr Kushlaf: So, I'm Hani Kushlaf. I'm a professor of Neurology and Pathology in the Department of Neurology and Rehabilitation Medicine at the University of Cincinnati in Cincinnati, Ohio. Dr Monteith: And what got you interested in muscle disorders? Dr Kushlaf: So, this is a long story. At first in residency, I was interested in epilepsy, but the moment I started doing a rotation in neuromuscular, I became enamored with neuromuscular disorders. I remember seeing the first patient in the rotation who had myofibrillar myopathy, and I found out that there is very little known about muscle disease at the time. So, I became very interested and I immediately changed direction as I started doing nerve conduction studies and EMGs and having that procedure base, I just decided that I want to pursue neuromuscular disorders. Dr Monteith: Well, that doesn't sound like a long story. It sounds like love at first sight. Dr Kushlaf: Yes, I think part of it is not very far from neuromuscular disorders and that it does have some electrophysiology involved. But muscle disease and nerve disorders, they need that detailed neurologic examination, which I love in terms of doing the localization exercise. Dr Monteith: Great. So why don't we talk about the objectives of your article? Dr Kushlaf: This article was written to review muscle channelopathies that include myotonic disorders and periodic paralysis, and also rhabdomyolysis, with emphasis on genetic causes of rhabdomyolysis more than acquired causes. Dr Monteith: So, why was this update so important? Dr Kushlaf: I think this area of muscle disorders hasn't seen a lot of progress in recent years, but there are interesting findings that we're learning that spark, hopefully, more research into the area, because we do have significant gaps that are related to understanding pathophysiology of some of these disorders. For example, in patients with periodic paralysis, it's clearly known now that these patients over time develop muscle weakness, and the muscle weakness is unrelated to how many episodes of weakness they have---basically, the episodic paralysis part of the disease. So, this is an important finding that I think we need to look more into it and understand that these disorders actually progress, even though that they may not have episodes of paralysis. In addition, there are genetic therapies that have been introduced into most of neuromuscular medicine at this time, including muscle disorders, while this specific part of muscle diseases has not had that luxury yet. And I'm hoping over time that there will be an introduction of gene therapies for these diseases. Dr Monteith: Great. So, it sounds like there's some clinical advances, then, as well as genetic advances. Now, you also spoke about rhabdomyolysis and that there's newer ways of thinking about that from perhaps when I was in residency. So why don't you update me on these newer approaches? Dr Kushlaf: The rhabdomyolysis… first, the definition of it is changing. We used to use a cut off, a CK of about a thousand, to call it rhabdomyolysis. And very recently it's clear that that level of CK is not sufficient to call it rhabdomyolysis. So, now the level went up in terms of exertion of rhabdomyolysis, up to ten thousand, has to be more than ten thousand. And in patients who haven't had exercise, the level is up to five thousand. So, it's no longer actually one thousand. And that refinement in the definition is important because there are some patients who exercise all the time and they may exercise at an athletic level and they have very high CK's. And those patients should not be labeled as having rhabdomyolysis. Basically, they are doing strenuous physiologic exercise. In addition, not only the definition of rhabdomyolysis is changing, it is our approach to which disorders to consider first and how we should work up patients with rhabdomyolysis. So, acquired rhabdomyolysis remains the most important etiology to be ruled out first. So, I always tell my fellows and residents that, think about acquired rhabdomyolysis first before you think about the genetic disorder. After you rule out the fact that it is not a toxic or metabolic or medication-induced rhabdomyolysis, then think about a genetic etiology. But when you get that consultation from the hospital that the patient has rhabdomyolysis and we want you to figure it out, always look at the medication list and make sure that there isn't anything on it that causes, actually, a rhabdomyolysis. And in many instances, you find out that it's actually a toxic or metabolic etiology for the rhabdomyolysis. And as part of this article, there's also an acronym that's now being used to identify those patients who would benefit from genetic testing. The acronym is called RHABDO. It's as is the word, RHABDO. R refers to Recurrent exertion of rhabdomyolysis. So, it's not just one episode. And the H refers to HyperCKemia, and the hyperCKemia should persist more than eight weeks after the episodes of rhabdomyolysis. And if it is exertional, then the person has not done an unaccustomed exercise. So, they have changed the way that they do exercise and now they are exercising for two hours instead of one hour or they have introduced a new way of exercise into their exercise regimen. Then that should not be considered. It's not considered necessary to test these patients for a genetic cause. Also, the muscle enzyme typically, in genetic causes of rhabdomyolysis, goes more than fifty times above the upper limit of normal, which is more than a thousand. So that has to be taken into account. And then for the D it's Drugs and medications. This has to be ruled out before you say yes, we need to find the genetic cause of rhabdomyolysis. And then the O, it's basically family history. If you find that there is other family members who are affected or they have a high CK, then of course that would point you toward doing genetic testing. Dr Monteith: Great. So, it sounds like there's some advances there in how we approach these patients. In what way is this practice changing? You mentioned, you know, really important to rule out these potentially reversible causes first. Dr Kushlaf: Yeah. So, once you identify the theology, it becomes easier to manage the patient. So, if it is a statin-induced rhabdomyolysis, you know that you want to stop the statin and you are not going to have this problem again. So, that's quite important. The statins, of course, will have to not be reintroduced in the future for that specific patient who developed statin-induced rhabdomyolysis. But for the genetic causes of rhabdomyolysis, if you go down the path of genetic rhabdomyolysis, of course we have no cures for these disorders. We may have treatments. One of the conditions that I have alluded to as one of the case presentations in the article is a patient who has riboflavin responsive multiple acid CoA dehydrogenase deficiency, and I wanted to highlight this disorder because it's a disorder not to be missed. It does have a treatment, which is riboflavin, and that comes from the name riboflavin-responsive. So that's why I put there in the article as part of the manuscript. However, some of these disorders, once you find the genetic etiology, there may be a way of preventing it in the future generation. Family planning, reproductive medicine technologies, can help in this instance and prevent this disease from occurring in the future generations. Dr Monteith: So, why don't we move on to episodic skeletal disorders? What is your general approach for these types of diseases? Dr Kushlaf: Muscle chan

24 min
5 NOV

Idiopathic Inflammatory Myopathies With Dr. Anthony Amato

Inflammatory myopathies are a large group of disorders associated with an inflammatory response targeting skeletal muscle. Treatment hinges on the use of evolving immunotherapies and diagnostic tools to quickly identify inflammatory myopathy, initiate appropriate therapy, and exclude underlying malignancy or infection of other organs. In this episode, Katie Grouse, MD, FAAN speaks with Anthony A. Amato, MD, an author of the article "Idiopathic Inflammatory Myopathies" in the Continuum® October 2025 Muscle and Neuromuscular Junction 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. Amato is the Brigham and Women's Hospital Distinguished Chair in Neurology and the director of neuromuscular research at Mass General Brigham, and is a professor of neurology at Harvard Medical School in Boston, Massachusetts. Additional Resources Read the article: Idiopathic Inflammatory Myopathies 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 Full episode transcript available here Dr Jones: This is Dr Lyell Jones, Editor-in-Chief of Continuum. Thank you for listening to Continuum Audio. Be sure to visit the links in the episode notes for information about earning CME, subscribing to the journal, and exclusive access to interviews not featured on the podcast. Dr Grouse: This is Dr Katie Grouse. Today I'm interviewing Dr Anthony Amato about his article on idiopathic inflammatory myopathies, which he wrote with Dr Kian Salajegheh. This article appears in the October 2025 Continuum issue on muscle and neuromuscular junction disorders. Welcome to the podcast, and please introduce yourself to our audience. Dr Amato: Thank you. And I am Tony Amato. I'm in Boston at Mass General Brigham. Dr Grouse: It is a distinct pleasure to have you here with us today, and I'm really excited to talk with you about your article. I thought it was a fantastic overview of the subject. And I'd like to start by asking what you hope will be the key takeaway for those who are reading this article. Dr Amato: I think it's kind of basic: how to make a diagnosis, describe about the inflammatory myopathy as approach to, again, diagnosis, and then a little bit on pathogenesis, which… and kind of leading to the treatments, and hopefully we'll have more treatments based on the distinct pathogenesis in the future. Dr Grouse: Can you give a brief overview of the categories of inflammatory myopathies you reviewed in your article? Dr Amato: So, I mean, the major inflammatory myopathies, radiopathic inflammatory myopathies, are dermatomyositis, antisynthetase syndrome, immune-mediated necrotizing myopathy, inclusion body myositis, and polymyositis. Now, that's been a big change, as you know. I mean it used to be, you know, we all started off it was dermato or poly. But I've kind of made a name for myself- a bad name for myself in the early 2000s saying, I'm not sure there's much of a thing called polymyositis. I think it's a hodgepodge and it's not distinct. And that's come to be, now most of those cases are- now we find out having antisynthetase syndrome or necrotizing myositis or IBM. Dr Grouse: Could you walk us through your diagnostic approach with a patient in your clinic presenting with symptoms that are suspicious for inflammatory myopathy? Dr Amato: So, you want to really make sure that they have inflammatory myopathy as opposed to some other kind of myopathy, a muscular dystrophy, for example. Taking family history first is going to be important, clueing in are they really weak or what they're complaining of is fatigue or muscle pain? Are they feel weak but what they really are complaining of is stiffness and rigidity from parkinsonism, or they have a sensory ataxia so they can't modulate? I want to know about other organ system involvement. Do they have a rash? Do they have joint swelling and pain that you might see with arthritis? Do they have shortness of breath that you might see with interstitial lung disease or ventilatory muscle weakness? Or do they have a cardiomyopathy? What kind of weakness do they have? Is it proximal weakness in the arms or legs? Getting out of a chair, climbing stairs. Do they have problems lifting their arms over their head---so, proximal weakness---or do they have more problems with grip, finger flexion, holding a pen, tripping? Do they have swallowing problems? Do they have ocular problems? So that's the big history on the exam. Again, I'm looking for pattern of involvement. So, on my exam, is there atrophy or weakness in muscles---you know, fasciculations---which would take it out of the motor? Is it mainly proximal? Is it distal? Again, is there ocular bulbar involvement? Is it symmetric, particularly in, like, the IBM? Most of the other inflammatory myopathies are going to be mainly and proximal and mainly symmetric. IBM is different, and that the- at least in the hands it's more distal, and it's finger flexors. So, you're looking at flexing the tips of the fingers, you're looking at the forearms, best looked at in a semipronated position to see if it's atrophied. And that leads you to an IBM if you see that. So that's the main things on exam. Dr Grouse: That's a really helpful overview. I was wondering, in earlier training days, the convention was you- once you've suspected myopathy, you get your CK, you get your EMG, then that may give you the information you need for your diagnosis. It seems that things have been turned a little on their head. We're often skipping those things to go straight to the antibody testing. When should we be going for the myositis-specific antibodies before considering other things like EMG or muscle biopsy? Dr Amato: I would always get a CK first. You know, in somebody who's weak. You know, the EMG, I don't need an EMG if the CK is two or three thousand. I mean, EMG is- localize it to, is it muscle, nerve, neuromuscular junction. If it's very elevated CK, it really doesn't help me there. Sometimes if I have myotonic discharges or something that might make me think of a myotonic dystrophy or something else like that. But you can see that with the inflammatory myopathy. So, if I'm pretty sure of a myositis, I don't always do an EMG, or- unless I really need it to help guide what muscle biopsies I do. if I'm suspicious then on my exam and I see the CK---or they come to me already with the CK, which often happens, and it's very elevated---that's when I'll do the myositis-specific antibody panels if I'm really thinking that. And the important thing to know from that is, you have antibodies for dermatomyositis and antisynthetase that are on the panel that are available, and even signal recognition particle, which is a necrotizing myositis. But what's not on the panel is HMGCR antibodies, which is important because that's 70% of the necrotizing myocidites are HMGCR, and then the IBM antibody and T5-C1A is not on that. So, you need to order those separately. If somebody doesn't know, they order a myositis-specific antibody and think that it's all-inclusive, but it doesn't have IBM or the HMGCR antibody. And the other test that I sometimes will do is a skeletal-muscle MRI to help in the evaluation. Sometimes, not all the time, but I'm not sure it's a dystrophy, is it a myositis when I see a lot of STIR signal, which is edema. And you can still see STIR signal in a dystrophy and toxic. But sometimes I'll do it depending on whether I need a biopsy or not. Dr Grouse: What is the benefit of an open biopsy versus a needle biopsy, and when should we be considering using one over the other? Dr Amato: So, it really is not our decision. It's the pass lab. So, it's the technicians and the pathologists who read the biopsies need to be able to process a needle biopsy, which might be much smaller. Needle biopsies show to be fairly accurate in a lot of the hereditary disorders where you might just look for central nuclear core, and they might be- so, mainly in kids, but in the inflammatory myopathies, it's really patchy. So, if I'm thinking of an inflammatory, I like an open biopsy. I think it's hit and miss. And so, I like open biopsies for the adults that I'm thinking of inflammatory. Dr Grouse: Do you have any other tips or tricks in the diagnosis of inflammatory myopathies that you could share with our listeners? Dr Amato: I would say first, in terms of what muscle to biopsy, you're not doing them yourself, but you're referring to a surgeon. You have to tell them what to do with the biopsy. And you want to pick a muscle that's about an MRC grade 4 because if it's a normal muscle, the muscle strength, the biopsy, is likely to be normal. If it's less than a 4, you might just have end-stage muscle. And saying you can't tell end-stage muscle from a bad myositis, from a dystrophy, from a severe end-stage neurogenic. If I don't have a muscle that I would typically biopsy that's an MRC grade 4---for example, somebody with an early weakness and they're only weak, say, in their in their hip girdle. So hip flexors, abductors, extensors, and we're usually not biopsying the iliopsoas or the gluteal muscles. Then what do you pick? That's when I like to do an EMG on one side of the body and look at proximal and distal muscles and select one that's irritable, you know, some fibs and positive sharp waves that I might biopsy. And then maybe consider doing a skeletal muscle MRI to go from muscle that's abnormal, that has a lot of edema in it, to increase the yield. Dr Grouse: That's really helpful. And then, I think, jumping from that, what are

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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

31/01/2024

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4 min

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.

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