Management of Neuromuscular Weakness in Patients Requiring Ventilatory Support

In this episode, Gordon Smith, MD, FAAN speaks with Casey S.W. Albin, MD, author of the article “Neuromuscular Emergencies,” in the Continuum® June 2024 Neurocritical Care 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. Albin is an assistant professor of neurology and neurosurgery in the departments of neurology and neurosurgery, division of neurocritical care at Emory University School of Medicine in Atlanta, Georgia.

Additional Resources

Read the article: Neuromuscular Emergencies

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Host: @gordonsmithMD

Guest: @caseyalbin

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 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: Hi. This is Dr Gordon Smith. I'm super excited today to be able to have the opportunity to talk to Dr Casey Albin, who will introduce herself in a second. She's well known to Continuum Nation as the Associate Editor for Media Engagement for Continuum. She's also a Neurointensivist at Emory University and wrote a really outstanding article for the neurocritical care issue of Continuum on neuromuscular emergencies. Casey, thanks for joining us. Tell us about yourself.

Dr Albin: Sure. Thank you so much, Dr Smith. So, yes, I'm Casey Albin. I am a Neurointensivist. I practice at Emory. We have a really busy and diverse care that we provide at the Emory neuro ICUs. Just at the Clifton campus, there’s over forty beds. So, although neuromuscular emergencies certainly do not make up the bread and butter of our practice - I mean, like many intensivists, I spend most of my time primarily caring for patients with cerebrovascular disease - this is a really interesting and just kind of a fun group of patients to take care of because of the ability we have to improve their outcomes and that some of these patients really do get better. And that's a really exciting thing to bear witness to.

Dr Smith: I love finding neurointensivists that are interested in neuromuscular medicine because I share your interest in these patients and the fact that there's a lot that we can do for them. You know, how did you get interested in neurocritical care, Casey?

Dr Albin: You know, I was always interested in critical care. It was really actually the neurology part that I came late to the party. I was actually, like, gearing up to apply into emergency medicine and was doing my emergency medicine sub-I (like, that was the route I was going to take), and during that sub-I, I just kept encountering patients with neurologic emergencies - so, you know, leptomeningeal carcinomatosis and obstructive hydrocephalus, and then a patient with stroke - and I realized I was just gravitating towards the neuroemergencies more so than just any general emergencies. And I had really enjoyed my neurology rotation. I did not foresee that as the path I was going to take, but after kind of spending some time and taking care of so many neurologic emergencies from the lens of an emergency department, sort of realized, like, "You know, I should go back and do a neurology sub-I.” And so, kind of, actually, late in the game is when I did that rotation and, like, dramatically changed my whole life trajectory. So, I have known since sort of that fourth year of medical school that I really wanted to focus on neurocritical care and neurologic emergencies, and I love the blend of critical care medicine and the procedural aspect of my job while doing it with the most interesting of all the organ systems. So, it's really a great blend of medicine.

Dr Smith: Did you ever think about neuromuscular medicine?

Dr Albin: Uh, no.

Dr Smith: I had to ask. I had to ask.

Dr Albin: No, I mean, I do really love neuromuscular emergencies, but I've known for forever that like, really wanted to be in an acute care setting.

Dr Smith: You know, I think it's such a great story, Casey, and I know you're an educator, too, right? And, um, we hear this from learners all the time about how they come to neurology relatively late in medical school, and it's been really great to see the trajectory in terms of fellowship determination dates and giving our students opportunities to make their choice, you know, later during their medical school career. And I wonder whether your journey is an example of what we’re seeing now (which is more and more students going into neurology because we’re giving them the free space to do that), and then also in terms of fellowship decisions as well (which was what I was alluding to earlier)?

Dr Albin: Yeah, absolutely. I think having more exposure to neurology and getting a chance to be in that clinical environment - you know, when you are doing the “brain and behaviors” (or whatever your medical school calls the neurology curriculum) - it is so hard and it's so dense, and I think that that's really overwhelming for students. And then you get into the clinical aspect of neurology, and sure, you have to know neurolocalization - and that is fundamentally important to everything we do - but the clinical application is just so beautiful and so much fun and it's so challenging, but in a good way. So, I totally agree. I think that more students need more exposure.

Dr Smith: Well, I mean, that's a perfect segue to something I wanted to talk to you about, which is you brought up the beauty of neurology - which is, I think, you know, neurologic formulation, really – and we talk a lot about the elegance of the neurologic examination. But one of the things I really liked about your article was its old-school formulation – you talk about the importance of history, examination, localization, pattern recognition – I wonder if, maybe, you could give us some pearls from that approach and how you think about acute neuromuscular problems and the ICU?

Dr Albin: Absolutely. I really do think that this is the cornerstone of making a good diagnosis, right? I will tell you what’s really challenging about some of these patients when they are admitted to the ICU is that we are often faced with sort of a confounded exam. The patient may have been rapidly deteriorating, and they may not be able to provide a good history. They may be intubated by the time that we meet them. And so not only are they not able to provide a history themselves, but their exam may be confounded by the fact that they're on a little bit of sedation, or they were aspirating and now they have a little bit of pneumonia. I mean, it can be really challenging to get a good neurologic exam in these patients. But I do think the history and the physical are really where the money is in terms of being able to send the appropriate test. And so, when I think about these patients who get admitted to the neuro ICU, the first thing that we have to have is someone who can provide a really good collateral history, because so much of what we're trying to determine is, "Is this the first presentation, and this is a de novo (new) neuromuscular problem?” or “Had the patient actually had sort of a subacute or chronic (even) decline and they’ve been undiagnosed for something that was maybe a little bit more indolent, but (you know, they had an abrupt decline because, you know, they got pneumonia, or they have bloodstream infection, or whatever it was allowing them to sort of compensate) they have no longer been able to compensate?”. And so, I really do think that that's key. And when I am hearing the story the first time, that's really one of the focuses of my history – is, "Was this truly a new problem?”. And then, when we think about, you know, "Where do we localize this within the nervous system?”, it's actually quite challenging because, you know, patients with acute spinal cord pathology may also not present with the upper motor neuron findings that are classic for spinal cord pathology. And so I think, again, it's a little bit recognizing that you can be confounded and we have to keep a broad differential, but I am sort of examining for whether or not there's proximal versus distal (like, the gradient of where they’re weakest), is there symmetry or asymmetry, and then, are there other, sort of, features that go along with helping us localize to something to the nerves (such as sensory symptoms or autonomic symptoms)? So when I think about, you know, where we're putting this, you can put anything in sort of the anterior horn cells or to the nerves themselves, to the neuromuscular junction, and then to the muscles. And teasing that out, I put in some figures and tables within the article to help kind of help the reader think about what are features of my patient's exam, my patient's history, that might help me to put it into one of those four categories.

Dr Smith: Yeah, I was actually going to comment on the figures in your article, Casey. They're really fantastic, and I encourage all of our listeners to check it out. There's, you know, figures showing muscle group involvement and different diseases and different muscle disorders and different forms of Guillain-Barré syndrome - it's a really beautiful way of visualizing things. I wonder if we could go back, though, because I wanted to delve down a little bit in this concept of patients who have chronic neuromuscular diseases presenting into the ICU. I mean, this happens surprisingly frequently with ALS patients or, like, myotonic dystrophy. I've seen this a number of times where folks are, just, they're not diagnosed and they're kind of slowly progressing and they tipped over the edge. Can you tell us more about how you recognize this? You talked a little bit about collateral history - other words of wisdom there?

Dr Albin: I would say this is one of the hardest things that we encounter in critical care medicine, because quite frequently - and I see this more with ALS than myotonic dystrophies - but, I would say, like, I don't know, once every six months, we have a patient who’s undiagnosed ALS present. And I think it can be extremely difficult to tease this out because there's something that's tipped them over the edge. And as an intensivist, you were always focused on resuscitating the patient and saving them from that life-threatening thing that pushed them over the edge, and then trying to tease out, “Well, were they hypercarbic and did they have respiratory failure because, you know, they've got a little bit of COPD, and is that what's going on here?” or, "Have they been declining and has there been sort of this increase in inability to ventilate actually because of diaphragmatic weakness and because of neuromuscular weakness?” Again, the collateral history is really important. One of the things that I think we are challenged by is how difficult - and I'm sure you can comment on this, as someone who is a neuromuscular guy - is how difficult it is to get a good EMG and nerve conduction study in the ICU in patients who may have been there for a little bit, you know? I think about this, sort of, the electrical interference, the fact that the patient's body temperature has fluctuated, the fact that they are, usually, by this time, like, they're a little volume overloaded – they’re puffy. You know, it can be very frustrating. I think, actually, you probably would know more about, like, what it's like to do that exam on our ICU patients.

Dr Smith: Sometimes, it's really challenging, I agree. And it's the whole list of things that you raised - and I think it goes back to the first question, really. You put a premium on old-school formulation, pattern recognition, localization, and taking a good history - you know, thinking of that ALS patient, right? I mean, one of the challenges, of course, that you have to deal with in that situation is prognostication and decisions regarding intubation, right? And that’s very different from (I'll give another scenario that sometimes we run into, which is the other extreme) a patient with myasthenia gravis who, maybe we expect to be able to get off a ventilator very quickly, but sometimes they’re reluctant to be ventilated because of their age or advanced directives and whatnot. I wonder if you could talk a little bit about how you approach counseling patients regarding prognosis related to their underlying neuromuscular disease and the need for intubation in a period of mechanical ventilation?

Dr Albin: Just like you said, it really ranges from what the underlying diagnosis is. So, one of the things that, you know, like you said, myasthenia - these patients, when they're coming in in crisis, we know that there is a good chance that they're going to respond pretty quickly to immunotherapy. I mean, I think we've all seen these patients get plasma exchange, and within a day or two, they are so much stronger (they're lifting their head off the bed, they're clearing their secretions), and every now and then, we're able to temporize those patients with just noninvasive ventilation. You know, when we're having a discussion about that with the patient and with the care team, we really have to look at the amount of secretions and how well they're clearing them, because, again, we certainly don't want them to aspirate - that really sets people back. But, you know, I think, often in those cases, we can kind of use shared decision-making of, you know, “Can we help you get through this with noninvasive?” or, you know, "Looking at you, would you be all right with a short term of intubation?” Knowing that, usually, these patients stabilize not all the time, but quite frequently, with plasma exchange, which we use preferentially. The middle of that is, then, Guillain-Barré - those patients, because of the neuropathy features (the fact that it's going to take their nerves quite some time to heal, you know) - when those patients need to be intubated, a good 70% or more are going to require longer-term ventilation. And, so, again, it's working with a family, it's working with a patient to let them know, "We suspect that you're going to need to be on the ventilator for a long time. And we suspect, actually, you would probably benefit from early tracheostomy”. And there was a really nice guidance that was just presented in the Journal of Neurocritical Care about prognosticating in patients with specifically Guillain-Barré (so that's helpful). And then, we get to the, really, very difficult (I would say the most difficult thing that we deal with in neuromuscular emergencies) - is the patient who we think might have ALS (we are not positive), and then we are faced with this diagnosis of, “Would you like to be intubated, knowing that we very likely will never extubate you?” - and that, I think, is a very difficult conversation, especially given that there is a lot of uncertainty often in the diagnosis. I would say, even more frequently, what happens is they have been intubated at an outside hospital and then transferred to us for failure to wean from the ventilator and, "Can you work it up and say whether or not this is ALS?” – and that, I think, is one of the most difficult conundrums that we face in the ICU.

Dr Smith: Yeah. I mean, that's often very, very difficult. And even when the patient wants to be intubated and ultimately receive a tracheostomy, getting them out of the hospital can sometimes be a real challenge. There's so much I want to talk to you about, and, you know, you talked about prognostication - really great discussion about tools to prognosticate in GBS, both strengths of things like EGRIS and the modified EGOS, and so forth – but, I wonder (given that I'm told time is limited for us) if you could talk a little bit about bedside guidance in terms of assessing when patients need to be intubated? You provide really great definitions of different respiratory parameters and the 20/30/40 rule that I'll refer listeners to, but I wonder if you could share, what's your favorite, kind of, bedside test - or couple of bedside tests - that we can use to assess the need for ventilatory support? And this could be particularly helpful in patients who have, let's say, bifacial weakness and can't get a good seal. So, what do you recommend? Is it breath count? Is it cough? Something else?

Dr Albin: I think for me, anecdotally (and I really looked for is there any evidence to support this), but for me, anecdotally - and knowing that there is not really good evidence to support this - whether or not the patient could lift their head off the bed, to me, is a very good marker of their diaphragmatic strength. You know, if they've got good neck flexion, I feel a lot better about it. The single breath count test is another thing that I kind of went down a rabbit hole of, like, "Where did this come from?” because I think, you know, it was one of the first things I was taught in residency - like, “Oh, patient with neuromuscular weakness, have them take a deep breath and count for as many breaths as they can.” We have probably all done that bedside test. It's really important to recognize that the initial literature about it was done in myasthenia patients who were in clinic (so, these were not patients who are, like, abruptly going to need intubation), and it does correlate fairly well with their forced vital capacity (meaning how much they're able to exhale on bedside perimetry), but it is not perfect. And I put that nice graph in the article, and you can see, there's a lot of patients who are able to count quite high but actually have a very low FVC, and patients who count only to ten but have a very good FVC. So, I do like the test and I continue to use it, but I, you know, put an asterisk by it. It's also really important - and I would encourage any sort of neurology trainees, or trainees in any specialty - if you're taking care of these patients, watch the respiratory therapist come and do these at the bedside with them. You'll get a much greater sense of (a) what they're doing, but (b) how well the patient tried. And it is really, I mean, we have to interpret this number in the context of, "Did they give a really good effort?” So, I'll often go to the bedside with the RT and be the one coaching the patient - saying, like, you know, “Try again”, “Practice taking this”, “Do the best you can”, “Go, go, go! Go, go, go!” (you know, like, really coaching the patient) - and you would be surprised at how much better that makes their number. And when you're really appropriately counseling them, that we actually get numbers that are much better predicting what they're doing. Then, you also have a gestalt just from being at the bedside of what they looked like during this.

Dr Smith: Yeah. I used to work with a neuromuscular nurse who was truly outstanding who was the loudest and most successful vital capacity coach ever. But, you know, she'd be doing it in one room, and you'd be in the next room with a patient. They'd be like, “What are they doing next door?” She was shouting and exhorting the patient to go harder and breathe better. So, it was always, “Wow, that sounds exciting over there”. All right, this is all in a prelude. What I really want to ask you, Casey, is, you know, whenever we do Continuum Audio interviews, we, like, look up people, and it’s not hard to look you up because you’re everywhere on the Internet. And come to find out, you’re a fully credential neuro Twitter star - and that's the term I saw, a star. So, what's it like being a Twitter star? I guess it's an X star. I don't even know what we call it anymore.

Dr Albin: I guess it's that. I don't know. I don't know, either. It's so funny, um, that that has become so much of my, like, academic work. I got on Twitter, or X (whatever it is) during the pandemic because, really, my interest is in, you know, innovatives and medical education, and I really had been trained to do simulation. So, I really wanted to develop simulation curriculum. I love doing sims with our medical students to our fellows. So, I was, like, developing this whole curriculum, and then the pandemic came along, and the sim lab at Emory was like, “Mm, yeah, we're not going to let people go in the sim lab. Like, that's not exposure that we want (people in a room together)”. So one of our fellows at the time was doing a lot on Twitter and he was like, "You would love this. You have cases that you want to teach about. You should really get on board”. And I, sort of, reluctantly agreed and have found the NeuroTwitter community to be, like, just a fantastic exchange of, you know, cases, wisdom, new studies - I mean, it's the way that I keep up with what is being published in the many fields that are adjacent to neurocritical care. So, it's very funny that that has ended up being sort of something that is a really big part of my academic time. But now that we're talking about it, I will give a plug for any of the listeners who are not on X. Dr Jones and I post cases, usually twice a week, that come directly from the Continuum articles or from our files (because, you know, sometimes we can spin them a little bit), but it's an amazing, sort of case-based, way to do some, like, microteaching from all of the beautiful Continuum articles, all the cases - and because there are free articles released from the issue, you know we'll link directly to those. So, for any of the listeners who have not, kind of, joined X for all the reasons that many people cite of not joining, I would say that there’s so much learning that happens - but Dr Jones and I are people to follow because of our involvement with Continuum and the great cases that we’re able to showcase on that platform.

Dr Smith: I think that's a great point. And, you know, there are certainly organizations that are questioning their engagement with X, and I'm on a board of an organization that's talked about not actually participating, and I brought up this point that I think the NeuroTwitter (NeuroX) community is really amazing. You'll have to give me some tips, though, I'm at, like, 498 followers or something like that. Do you know how many followers you have? I looked it up yesterday. I've got it for you if you don't know.

Dr Albin: I don't know recently.

Dr Smith: Yeah, 18,200 as of yesterday. That's amazing!

Dr Albin: Yeah, it's worldwide. We're spreading knowledge of Continuum across the globe. It's fantastic.

Dr Smith: That's crazy. Yeah, that's great work. It's really great to see the academic, kind of, productivity that comes of that. And I agree with you - Continuum has a really great presence there, and it's a great example of why you're the Associate Editor for Media Engagement. I think we're going to have to, I guess, gamify would be the right thing? Maybe we should, uh, see what the Las Vegas book is on the number of followers between you and Lyell Jones, I think.

Dr Albin: Totally.

Dr Smith: Yeah. Hey, Casey, this has been awesome. I've been so excited to talk to you - and I could keep talking to you for hours about your NeuroTwitter stardom – but in particular, neuromuscular weakness. I really encourage all of our listeners to check out the article. It's really, really, really, great - really enjoyed it. I learned a lot, and it reminded me a lot of things that I had forgotten. So thank you for the great article, and thanks for a really fun discussion.

Dr Albin: Thank you, Dr Smith. It was truly a pleasure.

Dr Monteith: This is Dr Teshamae Monteith, Associate Editor of Continuum Audio. If you've enjoyed this episode, please consider subscribing to the journal. There's a link in the episode notes. We'd also appreciate you following the podcast and rating or reviewing it. AAN members, go to the link in the episode notes and complete the evaluation to get CME for this article. Thank you for listening to Continuum Audio.