 So the inspiration for this presentation came about a month ago, a month or two ago when I was reminded of a story from medical school. Early on in my first year, there was an older student who had come to be friends with who I really thought was an intelligent, fun, easygoing guy. And I was not with some of his friends one night and was sort of taken aback by them telling me that they felt like he had what they called a darkness about them. They said they used to hear him audibly berating himself in the shower or other times. And I remember thinking to myself that that was crazy, that I needed to make sure that I never got to that point. But by the end of my first year, I literally caught myself berating myself in the shower. And it had taken the voice in my head becoming audible to realize how much of a toll that first year had taken. Over the subsequent years, I've had a fair bit of success with becoming more aware of that voice, recognizing it and quieting it. But it still pops up from time to time. And although it might not be quite as audible for everyone, I think this is something that many people, especially in medicine, experience to one degree or another. So there were several things during that same time period that initially brought that story to mind. We had two excellent and thought-provoking Taco Tuesday sessions, one on imposter syndrome and the other on wellness and cognitive distortions. I happened into a number of conversations with colleagues about the discomfort we feel in the face of uncertainty, either in front of colleagues or in front of patients. And then I came across a few podcasts by one of my favorite podcasters, physician Peter Atia, who talks a lot about negative self-talk and how it is particularly prevalent in medical trainees and about self-compassion as a potent antidote and even a more effective motivator than self-criticism. So I wanted to try to get to the root of whatever these problems are. And the problem goes by many different names, cognitive distortions, imposter syndrome, negative self-talk. And we know it's especially bad in medicine, just calling to mind the issue of physician suicide makes this abundantly clear. One of our very own here, Dr. Catherine, who refined these ideas and built a conceptual framework for how medical students, but also presumably how all practitioners get caught in a self-perpetuating cycle of shame, depression, anxiety, wherein dysfunctional thoughts lead to negative feelings, which themselves lead to emotional distress, which itself provides fertile ground for those distorted narratives to continue to grow. And there's several solutions that we've recently discussed as a residency, more of that seemed to be more widely discussed these days. Numerous wellness initiatives over the past several years have had measurable positive impacts on many of the residents. We discussed self-compassion practices in one of our sessions on Taco Tuesday sessions. And meditation is something that's fortunately becoming much more mainstream than it probably was in the past. But the idea of narratives being at the heart of the issue was something that kept coming up, and it brought to mind the idea of narrative medicine as an antidote to burnout and emotional fatigue. The topic of narrative medicine is something that had a bit less exposure to. I somehow committed myself to giving a grand round on the idea of narratives, and it was at about that time that I figured out I ought to do some research on the topic. So I borrowed a copy of Lindsey De Nino's The Principles, sorry, Lindsey De Nino's copy of The Principles and Practice of Narrative Medicine by Rita Chiron et al. and I worked through it over the past month or so. Lindsey had previously recommended this book to me based on discussion that we had, and so it's something I've had a peripheral interest in for some time and thought I'd take a deeper dive into it. And right off the bat, I was struck by this concept of thin stories that I immediately identified as what I felt was the foundational issue, the thing underlying so many of those issues that I just mentioned, and we can pinpoint exactly which type of distortion is present in a dysfunctional narrative and recognize that our negative self-talk comes in innumerable flavors and textures, but all of them are thin. They lack context, insight, reflection, and compassion. And the idea of a thin story comes from psychologist Michael White, who describes them as generally negatively charged, frequently characterized by conclusions that one is hopeless, a failure, incompetent, unworthy, hateful, inadequate, and so on. Now in this context, the word story being applied specifically to a personal narrative, but I think it's more helpful for reasons that will be clear in a minute to think of a story a bit more broadly encompassing the narratives that were told by patients, friends, colleagues, and even the narratives that we read. So the opposite of a thin story is, of course, a thickened story. It adds the context, it adds compassion, and in so doing, it creates a richer narrative and reveals insights to the speaker and the listener that exist far below the surface of a thin story. And this passage uses the word story in that much broader sense I was mentioning. So the authors say to narrativize and thicken a story is to tell it in a new way to a responsive listener, not to change the events of the story, but to discover novel significance in them, so that the thin conclusion no longer appears fixed and essentialized. And so then the question is, how do we thicken our own stories? And the practice of narrative medicine proposes to be a means of doing just that. The authors call close reading the signature tool of narrative medicine. And the term itself, they say, they talk about at length and it's been used in circles of literary criticism, starting around a century ago and was adopted as a foundational method of narrative medicine's teaching and practice. But as with anything that's been hanging around circles of literary criticism, the term is a bit esoteric and obscure. The authors do a fairly good job, though, of breaking it down into something tangible. They describe that close reading is not for the purpose of cultivating novelists or poets. On the other hand, it is for increasing one's capacity to notice things, to be curious about words, to gain insight into one's own biases and interpreting stories. And they propose that a method for teaching close reading by reading non-medical literary fiction with an eye toward the author's use of time, space, voice, and metaphor. But that's still something that's a little hard for me to wrap my head around. I feel like I need a teacher to guide me through it. I think an idea that might be more palatable or comprehensible to a lot of people is just that of engaging with art and creative works on whatever level that might be for an individual. It could be literary fiction, poetry, film, music, visual art, or any other creative work. And this talk actually initially included a brief history of how Western medicine came to be so focused on the biological sciences, often to the exclusion of the humanities. And while the reasons for that may seem obvious and the resultant benefits of scientific advancements over the past century undeniable, there's a reason to think and increasingly evidence to suggest that it might come at a cost. So, Ken Costano in 2016 published a study in science showing that reading literary fiction as opposed to nonfiction, popular fiction, or nothing improves theory of mind, which is the ability to understand other people's mental states. Manjion et al. in 2018 showed that exposure to the humanities during medical school correlated with increased empathy, tolerance for ambiguity, wisdom, emotional appraisal, self-efficacy, and spatial skills was inversely correlated with burnout. Winkle et al. in 2016 reported on the results of a one-year narrative medicine seminar where OB-GYN residents from three programs would meet once a month for an hour. And over that year, those with higher narrative medicine session attendance had a decrease in emotional exhaustion compared to those with low attendance who had an increase. And all these studies seem to suggest in one way or another that engaging with creative works and cultivating creativity can be beneficial, either to the well-being of medical trainees or to patient care. But there's an additional consideration, I think, in the author's posit that the medical field has a particularly tense relationship with creativity. Most patients probably don't want a doctor known for her creativity. But the authors propose that this is a flawed view of creativity, that the very act of listening to a patient's story, examining a patient, and putting all of that information and data together into a unifying diagnosis is itself a creative act on the part of the practitioner. Just the other day, Dr. Shakur told me I had to stand back and look at an ICG like the work of art in order to appreciate a subtle core ioditis. So I think, again, that there's reason to believe that the idea of exposing oneself to and fostering one's creativity is not just a boon for the individual, but also potentially beneficial to patient care. And I want to go into some more tangible examples of what this actually looks like in practice, but I wanted to pause here first and ask if anyone has any thoughts on this personal experiences or anecdotes about how exposure to the humanities has either improved personal well-being or their patient care. Or maybe even people have other creative outlets that I haven't mentioned. It occurred to me last night that the culinary arts might deserve to be included among things like literature and music. So you can feel free to ask to be unmuted or just put ideas in the comments if you'd like. Give a few moments here if anyone would like to. I can unmute myself quickly. So I feel like Randy always gets to brag that he's a math major and I never get to brag that I'm a humanities major. So that's my first time ever in medicine. Thank you for that. It seems like you're trying to connect the dots between, I'll just say, engaging in a creative opportunity with improved mental well-being. And just a comment for consideration. So often we talk about all the self care, right? All right, residents, you're unwell. Do more yoga, get more sleep, et cetera. And yet we have a system that doesn't allow that. We've really worked hard to address that in the residency with your admin half days and different things. And so one of the questions I have, the skeptical side of me is, is it just a matter of giving people more time to engage in the things that make them well? Or is there something unique to this creative humanities outlet that you feel is fundamentally important for this issue? I do think that there is something unique about the exposure to the humanities to creative expressions. And I think I'll sort of get into some of the examples of that. But I absolutely agree. And something that Dr. Dredgeen talks about all the time is the irony in making things mandatory in the name of wellness, adding things to residents' schedules in the name of wellness. And that's certainly not what I'm proposing. But I do think that many people, and this may not apply to everyone, probably find some outlet in engaging with creative works in humanities, but may feel that that is sort of removed from their practice of medicine. And I think that some of this evidence and some of the things in the idea behind literary medicine supports an idea that it actually contributes to building your medical practice. Yes, it contributes to your wellness, but also has potential to create a better physician in the room with the patient. Thank you, John. Looks like there's just two other questions. I'll just kind of read up while you're here. Again, you may need to move on. But Griffin just noted, I've often found playing the piano or listening to instrumental music be very therapeutic, especially when stress is high. So nature, this is from Dr. Olson. So nature and appreciation of finite status has been great to give me perspective and humility, especially that it is not about you. I also love well-done biographies to show how ordinary people really were capable of extraordinary things with an emphasis to their very humanity from Dr. Olson. And then Chris Keith, I come to my technical field via creative work, film production of fine art photography, love everything about the way I think to creative work and the forms of angle I take and the problem-solving can serve me well. So no specific questions there, Sean, but want to do at least let you know of those comments at this point. Dr. Olson is muted. Hey, listen, if I could just go ahead and comment a little. I think what you're saying here is great. And that is is that because medicine can be so stressful and so all-consuming, I think we sometimes get ourselves cocooned in that. And because, sadly, if you make a mistake and maybe your sentence isn't quite right or your painting isn't quite right, we make a mistake and our patients can pay a price. That's a heavy burden. And so I think that we have to learn how to cut ourselves some real slack. And we have to learn to appreciate that you can't change the past. You want to learn from it, you got to move on. That's why I think it's important to have balance. And I think that's what you're talking about here, really. And all of these things help give balance. And to the point that we don't allow balance in our life. And I've seen people like that. Medicine is a rough taskmaster that just can eat you up. And so I'm a big believer that that balance is critical. And I don't know. For me, I've loved getting way away back up in some of the wildest areas in the Wind Rivers, for instance. There's just a sense of both appreciation of the grandeur and a sense a little bit of your own significance in a certain way where it's not all about you. You're part of what's going on. And so you can't take it so personal. You've got to be thinking more of others and yourself. And then it seems to me like a lot less of a problem. Anyway, those are just some things that, for my long career, have been very helpful. If I don't get time out, exercise is another way for me to think about things in a different way. If I don't have time for that, I can feel the stress building. You've got to work to be able to find a creative outlet to release that. Absolutely. Absolutely. And I think those times in nature or whatever it is in our creative outlets are times when we were able to be so much more aware of all the nuance in our surrounding. And that's what narrative medicine tries to get at as well. So let me try and make it even a little bit more tangible in terms of what the practice of narrative medicine looks like. So that last study I mentioned there, and thanks to everyone who shared. I also want to be clear that the Murray and I Center in particular in this residency program has done a phenomenal job, especially with some of the wellness initiatives. And like I said, I think that a lot of residents have experienced measurable benefits from them. And this is just one thing that I'm sort of adding to the list of things for consideration. So in any event, that last paper by Winkle et al. showing that residents with the highest attendance at narrative medicine seminars had decreased emotional exhaustion after one year. It happens that the book contains specific excerpts from some of the sessions that were part of this study. And I want to share one of those. Basically, they had 15 session curriculum designed to run over a two-year period of time. And each session had a theme. So calling, why do you do what you do, work-life balance, making mistakes, so on and so forth. And each had a reading assigned to it. And the reading often was not obviously tied to the theme. One of those, I believe it was session five on managing expectations. In that session, the OB-GYN residents were asked to write a definition for some of the ACGME core competencies. And the idea was to somehow make these competencies meaningful in a way that the residents could appreciate. Now, the ACGME defines practice-based learning and improvement as the ability to investigate and evaluate patient care practices, appraise and assimilate scientific evidence, and improve the practice of medicine. So one of the fourth-year residents at the start of the seminar or the start of the session gave this definition, using experiences in daily care of patients to guide further decision-making. Now, I think that's a perfectly good definition. But after writing that definition, they read Jamaica Kincaid's Girl, which is a short story, and they were then asked to write instructions to themselves in a similar style. And this is what one of the fourth year residents wrote. Don't wear scrubs outside the hospital. If you want to have scrubs your size, you're going to have to bring them home. Make sure to eat breakfast. If you skip breakfast, you'll be grumpy by noon. Don't be grumpy. Not in smile while I yell at you. I'm not yelling at you. Why did you do that? Present the list of grand rounds. Tell the story so I know what you were thinking. Why did you think that? Do it this way. Wait, I have something else to tell you. Hold on a second. There's something else. Don't be late. Try to move a little more quickly. There are a lot of patients on the labor floor right now. You should really check that the junior resident checked that lab. I'm going to need to use a scarf for this case. Teach your intern to do this another way. Why did you do it that way last Tuesday? Stick around. There's something you should tie up before you go home. Why'd you go over work hours? Change back into your flip-flops before you go home. So this student chose to write instructions to herself about medicine, about life at the hospital. Other students chose to write things completely separate, unrelated to medicine. And then at the end, they were asked to revisit the practice based learning and improvement competency. And of course, whether or not this is better is highly subjective. But to me, it seems richer. So the definition before is using experiences and daily care of patients to guide further decision making. And the same fourth year student's definition afterwards was incorporating the lessons of the day's work and the experiences around me with the technical and cognitive knowledge I've gained into a more whole image of a doctor. So that is one example. It may be compelling to some, maybe not to others. Another example I wanted to share, and I think I'm coming up on my time here, but this won't take too much longer, was this one. I know this slide violates everything you're not supposed to do in a PowerPoint presentation. But I just love this example of a creative piece a student wrote after a six-week seminar about a patient encounter. So the student wrote, what I wrote was the patient has a prior history of falling when trying to cross the street. What I wanted to write was Ms. W, who's 90, was jaywalking in NYC while returning to her apartment from the liquor store. She, quote, must not have seen an indentation in the road, end quote, when she tripped and fell into the street as her newly purchased bottle of Chardonnay rolled away from her. Ironically, though, she endured iterations and scrapes all over her face and arms. The wine bottle remained miraculously intact, untarnished, and ready to drink. Ms. W laughed at the irony of the situation in telling the story and noted that she had retrieved the bottle before returning to her apartment. What I wrote was, patients MSE was significant for zero out of three objects recalled after three minutes. What happened was I asked Ms. W if it would be all right to test her memory, given that she'd noticed difficulty finding words over the past year. She replied, I know one of them is going to be soldier, right? Sure, it can be. I replied, grinning, falling into her infectious rhythm. Let's make it soldier, apple, and pen. She repeated the three words. She thought hard about them, but when it came time to remember, she could not. Do you remember that you suggested one of them to me, I asked, rooting for her? Soldier, she said triumphantly. What I wrote was the problem list, an assessment and abbreviated adulterated formulaic version of the rich encounter I'd had. I was schooled numerous times by this way fish comedic, lovably difficult woman, but here I was leisurely breaking her down to finger arm, eye, head, from a lofty spot miles away, deleting the character, leaving the complaints. I know this patient, but who else will? Now there's an impracticality here, of course, and I'm all for brief and to the point documentation, but it calls to mind a particular patient encounter that's actually been ongoing for me recently. That's quite sad and that several people here are involved in. And it's an interesting and perplexing case from an academic standpoint. And often the discussion with someone unfamiliar with case starts with the medical facts, but where the story really evolves and where people's expressions and investment in the story changed dramatically is in little things I picked up along the way. A photo of the patient riding a horse years back that he keeps as his background, his sister's account of how much faith he's put in his healthcare providers over the past several months, the way he tried to communicate that he was scared through a trade or conversations with his wife who's unable to be in Salt Lake with him. I know I personally find that it's a chore to go and re-examine his eye, but those little nuggets that thicken his story are really revitalized for me. And I can see that they are for others who hear his story as well. Now, you may never recall at this point that this started off as an inquiry into our own dysfunctional personal narratives and what we can do about them. And at the risk of becoming a bit difficult to follow, I think it's essential to realize the knowledge that our own stories and the stories of our patients sufficiently thickened are incontrovertibly intertwined. I know that personally, when I find myself in either a particularly good or bad mood, it's easy to track that feeling back through a chain of interactions with colleagues, patients, friends, family, and strangers. Philosopher Alastair McIntyre provides a caricature of the chain of events that unfold in such a situation like this. What I feel is in large part a response to what I take others to feel or not to feel. You are resentful of my lack of gratitude at your generosity in the face of my anger at your lack of sympathy for my depression over your sentimentality. And our ability, therefore, to understand our own emotional state, our own narrative is contingent on our ability to do the same for others. So I have this last quote here. I won't read it in its entirety in the interest of time, but in order to bring this full circle, essentially, Rita Sharon says that through close reading, and I think we can substitute any sort of creative outlet for close reading, the close reader is accompanied on the readerly journey not necessarily by another person who will actually say things back by similarly attentive presence, the self-increasingly known. And ultimately, the first step I think in changing negative personal narratives is recognizing them, getting closer to them, investigating their origins and thickening them. And it may be a bit of a leap, but I believe engaging with creativity, whether in literary fiction, painting, piece of music, or anything else, and learning to appreciate the subtleties and nuance contained within it is an invaluable tool for cultivating the skills that allow us to thicken the story, whether told to us by a patient or by ourselves. And that's all I have. I want to go down to Lindsay Dunino who gave me the initial inspiration, let me the book, and whose office I'm borrowing right now. Griffin Jardine who advised me very helpfully on this presentation yesterday. Any shortcomings are my own. And Eric Hansen who was encouraging of this topic. Here are my sources. I'd love to hear any comments. Your presentation is very well thought out and very thought provoking, I appreciate it. There's some excellent comments in the chat box. If you'd like to review those, in the interest of time, we will unfortunately have to keep moving and we'll move on next to Dr. Simpson. Dr. Simpson is some quick things about her. She's an award-winning baker. She won the Iowa State Fair. And also an effort to embarrass her. Her birthday was earlier this week, so happy birthday. And she will be presenting a case of a two-year-old with ptosis. Dr. Simpson, please take it away. Thanks Abigail. She's cute up. All right, so we're gonna switch gears and talk about a case presentation that I saw in a two-year-old with ptosis. So she presented to the PCHED at the beginning of August last year with her dad with the complaint of right eyelid droop. HPI was about a week prior. She had nausea, vomiting, and poor pio intake and was overall fussy. And then about five days later started to develop right eyelid droop in which she was intermittently rubbing her eye in her head and dad was unsure if it was painful or if the ptosis was just bothering her. Past medical history is really unremarkable. Normal birth, normal development, no past ocular surgeries. No medications or allergies. Her family history was a negative for inherited eye disease, amblyopia, glaucoma, headache and migraine. And she currently lives in Utah with her dad. On exam, unfortunately with the ptosis we were really unreliably able to get a vision because she was not tolerating manipulation of that right lid. Hey, I saw your message. I wanted to take some call. In the left eye, she had an unreactive pupil on the right side. And from the best that I could tell, a right exotropial and hypotropia on the right side with adduction and superduction deficits. The rest of her exam was notable for ptosis on the right side around a non-reactive pupil on the right. And her DFE was just with an elevated cup-to-disc ratio of 0.8 in both eyes, otherwise normal. And so now, now what do I do? Do I panic? Do I leave the ED and have PCH consult pediatric neurology? Or do I call my friendly neighborhood a neuro-ophthalmology fellow? I ended up doing the latter after considering and probably doing the first and second. But I knew that this new neuro-ophthalmology fellow was going to expect a differential diagnosis for me for what seemed to be a cranial nerve three palsy. And at this point in time, I have an extreme amount of experience in exactly one month of PGY2 year under my belt. But what I remember from being a VA intern was that if a cranial nerve three palsy was pupil sparing, it was ischemic, microvascular. And if there's pupil involving, it was aneurysm and I should send them to the ER. However, I knew she'd probably expect a little bit more from me. So I came up with this list, which of course still has ischemia and aneurysm, but also has other ideologies like infection, demyelination, lesions, that sort of thing. The problem I had with this list and the problem I had with this case to start was that she was two years old. And so I had to look up what were the common causes of cranial nerve palsies in children. And this was the data that I found for cranial nerve three or three, six or four palsies in children and congenital and trauma being the most common. However, her history didn't really fit with that. So I knew that that probably wasn't gonna be a good differential for my neuro-ophthalmology fellow. So what we decided to do was we came up with a plan and generally it would go after some of those mass lesions and aneurysms with the things like a brain and orbit MRI and an MRI head, but we were also gonna work up for other demyelinating inflammatory or infectious ideologies with lab work and a lumbar puncture. And overall, she had a really unremarkable workup. She had an LP with normal constituents, no bands, no meningitis encephalitis. Her autoimmune demyelinating panel was negative and her infectious workup by lab work but through serum was also unremarkable. The only things that came back positive was a slightly elevated ESR and EBV-IGM and IGT that were both elevated. On her imaging, so this is a T1 with contrast image that shows that she had enhancement of the root entry zone of the right cranial nerve three exclusively, very isolated, the rest of her MRI was normal. And on her MRI, unfortunately, the cuts didn't really lend itself well to this presentation but she had a right fetal PCA that was read as kind of displacing the right cranial nerve three root posterior laterally. So her hospital core, she was admitted for a couple of days. They ended up consulting ID to help kind of manage and interpret the multiple infectious labs that were taken, which they thought that there was evidence that she had mono likely within the past six weeks and that her current presentation was most consistent with just biogastroenteritis. Neurosurgery was also consulted given this fetal PCA that could have been pressing on the cranial nerve three. And they felt that there was no need to do an angiogram and that cranial nerve three compression is case reportable and that there was other causes that were much more likely than her fetal PCA causing any problems. So now what? I found that getting more history is never the wrong answer but in this case, I am kind of leaving some key things out that I'll fill you in on. So about one year prior to your presentation, she had right eyelid ptosis and a turned out right eye that resolved over the course of about six months. And dad was told that imaging at the time was done, he wasn't with her, she was with her mom but they were told that she had an abnormal cranial nerve three and she was told by a neurologist that this may happen again. She then was seen in our clinics on the fifth, or in May, excuse me, where she was noted to have an intermittent exotropia but no pupil or ptosis abnormalities. And then the day prior to her ED presentation, she actually came to clinic where she was noted to have a constant right exotropia, complete ptosis and a poorly reactive pupil. And there was concern for cranial nerve three positive issues she was sent urgently to the emergency room. So a few weeks after she discharged from the hospital, she was seen in follow-up, which her ptosis had improved some but was not completely resolved. She was able to fix and follow in the right eyes. She still had some anisocorrhea and she still had a superduction and adduction deficits and a large right exotropia and hypotropia. So patching was started for her. A few months later, this was last month now, she was seen in clinic, ptosis had resolved, her anisocorrhea was resolved, her EOMs were full but she was still having a right exotropia with concern for development of dense amblyopia in the right eye. So patching was continued but very heavily considering stirbismus surgery. Her neurology team brought her back in November for a repeat MRI, which showed improved enhancement of that area that was previously enhancing that had a result and there was no evidence of a remaining tumor. So what we have is a two year old girl with what sounds like two attacks of cranial nerve, three palsy, which seemed like they had resolved mostly entirely and this reversible enhancement on MRI and imaging. And all that put together came up with us for the diagnosis of recurrent painful ophthalmopolegic neuropathy or ophthalmopolegic migraine is what it was formerly known as. And so the epidemiology is very rare as about 0.7 per million people and it's largely a pediatric disease, typically less than 10 years old with the onset usually being around eight years old. There is slight female predominance. Earlier studies suggested that there was male predominance but I think that as more studies have been looked at there there's actually more females affected and a large majority of these patients have a personal or family history of migraine. So what is recurrent painful ophthalmopolegic neuropathy? It is characterized by an ipsilateral headache followed by a palsy of one or more cranial nerves. The headache does not have to be a migraine but in a large majority of the animal about 60% that there is migraine or migraine features. The palsy typically starts between zero and 14 days after the headache onset which can be difficult if you have a headache and then two weeks later have cranial nerve palsy to kind of correlate the two. By and large it's usually the cranial nerve three that's affected about 70% and there have been reports of cranial nerve six and four being affected. The ophthalmopolegia usually lasts around a few weeks but can persist up to months and unfortunately the repeat attacks are really poorly predicted. Sometimes it's a week, sometimes it's five years before they'll have another attack of RPON. So as I mentioned before this was formerly known as ophthalmoplegic migraine as it was felt to be related to a migraine phenomenon. However, in 2013 it renamed it and felt that it was actually more related to a painful cranial neuropathy. And so it falls under the category of painful cranial neuropathy and facial pain in the ICHD diagnostic criteria now. And what I wanna point out is that it is a clinical diagnosis but that we need to exclude kind of getting to criterion C and D other causes of cranial nerve three palsy. So a lot of times these people are getting to work up to rule out other causes. And we get a lot of aid in this from our imaging findings. And the imaging findings that I discussed before are actually part of the reason why it was recategorized from a migraine to a neuropathy. The pathophysiology is really difficult for us to kind of get a handle on. It's based basically on case reports and radiographic finding. Initially it was felt that it was possibly compressive and related to migraine, which caused compression of the cranial nerve root. However, it didn't really explain pupil sparing palsies and why the palsy would be so persistent even after the headache resolves. Some have felt that this is related to ischemia and that perhaps vasospasm causes breakdown of the blood-brain barrier, which would allow us to see those MRI findings of enhancement and that they've drawn correlations between the time course of resolution between a diabetic third and RPON. The last one that they've kind of entertained is denomyelinating or inflammatory condition. And this is because of those MRI findings of enhancement of the cranial nerve and thickening. They thought perhaps this is post-viral or related to a condition, something like CIDP or Guillain-Barre syndrome. But again, and a lot of these people, they're work up with serum lab work and with CSF is negative. So maybe a mix of all three, it's really been difficult to kind of parse out what is actually causing RPON. So these are the very classic imaging findings. This is a photo from one of the case studies, but I could have easily put our patients' images up here. And what they have found that there is enhancement and or nerve thickening, typically in the cisternal portion of the cranial nerve or in the root entry zone. And this is present in about 75% of patients who have been diagnosed with RPON. And typically this enhancement, like in our patient resolves over the course of weeks, kind of as the cranial nerve palsy gets better. And in a study that they looked at that they had also gotten vessel imaging, it was normal in 93% of patients, which called in a couple of big questions for me for this case. And the first one was, what is the significance of her fetal PCA? As suggested before, the overlap, there's only maybe 7% of patients that have abnormal vasculature when they're being worked up for RPON. But there is a few rare documented cases of concurrent RPON and vascular abnormalities. And they've suggested that the proposed mechanisms either be direct compression of this vascular abnormality or perhaps an altered trigeminal vascular response and that the proximal portion of the PCA has a lot of density of receptors. And so edema in that area can trigger the cranial nerve palsy as well as the headache. And I kind of wondered about the clinical relevance of this since neurosurgery said, this is a very rare case reportable. And it's true, there's just a few handful of cases largely in people who are old or 50 to 70 or so years old in which they had cranial nerve three palsy that they felt was related to vascular compression and they decompressed them. These patients, all of them got better or completely resolved in some sort of way. But they also noted that in 70% of people who they managed conservatively also got better. And so they recognize that there's a great overlap between RPON and these people that they're decompressing and that even it's a really difficult diagnosis to make even with imaging. So fetal PCA probably incidental, definitely not the full story with RPON. The next question I had is why did we repeat the MRI in November? We have two clear attacks. We have the classic MRI finding of that nerve root enhancement. Why do we need to repeat? I mean, if she had a cranial nerve palsy and got nearly completely resolved, it's unlikely to be a tumor, right? That's kind of what I was thinking, but actually there are a decent amount of case reports in which they've had schwannomas mimic RPON. And there are a couple reasons why they think that this could be the mechanism. And one is that perhaps these initial attacks of RPON are actually schwannoma in which the tumor is just too small to be seen in an MRI. The other thought is perhaps these repeated attacks of inflammation and demyelination actually cause cellular transformation and eventually will cause a schwannoma to develop so that RPON is actually a risk factor for the development of a schwannoma. The main takeaway, again, this is just a small percentage of people who have been diagnosed with RPON and it's likely not the full story. But what I gathered from looking into this was that we need to repeat the MRI at each attack. And there's a couple of reasons. One, if there's no, just because someone has RPON doesn't mean they can't develop an aneurysm or something you would hate to miss. And two, they could have also developed a schwannoma that maybe is now more evident on imaging. Some have suggested repeating imaging at a more regular interval in between attacks, but that's really poorly described. So the treatment, as you probably gathered, we're working on a handful of people, fractions of percents to kind of gather what we should do and how we should treat these patients. So there are no controlled studies. The biggest body of data that we have is on the use of steroids, and particularly in those that have enhancement on their MRI. And what they found is that about 50% of those responded to steroid treatment. And that could have been just a little bit of an improvement in ptosis or medriosis or complete resolution. And they kind of described it as that, of how that we'd use steroids and optic neuritis and that it may hasten the recovery but might not change the overall endpoint of how much function they'll regain after their cranial palsy. And the last thing is that there's no clear evidence for migraine prophylaxis. Back when we thought this was related to migraine, people were being put on calcium channel blockers and beta blockers to help prevent recurrent attacks. And what they found that there's no clear evidence to do this, and that the only reason that they should be on it is if they also have concurrent migraines, which a lot of these folks do. And so the prognosis, it's generally favorable. A lot of these folks will recover, functioning about over the course of a few weeks. But in one study, they found that about half of them do not recover completely. And this kind of was the whole spectrum of patients. Those who had a couple millimeters of ptosis or a little bit of medriosis, this remaining to those who really had virtually no recovery of their cranial nerve palsy. And that they did find that these repeated attacks have worse recovery. And I feel like that's shown really well in this patient that she had a decent recovery, just an intermittent exotropia after her first attack. And now she's having more problems with strabismus after her second attack. And the long-term treatment is really not well described. And so we kind of focus on the symptoms. So things like prism glasses for double vision and strabismus and ptosis surgery for those that still have remaining ptosis and strabismus. And in her, her amblyopia is really a great concern. She kind of has risk for strabismus and deprivational amblyopia in that right eye. So I would suspect that they'll be moving forward with strabismus surgery in the near future. These are my references. I'd like to thank doctors Warner, Redfern, and Bagunta for helping me prepare this presentation. And I'm happy to take any questions now or at the end so we can get on to the next presentation. Ali, we have time for one question. Dr. Petty put one in the chat box. I'll read it for you. With so much variability in the potential presentation, headache or not, migraine or not, up to two weeks before, imaging normal or not, is it just the basket to put any idiopathic cranial nerve palsy, or are we convinced it's directly tied to migraine or is migraine the basket? That's a really great question. I think it kind of gets at what we think this is, which has been difficult to describe. I don't, I'm not convinced based on my study that this is necessarily related to migraine. I think that there must be a correlation between patients that suffer from migraine and develop RPON. But I wish I had a better answer with as to why this cranial nerve enhancement develops. As far as, I'm sorry, what was the second part of the question Abigail? The diagnostic criteria, if it were. Yes, if, because it's so variable, is it, is any idiopathic cranial nerve palsy kind of related to that? Or, yes, Dr. Petty, if you want to chime in. Is it directly tied to migraine or not? Or is the overarching kind of umbrella or basket migraine? I guess. Can we do that correctly? So I think there might be an overarching migraine kind of predisposition in these folks, but I don't know that they're necessarily related to migraine because of the way that kind of natural time course of these. And I don't think that any idiopathic cranial nerve palsy, I guess I could see where that would be the case. I would have to be more convinced of it. And as we've, as the diagnostic criteria suggests, if a person has one attack of a cranial nerve palsy and their workup is otherwise unremarkable, someone might tend to favor that to be ischemic. But it's not until we have a second attack that we're able to comfortably make that diagnosis. Thanks so much, Allie. And thank you for your question, Dr. Petty. We'll move on next to Dr. Twitchel. He is a neurosurgery PGY1 originally from Utah who last year amongst, as all of us did, surviving the pandemic. He had quite a year as he couples matched, graduated medical school, got married, bought a home, got a puppy and started residency. He will be presenting a case of ocular myotonia. Thank you for the introduction. I'm happy to be here to join you and to discuss a little bit of what I've learned and if this will work for me. Start off. So this gentleman was a 54-year-old gentleman who back in 2016 had six months of very non-specific symptoms. He had difficulty ambulating fatigue. He had leg weakness and swelling and sores, blurry vision and was diagnosed with cataracts. And then he had stalking glove numbness and decreased libido and impotence. He has a history of osteoporosis with multiple spine fractures and diabetes. And eventually, through all this workup, he ended up being found to have a pituitary macrodenoma and a planem sphenoidalaminin angioma. So he underwent a transfenoidal resection. Spencer, are you still there? I think your audio went out. Can you hear me? Yes, we can hear you now. You're kind of going... Well, hopefully it holds out, but on the left, we can see a coronal T1 MRI post-contrast demonstrating the planem sphenoidalaminin angioma with the typical dural tails that you see evidenced here that are very pathognemonic of meningioma. And on the right, we have a pre-contrast image demonstrating the pituitary mass as well. So the differential of a cellar mass includes rathkis cleftis, specific pituitary adenomas, pituitary apoplexy, arachnoid cysts, epidermoid cysts, teratoma, and cranioferringioma. In this situation, it was more likely that this was a pituitary adenoma, but any of those are possible until we see pathology. The differential for that skull-based mass, first and foremost, would be meningioma. It could be cranioferringioma, astrocytoma cordoma, chondrosarc, or a giant cell osteosarcoma, or any of these other tumors, but they are less likely due to the review of the imaging. So after surgery, he had a resolution in his visual symptoms, but as he was managed with postoperative chemotherapy, he had worsening of his Cushing's disease. And then in June of 2018, he was seen to have a recurrence of his pituitary macrodenoma in the cavernous sinus. This was treated with radiotherapy. And then in 2018, he had a bilateral adrenalectomy for his Cushing's disease. And then in 2020, he had a new onset of episodic diplopia with MRI that demonstrated his progression. He was treated by radiation oncology with radiotherapy, his second round, and he had no improvement in his visual symptoms. It was only at this point that he was sent to neuroophthalmology for evaluation. Here you can see his Humphrey visual field testing. The top line is prior to surgery. The middle line is just after surgery and the bottom line is most recently. And you can see that he's had resolution of his previous visual deficit there in his right eye. On examination when he presented into the clinic in November, you could see that he had reason for his diplopia with his alternate cover testing. He had exotropia and an isotropia. And then with his Maddox rod testing, you can see that he was having difficulty with aligning his eyes correctly in various positions. His color plate testing though was entirely intact. And then his visual acuity as well was excellent. This is a video of the examination. So in the interest of time, we'll move on, but the differential diagnosis for his presentation included an ocular neuromyotonia, convergent spasm, ocular myestinia gravis, superior oblique myocymia, or a decompensated heterotropia. In the end, it was concluded that he had ocular neuromyotonia. Ocular neuromyotonia is very rare, but it's an involuntary episodic contraction of the muscles supplied by the ocular motor nerve. It's due to nerve hyper excitability resulting in a persistent contraction of one or more of these extraocular muscles controlling their vision. It has been noted to be associated with certain neoplasms such as thymomas, small cell lung cancer and Hodgkin lymphoma. And it's also been noted to be associated with neurovascular compression syndrome. The primary muscles implicated are distributed pretty equally between the vertical and horizontal muscles as noted there. And the episodes have been noted to occur but every 10 to 40 minutes and last seconds to minutes, which you can imagine would be very disturbing to your quality of life and your function. The studies that I read reported that radiation is the most common cause of ocular neuromyotonia. And ocular neuromyotonia is a distinct entity from neuromyotonia because neuromyotonia is a peripheral process involving peripheral nerves, most commonly a perineoplastic syndrome and associated with antibodies. The treatment for ocular neuromyotonia resides in stabilizing the membranes and it is typically carbamazepine, phenytoin, gabapentin or diazepam. And it was about 70% of people reported improvement in their symptoms, though it was not common to have complete resolution. It is also possible that if this is consistent and persists through medical therapy that a binocular fusion surgery or microvascular decompression are potential surgical options to be considered. So that is all that I have. Any questions? Thank you, Spencer, for your presentation. We have some excellent presentations today regarding the humanity side of medicine as well as two excellent neurothemology cases. We have time for maybe one or two questions if there's any or any comments that you might have. Dr. Warner has her hand up. Hi, so it was a great case. I think that it was quite a long video which showed that over time, the spasm of the muscle resolved. And I think it was, it's a rare disorder, quite unusual but absolutely characteristic when it occurs. I don't believe it's associated in a perineoplastic with any of the systemic disorders like lymphoma, et cetera, but very much related to tumors in the cavernous sinus radiation. And it has been also described with thyroid eye disease, although that's really unusual. The nice thing about it is it's extremely responsive to treatment. One of the puzzles in this case was when Spencer and Irina were in there trying to do the measurements, things were fluctuating wildly. And when you have that kind of fluctuation in your measurements, I think we all start thinking myosinia, but in this case, not so much. Just a really great case and video. And I hope that Spencer will put that on our core. It'd be great to have that for everybody to be able to see. Yeah, I'd be happy to. And then Ali, terrific job with your presentation. I think we actually had an eight-year-old boy with his second or third attack of a family-plegic migraine, formerly known as about a year ago. Maybe not even that long, that's quite a run. But I think that some people believe that steroids help the pain resolve, but I think that most people believe that it doesn't help the family-plegia itself resolve. I have a patient who's swore by shark fin cartilage or something crazy like that. But the term migraine came about just because of the generally painful nature of this disorder. And it is a migranous kind of headache in many instances, but I think Tom Carlo, who's a neuro-ophthalmologist, gave a terrific, one of those named lectures at AAO about reclassifying it, basically not calling it a family-plegic migraine anymore because so little of it was really characteristic. And then once MRI came along and started to show those enhancing lesions, that really put the final hung the crepe on that as a title. But it's so much more mellifluous and easy to remember. Thank you, Dr. Warner, for those clinical pearls. And I like the word mellifluous. Dr. Jardine, do you have a comment? I saw your comment in the chat box. I can read it out loud as well. I think we can unmute you too. But I'll just read it quickly since you have a minute left. For Ali's patient, I am monitoring the strabismus and amblyopia and the strabismus cranial nerve three motility deficits are unfortunately not improving as much this time around as predicted. Contributing to her amblyopia, she will likely need strabismus surgery. Thank you everyone for attending this Moran Eye Center Grand Rounds. We will see you next time. We'll end a minute early here. Thank you. Thank you. Thank you, everyone. Excellent job, Abigail. That was well done. Thanks, everyone.