Advocacy and Awareness Efforts in the Headache Community

Cranial neuralgias comprise a distinct set of disorders typified by short-lasting attacks of intense pain in the distribution of a particular nerve in the cranium. Cranial neuralgia syndromes are rare but can be debilitating and go undiagnosed or misdiagnosed for years.

In this episode, Lyell Jones, MD, FAAN, speaks with Stephanie J. Nahas, MD, MSEd, FAAN, MD, an author of the article “Cranial Neuralgias,” in the Continuum® April 2024 Headache issue.

Dr. Jones is the editor-in-chief of Continuum: Lifelong Learning in Neurology® and is a professor of neurology at Mayo Clinic in Rochester, Minnesota.

Dr. Nahas is an associate professor of neurology at Thomas Jefferson University and assistant director of the Headache Medicine Fellowship Program at Jefferson Headache Center in Philadelphia, Pennsylvania.

Additional Resources

Read the article: Cranial Neuralgias

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Host: @ LyellJ

Guest: @stephanieJnahas

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 Jones: This is Dr. Lyell Jones, Editor-in-Chief of Continuum: Lifelong Learning in Neurology. Today, I'm interviewing Dr. Stephanie Nahas, who has recently authored an article on cranial neuralgias in the latest issue of Continuum on headache. Dr. Nahas is a neurologist at Thomas Jefferson University where she is an Associate Professor of Neurology and serves as Assistant Program Director of the Headache Fellowship program there. Dr. Nahas, welcome, and thank you for joining us today.

Dr Nahas: Thanks for having me. Glad to be here.

Dr Jones: So, for our listeners who are new to Continuum, Continuum is a journal dedicated to helping clinicians deliver the highest possible quality neurologic care to their patients, and we do so with high quality and current clinical reviews. Dr. Nahas, your article is a perfect example of that - it's full of really helpful (and I think clinically relevant) recommendations for neurologists who take care of patients with cranial neuralgias. And now that at this moment (during this podcast interview), you have the attention of a huge audience of neurologists - what's the one most important practice change that you would like to see in the care of these patients? 

Dr Nahas: I would like to see the recognition of these cranial neuralgias and related syndromes as distinct and overlapping with other primary headaches much more often. I think far too often, clinicians will try to pigeonhole these headache and facial pain diagnoses and try to make just one diagnosis the main one, and any other symptomatology that comes along with it – “Oh, that's just a weird part of your primary syndrome, right?” I know I've fallen into this trap a number of times, because mostly what we see in a headache clinic is going to be migraine, so we kind of have a laser focus towards migraine-type symptoms (and we know migraine can do just about anything). So then when we hear a little bit about a facial pain, a little bit about some sort of neuralgia, we just try to wrap it up into migraine - but that's not always necessarily the case. You know, we know that any person on the planet can have as many diseases as they darn well please, so why not ascribe two diagnoses when it's appropriate? That can lead to better treatment outcomes, in fact. If you are focusing your treatment on two distinct, but overlapping, entities, you tend to get better results, because the treatments may not be identical (and they rarely are).

Dr Jones: And that's a great example of it's Occam's razor on one side (there's one problem) versus - what is it, Hickam’s Dictum?

Dr Nahas: Something like that.

Dr Jones:  - where you can have as many problems as the patient wants to have, so I think that's a great example of that. And, earlier, in the same issue on headache, we do have a wonderful article by Dr. Deb Friedman, who walks through that really important history component of trying to, you know, determine which headache syndrome the patient is dealing with (which is obviously a prerequisite for the diagnosis and management) - so that's a great point. So that's the one takeaway - recognition of cranial neuralgias as a distinct entity. Keep it in mind – otherwise, we'll miss it. Is that right?

Dr Nahas: You got it.

Dr Jones: Okay, good. If we learn nothing else, we'll take that away. So, speaking of the history, Dr. Nahas, for many pain syndromes (including these), the history is really paramount in establishing the diagnosis for patients, specifically with trigeminal neuralgia. How do they usually describe that pain to you? 

Dr Nahas: The whole spectrum of descriptors for trigeminal neuralgia-form pain is, actually, maybe broader than you would think, and I actually find that, sometimes, patients have a real hard time verbalizing and describing the way it feels, because it's so unusual - it doesn't remind them of anything they've necessarily felt before.  Sometimes, it can. For example, a patient who's no stranger to having lots of dental work - that pain that when they drill in or if they hit an irritated part of the tooth or the gums, that's usually kind of neuralgia form-like. But at the same time, patients will say, “It's still not quite like that. You know, it's really hard for me to explain. It's sharp and it's terrible like that, but it has a different quality.” And I think they just don't necessarily have the terminology, but I encourage them to try to be creative. You know, some of my patients will personify the pain - they'll describe as if there's some little creature in there that's clawing, or scraping, or pulling, or stabbing. Or they might use other descriptors, such as burning like a fire (like a blow torch is there). Or they may even use colors. You know, some of my patients are really creative, and I don't know if they actually have synesthesia or they're just bordering on that, but they'll describe different colors for the qualities of pain. (“Is it more red? Is it more like icy blue? Is it black or white?”) I don't hear that too often, but I do like to just open the door and let my patients describe for themselves in their own words - and if they can't have any words, I give them some examples and that usually gets the ball rolling.

Dr Jones: So, a combination (like we usually do) with some open-ended questions, and then some directed ones to kind of clarify. That's really interesting, and it gives you some immediate empathy and sympathy for the discomfort these patients have to deal with, right (as when they describe it in those burning, clawing kind of terms)?

Dr Nahas: Exactly, and they'll also put it into context for you - so not just describing what the quality of the pain is like, but they'll give you good examples of when they feel these symptoms, what brings them on, what alleviates them, how the symptoms may change from day to day depending on the situation or circumstance. And again, it just gives them an open door to express themselves, and it really does help to strengthen that alliance you're trying to create and maintain with your patient. You do get useful and valuable information when you just let them go on and describe things.

Dr Jones: So, there are, I think, misconceptions in the popular world and also in the clinical side of care that, you know, folks will have a perception of a disorder that maybe doesn't really match reality. What do you think is a common misconception you've encountered in taking care of patients with cranial neuralgias? 

Dr Nahas: The patients that I see tend not to have the clear-cut textbook descriptions  (like it's almost as if they're reading the criteria when they tell you your symptomatology) - because those cases are a little bit easier, they get identified more readily, they get appropriate treatment sooner, their disease doesn't necessarily progress and become complicated by, you know, any number of things that can happen with unmanaged neuralgia-form craniofacial pain. The ones that I see - they've been around the block several times, because maybe their syndrome isn't quite so typical. Maybe they didn't really have the terminology to be able to describe their symptoms. Maybe nobody really opened that door for them and invited them to just talk about what it is. Perhaps they, or whoever they were seeing, were more focused on diagnostic testing, and so their focus is more on, “Why is my MRI not showing anything? Why is my x-ray completely normal? You know, I have these symptoms. There must be an explanation.” Because that's what patients want - they want solutions. They have a problem, they want to know why they have it, and they want a solution to it. And they can get too focused on the hard data and ignore that it's a subjective experience that really guides us to help treat their symptoms, especially when we don't have necessarily an anatomic target to go after. (When we do, that's great.) But again, these straightforward cases tend not to come to me, because they’re easier to take care of.

Dr Jones: Still, just as legitimate a diagnosis, even with a normal MRI, right? I do find it's sometimes hard to kind of get around that with a patient, isn't it?

Dr Nahas: Absolutely, it is. You know, they're both relieved and disappointed. I often find if we order imaging for an unusual syndrome (or even a typical syndrome) and they see that, “Well, there's nothing on this report to go for. What does that mean? Does that mean that I'm crazy? Does it mean that this is all in my head, that I'm imagining it, that I'm amplifying my symptoms somehow? Is this my fault?” You know, all this self-doubt comes in, and you have to reassure these patients that, “Yes, your symptoms are real. They are in your head, because your brain is in your head, and your brain is the source of your perception and your experience. So, let's take your symptoms at face value and let's give you treatments that are directed at those symptoms.”

Dr Jones: Well said, and that's where we like to keep it, the brain inside the head. I think that was day one of neuroanatomy. I know that the treatment for many of these cranial neuralgias overlaps, right? There's some common approaches to several of these. There are some things that we put in our academic writing, but there are some things that we just kind of learn from experience. Do you have any tips or tricks that you would like to share with our listeners about the management of the cranial neuralgias? 

Dr Nahas: First and foremost (and I think this kind of goes for any of the disorders in the spectrum of headache and facial pain) is you need to be patient, and you need to set up appropriate expectations that, by and large, this is a trial-and-error process where we need to introduce a therapeutic intervention gradually and titrate the dose gently to effect while following for clinical response, but also keeping an eye on what our guardrails are. What do I mean by that? Let’s say, for example, we’re using oxcarbazepine for some sort of neuralgia-form disorder (I mean, take your pick for any of them – it’s fair game for most of these as a good initial trial).

Dr Jones: Sure. Yeah.

Dr Nahas: So, you want to start it at a low dose, start building it up slowly, and in addition to following for their clinical response - which I counsel them it may take a while  (even once we hit a target dose, it may take several more weeks, we've got to give it time) - you can monitor a serum level of oxcarbazepine and certain other antiseizure medicines for that matter. So, that can help guide you to know how high you can go. This is a little bit different from the situation with epilepsy, where you're checking levels to ensure that it's in a therapeutic range to make sure that it's not toxic - maybe to assess for adherence - but here, we're using it as a guide to know how much farther can we push the dose on this drug. And, of course, also, you want to be monitoring for any adverse events that can occur with that drug (such as hyponatremia, or changes in the CBC, et cetera) - so I do monitor these folks a little bit more closely than I otherwise ordinarily would, especially when I have a therapeutic intervention where I can actually monitor the drug level of it and be very, very precise in trying to maximize and optimize their treatment.

Dr Jones: Got it. So, patience with each trial, and then patience that there might be (and I mean patience with a ‘c’ that there might be) multiple trials – I think that's a good takeaway for all of these cranial neuralgias with pretty much all of the medication treatments, right?

Dr Nahas: Yes, and I do find that in some cases, one treatment is not quite enough. Because most of the treatments we draw from our antiseizure medication category, it can get complex trying to balance two, or even three, antiseizure medicines and finding the optimal dose for each. Do we push all of them to the max? Do we say this one is the undercurrent (we just want to keep it at a low level) and these other two are going to be doing the lion's share of the work? It becomes kind of fun if you like uncertainty and if you like to be creative. If you're the type of person who likes checkboxes and checklists and cut and dried results, you know this is not the game that you want to play - but that's one of the reasons that I enjoy doing this, because I have so much freedom to be creative and really finely tailor and tune the treatment specifically to the individual patient’s needs.

Dr Jones: That's fantastic, and in a minute, I think we can come back to maybe what drew you to this - I'm curious to hear that. But before we get to that, you know, when we think about the medications that are available (and again, your article does a phenomenal job summarizing the therapeutic approaches to the cranial neuralgias) - what do you see on the horizon, Dr. Nahas, for the care of these patients? 

Dr Nahas: I want to see a lot more research being done in this population of patients and across this spectrum of disorders. What makes it so hard is because they are somewhat rare, and because they very often co-occur with another primary headache disorder - so that makes it extraordinarily difficult to create a research study on a population that's so heterogeneous, right? That's, I think, the biggest challenge - is that we have so little to guide us other than our own clinical experience. There are not a ton of clinical trials for any of these disorders. I think one in particular that can be both underdiagnosed and overdiagnosed is occipital neuralgia - and I mentioned before that I, myself, have found myself falling into this trap of once I see a signal for migraine, I just call everything migraine, right? And, sure, with migraine, there can be allodynia in the scalp, and oh, sure, we all hear that if you push on something sore, you can have some lancinating pain. Oh, that occipital neuralgia that somebody told you about? No, no, that's just part of your migraine. You don't actually have occipital neuralgia. Well, you know, if you look at clinic-based studies (there's one in particular that I cited), most of the presentations of occipital neuralgia actually co-occurred with another headache diagnosis (either primary or secondary), and very commonly, it was migraine or probable migraine or chronic migraine. And why this is important is because you need to validate for these patients that they do have more than just migraine. They have a separate problem that, yes, it's interrelated, it's interconnected, they can influence each other - but we might have to treat them both differently. So, you have your suite of migraine treatments which might not include an antiseizure medication. Then, for the occipital neuralgia, maybe you are pulling in an antiseizure medication, or maybe you're focusing more on peripheral nerve blockade or physical therapy - or even considering a surgical referral, because as surgical treatments for nerve decompression or ablation or other interventional procedures also continue to evolve, that helps to give us some more hope in giving  these patients more relief with fewer complications. I'd also like to see some more creative solutions, not just more antiseizure medicines, not just more targeted anatomic interventions. But, hey, is there a role for some other peptides or neurotransmitters that we just haven't identified yet? Might some novel treatment approaches actually be useful for some of these patients? And, you know, again, how do we get at those answers? It's going to be challenging, because the patients - while they're out there, they're not really a homogeneous group, and the results from a particular study might not be so generalizable.

Dr Jones: And we've seen such great success in the world of migraine, right (looking for novel targets) And so it would be nice to transport that over to the cranial neuralgias, right?

Dr Nahas: Yes, absolutely.

Dr Jones: Yeah. We should always be mindful of disparities in care of patients who have neurological problems. Are you aware of any literature around the care of these patients related to health care disparities that our listeners should be aware of?

Dr Nahas: Nothing focused specifically on disparities in this population or subpopulations within this population (based, for example, on ethnicity, or race, or socioeconomic status). You're looking for subpopulations within a huge population, almost like a needle in a haystack - not quite that difficult, but again, it takes a lot of effort and diligence to try to find these individuals and then to get them to agree to enroll in some sort of research study, even if it's just a survey study or doing interviews with them trying to understand their symptomatology better. It can be quite challenging. And then again, let alone designing a rigorous clinical trial for these folks - who, again, such a heterogeneous presentation - and the willingness to participate in a placebo-controlled trial for pain that can be so heinous can be very, very challenging. You know, we've seen this as a challenge with cluster headache, too - not just because of the nature of the disease (when the cycles come and go somewhat unpredictably). But these folks aren't necessarily willing to forgo treatment for the purposes of a clinical trial - I mean, many are, and I thank them - this is another one of the reasons that research is really lacking in some of these rarer syndromes.

Dr Jones: So, another part of the rationale for more investigation for these uncommon and probably underserved disorders. So, Dr Nahas, I know caring  for patients with craniofacial pain, I imagine it can be challenging. I can imagine it's also pretty rewarding as well. What drew you to this work, and what do you find most exciting about it? 

Dr Nahas: Well, what brought me to headache to begin with was kind of random chance, and really, it revolves around mentorship. When I very first started as a neurology resident, Dr. Silberstein took me under his wing and wanted to turn me into a headache specialist (that was one of his goals). And, thankfully, he was successful, although he didn't really have an easy job of it, because back then, I didn't really see or understand how studying headache and facial pain could really satisfy that hunger that I have to understand the brain and the nervous system. I mean, that's why I became a neurologist in the first place, right? (I think that's why most of us did.) You know, not only are we drawn to medicine to help people and be altruistic and to study a fascinating topic, but particularly with the brain and the nervous system - I mean, this is what makes us human. This is what's so fascinating to me. And until I started to learn more about headache, I thought the best way to really learn about brain function is through disease (such as stroke or epilepsy, or movement disorders, cognitive disorders, degenerative disorders). This is how we learn, right? This is what I was taught, at least in college and med school. And then you get to the real world of actually practicing medicine or being in training. You start talking with these folks, and you hear their stories and how distinct they are from the textbooks. And again, when you invite them to really describe their experience, you see the human side of it, and you listen to them describe their symptoms - and you start to imagine yourself, what's really going on in their brain and their nervous system for them to experience that? So you start reading a lot of the literature about cortical spreading depolarization and how that can activate the trigeminal system and sensitize it - how that might be linked to the expression of aura (for example) - then, you can actually really parse out the anatomy and understand why somebody experiences those symptoms when you understand the anatomy. And there are just countless examples of this - about how studying the symptoms and what brings them about, what the pathophysiology is, and then what the treatment is, how that really informs our understanding of how the brain functions - that's really what's kept me excited about this. That, and again, forming relationships with patients and sometimes being the first person who ever just sat down and listened to them and let them talk, and they really feel like they're cared about and like they're important - because they are. I think far too often, patients with headache and facial pain disorders are stigmatized, and they're left feeling like it's not worth it trying to get better, that there is no solution. Society has beat them down, the medical system has let them down, and they just want to give up. Then, when we can finally sit and listen and give them some hope, and they see some improvement - the transformation that occurs right before your eyes is extraordinarily gratifying.

Dr Jones: So, it's fascinating, and you can help people - and I can't think of a better advertisement for headache fellowship for all those neurology trainees out there.  Well said, Dr Nahas. So I've got one more question for you before we close. And I know that the headache community, including yourself, are very strong advocates for your patients and for more research (as we've talked about today) into headache disorders, understanding the pathophysiology, developing better treatments. What is it about purple hair? I've seen several headache specialists (and maybe someone on this call) post online some purple hair. What's the story behind that?

Dr Nahas: A number of years ago, as part of advocacy efforts, we recognized there's got to be a way to really improve the awareness of such a common condition, of headache in general. It affects so many people, it almost becomes, again, brushed off. We say headache, it's just a nuisance. Well, no it's not. It's actually fascinating as part of the human condition. One of the things we needed was a color - our signature color - and we chose purple. We know that we share this color with other advocacy groups, but it's a great color, it's eye-catching, and you can utilize it in a number of different ways. One of the early ways was people dressing up in all kinds of purple garb - putting purple makeup on, purple sunglasses, purple tutus, purple T-shirts, and even purple wigs. A lot of us have been donning purple wigs for advocacy and for awareness efforts, particularly for events (such as Miles for Migraine, for example) - but some of us have been so bold as to not just put on a purple wig, but to actually go to a salon, bleach the hair, and dye it bright purple. I have at least one male colleague who also did this to his beard. Last year, we did it together at the same salon, took a bunch of pictures to post about. It really created a big splash online and for our social media efforts and outreach, and it caught on. Lots more people now are thinking about dying their hair purple. One of our current fellows actually did it this year. At our center, we have about 30 different purple wigs that we bought with some funds that we procured, and on the Shades for Migraine Day (June 21), we all went out parading around Center City, Philadelphia wearing our purple T-shirts and our purple wigs, and handing out flyers trying to raise awareness. We got a lot of strange looks, but we also got a lot of good feedback. And I think we actually reached some people who didn't realize that there's such a thing as a headache center that they could actually come and see us and get relief for this problem they thought was just a part of everyday life. That was kind of a long-winded answer, but -

Dr Jones: No, that's great, and it worked. It got me to ask you about it, right? And I will say I admire your commitment and dedication. The best I could do today, Dr Nahas, was wear a purple tie, but I'm sure your patients appreciate that level of investment, too. It’s really, really cool. Really impressive.

Dr Nahas: Yeah. A lot of them this past year have asked me, “Where's the purple hair? I thought you were going to do it every year around this time.” And, you know, it is a bit of a commitment.

Dr Jones: It's a commitment, yeah.

Dr Nahas: And there's some upkeep that is required and you're kind of stuck with it for a while (unless you want to go to the trouble of reversing the process, but that's really just covering it up). I said, "We've moved beyond dying the hair. We're doing wigs, and we're thinking of the next thing.” 

Dr Jones: Good for you. Dr Nahas, thank you so much for joining us, and thank you for such a thorough and fascinating discussion on symptomatic management of cranial neuralgias and such a wonderful article in the latest issue of Continuum.  Really appreciate you being here today.

Dr Nahas: I can't thank you enough. It's been my pleasure.

Dr Jones: Again, we've been speaking with Dr Stephanie Nahas, author of an article on cranial neuralgias in Continuum's most recent issue on headache. Please check it out, and thank you to our listeners for joining today. 

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