Thank you for joining us for this migraine education series. I am Brittany Mays. I'm a headache specialist at the Vanderbilt University Medical Center. And I also work as a headache specialist in our Headache Center of Excellence at the Nashville VA. And I will be continuing the series with talking about migraine diagnosis and classification criteria. I do not have any disclosures pertinent to this presentation. At the end of this lecture, I hope that you'll be able to differentiate between primary and secondary headache disorders. I hope that you'll be able to understand the current international classification of headache disorders guideline and diagnostic criteria for migraine. And through this presentation, we'll review several subcategories of migraine, which include, but are not limited to, migraine with and without aura and differentiating between episodic versus chronic migraine. So to be able to understand migraine I think it's important to start at the overall headache classification. So we talk about primary headache disorders and secondary headache disorders and how do you differentiate the two? So I like these infographics. I find them quite helpful because with primary headache disorders, this is basically something that's happening on a macro and microscopic level that's contributing to headache. And believe it or not, 90% of clinical headache presentation is attributed to a primary headache disorder versus a secondary headache disorder, something outside or extrinsic is causing the headache itself. So with primary headache disorders, it can be further subcategorized into migraine, which we'll talk about, tension type headache, which is actually the most common primary headache disorder, and then trigeminal autonomic cephalalgias, which are a bucket of primary headache disorders that are characterized by sidelocked headaches with these cranial autonomic features versus secondary headache disorders can be caused by trauma to the head and or neck. So post-traumatic headache, vascular disorders, tumor infection, et cetera. So my colleague highlighted migraine and pathophysiology quite well. So just for review, when my patients ask me, what is migraine? I like to share that this is a complex neurobiological disorder that typically has a three-to-one female-to-male predominance. However, in pediatric headache clinics and pediatric migraine presentation, typically the young boys that are more likely to present with migraine or show symptoms of migraine, and then around puberty and menarche, there's that shift to the female predominance. With migraine, there is a strong genetic predisposition that we'll talk about a little bit later. And lifetime prevalence of migraine, there's a bell-shaped curve, but for most women, migraine will present itself by age 30 and a little bit later onset for men around age 40. About a third of women will report migraine onset around their first menstrual cycle. So there is potentially that hormonal contribution. And sadly, with us being physiatrists, we always are thinking about the impact of quality of life of these disorders. And migraine is the third leading cause of disability globally in those patients under 50 years old. And as my colleague discussed in detail in the previous lecture, migraine has such a complicated pathophysiology and still pathways that have yet to be identified. But to simplify things for review, there's this neurovascular theory that there's activation of the trigeminal vascular system, there's cortical spreading and depression, central sensitization, and activation of the trigeminal cervical complex. And then as for review as well, migraine is a multiphasic syndrome comprised of prodromal symptoms that occur hours to days before the headache begins. About 30% of patients will present with aura, then the headache piece and the post-trauma resolution or recovery period. Now, just clinical features. How do you differentiate the primary headache disorders altogether? Because there are some overlapping features. So with migraine in general, the duration is four to 72 hours. Patients will share that it feels like a pulsating or a throbbing. It's usually characterized by a unilateral headache presentation that can alternate sides, but sometimes it can be bitemporal, bifrontal, can be homocephalic. In general as well, patients will share that it's moderate to severe in intensity. And it's always important to ask patients about the true headache behavior. So is the patient more restless and agitated or for migraine purposes, they tend to prefer to be in a low stimulus environment away from bright lights and loud noises. And migraine is also categorized by the associated features. So namely that it worsens with physical activity. So most patients will wanna lay down. There's the light sensitivity, which is the photophobia, phonophobia, sound sensitivity, nausea, and then 30% of patients may have or accompanied this. As I mentioned, tension type headache is actually the most common primary headache disorder. So what differentiates tension headache for migraine? So a tension type headache, it can occur, the duration can be hours to days, quite variable. In general, patients will describe kind of a band-like sensation across their whole head or the spice-like pressure sensation. It can be homocephalic, bitemporal, multiple different locations, can be variable as far as intensity. And as far as headache behavior, most patients will say that they can function quote-unquote normally, but if you really pry into this, they may prefer to actually lay down or they would prefer to take over the counters, but they'll try to push through this headache type. And contrary to clinical belief, there can be some associated features with tension type headache, but there's typically only one. So they may have mild light sensitivity, mild sound sensitivity. For me clinically, when thinking through tension type headache versus migraine, if nausea is present, that typically for me tips them over into the bucket of migraine and I'll treat them as such versus tension type headache. Lastly, the trigeminal autonomic cephalalgis, which we refer to as TACS, that's the third type of primary headache disorder. So these are characterized by very short lasting side-locked headaches that are severe. Patients will describe them as sharp, stabbing, ice pick. It's pretty much an abrupt onset and the pain is usually so severe that patients become restless and agitated. They can't lay down because the pain is so severe. And this headache type is also associated with cranial autonomic features that present ipsilaterally to the headache. And these cranial autonomic features include nasal congestion, conjunctival injection, lacrimation, ptosis, rhinorrhea, as well as facial and forehead sweating. I believe this infographic is quite helpful if you're thinking through the primary headache disorders, if you want a cheat sheet to try to remember how to differentiate the three. So again, migraine, more so unilateral or side-locked headache, but can alternate sides. Tension type headache, more of that whole cephalic or band or vice-like quality, and cluster, which is a subcategory of trigeminal autonomic cephalalgias, truly is that strictly side-locked headache, typically affecting the periorbital region with those cranial autonomic features. So we use the International Classification of Headache Disorders, and we're on our third edition, to guide us as far as the diagnostic criteria for migraine. So this pretty much summarizes what we've highlighted in that the diagnostic criteria includes patients must have at least five attacks of headaches lasting four to 72 hours that have at least two of the following four characteristics, that unilateral location, pulsating quality, moderate to severe intensity, and aggravation, or causing avoidance of routine physical activity. And then as we discussed, it has to have associated features, whether that's nausea or photophobia and phonophobia. And it's not better accounted for by another International Classification of Headache Disorder diagnosis. So about 30% of patients will have migraine with aura. And what exactly is aura? And so aura is a phenomenon where there may be some positive symptoms or things that are showing up associated with headache. And so with migraine aura, it can occur with and without headache, and it can evolve in presentation over the patient's lifetime. So if patients present later in life, it can be just aura without headache, and that's possible. So there are three typical types of aura, visual, sensory, and language. And we'll dive into those here now. So with visual aura, these are typically positive symptoms, meaning that things appear in the vision. And we use a visual aura rating scale to try to denote whether it's true aura or not, because aura does carry with it an inherent risk of stroke. It's very, very small in comparison to the general population, but it's present. And so when you're counseling patients about, for example, oral contraceptive use, you wanna make sure you know yes or no if they have aura. And so with visual aura, this rating scale that we use, if the symptoms are slowly progressive in the vision, so correlating with that slow march of cortical spreading and depression going on in the brain, that gives you a little bit higher score. If it's a positive symptom, if it lasts for a certain period of time, the higher the number on that scale, the higher the probability of it being visual aura. So visual aura can be further categorized into the stigotoma, which this little video is showing. It's a positive blurred spot in the vision that's appearing and can slowly progress throughout the vision. So that's a stigotoma. Scintillations or bright lights, flashes of lights can also appear. Patients can look at objects and look away and have these persistent visual after images. Macropsia or mycropsia, where things look either much smaller or much larger than they normally are. Pitopsia, which really are those brighter flashes of light. Teleopsia, where there might be a mosaic appearance to the images. And then metamorphopsia, where there's truly distortions as far as size and angulation of an image. So those are visual auras, which are most, and visual auras most common. Then there's sensory aura, which again, correlates with that slow progressive spreading, cortical spreading depression and happening in the brain during this third phase of migraine. And so with sensory aura, patients will typically present with paresthesias, numbness that affects the face, lips, fingertips, but truly any extremity can be involved. But one thing that should tip you into thinking about aura is that it usually will involve the face and an extremity, and it would be slowly progressive and migrating, lasting for maybe 20 to 30 minutes on average, but it can last the duration of the migraine phase, all of the migraine phases. And then lastly, as far as typical aura, there's language aura, which can present as expressive or receptive dysphagia, paraphagias where patients are truly substituting their words. And again, this can last for about 20 to 40 minutes prior to the headache presenting, or it can last the full duration of the migraine syndrome. Now, there are such things as atypical aura, and atypical aura should always pique your interest that there might be a secondary cause of the aura symptoms or a secondary cause of headache. And so that's why we call it atypical aura. It often mimics something big, bad, ugly that could be happening with the brain. So for example, with motor aura, we also call this hemiplegic migraine. The estimated overall prevalence is very low, so 0.01%. And it's a monogenic subtype of migraine aura, and it can be characterized either by transient hemiparesis that can be mild, or patients can develop quite dense hemiplegia, which of course that would tip you off. Maybe there's a secondary process like stroke happening or a TIA. So we have been able to identify a strong genetic predisposition for hemiplegic migraine and familial hemiplegic migraine. There's this autosomal dominant inheritance pattern, and we've been able to identify mutations affecting several genes. Some are voltage-gated calcium channel mutations, some are the sodium potassium ATPase, as well as the voltage-gated sodium channel mutation. And then more rare includes a sporadic de novo mutation that can cause this presentation. Using the ICHD-3 criteria for atypical motor aura or hemiplegic migraine, patients present with fully reversible motor weakness, and then they typically also present with a typical aura, like visual, the sensory, or the language symptoms. And patients average maybe three attacks of hemiplegic migraine per year, and it usually presents earlier in life. The typical age of onset is around 12 to 17 years of age, especially for familial hemiplegic migraine. For most patients, this truly is self-limited to maybe a few hours, maximum usually 72 hours. But there are those cases where it can persist for up to six weeks, and in those patients, they actually might require an outpatient rehabilitation course, especially if they're presenting with more dense hemiplegia. And as we discussed with that cortical spreading depression happening with the brain that we see with the typical aura, that slow, progressive nature of the symptoms appearing, motor aura is no different, that it's typically more gradual onset, and it's more series of consecutive and spreading symptoms. Another atypical aura presentation is brainstem aura. And so this is categorized by at least two of the following, either dysarthria, vertigo, tinnitus, that double vision, hypoacusis, ataxia, or decreased loss of consciousness. With this specific aura presentation, there are no retinal or motor symptoms. But again, if those symptoms truly are appearing, the true dysarthria, double vision, ataxia, even the decreased loss of consciousness, that should tip you off that this might be a secondary process and that would need adequate workup, and this would truly be a diagnosis of exclusion. The last atypical aura that we'll discuss is retinal. And you probably are wondering, huh, what's the difference between retinal and visual aura? So with retinal aura, this is a monocular visual field defect. So it's only affecting one side. And so this is a quite rare entity with the overall prevalence and incidence unknown, poorly understood at this point. And so the ICHD-3 criteria defines this as an attack-fulfilling migraine aura characterized by a fully reversible, monocular positive or negative visual field phenomenon. And then this is confirmed during attack. So patients may do a visual, the provider may do a visual field examination, and then the patient might actually be able to draw what their defect looks like. And in my clinical experience, true retinal migraine mostly presents as negative symptoms, meaning that there's loss of vision. So there could be that blurring, grayouts, blackouts, causing partial or complete blindness. And if you hear, again, those symptoms, probably as a diagnosis of exclusion, want to make sure that there's not a secondary cause of those symptoms. Also with retinal migraine, it should be slowly progressive, just like with the other auras we've discussed, spreading over five minutes and then lasting maybe five to 60 minutes on average, and then with the headache occurring 60 minutes thereafter. And like we discussed, could be positive, but mostly, again, from my clinical experience, it presents with the monocular visual field defect of negative symptoms. Now, the ICHD-3 criteria for migraine further differentiates migraine into episodic and chronic migraine. And this is really important and will lead into how we approach treatment for migraine later. So episodic migraine is migraine that occurs zero to 14 days per month on average, versus chronic migraine is 15 or more migraine days per month for at least three months. Now, two to 3% of patients per year evolve into chronic migraine, which tends to be a little bit more refractory. And we have been able to identify some risk factors for the development of chronic migraine, such as cutaneous aledinia, about one and a half times more likely, patients with cutaneous aledinia are more likely to develop chronic migraine, as well as a coexisting mood disorder, just such as depression. Patients with uncontrolled depression are two times more likely to evolve into chronic migraine from episodic, which is really problematic. And then there are complications of migraine as well. So there's migrainess infarction where there's actual ischemic changes on imaging, migraine aura triggered seizure, there's persistent aura without infarction. So if the aura symptoms are lasting more than seven days, which again would be a diagnosis of exclusion, we'd wanna work that up further. But the most common complication that you may encounter is status migranosis, which is migraine lasting more than 72 hours. And those can be more difficult, those cycles can be more difficult to treat. I just wanna highlight that at this time, the international classification of headache disorders does not recognize a diagnosis of complex migraine. So when I hear this clinically from referring providers, this is often referring to an aura. And so you just wanna make sure that you're using the nomenclature correctly. So in conclusion, migraine is a primary headache disorder characterized by distinct clinical features. About 30% of patients will present with aura and we discuss typical versus atypical aura. And it's important to remember if you're suspicious of atypical aura, these are diagnoses of exclusion, you need to work them up for a secondary etiology first. And then migraine can be further subcategorized into episodic versus chronic based on the total number of headache days per month, which will guide treatment in the next lecture that we'll discuss. And status migranosis with migraine symptoms lasting greater than 72 hours is a common complication that you may encounter. Thank you for listening. I hope you tune in to the next lecture when we'll be discussing pharmacologic treatment options for migraine.

migraine diagnosis

headache disorders

migraine pathophysiology

quality of life

status migranosus