In this fast-paced guide, pediatric neurologist Jennifer Yuen, DO, makes sense of the many seizure types and epilepsy syndromes, so that primary care providers can efficiently reach a likely diagnosis and determine which patients need a workup (including when and how to work up a febrile seizure). She offers tips on getting parents to describe seizures in useful ways, the typical appearance and onset age range for various epilepsies, and pearls of wisdom regarding drug use and how to improve medication compliance, especially for teenagers.
And share. So I'm gonna be talking about seizures and hopefully this works, you know, in terms of things that just kind of updating you as well as, um, you know, learning some new things about seizures, so. All right, so, so no disclaimers. And then basically we're gonna talk about seizures, febrile seizures, and the various types of epilepsy that you may see or encounter, and then just other seizure considerations. So what are seizures? Abnormal neuronal activity in the brain. Um, and so basically described it a little short circuit of what's happening in the brain. Um, and that's what the seizures are clinically speaking. So these are other terms that I just kinda found in terms of um what uh patients have said or I've read, you know, convulsions and twitches, tics and absent and absentee seizures like that. Comes up a lot. Um, episodes, attacks, spasm fits, and then the grand mal, petite mall that everybody hears and nobody seems to ever get correct, um, in terms of what they're saying. So whenever you hear these terms, I always say it's great that you're describing Describing them in terms of these, you know, um, using these words, but a really good description in terms of what you're asking your patients to give you is like painting a picture. You want to know all the little bits and pieces of what occurred um during the event that they had. Um, and so different types of seizures. Uh, there's a tonic-clonic, and so, um, everyone hears about the generalized tonic-clonic seizure. That's just kind of big seizure is what they call it. So that's your grand mal seizure is your generalized tonic-clonic seizure. Um, and so basically, it's the tonic phase is the part that's like stiff, and the clonic phase is the part that's moving. So we have the tonic, clonic, um, and that's really what it is. Um, and you can have a focal tonic, clonic as well, it doesn't have to be both sides. Um, and so, um, I will, uh, and why it's important to, to, it's nice that we have these terms that I've listed here, but it's also good to have a, have a description because some parents, some, sometimes you'll hear, um, also generalized tonic-clonic, but it really is just a tonic seizure and I will let you know, I had, I had an ICU doctor tell me a kid was had a generalized tonic-clonic seizure, and I had them describe it to me and the kid was an epistheonist and was herniating. Completely different treatment, completely different urgency, and that's why I I'm OK with using some of these words, but if you're really wanting to give me a better picture of what's happening, the, the smaller words are fine, like stiffening, shaking, jerking, I'm fine with all of that because then I can get a better picture of what's happening with the kid. So tonic is that stiffening and they can have that shaking, right? So you can be stiff and shaking and some people will call that generalized tonic clonic because they're like, well, it's just shaking, but they're really just kind of tense and stiff. Um, and then you have the myo clonic, which is really quick, really rapid jerk of, you know, an extremity really rapid. Um, atonic, um, is loss of tone. Uh, and so, basically what you see in certain kids, you know, parents are really concerned, they're like, my kids having a head drop. And their head is dropping, and that's an atonic seizure, um. And then we have the absence, and I will tell you I have had absence used for all sorts of different types of seizures, including tonic-clonic seizures. It is a specific type of seizure, um, oftentimes involving it's absence epilepsy, and so oftentimes triggered by hyperventilation. Uh, kids are staring off. Sometimes there's eyelid fluttering, sometimes they have like mouth chewing motions, sometimes they got like automatisms of their fingers and they'll kind of move their fingers a little bit, but it really is just kind of a stare off. And then they'll come back. Um, and then a spasm, which are, which a lot of people are like, well, what's the difference between a myoclonic and a spasm? So, hold on one second. So a myo so a myoclonic, I, I said was really quick, correct? A spasm is a lot, um. It's a little bit longer. So, my clonic is here, spasm is you, you have this jerk, but there's a little bit of a linger underneath and particularly concerning when we talk about infantile spasms. Um, and I'll talk about it later, but it is that, that quick movement and that hold, and that's what a spasm is. So, again, it's really important to get a good description. Um, of the event. And no, no need to use these big words, you can, um, but I always have my patients tell me in the, the whatever words they have, they can give me, don't need to impress me with the fact that they know what Grand Mall is. I just want to know what their description of the, um, movement is. All right, so, Focal and generalized seizures. It's really just kinda plain and simple segregation of the different types of seizures we have. Um, we have focal, so basically, one part of the brain has abnormal activity. Um, and so, uh, uh, You have, you know, like your right arm, they got jerking or your leg, um, or just even kind of, you know, head twitching and things like that, so focal, focal, um, seizures generally, um, don't have complete, uh, loss of awareness. Generalized seizure, the whole brain goes all off at once, having abnormal activity. There is loss of consciousness with this type of seizure. Um, and then, uh, you have focal with secondary generalization. So basically, one part of the brain. Starts having it and then it just kind of triggers an avalanche and the entire brain goes. So you might have patients that um have some awareness at the beginning and they're like, I don't know what happened and their family members like, no, they fell to the ground, they were shaking. So that's likely focal with secondary generalization. Important to know these um or important to ask these questions in terms of, you know, like, oh, do you have any idea? Was it, was it one part of your body that started first? Did you feel anything funny beforehand? Because it does, uh, Um, change workup, um, in terms of looking at, um, the brain and see if there's anything structurally wrong. Um, I know we're in pediatrics, but even more important for adults, if it's focal, you, you really have to scan them. Um, and then, um, so I'm gonna go straight into febrile seizures. It's not an epilepsy. So epilepsy is two or more unprovoked, um, seizures, and a febrile seizure is provoked. Um, so, onset from 6 months to 6 years, um, Roughly speaking, there's two different types. There's simple febrile seizures and complex febrile seizures. Simple febrile seizures, they're generalized, less than 15 minutes in length. I know that sounds really long, but it's less than 15 minutes in length, and there's 1 in a 24 hour period. And the fever can be at any time during that kind of 24 hour period of time. Um. So it doesn't have to be right at the, right at the time, you know, they're like, well, they didn't have a fever when EMS came and they came to the ER and like an hour later, they're spiking a temp and it's 40 °C, right? Fair seizure. Um, complex, it means that it's focal. It can be focal, secondary, generalized, and it's greater than 15 minutes, and you have 2 or more in a 24 hour period. The reason to really kind of figure out if it's simple or complex febrile seizure cause it changes your management. So simple febrile seizures, no neurologic workup, reassurance, it may happen again. Again, 6 months to 6 years that it can occur, you can always give them um a rescue medication uh for those because our rescue medicines, talk to you about later. Um, pardon me, sorry. Um Mhm. Here you go. Um, rescue medicines, we like to give them for seizures longer than 3 to 5 minutes, so it's totally OK to give a rescue medication for these, um, febrile seizures. And the complex febrile seizures, particularly if it's focal, really deserves a workup. So EEG and an MRI and then again reassurance. So if, if EEG, EEG is normal, MRI is normal, still reassurance can still occur 6 months to 6 years. Um So if there, if there are kids that are less than 6 months and have a fever with a seizure, you need to do a full workup, you know, subsex workup. Plus other workup for the potential for the kid is having an there's an underlying cause for them to have epilepsy and just because they got sick. It kind of just kind of brought that back up. Um, it just kind of kicked it into gear. And then I grant, if they're over 6 years, and with a, a seizure with or without a fever, needs a workup. I would say if your first febrile seizure is at 5. Probably deserves more of a workup. The peak age of febrile seizures to start at 22 months. So 2 years is the typical time that um a kid would have it. And then again, whenever we talk about febrile seizures, I'm always concerned about the cause of the fever in terms of if they're really young, they need a septic workup, are there any other signs, meningeal signs, things like that, um, important to have. All right. And so here we are to epilepsy. Um, so epilepsy again I said 2 or more unprovoked seizures. Um, and then again, the seizure is a symptom. I always, you know, worried in terms like, oh, the seizure. Well, the seizure was caused by something. Some of the time we don't know, but it doesn't mean that we don't go looking. And so, um, I always tell families, you know, we have two roads that we travel and sometimes they're together and sometimes they kind of merge off and sometimes we kind of travel one more than the other and it's really um the diagnosis and treatment. And so at the very beginning, diagnosis and treatment for epilepsy tend to kind of run parallel. Um, and so, You know, some parents are like, well, I really just want to just know why they have epilepsy but not treat. I'm like, you're having a seizure once every 2 weeks, we're gonna treat you. And then let's talk about doing a workup. So we have to follow both pathways. They're separate, but they kind of um are right next to each other. All right. Types of epilepsy, so, um, I'm gonna talk about generalized epilepsy, common types that you may see in clinic and focal epilepsies, common types that you see in clinic, childhood types, um, ones with unknown etiology and known etiology of focal epilepsy that you, um, may run into, have already run into, um, and hear about. So if you have any questions about or heard of any of those type of epilepsy, you can feel free to ask, um. This presentation I made for um child neurology residency. So I put like the tail end, I have like slides on other like rare types of epilepsy. I don't think we had time to go into that today. So, here we have the primary generalized epilepsies. Um, And the ones that you might see often are childhood absence epilepsy. There's a juvenile absence epilepsy, juvenile myoclonic epilepsy, and then this one you may or may not have seen or heard in terms of generalized epilepsy, febrile seizures plus, which is GEFS plus, but important to know because I talked a lot about febrile seizures because some of these kids end up with febrile, they start off with febrile seizures, and then they have an unprovoked. Seizure, and then we have to start worrying about this, right? So as I said, it doesn't actually become a concern if it, if it's, unless it falls outside of typical febrile seizure category. All right, so absence epilepsy. So there's two, childhood and juvenile. Childhood absence epilepsy, this is, um, parents come to you and like, oh, they're staring. Sometimes the parents actually don't know until they get into school, and they're like, yeah, the teacher's noticing that they're just kind of staring off. Um, typical onset, 5 to 8 years, and the seizures absence. So they're staring off, sometimes they have eyelid fluttering, sometimes they have these mal chewing motions, and they just kind of stare off. And then they'll come back and it's almost like nothing happened. Um, the typical EEG findings, 3 hertz like in wave, um, gold, gold standard treatment is ethosuccimide. Um, and so, um, I, this is, uh, we don't have a whole lot of epilepsy with the gold standards, and this is one of them. Um, and so I always like to, um, treat it with the gold standard. There are other medicines that will work for this in terms of generalized broad spectrum anti-seizure medicines, but ethosuccimide is it's Old standard. So if you um have um seen a patient and I know that we're in a bigger city and I, but I practiced in Arizona, I, I had some pediatricians that were out um on the reservations and things like that and they couldn't come in to see child neuro child neurologist. I said, if you're really convinced and you can, uh this is something that you will, you can actually Provoke in clinic. It kind of stopped a little bit with COVID cause nobody wants anyone breathing, um, hyperventilating around them. But prior to COVID, you would just have them hyperventilate and if you, if you time them for 3 minutes, they will have an absolute seizure. So you can actually trigger one in clinic and then you Have your diagnosis. And I, I've done this before. Again, less since COVID. But I've done it where I'm like, you know what, parents, I'm going to just, you know, have your child lie down. I'm gonna have to hyperventilate for 3 minutes. And if they have a seizure, we have a proven diagnosis. I will still do the workup. We will still get the EEG. I'll do all this, but I will start treatment right now today. Um, so, if you were so inclined, and say you can't get them in to see a child neurologist for a month or two, and you trigger an episode in clinic, you can go ahead and you can call us and we can advise you. But ethosuccinide is the medicine of choice. Um, is, is the medicine of choice. Um, and this is one where, you know, parents were like, oh, this is the one that you can outgrow. So I don't love that term, but it's one that doesn't necessarily continue into adulthood. But if it goes untreated, so I see when people say, well, they'll outgrow it, I don't have to treat my kid. No, this one you need to treat because they are having 50+ seizures a day, and you can't lay down a good foundation of all the education that their teachers are giving them. Um, when you're having 50+ seizures a day. So I tell them if it goes untreated, you are going to notice a definite decrease in their ability to attend, to be able to learn, to comprehend, and their development just kind of goes down. I've had plenty of parents to say, well, you know, the teacher was complaining of staring spells, and they were getting A's and now they're getting D's and F's. And I'm like, OK, this sounds very much like Absence epilepsy. Let's get you, let's get you diagnosed and treated, and sure enough, their grades go right back up. Um, so just an important thing as parents if they say, well, they're outgrowing, I started decide to start them with seizures. No, the brain is still very much developing. Brain isn't developed till the 23, 25, and so if this is starting at 5 years of age, that's 22 decades of brain growth that is affected by absence epilepsy. So the next one is juvenile absence epilepsy. And so this one, a little bit older and onset, um, and they have absence, but they also have generalized tonic-clonic seizures. This one, their EEG findings are a little bit different than the 3 hertz spike in wave. Um, and this one you can use, this one doesn't actually have a gold standard. Now, if, um, if at first we, you know, they have, um, Absence seizures and we start on the ethosuccimide cause like oh let's try it out. But once they have a generalized tonic-clonic seizure, you have to start a different treatment. The ethosuccimide only works on the absences. Sometimes I've had it where I'll put them on the, I'll keep them on the ethosuccimide cause if their absences are completely treated, and then I'll start them on a different medicine for the generalized tonic-clonic seizure. So, um Depakote, Keppra, lamotrigine, those are all good medications for this medic uh for these patients. Typically, these don't outgrow. So most of the time they are um lifelong. And then these are just the type of, this is the EEGs that you would see in absence epilepsies. I like to say absence epilepsy EEGs are, are pretty, and looks a little bit like wallpaper. It's a real nice pattern, you know, so therefore, these are very rhythmic, very rhythmic. This is what their EEG looks like. All right. So other primary generalized epilepsies, you have the juvenile myoclonic epilepsy. So again, happened in adolescence. And as its namesake says, it's a myoclonic. So you have those myoclonic jerks. These are the kids that are, you know, they're 1415, and they, they come in, they come into the ER because they um were sleeping over at a friend's house and then they had a seizure in the morning. And then neurology comes in consults or you guys see them in clinic, and a good question to ask a teenager who may have been, you know, maybe study up late playing video games, what have you. Um, I, hey, have you noticed that when you're like, you know, if you're showering in the morning or you're holding your cereal bowl that suddenly, you know, your, your hand just jerks and you drop. Things it's really kind of, are you, are you kind of a butterfingers in the morning, you're just dropping things and they're like, yeah, that actually may just kind of be that myoclonic jerk that they're like, well, that's happening, coupled with the generalized tonic-clonic seizure, and you have your um suspicion of a juvenile myoclonic epilepsy. Their EEG findings a little bit faster than the absences. So 4, 4.5 hertz poly spike and wave is not as pretty as the um absence EEGs. Um. This, um, treatment, you can use any kind of broad spectrum, um, Anti-seizure medication, Depakote, Keppra, lamotrigine. Um, this one can be lifelong, um, and it is a definite push that I tell families and patients. I'm like alcohol, sleep deprivation, recreational drugs, including marijuana, will make this worse. Um, I always tell them they're like, even no matter what social media tells you, and you can have your marijuana for your epilepsy, this is not the one. I've had patients that I had treated on lamotrigine doing really well. They had breakthrough seizure and guess what they did? I smoked pot. I'm like, well, here you are. What did I tell you? This is what happens. Um, so very much so important to, um, Just kind of educate the patients. I mean, we want to educate that we don't want any of this. Like, you shouldn't drink, you know, uh, we're pediatrics, right? Like, none of this is legal. But definitely, for sure, if you have juvenile myoclonic epilepsy, you can't. And I often preface it like, do you wanna drive? Like, yes, OK, well, then, if you have a seizure, you can't drive for a certain period of time. Um, and so sometimes that's uh a nice little push to kind of help them like over the hump of I need to take my medicine. And then we have guests plus again, I kind of dabbled in that, uh. You can have, um, if they have febrile seizures and suddenly have non-febrile seizures, something to look into the genetic type of epilepsy. Again, broad-spectrum anti-seizure medication. So when I talk about primary generalized epilepsy, I wrote on here specifically gifts plus. There's no carbamazepine, no oxcarbazepine, no phenytoin, any medication or uh leukosamide, anything that is specifically for a focal epilepsy, so channel acting medication, you probably shouldn't use it for any generalized epilepsy cause it will make it worse. Um All right, and so these are some of the EEGs for us. See, again, it's there and it's not as pretty as the absence epilepsy, but that's just kind of what it looks like. Um, focal epilepsy. So we have childhood type of epilepsy that you guys might run into or see or just kinda hear about, um, from patients, and then we have other ones. So, childhood epilepsy. Let me tell you, the International League Against Epilepsy loves to change names. So, What was known before as benign romantic epilepsy. During my period of, of being a resident into my, my first decade of child neurology, it ended up being benign epilepsy or childhood central temple spikes or Becks, and very most recently, 2022, 2021, something like that is now self-limiting epilepsy with central temporal spike selects. So, any of all that is still benign romantic epilepsy is kind of what most people have been trained on and what they hear. And then you have um benign focal epilepsy with occipital uh paroxysms or benign childhood occipital epilepsy or panatopolis syndrome. Um, and then we have other ones based on their, um, locations in the brain, and I'll go over all of them. So here we have selects. So school age to older school age. Um, So in the tweens, um, this is focal. Usually what happens is you get a story that the parents are like, oh, they had a seizure. What happened? Well, I sent them to bed, and I think that they were drifting off to sleep, and then they come into my room, and one part of the face is twitching, and there's slurred speech. And I don't know what happened. And then they, you know, they either stopped or they went to the ground shaking all over. Um, and so that's typically early onset is, uh, or onset is early stages of sleep, and then sometimes when they wake up as well. Um, and the EEG shows central central temple spikes. This, uh, the treatment for is observation versus treatment, 98% of them outgrow it. Now, There's some controversy as to whether or not to treat all of them. Um, before it was, well, if they're having really rare seizures, don't treat and, and some neurologists like, well, their EEG is abnormal, go ahead and treat for two years, um, to just to decrease the chances of learning disabilities, ADHD and things like that, and so. Um, oftentimes it's hard to talk families into starting a medicine after one seizure, but if they have two, I'm more in line of, you know, if you have frequent enough, I'm gonna treat you regardless of whether or not you outgrow this. Um, a little different than absence. Absence, you're having like 50 seizures a day. This one is not. Um, So there's a little bit of uh difference there. So you may notice that you have some patients that have it and some are on treatment, some are not. And this is kind of the thought process for that. But they do outgrow it. And then we have the benign childhood, um, occipital epilepsy or paniatopolos syndrome. Um, this one occurs in younger kids, 5 to 7 years old. And these are the kids that come in, they have vision changes, hallucinations, vomiting, sometimes headaches. So this one is kind of a, a stranger one where, you know, where they'll come in and we're like, well, maybe they have headaches, maybe they have migraines. Um, but if I get, you know, if they're really just You know, episodic and don't last very long, and otherwise it just doesn't sound a whole lot like actual like headaches or migraines. This is something to think about. Are they having occipital epilepsy? Um, EEG shows uh occipital spikes in sleep or when the eyes are closed on the EEG, uh, so treatments, um, uh, anti-seizure medicines that work really well for focal epilepsy. So carbamazepine, oxcarbazepine, um, Glucosamide works fine with us, um. And then we have focal epilepsy by location of the brain, um, so those in the frontal lobe, um, tend to be really short seizures and they cluster in sleep. And these are the ones that if you hear the story, you're like, are they, are, are these really epileptic or these non-epileptic, are these psychogenic or functional type of seizures, uh, because they're like, you're like, they don't sound, they don't sound like seizures. But, um, but these are ones where, um, If able to to capture one on EEG just because it can't because it can be difficult to diagnose unless you capture it on EEG. Um, um, there have been a few times, you know, The time I've been a child neurologist and particularly my 1st, 1st 1 during residency, my attending and I very much convinced that this, that whatever this kid was having was non-epileptic, routine EEG is normal, and the kid was not having enough to put in the epilepsy monitoring unit. But then as they start to happen more, cause I always say epilepsies are going to present itself. It's not going to just See you without any treatment. If it's gonna be an epilepsy, it's going to, it's going to present itself, it's going to continue to happen. Finally got to the point of having enough episodes, captured one, sure enough, frontal lobe, uh, frontal lobe seizure. Um, so, Um, oftentimes it's nice to capture on EEG. And then, um, treatment, I'm gonna say the treatment for a lot of these are the ones I'm gonna talk about next are the, in terms of carbamazepine, oscarbazepine, leucosamide. You can use broad-spectrum anti-seizure medicines with this as well. And we have other options that aren't, uh, pharmacological, so, uh, VNS or an RNS now, we have non-lesional surgery or lesional surgery if you find a lesion that's causing you to have, um, the seizures. Um, temporal lobe seizures, they're repetitive, you have automatism, so lip smacking, rubbing the hands together, deja vu, fear, anxiety, and odd smell, and odd chest sensation, right? So a lot of these things that you hear, and if they only give you one, you're like, oh, you know, it doesn't really sound like a seizure. The odd smell generally is um smelling of burning rubber, and then the taste that they can have is like a copper penny. Um, that they can have. So it's very particular. So if they say, here an odd smell, and you're like, ask them what they smell, and they're like, oh, it's, it smells like, you know, like a pumpkin spice candle. Probably not. Probably not it. But it's, it's, you know, if they tell you like, oh, I smell something funny, and each and every time it's the same thing, then you start thinking, OK, it's a temporal lobe. Doesn't mean that every time it happens, it's a temporal lobe seizure, but it definitely should ring some kind of like alarm bells to be like, oh, maybe you need to see a neurologist, maybe need to do some imaging. Um, again, treatment, um, similar to that of all the other, um, focal epilepsy. Um, all right, so We have parietal lobe. So I, so we have, we had our frontal and our temporal lobe and parietal lobe now. So we have our Jacksonian march. I'm sure that you guys have heard about sensory changes. So the feeling of the sensation marching, so face to, you know, in terms of face to leg, um, they can have vertigo, visual um illusions, uh, distortions of the reals or hallucinations that are not real. Typically, Hallucinations and seizures are not formed hallucinations. So if they tell you, no, I definitely saw a figure and they were standing there and they were, you know, coming at me or if I'm hearing something and they're talking words to me, unlikely to be a seizure is going to be the hallucination, whether it's auditory or visual, um, is going to be less formed, it's distorted, it doesn't look real, um, or sometimes they see colors. Um, just kind of wavy colors, whooshing sounds, um, things like that. So if they tell you that my, the whoever is talking to me was actually I could hear what they were saying, they were calling my name and telling me stuff, probably not a seizure. Um, and then occipital lobe, visual changes, um, you'd have loss of vision, um, illusions, hallucinations, abnormal eye movements, um, and so the same treatment, um, categories with this. And so whenever you're, whenever you're getting a history of seizures or episodes that kids are having. If they are episodic, if they are always the same, start worrying about seizures. Even if it doesn't sound like a seizure, if you're like, huh, every single time this kid is telling me exactly the same thing that's happened 5 times in a row, then start thinking, start thinking, um, epileptic seizure. All right, and then we have our epilepsy syndromes, um, with syndrome, infantile spasms, Lennox-Gasteau syndrome, and Landau Clefner. Not that you're necessarily going to, um, diagnose any of these, but you might come into them and, and, and, and, and see these kids or send into us or when to be concerned, send to us. Um All right, so epilepsy syndrome. So we have West syndrome. So try out of infantile spasms. So remember, I told you it's the spasm is that kind of quick jerk and that kind of like, um, that subtle kind of hold, and then down. So, if they are infancy, so 4 to 6 months. If, if you have an 18 month old, and you're like, oh, they're having spasms. And if they were totally normal before, not infantile spasms. Um, Could be something else, but not typical infantile spasms. Um, so if you see any of them, you know, in terms of flexion spasm or extensor spasms, and sometimes they can cluster, they can cry. And oftentimes, um, in the morning times, and they're of that age. Get concerned, you know, call your local neurologist, send us a video, send us to the ER, um, we can definitely get a workup. Their EEG at the bottom, uh, corner there, it's, it's not very pretty. You've seen some of the other EEGs before. This one is, is, is a, is a kind of a big mess. So, It's very messy, disorganized brains. That's what it is. It's a very disorganized EEG. This little portion here that I'm circling with the mouse, you can see that the part that looks like it's not disorganized, kid had a seizure. So it's an electrodecrement. That's their, um, the EEG finding. So when they have an electrodecrement where the brain actually looks like it's organized, is actually when they have the seizure. So the brain just kind of shuts off, has a seizure, and it goes back to being disorganized. Um. So that is included with the sweat syndrome, hips arrhythmia is the name of the EEG abnormalities, and they have developmental regression or delays. Um, typically 4 to 6 months is when I see it, um, but, you know, 3 to 12 months of age, uh. Usually younger in infancy. Um, and then the ideology, the cause of it is there's many different types of causes and sometimes it becomes unknown, it remains unknown. Um, treatment for them, ACTH, that's like gold standard. It's so expensive and not everybody has it. And sometimes parents don't want to give their kids injections. So high dose prednisone it is. Um, and then vigabatrin and there's other anti-seizure medicines that other people use, but steroids is where it's at. So we have just kind of immunosuppressants is really what, um, what we start them with while looking for the cause. And um there's some kids whose infantile spasms. Um, end up turning into Lenax-Gasteau syndrome. So sometimes they can just kind of, it's on a spectrum. So we have, like in neonates, this Otahara syndrome that turns into West syndrome, that turns into Lanax-Gasteau. So there's a big spectrum of where these kids end up. Some of them do. Some of the kids just kind of pop in here and there, but a lot of kids, they'll have this whole spectrum that they've, they've written and the name changes because of their age, and the type of seizures. So, Lennox-Castello syndrome, you have multiple different types of seizures that are difficult to control. And so, they can have generalized tonic-clonic seizures, they get myoclonic seizures, tonic seizures, and the infamous atonic seizures. So if you've got a kid with multiple different types of seizures, they've got developmental disabilities, and suddenly they have atonic seizures, you probably, you know, in terms of Lennox-Casteau is probably what they have. Um. EEG findings, it is pretty. It's still like the absauces because, but it is slower, it's 2 2.5 hertz, um. And this is kind of younger, 3 to 5 years of age. Um, again, various causes, most, um, unknown, but now we have a lot more genetic testing and genetic type of epilepsy that we run genetic panels, so we're finding out more, but there's still a vast number that are unknown or uh What they call it cryptogenic idiopathic, um, type epilepsies, and then again, patients with infantile spasms can develop into Lanox Gaste. And then this one just various different types of medications for generalized epilepsies, we just kinda broad spectrum, hope, hope for the best, um, in terms of um some of these medicines. There are some medicines that have been studied that uh people use specifically for Lanox-Gaste syndrome, um, like Clobezan or Ompi. Um, refinamide, um, forgot the brand name for refinamide, but refinamide is one of them, um, selbamate, that's an old one. I, I use that early on in my career and I don't use it very much anymore, but that used to be a Lennis Gasteau um medication. Um, so you might see these patients in your clinic. And then the next one, so Landau-Claffner syndrome, rarer. Um, but something to think about when you see kids and suddenly they have speech communication difficulties, um, and comprehension can comprehension can affect their expressive language, regression and development. In a kid that's 3 to 7, you start thinking, oh, is it autism, right? But, If, if really the only thing is the speech and you have this regression, and they're typically a little bit on the older side than a kid with autism with regression, think about this, um, so it's an acquired epileptic aphasia. So, 202022, I said that it changes a lot, the International League Against Epilepsy, which I really think is like the coolest name for Uh, a group of people, of doctors, um, they recommend that Landau-Lffner is a subtype of a developmental epileptic encephalopathy with spike wave activation and sleep, so you can have, um, electrical status epileptic consistently or just continuous spike and slow wave during. Sleep, slow wave sleep. So you don't even have to have a seizure occur during this. You just grab an EEG, hope they, uh, you know, get it, get a 24 hour capture sleep and if you have this with the clinical diag with the clinical picture, you have your diagnosis. Um. And so the EG can be normal during awake, so you want sleep. Um, and again, this one, you can various uh anti-seizure medicines, immunosuppressants, uh, maybe it will help um with this including an IVIG can help, um, surgery, VNS RNS, those type of things, uh, that we have. And so this is kind of the EEG awake EEG, this awake EEG is not normal. But in here, we have our continuous, uh, slow wave, continuous spike in slow wave sleep. Here. All right, so other concedure considerations, I'm watching the time. Um, status epilepticus. So this one, status epilepticus. Definition changes a little bit over, you know, over the, over time. So it's greater than 5 minutes of continuous clinical and or electrographic seizure activity. You can also have multiple seizures that cluster without regaining consciousness for longer than 5 minutes. So if If you guys had, you know, in terms of learned about seizures a while ago, it used to be 3 seizures in 30 minutes without regaining consciousness, we've changed this. And it can be uh convulsive, so one that you can see in terms of clinical or non-convulsive, so. Um, oftentimes if, uh, kids say they, they come into the ER and they're like, oh no, they have generalized tonic chronic seizure, and, you know, suddenly it stops, but the kid is hunted, they're not responding, they're not, you know, they're this, it's just like, oftentimes like, well, are we now concerned that they're having non-compulsive status, you know, versus um being postsickle. Um, so, EEG can be helpful in that standpoint and then again, medical emergency that lasts longer than 5 minutes, it's likely to continue if not treated. So this is one where um we give our rescue medicines, um, you know, seizure over 3 to 5 minutes. Most seizures are under 5, the majority of those are under 2 minutes long, um, although it seems like forever, um, when we see them, um. That, um, I always tell parents, I'm like, it's not, you know, give you seizure medicine to stop it. It's not because suddenly, your brain is going to be damaged or something bad's gonna happen. I'm like, if it's gonna last longer than 5 minutes, it may actually last, it may continue, and then you're gonna pile on. Even more medications and oftentimes, the, the reason why a kid needs to be intubated or given oxygen in the ER for status epileptic is because us, uh, right, we give them Ativan, we give them, we give them, you know, Keppra, Depakote, phenobarb, what have you. Um, all of that is what's gonna cause the apnea, and we're gonna protect their airways. So we're gonna intubate them. Um, and so I always tell parents, it's not that something bad's gonna happen. It's just that if I can get it stopped early, I don't have to pile on more medications. Um, and so So that kind of helps them realize like, OK, all right. So that's why I want to give my kids medicine. Honestly, with the with the rescue medicine, I'm like, you know what, if you're freaking out and it's only been a minute and they continue to have the seizure, go ahead and give it to them. It's not gonna hurt, right? It's shallow breathing, they're gonna be tired. But if it stops the seizure from continuing, go ahead. I'm, I'm not stopping you. Um, but again, if you're a parent with a kid, if you have a parent with a kid who has seizures, um, you know, daily. They might not want to use a benzo every day for it, you know, say they're on 4 medicines and they have a VNS and this is kind of their life now. I say, you know what, that's, you may not want to use the benzo every day. So maybe do keep time um for these seizures or however many clusters. Um. So side effects of seizures, we have um short term, we have post, the postictal phase, or post-seizure phase, tired, lethargic, grumpy, aggressive, confused, dazed. Some don't even have a postictal phase. Um, many have no side effects in the long term. Um, you can have, um, I didn't write it on here, you can have weakness. So Todd's paralysis after a focal seizure, um, so weakness on one side that resolves after a couple of hours. Um, if, if all that is, you know, they come into the ER and say parents don't, they're like, oh, maybe they had a seizure and then they have weakness. And of course, you know, they're like, oh, is it a stroke and things like that. But sometimes and then it's like, oh, no, just resolved after an hour. It can be Todd's paralysis, and that's can be a post sick phase. Um, and then as I said before, frequent seizures in young developing brain can cause learning and memory issues. So there are higher rates of ADHD and learning disorders. One of the gold standards for kids with epilepsy is to see neuropsychology, but As you very well know, that's hard to get into, to see neuropsychologists or if they, or it can be costly if they go outside, um, you know, in terms of self-pay. But if, if this were, you know, the gold standard world, I would love it for every patient with epilepsy to see a neuropsychologist, um, routinely, you know, at the very beginning of their epilepsy and then on routine and throughout however long, um, they will be seen. Um, and then prolonged seizures can cause brain cell death. Um, and so just because of the, the brain cells just tire out, so when they tire out, They quit working and if they can't recover, if they don't get enough rest and recover, they'll die. And, um, and brain cells don't regenerate. And so, but this is usually hours long. That you start seeing kind of brain cell death. Sudep, sudden unexplained death and epilepsy. So kids, kids or adults, people with a history of epilepsy, it's sudden death, it's unexpected, unexplained despite investigative um efforts including autopsy. Um, risk factors include, um, those with generalized tonic-clonic seizures. Um, if you've had 3 or more generalized tonic-clonic seizures in a year, your risk goes up. Uh, frequent uncontrolled seizures, uh, patients who are on multiple medications, those that are non-compliant with their medications. I have unfortunately had Two of my patients um ended up being sued up and both of them decided not to take their medications. Um, and so that's, it's really unfortunate, um, for it. Um. And then, um, the younger they are at onset, um, can increase the risk, um, and also many years living with epilepsy. Now, the rate of pseudap is actually higher in adults than it is in kids, but the younger they are at onset does increase the risk of it. And then of course, risk factors include etiology. If you have a genetic cause of, of epilepsy, you're at higher risk, um, for it. And then seizure threshold, um, we all have a threshold, um, we could all potentially actually have a seizure, um. There are things that provoke, uh, like illness, dehydration, low blood sugar, even high blood sugars can cause it, um, changes in electrolytes above or below normal, changes in blood pressure, stress, medications, drugs. Um, and then patients with epilepsy are even at higher risk, um, for a seizure becau uh due to any of these things. So, when, you know, patients like I had a breakthrough seizure, are you ill? Did you miss medications? Those are, you know, some of the things that we ask because those are really common for them to have a breakthrough seizure due to. And then medication compliance, important to discuss with um our patients and their families, important as the medication compliance. So missing doses cause increased seizure risk. Um, changing the doses without physician approval can have consequences. And then teenagers, teenage population compliance can be as low as 50 to 75%. And then, of course, I've had it many times. I'm like, well, who gives them the medicine? Well, you know, my, my kid is 12 and I want them to take responsibility. So I'm letting them. Take care of their medication. I'm like, that's OK for you to have them draw their medicine or take them out of the bottle, but you should be watching them, and you should be there, um, being there because their brain is not mature, right? As I said earlier, 223 to 25 is when their frontal lobes are completely mature. So at 12, not a whole lot of mature. I think your auditory, auditory centers are mature by that time. Uh, now, whether or not they, they choose to actually listen to what you're saying, it's different cause they're teenagers. But again, the frontal lobes are not mature at that time. So, um, definitely, I'm reminding our parents, I'm sure that you guys do with all the other medications, and you pre uh prescribed that parents should be the ones monitoring their medication compliance. Seizure first aid. Um, so you want to lay, you know, just to kind of reiterate some of the things to tell parents, uh, lay the child on their side, any side doesn't really matter. It's just to allow, um, anything coming out of their mouth to come out and not into blocking their airway. Um, and then don't stick anything in their mouth and just introduces objects in the airway. And many times our parents be like, oh, I have a band-aid, you stuck your finger in their mouth. Yes. I'm like, you won't do it again? No. So lesson learned. Um, and then rescue medicines, intranasal Versed, um, Um, Diastats, um, oral Ativan, clonazepam wafers, rescue medicines, you know, parents, I was like, well, which one works the best? Whichever one you're going to be comfortable giving is going to work the best. As much as, you know, some parents will like, you know, be like, well, maybe, you know, intranasal versed is quicker. But if they're like, well, I don't, I don't really love to shove anything up their nose, and I'm like, then it's not gonna work. You know, if, if parents are concerned that they're sticking their fingers um in their mouth, granted, when the clonazepam wafer goes in, I would say put it in the cheek, don't go past the teeth. But some people, you know, of course if they, they're like, oh, I don't really want to put anything in their, their cheek or I don't want to give a suppository, then it's not going to work no matter. So all of these are going to work. Um, relatively the same, whichever one you're gonna be comfortable giving is the one that you, that, that I will prescribe to you, done and done. Um, and then, of course, calling EMS, um, sending to the ER.
