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Seizures in Kids — Practical Diagnosis, Treatments, and When to Refer · Pediatrics Now: Cases Updates and Discussions for the Busy Pediatric Practitioner

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I'm Holly Weymant, and this is Pediatrics Now, cases, updates,
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and discussions for the busy pediatric practitioner.
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Today, I'm bringing you Grand Rounds. Let's listen in to this talk by pediatric
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neurologist Natasha Varagis.
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It is my great pleasure to introduce our speaker for today, Dr.
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Natasha Varagis, who joined us just this past year.
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Dr. Varagis completed her medical school and child neurology residency at UT
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Southwestern in Dallas. and then she completed her Epilepsy Fellowship at Vanderbilt in Nashville.
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She is board certified in neurology, the specialization in child neurology and
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epilepsy, and her medical interests include complex genetic disorders and syndromes
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and refractory epilepsy.
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So everybody, please welcome Dr. Varagis.
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Morning. Thank you so much for the kind introduction, Dr. Ranch.
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It is definitely a privilege to get to be part of Grand Rounds today.
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Today, I'm going to be talking about childhood epilepsy syndromes,
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and I've targeted the information to a depth and level that will hopefully be
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appropriate and interesting and useful to the pediatrics residents as well as
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the general pediatricians.
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I'm focusing just on the childhood epilepsy syndromes, the ones that present
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between ages like 3 to 15 years or so, because the neonatal and infantile epilepsy
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syndromes certainly deserve their own hour.
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So hopefully this will be interesting and again the goal is to provide the level
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of knowledge that would be useful for a general pediatrician.
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Why should we talk about epilepsy? Well, it's certainly, honestly,
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it is a relatively common condition, certainly not as common as asthma or ADHD.
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But at a large children's hospital, you're certainly seeing epilepsy at least
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once a week between the ER, the floor, or the clinic.
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The Epilepsy Foundation, our chapter, estimates that there are greater than
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160,000 people between adults and children in Central and South Texas.
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There are at least well over 3,000 pediatric patients with epilepsy just in
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the San Antonio metro area and probably an equal or greater number in the Valley region.
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And this matters, of course, not to recap common knowledge, but this matters
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because of the significant morbidity and mortality.
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Almost all seizures certainly do have a negative impact on development,
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cognition, and memory to some extent, some more than others.
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And of course, having paroxysmal, unpredictable lapses of consciousness and
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control of your body be to all kinds of unintended injuries that can go all
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the way to early mortality from droning or suffocation and such.
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So I've organized our talk, this talk as follows,
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talking first about the self-limited epilepsy syndromes, then bringing out the
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focal epilepsies versus the generalized epilepsies, and finally more of the
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developmental encephalopathies or progressive epilepsies.
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And again, if you think, if it feels like I've left out some important syndrome,
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that's because I'm focusing on the ones that present between about ages 3 to 15 years of age.
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We'll start with the self-limited epilepsy syndromes.
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This is actually a patient that I saw in residency. This was a seven-year-old
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little boy, completely healthy, completely normal, very disciplined family.
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They had good sleep hygiene.
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They always went to bed on time until one day when they got a deal on a flight
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to go on their family vacation that left at six in the morning.
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So they get up early. They get their children up at 3.30 in the morning to go catch their flight.
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And then all of a sudden, as they're going through security at five in the morning,
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he has a convulsive seizure for the very first time in his life.
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Parents freak out. And of course, by 6 a.m., they're in your emergency department
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instead of boarding their flight as they had previously planned.
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And when you ask them for details, the parents describe that the left side of
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his face and his mouth first began twitching and they asked him to stop,
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but he wasn't able to talk to them by the time when they tried to interact with
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him, and then he started drooling profusely, and then his whole body developed a convulsion.
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Local paramedics were called, and they came to the emergency department.
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And in your ER, he has a normal head CT, but the EEG shows sleep-activated bilateral
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central temporal spikes with a bifrontal dipole.
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And for some of you who've been studying for boards, you already know what that answer is.
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That little phrase is pathognomonic for a very common childhood syndrome called
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self-limited epilepsy with central temporal spikes, previously known as the
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nine-rolandic epilepsy.
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This is highly testable on the pediatrics boards. It's also something you will
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absolutely see as a general pediatrician.
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Six to seven percent of all childhood epilepsies, that's the number the International
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League Against Epilepsy has estimated in their most recent papers.
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Usually happens in four to 10-year-olds approximately.
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So early elementary school, and it almost universally resolves by puberty.
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Status epilepticus is very uncommon. The head and imaging is normal.
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And this is in patients with normal development other than maybe a little learning disability or ADHD.
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There is possibly, there is kind of a thought that poorly controlled selects
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does worsen learning to some extent.
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So it's, again, it's not completely benign, but it is self-limited.
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And there is no developmental regression. That's one of the big hallmarks of this case.
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The patient's development remains normal as these seizures present.
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Another common, this case illustrates another common childhood epilepsy.
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This would be, and it just is another sort of to start to illustrate how tricky
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seizures can sometimes be and how often they can mimic other conditions.
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This five-year-old boy was seen by his PCP because he had two episodes in the
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middle of the night over the past few weeks of severe abdominal pain that woke him up.
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PCP said he was constipated, but the pain occurred again a few weeks later.
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This time he was vomiting.
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He was very, very pale. The family swears that he had altered mental status.
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He was not acting himself, and this lasted 10 minutes, and then he was sleepy afterwards.
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PCP is still thinking that it's related to the stomach because of the stomach
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pain, but finally after he does a little workup, he reversed to neurology for
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abdominal migraines, but just a couple days before his scheduled neuroappointment,
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the boy has another episode and this time it evolves into eye deviation.
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It's a little bit of tonic-clonic activity at the end and so EMS comes,
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but by the time they come, the event has resolved.
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This is another very common childhood epilepsy that you will likely see at some
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point as a general pediatrician, self-limited epilepsy with autonomic seizure.
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Now, this one is responsible for about 5% of childhood epilepsy under age 14,
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according to the International League of Gas Epilepsy.
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And in fact, it's between ages 3 to 6.
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Again, according to the ILE official papers, it's responsible for about 13% of epilepsies.
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This is another one that typically resolves a couple of years after it presents
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and doesn't tend to have a very high seizure burden.
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These seizures, though, do tend to last a little bit longer and have a very
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long prodrome that can be a little bit confusing for patients when it first
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starts in terms of the diagnosis.
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This one, again, the high-dimaging is normal, but the EEG shows either occipital or multifocal spikes.
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Again, you consider this diagnosis in patients who are developmentally normal.
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The self-limited epilepsy is also called the SELFIES, a very nice little acronym,
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as we highlighted or current otherwise healthy children with normal development,
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not associated with regression, typically resolved by puberty.
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This as a group, as a larger group, is responsible for up to 25% of all the childhood epilepsies.
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This is good news to share with your patients, okay?
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25% of children who present with seizures have one of these syndromes that they
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will grow out of, maybe even by the time that they're a teenager.
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We don't call them benign anymore. That used to be part of the nomenclature
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that in the most recent sort of iteration of the International League of Sun
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Palypsi, in the most recent set of position papers, the word benign was removed
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because seizures are not inherently completely harmless.
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There's still the possibility that you could have one when you're in the bathtub
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and, you know, or you could suffocate on a pillow in the middle of the night, that sort of a thing.
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They're not completely harmless, but self-limited sort of describes their nature
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a little bit more accurately.
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There was a time, again, a couple of decades ago that it was recommended not
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to treat with medication since the seizures itself resolved.
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Now that medications have a lower side effect profile than they did back then,
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it is definitely recommended to treat them for the period of time that the seizures are active.
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Usually, they're treated with a sodium channel blocker, triloptole or Vempat.
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Kepra is also perfectly reasonable. Just pick one that has a low side effect profile.
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Usually, a low dose of a single medication is enough to control the seizures
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for the couple of years that they are active.
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And again, this group of self-limited epilepsies is kind of a class.
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The most common ones are CELETs and CELES.
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There are a few other usually arising from the occipital lobe.
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These also tend to resolve within the first couple of years.
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They're all part of this group these are the self-limited epilepsies.
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Now, those are distinct from the more true focal epilepsies.
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Those epilepsies might feel focal because they do have focality on their EEG,
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but they should be distinguished from the more true focal epilepsies that come
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from a true lesion in the cortex.
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You might not see it on MRI, but the focal epilepsies come from some sort of
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lesion in the cortex that is the trigger for their seizures.
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This is another illustrative case of a seizure that could be a little tricky
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when he first presents to you.
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This nine-year-old little boy in foster care tells the school nurse that about
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every couple of days, he's having these episodes where his throat feels very,
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very tight and his whole body feels hot.
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It'll last a few minutes and go away. So the nurse, very understandably and
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appropriately, assumes their
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panic attacks, which is a very reasonable assumption, talks to foster mom.
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Foster mom goes home, works on behavioral strategies for anxiety.
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He's still having them every couple of days. She talks to the PCP,
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the PCP prescribed Alexa Pro, which again is a very reasonable course of action for panic attacks.
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But these freak events just continue.
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And then he starts to look like he's fidgeting with his hands every time during the episodes.
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And then finally, during at the end of one of the prolonged episodes,
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his head starts to deviate to the left sides and he develops a generalized tonic-clonic seizure.
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And I include that case as sort of an intro to some of the more,
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some of the, just to introduce the variety or the really massive landscape of
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potential auras and semiologies for focal seizures.
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This table was taken from the standard textbook on surgical epilepsy.
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It's certainly not a comprehensive table, but it kind of illustrates the variety
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of symptoms you can have at the onset of a seizure.
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You can have unprovoked fear.
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And that by itself might be the only symptom of the seizure at the beginning
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until it sort of progresses and declares itself.
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I had one young adult, like a 20-something-year-old young man patient tell me
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that the only way he could tell the difference between his emotions and his
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seizures were like sort of the context.
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If he felt very, very afraid and he didn't have a reason to feel afraid,
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then he knew this must be his seizure coming on.
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If he felt afraid and there was a clear situational reason, then he wouldn't
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be as afraid that this is a seizure coming on.
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And he got pretty accurate at that, but it wasn't that he could tell the difference between the fear.
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It's because it would start when he was still conscious enough to assess the
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context and see what made sense.
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Blinking is one of the symptoms of occipital lobe seizures. There's focal motor
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movements, swallowing, hallucinations.
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There are some seizures that begin with sort of this repetitive auditory hallucination
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of a song that you have actually like heard before in the past and that you
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normally would not be surprised about thinking again.
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Again, all of these specific subtypes are relatively uncommon.
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And of course, you shouldn't treat every panic attack as if it's a seizure,
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but it's worth knowing about these just in context.
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If, you know, treating empirically for stomach pain or anxiety or that sort
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of a thing is not making any progress, it is worth knowing.
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It's important to know that seizures can have very tricky, very focal semiology.
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And it can be worth, and again, it's the job of a neurologist to definitively
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diagnose one way or another, or whether something is a psychogenic neurologic
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symptom, or whether this is actually a seizure that we should be worried about.
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Most of those focal seizures come in children, at least, come from vocal cortical dysplasias.
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That's just a malformation in the cortex that can occur anywhere.
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A lot of them do run in families, okay?
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So just because something is due to a brain malformation does not mean that
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it cannot be hereditary.
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A lot of those mutations in the amateur pathway do run in families with incomplete penetrance.
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These seizures can occur anytime from infancy to late adulthood,
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but this is just, again, a common etiology at this age.
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They tend to they're more likely to be resistant to medications
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that would be one of your first clues that this is not one
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of the self-limited epilepsies and again
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it depends entirely on where it's located in the cortex the
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mTOR pathway is just worth reviewing very quickly
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one of the signaling pathways that leads to cell growth in many different organ
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systems the sort of the path the most common syndrome which are the tuberous
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sclerosis but quite a number of mutations in various points along this pathway
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can lead to hereditary focal epilepsies from focal cortical dysplasias.
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So these are some of the common genes tested for genetic panels.
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The most common focal epilepsy really in both children and adults is temporal lobe epilepsy.
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Again, this one is much more common in adults, but it still happens frequently enough in children.
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Children usually do have the same auras as adults once they're old enough to
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articulate their symptoms.
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And that can be stomach pains, cabbiness, fear, deja vu, and then progressed
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sort of hand automatisms, like sort of a picking, rubbing, dystonic posturing.
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All of those symptoms can help localize where the seizure actually started,
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mouth smacking, and then eventually like head version and generalized tonic-clonic convulsion.
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You can absolutely have like short seizures that stop before the convulsion
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or before the head version as a prodrome to the deaf, for sometimes a long time
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before they have their first convulsions.
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Sometimes, as I mentioned before, they might run at families from emptor things.
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Sometimes there are other lesions in the cortex.
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One very well described etiology or mechanism for temporal epilepsy are actually
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prolonged febrile seizures in early childhood.
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So when we counsel patients, children or infants with febrile seizures,
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that they have only a 3% risk of epilepsy down the road, this is part of that 3% risk.
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Some patients, I know we counsel most people that febrile seizures are benign,
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and they usually are most of the time, but it is true that they can actually
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see the mesial temporal lobe leading to epilepsy down the road.
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The MRI might look normal for many years, even if the pathology exists.
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The temporal lobe, of course, overlaps with both of your language centers of memory,
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hearing, and that leads not only to those auras that we talked about,
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but can also over time lead to progressive damage in things like learning and memory.
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Okay, so almost all of the medications can be used. Usually,
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you know, you start with whatever makes sense based on the patient's,
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on the side effect profile of the medication, the patient's other comorbid medical conditions.
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But this is a good place to bring up the fact that in general for epilepsy,
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if a patient has failed two medications, whether from side effects or just because
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the medication was not effective enough.
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The standard of care is to start considering pre-surgical evaluations,
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start considering whether the patient would be a good candidate for a surgery
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to better, to resect, to better control their epilepsy.
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In patients with focal epilepsy, early resection does lead to the best seizure outcomes.
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It's not necessarily something you should think of as a last resort.
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Part of that is because,
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The resection, if it's done early enough, leads to the best seizure outcomes.
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And if the surgery is delayed for too many years, other parts of the brain,
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whether it's the contralateral temporal lobe or other foci in the brain,
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they become entrenched.
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It seeds the other parts of the brain. They become entrenched in the seizure propagation network.
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So delayed surgery, if you wait too long, can be significantly less effective.
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The way I explain it to patients is that your brain becomes really good at anything that you practice.
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If you practice playing the piano, your brain is good at that.
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If you practice playing basketball, your brain is good at that.
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If you practice being grateful, your brain becomes good at that.
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If you practice having seizures, your brain becomes good at them and it's harder to stop them. Okay.
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So earlier surgery leads to better seizure outcomes. It also in younger children
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leads to better rehab potential.
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Sort of the younger you remove the not useful piece of the brain,
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the part that's causing the seizures, the more neuroplasticity you have at your
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disposal for rehabilitation.
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Temporal lobotomy had the best results in focal cortical splashes depending,
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again, it depends on the center and on the mode of selection,
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but they can have the 50-60% seizure freedom in well-selected patients.
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The pre-surgical workup is outside the scope of this talk and would be kind
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of another hour, deserves its own hour by itself.
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But there are a number of imaging modalities and other forms of testing that
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you would do to, first of all, make sure that the lesion is not overlapping on eloquent cortex,
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that resection is not going to result in permanent loss of language or memory
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or certain motor functions or certain visual fields.
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And then you also try to localize it well to make sure that you truly understand
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exactly where the seizure is coming from and you're going to have a good epilepsy result at the end.
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Again, and that specific workup is very individualized to the patient,
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and it always should happen in the context of a large epilepsy team.
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Now, there are, for patients for whom resection is not, medications don't work,
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resection is not an option either because it's in too many locations in the
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brain or overlaps too heavily on eloquent cortex.
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There are other implants, and I think the most important one to bring up is
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responsive nerve stimulation.
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This is probably the latest greatest technology available
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to us now in epilepsy but it's been used in adults
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and children for really for quite
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a few years now essentially you put a little computer chip in a little what
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looks like a pacemaker like right on the surface of your brain this is a brain
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implant and then you have two little electrodes that go that you permanently
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implant inside the brain and those electrodes detect when your seizures are happening.
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Store it in the little device and then send a signal to try to disrupt the seizure
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to stop it okay and this device is able to gradually over time learn what your seizures look like,
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and learn what kind of stimulus is necessary to break the seizures in your case
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and what happens is the all of that data that it's detecting as it's sort of
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continually monitoring the brain gets stored in the device that gets uploaded
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to a computer about once every couple of weeks once a month,
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and then the epileptologist as well as the device engineer will work with the
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device to train it to change the settings to help it learn your patient's epilepsy
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and your patient's best stimulation settings.
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It's a big deal to implant people. I don't think anybody's certainly doing it
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under the age of six or seven years old, but it is a very effective option for
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patients for whom surgery is not an option.
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The results for medial temporal lobe epilepsy are a 70% reduction seizure frequency.
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And then according to the clinical trial, 30% of patients achieve seizure freedom
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for greater than six months.
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So with the focal epilepsy, we do have a wide array of options beyond just medications.
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Of course, we just talked about the focal epilepsy, so let's move on to the
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generalized epilepsies.
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This is another large fraction of the seizures that we would see on a regular
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basis in the emergency department. What is this?
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It's just any seizure whose onset involves all lobes of the brain simultaneously,
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and they can be generalized tonic convulsions, but absent seizures are also generalized.
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Many atonic seizures are generalized, sometimes myoclonic seizures.
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The different combinations of those various semiologies, all of these can come
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as a generalized seizure.
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And just an EEG example to illustrate, it involves all lobes of the brain right
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exactly at the same time, the same millisecond it hits all of them.
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So here's a good typical example that you might see in your general pediatrics clinic.
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13-year-old girl comes to the ER for a first-time seizure.
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This time, she presents with a convulsion lasting three minutes at school,
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and it just occurred without warning. There was no clear trigger.
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Nothing else was going on.
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And you talk to her more and you're asking about what's been going on in the past.
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Does she have any other medical conditions, any prodrome, anything in the last
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few months? And she reports that she is a little bit clumsy in the morning for the past year.
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Often she's always dropping her toothbrush or dropping like her breakfast and
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her siblings have been teasing her about that.
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And they've just been sort of brushing it off as a joke.
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Otherwise, though, this is a perfectly healthy child on enroll classes,
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traveling for basketball.
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There is a family history too. Grandmother was on phenobarbital,
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and then father had this funny history where he was spacey.
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He didn't do well academically in his elementary school, but then he kind of
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came into his own as he got older.
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So this is like a very classic case of juvenile myoplinic epilepsy.
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Again, this is another one that all of you will see at some point as a general pediatrician.
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It's 9% of all epilepsies. This age of onset's a little bit later than the ones
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we've talked about before, usually in late elementary school or early adolescence.
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And it's the combination of myoclonic and generalized tonic-clonic seizures.
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So that clumsiness in the morning that she was reporting was from myoclonic seizures.
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That's usually how it presents. The patient thinks they're clumsy,
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and in retrospect, they were just having myoclones intermittently early in the morning.
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This occurs in patients that are essentially like abnormal development.
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If the patient has cognitive disability, you need to do more workup.
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You can't immediately assume that it's juvenile myoclonic epilepsy.
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This is another one that is normal head imaging. The EEG is characteristic.
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But this one, unlike the self-limited epilepsies, tends to have lifelong disease,
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at least for the next several decades after the initial diagnosis.
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So this is a patient where you have to sit down and counsel them that they need
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to choose medications that will be...
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Safe for them to stay on for the next several stages in life.
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If it's a girl, please don't put her on Depakote or Topiramate if you can't
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help it, because you want her to be stable on some combination of meds during
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her childbearing years, which are just coming up in above a decade or so.
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Try to avoid things that have the potential to exacerbate other common conditions in adulthood.
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Now, the good news is 60 to 90% of them respond well to medication.
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So you need to counsel them, counsel most of these patients that they probably
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shouldn't try to be a commercial airline pilot. They probably shouldn't try
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to go into active combat in the military, but they can do.
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Since most of them are likely to be well-controlled, they are probably going
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to be able to do almost anything else career-wise and life-wise.
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They're probably going to be able to learn to drive, go to college,
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all of those, and they have children, all of those things.
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And so recognizing this syndrome and like diagnosing it correctly early on is
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really essential in giving them the correct counseling and management early into the course.
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And this is one of a group called the idiopathic generalized epilepsies. Okay.
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This as a group is 10 to 20% of epilepsy in children.
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So another thing, again, you are going to see this as a general pediatrician.
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This one is, it's heritable. It runs in families, okay, in the sense that maybe
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the dad has juvenile myoclonic epilepsy and the daughter has childhood absence.
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Or maybe there's a cousin that has just generalized chronic seizures and then
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there's, you know, one child that has childhood absence seizures,
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but then one cousin another generation later has juvenile absence.
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Like that's how it runs in families. There's not necessarily a particular,
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like one phenotype doesn't necessarily run in families by itself.
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Most of the time, these are controlled on medications, not all the time, but often.
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This is the subtype of epilepsy that is most likely to activate with either
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photic stimulation or hyperventilation.
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So you know how on EEGs, we always flash the lights in their face or have them blow on a pinwheel.
25:21
It's mostly for the diagnosis of this little subcategory of epilepsies because they're so common.
25:26
And if this is the correct diagnosis, they do not evolve to an epileptic encephalopathy.
25:34
Just to talk in a little more depth about some of the other subtypes of the
25:39
IGEs, idiopathic generalized epilepsies, child's adoptions, epilepsy,
25:43
everybody memorizes this one in med school.
25:45
18% of epilepsy in school-age children starts around 4 to 10 years of age.
25:49
It's the 3-hertz spike in my discharges. That one, every medical student has
25:52
memorized at some point.
25:54
The one thing to keep in mind is that I think we memorize this as a universally
26:00
self-limited epilepsy.
26:02
It is true, most of them resolve around adolescents, but the official number
26:07
from the position papers of the International League Against Epilepsy is that
26:11
it's really only 60%. I would have expected that number to be higher the way
26:14
that we are taught it, at least in medical school.
26:16
So that means there are 40% that are going to eventually evolve to a different
26:21
type of idiopathic epilepsy.
26:23
So you cannot necessarily counsel these families that their child will grow
26:27
out of it for sure and never have to think about it again.
26:30
They have a high chance of growing out of it and never having to think about
26:34
epilepsy again. But there is a significant minority that eventually develop
26:38
generalized tonic-plonic seizures.
26:39
Okay, so that is an important part of the counseling to include when you talk to these families.
26:46
The other piece of it is that at about one-tenth, the incidence of standard
26:51
childhood absence epilepsy, there is also a thing called juvenile absence epilepsy.
26:55
The EEG can look very similar, maybe a little bit faster spike in waves,
26:59
but I mean, it might not be significant enough to pick up on the first diagnosis.
27:04
Seizures might last a little bit longer and less frequently.
27:07
And again, this is in patients where their head imaging is normal and development
27:10
should be basically normal.
27:11
But this is, it can look fairly similar at the beginning, but these patients
27:16
almost all develop generalized tonic-clonic seizures down the road.
27:19
And these are patients that require lifelong medicine. And again,
27:23
It would be very easy early in the presentation to confuse it with this more
27:28
common, more likely to be self-limited epilepsy.
27:32
And so, again, when you diagnose patients with a generalized epilepsy and abnormal
27:38
EEG, it is still important to talk to them about seizure precautions.
27:42
What do you do if your child has a generalized tonic-clonic seizure?
27:45
What is emergency management of a seizure? and it's still important to talk
27:50
to them about sort of the range of prognosis so that they're prepared for different possibilities.
27:55
Another thing that's worth keeping in mind is that 20% of patients, again,
27:59
these are numbers from the International League Against Epilepsy,
28:01
their position papers, 20% of these patients develop absent status epilepticus,
28:06
which is an entity that I don't think most of us really ever think about most
28:10
of the time, but this is a form of status epilepticus.
28:14
It's very confusing and it's almost a little bit freaky to watch.
28:18
The patient is going about their activities. They just have slowed response time.
28:23
They look dazed and they're just not remembering anything.
28:25
They can't remember that they went to the grocery store, but they did go to
28:28
the grocery store and sort of go walk around and buy their groceries.
28:32
And they did sort of walk around the house and try to do things,
28:35
but they're just not acting themselves and it looks a lot like they're intoxicated.
28:39
And there are patients, especially adults probably, where this can last for
28:43
a couple of days before they're brought to medical attention,
28:45
and then the EEG shows continuous or semi-continuous generalized low-spike discharges.
28:49
This can be precipitated by basically using the wrong type of seizure medication
28:53
in a generalized epilepsy or abruptly stopping at a fan or a benzodiazepine.
28:58
The main reason to think about this is not so much because it's common,
29:02
but because it would be very easy to confuse for intoxication.
29:08
So again, these are the idiopathic generalized epilepsies, childhood absence,
29:13
juvenile absence, and juvenile myoclonic, as well as just generalized tonic-clonic
29:17
seizures by themselves, and they all overlap a little bit.
29:20
But just so you don't get too simplified of a picture of generalized epilepsies,
29:25
okay, this is its own little box here, but there are quite a number of other
29:29
types of possible generalized epilepsies, some of which have, you know,
29:33
genetic etiologies, and most of which start to be less benign in terms of long-term
29:38
development and cognition, is a case that is kind of derived from patients I've
29:44
seen in clinic. It's an 11-year-old little girl.
29:47
She's been diagnosed with ADHD. She comes to Neurology Clinic because of these
29:50
movements. The PCP has diagnosed them as chronic motor tics.
29:53
She just has rapid eye blinking intermittently.
29:57
And of course, at first, PCP counseled, as you normally do for tics, just ignore it.
30:03
Let her, you know, it should evolve to something else over time.
30:08
She'll probably grow out of the tic. The more you think, the more likely it's going to go away.
30:11
But the episodes just continue happening frequently, especially when she looks
30:16
up or looks towards lights.
30:17
Again, and that doesn't necessarily make anybody think anything unusual.
30:21
Of course, you blink more often when you look at the sun or you look at the
30:24
light in, you know, in your classroom.
30:26
But at a certain point, the mother is getting frustrated because it's happening
30:30
too often. The patient's falling behind in school.
30:32
The mom thinks, okay, maybe the blinking is distracting the patient.
30:35
So you try guanfacine and there's no improvement.
30:36
So nothing's getting better. So you get an EEG and there you go.
30:41
Every time she has these blinking episodes, you see these sort of high-voltage
30:45
spike discharge, generalized spike discharges that sort of are bifrontal.
30:50
And they happen every time she has these little blinking episodes.
30:54
And they do seem to correlate with a little brief lapse of consciousness every
30:58
time she has these blinking episodes.
31:00
This is a classic syndrome that used to be called sunflower syndrome.
31:05
Obvious reasons, or Javon's syndrome after the person who described it,
31:08
but now has been updated to a more sort of descriptive term of the seizure semiology.
31:14
It's epilepsy with eyelid myoclomia. It's a rare epilepsy, but again,
31:17
it's easy to miss, easy to not pick up.
31:21
Onset is between three to 12 years of age. And this, the seizure semiology is
31:25
literally, they just have this rhythmic eyelid twitching with a little head
31:28
extension that's triggered by sunlight or photic stimulation.
31:31
You can understand how this goes undetected for a long time.
31:34
Of course, you blink when you look at the sun.
31:37
But it is associated with a lapse of consciousness. It's associated with EEG
31:42
changes with those blinking episodes.
31:44
And they often also have either absent seizures or generalized tonic convulsive seizures eventually.
31:51
Usually requires lifelong medication. And this might sound like a benign etiology,
31:55
but just imagine if every time you're driving and every time the sunlight hits
32:00
your eyes, you have a brief lapse of consciousness.
32:02
This is a very disabling disease, even though it doesn't necessarily look disabling on the outside.
32:07
And so it's important. It's very rare, but it is important to pick up when it presents.
32:12
There's another entity called GEFS+. It's not so much its own syndrome.
32:16
And I think it's really easy to get confused when people first teach you about it.
32:19
Generalized epilepsy with febrile seizures plus it's just
32:22
a patient with febrile seizures who again later in childhood
32:25
develops one other type of generalized seizure it's this
32:28
umbrella term family it's usually runs in
32:31
families where some patients in the family have simple febrile
32:34
seizures other have febrile seizures that just continue beyond the
32:37
expected age group others have idiopathic epilepsies you might even have to
32:40
worry 10 of these families with these clusters of various generalized seizures
32:46
occur with a particular type of mutation called SCM1A that is a sodium channel
32:52
mutation on the interneurons.
32:54
But there are quite a few other channelopathies that have been identified.
32:57
So it's not like this presentation doesn't necessarily formally diagnose you
33:03
with this condition, but it is a responsible for high fraction as well as some
33:06
of the other channelopathies.
33:08
Just to kind of introduce this sort of concept, this is another set of epilepsies
33:14
that just kind of run in families together.
33:17
The mechanism of some of these epilepsies is very well described.
33:21
Onset seizures are thought to be due to thalamic dysregulation.
33:25
So it, and this kind of makes sense.
33:27
The thalamus is sort of the realization for everything that is happening, like,
33:32
to the cortex. And so if the, is a relay station, right?
33:37
Like some, it controls many, many different parts of the cortex.
33:40
And if you were to, it's involved in like relaying to the basal ganglia, that kind of a thing.
33:45
And so if you were to have sort of dysregulation of the thalamus or excessive
33:49
activity of the thalamus, it would, you would imagine that it would project
33:52
to the entire cortex at the same time, which is the idea that's the mechanism
33:56
at this point is pretty well established for obstinate seizures by themselves.
34:00
That's why we use ethosexamide, because it specifically targets the calcium
34:05
channels that are most common in the thalamus.
34:07
Other types of generalized seizures come from channelopathies.
34:11
Again, that's fairly logical. If you have a genetic mutation in every sodium
34:14
channel in the brain, or every potassium channel, or every GABA receptor,
34:17
you would imagine that, you know, the seizure would start in all parts of the brain simultaneously.
34:24
Other generalized seizures, as we talked about with the idiopathic generalized
34:27
epilepsy, are often polygenic in nature.
34:29
Like they're probably multifactorial, just like your height and weight and things like that.
34:34
Also, a lot of nonspecific secondary epilepsies, like from chromosomal microbe
34:38
deletions, end up being treated empirically the same as a lot of the generalized epilepsies.
34:44
The treatment, the most important thing to remember when you're treating epilepsy
34:48
is to avoid the pure sodium channel blockers.
34:51
This is a situation where you should absolutely not use trileptal or VIMPAC.
34:58
Also, pure GABA activity, these are less common medications,
35:02
but that also can worsen both the epilepsy and the development associated with that epilepsy.
35:08
Usually, Kepler of Elproate are often first line, but lamotrigine is very reasonable
35:12
for most of first many of these epilepsies.
35:15
Clobizam, zonisamide is a really good option when you're more refractory parampanil.
35:20
We still have a good battery of medications that are very well established as
35:26
being effective in most of these syndromes.
35:29
The one thing to remember is that ethosexamide only helps with true absence seizures.
35:34
The only syndrome for which you really should be using ethosuximide by itself
35:39
at least is in childhood absence epilepsy because ethosuximide only affects
35:50
the T-type calcium channels.
35:52
It will not do anything for generalized tonic-clonic seizures or myoclonic seizures
35:58
or any of the other species.
35:59
As far as childhood absence epilepsy is concerned, of course,
36:03
ethosexamide we have already memorized in med school as first line.
36:06
The other two things that are shown in sort of the standard clinical trial for
36:09
childhood absence are valproate and lamotrigine, usually you pick based on gender.
36:14
Juvenile myoclonic epilepsy, you can use almost all of these depending on the
36:18
patient and so on and so forth.
36:21
There is a fraction, maybe 5-10%, depending on the specific syndrome,
36:27
that are going to be refractory.
36:28
And of course, if that were to happen, the first thing you'd want to make sure
36:31
is that you have the diagnosis correct, that you're not missing something more
36:34
progressive or some kind of a subtle focal lesion or something like that.
36:39
But in patients who truly have a idiopathic generalized epilepsy and who just
36:46
aren't responding to any of the medications or who have side effects on all
36:49
the medications you try, there are a couple other options.
36:51
Vagal nerve stimulators are a very reasonable thing, can decrease medication burden.
36:55
I think the generally quoted number is that it can decrease seizures by 50%
36:59
in about 50% of patients.
37:01
Certainly not a slam dunk. It's not perfect, but it's a reasonable thing to offer.
37:04
You can try dietary therapy, which I'll talk about down the road in a couple
37:09
of slides in the context of a different syndrome.
37:11
It has moderate efficacy. It's a perfectly reasonable thing to try if nothing else has worked.
37:16
And then currently there is a clinical trial looking at responsive neurostimulation
37:21
for the idiopathic generalized epilepsy as a result of that should be published next year.
37:27
So for the very, very refractory juvenile myoclonic epilepsy or juvenile absence
37:32
epilepsy, we might be able to offer RNS as a therapeutic option down the road.
37:39
But as I said before, if you have a refractory, really especially a refractory
37:46
generalized epilepsy, you should start to worry that maybe you need to think
37:49
about a different diagnosis.
37:51
Especially if the patient's development is now being severely impaired.
37:55
The developmental epileptic encephalopathies are refractory epilepsies associated
37:59
with significant developmental delay or regression.
38:03
So this is a variation of a couple of patients that I've seen in the last few years.
38:09
Three-year-old boy comes to your clinic with ticks. He lives in the valley.
38:13
Parents just think he moves funny sometimes. And they brought him to the pediatrician a few times.
38:18
And at first, the pediatrician said, this looks like an exaggerated morrow.
38:21
But you don't normally have exaggerated morrows in three-year-olds.
38:24
So he just wanted to rule out other problems. But he called it a tick.
38:27
He sent it to your clinic for ticks.
38:28
And the parents report that this patient is having sudden arm jerks multiple
38:32
times throughout the day. They don't know how many months ago this started.
38:35
They think the frequency is increasing.
38:36
Now, and up to this point, the patient was developmentally normal.
38:39
Like nobody had any concerns.
38:41
Nobody was thinking too hard about it. He just was kind of, he learned to walk
38:44
and talk at the normal time. But over the last two months, he's having daily
38:47
tantrums. He's getting harder to manage.
38:49
Grandparents are calling this just delayed onset of the terrible twos,
38:52
but he's not sleeping well anymore. He's not consistent in his potty training.
38:57
And in clinic, you get to see one of these events, but his bilateral arms jerk
39:01
outward and his head drops momentarily. and then he begins crying.
39:05
This would be a very classic presentation of a syndrome called epilepsy with
39:12
myoclonic atonic seizures, or EMATS.
39:14
It used to be called Doze syndrome, D-O-O-S-E, but again, the official name
39:21
is now EMATS because that describes exactly what the syndrome is.
39:25
It's not terribly common, but it is 2% of childhood epilepsies,
39:28
and so any neurology clinic will certainly see this subfrequency.
39:32
This starts between two to six years of age, usually in patients that are normally
39:36
have development, normal development prior to onset.
39:40
It doesn't, most of the time, there isn't one specific gene that you can pin this to.
39:45
Sometimes there are some, 5% have GLUT1 deficiency, but most of the time you
39:50
can't pin it off to just one single gene.
39:53
The EEG shows the generalized poly spike in waves, the head imaging is normal.
39:58
And this one has a very characteristic sort
40:02
of clinical course there's a stormy phase for several
40:05
years where you have myoclonic atonic
40:08
seizures and generalized tonic clonic seizures and sometimes some
40:11
other generalized seizure types and you start keprin you start valproate and
40:16
and they might slow them down or they'll stop the generalized tonic clonic seizures
40:20
but it's very difficult to control these myoclonic atonic jerks if you can control
40:26
it on a couple of seizure medications,
40:28
that's wonderful, but a large fraction of the time, the only.
40:32
Meaningful treatment for this one is the ketogenic diet or one of the modified versions of it.
40:36
Okay, so the people who most of the good studies on this epilepsy syndrome will
40:42
say very clearly that the ketogenic diet should be considered gold standard
40:47
for this particular syndrome.
40:50
Should be offered early. And part of the reason that this should be offered
40:54
so early and sort of pushed as a good treatment for this epilepsy is because
40:59
the seizures themselves often go into remission both three or so years after
41:04
onset and you're able to wean off the medication.
41:06
But the subsequent development can either go back to normal or you might retain
41:11
residual-like deficits.
41:12
And we do think that some of the cognitive development down the road probably
41:18
has something to do at least with how well the seizures are controlled.
41:22
And so the better you can control the seizures during this stormy phase,
41:25
hopefully down the road, the better their long-term development is going to
41:30
be once they get past this stormy phase.
41:32
So that leads us to just briefly discussing the ketogenic diet.
41:36
This is probably the best syndrome to discuss it.
41:38
And this is probably the best context in which to discuss this as a treatment for epilepsy.
41:45
This is not quite the same thing as the ketogenic diet for weight loss that
41:50
family doctors use to help their adults get off their diabetes medications.
41:54
Okay, it's a similar concept.
41:56
You're trying to gain your calories more from fat and protein as opposed to
42:01
carbohydrates, but it's a little bit more aggressive.
42:04
There's a classic ketogenic diet as it was originally developed,
42:07
and then other versions, including the modified Atkins, are a little bit more
42:11
tolerable for children who eat by melt instead of G2.
42:14
This is a normal diet, 55% carbohydrate.
42:18
So that's your apple and your oatmeal, this 30% fat, that's the olive oil you
42:23
put on your carrots, that sort of thing, and then 15% protein.
42:26
In the classic ketogenic diet.
42:29
Over 90% of your calories are coming from fat.
42:33
And then in the modified Atkins, like 65 to 70% of your calories are coming from fat.
42:39
This is not like a easy, standard, natural, healthy diet.
42:43
Sometimes parents come and try to figure out if there's any way to fix their
42:48
child's epilepsy just by cleaning up the diet and getting rid of the takis and
42:52
eating more vegetables and that sort of thing.
42:54
That doesn't really work that way, unfortunately. But if you're going to use
42:57
diet to treat epilepsy, this is what you have to do.
43:00
And the mechanism is, you know, at the surface level, at least the mechanism
43:05
is fairly straightforward.
43:06
Most of us use carbohydrates as a source of most of our energy.
43:10
Whatever we don't use gets stored in glycogen. And then whatever doesn't get
43:13
used, stored as glycogen.
43:15
Whenever the glycogen is not used over time, then eventually gets stored into
43:18
fatty tissue. And when we go into ketosis, when we use one of these dietary
43:24
strategies, we are bypassing glucose as a mechanism of energy.
43:28
We're bypassing a glycogen. We are going straight to fat as a source of energy,
43:32
which means that the circulating currency for energy in your body is no longer
43:36
glucose so much as it is the ketones.
43:39
So all of your cells are being forced to use ketones as their fundamental substrate
43:43
of energy rather than glucose. And for multifactorial reasons,
43:47
this leads to seizure control, being consistently in ketosis.
43:52
It's not an easy diet to follow, if I haven't said that enough times already.
43:55
And it's not necessarily a standard healthy diet, but it is an option.
43:59
And it is sometimes the best option for certain syndromes, most classically EMATs.
44:05
This is some of the variations of the diet. You know, it's a little bit of lots
44:11
of fat, a little bit of protein, a little bit of vegetable.
44:15
Now I started with that one because it's a good illustration of the fact that
44:20
not every refractory epilepsy is Lennox-Gustin.
44:24
Okay, this is its own separate syndrome.
44:27
I think that this term gets thrown around in medical charts a lot just to mean
44:31
a child who has seizures that we can't control very well.
44:35
But as you've seen already, first of all, there are plenty of examples of other
44:41
epilepsy types that just happen to be difficult to control.
44:45
And there are other syndromes involving developmental regression and refractory
44:50
epilepsy that are not exactly the same as Lennox-Gusto.
44:53
And that's important because each one of these different syndromes has different
44:56
prognoses, requires different counseling, and involves different treatment options.
45:01
Okay, Lennox-Gastaut, this is still the most common developmental epileptic
45:05
encephalopathy because it's the final common endpoint for many different brain pathologies.
45:09
So babies who had HIE might eventually develop Lennox-Gastaut.
45:13
A lot of children had infantile spasms when they're younger eventually become Lennox-Gastaut.
45:17
It just doesn't declare itself as Lennox-Gastaut usually until they're sort
45:21
of like toddlers to sometimes like much later in childhood.
45:26
This one usually has a very identifiable etiology, either genetic or structural.
45:30
Sometimes it might be found on a chromosomal microarray, not just a single gene.
45:35
You might have abnormal development before. It might be normal,
45:37
but it always worsens cognition and development.
45:41
Tonic seizures are mandatory for diagnosis, but you can have all of these other seizure types.
45:48
The EEG is also very, very characteristic.
45:52
In order to call something Lennox-Gastaut, you have to see less than 2.5 hertz
45:55
spike and wave, and also generalized paroxysmal fast activity.
45:59
So these are two buzzwords that if you see them on a test question or an EEG
46:02
report, these are cluing you in that now we're talking about Lennox-Gastaut.
46:07
And it's important to label this one correctly because almost universally,
46:12
these patients have refractory seizures, multiple seizure types,
46:14
and 90% are going to moderate to severe intellectual disability.
46:19
This is not a diagnosis you want to make incorrectly. You would never want to
46:25
confuse this, for example, with this one. The prognosis is so different.
46:30
And you'd want to make sure that when you're treating the refractory seizures,
46:35
that you're using medications and strategies that are optimal for this particular syndrome.
46:41
One other developmental epileptic encephalopathy that's really important to
46:46
mention is something called ESWASER spike wave activation in sleep.
46:50
This one used to be called Lendel-Kloffner, but as the phenotype was sort of
46:54
expanded and as the disease was better understood, it has been given also an
47:00
acronym that just tells you exactly what it is.
47:04
This is very rare. You don't see it very often. It's less than 1% of the epilepsy
47:07
in the tertiary care centers, but most tertiary gear cells will see this.
47:11
And when it happens, you don't want to miss it.
47:13
This is developmental regression that correlates with continuous spike wave
47:17
on the EG during sleep. Okay.
47:19
So the patient comes in with, they're just sort of, they've been doing well
47:25
in school and they are worsening or they were, like,
47:29
they were developing fairly normally, but now they're just significantly impaired
47:33
and nobody knows why they went to ENT and things like that.
47:37
And they, And nobody can understand why they're not talking as much.
47:40
But finally, somebody gets an EEG and you see this. Basically,
47:43
it looks like status epilepticus during sleep.
47:45
Normal during wakefulness or mostly normal during wakefulness,
47:48
but then it starts to look like continuous spike wave activity during sleep.
47:52
And the treatment for this is fairly specific to this syndrome.
47:57
If it's treated well, you can certainly rescue some of the developmental outcome
48:03
in a large fraction of patients.
48:05
Standard of care is either ontphi or diazepam, which are both basically benzodiazepines
48:11
at night, or daily steroids. Okay.
48:14
On fees, that's the lowest side effect profile. Most people start with it next.
48:17
But sometimes steroids are the only thing that sort of rescues the patient's
48:20
speech. You never know. So you have to try different things depending on the patient.
48:23
It does tend to be refractory to medication to some degree.
48:26
You don't really cure it so much. But the subsequent develop does depend partly
48:30
on how well you treat the spike wave burden during sleep.
48:33
And again, rare, but you don't want to miss it when it happens.
48:37
And as I've highlighted already, that when you're treating the developmental
48:40
epileptic encephalopsies, that preferred medications are highly dependent on the specific syndrome.
48:45
Lennox-Gastaut, it's the subject of many clinical trials, so it has a much wider
48:49
array of medication options than really any of the others, as it should.
48:53
VNS is often used to decrease medication burner.
48:56
That's vagal nerve stimulation. The ketogenic diet, this is another,
48:59
even for Lennox-Gastaut or continuous spike wave in sleep, ketogenic diet is
49:05
a very reasonable option, especially in G-tube-fed patients.
49:10
And then other implants or neuromodulation devices are being studied.
49:15
Right now, there's a phase two clinical trial going on for RNS for really,
49:18
really refractory Linux-Gistos syndrome.
49:21
And so that sort of, the syndrome that's covered up till now are sort of the
49:28
majority of the important syndromes you should recognize, like for the epilepsy themselves.
49:34
I'm just going to now quickly highlight a couple of other more rare genetic
49:39
or irreversible conditions that you just should be aware of so that you recognize
49:44
them again if they were to come to the hospital.
49:47
Rett syndrome, I'm not going to go into this. This could also,
49:49
this syndrome by itself could be discussed for an entire hour,
49:52
but this is a rare genetic syndrome caused by a mutation in MEK-PTU,
49:56
a transcription factor that affects the nervous system as well as other organ systems.
49:59
It results in loss of speech, loss of ability to walk, seizures,
50:03
movement, disordered vital sign, abnormality.
50:05
It starts with normal development and then regression. And the main thing I
50:09
just bring it up is because you don't want to confuse this with the other epileptic encephalopathies.
50:13
It's very clearly diagnosed on genetic testing.
50:16
There are a couple similar syndromes that are caused by other mutations that
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have similar sort of dramatic effects on brain development.
50:23
This is not curable, but it's a subject of ongoing active research.
50:27
Progressive myoclonic epilepsies are another subset of epilepsy. They have no cure.
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They're always initially on the difficulty for myoclonic seizures.
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Very, very rare, but when they do present, they're usually initially misdiagnosed
50:41
to juvenile myoclonic epilepsy, but then we get genetic testing,
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and we find that never mind, you actually have an incurable disease.
50:49
These pictures are examples of patients from one of the patient-like advocacy websites.
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This girl that the website was named after, you know, completely healthy,
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normal teenager, beautiful soccer player.
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As a teenager, was diagnosed with this case, it was Lefora disease,
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and within a few years, she's wheelchair-bound, G-tube dependent,
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and then she dies in her early 20s, same thing happened to this girl.
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Completely healthy, normal, active teenager, diagnosed with,
51:16
started having myoclonus as a teenager, initially diagnosed with juvenile myoclonic
51:19
epilepsy, and then within the next 5 to 10 years, she's wheelchair-dependent
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G2 fed, cognitively delayed, and dies in her early 20s.
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This is one I just highlight because, again, you don't ever want to miss it
51:32
for the sake of prognosis, and it's often,
51:36
this is one of the, again, one of the things that generally gets misdiagnosed
51:40
as a more benign syndrome before it gets diagnosed properly.
51:43
And the treatment for many of these progressive epilepsy syndromes is you treat
51:46
it aggressively based on the mechanism of disease, but they're mostly areas
51:49
of basic science research, just ones you should be aware of.
51:53
So just kind of to summarize this whole presentation, I haven't gotten the impression
51:58
by now, childhood epilepsies are very heterogeneous in their symptoms and their
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treatment and prognosis,
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and the correct management is heavily reliant on early diagnostic testing, okay?
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There are some seizures for which you should prescribe the sodium channel blockers,
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others for which they're contraindicated, and so the understanding which should
52:19
be used in which context depends on understanding the underlying syndrome correctly.
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Also, another good take-home point for a general pediatrician,
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some seizures can be tricky.
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Just be very careful with chronic motor tics when they don't respond to initial treatment.
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Be very careful with panic attacks when they happen exactly the same way every
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single time and just don't respond to other management strategies.
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I mean, it's never wrong to refer to neurology just to rule things out.
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Anti-seizure medications, of course, are the mainstay of most treatment,
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but dietary therapy, receptive surgery and neuromodulation devices are also
52:50
important options for the appropriately selected patients.
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And with that, that is all I had for you guys today. Thanks for listening to that fabulous talk.
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Don't forget to click on the link for free credit. I'm Holly Wayment. I'll see you soon.
53:06
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53:10
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53:22
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