The El Paso Physician
New Advances in Pediatric Neurosciences in El Paso
Season 27 Episode 3 | 58m 25sVideo has Closed Captions
Advances in Pediatric Neurosciences Panel Discussion
Advances in Pediatric Neurosciences Panel | Dr. Ziyad Makoshi and Dr. Alexandria Melendez-Zaidi This program is underwritten by : El Paso Children's Hospital.
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The El Paso Physician is a local public television program presented by KCOS and KTTZ
The El Paso Physician
New Advances in Pediatric Neurosciences in El Paso
Season 27 Episode 3 | 58m 25sVideo has Closed Captions
Advances in Pediatric Neurosciences Panel | Dr. Ziyad Makoshi and Dr. Alexandria Melendez-Zaidi This program is underwritten by : El Paso Children's Hospital.
Problems playing video? | Closed Captioning Feedback
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My name is Dr. Luis Munoz, and I'm the president of El Paso County Medical Society.
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There are many different types of neurological conditions.
Neurology and the field of neurosciences is the specialty dedicated to the diagnosis and the treatment of issues affecting the nervous system.
And that includes the brain, the cervical spine, peripheral nerves and muscles.
Epilepsy is also a big part of what we're going to be discussing here this evening.
During the next hour, we have experts focusing on and answering questions about pediatric neurosciences in the borderland.
This evening's program is underwritten by the El Paso Children's Hospital.
And we also want to thank the El Paso County Medical Society for bringing this program to you.
Since 1997.
I'm Kathrin Berg, and you're tuned into the El Paso physician.
Thank you so much for joining me this evening.
Tonight, we're going to be talking about pediatric neurosciences in the Borderland.
And we have a veteran with us who's been in El Paso for a whopping a little bit more than two years.
But he's been on the show before.
And this is Ziyad Makoshi And we're going to ask you about what your favorite Mexican food restaurant is here in town.
You are the medical director of pediatric neurosurgery at El Paso Children's Hospital.
And you brought your friend, she says she doesn't have any friends, but I know she does.
You brought your friend and she is Dr. Alexandria Melendez Zaidi.
But I see it right.
He's a Zaidi.
There you go.
And she's a board certified pediatric neurologist and an epileptologist at El Paso Children's Hospital, also a Texas Tech physician.
So, Dr. Alexandria, we call her Allie.
I understand, Melendez, you've been in El Paso most of your life.
You're a Thunderbird, Coronado High School.
And I said, just to kind of lighten the mood a little bit.
Number one, we had to ask.
We went to high school.
That's the El Paso question.
And number two, what is your favorite native Mexican food restaurant in town?
This is like a plug.
We shouldn't say this.
Actually.
You know what?
Never mind.
We'll talk about that after the show.
Oh, yes is it rellenos, or is it enchiladas.
I like Machaca.
Oh really.
Oh yeah.
The place good, right?
Yeah.
Okay.
I'll be quiet again because we try not to give plugs.
Yeah.
Yeah.
I have a place, but I have a plate.
We'll talk about that after the show.
Okay.
Makoshi, how about you?
I was thinking classic with the tacos, actually.
Oh, with the tacos.
Very nice.
Beef, potato.
You didn't have to go there, but.
Yeah, and all of the above.
So, Makoshi.
Let's talk about you.
So, again, you a medical director of pediatric neurosurgery at El Paso Children's?
What does that mean for people who are tuning in?
And again, and you know, you're not a neurosurgeon, but you are what does that mean to the general public?
So the pediatric neurosurgery is one of the specialties that we don't have enough in North America.
So there are few and far in between there, sometimes in major centers where you'll have two or three pediatric neurosurgeons.
But then similar to El Paso, the closest centers may be as far as an 8-10 hour drive.
So pediatric nurse surgery is a subspecialty to manage any of the pathologies that involve the brain, the skull or the spinal cord and even the peripheral nerves.
So we've had a lot of kids that were born with abnormalities that developed in utero, like open spinal cords that need immediate surgery, a lot of water on the brain, hydrocephalus, the big heads sometimes need shunts in order to live normal lives.
And these kids were transferred before to other centers that were far away.
So it was travel for family, a lot of expenses, not a lot of as far as support.
So bringing that here, where we can manage those conditions and growing the neurosurgery program has been one of my jobs as medical director to continue to offer what we can for El Paso in the surrounding region and keeping those kids from having to travel with their family as a far distance to get treatment.
Follow it.
Which is so important because we talk often on this program about the support system and when, especially as a child, when they leave their surroundings, it's it's very difficult for them to get their bearings, so to speak them.
Thank you for being here, Number one, Dr. Melendez, it's talk about you again, board certified in pediatric neurology and also an epileptologist, which is just fun to say.
Not that I practice it all the way over here, but in the world of epilepsy.
And I think we talked about this several years back, there was always a child or two in elementary school as we were growing up, and we all knew that if there was some kind of a seizure happening that we would get out of the way, make sure that maybe they were turned on their side.
So there's these trainings that we went through when we were kids, bring that into what it is that you do all day, every day, and what is epilepsy and what is the cause who children, do.
they grow out of it?
Which is something else that was an issue way back when...but maybe talk about what you do all day, every day.
So I can maybe I'll talk about epilepsy first.
So so epilepsy is is kind of a fancy way of saying someone who's predisposed to have lots of seizures or a few seizures.
So like any one of us could have a seizure, you know, if you got hit in the head hard enough, if you had a electrolyte imbalance, you know, with your chemistries in your body, anyone could have a seizure.
But someone who has epilepsy is someone who has what we would call unprovoked seizures, meaning that they just kind of happen out of out of nowhere.
So generally we say that if you have more than two unprovoked two or more unprovoked seizures, you have epilepsy.
If you have one unprovoked seizure and like a very abnormal kind of electroencephalogram or EEG, which looks at your brainwaves, then that would be another definition of epilepsy.
And epilepsy tends to be more common in kids.
There's there's probably a ton of like basic science reasons as to why.
But for that reason, even like, like general child neurologists end up seeing a lot of epilepsy.
So training in epilepsy just gives you a little bit more experience.
I would say managing the kind of like plethora of like different epilepsies that there are understanding of how to read and interpret EEGs, understanding the the kind of role of surgery in epilepsy care.
So on that note, when I think of and maybe we can look at the first seizure, whatever that seizure looks like, I know it's different with every child.
You said there are different types of epilepsy.
When you're looking at children.
Is there a specific age or an around about age when this starts happening to someone who will be a child that has epilepsy and again, different types.
But yeah, and how do they know what to look for?
So the the the kind of scary thing is, you know, epilepsy can start at any time in childhood because it happens for all different reasons.
So one of the most common causes is like a structural epilepsy.
So that can be from a genetic disease, meaning, you know, you're born with something and that genetic disease changes the structure of your brain and predisposes you to epilepsy on a and it's kind of similar note in the structural epilepsy category.
You know, some some kiddos have a very rough time with during birth and either have some kind of some degree of injury, maybe like a hypoxic we call it ischemic injury that can cause structural problems as well as others and that can predispose them to epilepsy.
That would be more like the kind of neonatal period, less than 28 days.
And then there's there's many other causes of epilepsy.
And those are the ones that tend to present later on in childhood.
Those are your what we call kind of idiopathic epilepsy, meaning we don't have a cause for them.
The kiddos have a normal MRI, we maybe do genetic testing.
The genetic testing is negative and, and those can kind of go one way or the other in terms of kind of petering out and not having epilepsy after several years or some, you know, rarely will go on to continue to have it in adolescence and adulthood and so seizure and I'm somewhat familiar with them.
My my grandmother had seizures and it wasn't at all what I expected a seizure to look like, which is why I was asking what it is that a parent can look for, especially in a brand newborn an infant, because you think a seizures and somebody is moving around or there are other seizures where there's absolutely just stiffness, which is about my grandmothers were not that what's happening I had no idea was happening and so for the sake of the audience in trying to describe what they can present as Yeah.
So that we kind of know what happened.
Well they had, you know, two or 3 seconds where things just went wild and then we don't know what happened.
So if you can describe them, I know they're they're very different with every person I get that maybe give us a little bit of of help there.
So, yeah, you're absolutely right.
Actually, the the International League against Epilepsy, the ILAE has a huge list of the different types of seizures that you can have that range from kind of strange ones that are called gelastic seizures.
So those are like laughing seizures to, you know, kind of your more typical ones like you see on TV where you have like kind of stiffening and rhythmic movements.
That's called like the tonic clonic seizures.
So those are those are probably these tonic clonic seizures.
And gtc for short are probably the most kind of common.
And like classic ones that you would see on on TV or in movies, those are quite common.
Other other things that can look like seizure, as you said, kind of like a forced stiffening.
And then there are many seizures that just kind of look like an absence of awareness or loss of consciousness.
Not necessarily any changes in what the person is doing motor wise, but maybe they're, you know, they just stop responding to you or they're staring off into space, or they all of a sudden lose their ability to talk kind of reasonably and start kind of saying that same thing over and over again.
So I would say that it's all over.
It's it's really all over the place.
You know, it's kind of like you just have a little bit, you know, lasts just a couple of seconds.
But he completely loses all awareness falls down his.
Has hit the shower.
You know, it can be something that lasts a couple of seconds.
It can cause a couple of minutes.
Yeah, it can last a few seconds.
A few minutes.
Most seizures lasts less than 2 minutes, But but there are, you know, the more rare cases where they can last long, they can last 5 minutes or longer in those circumstances, that's when, you know, we start to get worried.
And now we're going to bring you in there, Mr. Canada.
Man.
sorry.
We have these fun things before the show starts to get everybody a little bit loose and have fun.
So when it there needs to be intervention again.
Neurosurgeon When I don't know how to ask this question exactly.
So when is it that Dr. Melendez has a concern enough to wear?
It's now time to see what the interventions are going to be and maybe we can start there without going into surgery.
First, What can some of the interventions be with Again, and I know we talked about different types of seizures, but what types of seizures belong to what interventions and then maybe will go into surgeries after that.
Yeah, I know.
That's a great question.
So what we look at is having a proper workup and that's where kind of both of us will get involved originally and little see as far as they should see a specialist because we need to get the appropriate diagnosis.
Is this epilepsy.
If it is epilepsy, are they being appropriately treated with the right medications or not?
If it's not this medication, that's a good response.
Do they need another?
So that original workup will start.
And usually with Dr. Melendez beyond that, we start to look at people that are either refractory where they're still having seizures despite the medications and medications aren't enough, that they're not tolerating the medications.
And they've tried multiple medications, but the seizures are still debilitating.
They're affecting their quality of life.
So then we talk about other alternatives, and those can be surgical or we're not treating the brain itself, although sometimes we have during the areas that are triggering a seizure, we can actually put something in to almost depolarize the brain, kind of disrupt those pathways to stop the seizure, other things like vagus nerve stimulator.
So those are kind of little battery driven with the big nerve in the neck.
And that helps a lot of kids maybe not completely go into remission, but decrease the severity and the frequency of the seizures, which can make a huge difference for some kids that are seizing up to 50, 100, some even 150 times a day, just many, many seizures that they can't even leave the house, which makes a big difference.
The other part of it is where during the workup to find out why they're having seizures, is there something in the brain?
Is there, for example, a tumor is or a bleed is or an old injury.
So if there's some cause or we could target, that was surgery to take it out and have a good response if that's where that seizures are coming from.
So it's a it's a detailed workup involves multiple different people, not only us, but also getting as far as a neuropsychiatric testing, getting as far as something called functional MRI is where we actually for kids that are able to cooperate, doing tasks where we look at their brains and seeing what parts of the brain light up.
So all those are useful information to localize.
Well, this is where the speech area has.
This is where their memory area is.
This is where the seizure might be coming from.
It's affected this or affected that.
So it's not a simple thing.
When they come to see us, we put them through a battery of workup in order to give them the best recommendations to get a better quality of life.
And so it'll be at the end of this as they get their seizures, if not cured, at least better controlled, so that they can live a better life afterwards.
So here's almost a question that I should have asked prior to that.
So to diagnose, to diagnose physiologically in the brain what is happening, What are those tests that you're looking at?
You were talking about MRI, you were talking about different ways of taking pictures in the brain.
But also and I remember now we talked about, you know, the TV shows that you see and there's operations and the person's talking and there's a doctor behind them operating on the brain with all that sticks in my head.
So we're going to talk about that again, just in case people didn't watch the show last time.
That fascinates me.
That absolutely fascinates me.
But to diagnose what type of seizures are occurring, the zappies versus epilepsy, you know, 2 to 5 minutes, how how is that done?
I mean, I know there's the clinical way of watching, listening to what maybe people people around them have witnessed because a person having the seizures often cannot tell you.
Correct.
How I'm throwing that to either one of you Who wants to take that.
The first one is there you go.
How does one really find out what type of seizures are are going on?
Yeah.
So it's hard.
I know.
It's it's it's hard.
It's so yeah, you're right.
Often we kind of listen to the what we'd say the clinical history is like what what the patient looked like, what the parent saw, what the what sometimes what the patient felt, you know, because what some types of seizures, there's very kind of characteristic and what we would call like an aura, you know, like, oh, I felt like like something rising in my stomach, like some nausea or I hear something or, you know, there's these different little kind of symptoms.
So so we we take a very, like I would say, thorough history of what the clinical seizure looked like.
And then often, you know, we'll then get this the brainwave test.
So the electroencephalogram or the EEG where they basically kind of put special stickers all over your head and then you're able to look at the kind of baseline brain activity and not all the time, but often patients who have epilepsy will have some some kind of subtle or or very obvious in some cases, abnormalities on their EEG that point us in the direction of, okay, this this really is epilepsy.
What are those things?
Those are abnormal activity that we call kind of like spikes or sharps that the analogy that we often give is, you know, if a seizure is is the fire, then what you're seeing on the EEG are, are the are the sparks.
Yeah.
So it's just it's evidence that something is building or something could, could build or has been there.
So you know that the EEG we ranges from often it's like a routine, it's like an hour long study and most of the time that's sufficient to get to get the information, particularly if the kiddo, you get them to go to sleep.
But sometimes we do have to do longer studies and I would say those are generally necessary.
And like a surgical patient that you're wondering, like, you know, is this epilepsy coming from one part of the brain in particular that that Dr. Makoshi can remove?
So even if they're not having a seizure episode, the tests are still able to see how the brain is functioning.
So someone does not need to be in.
And I know you said sometimes it's 150 episodes a day, but say it's someone who has three a day and they're in for that hour.
You can still kind of see where the misfiring will be or whatever the wording would be in that case.
Yes, there definitely is that possibility.
When when we're doing the surgical workup as part of that workup, one of the things that we aim to do is actually get someone capture someone we say on EEG as they're having a seizure.
So we see exactly, you know, which part of the brain is it coming from or is it coming from kind of all of the brain at the same time, knowing if it's focal meaning, it's coming from here or here versus generalized meaning?
It's coming from kind of everywhere at the same time is very important because it tells us basically whether whether someone's a surgical candidate, what type of surgery may be offered, because you can offer surgery to either and even, you know, backtracking.
Also, what type of medication would be best for that patient?
That's a perfect segue way because that that's my next next question.
I know surgeries down the line, but I feel like with a lot of these programs, we wait to see what the other interventions are.
So we talked about refractory means.
The seizures are still happening even on medication and seizure prevention medication.
And I know there's a lot out there when and how and what is it that we are giving to individuals?
Does it depend on the age?
Does it depend on the severity?
I think dosages are like that, but I don't know if there are a lot out there.
We don't have to say the names of them.
But just just in general, when someone is newly relatively diagnosed with having seizures, what is the next step from there?
And again, I'm throwing that out.
Dr. Melendez, When we talk about cutting the brain, you can take out with the cutting.
I'm going to put him on the hot seat a little bit because we're going to make a TV show out of him.
Not that we aren't already, but as far as the medications go and treatments in general, how how do you start and do you tweak that as time goes by?
Yeah, absolutely.
It's the latter question about tweaking it.
So so you choose medications based on a lot of things, based on age range, you know, where you're treating the seizures based on the type of seizure, whether you think it's focal or generalized.
I'll give you an example of both.
So there's there's a lot of literature, very kind of famous literature that shows that in like neonates, for instance, we use a really old school drug called Phenobarbital, which has been around for over a hundred years.
It might be exactly 100 years, not a very nice medication, to be honest with you.
Lots of side effects.
It is very, very sedating.
But it's it's it's unequivocally the best for that age range.
And they tell me the age range, again, that's typically like neonatal seizures.
So a young young.
Yeah, Yeah.
Like, like like kiddos who unfortunately experience them around birth.
Okay.
And then I mean, there's there's a like a slew of other medications that we have to offer.
And again, there are some that are better for focal seizures or some that are better for generalized seizures.
There are some that are more kind of powerful medications that are that are good as we move like into that refractory epilepsy category.
As you know, kiddos are moving through all the typical ones and we're needing something that's a little bit more, more powerful.
All of those tend to have, you know, as you might imagine, like worse side effects or more scary side effects potentially.
Okay.
So so there's there's a lot to choose from.
And then there's also, you know, kind of specialized medications for certain seizure types, like very, very special ones.
So like patients who have tuberous sclerosis complex and a C, which is a genetic disease and seizures.
Because of that, they should get like above trend patients who have infantile spasms, which is another kind of infantile epilepsy.
They should get some type of steroid generally.
So some hearing when you said tubular that I said that right.
Sclerosis, there's some kind of scarring in areas of the brain.
And my understanding that correctly?
It's that's that's a very interesting disease where it's actually they're called tubers, tubers that grow and they're almost like benign tumors that kind of grow everywhere in the body.
There's potentially but I would say like the most part in some kind of aspect is, is that they grow in the brain and they're very epileptic genic.
So they have a very high potential to to cause seizures.
Okay, That's a good transition to now into brain tumors, which is I know a specialty that you're like, Yes, yes, it is now my turn.
So let's let's do talk about brain tumors.
And again, just to say the words brain tumor is scary.
But there are some and I remember is talking about it last time, that there are some that are not so scary.
You can take care of them and there's others that are pretty dang scary.
Talk about the gamut for us.
I mean, brain tumors, any time you're talking to any parent paralyzed with fear, Oh, of course, you just hear the words like so it's important when we go through it.
Is that you want to be confident that you're going to be treated the best possible treatment for your child with the best possible outcome.
And that's what we aim for.
It's hard to say that all tumors are going to have fantastic outcomes, but we can aim for the majority because we try and diagnose them early to catch the symptoms, get the appropriate imaging to know exactly where the tumor is and what type it is even before we offer surgery and then discuss with the family so that they're prepared to know, well, this is what we think it is.
This is what we can do to remove it safely so that your child recovers from this.
Sometimes they can end up being cancerous tumors that need other types of treatment.
So the mainstay of treatment is to remove all of it, to do the best we can at the beginning.
And some will need as far as chemotherapy and if they're a bit older, radiation, other tumors are more not as cancerous or benign, or if we take it all out, then it's a cure.
So once we've done that well enough, people are very scared of brain surgery.
If you're going to operate on my child's brain, are they going to be the same?
We always try and go in and it's hard.
We never can make promises to say that we're always going to be 100% fantastic.
But generally in pediatric surgery, when we're going to operate on someone that's coming in, well, the aim is that your child comes out of the surgery the same or better.
And that's the main idea, is that we've removed the tumor without hurting your child so that they can live the rest of their life and do fantastic.
So the majority of our kids are going back to school, going back to living their lives.
You wouldn't even know that they had brain surgery.
So it is scary and it's going to be scary to anyone.
But it's important to know that the best treatment is available.
And that's what we're going to offer with everything that we can from the best modalities of imaging, from intraoperative monitoring, where we have a whole new operative muttering.
So we actually as far as monitor the function of the brain around the tumor to make sure we don't go through any areas that may cause injury to the brain.
Is that that segue way?
I've been looking for?
Sure.
Okay, there it is.
Because when the person is child and I guess this is also age related to some of these able to communicate with you what is happening.
So when you say being able to know with the functioning of the brain is as you're operating, talk about that because that again, it almost seems sci fi and I and I know I've seen TV shows, I've seen movies, but talk about that in a realistic format so that we can know, okay, the arm is moving, can you speak, can you say this word correctly, etc.?
Can you hear this?
Can you you know what have you.
Take that a little bit and I'm joking about it.
This is really no joking matter.
And I and I completely respect that.
But again, it's fascinating to me because your brain operates your little toe, you know, how often do we connect those two things?
But we do it matters you.
So I'm glad you take that anywhere you'd like to take it.
So thank you for laughing.
I like you.
And you were worried about this, right?
See, we're having a good time already.
I'm just amused and it's an important question is that and I'll go back to where parents are always afraid of brain surgery because you're afraid of what you don't know, which makes sense.
And that's where I come into explaining that.
No, the whole idea of doing surgery is to do it safely, not to injure anything so that your child comes out walking, running, playing, going back to living their lives.
So there's a few ways to do that.
Sometimes in older kids, when they had two teenage years and definitely in adults, if it's in the area that's next to the area that control speech or movement, we can do a week surgeries.
It sounds scary, but actually patients do quite well, will play music in the background for them.
They can sing along if they want to.
They wake up and then we actually stimulate with a little electrical pulses around the tumor to find out.
Alright, this area controls speech.
Different areas can control different things.
This areas English, this area, Spanish.
So it's even more complicated than that.
This area names, objects, this area names, animals.
So it gets very detailed.
In fact, what you can do with awake surgery.
So it's rather fascinating.
And that keeps us safe to know you're going to leave this surgery removing as much of the tumor, if not all of it, while safely preserving that function.
And that's for older people that can tolerate kids.
I don't have that luxury sometimes when they're too young.
So we have other correlates where we have our monitoring and we actually stimulate the same area of the brain.
And on a monitor we can see if we have as far as stimulated their hand or their leg.
Interesting.
And that gives us that feedback that we're very close to that.
Now on the monitoring, are you seeing that in the brain or are you seeing that physiologically with their hand or foot?
We form a circuit so we stimulate here and you have as far as little needles that are sitting in the muscles, in the nerves.
So if we stimulate here, naturally that fiber goes all the way down to, as you said, your little toe and you get that little bit of movement and they see it on the monitor.
So we know that's the area that controls that.
We can stay away from that.
So there's a lot of things that keep us safe.
And that's what taking away the scary to say, no, we have these abilities to do this safely and that's that's the main goal is to do these surgery safely, to give the best outcome for the kids to go back and live their lives the way they should.
Right.
Exactly.
So when we're looking at I know we're looking at brain tumors and all kinds of other neurological issues, we're looking at brain vascular malformations to.
I do I know we're talking about the tumors, but when we're looking at vascular issues, because you've got a lot of threading of in there, talk about some of these malformations.
Are these usually something that is congenital, something that people are born with?
Is it something that's developed over time?
How would you how would you define a vascular malformation in the brain?
So vascular malformations for the brain or anything that involves blood vessels or blood.
So there are a few congenital and we see a lot more of them here in El Paso than we do in other regions because there are some genetics for little pools of blood called cavarnomas.
that have a gene associated more commonly in Hispanics than otherwise.
So there is a mutation that could run in families.
So I get a reasonable number of patients that are coming through that have multiple of these small little areas of blood.
Doesn't mean that they're going to have any symptoms.
But that's a discussion that we have to have with the family.
Some of them can cause bleeding repetitively and need to come out because they can cause symptoms, they can cause seizures.
And those are the ones we and for others, we just watch and they behave themselves.
How do you know to look for them?
So is this something where something an episode must have had happened for you to say, okay, we need to look at this?
Usually that's how it comes to either they've had a sudden headache, either they've had a new seizure either because if it's big enough, it's caused a problem with speech or movement, and then they get a scan.
Some of them are incidental where they just are playing sports, they fall, they get a scan and you see them or they're getting scanned for a different reason.
So there's both of them are a possibility, and I've seen both come through the clinic.
Goodness gracious.
Okay, so now we're at this point and you see what you say.
Somebody fell down.
They had a concussion and you're looking at the image.
And the image does show these different pools in there.
Now there's a discussion with the family.
What is that discussion?
What does that discussion exactly?
And that's where as far as we need the family to understand that there could be a genetic component and testing for genetics to know also the risk for the rest of the family.
And important to know if there's something that's going to run the family.
Well, we found one person with it are the siblings or as far as if they're going to have kids, do they have a risk, too?
So the information is power.
We need to know what's going on and then to educate them on what to watch for.
So if you're having a sudden headache that you hadn't had in the past, it's important to come to get a picture instead of waiting for a while with this headache to go away and they're getting worse or causing a seizure.
So education becomes a primary thing is what do you have in your brain?
What are we going to watch for and when are we going to need to do something surgical?
So having that education gives the patient's power to know throughout their life what they need to watch for having that discussion to keep them safe.
So, Dr. Melendez, I have for you one of your topic points, because I feel like this is a perfect segue way into this as well, is neuro genetics, right?
So this is kind of the same discussion, but taking it to your view of how that testing is done, you talked a little bit about it, touched base on it.
When do you think the testing should be done within certain families, etc.?
Yeah, I guess it depends on on why why you're doing the testing.
Often I think we're trying to do testing for for similar things.
So I think as you know, as time has gone by, we found that there's lots of genetic causes for different diseases, genetic causes, meaning, you know, there's some change in your your genes that, you know, make you who you are, make your make your hair red or, you know, blond or whatever.
And those same changes can cause sometimes seizures or they can cause these vascular malformations, sometimes they cause intellectual disability, or sometimes they can cause problems with the muscles and for a long time, we you know, we did genetic testing because often not just us, but families really wanted to know know why they want they want an answer.
You know, even if you couldn't, it didn't change your treatment.
It made people feel better to know why this happened or, you know, it may help inform you, you know, what to look for in the future, what your kiddos going to look like, you know, 15 years from now.
Are they going to be independent after high school?
Like what?
Or am I going to have to worry about, you know, progression getting worse?
So so for a long time, that's why we did the neuro genetics and that was the important piece.
But over the last, I would say like a decade, maybe more or less, there have been a lot of like really, really amazing things that have been done in pediatric neurology in general, but definitely in pediatric neurology that have like revolutionized what we can do for kiddos.
One of the kind of most, I would say, important things that genetic testing has has given us is it's helped us to make like targeted therapies.
So I don't there's, you know, kind of very specific muscle diseases that many people have heard of, maybe like Duchenne muscular dystrophy or spinal muscular atrophy.
These are diseases that, you know, you used to to kill children and now there's therapies that that can give them essentially a normal life or a much better quality of life.
When you say the word therapies are you talking about medication therapies?
You talk about physical therapies, a combination of the two.
I'm talking about gene gene therapies, Gene therapy.
Okay.
So right with that is so gene therapy is basically an option for different sets of diseases where you have essentially a malfunctioning or poorly functioning or you don't have the gene, you don't have the proteins, and it's not doing the things it's supposed to be doing for the body.
So in some cases, you know, if the if the gene is amenable to this, then you can basically replace it.
So often, you know, you're giving patients a lumbar puncture, you know, where you're actually accessing this, that the fluid around the brain and injecting like a medication.
Sometimes rarely you can you can just give it in the veins.
But it's it's it's pretty amazing, right.
It's like science fiction except it's now.
How long has this been a way of treatment?
for this type of therapy, neuro genetic therapy?
I mean, it's it's relatively it's relatively new.
So like, the first big one was for Spinal Muscular Act or I would say not the first big one period, but in our realm of pediatric neurology, the first like real, I would say life changing one is probably for spinal.
Sorry, spinal muscular atrophy, SMA.
Okay.
And which is a childhood disease.
And I want to say that was in in the last decade.
Oh, and it's only recently taken off because of access to the therapies.
And then last year, actually, the FDA approved a gene therapy for Duchenne muscular dystrophy.
You said that before the show.
Describe what that is.
Duchenne muscular dystrophy.
I know muscular dystrophy, but this is a specific type, a branch of or.
Yeah, yeah.
So so yeah, there's all different kinds of muscle problems that you can have that cause weakness, that cause, you know, bad worse things down the line.
But Duchenne muscular dystrophy is just a very specific type that cause that's caused by a mutation in a specific gene, and it's almost exclusively found in boys because of how the genetics are are passed on from generation to generation.
And it's it's I mean, it's fatal.
Unfortunately.
You have kiddos who who might be a little bit slow and you know, when they're toddlers and then they're able to obtain milestones but those kind of go away rapidly.
And if if we know you know now we have we have a treatment for it.
What is the demise the atrophy itself of the muscles?
yeah, and I know I'm being physiological, but it's just it's a blunt question, but by age and I'm trying to think of a like what it looks like, what it looks like a growing child, the muscles.
And it's just that the muscles don't develop.
Is it that they develop and then?
So they're the, the muscle tissue is basically disintegrated, replaced by by by fatty tissue initially.
And then it's the structure of the muscle itself is is not it's not it's not well formed.
Okay.
So, so and then it kind of just worsens in integrity over time.
And so that's why, you know, these kids are able to, you know, most of them can walk and kind of looks like they function relatively normally when they're younger.
But then as time goes by, you know, they're they're unable to walk.
Maybe they're unable to breathe as as well, they're unable to eat as well.
So, you know, I'm asking you this not to be icky about it for the lack of a better word, but when you're talking about these new ways of treating I mean, we're talking about neuro genetics.
20 years ago, the picture looked a lot different, is what I'm understanding from you of how some of these treatments have come into play.
And you said earlier science fiction, right?
I mean, sometimes you see and you just I talk about this when it comes to the world of research.
And people often have fundraisers for different organizations that are researching, researching.
So this coming in to from neuro genetics, from researching this, would you say that there is a population of 20% of children are able to now carry on and grow into a full lifetime or not?
And if I'm being difficult, we can we can we can change to a different subjects.
I know we've got a lot of neuromuscular diseases that we that we're talking about, too.
Yeah.
Yeah.
I I'd be hard pressed to tell you percentages on.
Yeah, it's a hard subject but because most of these are somewhat like rare conditions.
Okay?
Even though like muscular dystrophy is, is very common, still a rare disease.
But, you know, it's it doesn't matter if it's rare or if it's if it's your kiddo.
Yeah, exactly.
Yeah, exactly.
I kind of don't know where to segue from here, but I do want to talk a little bit more about the brain and how the neurons are shooting within every sector of our body.
And, Dr. Makoshi, I'd like to talk a little bit more about brain surgeries now.
So going back to epilepsy, and you said that there's a certain point to where if there is a refractory type of epilepsy that is not being made better by any kind of medications, where then would you as a surgeon, go in and perform some kind of a surgery?
What do you start looking at there in the world of epilepsy?
So that's a perfect question.
Taking it back even a step before that, we know that anywhere from about 1 to 5 or one in ten will have medically refractory seizures and need something more.
Otherwise they just continue to get worse.
The problem is, is that sometimes whether pediatricians or even neurologists are sometimes unaware or hesitant to refer to an epilepsy center because some people either don't know that it exists or are worried that they're going to go there, they're automatically going to get surgery, they're going to cut into my child, which is not the case.
The whole idea of going to an epilepsy center is getting a full workup in order to give the best recommendations to the family.
This is what your child has.
This is what we think the cause of the seizures.
And these are the options for treatment and how they may help your child to live a better life.
That's the whole idea is to get a full assessment.
So what we try and do, we have the epilepsy monitoring unit that's opened recently in El Paso Children's, which is fantastic because that's a dedicated unit where we can monitor the kids to catch enough seizures to hopefully get enough information.
Dr. Melendez looks into the EEGs, interprets for us and tells, we this is the abnormality.
It looks like we have one area.
Do we need to look at it more closely?
And sometimes that's where again the rest of the workup with the imaging will say that in this specific area of the brain we see some abnormality.
This is the cause of the seizures.
It's unclear.
Sometimes we need even more workup.
So we have what's called stereotactic, and that's using our little navigation system to put little electrodes into the brain and within even less than a millimeter, actually find out exactly where that seizure is coming from.
Oh, my goodness.
Now, this used to be an issue for kids that need to be referred to other centers because you can imagine if you're a big center, are getting referrals from all over, the waiting list can take forever.
So these families are waiting with seizures and can get this treatment just to find out where the seizure is coming from.
Are they a surgical candidate or are they not a surgeon?
What's going to help my child?
So we're happy to have it here so that these children don't have to travel anymore so that we can take care of them and do extra step if we need to it again, get those answers.
So walk us through what this looks like.
We have a child now and you said within millimeters, how they have electrodes on their head, brains and you're able to watch on a monitor as different things are happening.
Just walk us through what that visually looks like, what you particularly are looking at.
I'm not looking at it.
That would be Dr. Melendez.
Okay.
Then to be looking at pretty similar, look at it all.
So that's a lot pretty similar to getting a very accurate instead of putting it on the scalp.
Or you can imagine if you're putting little leads on the scalp, you're kind of getting that end results as far as that electrical activity.
Okay it's starting from a little area.
It's already spread to a larger area by the time you catch it at the top.
So if you're putting in little leads in different areas of the brain now, you can know exactly which lead it's starting from and you're moving them in real time.
You put them in before.
So what we do is we'll plan out the areas of interest based on the first EEG, the ones that we put on the scalp to say it looks like it's mostly coming from the right side.
It's mostly coming from the frontal lobe or the temporal lobe or the parietal lobe.
And then we need to put the electrodes more into these areas.
So the number of electrodes that we put anywhere that we put them in depends on what we find on the imaging, what we find on those scalp edges that helps guide us.
Sometimes we don't know when we have to put a larger number to get more information, but usually we can identify to say it's mostly on the right, mostly on the left, and which lope it is.
And that way we put in the electrodes into different areas and those are electrodes similar to the EEG, then just have wires connected to a machine that catches the activity.
It adds another benefit.
Dr. Melendez can then come in as far as stimulates or in areas of the EEG and find out exactly what function that area of the brain does.
So not only can we know where the seizures are coming from, is this area important for a critical function where we shouldn't remove it with surgery or no?
Is it a quote unquote silent area or if we take it out, will take care of the seizures and not harm the child?
So this is like a very tagged team type of a situation.
Yeah.
So here's a question we heard about 15 minutes before the end of the show, and this is this is where I usually stop all my questions say, is there anything you want to talk about this, even though we haven't yet, But what I'd like to do prior to doing that.
So just think of some questions.
You want some questions you want me to ask you before that, However, what do you see?
Like you said this a lot of the stuff with neural genetics is is a year or two or three years old.
What do you see 5 to 10 years now going forward in?
Because I feel like there's so much that is like on the forefront of happening.
What can you to the best of your ability?
Yeah.
And your wishes, perhaps also ability, but realistic wishes, best educated guesses, wishes.
Do you see happening in the next 5 to 10 years?
I guess I'll I'll speak to maybe general pediatric neurology and epilepsy and then try to make it more specific to us in El Paso.
Okay.
So I think, you know, in the next few years, the the the hot thing that's going to continue to be to be big is is this genetic testing getting answers And then, you know, at the same time as, you know, we're diagnosing what the patients have, the basic scientists and clinical scientists are working to come up with with more kind of specific cures, or we would call them like precision precision therapy.
So whether it be gene therapy or special medications that are for that for those particular patients, you know, with like this particular gene that's that's different or this particular epilepsy.
So that that field is is is just growing.
A lot of the pharmaceutical industry and other kind of companies have really I think, invested a lot in that because they they realize the potential and how important it is and how life changing it can be for people.
Right.
Like I said, can kids use to to die from some of these diseases and now and now they can have an essentially normal life, which is amazing to see.
I so I think that that's going to continue to be very important.
I think specifically here here in El Paso, I think that we are trying to do a lot more genetic testing.
And there are certainly, I would say, you know, sometimes it can be tricky with with insurance and and kind of resources.
But but, you know, people are are working hard, I would say, to kind of surmount those obstacles.
And then there's also assistance from from other like kind of outside programs who who understand the importance of the testing.
So I think that that's going to be very beneficial because then it allows us to to bring those those precision therapies to the kids in the community who previously, you know, it was it was limited because you didn't even know what they had.
Right.
So so that's that's amazing.
And then there's a lot of work that's being done in an epilepsy surgery.
And there's there's a lot that's done for adults and even more in the like the last few years for pediatric epilepsy surgery in particular for the use of these like devices that you can implant in the brain or in the nerves and, you know, help seizures when you can't, you know, resect or like remove the tissue.
There's a lot of data that's coming out on the utility of those devices.
So I think that that's going to be that's going to be super exciting.
And that's, you know, just like another another another tool that we have to to help kiddos with epilepsy and.
Makoshi, that's the perfect.
So we're going to talk about brain implants now and.
Okay, no, and that's a that's a perfect discussion.
Yeah.
So, you know, we talk about remember when the pacemaker was what you're going to put that inside your body and it's going to monitor thing, you know.
So this is not that far off from when the first person had the pacemaker that was the size of a hockey puck.
But let's talk about some of these implants that help and they're actually similar batteries.
So as far as well then I guess that need to be replaced every few years and they're working on that too to find a rechargeable batteries as well instead of having to do surgery 5-10 years down the road.
So there's a lot of good work in progress for the implants to stay implanted and need to be revise that having another surgery.
We're still a little time away from that, but I'm hoping soon that it is rechargeable that we don't have to do surgeries, but there is different type of implants.
There is one of the oldest ones that we talk about with the vagus nerve stimulators, which is kind of the catch all for all epilepsies that may not be responding to medications.
So it's a little incision in the neck and the electrodes that sit on one of the biggest nerves in the body, the vagus nerve, and then a battery that's implanted into the chest.
And there are smaller batteries.
They they keep getting smaller and smaller, which is nicer for the younger kids.
And what it does is it gives as far as these stimulations to interrupt the pathways and thereby stop seizures or decrease the frequency or the severity of the seizure.
They have gotten more advanced.
Or if you notice that your child is having a seizure, you have a little magnet, you can give an extra boost to stop the seizure.
So a lot of these changes have happened over the few years and we're just getting smarter and smarter to treat it better and better.
What I'm going to do is stop you just for a second, because this is so important.
I think that we kind of got to it late last time.
Explain with the vagus nerve does.
Why is that?
The the the the the important nerve.
And then I'd like to continue with this discussion.
So I feel like if we don't understand what that is, then it's hard to understand why it's implanted by the vagus nerve.
The vagus nerve is one of the biggest nerves in the body that connects with multiple things below the neck where the majority of the cranial nerves, the ones from the brain, sit inside the head.
Not only that, it has the most to relay of any of the other nerve where it's connected to so many different pathways.
So it seems to be both in location and how many connections.
It has a perfect target.
If you're going to try and neuro modulate the brain to change it.
That's the one to aim for and that's what they try and it seemed to work out.
There are still theories as to why it works, and we're getting better at understanding it, but we don't know for sure, aside from it has so many connections and that may be why.
And the vagus nerve stimulators are one of the few things that if you have a good response after a few months, your response is actually better at a year, two years and five years.
So you get even better benefit over time.
So it's one of those things that over time you can show more improvement.
So that's the beauty of as far as using the vagus nerve.
And one of the benefits of if you're stuck against a wall with medications, there are sometimes options that may help your child.
That's important too.
So I know vagus nerve.
I'm that person that has her blood drawn and likes to faint.
And I remember somebody said, Well, that's a vasovagel thing.
I'm like, Wow.
And that's the first time I heard about the vagus nerve.
And I didn't understand it.
I still honestly don't quite understand it.
But it also fascinates me that this is a nerve that affects so many different things.
In your experience, Dr. Melendez, we talked a lot about the brain, but we haven't really.
We're kind of just getting to the vasal nerve right now when the parents come to you and there are questions again, I feel like I'm going back to the beginning of the program.
But when parents come to you and say, you know, I'm noticing that there are, you know, 30 seconds a minute or two.
My child is clocking out.
And again, it's going back to the beginning.
But what is it from there that you have the discussion with the parents?
Like what what leads you to that first test, if you will, to find out what's happening?
I mean, really listening to the parents and and taking a good history, which is, you know, basically just knowing what questions to ask.
And again, I'll say it again, like listening to what your what your family say, because, you know, often like they're your kids.
Right.
So and you notice every Yes, you notice everything.
So depending on what the parents say, like your your example of a staring off a common kind of seizure type that we see in kids is what's called absence seizures.
So those are like the mini kind of seizures where you just stare off for a little bit and then you're okay and last a few seconds.
And those have kind of very different treatments and very different causes of them or ideologies.
As someone who's staring for, say, like a minute or two, right.
Which in that case I would I would start wondering whether they actually had like a a temporal lobe epilepsy, which is which is very different from the absence kind of seizures and the associated epilepsy.
And so really it's asking the history and history and if they are relatively short lived.
So I feel like, you know, with my son again, we there are seconds at a time.
We just think that we didn't know what to look for, what to ask for until he actually fell down in the shower.
You know, it was one of those things that we just like.
And again, it well, "I had a zappy."
He's like, What do you mean?
He had his zappy.
You know, he's a full on teenager at this time.
He's he's older now.
But and I'm saying that for people to ask for some help if they can and maybe there is a website out there I don't ever encourage people to do doctor Google because you can get scary and you go down the rabbit hole that you don't want to go to.
But again, if you're watching this right now, feel free to call the El Paso County Medical Society or info at the El Paso County Medical Society and ask your question will make sure that a medical person gets back to you with that.
On that note, I would love to maybe open it up to something that you want to talk about this evening.
We didn't quite get to or something you'd like to talk a little bit more about.
And the veteran always gets to go first.
Since you've been here once before, what is it that you'd like to talk about this evening that we may not have touched base on?
I think the only thing actually is mostly just to reinforce, okay, because we know that a large number of people just aren't coming to see us.
Yes.
And that's kind of that point of mean.
They do that.
Exactly.
And what what's been shown is that even the kids that are referred to epilepsy Centers for Assessment and Management that don't end up getting surgery still do better than those that haven't been referred for epilepsy assessment.
So just because we're coming to see as far as an epilepsy neurologist and an epilepsy neurosurgeon doesn't mean that surgery is going to be the right thing.
Sometimes it is Sometimes it's a fantastic the best option for a child, sometimes it's not.
But knowing that and knowing what to change, whether with medications, with regimens, what to watch out for that education from getting that full assessment, including your child has issues with memory specifically so they get a benefit for taking more time with this with language, for example.
So they may need to pay attention to this.
So just getting that full assessment is useful to parents.
So it's important if you think your child has epilepsy or they do have a place to get assessed and you don't need to travel to as far big centers because you were trained at big centers and came here to offer that to the patients.
So I'm going to throw this out as a plug, but it would be the regional pediatric Epilepsy Monitoring unit.
So let's say somebody is listening and they're like, Oh, who do I call El Paso Children's Hospital?
And then again, that is where something you can put into the search engine if you have a phone number.
Great.
I didn't ask you ahead of time.
So of course you're not prepared because all of us just have everything on the phone.
But again, it's called the regional pediatric epilepsy Monitoring Unit, and that is where the magic happens.
We try to figure out what is happening going on in the monitoring.
Monitored monitoring is great.
Anything that we haven't talked about on your plate, Dr. Melendez.
I know we covered a lot.
It's an awful lot that we have, and I almost want to cry.
When you were talking earlier.
Oh, yeah, But you're right.
I mean, these things are very rare.
Until it's your person.
You know, if it's your child, then it's it's not rare at all.
It's what you deal with all day, every day.
Is there anything we haven't touched base yet that you'd like to talk about?
No, I guess I have.
Dr. Makoshi She had a I mean, I, you know, second everything that he said, but.
But I do.
I'm very excited for everything that they're doing at El Paso Children's and hopefully everything that we're trying to build.
Because.
Because yeah, it stinks to have to leave to go out of town.
It stinks to leave your family, your other kids, your job, that is, you know, giving you your insurance to pay for this place.
Right.
And yeah, I think that, you know, we as as definitely as as a team and with with the rest of the children's hospital and members of UMC and Texas Tech like like Dr. O'Neill, like Doctor Dr. Lopez, I think that we're we're building something where we're really going to be able to to help the kids with within this not just El Paso, within the region.
And, you know, something that can well last.
There are three different places that you can go to watch us again.
One is PBS El Paso .org, and you would find the El Paso physician on that website.
And you can go there and you can see this as well as other shows that have been aired.
Also, the El Paso County Medical Society, which is EPCMS.com EPCMS.com.
And then also YouTube, you can almost find anything and everything on YouTube.
It's a little scary.
So I thank you so much for being here, Dr. Melendez and Dr. Makoshi I just like seeing your name.
Dr. Makoshi and again, any time I watch some kind a show when they're operating on the brain and somebody is lifting a bell, I will tell you, I think about you every single time.
But pediatric neurosciences in the Borderland was our topic this evening.
Thank you so much for joining us.
I'm Kathrin Berg, and this has been the El Paso Physician.
Good evening.
I am Dr. Allison Days, a past president of the El Paso County Medical Society, the El Paso County Medical Society has put on the El Paso physician TV program for the last 26 years.
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