There was a great video once of Michael J.

Fox, like, basically being on a talk show and taking a break and then taking his medicine and coming back out afterwards.

And the improvement that you get from carvedopalevodopa is so dramatic.

I tend to ask these patients, what is your dose?

When did you last take it?

And when are you due?

And do you have it with you?

If so, you know, just let the nurses know you're gonna take it.

If not, I'll order it from the pharmacy now because it'll take a couple hours to get there.

So the more you get out ahead of this, the more you can can make sure that these people stay in the happy place.

Hi, everyone, and welcome to another episode of Amplify.

I'm your host, Sam Michoud.

Before we dive into this month's episode, I wanna say thank you for joining us, and I wanna remind you that you can go to ebmedicine.net where you will find our three journals, emergency medicine practice, pediatric emergency medicine practice, and evidence based urgent care, and a multitude of other resources like the EKG course, the laceration course, interactive clinical pathways, just tons of information to support your practice and help you in your patient care.

And if you're not a subscriber, try us out, and we will meet all of your CME needs.

And now let's jump into this month's episode.

Alright, ladies and gentlemen.

Welcome back to the podcast.

I'm Sam Ashu, one of your hosts.

And on the other end of the microphone is doctor T.

R.

Eckler.

Could not be more excited to talk about all of these rare and interesting actually, maybe not as rare as I thought, neurologic conditions.

Yeah.

For sure.

And today, we are having another episode of trivia with TR as we talk about neurological diseases in the emergency department.

This is the May 2025 issue issue of emergency medicine practice called the emergency department management of patients with complications of chronic neurological disease, specifically, really just three neurological diseases.

We're gonna talk about Parkinson's, myasthenia gravis, and multiple sclerosis.

And I'm sure if you're listening to this podcast, you know someone with at least one of the three of these.

I have a family member with Parkinson's.

I have multiple friends with multiple sclerosis.

I don't know anybody with myasthenia gravis, but that doesn't mean it's not an important disease, and we're gonna cover it today.

But before we do, trivia question number one.

Are you ready?

I'm ready.

68 year old man with Parkinson's disease is brought to the ED with chest pain.

He missed his morning medications, and he's been waiting in the ED for, drum roll, please, eight hours, which would never happen in your ED.

I understand, t r.

But just in case, we're just gonna set the bar there eight hours.

And while attempting to stand, he falls and sustains a subdural hematoma in your emergency department.

Which ED management principle was most likely overlooked in this scenario?

So we're not casting blame, but if you were gonna pick one, what would it be?

A, you didn't give him aspirin for chest pain.

B, you should avoid anticholinergic medications in this population.

C, you should evaluate troponin levels promptly and not let them wait for eight hours, d, maintaining the patient's Parkinson's medication schedule is important, or, e, performing a head CT on arrival for all Parkinson's disease patients is the critical piece that you missed.

Given how hard it is to get a radiology read these days, I'm not scanning every Parkinson's patient.

Not a chance.

Wrong reason, but good choice.

My chest pains are more often GERD in this stressful time, so I actually am struggling to make sure I give all my appropriate chest pains aspirin.

Because I think if I gave every chest pain aspirin, I would make so much GERD so much worse that I just can't do that.

But I will tell you that of the few medications that I worry about, Parkinson's medicines are so important to time well because the improvement it gives the patient in their stability and their movement and their coordination is just, it's remarkable.

There was a great video once of Michael J Fox, like, basically being on a talk show and taking a break and then taking his medicine and coming back out afterwards.

And the improvement that you get from carvedopalevodopa is so dramatic.

I tend to ask these patients, what is your dose?

When did you last take it?

And when are you due?

And do you have it with you?

If so, you know, just let the nurses know you're gonna take it.

If not, I'll order it from the pharmacy now because it'll take a couple hours to get there.

So the more you get out ahead of this, the more you can can make sure that these people stay in the happy place that is their their peak and trough.

Yeah.

Absolutely.

And that is really the perfect answer to the question.

Well done.

The ideal is to keep them on their regular schedule.

That's anybody with Parkinson's disease.

And Parkinson's, as the article authors point out, is really more often a comorbidity.

It's not typically the reason that they're presenting to the emergency department.

It's not something you're gonna diagnose in the emergency department, but it is a frequent comorbidity in the elderly in that kinda Medicare geriatric population.

And carbidopa levodopa is the most frequently prescribed medication in the way of treatment.

There is no cure for Parkinson's disease, and the disease is caused by a central deficiency of dopamine, which leads to all kinds of symptoms, including things like rigidity, poor movement, poor balance, puts them at risk for falls.

And unfortunately, carbidopa levodopa is something that can wear off while they're in the emergency department, and then they can become more symptomatic.

And that complicates matters because now you're dealing with a patient who came in for some other reason, whether it's chest pain or ulcerative mental status or confusion or UTI, and now you've thrown in these other symptoms, and it becomes unclear.

Are they worsening because of the UTI or infection, infection?

Or do they just need their medication?

So getting that information upfront and making sure they stay on their schedule, critical action.

Well done.

I also think just as a an aside, as I learn more and more in this job, I used to just think of this as, like, a stability and a strength thing related to their Parkinson's.

But the autonomic dysfunction that they develop and the nature of the lability of their blood pressure is something that especially as you talk about when they get, you know, some kind of illness or some other thing, like they're overdosed on their heart failure meds and they're too dry, that that is exacerbated by the autonomic dysfunction of their Parkinson's.

And they're more likely to syncable episodes.

They're more likely to have their blood pressure run high, and they'll take more medicine than they should have, and then it runs real low.

So you gotta be cautious in the blood pressure on these people.

And I think orthostatic vitals on these people are a great thing to think about just so you can have a sense before you try to safely discharge them as to if they're gonna fall because they're unstable or if they're gonna fall because their blood pressure's gonna drop through the floor.

Yeah.

And it you know, they get this dystonia, which can often be mimicked by dopamine antagonists.

So, you know, we used to think of things like typical antipsychotics, but now there's evidence that even the atypical antipsychotics and even some seizure medicines like valproic acid and antiemetics like metoclopramide, some of those can actually cause similar dystonia.

And so in an elderly person who has dystonic symptoms, you may end up seeing that initial presentation of Parkinson's, or it may be a medication side effect, or it could be actually Parkinson's made worse by a medication that's been recently prescribed.

I thought there was a excellent case example in the article about an elderly patient who was recently prescribed metoprolol, because it turns out beta blockers can affect dopamine.

Not very much, mind you, but in somebody who has Parkinson's, that may be just enough to tip the scales and make their symptoms worse.

And so it's just as important to get the history about their medication dosing schedule as it is to ask about new meds and new prescriptions that they've started.

The carbidopa levodopa is the most typical medication we prescribe.

It's a combination of two medicines, levodopa, which increases dopamine levels, and carbidopa, which prevents the peripheral conversion of levodopa to dopamine so that you don't get peripheral effects.

It increases the levodopa conversion in the CNS, so carbidopa doesn't cross the blood brain barrier, and levodopa does.

So levodopa will go into the CNS and get converted to dopamine and therefore help with Parkinson's and the symptoms of poor quantities of dopamine in the central nervous system.

That's how the medication works.

And it comes in short acting and extended release preparations, so it's important to know which ones your patients are taking.

Sometimes people will take this once or twice a day.

Sometimes they will take it up to five times a day.

And so it can be pretty easy to miss doses in a emergency department visit, even if it's just for a couple hours.

It doesn't have to be an eight hour stay.

They also mentioned that some cases for severe patients, they'll they'll basically administer it continuously by NG tube.

Yes.

So this where you've got a patient that's got an NG tube that's been dislodged, and they seem like they're starting to have symptoms that are getting worse.

It's something to consider that they they could have been getting this, and then they can get a withdrawal syndrome from basically not having their carbidopa levodopa, which is called Parkinsonian hyperpyrexia syndrome, which kinda resembles neuroleptic malignant syndrome.

And it is something that that I think I would immediately be thinking, oh, this person's getting septic.

This person's getting, you know, altered.

And and I think that'd be the the thing I'd wanna take away is, did they run out of their carbidopa levodopa, and have they been off of it for a couple of days?

Or did they suddenly have this administration of continuous through their NG tube get stopped, and that's why they're developing this syndrome.

It's something to keep in your differential.

For sure.

In fact, I thought the feeding tube thing was pretty interesting.

I've never seen a patient with a PEG tube or a PEG j tube that is a continuous infusion of carbidopa levodopa.

The idea certainly makes sense, but they drive home the point that opposed to someone who normally might dislodge a feeding tube and be a candidate for just replacement in the ED with a Foley or something temporary, this is someone who cannot miss that infusion.

And so you have to make sure that if they're unable to get the continuous infusion, you have a plan for what is going to then take its place until they can, especially if they can't hook up that tubing again into whatever temporary device you've placed.

And secondly, that if you end up intubating someone with Parkinson's disease, that placing an NG tube and making sure they stay on their medication regimen is critically important because even in the ICU, they can go through the withdrawals from their medication, and this can affect how long they stay on the ventilator, whether or not they can be extubated, and so on and so forth down the road.

So, you know, NG or some kind of enteric modality for providing that medication is very, very important.

All great points that the authors brought up.

And then, of course, there is one other modality for treating Parkinson's disease, and that's central stimulation with deep brain stimulators.

So sometimes patients will come in where they've tried carbidopalevodopa, and it's been unsuccessful or just partially successful, and they have a deep brain stimulator.

And in that scenario, it's just like any other stimulator device.

You need to make sure it's on.

You need to make sure it's functioning.

You need to make sure they haven't been through a recent MRI or device adjustment that has affected the settings, and now they're actually going through acute withdrawal or being under dosed.

So all things to keep in mind when someone with Parkinson's presents to the emergency department.

So don't miss their meds.

Make sure that they're getting it even if they're NPO or intubated.

Make sure their stimulator or their PEG j tube is working and is still hooked up.

Look for those physical signs of Parkinson's disease, which include rigidity, bradykinesia, which is really just they're very slow to move.

They'll have tremors typically in one extremity, and then they'll have this kind of stooped over posture that comes late in development.

And then, orostatic hypotension, like you mentioned, especially if they're coming in with syncope, this is a very common complaint.

And if you're blessed enough to have physical therapy in the emergency department, this is one case where their assessment can certainly be exceedingly helpful early on because you can take someone who might be a good candidate to go home.

You know, they have a mild UTI, not a big deal, but this has exacerbated their Parkinson's symptoms, and their orthostatic hypotension is worse, and their movement is a lot worse, and now they're a huge fall risk.

And you might not have the time to pull that out of your history and physical, but a therapist would be an exceptionally good way to objectify that and give you some evidence that this person actually needs to be admitted to the hospital.

Alright.

Here's another trivia question for you since we just completed the Parkinson's discussion.

The deficiency of dopamine that occurs in the brain with Parkinson's disease occurs in which part of the brain?

Now if you have the article, there's a great little image, but I'm gonna give you five choices.

Here we go.

The amygdala, the cerebellum, the hippocampus, the substantia nigra, or the thalamus?

Which one of those areas is the area that loses the dopaminergic neurons?

Can I tell you, I was worried that you were gonna give me the larger structure around the substantia nigra, and I was worried because then you start I'm like, well, where is he going?

But substantia nigra is the answer.

It's always their substantial nigra isn't as substantial as it used to be, and that's the problem.

And that's why they need to have somebody put really expensive fancy wires up there to make it, you know, get a little more substantial.

There you go.

Very good.

Alright.

One more question.

I covered this earlier, but let's see if you were listening.

Which best describes the role of carbidopa in the treatment of Parkinson's disease?

One, it acts on dopaminergic receptors.

Two, it blocks dopamine reuptake in the brain.

Three, it enhances central conversion of dopamine.

Four, it inhibits peripheral breakdown of levodopa.

Or lastly, it replaces deficient dopamine in the central nervous system.

I think it's four.

It is four.

It inhibits the peripheral breakdown of levodopa so you don't get any peripheral nervous system symptoms or side effects, and the levodopa ends up getting converted centrally.

You are absolutely correct, sir.

I wanna just make a plug here for the students out there, both young and old.

I think this is such a cool medicine to look at from a history of medicine standpoint because we knew that we had to get more dopamine into these patients, but we knew we had to figure out a way to get it into their central nervous system.

And I think just the the biochemical research and the trials and the history of, like, the the creation of this medicine is just an unbelievable miracle that is something that I think is worth looking at because I think it's easy to lose track in the era of modern medicine of all the interesting miracles that you just casually get to use on a daily basis, and you don't know the full background story too.

So I think this is a good one to to look into and enjoy a little deep dive into the things that we didn't used to have that now we do, kinda like insulin.

Great point.

Alright.

The second disease is myasthenia gravis, And this disease, unlike Parkinson's, is autoimmune in origin.

And people have autoantibodies that attack their nicotinic receptors on the postsynaptic membrane.

And if you don't know what I'm talking about, there's a great image in the article for exactly where this is taking place, and this causes muscular weakness.

And in its most critical form, it causes ventilatory failure.

So it affects their patient's ability to breathe, to ventilate, and then they decompensate very quickly.

And the most common triggers for patients who have myasthenia gravis, even when it's controlled and treated, would be things like infection, medications, especially the ones that we're used to hearing about, things like fluoroquinolones, macrolides, and beta blockers.

All of these can precipitate a myasthenic crisis, which is when someone goes from stable, relatively controlled myasthenia gravis to all of a sudden now being very weak, maybe experiencing symptoms, having shortness of breath, having trouble breathing, and then showing up in your emergency department.

And that's when we run into trouble.

So when it comes to myasthenia gravis, there are some things you can do in the emergency department with the patient who is complaining of shortness of breath or any kind of respiratory complaint to see if they're exacerbating to the point where they need to be in the hospital.

So I thought the authors again did a great job of driving home the point that you can't just tell by looking at the person with myasthenia gravis that they're not having a crisis.

So there there will not be tachypnea.

They won't look like they're having respiratory distress.

It doesn't present like, say, a COPD or an asthma exacerbation where you go, oh, that's respiratory distress.

This is going to be quiet.

It's going to be less noisy.

They're going to look super relaxed, and it's not because they are relaxed.

It's going to be because their muscles are failing, and they're unable to contract that musculature, and it's not going to present with the same alarms as you might expect.

So their vital signs might be completely normal.

Their oxygen saturation will be completely normal.

And if it's not, that's a very, very late finding, and you don't wanna wait until then to make the diagnosis.

And worse, you don't wanna send somebody home saying, oh, you have normal vital signs.

You're gonna be okay, and miss the fact that they're having a crisis.

So there are some tests.

The diagnostic test of choice is the negative inspiratory force or the NIF, which is when they inhale on this device against pressure, and it's measured in centimeters of water.

And anything less than 20 centimeters of water is indicative of a severe myasthenic crisis, and that person should be admitted to the hospital and treated and not sent home.

But I thought it was pretty cool that they gave some alternatives there.

Have you ever tried any of these kinda bedside alternatives when maybe what NIF wasn't available?

So can we just back up for a second and have a moment of silence for edrophonium, which for my entire medical career has been if you wanna diagnose myasthenia gravis, you did a Tencelon test.

And I looked this up today.

That is that is Tencelon was the trade name for eidrofenium.

That's right.

And that medicine has been discontinued by the United States Food and Drug Association as of 2018.

Yeah.

So I have now arrived at the point where my medical education is so outdated that the diagnostic test of choice does not exist anymore.

And I'm just proud to be so humbling and such a geriatric moment for me that I needed to just stare at the clouds for a minute and remember that time is fleeting and goes by so fast.

So one moment of memory for the old 10 swatches that I never did and because no one would ever give it to me, and I now I can't.

So we are.

That's right.

So I am here to celebrate the fact that I do think I if I bother my respiratory therapy people or my upstairs people enough, that a NIFS machine would come downstairs, and I could do this.

And I think that it intrigues me from the standpoint that I think that myasthenia flares I think I underappreciated how risky they are in terms of respiratory status.

And I think some of these patients, when they're not doing well on BiPAP, I'm attributing it to their infection and everything else.

But I think that's the scary part of these patients is, I think for, like, bad, you know, URIs that their intubation rates in studies are up to sixty percent.

Like, the rate at which these people fail.

And I think some of that stuff is in a pre BiPAP CPAP era.

And I like to consider it in a pre COVID era when, like, we weren't as aggressive about high flow oxygen and some of the interesting different ways that we use BiPAP and CPAP now.

I think that those rates are lower, but I still wanna have in the back of my mind the idea that I need to watch these patients very carefully because even if I do everything right, they still may need to be intubated.

And that was an interesting thing for me to take away from this that I need to accept that that may be the endpoint for these patients even if I do everything right.

And therefore, I should treat it as more something to plan for and less of something to think that I'm failing if I get there.

Yeah.

So many great points there.

Absolutely.

So, yes, the Tensilon test is gone.

The diagnostic test nowadays is an antibody assay, and so you can actually test them directly for these autoimmune antibodies, and make the diagnosis that way.

And, yeah, you made some great points there about ventilatory management.

So in an era where we used to only have two things, supplemental nasal cannula and a ventilator, there was a very black and white distinction there, a or b.

But now we do have a lot of tools at our disposal.

And, yes, absolutely, it's okay to use things like CPAP or BiPAP or high flow nasal cannula or these kinda intermediate measures.

They're still representative of respiratory failure.

So that person is still at critically high risk and still needs to be treated for a myasthenic crisis.

But, hopefully, if you can avoid intubation, they can then avoid those downstream complications of extended intubation and extended trials of extubation and so on and so forth.

Those complications can hopefully be avoided by bridging with some of these other noninvasive measures.

But I do wanna be clear that that all still represents respiratory failure.

And then, you know, if you don't have the negative inspiratory force test, the author said there's actually good evidence that just doing a counting test can be diagnostic.

And this is done by having the patient inhale deeply and then count out loud at a rate of two numbers per second while they breathe out.

So take a deep breath, and then one, two, three, four, five, six.

They should be able to reach 30, but anything less than 20 is equivalent to a severe compromise and equivalent to a negative inspiratory force of less than minus 20 centimeters of water.

You can do the NIF, which is ideal.

But if you can't do the NIF, do the counting test at the bedside with your patient.

And if they fail that, that's just about diagnostic for a severe crisis.

And then when it comes to treatment, because it's autoimmune, we're looking at all things at our disposal.

Now you're likely reaching out to your neurology specialist at this point as you should, but lots of things are at their disposal, things like steroids, things like plasmapheresis, things like specific targeted immunosuppressants, and even things that we don't normally think about, like cyclosporine and methotrexate.

All of these can be prescribed acutely, but even for long term control.

So you might see somebody, for example, with myasthenia gravis who's on rituximab.

But in the acute phase, steroids, plasmapheresis, and IVIG are kind of the mainstays for what you're gonna give them.

And the sooner you start that, the better.

Ergo, get your neurologist on the phone once you have their breathing stabilized.

And I think from a rural and critical access standpoint, I think these are the kind of patients that they can look better on oxygen on BiPAP, but you're still wanting to transfer these patients out from your facility unless you've got an ICU to care for them because they're gonna be delicate and challenging from a, you know, maintaining their ventilation and oxygenation standpoint.

And I think that you should move them while they're stable as opposed to waiting for them to become more unstable, especially if you have trouble managing ventilated patients in your setting.

Yeah.

And in a world where we still debate succinylcholine versus rocuronium, I thought the authors did a good job stressing that rocuronium should be avoided in this population.

And succinylcholine should be used because it dissociates quickly.

The one thing we don't wanna do is provide prolonged paralysis for these patients, and so succinylcholine is ideal.

Now you can do rocheronium with a reversal agent if you really want to shortly after intubation.

That's an option as well.

But succinylcholine is what the authors recommended.

Something to think about.

I think that the biggest thing I took away from this, especially given the prevalence these days, is that myasthenia gravis patients really should not be exposed to Botox.

That is one of the medications that will trigger a crisis for them.

And I think given the ubiquity and ease of getting Botox, and there's just so many places you can get it now, I'm not sure everyone's gonna make sure you don't have myasthenia gravis.

And I think that that's another question that I'm asking more and more of in my patients with weakness or my patients with stroke like symptoms is when is the last time you got some kind of paralytic or neurotoxin injected?

Because I think it matters now in terms of trying to figure out what could be causing symptoms and what could be triggering things.

Yeah.

Yeah.

In fact, there's a good table on page seven, the precipitants from myasthenic crisis.

So fever and infection at the top of the list with good evidence behind that.

Tapering of their immune modulating medication if they're undergoing some kind of medication change, lots of evidence behind that as well.

Non depolarizing neuromuscular blocking agents and Botox all also have good evidence behind them.

There is also evidence for things like beta blockers, calcium channel blockers, fluoroquinolones, aminoglycosides, magnesium supplementation.

That's over the counter, so you gotta be careful and ask about that class of medicines as well.

Class one a, antidysrhythmics and macrolides.

And then there is some kind of soft evidence that penicillin even may be something that can precipitate a crisis.

It's hard to say in that scenario if it's the infection that they're treating or if it's the penicillin, but either way, just know that there are a lot of things that can trigger a crisis.

And you wanna ask about all of those, including over the counter meds.

I'm pretty sure all these patients are allergic to penicillin, though, so I'm not really that worried about them getting a reaction.

That's a whole another podcast right there, sir.

A whole another podcast.

Okay.

Then let's get into some trivia questions.

A 41 year old man with myasthenia gravis is presenting with new shortness of breath.

He was recently started on metoprolol.

Which is the most appropriate immediate ED action?

This is such a great board style question.

The most appropriate immediate one.

A, administer steroids, b, begin CPAP for oxygenation, c, discontinue metoprolol and assess respiratory function, d, order a head CT because why not, and and e, start empiric antibiotics.

I think c felt like a good answer to me.

I'd stop the metoprolol and I'd do some counting or see if I could steal a NIFs from upstairs.

Perfect.

That is the perfect answer.

Yes.

Administration of corticosteroids is not the first immediate action.

Beginning CPAP is great if they need it, but you gotta assess the respiratory function first.

Of course, ordering a random head CT is never the right answer unless you have an indication for it.

And then starting empiric antibiotics is also not the answer.

Now we did say that infection is something that can frequently cause an exacerbation, but you do have to figure out that they actually have the infection before you give them something that in and of itself might trigger a myasthenic crisis, like an antibiotic.

Alright.

One more question for the myasthenia gravis.

Which of the following medications should be avoided in patients with myasthenia gravis due to the risk of exacerbating symptoms?

Acetaminophen, amoxicillin, ceftriaxone, fluoroquinolones, or loratadine.

Oh, I was gonna go with fluoroquinolones, d.

Yes.

Can't ever go wrong with blaming a fluoroquinolone for anything in medicine.

Not in this era.

No.

Especially in this patient population.

But, yes, there is very, very good evidence that fluoroquinolones can exacerbate myasthenia gravis.

So that is the correct one.

Two, just kinda parting thoughts on this that I thought were really interesting.

One, I found that when you're looking at the very ill myasthenia gravis crisis, you have basically equivalent value in terms of improving their condition with IVIG and plasmapheresis.

But there are certain populations that can't do one or the other.

IVIG cannot be combined with dialysis.

So if you've got a dialysis patient with myasthenia gravis, there's someone that's gonna get plasmapheresis.

And if someone is septic, you don't wanna do plasmapheresis and deplete them of their antibodies at that time.

So there's someone that's gonna get IVIG.

I thought that was a valuable, you know, kind of thought to have in the back of my head as to what is the treatment I'm gonna want for which patient in here.

And the other, just looking at the cost of some of these medications for myasthenia gravis, I think we touched on how tapering down their medications can cause a crisis.

But like so many of our patients on blood thinners, it is not sometimes that they're titrating or going down on their medications by choice, but because they can't afford more of them.

So trying to ask your patient honestly if they have their medication or if they've been running out or if they've been trying to stretch it to make it last longer or to make it last until their next refill, I think that can easily be one of the most common causes that precipitates these crises.

And if you don't tease that out and try to figure out the supporting things to make sure to get them their medications, then we're gonna be back here very shortly.

All great points.

Alright.

Let's jump into our third and final disease.

This is multiple sclerosis or MS.

It is another autoimmune disease, but this time targeting myelin or the lining of nerves is in the central nervous system leading to inflammation and eventual damage to the surrounding neurons and then manifesting in symptoms.

Interestingly, it does have a female predominance ranging from two to three affected females for every affected male, and a typical presentation between 10 and 50 years of age with a peak between 20 and 30.

So this is a disease of the relatively young, unlike what we just discussed with myasthenia gravis and Parkinson's.

And the condition, again, has no cure, much like the other two conditions, but the therapies can decrease the frequency and the severity of the crises that bring them to the emergency department.

A few things to know about multiple sclerosis in the ED specifically, the manifestations depend on the location of the neurons that are affected, which seems kinda silly to say.

But people with multiple sclerosis who have it start to recognize, okay, I've got a new neurological symptom I haven't had before, and so I'm going to the emergency department because this is an MS flare.

But if they come in with things that could be stroke mimics, most commonly, it's affecting the vision and optic neuritis is their presenting symptom.

And so their neurological symptoms are going to map to wherever the deficiency is in the central nervous system.

When it comes to treatment in the emergency department, making sure, of course, that they don't have an acute stroke or some other mimic going on is critical.

The diagnosis is typically made by MRI and lumbar puncture if it's their first ever presentation for multiple sclerosis.

And even though you're going to get MRI and LP, if you know the diagnosis of multiple sclerosis, then treatment with steroids is the recommended therapy and shouldn't be delayed pending MRI and LP.

So it's not one of those instances where somehow if I give you steroids now, the LP results will be ruined and they can't use them later on.

That's not the case.

Don't withhold steroids in this scenario because the longer it takes for them to get the steroids, the more damage to their central nervous system is going on.

I think this also made me a little more curious about how they were diagnosed because I did appreciate kind of the discussion of the MRI and the LP kind of depending on the picture that they give you and how certain it is.

And I think for someone that was presenting saying, oh, I'm having another MS flare.

My controller medications aren't working.

This gave me a little more of an interest in saying, did you ever get an LP?

Like, what was the MRI that they did?

You know?

And, like, have you seen a neurologist?

Like, how deep, how complete was this workup?

Because I think I'm gonna be more interested, especially in these patients that have some of these recurrent flares that don't seem like they're being controlled.

I'm gonna go looking for more of these unusual, you know, different things on the differential by getting that LP done.

Yeah.

And when it comes to imaging, there are some fantastic pictures in the article about MRI imaging and how you can see these white lesions on MRI all over the brain and the spinal cord.

Now it's important to keep in mind that the spinal cord is frequently involved because it's all part of the central nervous system.

We typically think of this as being a brain phenomenon, but it's brain and spinal cord.

Steroids, like I mentioned, are the mainstay of therapy, and we're talking large doses.

And I found it interesting that there is a equipotence between oral and IV dosing of these massively large doses of steroids.

So we're talking Solumetrol, a thousand milligrams IV, or prednisone, one thousand two hundred and fifty milligrams orally per day, which is just mind boggling to me that someone could take that much oral steroid and not immediately rot a hole in their gut.

But, yes, it is proven to be safe, carefully administered, and that is the actual dosing necessary.

So there is a oral route.

It doesn't always have to be IV, which, again, I found interesting because it seems to me like at least the ones that showed up in the emergency department always needed IV access and IV Solu Medrol, and we're talking home health and setting this up as an outpatient, and it all took time and frequently resulted in an OBSTAY.

We never really discussed oral options.

You ever seen anybody take that much orally?

I have not, but I think this is something that intrigues me as a you know, if someone is someone that's coming in more frequently.

Or in this day and age, people are just so busy.

I have the same discussion with antibiotics sometimes when using long acting antibiotics that people are just like, yeah.

I just I can't be admitted right now.

And I think I would entertain this as a treatment option for someone with well established MS that had, like, a, you know, relapsing remitting course and had tolerated oral steroids well before, and they were asking for it and saying, hey, I understand you wanna admit me to make sure we get this under control, but I just can't right now.

I don't think based on this that I would hesitate to then provide it to them.

Great point.

There is also a role for baclofen in these patients.

So baclofen, the muscle relaxants can help with the subsequent spasms and with symptomatic treatment.

And it's usually provided in the way of oral medication or baclofen pumps, so people can have continuously infusing baclofen through an intrathecal pump, for example.

And that can cause problems in and of itself with withdrawal if there's ever a problem with the pump.

So much like we talked about with Parkinson's and people with deep brain stimulators, if you've got a device that you're requiring to be on and running twenty four seven and you undergo an MRI or have someone adjust this device, you need to make sure that the reason for their presentation isn't that this pump is now no longer working.

And in the case of baclofen, the withdrawal becomes a life threatening withdrawal.

We're talking things like intractable tetany and muscle spasms that affect respiration and lead people to become intubated and ventilated in order to prevent life threatening muscle contractions, typically treated with benzodiazepines in the hospital.

So, again, not somebody you're going to send home until you're able to fix the problem and just something to be aware of.

They can develop seizures.

They can develop hyperthermia.

They can go into rhabdo.

So this can easily throw you off as a sepsis patient or, you know, encephalitis patient that you think is now febrile and seizing because of that.

But I think as soon as you find out about that baclofen pump, you need to reach out to your neurosurgery colleagues, see who's filling it, see who's the one that's maintaining it, and can run a diagnostic on it to make sure it's working.

And then see if they're interested in coming in and doing an LP to inject intrathecal baclofen while they're working out what's going on with their device, which I thought was one of the more elegant solutions to the problem that you could do the LP, get your diagnosis out, and then put your treatment in, and really just feel like you're a pretty hero doctor when you try to admit that one to the floor.

Yeah.

That is pretty amazing.

Or the ICU, I guess.

I guess that would even if you stabilized it, they'd probably still wanna put that in the ICU.

I agree.

I did mention earlier that optic neuritis is the most frequent presenting symptom, and there is evidence for a point of care ultrasound in diagnosing optic neuritis, specifically, in this small study that the author cited, that optic nerve diameter and optic nerve sheath diameter are significantly larger in the affected eye than the unaffected eye, and larger in patients with optic neuritis than in healthy kind of matched cohorts.

And if you are adept at using the point of care ultrasound, and you're doing your ocular ultrasound, this is an easy measurement to make.

I'm a big proponent of eyeball ultrasound for all kinds of things, including retinal detachments, but that's going down the rabbit hole for just a second.

It's not that hard to make this measurement, and, you know, it takes way too long to do an MRI, and you could do this at the bedside in, like, five minutes and make this diagnosis.

We had a lens dislocation in the ER the other day that was really cool.

I'm looking on the ultrasound.

So I'll bet.

Think that the the benefit of this, if you can see a significant difference in their optic nerve, especially if you're getting pushback to admit these people for their MRI and their workup, I think it's another data point you can use to encourage your upstairs colleagues that there's something here to be worked up more.

Yep.

Important things for us in the emergency department are things like not missing infectious causes for a flare.

You know, people with MS can flare for multiple reasons, but infections are one of them.

And so we do wanna check because they're frequently on immunosuppressive therapy, much like our patients with myasthenia gravis.

So you don't wanna miss an infection if that's what's causing their flare.

And keep in mind that people can get flares or pseudo flares triggered by things like fever and even heat.

Now I found this pretty interesting that, you know, even a viral infection causing a little bit of fever can trigger a pseudo flare, meaning that their symptoms are just acutely worse because of the fever, and that control of fever can actually help in that scenario.

Much like the other two diseases, lots of things to know and a ton of information in this article that I thought the authors did a really good job of presenting.

Alright.

And on that note, let's do a couple more trivia questions for you, sir.

When it comes to brain MRI findings, typical of MS, what do white matter lesions represent?

A, hemorrhage, b, calcifications, c, demyelinating plaques, d, infarcts from emboli, or, e, tumor metastases.

Think in the the MS patient this is c.

It is.

C is demyelinating plaques.

That is indeed the typical appearance of the multiple sclerosis white matter lesion.

Alright.

One more on multiple sclerosis.

Which of the following is the most common initial presenting symptom in patients with multiple sclerosis?

A, bulbar weakness, which is facial muscle weakness, double vision dysarthria or dysphagia, double vision with neck flexion, Lhermitte sign, which is that electrical shock sensation shooting down the spine caused by neck flexion, optic neuritis, or Yudhoeff phenomenon, which is transient worsening of previous MS symptoms as a result of increased core body temperature.

That's the pseudo Well, it's a cool name.

But I'm gonna go with, I think wait.

Which one was Lermi?

Lermi was d?

Was the oh, c.

Lermi was c electric shock.

I want d d was optic neuritis.

Optic neuritis is where I'm going.

Yes, sir.

Finally.

That's right.

It accounts for twenty two percent of first time presentations is optic neuritis.

So that's when you pull out your point of care ultrasound.

Alright, ladies and gentlemen.

Well, we covered lots of information, but there is a ton more.

So if you have access, I can't recommend enough that you go and read this article about Parkinson's, myasthenia gravis, and multiple sclerosis, and digest and ingest all of the information here and get your four hours of CME.

It's really quite the volume of information on all three of these disease processes specific to the ED.

Now we didn't mention in any of these really pre hospital care because it's challenging enough to make and treat these diagnoses in the emergency department in the pre hospital setting.

It's often focused on rapid assessment, knowledge of what medications they're currently on, and bringing those with them, especially if they have Parkinson's so they don't miss doses.

If they have assistive devices, bringing those, and then getting that history from someone on the scene who can help, all of those are critical things to do in the pre hospital setting, and we didn't dive into that much today.

But there is a good section on each of those disease processes for the pre hospital setting, and lots of pictures.

MRI, ultrasound.

It's a fantastic issue.

I highly recommend you go read it.

I completely agree.

These are three diseases that if you just take the time to, like, learn the history and the background of them, it'll give you just a profound appreciation for the progress that we're making.

I have hope for the future, and I am just always interested in, you know, the casual way that I get to throw miracles around in tiny pill bottles in this job.

And I try to maintain a healthy respect for it.

There it is, ladies and gentlemen.

Doctor t r Eckler, ten out of 10 today.

Got all the trivia questions right.

Hey.

That's a giant round of applause for him right there, ladies and gentlemen.

I think we'll bring him back for another episode.

One more One more time.

Just one more.

And that's a wrap.

Thanks for joining us for this episode of Amplify.

I hope you found it informative, and I wanna remind you that ebmedicine.net is your one stop shop for all of your CME needs, whether that be for emergency medicine or urgent care medicine.

There are three journals.

There's tons of CME.

There's lots of courses.

There's so many clinical pathways, all this information at your fingertips at dbmedicine.net.

Until next time, everyone.

I'm your host, Sam Ashu.

Be safe.