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
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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.