-
Hello everyone and welcome back. As a
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reminder, you're listening to the
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Insights edition of the NCS podcast. As
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a reminder, Insights is a teaching
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podcast geared to residents and non
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neurologists and non neuro intensivists
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for sort of a broad overview of how to
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approach neuro critically ill patients
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As a reminder, all of the content that
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we features comes from the on call
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chapters on the NCS website. We really
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invite you to check those out because
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All right, hi guys, hi, Casey, to
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Cecilia Neurice, you've read me since I
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hopkins. Glad to be here again. Today
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we're gonna talk about neurological
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emergencies, or I'm sorry,
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neuromuscular emergencies, and this big
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umbrella of neurological emergencies.
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So, Casey, I know that these patients
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can be really complicated, especially
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for a non-neurologist, which is me. So
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when you see these patients, how do you
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usually assess them first? Can you walk
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us through maybe the first few steps of
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how you would go about assessing these
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patients? Yeah, absolutely These
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patients are not as patient. as some of
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the other patients that we've talked
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about. So, you know, I think up to
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this point, we've covered acute
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ischemic stroke and ICH and status,
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which are all much more common than
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neuromuscular emergencies, which I do
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think creates a little bit more
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apprehension around how do you assess
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these patients. The same is true for
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basically all emergencies that you want
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to start by assessing or AV and Cs.
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What's different about these patients is
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so many other patients in neurocritical
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care need protection and ventilation
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because of altered mental status. Our
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neuromuscular patients actually have a
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totally different pathology that's gonna
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lead them to needing intubation. And
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that is that they have weakness of the
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really complicated musculature that
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controls breathing. So if you think
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about breathing, it's actually covered
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with a pretty complex and really amazing
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process That starts with, you know, um,
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pattern, rest of the duration. in
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generators in the brainstem and then
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involves all of our muscles of breathing,
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namely the diaphragm. So the diaphragm
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is really what is the driver of
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inhalation and exhalation, but it's
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also supported by some of our accessory
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muscles, which become more inpatient,
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more important as that diaphragmatic
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weakness takes over. And so these
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patients are really at high risk of
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having hypercarbic respiratory failure
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because they cannot maintain that
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ventilation drive because
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of, but in addition to that hypercarbic
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respiratory failure, they can also
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suffer adelectasis, and they also have
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weakness as some of those oral
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pharyngeal muscles that control
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secretions. So they also are at higher
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risk of aspiration, and those two
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things together can actually put them at
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risk of hypoxic respiratory failure.
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And what
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I'm most interested in when I walk in
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the door, with anyone with suspected
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neuromuscular weakness, is how well are
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they doing on gas exchange? And some of
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this is pretty easy to kind of just get
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a sense of at the bedside. Is the
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patient able to talk in complete
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sentences? Are they looking short of
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breath? If those two things like the
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patient's talking to me and can give me
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a complete history of when their
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weakness started, I'm way, way, way
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less concerned that they're not able to
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maintain their ventilation versus a
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patient who can barely get out one or
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two works.
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Then beyond that, I think there are
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some sort of sort of targeted things
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that we can look at at the bedside. So
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we know that neck flexion can be a nice
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marker
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of diaphragmatic strength. So the
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patient who really can't even lift their
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head off the bed, that patient probably
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has pretty significant diaphragmatic
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weakness. The patient who can't count
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to 20 in a single breath probably
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doesn't have good inhalation or
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exhalation to maintain that ventilation
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This gets, um, that are elucidated by
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actual respiratory mechanics. These are
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sort of portable spermetry that can be
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done at the bedside. Just to kind of
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introduce some of those terms, we talk
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a lot about the NIF or the negative
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inspiratory force, which confusingly
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can also be called the maximal
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inspiratory pressure. So NIF
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is NIP, which is just, you know, to
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make things complicated We also talk a
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little bit about the maximal, maximal
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expatory pressure or the MIP, which is
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really kind of a nice marker of like how
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well the patient's able to cough. And
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then finally, we can measure how much
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error is the patient able to take in in
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a forced vital capacity? Like when we
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ask them, how much air can you fill up
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all the way to the top? And then
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breathe it all the way out. How much
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air are you able to breathe all the way
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out after your maximal inhalation.
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Um, so those are sort of the things,
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so again, that's forced Bible capacity.
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Those are the three tests that very
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frequently will ask our respiratory
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therapist to help us objectively measure
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at the bedside.
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Just some numbers to keep in mind. This
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is, this is again, like you have to
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take the whole patient into
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consideration, but we know that
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patients who have a negative inspiratory
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force that myth That falls below less
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than negative 30, those patients are
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not doing very well with gas exchange
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and have a higher rate of progressing to
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needing innovation. Similarly,
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patients who really can't take a force
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file capacity of 20 milliliters per
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kilogram of their ideal total body
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weight, which like ends up being
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somewhere around 15 liters Those
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patients again are also not doing a
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great job with gas exchange or and have
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the potential to progress. respiratory
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failure. The trick with all of this is
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that we really want to intervene and
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safely give these patients either
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support or intubation before they have
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any sort of, you know, blood gas
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abnormalities. Waiting for that PCO to
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to rise, waiting for them to become
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hypoxic, that's really not the goal.
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We want to intervene before then with
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some sort of safe ventilation depending
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on what the primary process actually is.
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Great. Excellent. That was really nice.
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Thanks for breaking it down, making it
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simple to understand. So let's say we
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talked about all of this and we took
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care of the respiratory status. What
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about all the other things? Let's talk
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about all the other fluonura things that
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you're going to look for in these
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patients. Right. So these are the
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patients we actually have to do a really
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comprehensive neuro exam to figure out
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why they are weak. The most common
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reason that people come into the, you
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know, to the emergency department, to
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our neuro ICUs with weakness is because
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of a central nervous system process.
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Here, we're really focused on the
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peripheral nervous system, meaning that
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there's a potential problem either
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within the nerve cell body, which is
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for motor neurons, the anterior horn
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cell, a problem with the nerves
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themselves, so a neuropathy, a problem
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with the neuromuscular junction, so one
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of those junction pathologies, or a
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problem with the muscle. And each of
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these is gonna have sort of a signature
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based on our neurologic exam. You know,
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to go through that comprehensively, I
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really invite people to look at the
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on-call chapter, but a couple sort of
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key bits to kind of keep in mind is that
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neuropathy pathology, so pathology of
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the
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nerve tends to involve sensory because
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the nerve has both motor and sensory
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components. So patients who do not have
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any sensory deficits and don't complain
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of any sensory pathology probably don't
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have a nerve pathology. They probably
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have either a
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neuromuscular junction problem, a
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muscle problem, or an anterior horn
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cell problem.
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Similarly, neuromuscular junction, we
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can think about presynaptic or
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postsynaptic.
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The most common of these is myosynia
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gravis, which is a postsynaptic
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disorder. As such, it doesn't involve
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any smooth muscle cells. So the pupils
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are never involved. So I think that's a
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nice kind of pearl. And then muscle,
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people tend to have like muscular cramps
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or they have sort of muscle aches And so,
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you know, again, that's another way to,
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you know, get a better history and try
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to figure out, you know, where is this
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problem within the peripheral nervous
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system? Reflexes tend to be spared in
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neuromuscular junction problems and in
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muscle problems. Muscle problems, if
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they're very severe, you can lose the
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reflex. But loss reflexes also are
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another way to point to a neuropathy
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problem So again, you really are going
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to have to get out the reflex. you're
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going to have to really assess, is
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there proximal involvement? Is it
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distal involvement? You're going to
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really need to build a comprehensive
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differential. But.
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Now, the most common two neurologic
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emergencies from a neuromuscular
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perspective are myosynia gravis, which
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is a neuromuscular junction problem,
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and Guillain-Barré syndrome.
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Guillain-Barré syndrome is
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a catch-all syndrome
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syndromic name for a group of
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neuropathies that have a variety of
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presentations. So again, this is
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affecting the nerve root and the nerve
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itself. They have proximal and distal
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weakness, but the classic pattern is
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that it's ascending. It starts in the
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toes and they often have some sensory
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deficits, like a little bit of tingling,
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a little bit of back pain, and then it
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starts to get worse and starts to kind
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of almost creep up. Now, there are a
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lot of different variants of
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Guillain-Barré, so they don't all
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present like this,
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but that is sort of like the classic.
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So again, that's a neuropathy.
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The hallmark of that disease is that
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it's a fattigable process. The
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neuromuscular junction, as you're
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asking it to exercise more, it becomes
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more pronounced the weakness that the
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patient has. Some ways to look at that
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at the bedside are looking for ptosis,
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especially after sustained upgaze,
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diplopia, sort of weakness of the face,
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difficulty controlling saliva And then
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that, again, fatigueable component of
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weakness.
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These patients are a little bit
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difficult to assess with respiratory
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mechanics, quite often because they
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have facial weakness. That makes it
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really hard for them to form a seal,
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which is how we get these accurate
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numbers with spirometry. So again,
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you're using your neuromuscular exam to
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localize where the likely pathology is,
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knowing that many, many patients will
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kind of fall into one of those two
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categories. Please go check out the
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on-call textbook. because there's a lot
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more detail within there about how to
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assess these patients. But one of the
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things that they all sort of share in
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common or many of our acute
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neuromuscular emergencies share is that
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they are inflammatory processes, which
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means that they require either induction
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immunosuppression if they've never been
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on immunosuppression or an increase in
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immunosuppression if this is a chronic
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pathology that is well known. And so,
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Sally, I was hoping that you could kind
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of walk us through, you know, very
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commonly we're using IVIG or plasma
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exchange. And there really are some
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nuances about when to choose what and
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what the contraindications and
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complications might be. So can you walk
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us through like, how do you choose one
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and what should you be aware of? Yeah,
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great. You're absolutely right. I
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think we talk about immunomodulation a
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lot when we talk about taking care of
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these patients which rat were gonna go
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So, um, I think for simplicity, we're
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going to keep it at IVIG.
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my exchange. Obviously, there's a
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whole lot of other newer options that we
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could talk about, but I think these two
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therapies or interventions are very much
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commonly done. I'm going to start with
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IVIG and then we're going to kind of
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move to plasma exchange or PLEX after
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that.
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I think IVIG is something that we've
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probably seen historically a lot more,
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you know, one of the things that a lot
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of people are using this intervention
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for is that it is a lot less complicated.
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So let's say if you have a very old
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patient, someone without a central axis,
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someone that you just have a peripheral
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axis and you know that you really can't
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push that any farther. IVIG may be a
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very reasonable choice. Again, these
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are all immunomodulatory As you talk
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about, it's going to be neutralizing
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the antibody. This is going to be kind
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of suppressing that pro-inflammatory
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cascade that can be going on or maybe
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going on. So a few things with IVIG,
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it can be perthrombotic. So that's
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something to keep in mind when you
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infuse that. I'm sure a lot of
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institutions have their own protocol as
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far as how to give it the rate of
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administration because the
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infusion-related reaction could be
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pretty significant if you give it fast.
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So people have those marks of shivering,
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rikers, maybe a fever and things like
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that. So those are some of the things
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to think about. If someone has IgA
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deficiency, this could be an inflexus,
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so just something to keep in mind. We
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commonly talk about that. But again,
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the nice thing about IVIG is that it can
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be given via peripheral line. This is
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probably something that may be also
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easier for someone who has a lot of
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medications on board because when we
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talk about plasma exchange replex, we
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talk about the fact that you need to
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think about drug removal also when it
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comes to plex. And I'll expand on that
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a little bit when we talk about plex.
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So that's really IVIG. And the dose
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thing, you know, we talk about 04
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grams per kilogram, per kilogram over
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5D. So the total dose is 2 grams per
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kilogram. But guess what? We have to
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break this down over 5 days because you
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can't give that much drug.
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Now, moving on to plasma exchange,
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this one does require a central line.
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So is this something that is doable?
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Operationally, you do this on
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alternative days and it's usually going
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to be five treatment options. So you're
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kind of dealing with this situation for
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10 days. So again, it's a commitment.
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Now what about drugs? So just like it
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sounds, any drug that has a higher
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tendency to stay in the plasma will
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possibly be removed by a plasma exchange.
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And what are those drugs? When you
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think about it pharmacokinetically,
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drugs that have a lower volume
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distribution meaning the drugs that are
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going to be staying in the plasma and
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the drugs that have a higher protein
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binding, again, the drugs that will
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stay in the plasma, guess what?
-
They're going to be removed probably
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higher. So again, if you have a really
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complicated case, right, you have
-
someone who's on a lot of antibiotics,
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someone who's getting life-saving
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medications,
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plaques may not be that safe. or you
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really have to think about looking at
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drug removal, talking to your clinical
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pharmacist, making sure this is the
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right approach for them. Another thing
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that you see commonly with Plex is mild
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degree of coagulopathy. So, if
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someone's getting anticoagulation at the
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same time, you may see your numbers
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kind of changing, have seen elevated
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PTT in this setting. Again, remember
-
that your coagulation cascade could be
-
affected by this because of the
-
coagulation. Cofactors and proteins
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could be affected by this So that's just
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something that is expected and may be
-
something that you want to think about
-
before starting Plex. One last poem
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about this is that you really don't want
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to Plex someone after you give them IVIG
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because Plex will remove your IVIG. So
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this unfortunately happens in practice.
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My recommendation is that if you think
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your IVIG is not effective, have you
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given it enough time? You really want
-
up to two weeks to see if your IVOG has
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been working enough. Another, again,
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Pearl is that if you're in a rush, if
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this is something that you wanna see if
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you see the benefits sooner than later,
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maybe Plex is something you wanna do
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first just because you commit someone to
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IBIG, and then right after it's done,
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you Plex them, you're gonna get out all
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of that IBIG from the system, which
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obviously is not gonna be efficient.
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I think that's such an important Pearl I
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feel like we frequently see second doses
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of immunomodulation therapy given,
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whether that's IBIG and then more IBIG
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or IBIG and then Plex, which again,
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not a great idea or Plex and then IBIG.
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And at least within the Guillain-Barre
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population, there have been multiple
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trials that just didn't show a benefit
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of that. In fact, there's maybe even a
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signal of harm with more of these
-
treatments So again, pick one, stick
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with it, really give it time, at least
-
a month, before you really have said
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this. did not work at all.
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The other thing that comes up so
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frequently, especially for myosynic
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patients is that they have sensitivities
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to a lot of drugs, or there's a lot of
-
concern that a drug might worsen their
-
myosynic crisis. So
-
yeah, I know that list is like 50 drugs
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long, but are there common things that
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we see again and again in the ICU that
-
we really do need to be mindful of with
-
these myosynic patients Yeah, I think
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this is such a good point and you're
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right. I think when you look at the
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list is over 50 medications and there's
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contraindications and there's relative
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contraindications and there's definite
-
and there's possible. So, I think for
-
the sake of this conversation, I would
-
limit it to what I think is absolutely
-
necessary to know and what is absolutely
-
necessary to think about A lot of
-
antibiotics are on that list but I think
-
there is three classes that I really
-
think you have to be very cautious
-
I mean, it's like a side. that's one
-
class, that's a very definite
-
interaction, your patients will be
-
weaker. So if you can avoid that class
-
altogether, that would be amazing, and
-
that would be my recommendation.
-
Luckily, there's a lot of other
-
alternatives, so we don't see a lot of
-
gentamicin, or tuberamisin, or
-
amicasein anymore. Another class is
-
fluoroquinolones, and then macrolides.
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So those three classes in the antibiotic
-
group, so immunocosides, macrolides,
-
and fluoroquinolones, you want to avoid
-
them. Magnesium supplementation is
-
another, I think, big topic. Now,
-
although it's a definite interaction, I
-
think you have to keep an eye on the
-
numbers and monitoring these patients
-
closely. Obviously, if someone's
-
magnesium is 11, right? And you have
-
a cardiac patient, you have to provide
-
enough supplementation and repletion so
-
that your patient is not experiencing
-
those side effects. But this is just,
-
to treat people, you know, with more
-
caution to make sure that you're on top
-
of their levels, to make sure that
-
you're looking at that more closely than
-
other patients.
-
Another big class is neuromoscore
-
blocking agents. I know a lot of
-
institutions have actually very specific
-
guidelines for rapid sequence intubation
-
for these patients. The dosing is
-
usually lower. We talked about how,
-
you know, you have to reverse these
-
patients quickly. A lot of these people
-
post a war actually come to a monitor
-
setting very quickly just because you
-
want to monitor them because you want to
-
make sure that they're breathing fine
-
and they're waking up okay and all that
-
in health and aesthetics. That's
-
another class that, again, could be a
-
problem and you have to really watch
-
these patients. And then one last class
-
that I think is pretty controversial is
-
corticosteroids. We do use
-
corticosteroids for mycenae and
-
absolutely that is part of, you know,
-
the algorithm for treating these
-
patients. But how does corticosteroids
-
can possibly exacerbate myesthenia
-
initially. And that's why when you see
-
high
-
dose corticosteroids, you see IVIG with
-
that, or you see that these patients
-
are intubated during that time. And
-
that's when we utilize that 'cause we
-
wanna give them that extra support so
-
that they can get that extra support
-
while they're on high-dose, you know,
-
methyl pred or other, you know,
-
corticosteroids. Usually in an IV form
-
initially with the high doses. But
-
those would be my recommendations, you
-
know, specific antibiotics we talked
-
about, again, intubating with a lower
-
dose of neuromuscular blocking agents,
-
reversing them quickly, talked about
-
magnesium and then corticosteroids.
-
Those are really important. And they
-
come up all the time, I feel like in
-
clinical practice. You know, these
-
patients are not patients that we see
-
day in and day out, but they do come up
-
frequently. And I think this has been
-
such a fun way to kind of think back on
-
how do you approach them with a
-
conceptual framework? How do you make
-
sure you're stabilizing them?
-
respiratory perspective. And then
-
really, how do you be thoughtful about
-
which immunosuppressant you're using and
-
the other drugs that they may be exposed
-
to in the neuro-ICU? So with that,
-
we'll actually wrap up any final words,
-
Salia? No, I think just having a
-
neuro-intensive is to help you in a
-
clinical pharmacist and this podcast
-
hopefully should get you ready for that.
-
I love it. This was a wonderful topic.
-
So until next time, guys,