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Episode 98: INSIGHTS - Neuromuscular Emergencies

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Description

Listen to the seventh episode of NCS' INSIGHTS series, this time on Neuromuscular Emergencies.

The INSIGHTS series is hosted by Casey Albin, MD and Salia Farrokh, PharmD, and covers different topics from Neurocritical Care ON CALL®, the only up-to-date, comprehensive resource to offer content exclusively dedicated to the practice of neurocritical care. Learn more about ON CALL®.

This episode is sponsored by Biogen.

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The NCS Podcast is the official podcast of the Neurocritical Care Society.

Contributors

  • Salia Farrokh, Pharm.D., BCPS, BCCCP

    Salia Farrokh, PharmD, BCPS, BCCCP is a neuro ICU clinical pharmacist specialist at Johns Hopkins Hospital. Dr. Farrokh received her PharmD degree from Saint John Fisher College, Wegmans School of Pharmacy in Rochester, NY. Her postgraduate training includes residencies in Critical Care and Pharmacy Practice at Yale-New Haven Hospital. Dr. Farrokh’s research interests include effective antiplatelet therapy in neuro intervention patients, optimal pain management in neuro ICU patients, and use of neurostimulants in this setting. Dr. Farrokh is passionate about training and precepting students and residents and is a certified ENLS trainer.

  • Casey Albin, MD

    Casey Albin, MD is an Assistant Professor at Emory University School of Medicine where she is a member of the department of Neurocritical Care. She completed both her neurology residency and a fellowship in Medical Simulation at Harvard Medical School/BWH/MGH. She completed Neurocritical Care fellowship at Emory. Dr. Albin’s research interests focus on educational innovations in acute neurologic emergencies and neurocritical care. In addition to running simulation courses, she is the editor of a best-selling textbook The Acute Neurology Survival Guide and is passionate about open access neurologic education through Twitter, blogs, and podcasts. She serves on the Education Committee of the Neurocritical Care Foundation.

  1. Hello everyone and welcome back. As a
  2. reminder, you're listening to the
  3. Insights edition of the NCS podcast. As
  4. a reminder, Insights is a teaching
  5. podcast geared to residents and non
  6. neurologists and non neuro intensivists
  7. for sort of a broad overview of how to
  8. approach neuro critically ill patients
  9. As a reminder, all of the content that
  10. we features comes from the on call
  11. chapters on the NCS website. We really
  12. invite you to check those out because
  13. they have a much more comprehensive look
  14. them what we're able to provide in the
  15. podcast We are also so grateful for our
  16. sponsors, Biogen and Cerebell, and now
  17. a word for one of our sponsors. Founded
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  19. biotechnology company that has pioneered
  20. multiple breakthrough innovations,
  21. including a broad portfolio of medicines
  22. to treat multiple sclerosis the first
  23. approved treatment for spinal muscular
  24. atrophy and to co-developed treatments
  25. to address a defining pathology of
  26. Alzheimer's disease. Biogen is
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  35. All right, hi guys, hi, Casey, to
  36. Cecilia Neurice, you've read me since I
  37. hopkins. Glad to be here again. Today
  38. we're gonna talk about neurological
  39. emergencies, or I'm sorry,
  40. neuromuscular emergencies, and this big
  41. umbrella of neurological emergencies.
  42. So, Casey, I know that these patients
  43. can be really complicated, especially
  44. for a non-neurologist, which is me. So
  45. when you see these patients, how do you
  46. usually assess them first? Can you walk
  47. us through maybe the first few steps of
  48. how you would go about assessing these
  49. patients? Yeah, absolutely These
  50. patients are not as patient. as some of
  51. the other patients that we've talked
  52. about. So, you know, I think up to
  53. this point, we've covered acute
  54. ischemic stroke and ICH and status,
  55. which are all much more common than
  56. neuromuscular emergencies, which I do
  57. think creates a little bit more
  58. apprehension around how do you assess
  59. these patients. The same is true for
  60. basically all emergencies that you want
  61. to start by assessing or AV and Cs.
  62. What's different about these patients is
  63. so many other patients in neurocritical
  64. care need protection and ventilation
  65. because of altered mental status. Our
  66. neuromuscular patients actually have a
  67. totally different pathology that's gonna
  68. lead them to needing intubation. And
  69. that is that they have weakness of the
  70. really complicated musculature that
  71. controls breathing. So if you think
  72. about breathing, it's actually covered
  73. with a pretty complex and really amazing
  74. process That starts with, you know, um,
  75. pattern, rest of the duration. in
  76. generators in the brainstem and then
  77. involves all of our muscles of breathing,
  78. namely the diaphragm. So the diaphragm
  79. is really what is the driver of
  80. inhalation and exhalation, but it's
  81. also supported by some of our accessory
  82. muscles, which become more inpatient,
  83. more important as that diaphragmatic
  84. weakness takes over. And so these
  85. patients are really at high risk of
  86. having hypercarbic respiratory failure
  87. because they cannot maintain that
  88. ventilation drive because
  89. of, but in addition to that hypercarbic
  90. respiratory failure, they can also
  91. suffer adelectasis, and they also have
  92. weakness as some of those oral
  93. pharyngeal muscles that control
  94. secretions. So they also are at higher
  95. risk of aspiration, and those two
  96. things together can actually put them at
  97. risk of hypoxic respiratory failure.
  98. And what
  99. I'm most interested in when I walk in
  100. the door, with anyone with suspected
  101. neuromuscular weakness, is how well are
  102. they doing on gas exchange? And some of
  103. this is pretty easy to kind of just get
  104. a sense of at the bedside. Is the
  105. patient able to talk in complete
  106. sentences? Are they looking short of
  107. breath? If those two things like the
  108. patient's talking to me and can give me
  109. a complete history of when their
  110. weakness started, I'm way, way, way
  111. less concerned that they're not able to
  112. maintain their ventilation versus a
  113. patient who can barely get out one or
  114. two works.
  115. Then beyond that, I think there are
  116. some sort of sort of targeted things
  117. that we can look at at the bedside. So
  118. we know that neck flexion can be a nice
  119. marker
  120. of diaphragmatic strength. So the
  121. patient who really can't even lift their
  122. head off the bed, that patient probably
  123. has pretty significant diaphragmatic
  124. weakness. The patient who can't count
  125. to 20 in a single breath probably
  126. doesn't have good inhalation or
  127. exhalation to maintain that ventilation
  128. This gets, um, that are elucidated by
  129. actual respiratory mechanics. These are
  130. sort of portable spermetry that can be
  131. done at the bedside. Just to kind of
  132. introduce some of those terms, we talk
  133. a lot about the NIF or the negative
  134. inspiratory force, which confusingly
  135. can also be called the maximal
  136. inspiratory pressure. So NIF
  137. is NIP, which is just, you know, to
  138. make things complicated We also talk a
  139. little bit about the maximal, maximal
  140. expatory pressure or the MIP, which is
  141. really kind of a nice marker of like how
  142. well the patient's able to cough. And
  143. then finally, we can measure how much
  144. error is the patient able to take in in
  145. a forced vital capacity? Like when we
  146. ask them, how much air can you fill up
  147. all the way to the top? And then
  148. breathe it all the way out. How much
  149. air are you able to breathe all the way
  150. out after your maximal inhalation.
  151. Um, so those are sort of the things,
  152. so again, that's forced Bible capacity.
  153. Those are the three tests that very
  154. frequently will ask our respiratory
  155. therapist to help us objectively measure
  156. at the bedside.
  157. Just some numbers to keep in mind. This
  158. is, this is again, like you have to
  159. take the whole patient into
  160. consideration, but we know that
  161. patients who have a negative inspiratory
  162. force that myth That falls below less
  163. than negative 30, those patients are
  164. not doing very well with gas exchange
  165. and have a higher rate of progressing to
  166. needing innovation. Similarly,
  167. patients who really can't take a force
  168. file capacity of 20 milliliters per
  169. kilogram of their ideal total body
  170. weight, which like ends up being
  171. somewhere around 15 liters Those
  172. patients again are also not doing a
  173. great job with gas exchange or and have
  174. the potential to progress. respiratory
  175. failure. The trick with all of this is
  176. that we really want to intervene and
  177. safely give these patients either
  178. support or intubation before they have
  179. any sort of, you know, blood gas
  180. abnormalities. Waiting for that PCO to
  181. to rise, waiting for them to become
  182. hypoxic, that's really not the goal.
  183. We want to intervene before then with
  184. some sort of safe ventilation depending
  185. on what the primary process actually is.
  186. Great. Excellent. That was really nice.
  187. Thanks for breaking it down, making it
  188. simple to understand. So let's say we
  189. talked about all of this and we took
  190. care of the respiratory status. What
  191. about all the other things? Let's talk
  192. about all the other fluonura things that
  193. you're going to look for in these
  194. patients. Right. So these are the
  195. patients we actually have to do a really
  196. comprehensive neuro exam to figure out
  197. why they are weak. The most common
  198. reason that people come into the, you
  199. know, to the emergency department, to
  200. our neuro ICUs with weakness is because
  201. of a central nervous system process.
  202. Here, we're really focused on the
  203. peripheral nervous system, meaning that
  204. there's a potential problem either
  205. within the nerve cell body, which is
  206. for motor neurons, the anterior horn
  207. cell, a problem with the nerves
  208. themselves, so a neuropathy, a problem
  209. with the neuromuscular junction, so one
  210. of those junction pathologies, or a
  211. problem with the muscle. And each of
  212. these is gonna have sort of a signature
  213. based on our neurologic exam. You know,
  214. to go through that comprehensively, I
  215. really invite people to look at the
  216. on-call chapter, but a couple sort of
  217. key bits to kind of keep in mind is that
  218. neuropathy pathology, so pathology of
  219. the
  220. nerve tends to involve sensory because
  221. the nerve has both motor and sensory
  222. components. So patients who do not have
  223. any sensory deficits and don't complain
  224. of any sensory pathology probably don't
  225. have a nerve pathology. They probably
  226. have either a
  227. neuromuscular junction problem, a
  228. muscle problem, or an anterior horn
  229. cell problem.
  230. Similarly, neuromuscular junction, we
  231. can think about presynaptic or
  232. postsynaptic.
  233. The most common of these is myosynia
  234. gravis, which is a postsynaptic
  235. disorder. As such, it doesn't involve
  236. any smooth muscle cells. So the pupils
  237. are never involved. So I think that's a
  238. nice kind of pearl. And then muscle,
  239. people tend to have like muscular cramps
  240. or they have sort of muscle aches And so,
  241. you know, again, that's another way to,
  242. you know, get a better history and try
  243. to figure out, you know, where is this
  244. problem within the peripheral nervous
  245. system? Reflexes tend to be spared in
  246. neuromuscular junction problems and in
  247. muscle problems. Muscle problems, if
  248. they're very severe, you can lose the
  249. reflex. But loss reflexes also are
  250. another way to point to a neuropathy
  251. problem So again, you really are going
  252. to have to get out the reflex. you're
  253. going to have to really assess, is
  254. there proximal involvement? Is it
  255. distal involvement? You're going to
  256. really need to build a comprehensive
  257. differential. But.
  258. Now, the most common two neurologic
  259. emergencies from a neuromuscular
  260. perspective are myosynia gravis, which
  261. is a neuromuscular junction problem,
  262. and Guillain-Barré syndrome.
  263. Guillain-Barré syndrome is
  264. a catch-all syndrome
  265. syndromic name for a group of
  266. neuropathies that have a variety of
  267. presentations. So again, this is
  268. affecting the nerve root and the nerve
  269. itself. They have proximal and distal
  270. weakness, but the classic pattern is
  271. that it's ascending. It starts in the
  272. toes and they often have some sensory
  273. deficits, like a little bit of tingling,
  274. a little bit of back pain, and then it
  275. starts to get worse and starts to kind
  276. of almost creep up. Now, there are a
  277. lot of different variants of
  278. Guillain-Barré, so they don't all
  279. present like this,
  280. but that is sort of like the classic.
  281. So again, that's a neuropathy.
  282. The hallmark of that disease is that
  283. it's a fattigable process. The
  284. neuromuscular junction, as you're
  285. asking it to exercise more, it becomes
  286. more pronounced the weakness that the
  287. patient has. Some ways to look at that
  288. at the bedside are looking for ptosis,
  289. especially after sustained upgaze,
  290. diplopia, sort of weakness of the face,
  291. difficulty controlling saliva And then
  292. that, again, fatigueable component of
  293. weakness.
  294. These patients are a little bit
  295. difficult to assess with respiratory
  296. mechanics, quite often because they
  297. have facial weakness. That makes it
  298. really hard for them to form a seal,
  299. which is how we get these accurate
  300. numbers with spirometry. So again,
  301. you're using your neuromuscular exam to
  302. localize where the likely pathology is,
  303. knowing that many, many patients will
  304. kind of fall into one of those two
  305. categories. Please go check out the
  306. on-call textbook. because there's a lot
  307. more detail within there about how to
  308. assess these patients. But one of the
  309. things that they all sort of share in
  310. common or many of our acute
  311. neuromuscular emergencies share is that
  312. they are inflammatory processes, which
  313. means that they require either induction
  314. immunosuppression if they've never been
  315. on immunosuppression or an increase in
  316. immunosuppression if this is a chronic
  317. pathology that is well known. And so,
  318. Sally, I was hoping that you could kind
  319. of walk us through, you know, very
  320. commonly we're using IVIG or plasma
  321. exchange. And there really are some
  322. nuances about when to choose what and
  323. what the contraindications and
  324. complications might be. So can you walk
  325. us through like, how do you choose one
  326. and what should you be aware of? Yeah,
  327. great. You're absolutely right. I
  328. think we talk about immunomodulation a
  329. lot when we talk about taking care of
  330. these patients which rat were gonna go
  331. So, um, I think for simplicity, we're
  332. going to keep it at IVIG.
  333. my exchange. Obviously, there's a
  334. whole lot of other newer options that we
  335. could talk about, but I think these two
  336. therapies or interventions are very much
  337. commonly done. I'm going to start with
  338. IVIG and then we're going to kind of
  339. move to plasma exchange or PLEX after
  340. that.
  341. I think IVIG is something that we've
  342. probably seen historically a lot more,
  343. you know, one of the things that a lot
  344. of people are using this intervention
  345. for is that it is a lot less complicated.
  346. So let's say if you have a very old
  347. patient, someone without a central axis,
  348. someone that you just have a peripheral
  349. axis and you know that you really can't
  350. push that any farther. IVIG may be a
  351. very reasonable choice. Again, these
  352. are all immunomodulatory As you talk
  353. about, it's going to be neutralizing
  354. the antibody. This is going to be kind
  355. of suppressing that pro-inflammatory
  356. cascade that can be going on or maybe
  357. going on. So a few things with IVIG,
  358. it can be perthrombotic. So that's
  359. something to keep in mind when you
  360. infuse that. I'm sure a lot of
  361. institutions have their own protocol as
  362. far as how to give it the rate of
  363. administration because the
  364. infusion-related reaction could be
  365. pretty significant if you give it fast.
  366. So people have those marks of shivering,
  367. rikers, maybe a fever and things like
  368. that. So those are some of the things
  369. to think about. If someone has IgA
  370. deficiency, this could be an inflexus,
  371. so just something to keep in mind. We
  372. commonly talk about that. But again,
  373. the nice thing about IVIG is that it can
  374. be given via peripheral line. This is
  375. probably something that may be also
  376. easier for someone who has a lot of
  377. medications on board because when we
  378. talk about plasma exchange replex, we
  379. talk about the fact that you need to
  380. think about drug removal also when it
  381. comes to plex. And I'll expand on that
  382. a little bit when we talk about plex.
  383. So that's really IVIG. And the dose
  384. thing, you know, we talk about 04
  385. grams per kilogram, per kilogram over
  386. 5D. So the total dose is 2 grams per
  387. kilogram. But guess what? We have to
  388. break this down over 5 days because you
  389. can't give that much drug.
  390. Now, moving on to plasma exchange,
  391. this one does require a central line.
  392. So is this something that is doable?
  393. Operationally, you do this on
  394. alternative days and it's usually going
  395. to be five treatment options. So you're
  396. kind of dealing with this situation for
  397. 10 days. So again, it's a commitment.
  398. Now what about drugs? So just like it
  399. sounds, any drug that has a higher
  400. tendency to stay in the plasma will
  401. possibly be removed by a plasma exchange.
  402. And what are those drugs? When you
  403. think about it pharmacokinetically,
  404. drugs that have a lower volume
  405. distribution meaning the drugs that are
  406. going to be staying in the plasma and
  407. the drugs that have a higher protein
  408. binding, again, the drugs that will
  409. stay in the plasma, guess what?
  410. They're going to be removed probably
  411. higher. So again, if you have a really
  412. complicated case, right, you have
  413. someone who's on a lot of antibiotics,
  414. someone who's getting life-saving
  415. medications,
  416. plaques may not be that safe. or you
  417. really have to think about looking at
  418. drug removal, talking to your clinical
  419. pharmacist, making sure this is the
  420. right approach for them. Another thing
  421. that you see commonly with Plex is mild
  422. degree of coagulopathy. So, if
  423. someone's getting anticoagulation at the
  424. same time, you may see your numbers
  425. kind of changing, have seen elevated
  426. PTT in this setting. Again, remember
  427. that your coagulation cascade could be
  428. affected by this because of the
  429. coagulation. Cofactors and proteins
  430. could be affected by this So that's just
  431. something that is expected and may be
  432. something that you want to think about
  433. before starting Plex. One last poem
  434. about this is that you really don't want
  435. to Plex someone after you give them IVIG
  436. because Plex will remove your IVIG. So
  437. this unfortunately happens in practice.
  438. My recommendation is that if you think
  439. your IVIG is not effective, have you
  440. given it enough time? You really want
  441. up to two weeks to see if your IVOG has
  442. been working enough. Another, again,
  443. Pearl is that if you're in a rush, if
  444. this is something that you wanna see if
  445. you see the benefits sooner than later,
  446. maybe Plex is something you wanna do
  447. first just because you commit someone to
  448. IBIG, and then right after it's done,
  449. you Plex them, you're gonna get out all
  450. of that IBIG from the system, which
  451. obviously is not gonna be efficient.
  452. I think that's such an important Pearl I
  453. feel like we frequently see second doses
  454. of immunomodulation therapy given,
  455. whether that's IBIG and then more IBIG
  456. or IBIG and then Plex, which again,
  457. not a great idea or Plex and then IBIG.
  458. And at least within the Guillain-Barre
  459. population, there have been multiple
  460. trials that just didn't show a benefit
  461. of that. In fact, there's maybe even a
  462. signal of harm with more of these
  463. treatments So again, pick one, stick
  464. with it, really give it time, at least
  465. a month, before you really have said
  466. this. did not work at all.
  467. The other thing that comes up so
  468. frequently, especially for myosynic
  469. patients is that they have sensitivities
  470. to a lot of drugs, or there's a lot of
  471. concern that a drug might worsen their
  472. myosynic crisis. So
  473. yeah, I know that list is like 50 drugs
  474. long, but are there common things that
  475. we see again and again in the ICU that
  476. we really do need to be mindful of with
  477. these myosynic patients Yeah, I think
  478. this is such a good point and you're
  479. right. I think when you look at the
  480. list is over 50 medications and there's
  481. contraindications and there's relative
  482. contraindications and there's definite
  483. and there's possible. So, I think for
  484. the sake of this conversation, I would
  485. limit it to what I think is absolutely
  486. necessary to know and what is absolutely
  487. necessary to think about A lot of
  488. antibiotics are on that list but I think
  489. there is three classes that I really
  490. think you have to be very cautious
  491. I mean, it's like a side. that's one
  492. class, that's a very definite
  493. interaction, your patients will be
  494. weaker. So if you can avoid that class
  495. altogether, that would be amazing, and
  496. that would be my recommendation.
  497. Luckily, there's a lot of other
  498. alternatives, so we don't see a lot of
  499. gentamicin, or tuberamisin, or
  500. amicasein anymore. Another class is
  501. fluoroquinolones, and then macrolides.
  502. So those three classes in the antibiotic
  503. group, so immunocosides, macrolides,
  504. and fluoroquinolones, you want to avoid
  505. them. Magnesium supplementation is
  506. another, I think, big topic. Now,
  507. although it's a definite interaction, I
  508. think you have to keep an eye on the
  509. numbers and monitoring these patients
  510. closely. Obviously, if someone's
  511. magnesium is 11, right? And you have
  512. a cardiac patient, you have to provide
  513. enough supplementation and repletion so
  514. that your patient is not experiencing
  515. those side effects. But this is just,
  516. to treat people, you know, with more
  517. caution to make sure that you're on top
  518. of their levels, to make sure that
  519. you're looking at that more closely than
  520. other patients.
  521. Another big class is neuromoscore
  522. blocking agents. I know a lot of
  523. institutions have actually very specific
  524. guidelines for rapid sequence intubation
  525. for these patients. The dosing is
  526. usually lower. We talked about how,
  527. you know, you have to reverse these
  528. patients quickly. A lot of these people
  529. post a war actually come to a monitor
  530. setting very quickly just because you
  531. want to monitor them because you want to
  532. make sure that they're breathing fine
  533. and they're waking up okay and all that
  534. in health and aesthetics. That's
  535. another class that, again, could be a
  536. problem and you have to really watch
  537. these patients. And then one last class
  538. that I think is pretty controversial is
  539. corticosteroids. We do use
  540. corticosteroids for mycenae and
  541. absolutely that is part of, you know,
  542. the algorithm for treating these
  543. patients. But how does corticosteroids
  544. can possibly exacerbate myesthenia
  545. initially. And that's why when you see
  546. high
  547. dose corticosteroids, you see IVIG with
  548. that, or you see that these patients
  549. are intubated during that time. And
  550. that's when we utilize that 'cause we
  551. wanna give them that extra support so
  552. that they can get that extra support
  553. while they're on high-dose, you know,
  554. methyl pred or other, you know,
  555. corticosteroids. Usually in an IV form
  556. initially with the high doses. But
  557. those would be my recommendations, you
  558. know, specific antibiotics we talked
  559. about, again, intubating with a lower
  560. dose of neuromuscular blocking agents,
  561. reversing them quickly, talked about
  562. magnesium and then corticosteroids.
  563. Those are really important. And they
  564. come up all the time, I feel like in
  565. clinical practice. You know, these
  566. patients are not patients that we see
  567. day in and day out, but they do come up
  568. frequently. And I think this has been
  569. such a fun way to kind of think back on
  570. how do you approach them with a
  571. conceptual framework? How do you make
  572. sure you're stabilizing them?
  573. respiratory perspective. And then
  574. really, how do you be thoughtful about
  575. which immunosuppressant you're using and
  576. the other drugs that they may be exposed
  577. to in the neuro-ICU? So with that,
  578. we'll actually wrap up any final words,
  579. Salia? No, I think just having a
  580. neuro-intensive is to help you in a
  581. clinical pharmacist and this podcast
  582. hopefully should get you ready for that.
  583. I love it. This was a wonderful topic.
  584. So until next time, guys,