Chapters Transcript Arteriovenous Malformation um, So I'm going to talk about, uh, you know, cerebral arteriovenous malformation. Um, if you talk about, uh, you venous malformation of the whole nervous system, that's gonna take a little longer time, But they do occur in the spine to where? But we're just going to focus on the on the brain part. Disclosure got two kids and a dog. Those are my kids and a dog. Okay, Other than that, I don't have any financial disclosures. Okay? Okay. Um, like, today's objective is really to understand the natural history of arteriovenous malformation, Understanding the treatment options available for it. And exactly where do we stand in 2021 there. So first, What is it That so, you know, in a normal brain, you have arteries. That branch, like a tree, eventually becomes smaller and smaller to the Oregon. They become capillaries. That's where the diffusion of you know, um, oxygen and glucose to the organ. And then eventually those capillaries returns the waste product back to the veins. You know what you have inside information? The They give this capillary network essentially and you get this essentially this ball of vessels. Um, it's basically a shunting is technically there shouldn't be any brain in between in that mass of tissue, the faster tissue where, um and basically the brain just go. The blood is from the arterial side. Just go straight to the veins, and we returned back to the heart. Um, if you look at the demographics, it's actually less than 1% of population. Has it compared to aneurysm, which is about one in 50 people? This is still much more aware most No, most of them are congenital. I would say probably 99% of them are congenital but there are some that form later in life. Um, the theory behind that is that you know, a two hit theory. Basically, your brain is prone already have, like a maybe a genetic defect. And then something else happened in your life, like a hematoma that occurred that caused this a second hit, and this forms later in life. And I'll show you a case that, actually one of the rare cases there that I actually had an encounter when I was practicing New York that this guy developed a arteriovenous malformation later in his life. Um, they're very rarely familiar. Um, they can be associated with genetic disease like Oscar rappers when do syndrome or search, Web or search represent your man and they tend to happen more than men than women. Um, if you look at the natural history, about 50% of people will present with a hemorrhage and you remember most of time they don't even know they have it. Because remember, this is congenital. Unless, for some reason you got your head scan he's just gonna walk around without knowing it. Um, The annual risk, we think, is somewhere around between 2% and 4%. So we typically use 3% of the average. And, you know, this is a little bit counterintuitive, you know, you think of we know smaller. A BM tends to rupture at a greater weight than, uh than the large jbm. You know, we we would think of large avian would capture more, actually, but think of like you have a larger a bm. The pressure inside that kind of spread out more Dennis of small A B M that the pressure is all centralized, and I think that's part of the reason why small avian tend to rupture than a large area, And about another 9% patient will actually present with seizures. Um, there are some people were with complained about headache. It's, uh, it's a little on the same side of the A V M um, the one kind of migraines. The oxygen Migraines can occur with oxide baby m so the migrants go away if you take out the A B M. And then the last thing is the vascular steal syndrome. If it's like near the motor strip or the set of the sentence sensory cortex because of the bypass of their blood straight from the arterial into the venous side, the surrounding tissue actually get less blood so you can actually get, like this stroke like symptoms. Uh, that's temporary. If you look at the natural history still, um, pregnant women, for some reason that there's some things can get worse. It might be duty because the change in blood volume, because the blood volume at least increased by 50% and the blood pressure changes with the baby as they get through the trimesters. And when When a VM watchers, the first time you do have a it's not that you don't actually have to treat it all the time. There is a slight increase compared to its natural history of an unwrapped A vm. During the first year, you can re rupture about 68% the first year, and then you will drop back to it's kind of natural history again. um, things that are high risk on a VM that makes it more to hemorrhage. Of course, if you present the initial hemorrhage, we'll talk about what the venous drainage mean, and then, uh, they associated with flow related aneurysm or international aneurysms. And then the other last thing is the location there. Uh, if it's deeper or versus superficial. So, for example, this is a special Martin grade, Uh, special was actually, uh, attending here in the 1980s there, and this is some of the work that he did, Um, and he published. Um, this is a this grading scale. It's actually a surgical grading scale. Um, but this is the most common use grading scale to a great navy. And when we diagnosed one of them, um, there's some little bit changes has gone on, um, in terms of adding more things to or that, but this is probably the best, um, in terms of understanding Which one should be operated on which one shouldn't be a. So you get a point for size between 0 to 33 to 67 million, greater than 60 then greater than six centimeters. Eloquence, you get 001 1.478. Um, important area. Remember, Not all parts of brain are created equal where some parts is not as important. You know, um, but the important point, like language motor, the deep structure to help with elements internal capsule, arrange them. Um, those account for one. And the last thing is, venous drainage is the is. The drainage pattern is a vein where the A. B s training is a superficial or the drain into a deep system, like in town. So we were vain, straight signers. So that's what the deep drainage mean there, Um so this is, for example, a grade to a B. M, where this is located in the motor strip And it's got superficial drainage, and the size is less than three cm. This is an example of a great three a B m, where it's located deep. It's probably around somewhere where the thalamus or hypothalamus is it's got a deep drainage. You can see it where, Uh, yeah, the chain into the the straight Sinus and And the size is less than three cm. And really, between four and five is just the only thing that's really different is the size one is 3 to $6 million and one. It's a greater than six cm, 6 cm and you can see on the M. I just basically covers the whole right side of the brain, just a B M. And that's something probably inoperable way. Uh huh. So when we talk about treatment options, treatment options, there's always observation. When you find in a VM doesn't mean you have to fix it there. So, you know, we kinda have to balance. You know, this is a lifetime risk of hemorrhage for someone forces each individual treatment options because each one has its own inherent risk. Um, you know, when we talk about observation, you know what it was like a great 4th Grade five. They never had much. There's no reason to touch it because the surgical morbidity, mortality and morbidity is significantly high for those a b m other things include immunization. Uh, organization can be used as a sole treatment. Um, versus palliative, Um, microsurgery about specifically for Grade one grade. You were moving and in some grade threes. And the last thing is radiosurgery using a gamma knife at, um to try to shrink the A B m. So observation really are for interrupting a bms. Especially great for games. Grade five, Um, and older people, older patients. You know, we got one recently. Just fixed it. Um, she's got a float with a dancer, but she's got a grade three a b m. She's in her 70s. No, we're not gonna operate on that A b m. Um, If you think, think about she never had a hemorrhage before. She's lived 77 years old. Why? Why? Why we do anything to hurt, you know, And the last thing we talk about the rubber trial, which was published in 2014. Then there was a five year study yet In 2020, and and this trial is, uh, extremely controversial where, um, you know the reason why this trial is extreme controversial was is basically randomizing medical treatments, um, versus medical training person invention for a network to a B. M. Um, in over six years, they were only able to enroll 226 patients out 39 sites they were hoping to get. Like something like 800 900 patients of 100 and some centers. The trial was stopped because they found there was a 3-fold increase in the eventual group. Um, the primary endpoint was to see, um, how many died or how many have stroke from any causes. It doesn't matter what the causes, And they found that medical in the medical patient group there was a 10% risk of symptomatic stroke or death, and medical intervention goes about 30%. And the definition of stroke is also kind of a little bit. They basically any symptoms, seizures, headache with new blood or stroke on the CT MRI, Um, the biggest Christmas about this stroke is one is is such a low enrollment. I mean, there's only 226 patients 26 of six years, and when we talk about a VM, we really have to talk about long term follow ups. You have to, you know, in most studies, you know when we talk about follow up long term, couple of years is long term. But this couple of years is actually not long. You know, a true long term study in an A V M study. You talk about decades, 10 years, 20 years. The reason why is we're talking about lifetime risk of hemorrhage. Um, so this follow up to the 2014 study, they only had they follow up these patients with only 33 months. and but they did find similar results at five months. The You know, the one of the biggest criticism about this is that there's such a, um, low representation of surgical treatment is on. So you remember they kind of just group the invention. Does anything radiosurgery accusations, surgery or Oh, it's Yeah and microsurgery in our literature neurosurgical literature. Um, we usually recommend microsurgery for grade one and grade to a B. M as long. It's not located in the elephant cortex. Um, but in this series, um, only five patients have microsurgery. The microsurgery Cold point was only represents 15 15% of the overall intervention, and you can see on the number here. You know, there's essentially a hodgepodge of of treatment option here, and they kind of just group everything in, um, together without really trying to standardize what the risk for each procedure was. Uh, and the one thing is, a lot of since this study was published, a lot of individual center published their own series, and it basically showed that there see, the individual center series is the data is significant Better than this trial. Um, but it does show what the important thing that they really get out from this is. If you look at it, the annual hemorrhage risk, you can see it. There was 2.2 Percent per year and a five year was 10%. So you can see if someone lives 30 years. You almost essentially flip a coin. That's about 50% chance this is going to hemorrhage in your lifetime. Okay, embolization. So embolization just means blocking something off. You know, um, it's typically used as an adjunct for microsurgery and radiosurgery. Very rarely. You can use this as a sole treatment as a cure, because, um, when I say rarely, we don't know enough about it. Because, like I said, you have to have long term data and There's no long term data for the sole treatment as a cure. Um, and the last thing is palliative treatment for grade four and grade five A B M. Things are recurrent hemorrhages, high risk features. Um, those are these. Now I'll show you examples of each one of those. And then this is the microsurgery. Um, like like I said before, um, in our literature north surgery literature, we recommend grade one and grade to a B. M two for surgery as long as they're not in locating eloquent cortex. And this is a special Martin Greater Scale web that was published, Uh, in 1986 and you can see great, great to you. The social outcome, um, with major mind deficits is very low. But as you can see as you approach grade three and Grade four and Grade five, The risk is significant there. And that's why most people most centered for Grade four and Grade five. They don't really operate on those. And we know, for example, though the hemorrhage rate and those grateful in grade five probably a little bit lower, probably somewhere around 1.5% versus truly high up like at 4% and then radio surgery. So radiosurgery is really reserved for treatment Eloquent cortex or someone who's got a hemorrhage. But they're older. They're not really a good candidate for any kind of surgery. Um, the obliteration rate, it's about for a V in less than three centimeters. Um, it's about 89%,, But it does take 3-5 years for that effect occurred, um, the those vessel has to thicken, Um, and in order to occur, and it doesn't occur immediately. So in that time between the 3-5 years the hemorrhage risk still exists during that period. And there are some with some where side effects, Um, including radiation necrosis. You know, I don't You know, this is not like getting a sun tan, you know? Um, so and the other thing is, there is something called chronic encapsulating interest, intracranial hemorrhage hematoma that can develop years later. And this is a really weird phenomena. And I will show you a case of that day that we actually operated on when I was a fellow away. Um, never even heard about it and tell her, um, until I was a fellow away. So when we talk about the organization, and so as a sole treatment, you really just was selected as a VM. Because, like I said, there's no no long term data. The material that we use for embolization Only been approved, probably for the last 20 years. Um, And then last thing is, you know, obviously we do palliative embolization of grade four and grade five because of recurrent images or highways features. So this is the stuff that we use, you know, essentially super glue. But it comes in a different vial. Okay, A little cleaner, um, and then coils to slow down the flows. Um, And then, for example, this is a VM, um, on the on the left on my light. Um, you can see it, um, the middle cerebral artery feeding into this This notice if I can point to it for this thing. I heard this is a VM. This is the arterial feeder. Mm. Ca. And you can see a draining. There's there's a draining Patrick coming faintly. That's the Venus outflow. Um, okay, so this is the material that we used to That's FDA approved for a B immunization. So this stuff is essentially a f mean final alcohol co polymer. It's dissolving the MSO. Um, some of the nurses nowhere. And they get a patient after immunization, they come back to the I C u. They smell like garlic. That's the stuff that we're using at, um, belies the IBM essentially, what it does this this this material kind of based on flow. Well, we try to build a plug first distantly around the catheter and then eventually pushed the the embolization material into the A B m the whole goal. This is like I said, this is an adjunct for microsurgery with your surgery. Um, the reason why is essentially turned this vascular bloody tumor and turn it into a, uh turned into the solid tumor makes it life a lot easier when you take it out. Um, I always compare when you take out in a VM that hasn't been analyzed. You get into something in new bleeding. It looks like a submarine just went to deeper and all the pipes started break there. Uh, so there's an example of an embolization as a sole treatment. This guy, um, he's in his sixties. Uh um, he had a history of wealth trade as a VM at eight years old. So Probably 1950s when he was eight years old and it ruptured it. I don't even know how they diagnosed because there was no CT scan at that time and he at work, he collapsed and become comatose there. And you can see, um, you know, they you can see part of the A V m. Here. You can see all this blood here, um Castle Adventure courseware. So basically, you have obstructive hydrocephalus there, and this is a VM. This is not a social a VM because it's in the warning area and you see the A V m here. This thing here with PC a feeder into it. Um, this is a lot of you, The same thing. You can see it in his native you trying to use a VM, and it drains you too deep Sinuses there. And I actually did this that, you know, I you know, because he was so sick. And so I immobilized him. You can see my micro catheter, all the lower turns to get into it. You know, it's like a when you go here to get to the VM. You can see the tube going around around there. And then basically, I emphasize this. Um it wasn't intentional to as a cure, but I was able to get the whole thing. You can see the Onyx cast is the cast where the the the polymer was and amazingly to somehow this thing was completely occluded there by this cast, and there was no residual ADM. And I will tell you, at that time I wouldn't. And he actually made a great recovery. Um, and he was he had seen a specialist over in, uh, in Stanford, and he was told that this was cured. Um, the guy Has seen about 1200 RPM in his lifetime. And this is the 12th cases he had that this was completely cured by the organization. Um, palette the treatment. So, for example, this is a 50 year old with IVH. You can see all the blood in here. You can tell the GM is deeper in the basically. You know what? My basal ganglia right here with the calcification in it, um, and when you do the when you do angiograms a lot of times kinda hazy. The reason why it's hazy. It's The flow is so fast you can see the normal festival very well. Well, because this flow just go right in. Come right back to the vein, right back to the heart. But when you when you do the angiogram, you can see there's an international aneurysm here. This is probably what ruptured and caused the, um the I V h So you can see when we take the micro Katherine all the way up. Why? To just in front of it. You can see this is the night of the the aneurysm. And basically, this is glue. This is a super glue. This is the N b N c b a NBC, a glue that we used to where included, Then you can see before and after. So this is a case where we this is a non operative case, and we just probably treat the highways feature, um, radio surgery. Um, For example, this is a 44 year old man with recurrent seizures, and he's got an AVM in his motor strip. Up here, you can see this is supplied by, and we will always be here, and it's a superficial drainage here you can see it's draining to cortex and eventually superior sagittal Sinus. There's two veins here. Um, it's in the motor strip. So this is not something you operate off without causing him to be completely white hemiplegic. So he got gamma knife, And this is three year follow up angiograms. You can see the IBM is gone. I will tell you. I have seen a VM that's gone an angiogram. Um, and somehow, over time it comes back, it's pretty rare. So, you know. So the problem is, like I said, these, You know, gamma knife has only been around for probably the last 20 years. So data We talked about long term data 2030 years. So people are still accumulating these datas. And a lot of times, when you read the literature, they will put long, long term follow up for gamma knife. Five years. I mean, five years is not long enough. You really have to see 10, 20 years. Like I said, you know, for these, uh, these studies there, um, this is a This is actually one of the cases here. So there's a 35 year old who was actually, like 34 35 weeks pregnant at, um, she become abundant in the at a meeting. Yeah, and she was blue and white people on the way here. Yeah, um, you can see this big white temple. I c h r i bh I mean, so we you know, obviously a young patient, most likely suspect. Um, there's an a v m there, so you know, we can We couldn't figure out what we're not gonna do a diagnosis because she was actually dying. So we actually took the overall for Hemi craniectomy just to relieve the pressure. And then if you look at the when we did the angiogram the next day, you can actually see the A V M right here. She's got this. She's actually got a I I guess you can call a normal variance or abnormal vessel. She actually got an accessory. Middle cerebral artery usually just got one military audio on one side, but she's got actually got two of them on the wayside and one of them, actually, all it does is supply this a VM here, and you can see the drainage in the back. And then this is a white for cable audio injection. There's a PC, a feeder into here, And this is the A V m. So we actually symbolized it. You can see the micro catheter, Um, to the A v m. This is a VM, and you can see the drainage pattern. And then once we emphasize is gone actually, uh, and then from the same thing from the other branch. And we took it to the old war and, uh, took out the A V m the clock and everything. She delivered the baby. She's about two years out now. She's made a complete, remarkable recovery yet, um, she actually doesn't live here. She was a different state there, but she made a recovery agent. She sent me a Christmas card last year with a baby. Yeah, I don't know. That was That was great. Yeah. Um, this is a 23 year old patient presents a headache and left, right, left sided weakness. You can see this big hematoma here. No, 23 year olds shouldn't have a hemorrhage in their head, and you can see the ABC right here. Um, and this is the angiogram. You can see this, uh, a VM supplied by a c a m c a into this thing here. And then you can see the superficial drainage and then into the superior sagittal Sinus. Oh, You can see there's a 3D reconstruction there of the what we do in the angiogram where you were, um, to show the A V m and everything and how to get there. So we emphasize some of this took it out, and this is a post op angiogram. You can see the the whole here, where the a b. M was. You don't see any blood vessel here because there's a hole in a gray area where this was, um This is a 65 year old woman came in with headache, Nausea, vomiting, And you can see the blood in the 4th ventricle way here complete. Cast it. She was actually awaken amazingly and just the angiogram show. There's a posted false IBM supplied by Esa and pica. And you can actually see this Internet the aneurysm here. So this is what we're not in tonight. And this is a flow with the Anderson with fluid, and I mean there's an ant on the branch of the festival. The supply of the A V m because of the increased flow in the vessel. Because last year's stress on the water festival and you will actually cause an aneurysm. And sometimes when you take the A V m out, this thing will actually disappear on its own when the flow return to normal. This has happened there. Okay, so this is the for orientation sake. This is she's sitting. This is sitting craniotomy. So she's sitting there at the top of their heads here. That buys down here. And you can see this is we're in the middle of taking this A B M out. We turn it into a tumor organization. There's some attachment into the veins here, Um, and we basically by pulling it out, going around it, try to this is on her left side because we're looking from the back. Take it out. And this is the whole thing. Is the a V m here? Despite this is a section that was a bipolar. This is a micro scissor. So the bipolar is to kind of coagulate. Um, that blue thing is to coagulate, um, the blood vessel. And then you cut and you can see we detach it from the top. Basically where the drainages and then the seating position is really great because gravity kind of retract everything for you. It just pulls everything down there. Um, and basically at the end, which is detaching from the West. And then that goes to pathology. Okay. No one. No one gets to keep that. Unfortunately, no, I have patient. Requested it to keep that. Yeah. Um, and so you can see pre op. You can see the VM here and post out. You can see some of the onyx material whether it was blue in there, so you just leave that stuff in behind. But the A B M has gone there. Um, so these are what I call kind of oddball ball. Case is kind of weird phenomena, right? So we talked about Daniel Day Nova Annual A VM. Um, and most of them are congenital. Very rarely. You get to know there's probably 50 cases reported in the literature about dane over a B. M. This is one of the cases I had when I was in New York. This is a 24 year old guy with a history of seizure. His first episode was a 15 when he had a season. He burn himself on a home home heater. Um, and the whole work up when he was 15 was negative. The top was the top. Right here is actually this is my at that time at 15 and then keep having seizures, and then eventually you came back and you can see, um, nine years later, he got he's got this a VM here, which is kind of weird. So this is a case of Dino behavior as an adult. And here's the A V m basically supplied by the CIA, um, supplying this and then it changed into the superior sagittal Sinus. So this is part of this is what I call it great one a b M. So we actually respected it, and he would and subsequently was seizure free. Um, so this is the last thing is this, uh, chronic encapsulate intracranial hematoma? Um, like I said, I never even heard of that. That doing residency? Yeah, I only saw when I was a fellow. So this is a 44 year old guy who had gamma knife for a left power a BMW in 1997. Um, and he had to follow angiogram to show the ADM was gone. And then he came back with new, medically refractory seizures. And this is what it looks like on the M R. I so you can see this looks weird. As you can see, there's something like like a bubble way, you know, and you can see this, you know, he's lying down. That's why this is blood here, and it's and it's layering. So something is bleeding in here. And we did Andrew, you know, and you can see here. There's something enhancing inside in this and he's the angiogram on and you don't see anything. There's nothing there. There's no early drainage. There's nothing. Um, so there's no signs of active a bm at all. And this is what looks like inside when they get gamma knife. Did this a VM even though it's gone. So some reason this is almost what I would describe kind of like a subdural hematoma. Somehow it forms a capsule around it. The capsule has a lot of new vascular stations and start leaking blood people actually taking the fluid from this inside, and there's a lot of actually a lot of, um um and you feel like basically industrial growth factor in there. Um, and it's basically almost like I said inside of it, you'll see the capsule completely new, has new vascular ization, and, uh, and you remove it. And when we do like what we call in the sound of green angiography in there, some of the festival actually lights up inside of this, and and, uh, you essentially have to remove this whole encapsulated, um, abnormality. So we're making a court economy. Here you go through it. And basically, you know, this is the 11 blaze. This is a very sharp blade making through the brain. You can see inside. This is just dark. It looks like a human literally just yellow is fluid. Essentially, it's just like what we encountered a lot of time when we evacuate subdural hematoma. This brownish liquid here, um, and the cap. And there's all this new vessel that growth onto the wall, the capsule where, um, and within the capsule, there's expectations and all the temptations almost. It's very similar to like I said, it's not going to tell me where it is. Um, different compartments over there, Um, and essentially, you have to get rid of the wall, and that's what's leaking at um, and it's. And if you don't do this, they were the wall. All it does is just keep expanding. Yeah, um, and I guess this is a pretty pretty rare phenomena, Um, to see here I do have. There's a patient I'm going to do an angiogram. They did get gamma knife a few years ago, and he looks like he started to develop one of these in his motor strip where this was where he was, where it was gamma knife on the MRI recently. It looks like it's starting to become one of these things. You know, to question this. No. Like I said, we would do an angiogram to see to make sure there's no residual a vm. But you know, he's having refractory seizures to where, just like this guy. But you can see you know you have to essentially moved the wall or the whole thing. So in conclusion, basically a three way VM is really just a really horrible group of vascular ation that there is. No one should fit off to treatment for these, and it's difficult sometimes to make a decision that on these patients, um, there's a lot of factors to go into a patient's age. What greatest thing looks like, What kind of symptoms are they having and what treatment appropriate for them? You know? Um, so a lot of time, we use a combination of everything above, you know, observation, immunization, microsurgery or even radio surgery. There are some centers that are even trying to fractionated radiosurgery for large A B M. To see you can shrink like a great for a B M six. You know, 600 IBM, two small sizes And then eventually, after like 10 years, they've become more manageable and take it out. So those are the things you know. It's like a It's like most treating diabetes or high blood pressure chronically, you know. But these are things that we're still trying to figure out how to manage these. Like I said, No, this is a lifetime risk of hemorrhage. First, is there the risk of these treatment modality? That's it. Very great movies. I always love those movies. So now we're gonna talk a little bit about nursing and pharmacology, implications of a condition called sympathetic Storm which is something that can complicate, uh, epilepsy stroke, intracranial hemorrhage, a number of different conditions and our first speaker discussing nursing implications is Jen joke. Um, she's the lead nurse practitioner in the Rhein Berg Neuroscientists Critical care unit. Good morning. Thank you, Dr. Silo, for the introduction. I am Jennifer Jack, um, high interest practitioner networks at university hospitals, Cleveland Medical Center. I am going to be talking to you today about paroxysmal sympathetic hyperactivity. First recognize. First off, I have no disclosures. I don't have. I wish I had a cute picture of my dogs. Would rival doctor who's but this presentation today is gonna be pertaining to their adult population. 1st 1929 Report in 1929. What's now known as paroxysmal sympathetic hyperactivity has confounded politicians over the years from its various symptoms and the differences between patient to patient. For over 70 years. It was a syndrome that didn't have its own name. It had about 30 names. And if you can see from the screen, one of the oldest terms was Diane Stephane Dion's a phallic fit or seizure. Does Otto know Mia or what? We talk about it as sympathetic storming In 2014, an expert consensus panel got together and decided they were going to give it a definitive name. And they gave it paroxysmal, sympathetic hyperactivity or psh for what we'll call it today. Psh! They also give a definition to make research a little bit easier. They define it as a syndrome, recognizing a sub group of survivors of severe acquired brain injury of simultaneous proximal transient increases in sympathetic and motor activity. They outlined the symptoms as tachycardia, hypertension to Kenya, different sis posturing and fever. They described a symptom or episode or paroxysm, that when someone gets a non noxious stimuli such as turning or suctioning, you get this exaggerated, sympathetic response. Not all patients have are. They do have spontaneous paroxysms. Most have stimulated induced paroxysms, and not everybody displays all five or six of those symptoms. At the same time, it's usually a combination, and take a cardia seems to be the most common symptoms. Psh is often described as a pattern of recurrent bursts. Dis regulated sympathetic activity resulted from severe brain injury or an increase in sympathetic nervous system activity caused by a disassociation, or loss of balance between the sympathetic and parasympathetic parasympathetic nervous system. There's a couple of different theories. There's multiple theories, but the 24 runner front runners, um, our disconnection theory that suggests there's a loss of inhibitory political centers control over the sympathetic control centers. The other theory. Excitatory inhibitory ratio. The two stage process. First, with expectation originating from the disconnection that descending inhibitory pathways. And the second the episode is halted by the recovery of the inhibitory factors. It's not that there's a failure to modulate spinal cord excitatory sensory circuits by inhibitory centers within the brain stem of acquired brain injury and patients that develop psh. 80% of those are traumatic brain injury, or TBI, Of TB patients. About 8-10% of those patients develop Psh. The remaining there's 10% that develop Psh from hypoxia, hypoxia or anoxic injuries, 5% from Strokes, And the rest of the 5% is a mixed bag of hydrocephalus, tumors, infection, an autoimmune encephalitis or hypoglycemia. We see PSH more in men than women, younger adults than older adults and patients with lower GCS. There's not one specific diagnostic test that can say someone has G S H. It's a diagnosis of exclusion. So as a clinician, I need to ensure that I've ruled out things like seizures, pain that hasn't been controlled. Infection P E D V T. Hydrocephalus. Any condition that might be drug induced, like neuroleptics, malignant syndrome or rhabdomyolysis or dehydration in 2014. During this conference, they did develop a tool called the P S H A M Tool Assessment Measure tool. It has two components as a clinical feature scale that rates the severity of the symptoms that are outlined in the definition. The second half is a diagnostic likelihood tool Kind of outlines about 11 criteria, um, that include The features persist greater than three days. They are seeing two weeks or greater post injury that there was an anti Semitic brain injury. Medications used to treat the alternative diagnosis or not effective, and medications that are given are resolving these practices, um, or episodes. It is becoming recognized that patients that experience that have poorer long term outcomes with psh our patients that have a lower GCS score have early onset symptoms and longer duration of symptoms. Episodes of the paroxysm can appear 24 hours after injury up two weeks a lot of time in nursing. What we'll see is, once we start winning a sedation down from someone who is ventilated, you start seeing the episodes. Episodes can last anywhere from minutes to hours if they're left untreated. Typically, you might see 3-5. practicing today depends on the stimulus, and typically it's showing that from presentation of symptoms after about two weeks, you should. You should see a decrease in the severity and symptoms, but they can last up to months to a year. Prevention and treatment of psh. There's three goals eliminating the predisposing factors that are precipitating the proxy ISMs, mitigating the excessive, sympathetic outflow and supportive therapies. It is really important to note that not treating the practice is, um the episodes of psh can lead to secondary injuries. Excessive hyperventilation can lead to vaso constriction. Excessive are uncontrolled hypertension. Prolonged arrhythmias can have cardiac involvement. You could have a re bleed for somebody that had an I. C H, and you can also have neurogenesis pulmonary edema. Treatment is focused on preventative therapies and a board of therapies, and those are pharmacologic. We're going to talk a lot more about that in Maribel's presentation, so I'm not going to cover that here. But briefly just say we work in combination with opioids, benzodiazepines, gabbas, dopamine agonists and alpha agonist. Nursing your essential in the management of this condition the communication that you have between nurses and families regarding triggers, therapies that are working and not working. These can make the difference for somebody having a prolonged stay needing more medication. It's important for you as a nurse, often the first person to see the the onset of symptoms and the one to treat them that what therapies are working for them. What medications are working, what's not working things that you want to focus on. Two is if somebody is in pain, make sure that we're treating pain, that this is not a trigger if you notice that somebody's federal making sure that we are treating recently, um, lot of facilities have gone to straight calf protocols using bladder scanning rather than Foley's And these patients you want to avoid bladder, distention and constipation. These are the patients that you want to ensure. Having every floor of six. Our Bladder Scan That can potentially eight on episode with these episodes are expending a lot of caloric. Um, they're they're needing. A lot of clerk needs, ensuring that they're hydrated, ensuring that they're eating. If you have a patient who's not an I V fluids N p o intermittently or just MPO, talk to your providers about getting them more nutrition or maybe talking with a dietician getting a consult. Mhm. Things like turning and sectioning. There seemed to be one of the common triggers for these episodes, so perhaps pre medicating before them also thinking about the environment. Is it too cold in the room? Is it too hot in the room? Alarms Too loud. All of these things can trigger episodes. So as a nurse, communicating between each other is very essential. But I think the most essential piece for you as talking with family again. You're the ones that are seeing the family more often than the provider. So discussing with them what we're doing, as far as you know why this is happening, what can we do to avoid the symptoms and the episodes? Lot of times you know, someone stroking someone's hand or maybe stroke in their face, and they're having an episode redirecting the family and letting them know that they're not hurting them, but maybe offering a different way that they can show their love for their family member. Thank you. I appreciate that. And the next presentation will be miserable talking about those pharmacological interventions. Those resources. Yeah. All right. Well, that was wonderful. And, um, now we're going to hear about the pharmacologic aspects. So Maribel Rangel is one of the farm pharmacists. Uh, she's critical care pharmacy resident in our program. So welcome, Mayor Bill. All right, so my name's Mirabel. I'm a critical care resident, and today I will be talking about farmer pharmacologic treatment in sympathetic Stormy. All right, And then I don't have any disclosures. And some objectives for this presentation are going to be to recognize drug classes using sympathetic storming, and be able to identify key drug adverse effects that you can see in some of these drug classes. So treatment is not a one size fits all, so it's very individualized, and it varies depending on what type of symptoms the patient is having. So different types of symptoms will be treated with of wide range of medication classes. So, like I mentioned, most patients will not just be on one medication they will be. Most of them will require multiple medication classes for management, depending on their symptoms. So with that being said, there are a lot of, um, pharmacologic drug classes that I can talk about today. Um, I'm only going to be focusing on a couple, so these are a select few. So we have our beta allergic blockers, Alpha two agonists. We have our neuro modulators, virtually acting muscle relaxants, opioids and then our benzodiazepine. So, um, like I said, I can talk a lot about all these medication classes. I'm only going to be focusing on the ones highlighted here. Um, I wanted to kind of point out some key, uh, medication classes that we don't typically see all the time. So I'm not going to be talking about opioids or benzodiazepines for this presentation. So just to start off with our beta blockers or beta blockers, we typically use are non selective beta blockers such as propranolol a beta law. But we can actually also use meta pre law. So these agents work by, um, stabilizing the effect within the central nervous system or the C n s. Um, to help reduce the role of our catacombs means so we typically will start our beta blockers in target. Um, patients who are experiencing hypertension, tachycardia and die a fiery says so, uh, those are going to be our target features for our beta blockers. And these are going to be, um, administered orally. Uh, there are some patients that might not have oral access. So I did want to point out that propranolol does come as an oral solution that can be given the other feeding, too, as well as moto BLR, our immediate release. Um, some adverse effects for our beta blockers. You can see it can they can actually potentially are hypoglycemia and also mass signs of hypoglycemia. So patients may be having they may have a low blood sugar, and they won't be having those typical signs and symptoms that we think of that comes along with our low blood sugar. Um, stopping our beta blockers can actually have a rebound effect, you know? So we stop them abruptly so we can see a cute tachycardia, hypertension and or ischemia. So just some things to be aware of when we stop them abruptly, um, moving on to the next drug classes are Alpha two agonist. So in this drug class will see our decks Meta, Tommy Dean and then our comedy. So, Dex, Metta Tom Brady is only gonna come in as an I V formulation, and then our clonidine can come as a tablet or a patch. So these, um, Alpha two agonists they work on the central Alpha two agonists to kind of prevent norepinephrine release has seen in the picture here. And like I mentioned are precedents or decks. Meta Tommy Dean is going to be given as an I V infusion. Um, be really cautious when giving it as a bullet because it can actually cause profound hypotension hypertension. So we most institutions will omit the bullets of precedents or decks matata machine and just start, uh, continuous infusion are clonidine, um can come as a transdermal patch and that one has to be exchanged weekly, and then we should be rotating sites. So when we're gonna use our alpha two agonists, um, in patients who are hypertensive, tachycardic, dystonia, if they have pain or anxiety, um, some adverse effects of this drug class are going to be hypertension. Like I mentioned, just be cautious when giving press Adex As Ebola as you will see, hypertension bradycardia is also a big one. Um, with our alpha two agonists, especially when starting our precedents or decks. Meta Thomason infusion Sedation. Um, so it does not suppress our respiratory drive so we can't actually use our precedents infusion and patients who are not intubated or have a secured airway. Um, and it can also cause withdrawal symptoms when we give it for over 24 hours and then stop it. So a lot of the times you'll see patients on precedents in our intensive care units, and then when they are ready to go to the floors, we'll try to wean them off. If we kind of have a hard time weaning them off of our continuous infusion, then we'll actually add clonidine on to try to help wean them off of the, um of the precedents. And then they can go to the floors on clonidine, and then we quantity and later, right next up is our neural modulator. So the main drug that I think of with your module is going to be our propofol. So this is a gabba receptor agonist and an n M D a receptor antagonist. So it leads to CNS depression. And this propofol only comes in a solution for ivy administration, and it can be used in refractory symptoms. So Adverse effects of propofol, um, deep sedation. So we have to make sure our patients have a secured airway before starting. Propofol can also cause hypertension and things. It does come in a 10% fat emulsion solution. We should expect to see hyper epidemiology and hyper triglyceride anemia, so we'll be monitoring for trade. Our triglycerides, um, pretty often went on propofol infusion. And again because of the 10% that indulgent solution that it comes in it provides. It provides a perfect environment for bacteria to grow. So we do have, Um, want to be cautious. It does carry a risk for infection, so we should be discarding any tubing and any unused propofol solution after 12 hours to try to minimize the risk of infection. It can come with infusion reactions, and then propofol related infusion syndrome, or Priss, is, although very rare, um, just something to be aware of that if patients are on propofol for long periods of time and if they are on high dose. Um, Chris is something that can happen, but again, pretty rare. But I like to always point out that it is a possibility. Next neuro modulator that we're not typically familiar with is Romo. Christine. So this is a dopamine two receptor agonist, and we typically use, um, Romo Christine to treat hypoxia and sweating, and it only comes as a capsule or tablet, and we try to tell our nurses to give it with food because it does cause some GI distress. You can also see hypoglycemia with promo Christine so it actually can be used for type two diabetes. It's not one of the first agents that we go to, but again, um, one of the side effects is hypoglycemia, such as something to be aware of. Um, it can cause lack of energy. Or patients can feel drowsy and confused. Um, it can cause visual or auditory hallucinations and, um, dyskinesia. And then it can also reduce our seizure threshold. So something to keep in mind, especially in our population that we're treating and last neuro modulators are back often. So this is, uh, as you can see, the pictures that gabby agonists and it decreases are excitatory neural modulator release. Um, we will typically use baclofen for spasticity, dystonia, closeness, um, and then post traumatic pain. And back often comes as an oral solution as well as a tablet. And then we also can use it intrinsically So for the oral solution in the tablet, we don't we can administer it at any time without regards to meals. For our inter sequel solution, it's always important that we have to be administering that inter secretly. We cannot use the Inter sequel formulation as ivy sub Q. I am or epidural E. So baclofen does have some adverse reactions, so G I distress, um CNS depression. It can also cause sedation. Our patients may experience lack of energy, and then if they have seizures and, um, start back laughing, they may lose control of their seizures. So also, with back off in, it does have a black box warning for, um, severe withdrawal symptoms. And we typically think of the severe withdrawal symptoms with inter ethical administration. Um, but it can also happen if they are on tablet or an oral solution for long periods of time and on high doses. So we mainly think of our black box warning for our interest. Equal solution. But again, it can definitely happen for the oral administration. So altered mental status, high fever, hypertension, muscle rigidity, bradycardia and seizures or something that we typically look out for in severe withdrawal symptoms. And last medication is our peripherally acting muscle relaxants. Um, and this is the one that Dan trolling we're not very familiar with. As far as like, this is not one that we typically see on, um, in patients. So it acts directly on skeletal muscles by interfering with the release of calcium ions. So we are going to be using, uh, the entry line for posturing and muscle spasms, and it comes as a capsule and then a solution for ivy administration. Um, I did want to point out that the ivy is a Mexican, so it can x travesty. And if it does extravagant, you want to gently aspirate the extra visited solution and then elevate the extremity for capsule we can administer without regards to meals. So some adverse effects seen with Dan Trillin are going to be flushing. You can see dermatologic effects, CNS, depression, muscle weakness, G I distress. Um, it does come with a black box warning, just like our back loaf in, Um, you typically see this one with the capsule over that I the Ivey administration of hip paddle toxicity. And you're mainly going to be concerned with the hepatic toxicity in patients who are taking a higher dose of the entry Lean. So 800 mg or more per day is going to have a higher risk of developing his paddle toxicity than those who are taking, um, Dan trillion at 400 mg per day or less. So that is the end of my presentation. All right, so for anybody taking their pharmacology test refresher So we're gonna have our speakers come up and, uh, opportunity for questions. So how do you decide what medications are working? I mean, is there like, an algorithm? Do you try them in a specific order or for a specific amount of time? Let me hear about answer first. So, unfortunately, there is not a guideline to kind of gear us on what medications we should trial first. Um, I will say, like give a patient experiencing like hypertension. Um, tech cardio will most likely start our beta blockers first. before we go into, like our I V infusion. So we'll try to do something orally and then, um, move from there. But, yeah, it's very individualized. There's not like if a patient experiencing hypertension, you give this. So it's really just dependent on the symptoms. And like, there isn't a guideline. Yeah, yeah. From our provider standpoint, it is difficult. So, you know, just thinking about the initial presenting symptoms with the type of cardio Mirabel said is a beta blocker aboard of therapy is using, like, a morphine or fentanyl. So it really is just seeing what the patient responds to that makes it very difficult. So, um, you had showed that gamma knife patient. And, um, are there some vascular malformations that are actually triggered by radiation therapy or a consequence of radiation for other conditions? Uh, yeah. Actually, um, you know, Cavan. Omagh's, um can be triggered by radiations. So Kavanaugh Mazar really just looks like a little mulberries, like, if you take it out literally. Just looking at it looks like mulberries. Just a little a bunch of little blips on it. Um, the reason why we initially we thought these were also congenital, but Then you had a bunch of pediatric patient with brain tumor. We got radiated. So you have a bunch of MRI scans over the years and awesome. This capital shows up in the area that they got radiated. So that's one of the, uh, basketball information that actually shows up with radiation, though. So we think that triggered somehow maybe a second hit to, uh in these patients and they grow these things. Like I said, it's not all patients with radiation gets it. So So there's a like, you know, probably a second hit to their brain. They were prone to it. They get, and they get one of these things. Is there any risk when you're doing these radiation on these E. M s that they would become unstable and it would actually cause it to rupture versus fixing it. Um, so the ratings show the radiation itself doesn't increase the risk of rupture. Um, so when you treat an A V M, it's really a all or non phenomena, you can't possibly treat something. Um, if you policy treat something, you have not reduced the risk of hemorrhage. So these things are you know, you're going to fix it all or leave it alone. Right? The only time we, like I said we only treat partially is for palliative reason. Because they are so large, um, or we current hemorrhage that can be taken out, um, about devastating the patients. Um, but The time frame takes about 3-5 years after the initial radiation. Because what happened? It cost the intimacy thicken. So essentially, eventually, these things blocked himself off. Um, but, you know, you're the question, then, is the long term follow up. I have seen cases of a VM that's gone from radiation somehow came back years later with just a subtle hint of the early venous drainage. And the patient is devastated by the hemorrhage. Right? Um, so the question is is that, you know, like I said, No. Like I said, these a lot of publications are only five years data, five years, not a lifetime. So, you know, that's the one argument against radiations of therapy is because no one truly knows. No, you gotta carry these patients for 30 years. 40 years to see the outcome. Um, but, you know, is it is it always gonna happen? I mean, there's always gonna be outliers. But in the meantime, while the radiation is taking effect, your wrist is still the same as as if nothing was done at that time, you know, so Created by
