Ep. 28: Paralyzer
You've heard of paralysis. But what if it came and went, triggered by rest, a meal, or exercise, and left no trace by morning? This week, Andrea walks Crystal through Periodic Paralysis, a rare and frequently misdiagnosed group of channelopathies that can leave patients unable to move for hours at a time. We break down how ion channels gone wrong can bring an otherwise healthy person to a complete standstill, why it often takes years to get a correct diagnosis, and what living with this condition actually looks like.
Medicine doesn't always look the way we expect. Sometimes the most dramatic symptoms vanish before anyone can see them.
0:06 Welcome to M4 Medicine, mystery, mayhem, and sometimes murder. I'm Andrea Marciano and RN with 35 years of experience across various fields. From direct patient care in hospitals to home health, geriatrics, and injury medicine, I face life, death, and everything in between. And I'm Crystal Miller and RN with 13 1/2 years of experience. My work spans home health, Hospice, emergency care, geriatrics, and occupational health. From the chaos of trauma based quiet moments with patients, I've seen stories that defy explanations. We've been friends for over 25 years and as nurses we've shared countless stories that have left us and our families speechless. Now we're bringing those stories and many more to you in medicine, mystery, mayhem, and sometimes murder.
0:53 Each week, we'll uncover cases that challenge the boundaries of medicine, sharing the human stories behind the science. From puzzling medical mysteries to shocking real life crimes, we'll bring you stories that'll leave you questioning everything you thought you knew while. We're both experiencing these nurses. We want to make it clear we're not here to give medical advice. This podcast is about storytelling, education and exploration, not diagnosing or treating medical conditions. This is medicine, mystery, mayhem, and sometimes murder. Let's dive in. Hey, Crystal, how are you doing? I'm. Doing great. How are you? I'm very well. Thank you. You got anything exciting to talk about? Anything's come up recently. Well, I had an exciting driving experience. I went to Utah to see my kids and my parents, my siblings, and we go through Kansas and Colorado and Wyoming to get there from Oklahoma.
1:46 And driving on the way back, we actually had a snowstorm in Kansas. And it was actually barely a snowstorm because there was just a little snow, but there was so much wind that it was blowing the snow off the drifts. And so it was only about 50 yards, maybe. Oh that's no fun. And with white hard to see edges. Yeah, exactly. So we were driving along going about 60 mph probably and all of a sudden there's a car in front of a truck in front of me and Holly Scouts car stopped. Oh my gosh. I honked my horn which I meant to turn on my hazards but in my think I honked my horn instead. I turned on my hazards and swerved around it on the left. It was in the left lane, swerved around it on the left and just missed it.
2:39 And then Odyssey was right behind me. She also she saw me swerve so she swerved and just missed it and then we both stopped. Had to get our adrenaline to calm down and then the guy just drove off. Odyssey thinks that he actually came off the other side going opposite direction and spun into our into our lane on the on the opposite highway. So it was wild though. Like it, I mean, it was turned sideways, so like the passenger side door was facing us and it was just the closest call ever. And in the snow and in low visibility, it was like a miracle that we got around it. Frankly, Oh my gosh.
3:26 Well, back in the day, I used to travel from California to Utah to go to school and I would come through in the winter time and I didn't drive back and forth that many times in the winter because it really is very dangerous, especially going over the passes. Donner Pass, not fun, but friendly. And there's a reason people got stuck and started eating their friends and family. But that's another story. Another podcast. I was one time coming and it was after, after the Christmas holidays and I was, I can't remember exactly how far I was, but I hit some black ice and spun off the road. Well, excuse, pardon me. I slid off the road and then I spun and I counted 12 revolutions there in the medium.
4:11 Just I, that's how many I counted. I don't know if that's how many actually happened. If there. I think there may have been more because I think by the time I thought of counting, I was already doing it a few times. And I had a friend who was traveling with me and he had been asleep. He woke up as we were coming out of the last revolution and he's really disoriented. I mean, just waking up and also coming out into a spin. I didn't feel like we were going to die because we were out of traffic, but it was not comfortable. And I was, you know, I doing all the things I was breaking and turning, but I think was helping because obviously it wouldn't, but I still felt like I had to be doing something. I was pulling the emergency brake and back in the day when we had them and and so as we came out, he started to get out of the car and I saw that he was still seat belted. So he was confused to the point that that made sense to him, but it wasn't a good idea.
4:59 So I said get back in. He looked at me and he did. And he shut the door. And just as he shut the door, we were hit from behind. Oh my gosh. Yeah, and I was riding in my favorite car ever, 1977 Toyota Celica. It looked like a Mustang and it was made of real metal back in the day. I think that if we had gotten hit and his head was outside the door he would have at least been injured if not killed after the other. The guy came came off and hit us and he said we got out and he said I hit some black ice I'm really sorry. And I looked and there was little damage to my car because it was real metal but his was kind of scrunched because his was a newer car.
5:39 And I said listen let's trade information and get the heck out of here because I came off you came off doesn't mean anyone else is going to stay on the road back here. So I'm going and he said no we have to wait for police. I said Nope, Nope, Nope, Nope. I so I got on. I got back on the freeway and traveled on and as I went I saw that there was no other spot for about 30 miles that didn't have a cement barricade between the lanes and about two miles up the road. Someone had probably done what I had done and they were in a Suburban. They had flipped over and the top of the vehicle was crushed down to the door frames. Oh my gosh. And I thought that could have been us.
6:20 I had. I had settled down because really nothing terrible happened. And when I saw that again, I had to pull off at the next exit, I was so freaked out because I knew that could have been us. There was another car that was driving with us, another set of friends in another car, and we all pulled off because we were watching each other. I think we even had little walkie talkies or radios or something. And because back in the day, you know, no cell phone. So I said I'm getting off the road. And we went off the road and we all jumped out and everybody hugged each other. And. They said did you see that? You didn't? All that was when we went off. All it was was sand. It was just a lot of stretch of sand between the lanes and I have no idea why that was there. But like you said, it was basically a miracle that we went off there and everything was OK.
7:08 Nobody got hurt. The insurance has ended up covering the small amount of damage I had, but it it could have been so much worse. And there was good visibility. That wasn't the problem. It just was black ice and black guy says, you know, is invisible. Yeah. And there's just nothing to do. People will kind of by habit slam on brakes, which is the very worst person thing a person can do. And I didn't do that because it hit and I went off and I never even had time on the freeway to do something. I just slid off and had no idea. So this person that may have done similar sort of thing, could they have come across from the other side? Like was there no barrier?
7:52 There, yeah, no. And and Ash, who was in Odyssey's car, saw the skid mark the the spin marks in the grass and the knees. You know, probably did the same thing I did, just wasn't as lucky and came across and hopefully they never got hit by anybody that is so. Scary. It's crazy. And it's interesting because for I, I sent Odyssey when she was 15 with her learner's permit to a defensive driving course called Driver's Edge in Las Vegas. And I, I didn't send any of the other kids. And I think it was a cost. No, 'cause it's free. I don't remember why I didn't send any of the other kids, but I did end up sending Odyssey and they teach all of these high hazard reactions. So it's, it's on the Motor Speedway there and in Vegas and like they have them go through an oil slick and they teach them how to go through snow.
8:38 And you know, when you start slipping, you do this. And so she actually had another point in the same trip where she was behind us and I noticed that she started that she started slipping, you know, so she didn't have control of the car. And so I slowed way, way down because this was like worse, this was like 5 foot visibility at this point. And so I slowed down. We went really slow for the next probably 15 miles, but she, she lost and gained control probably 4 times in that time. And just like it was really, it was. And she of course was like, I'm so glad I took that driver's edge training. So hey, I think it still exists. So if you want to teach your kids high hazard training? Nope.
9:18 Driving skills? Driver's Edge in Las Vegas. If it still exists and it's free, you just have to get there and have a place to stay I guess. Wow, yeah, that's that's huge. I remember when I was living in Utah, every winter I would go out somewhere that had no real barriers, like a big parking lot or something that was empty. And I just practice. I'd go and I'd push the brakes and start to spin and practice getting the feel of it again. Because after the short summer times, after four or five months, it's easy to forget how to respond. And I mean I would just. Cry and drive really slowly. That sounds like a good coping mechanism.
9:59 I actually preferred my method but and I did send my kids out that way too because I said if you just face it and you remember get that muscle memory back you'll be fine. But if you don't and just drive and cry, then you may not have as easy a time. I don't do that anymore. For the record, I've I've, I've learned the skill of driving in the snow now I just don't like it. I do not like it, but. No, I have come to realize I like heavy, heavy, heavy rain much less. Because the. Heavy, heavy rain. The lines are invisible. Yeah. I I. I think my scariest, I think my scariest driving experience was heavy, heavy rain at the point of the mountain in Utah. Yeah, it's all like, yeah, my in a storm we had not long ago, the visibility was so low and there was a semi truck that that was near me and kept shooting my window with water. I slowed down as much as I could and then all of a sudden I realized that I was not in my lane because I couldn't see the lines.
11:05 Anyway, that was harrowing. Well, that's probably enough of that. However, a little bit into our topic today. Well. That's fun. Yes, and you'll see why in a minute. At first you probably will say what? No, it doesn't, but it does a little bit. Anyway, today we're talking about periodic paralysis. Have you ever heard? That I have not. I hadn't either, and I'm going to, from here on out, call it pee pee because periodic paralysis is kind of a tongue twister. Yeah, I can imagine. This pee Pee is a rare group of genetic disorders that causes sudden, temporary episodes of muscle weakness, stiffness, or paralysis.
11:46 It's triggered by factors like exercise, stress, cold. Those are the things that segued into it, just in case you're wondering whether. Yes. Or high carbohydrate meals. They then have abnormal potassium levels. Oh wow, I know it's like what? I'll tell you more about this of course, but. We'll see you later, that's all. Thanks for listening. This to me was just fascinating because it's so intense the the situation anyway, so I'll tell you my sources. My sources were Sears, Cedars Sinai Organization, and in their health library they had some information about this.
12:30 National Institute of Health had a couple of articles and and studies I read about this. Mayo Clinic had some information, the Cleveland Clinic also, interestingly, the medical dystrophyassociation.org had some interesting information about periodic paralysis. And you'll, you'll see why that is in a little bit, that it is kind of an overlap for MDA muscular dystrophy, well, a association, but for the muscular dystrophy situation. So what is periodic paralysis or PP? It's a rare genetic disorder, which is really good because if it wasn't rare, this could be a much bigger problem because it is huge and it causes sudden attacks of short term muscle weakness, stiffness or paralysis. Now, this is one of the things that differentiates it from things like muscular dystrophy or Lou Gehrig's or other things like that.
13:25 And it is not progressive mostly. OK. Some it it itself is not progressive, but sometimes the effects of what happens will cause progressive problems. You see what I mean? It's not that it's guaranteed to progress, but some of the side effects become progressive if that makes sure all right. And these attacks may affect the whole body or just one or two limbs. Sometimes it's on one side unilateral or sometimes it's bilateral and they're not totally sure why it shows up that way in in people.
14:06 Some of the conditions, it's broken down into a few segments and some of them are called by names or things that relate to whether the unilateral, bilateral, but we'll talk a little bit more about that too. So there are several different forms, but they all involve defects in the ion channels. Do you remember what that is? Is that the calcium, potassium exchange? You're a genius. Yes, these are gateways that lead charged minerals, ions such as sodium and potassium, and calcium is one of them in and out of the cells. Now the reason that's important is because these ions are very, very responsible for things especially related to muscle. And you can have problems with calcium, which can cause heart issues are palpitations and potassium can as well. The thing that most of us think of when we think of low sodium, potassium or calcium is muscle cramps because again, it affects the muscles.
15:10 And so people will say, well, you had muscle cramps, you should have a banana or you have muscle cramps, you should you should have something with calcium and drink a glass of milk or things like that. Technically it's true. It may not translate quite as easily as that. All the bananas do really a lot. And that actually can be a problem because sometimes my mother had an issue with high potassium because she was eating a banana every day. So something about her body wasn't screening the extra potassium and so she started having heart palpitations. So that can be a problem on that side. So our bodies as you, I don't know if you remember the the teeter totter when they talked about these channels. And if you have too much, it's not going to work. And if you have too little, it's not going to work.
15:52 And I always remember the teeter totter, even if I don't remember why it was important. But but like I said, these flow of ions are part of how the muscles work, and in PP the ion channels sometimes fail and so the muscles don't work correctly. Now most forms of PP involve hypokalemia, and that's when blood potassium levels are too low. Some of them do hyperkalemia, which is when some of the potassium is too high. So I want to point out really quick for our listeners that it may sound strange that high potassium is called hyperkalemia and low potassium is called hypokalemia. But I want you to remember potassium is K on the periodic table, and that's because of kalemia.
16:36 KALEMIAI mean that's where kalemia comes from or vice versa, whatever. But I just wanted to point that out because when I before I went to nursing school, if you said hyperkalemia, I would have assumed you met calcium. Oh, yeah. OK, so good. Thank you. And so yes, cholimia is, is potassium calcemia is the calcium good? OK, thank you. And but most of them, like I say, is where the blood potassium levels are too lows, too lows too low. And a lot of us will have symptoms if our our potassium or calcium are too low or too high, as I mentioned related to my mom's episode.
17:17 And our potassium level should be at least three. But the normal range is considered 3.6 to 5.2 millimoles per liter. Usually we'll look at labs and it'll say millimoles or whatever the unit is. We're sure when we read labs at least I as a nurse look at it go, OK, 3.6 to 5.2, cool, we're good. Me too. I personally. Hear if it's millimoles or it's decibels or decibels. Inches or. When I come and and we're a better nurse perhaps I would care. But what I needed to know is where the numbers fall and people 3.6 is the normal of the low and at three people can start having significant symptoms, which is interesting to me because from the normal is .6 millimoles, which doesn't seem like a lot. But when you're only going up to three, I can see how a .6 can make a really big difference.
18:15 As we discussed, potassium is vital for nerve and muscle function. And heart function. Right. Exactly. Because heart is a muscle. Yeah, Yeah, yes. And we'll talk a little bit more about that. And one form of this PP that is significantly more dangerous than the others because of that. And this potassium problem will feed into what you were just saying, can lead to paralysis, respiratory failure or cardiac arrest with chronic deficiency, increasing the risk for high blood pressure, kidney stones and bone loss. And and so it feels like, how could this make such a difference? And do I have to get potassium in other ways?
18:56 Well the short answer is people who have a air quote normal system should regulate on their own. We should with just regular eating be OK. You know we want to avoid those highs. Like mom was doing a banana every day and sometimes she had two bananas a day that just put her system too high with potassium. Now if every one of us ate 2 bananas every day for the rest of our lives where we have problems, maybe, maybe not. Depends on our symptoms. I don't think I could eat 2 bananas every single day for the rest of my life for tears. But our body systems, typically, if they're working properly, can adjust and regulate by letting more or less of these ions into our cells.
19:40 We'll talk a little bit more about this later, but sometimes cells will burst for reasons that may or may not be related to anything specific. Sometimes when they die, they burst. And some things can cause cell death more than other things. We won't go into that today because that's another whole podcast. But when there's too much potassium that has left, or calcium or sodium has left the cells. Then they're not available for proper cell function and especially in the muscle areas, this can have a huge impact. Now just for general information, this PP attacks, these attacks can start in childhood or adulthood and when it starts can help determine which one this is.
20:21 But again, remembering this is a very rare condition and I'll talk about how many people get it in a minute. But one of the things to know is these episodes happen after certain types of trigger triggers in most people. And they may happen after someone works out hard and then while they're resting, they can have a problem. The symptoms may be mild or severe. They may last for minutes or days. It's all over the board, which then, as we'll talk about later, be so difficult with diagnosis. It can look like a lot of other stuff. This speaks to me saying that it is not a degenerative type disease, but this is something important to know. It may slowly get worse over time, but it doesn't have to. But it's more because of those side effects that I was talking about.
21:10 The hypokalemia may not get worse, but because of things being damaged, the person make it worse. Does that make sense? Yeah, it is a differentiation. Differentiation. Thank you. That wasn't right. Big words, mouth got stuck. But it is a difference because some things do progress and some things just get worse because of side effects. The frequency of these attacks tend to decrease this individual's age, which is good. And it makes me wonder if there's anything about puberty, anything about puberty that might change, because I know there's a couple of seizure disorders that when people go through puberty, they stop, and I don't know if they're related. Yeah, I've had three kids outgrow seizures actually. So I'm I am very familiar with that. So it makes me wonder if these things are somehow linked, but I don't know.
22:09 I didn't, I didn't look into that because that would also be another podcast. There is one case of one of these PP types and it's called Thyrotoxic and we'll talk a little bit more in detail about that in a minute. And that usually is on set after the age of 20. Interestingly, most thyroid abnormalities are manifesting after the age of 20. Not mine. Most there are outliers on both sides. The difference of this paralysis versus other types of things like Guillain Barre or you've got some types that are probably in the paralysis realm like trigeminal neuralgia, which can cause some paralysis. But this type, right, those are not in this category because they're not thyroid related.
23:06 And like I say, the most constant triggers are the rest that following strenuous activity and consumption of carbohydrate rich meals. The reason is these triggers increase plasm, plasma epinephrine or insulin level. So you eat a lot of carbohydrates, your insulin goes up and that shifts that intercellular ionic transfer that we were talking about and it takes more potassium and it lowers the serum potassium levels which then enter enters the episode. So the serum potassium goes down because these other things are going up and these heavy carb meals and I don't know how heavy. They didn't really say like you eat a whole container of spaghetti, is that the load? I don't.
23:50 Know right, Right. It does appear that many people who have this have different levels of tolerance and again, it's a rare thing so there's not tons and tons of people to to look at. But that shift at the intracellular shift is really interesting to me and how it can trigger pretty fast. But then you look at things like diabetes and insulin and sugar levels can alternate pretty fast too And sometimes depending on how air quote fragile a person is will depend on how fast these transfers occur. Now that's true in this, the thing about hypo PP as well, because different people have different tolerance levels, which is the one thing that I realized during all of the research I did is that everybody's different.
24:38 There are some general guidelines, but there are some things that are similar. And like I say, the less consistent triggers that we were talking about earlier about the car accident, stress and cold is excitement. So if someone has extreme excitement because they're there goes that adrenaline or stress, fear, cold temperatures, high sodium intake, because as you can see, that would change those pathways too. And alcohol consumption or anesthesia, those are things that can trigger these events, but they are less common. Attacks vary widely. Some have once a week, once a lifetime, several times a week.
25:19 It doesn't seem to matter. Even if there were seven people that had the same type of hypo PP, they may have two totally different presentations. This is the one thing I've seen about women versus men. Women tend to that is good. I mean, women tend to have fewer attacks than men. One thing we have less of, right, and of course I'm not making light of people having attacks, but really one thing I've ever seen that women have less than men and they don't really know why that is either. In the research that I did, they didn't really seem to have any particular reason. If I were a person that had this, I feel like it would be super stressful because if I'm a person who doesn't know if it's going to be attacked for minutes, days or weeks, I don't know how a person could get along.
26:10 Function. Yeah, Yeah, sometimes it's mild, like they just have generalized muscle weakness and they have more pronounced involvement of proximal, which is the near the body than distal muscles which is away from the body. So like fingertips are less affected than upper body or upper arms. You see what I mean? Severe involvement of these muscles can be life threatening. And we'll talk about that in a minute. Because when we think about, I think we forget how many of our systems are run by muscle contractions like the heart, Big 1. And I feel like people may not realize how many of the body systems are at least facilitated by muscles, even if they aren't run by it.
26:54 Because if you think about the lungs, the lungs aren't a muscle, but the diaphragm is, right? The diaphragm is what helps open and close the lungs. Right. So if your diaphragm is paralyzed, that's going to be a problem. That's Yep, that seems like a problem. Yeah, yeah. This is part of the general overview and I'm going to go deeper into some of them. But during one of these attacks, whether it's a muscle weakness or a complete paralysis during a physical exam, the muscle weakness is accompanied by hyporeflexia, which is a decreased or absent reflex response in your muscles. And if it's completely gone, it's called araflexia. And that's when somebody, not just somebody, when a doctor or someone with a reflex hammer, just somebody comes out, you, anybody. But when they use the reflex hammer and there's no response, where is typically, typically a response behind the elbows, behind the knees, behind the ankles, things like that. If there is no response or decreased response, this can be a symptom.
27:56 It can be a symptom of other stuff too, but it's one of the things that they watch for. But actually in front of the knees. But they do behind as well. But yeah, you're right. They do in front and behind. You're right. I've never had behind done, only in front. I feel ripped off in a more in a more complete neurological exam. They'll do more of them. And I was just envisioning an exam I witnessed the other day. But and what's interesting to me is many patients report porodromal or early symptoms such as fatigue, paresthesia, which can be some numbness and behavioral changes shortly before the episode. The brain man. Right. And maybe this is something that if people do get these, I kind of was thinking of it like an aura with a migraine to see things or my vision would change or or something would happen. And so I knew it was coming and if I can put some caffeine and ibuprofen on board fast enough, it wouldn't happen. So I feel like people that have these early symptoms could really lessen the effects because they will do what it takes, like increasing their potassium or decreasing them if they have the one more rare type. That's to me.
29:04 Behavior changes. That's wild. Like, Oh no, mom's suddenly being really nitpicky. I think she's going to be paralyzed soon. Exactly and exactly too. When you look at people who become hangry, many of them are hypo or low blood sugar episodes because our bodies, while they have a lot of give, there's some parts of our systems that are very tight. They have very little differences that it can tolerate before something starts to go wrong. I just think sometimes of how many systems in our bodies have to be working at every moment in time for us to do. Yeah, I agree. Anything primarily stay alive.
29:45 It's crazy how much of our body is autonomous. Also, we don't have to think about it functioning. Really what we do think about is not even necessarily any sort of function because it's like I am going to pick up my phone, right? But I didn't think about I'm going to take my hand, I'm going to out stretch my fingers. I'm going to place it down on the phone, then I'm going to in stretch my fingers I would say. And then I'm going to. Lift my hand. Like we don't think of that, right? We're just like, I'm going to pick up my phone and then our body picks up our phone. It's fascinating. It's fascinating. There's a lot going on in our bodies, that's for sure. Well, there is. And I always think of the fact we're just your example. I'm going to reach out and touch it. Well, I've reached out the right amount of degrees. I've put my arm in the correct angle and positioning and I have done all of these things without, like you said, even thinking about it.
30:35 I'm, I just reached for this. I could not solve this math problem in my in any way what it took for me to get there in a math sentence. But other people can. I can't, nor do I want to, but I just. Yeah. But my brain in the meantime has done all the things it took to create lifting or moving something and every bit of what happened there. So I think when we do things like forget or become distracted, we just get to remember all the things our brain does correctly, not those things. Yeah, exactly. And one of the things, back to our topic, one of the things that individuals have with the Hypo PP is they have mild muscle weakness between attacks.
31:16 So some they'll have their attacks and then between them sometimes they'll have mild muscle weakness and that can improve with mild exercise. So I feel like maybe this is hypothesis by Andrea, but I wonder if that's moving some of the muscle strands so that it can balance out those hypo or hyperkalemic cells. I that's my theory and I only just thought of it. I did 0 research about that so if I'm wrong and people are experts at hypo PP please. Forgive me. But like I said, there are some main forms of pee pee. The most common is the hypoclimic. We've been talking about the low potassium, and that is, like I said, the most common.
32:05 Now get this, This is the list of things that can trigger. We talked about a couple of these already, but we talked about heavy exercise and food with lots of sugars and starches, licorice, stress, cold temperatures and some medicines. Now you may be wondering about licorice. Yes, I am wondering about licorice. That was I licorice. I mean, it's on the list. Licorice. How is that a thing? Well I'm glad you asked because licorice can cause hypokalemia because of its active compound glycer cyreazine, which I may be pronouncing incorrectly, but it blocks an enzyme which is listed here. 11 BHSD 2 Where you of? Course, of course, we're talking about black licorice, correct? Yes, yes, because red licorice is not I, it's not truly licorice, but thank you for clarifying. So black licorice and if you want to look at that enzyme, go ahead.
33:10 But it blocks an enzyme in the kidneys that normally inactivates cortisol. OK, So when I was reading this, wait, cortisol. So that's interesting and it leads. Hormone. Right. It leads to excess active cortisol. This build up of cortisol excessively activates mineralocorticoid receptors. My. Goodness. Mimicking high aldosterone then causes the kidneys to excrete too much potassium and retain sodium. Wow. That results in low potassium and high blood pressure. So I'm good, but wow.
33:52 You're also good because it rarely causes PP. Only 40 cases have ever been described. Wow. But I thought, Oh my gosh, And how did it if there were 40 cases? No idea. However, it was a thing and has been a thing. And actually, we're going to talk about some case studies where it was a thing, which to me is wild because I've eaten black. I do like black licorice. I don't think I've ever eaten enough to get to this level of having all activating compounds. Dumping all your kidneys kidneys dumping all your potassium from your. Kidneys Exactly exactly so when I read the licorice I, I immediately had to go down a rabbit hole for that for a second I'm.
34:38 Sure I I would too I think. Anyway, hypo PP is a rare and I keep saying rare because I want people to understand it really is very rare and it's one person per 100,000 people. OK. So that's pretty rare. You're the one of the 100,000. It doesn't feel rare as most disorders that it doesn't feel rare when you're one that has it. But that to me was comforting and I would probably know by now if I had it. And yeah, yeah, probably one of the other things to know is that when the serum potassium levels drop below 3, like we talked about affected fibers, the muscle fibers develop sustained depolarization. Now the way muscles work is by polarizing and unpolarizing, but the, and that's why the ions that have charges are important because it changes the polarization. Now these fibers, when they're depolarized, cannot relax or tighten.
35:39 They can't do their their movements, so they're electrically inexcitable. They just can't be changed. Normal fibers undergo hyperpolarization in response to a drop in serum potassium. So if you and I had more potassium than we needed, ours would be hyperpolarized, which would then 'cause some of those spasms and things we think of with potassium, which is interesting because I know that I've had cramps. I've thought about take, I've eaten a banana, which actually is kind of contraindicated because if mine is probably already hyperpolarized. So that's another whole thing. But then it shifts the ions and there's all kinds of stuff that goes on behind the scenes, which is shocking to me. And so instead of being ready to go, their muscles are not ready to go.
36:24 They don't, they just don't respond. The types of hypo PP have been both familial and acquired. So many of these conditions are genetic. They've carried on through the family lines. This hypo PP does not always, more typically it does the not. But there are some types that occurs from other sorts of conditions, which we'll talk about in a minute. But mostly there's a mutation in the skeletal muscle calcium channel gene, which I won't even try to say because it's a big, it's a big one thing. It's the one that helps work on the regulation of all these muscle ion channels.
37:06 The mutations disrupt the normal flow of electric current through the voltage sensor domain of the ion channel. And there is actually a voltage sensor. It's not quite like what we use in our electrical processing, but it's it's the sensor that tells whether the ions need to charge or become positive or negative. And I just don't even know how well this works. And I figured it out again, no idea. Yeah, it is wild. The bottom line is this causes what's called flaccid paralysis, which just basically means the muscles can't move and. That's bad. Yeah, that seems bad. I think that that moving muscles are a good thing. I really feel that way too for we are caring professionals though, so take our word for it.
37:54 We know now that things working are good, and the most important thing in this hypo PP is prompt diagnosis and treatment. And that can be a challenge which we'll talk about in a little bit, because if it's not promptly identified, some other things can happen which can cause some morbidity. They can cause death because it leads to the cascading chain of events which can be fatal. Again, that part of it is not that common. Usually people have some time once they started to develop some things. The good news is, if a person is paralyzed, they usually go for care. They don't even ignore it.
38:37 Oh femor gosh, you know I can't. Oh, this is awesome. That's weird. I'll watch TV. And most people will go get some care, which is is good because it does facilitate an earlier diagnosis. Then a lot of things will sit around going well. It's probably not that bad, not much fell off. Right. Wait, am I having a heart attack? I can see a little bit. Yeah, it's probably indigestion. I have a funny story about that actually. As you know, and I don't think our listeners know, but I, I used to have SVT or Supra ventricular tachycardia, which means your heart just beats really, really fast because it's getting sent electrical signals that it's not supposed to be getting. And what happens is normally you have a sinus node on your heart that sends out the signals and that's what supposed to send out the signals. But your your heart starts sending out signals from other parts of the of the heart and so it gets confused. Anyway, Long story short, my heart rate when I was in SVT was 300 beats per minute, which as you can imagine is very very fast and very very frightening and. Very. 1st and very unpleasant so the first time I was in SVTI was driving and it's when it starts it's starts feeling like like heart palpitations, but then it just gets worse and I don't even know how to describe what worse is other than like my fingertips started to hurt and then I was having a hard time like catching my breath or yeah I mean it's just kind of hard to describe so I pulled over into a gas station before it got really bad but then as I was as I was sitting
40:11 in the car it got it was getting worse so I got out of the car turns out standing up makes it worse worse. So then I laid down in the back of the car and the whole this is the funny part of the story. The whole time I'm thinking to myself. Am I having a heart attack? And I don't want to call 911 because it will be so embarrassing to have an ambulance come to this parking lot. And I marvel that in that moment, I really was saying I would rather die than be embarrassed in a stupid. Gas. Station parking lot, it's and I'm a nurse and I was a nurse at that time, but there was just, again, obviously it wasn't a heart attack and I did call 911 and I did get it treated. I ended up having another episode before I got it treated and that was equally as scary and horrifying and terrible and fast. 300 beats per minute. But yeah, it's so it's there are people, myself included, I don't think I would be this.
41:03 I don't know. I don't even know. I don't know if I'd be the same way. But there are people, myself included, who will go wait and see. Right, right. I had a patient come into the clinic I was working at who had called me. I was just a triage nurse and I was on the telephones at the moment. He called me and he said I think I'm having a heart attack and 911, this is how old I am. 911 system was newly available and I said call 911, don't be on the phone with me. And he said, well, if I'm not having a heart attack, it'd be really embarrassing to have someone come to the office. I'm like, dude, and if you are having a heart attack, it might save your life. He wouldn't tell me who he was. And we didn't have reverse directory or anything. And I had no idea who it was. And he hung up.
41:44 And then I'm like, Oh my gosh, he's going to go die. Well, then about half an hour later, I didn't put this together until a little while later, about half an hour later, this guy came in and he had checked in and said he was having abdominal pain, he was nauseous and things like that. Turned out it was the same guy. And then he came in and he was having a heart attack. Oh, wow. And we found out later he was the same guy. But we didn't know it then because like I said, there's half an hour between. Why the heck would he come to an urgent care clinic and not the ER? Because the ER was only down the road, for heaven's sakes. But anyway, blah, blah, blah. But that was the thing. I would be embarrassed if it's not this. Yeah. So what? Yeah. Better than dead? I know it's, that's what I mean. Like I said, I did finally actually Odyssey called 911 because I was incapacitated, but EMS eventually came. But I just, it just, I still marvel at the fact that I'd rather be dead than embarrassed.
42:39 I can't imagine that's actually true, right? Like in my rational mind. But that's what I was allegedly supposedly thinking at that time, you know, because it could have been a heart attack, Who knows? I don't know. I don't know what SVT I do now, but I didn't know what SVT felt like. Well, in your defense, when the heart is acting inappropriately, there's less oxygen going to the brain. That's true. So maybe the decision making in that moment is reduced because there's less oxygen in the brain. But still, it's true. The embarrassed thing is, is a common thought. I've heard people say it a lot of times when they've come into the to urgent care or when I was working in the hospital and they had come in after they've been through the ER. They said, well, you know, anyway, yeah, it's wild.
43:20 It's important to get care immediately if you think something's going on because better better early than too late. Yep, I agree. Again, back to our topic. Thanks for the examples though. So hypocalemic PP often starts in late high childhood or teen years. The average is between 5 and 35 years of age. Oh my gosh, imagine being a 5 year old and all of a sudden you're paralyzed and you don't know why. Or the parent of a 5 year old. Or the parent of a 5 year old. Yeah, for sure. However, that gap to me was amazing. Five years. The average age is between. Is that even an average? I was just going. To say, can you call that an average? So I don't know, but that's what the information said when I was researching it. And attacks of the skeletal muscle weakness may last again from a couple hours to a day.
44:08 These tend to happen at night or in the morning. Which brings me back to the thought when they were saying it can happen after heavier exercise, the rest period. So that seems to be the case. They may not be able to move it all during the most severe episodes. That would be so scary, and I'll tell you something about that in a minute. But after age 50, it may cause permanent weakness that slowly gets worse, especially in hips and thighs. And again, the condition itself is not progressive, but the damage it causes can become progressive. And one of the things that removes it from like I said, the ALS or Lou Gehrig's or things like that. Now my partner Max had gown beret and he woke up 1 morning not really able to move his feet and legs quite right. And he he thought, well, that's weird as we do and and continued.
45:05 And by the time he called the ambulance, it didn't take that long. It had only taken about an hour or so until he really couldn't move. And it was getting progressed. It was progressing much more quickly. And he was in the hospital for, I think he said he was in the hospital for two weeks and then he had to go to rehab because he had to learn how to walk again because it became to the point that he was paralyzed to his neck. Did he get a tick bite? How'd he get young Beret? He still doesn't know and because of it he can't take some of the immunizations because of the inactive ingredients that can cause a flare in a virus. The Andre is a virus and some things can flare it. He can't take the flu shot. He can't take a couple other things that have too much active virus.
45:52 I guess in it is the problem. Yikes. But the one thing that is different about that is this is not periodic. That was an event and he is more susceptible to it in the future, but he doesn't necessarily ever have to have another event. Wow. And the these are periodic. So you might have one and then as they mentioned, maybe not ever again or in a few weeks or in a few years or or whatever. And that again to me would be so scary because you just don't know. It could, everything could be fine. The good news, I guess is that if it starts, it usually starts with weakness and then progresses on. But say I'm driving. That start feeling, my thought from the very beginning is somebody driving?
46:37 Yeah. And I don't think that it would be the same as epilepsy or something because they have to pass a certain period of time and there can be controlled by medication in order to drive if they've had a seizure after. I think it's a six month time frame, is that right? I can't quite remember. But it's a year. Is it a year you? Have to you have to be a year without seizures medication free. Torin just went through it. So it has to be medication free? Oh I thought it could be controlled with medication. I think it's three years controlled with medication. Oh, and that's a huge impact on life. I have someone I know who had had had seizures, had just passed the window of everything's OK and had a seizure. No.
47:23 And now she can't drive again. Yeah. And she's devastated because as an adult it affects every blasted part of her life to be able to drive and get herself around and her dependency on others. Now is is back and she has no not adult adult children, but driving age children who she can't go anywhere without them. Yeah, Holly and I were just talking about this. Yeah, Holly and I were just talking about the same sort of thing. Obviously I don't have seizures, but knock on wood, but when I had my hip replacement before, before I had my hip replacement. And then after I had my hip replacement, I was dependent on my adult and driving age children and even little things like like we would go to a seafood buffet and they would have to go get my food for me, right. Like, and, and it it because it's not just frustrating for, we'll say the patient or the, you know, it's frustrating for all those people too.
48:15 Those kids don't want to have to take their mom grocery shopping every time, you know what I mean? And kudos that they do. And you know, like, but it's just, yeah, that sucks. That's what I'm saying. It does. And fortunately now there are a lot more things that like DoorDash, Instacart and all of the things that people get stuff brought to them. But still it's there. It's always there. And then now we've gone through one type of this. We probably ought to move along a little bit. There's another PP that we had alluded to. It's hyperkalemic which is the high potassium and this may cause attacks and the triggers may include heavy exercise, fasting, stress, cold and some medicines which are similar.
49:02 Fasting one was interesting to me. Yeah, I would think and your testing would go down if you were fasting? I would think so too, but we get to remember that these people have their gates, their ion gates, if you will, which is really what it's called as an ion gate. But it's, it sounds like it's a made-up thing, but the ion gates are not working properly. So they let out too much or not enough potassium, sodium, calcium. In this case we're talking about the potassium and the symptoms for the hyperkalemic. The high potassium include weakness in the arms, legs, shoulders and back, muscle stiffness which is myotonia and attacks Usually asked minute to hours with normal strength between episodes. Be thinking to yourself, doesn't this sound very same? Yeah, except for the minutes to hours as opposed to months or whatever the.
49:51 Yeah, or days, right. And again, diagnosing can be really a challenge, especially because it's considered fairly rare and hyperkalemic PP often starts by the age of 10. These attacks of skeletal muscle weakness last an average of 30 minutes to four hours like we're talking about. So it's shorter and that's good, and still no bueno. Yeah, it's I would not like to be paralyzed for four hours even. No, I don't. Think I'd like to be paralyzed for 5 minutes. What am I? What? Am I really There's no time frame? I would like to be, but then we have some paralysis which we are planning an episode on another time, right? The attacks tend to be frequent but less severe than other PP form. So they may have them more often, but they're less severe, which in some ways seems to me would be more like disrupting. Yeah.
50:38 True, as people get older, they may have fewer attacks, but they also might have permanent muscle damage that slowly gets worse. Again, not because of the hyperkalemia, but because the results of the hyperkalemia. There's another kind called Thyro Toxic PP and this is a little bit clarified in its name, but it's high levels of thyroid hormone that might cause attacks. Thyroid related problems are also usually present. Other triggers may include exercise, meals with lots of carbohydrates, and stress. Wild. Yeah. And this form appears mostly in men, especially of those in Asian backgrounds. One of the things, yeah, the predominance of male versus female is 9 to 1.
51:22 So for every one female, there's nine males that have had this issue and and familial cases, which is the ones that run in family exhibit an autosomal dominant inheritance pattern, which means it comes through with the woman dominant gene is the way I understand it. It manifests normally with milder clinical expression and attack rates in women compared to men, meaning women again have less severity and it's less common. It tends to start between 20 and 40 years of age. The attacks happen anywhere between a few times a year to a few times a week. Again can last from hours to days.
52:04 And they also may have the thyroid latest symptoms such as anxiety, sweating, weight loss and an abnormal feeling of the heartbeat like palpitations. Some people with thyroid toxic PP don't have symptoms of thyroid disease, especially early on. So this is another thing where it itself hasn't progressed, but the thyroid problems may have progressed, which makes this worse. And some of those other symptoms will come up for people, like I say, the thyroid symptoms, and sometimes that is how they identified as those thyroid symptoms, like the sweating, anxiety, weight loss and palpitations. And the final one that we're going to talk about, there are a couple other kind of outliers, but these are the main ones.
52:45 But this is called Anderson Towall syndrome, and this is named because of the people who discovered it, as is common in these sorts of things. This also is a shift in the potassium levels and triggers may include guess what. Exercise, stress, and carbohydrates. Well, certain medications, exercise and stress, which makes sense to me really, because you've got those. We talked all about the cortisol shift and all of that, and that makes sense because things are in motion, things are moving. Those gates are going to get rusty and and not work properly and certain medications, like I say, can happen.
53:26 And with this form of pee pee, this is the only form of pee pee that they have a certain set of physical features. It includes a broad forehead, widely spaced eyes, low set ears and a small chin. And right, they have the genetic defect that can cause these. And some of the other physical things are like I said, it's called microganathia, which is the undersize or under belt low jaw. And it's people you see that have most of us have our, our chin will point out a little bit. It'll be a little bit outside of our jaw. It'll be pokey, if you will. I don't even know how to it'll protrude a. Little it'll protrude in a profile. Right there you go.
54:07 Thank you. And people with this syndrome, their chin is almost not even there. It just sort of from the. Like Mitch McConnell. Oh, does he have that congressman? Oh, OK. Maybe. I don't know if he would be actually diagnosed with it, but maybe. But it's I mean. Just the chin. I don't know about that I'm. Not diagnosing him but what's interesting is in profile I've seen it in people that have, but in profile they have just their lip and then goes down at an angle. There's no protuberance. What's that disorder where people are, They look really old and they get old really their bodies accelerate syndrome. No, that's not the real thing.
54:49 Anyway, it doesn't matter. But they have this underdeveloped jaw and they have clinodiactyly of the 5th digit, which is where the finger cursed sideways most often link little finger bending towards the ring finger and it's got an abnormally shaped bone. And then they also can have the syndactyl, which is a condition where two or more fingers are webbed. So they have a variety potential of a variety of physical attributes that will be telltale because of this condition and the the genetic disorder. They also tend to have scoliosis in short stature. These mutations are in the ion channel.
55:29 So they they start mutated more than some of the others. It sounds like from what I was reading, those multiple systemic systemic issues, this is is the one that is the most dangerous because it causes more problems with cardiac rhythm disturbances it can cause. That itself can be huge. And they're saying that Graves' disease is the most common cause of the thyrotoxic condition. If you've ever seen anyone with Graves' disease, they are the ones that have the buggy eyes sometimes. So this can fall into that. Graves' disease is not hypo PP, but Graves' disease can trigger hypo PP. It's the cause of it in some cases. It also has a greater incidence among men, although hyperthyroidism is more frequently diagnosed in women. So the incidence of this hyperthyroidism is more frequent in women, and the hypo is more frequent in men. Just for the record, Graves' disease is more common. Excuse me?
56:31 Graves' disease is an autoimmune disorder or the immune system mistakenly attacks the thyroid gland, causing it to over produce thyroid hormones. So that graved. With so is Hashimoto's thyroid toxicosis, which is what I have. I was actually diagnosed with graved disease incorrectly and then that diagnosis was fixed to Hashimoto's. But yeah, also an autoimmune disease that attacks the thyroid. So thanks a lot buddy. Yeah, well, and you go back to like we were talking about before, there were so many things that have to happen every single moment for a body to correctly. It's amazing most of us don't have so many more syndromes and. Conditions or Yeah. Yeah, right.
57:11 And actually, sometimes I wonder if the people who are air quotes normal are really the outliers. Yeah, maybe like, well, that was normal. Yeah. But really it wasn't, I don't know, back to what we were talking about. Most of these PPS affect the skeletal muscles, right? And again, the heart muscle for Anderson Twalley is the most problematic and the most life threatening and it Anderson Twalley syndrome is a group of diseases and they're inherited myopathies or myopathies. Different people pronounce it differently, but anyway, same word. And it causes problems with the tone and contraction of the skeletal muscles.
57:52 And that is obviously a problem. And the disease has onset in childhood or adolescence and starts before 18 is slowly progressive. This is The only exception to the progressive nature. The the Anderson trolley is progressive itself. That's why I kept making that differentiation before, because the other one is not the others are not. This one is with these physical manifestations is probably the easiest to diagnose too, because it's more obvious other people don't have physical manifestations and permanent weakness may occur with age. Men and women are affected equally. The attack lasts from 1 to 36 hours each time, right?
58:32 Yeah. So as a deeper dive, all of these forms of PPE have genetic problems. Each one has some genetic defect that causes this syndrome. If you will exclusively attacking the ion channel areas that control the mineral transfer in our muscle cells. Talk a little bit more about that. Each muscle cell has a covering or membrane that keeps the inside of the cell protected, right? Because it has to keep stuff in there. And when your nerves tell your muscles to contract, it sends a chemical signal to the gate. We've been talking about the ion gate that allows the sodium ions to flow into the cell, and the rush of charged ions change the electrical charge inside the cell. And I just have this little picture of this in my mind. Oh, everything's changing.
59:20 Yay. And it causes a wave of current that runs through the muscle fiber, right? So you can see that sodium goes in waves, come, gets the muscle fiber moving, and then calcium ions pour out of the sacs in the cells that make the muscle fibers contract. Again, that charge of those ions is huge because the correct charge has to be in the correct place at the correct time. And when these sodium, I mean, excuse me, when these ion gates are not working properly, even a little bit of problem can cause a real a real difficulty. So calcium ions have poured out of the sacs in the cells to make the fibers contract and then the potassium channels open sending potassium ions out of the cell and it causes it to contract. So you've got.
1:00:05 All of these things happening all of the time and we don't move muscles 1 cell at a time either. This is happening on probably the level of thousands at the same moment. I'm sitting here because I've got an Italian last name and when we do that we move all the time and thousands and thousands of contractions are happening. You think about sitting up when I'm using my core muscles, such as they are they all of these muscles are moving all the time. Any little teeny contraption, all of these activities, these are going to happen. So if you can see that NPP, the ion channels have flaws that disrupt the process, the cells then either don't contract or don't relax in the right way in response to the nurse signals. So the nurse signals could be correct, but the gates aren't. So the nurse saying, hey, hey, over here, let's do this. And the gates cannot or they cannot completely, which is why sometimes the action that occurs is just a weakness and other times it's a complete paralysis because the nerves are saying open the gates and the gates just don't do anything. That means that they're flaccid or unable to move. And that's where it can be such a problem. And then ultimately, because they do end this problem, this flaccidity ends.
1:01:20 That means the ion gates are opening, even just a little bit. So now, finally, things are worked. So many of these defects, like say, can cause the PP, but they always affect the sodium, calcium and potassium ion channels. That's just how it works. There are many unidentified defects in the PP chain. It shows that in most cases, PP is able to be contracted even if just one parent has the condition. And that is a lot because usually there has to be a higher concentration or both parents have to have it or it'll skip generations or something like that. But only one of the parents has to have it. And that unfortunately can be a big problem. Some of the symptoms we've talked about a little bit, but are attacks and muscle, excuse me, attacks of muscle weakness that last minutes today's and muscle pain and muscles after exercising again because those channels aren't working correctly. So you're going to have a build up probably of calcium that will cause the pain because it's usually calcium release and build up that causes muscle pain after a person's worked out and we all can get that.
1:02:26 However, ours will release as the gates open and things move in and out again. Muscle cramping and feeling tingles like tingly fingers or tingles in other parts of the body. The final symptom that was listed in this this general just overview is permanent weakness, but that's mostly later in life. It's differentiated by the exact gene that's affected. And the way that it's identified is blood test for potassium levels during an attack or even just after an attack because it'll still be either elevated or super low. Other blood tests to measure levels of other minerals and blood gases and genetic blood tests for known PP defects. And that would be if suddenly a person has a weakness or paralysis that shows up that someone else in the family has had.
1:03:15 So they'll do some genetic testing at that point nerve conduction studies and EMG's will do to be done to check activity, muscular activity in the in the areas. Also an ECG to check electrical activity of the heart, because obviously you can be paralyzed in your legs and you're like, well, that's a bummer. But if you're paralyzed in your heart, that's a big bummer. Yeah. And that has to be checked immediately. Yeah, of course. Things have to be reversed and assisted for that right away. Also, they'll check levels of thyroid hormones and most people if they go, if they start having some of these problems, if it's significant enough, they should probably take themselves to the ER by themselves.
1:03:56 I mean, not should as I'm recommending this. They, they mostly do go to the ER. Some people if they have the lower level, just the weakness will go to the primary care providers and then we'll probably moved be moved along to neurologist and then go down that pathway before things are identified because it can look like some other stuff. The treatment for these folks is to closely control potassium intake through diet and supplements. That's a really big a big part of it. Sometimes they get IV potassium treatments if this, the symptoms are severe from hypocalimate PP, low potassium control carbohydrates in the diet and what's interesting is carbohydrates come in so many forms. So that seems to me that would be quite a challenge, but vital for the folks that are affected by this. Honestly, probably vital for most of us, but we don't have that much feedback.
1:04:50 Usually. The biggest thing is controlling those carbohydrates also control thyroid function. If they have thyroid toxic PP, then they're going to be on thyroid assisting medications, whatever that looks like for them and their condition. A lot of people are recommended to get help from nutritionist to support those diet pieces because it's easy to be unclear about what is exactly a carbohydrate, how they show up in things where they can affect person and the other minerals. What does have potassium, calcium and sodium. Most people I think are familiar with sodium, but there's a lot of what people call hidden sodium, especially in the American diet and especially packaged things. I would expect that people that are what I say, considered fragile air quotes, that those are people that are not very easily controlled will have more little nuances to their treatment.
1:05:41 There are some medications that can help. Some of them are diuretics which cause increased urination because if they pull potassium out of the body, they'll use a diuretic to that out through the kidneys. That reminds me of nursing school and learning about potassium sparing diuretics and potassium leaking diuretics. Exactly which is how our body processes it back again to me being shocked at how people figure this out. I just don't even Yeah, true. How on earth and what's funny is it does just say talking about potassium spurring or diuretics and then certain types of anesthesia can be a problem because of possible complications.
1:06:23 Sometimes people with the Anderson quali have to use heart medicine or pacemaker and. I figured they probably would. I mean, you can't just have your heart stop, you know? They don't work. Out well contradicated to life it's true and once they know it they I'm sure have very close cardiac observation and treatments for that. Because like anything it's on a spectrum where it's sometimes really bad, sometimes not so bad. Some of the people with Anderson Tuoli don't have heart issues, but most of them do from what I read. Their advice to limit exposure to triggers, to keep exercise to a moderate level, not get super, don't run a triathlon.
1:07:06 You know what is it? Whatever is moderate for them. And that's probably something that takes some time to figure out exactly. There are some medications that affect the electrolyte balance we talked about using related to urine output with diuretic. Sometimes that helps and other times it doesn't. It just depends on the particular part of the disorder. Now, dear listeners, we've gone over our hour, which is what we usually record for, and we've got some case studies. So if you want to listen a little bit longer, listen to the case studies. Otherwise, we'll catch you on the next episode. Yay. Now for those who are still here, there's some police studies that I wanted to discuss. The first case study, and I'm going to read these because paraphrasing them will just be difficult. The first case study is a 36 year old male patient with no prior medical history presented to the emergency department with weaknesses, upper and lower limbs and have been ongoing for one day.
1:08:03 No complaints of double vision, no difficulty with swallowing or incontinence, and no history of trauma were reported. He had no significant family history. Upon physical examination, the patient was found to be alert and oriented with a power of two out of five in the lower limbs and three out of five in the upper limbs. They were slightly decreased reflexes in all limbs, but no significant findings otherwise. Initial lab tests showed an increase in white blood cells, a low potassium level of 2.5, a high thyroid stimulating hormone, THS TSH of 6.1 and a low a low and a couple other labs that don't appear to be related. That's my comment there.
1:08:46 Based on these findings, the patient was admitted for investigation and treatment of hypoclimic paralysis. During his admission, he was started on a potassium infusion of 40 millimoles over 4 hours and 50 milligrams of levothyroxine. That's a thyroid medication. Further investigations showed a high titer of anti thyroglobulin. Sorry about that pronunciation after two days. Two days after his admission, he was diagnosed with hypothyroidism and hypoclimic paralysis, and he was discharged on levothyroxine and magnesium hydroxide and potassium chloride, all of that to maintain his levels. And of course, he was referred for follow up in the clinic and genetic testing.
1:09:35 But it turns out for this case, they noted genetic testing was not done to due to the patient's financial issues. Yeah, no kidding. So the thing that for this case, what was amazing to me and, and I guess emergency rooms are more familiar with this than I am, but they thought of the fact that it was a hypoclimic paralysis almost from the get go it seemed like. And that low potassium of 2.5. Remember normal is 3.6, I think it was 5.6. But I think this is five. I think anything over 5 is bad. Is it OK? But 2.5 is quite low and like we talked about before, when the range is so short, any change is significant. And this was a big change.
1:10:18 So they noticed right away. But he had had no complaints, no prior prior problems and he was 36 when this happened. Wow, and that would be wild. Yeah, it would. Now the second case, this guy was a 27 year old man with no prior medical history presented to the emergency department complaining of muscle weakness which had lasted for one day. He did not exhibit any other symptoms such as slurred speech, double vision, headache, trauma, palpitations, or excessive sweating. He had none of those. This was his first episode and there was no significant family history. Physical exam reviewed GOSH words. Physical examination revealed a power of two out of five in his upper limbs and three out of five in his lower limbs, according to testing, and then accompanied by decreased reflexes throughout his body.
1:11:09 No significant abnormalities were discovered in other systems. Initial lab test results showed an elevated white blood cell count, low potassium 2.1. Wow. And. White blood cell count, huh? Right. And that's been consistent for last two, right? Yeah. And they don't address that. They didn't address that at all in any of the things I was reading. Although if you think about Graves' disease, that's an autoimmune disease that attacks the thyroid. So I wonder if part of this may be autoimmune. I would that white blood cell increase. No one's ever said that. That's another theory by Andrea. He also had a TSH of 0.2. He was admitted for further investigation and management of periodic paralysis highs caused by hypokalemia with hyperthyroidism, so he had low potassium and high thyroid activity. Yeah, a TSH of .2 is low.
1:12:05 Yeah. He was given potassium replacement therapy and during his stay has revealed a high titer of anti thyroid, thyroid globulin antibodies. Thank you. And he was started on carbamazole twice a day and I looked that one up. It's used to treat overactive thyroid, the carbon muscle. After a couple of days of from his admission, he was discharged and referred to outpatient department for follow up and genetic testing. Again, not done due to financial issues. I thought that was interesting that both of them mentioned that. Yeah, I agree. But genetic testing is expensive. But it seems to me, well, the only time at that point that it would matter is for having children, right? And this kid was 26.
1:12:52 The other one was 36 or no, excuse me, he was 27. All right, third case, a 37 year old male patient with a known history of grave disease and positive TRAB thyropropen receptor antibodies had not taken his carbamazole, which is the overactive thyroid stuff. So Graves' disease, he's supposed to be taking carbamazole for his overactive thyroid, but he hadn't been taking the medication for more than a month. Again, a lot of people, it says something about this later, but a lot of people change their medications because they can't afford them. Yeah. Yeah. At any rate, we won't judge that now, but we will later be presented with lower limb weakness that prevented him from walking or standing. He had no history of headache, fever, decrease or loss of consciousness.
1:13:40 He had no bowel or bladder incontinence or recent infections. He had no significant family history. And if you remember people who have this have had some of them have had family history of this disorder, but this guy hadn't either. His initial vital showed a pulse of 108 8, which is elevated of course. Blood pressure of 127 / 68, not bad. Initial examination showed a power of three out of five in the lower limbs and normal power in the upper limbs. No other significant findings were found. Initial labs showed a potassium of two and he had TSH was severely decreased as well.
1:14:21 He had a couple other things that were not totally related, but he had no other findings that were related in the in the blood draws. As a result, he was admitted for management investigation as the case of periodic paralysis with thyroid toxicosis. He was starting on IV of potassium replacement and he start he was started on Propralonol which is a beta blocker to slow his heart rate and it also works to decrease blood pressure, which in his case he didn't need blood pressure but needing his heart rate slowed because if you remember it was 108 and then the carbamazole then his testing later the calcium was our potassium was corrected to 4.4. So after he was given these things, his potassium went up to a normal range and his weakness improved. 3 days after admission, he was discharged on propolinol and carbamazole and referred to the outpatient department for follow up. Genetic testing was not done due to the patient's financial issues.
1:15:19 I hate it. The fact that they mentioned that every single time, it means a lot to me. And I believe that part had come from the Mayo Clinic #4 is a report of a licorice consumption case. I know I wanted to cover this. So this is a 36 year old previously healthy Palestinian man from Eastern Jerusalem presented to the emergency department with a four day history of rapidly progressive limb weakness, bilateral parasis, which is partial paralysis or muscle weakness. So he had either one or both of those, first developed his legs and hands, progressed to his arms, thighs and torso and caused extreme difficulty in walking.
1:16:00 On the day of admission he had difficulty sitting up or raising his hands from the bed. Now this is Andrea talking. At first blush I would start thinking Gan Beret because of the of the progression. However, I'm incorrect on examination the laying down patient because he could not sit up. He was unable to move was but was fully alert, cognizant and a febrile so we had no temperature. He had mild hypertension or blood pressure which was 150 / 100 which sounds a little more than mild to me. Yeah, especially that, that diastolic or low, low bottom number, right, That's a high number for that. Yes.
1:16:41 And for note to listeners who are unaware of this, the closer the number, so like 150 to 100, the closer those numbers are to each other, the more dangerous it is, right? Because that means that the, well, we'll go into that another time. But anyway, it's more dangerous. And his neurological examination revealed several bilateral, excuse me, severe bilateral weakness to the proximal and distal muscles. Now, if you remember, it often is only the proximal but not the distal muscles. So the ones closest to the body, but not the far ones away. One of the things I think that may have made this different for him is that it had been a few days. So he's progressed pretty far with this problem. All four limbs were affected and it was by bilateral so both sides. He also had absent patellar which is knee, ankle and triceps reflexes.
1:17:30 They were absent. Wow. And his plantar. The foot reflexes were incorrect. It was flexor instead of extensor. I know he's getting bad. The patient had no evidence of meningeal cranial nerve or central nervous system involvement, which to translate is basically like he didn't have meningitis or or brain injury at this point. He had no sphincter disturbance, so he had not been caught in a bowel, hadn't had muscle wasting or muscle tenderness. The laboratory studies were done and he had a lumbar puncture, chest imaging, CT and all of that showed no abnormalities. However, he had in his ECG had mildly elongated QT interval, which is an abnormal pattern which can be deadly. And he had severe hypokalemia.
1:18:21 Potassium, as we said, is 1.7. So he's getting really, really low. He also had elevated creatinine level of 800. And creatinine can indicate muscle wasting and damage, among other things, but that's one of the things. Now his weight was stable, he had consumed A balanced diet, and he didn't have diarrhea, excessive sweating or symptoms of hyperthyroidism, Cushing's syndrome or things like that. And he had no previous episodes of muscular weakness or personal or family history of any of these things. He denied the use of licorice chewing tobacco, which can have some licorice properties in it and prescribed over the counter natural herbal medications or abusive drugs, alcohol or laxatives and had no risk for human immunity, HIV. And they ask these things and these things can be related because of some of the side effects of some of these these herbal things over the counter stuff. People think that things like laxatives can be harmless, but they cannot because I mean, they can be very damaging because they can cause a dumping, as it were of enzymes and potassium, sodium and calcium are some of the things that they dumped the most because our body doesn't have time to absorb them. And then he had a 24 hour urine study which revealed high urinary excretion, which can indicate some kidney issues. And he just all of this was going on. They just weren't sure what was happening. But he was given a large dose of
1:19:52 intravenous and oral potassium, which resulted in the normalization of the serum potassium and creatinine levels after three days. It took three days to get normal and then he during that three day time frame had complete restoration of muscle strength and motor reflexes. He was gradually weaned from all the potassium supplements and his electrolyte levels remain stable and normal throughout the rest of his hospitalization. So he isn't presenting as some of these other folks have. A little bit, but not completely. Well, it turns out that upon further questioning, he admitted that during the previous year, he and his three sons had consumed half a bag of licorice candy daily. Oh my gosh. Yeah, so half a half a bag each one of them had.
1:20:33 I mean, they'd all consume that daily. So that's a lot. He also noticed that during the two weeks before his admission, he drank large amounts of tea prepared from an extract purchased at a E Jerusalem popular. It's called the SUS House SUS. And then house is specialized in the production of licorice, sweetened tea, and SUS is Arab slang for licorice. Now, one of the things that the investigating people noted is that because they didn't know what the slang was, they didn't understand what he had had, which may have resulted in delayed diagnosis because he had said that he had gone to the suss house, but they didn't understand what that meant. And I don't know if these people had been American doctors treating them in Jerusalem or or what, but that slang was a problem. They estimated that the patient consumed an additional 100 milligrams of this glyceraic acid with the T, which aggravated as hypoclenia and resulted in the development of the progressive life threatening paralysis. And if you remember all of his heart numbers or is his ECG and stuff was all a mess, too.
1:21:42 Yeah, yeah. Now, his children who did not drink the tea remained asymptomatic. And they had been helping meet the licorice, but they didn't have the tea. So the tea must have pushed him over the top. And it's probably good because these kids are are obviously younger and potentially quite young. I don't know for sure, but that could have been huge for them, probably deadly if they had more of that going on. He was discharged after 10 days of hospitalization with strict rules to avoid licorice and licorice flavored products. At an 18 month follow up, he was all normal, everything's fine. He was free of residual weakness. But Can you imagine death by licorice? No, that is, that's wild.
1:22:23 Wild all the time. But this is wild world. It is a wild world. I have two more quick cases. This next one is a 46 year old man who has a history of autoimmune hypothyroidism and currently it was on levothyroxine and presented with sudden symmetrical weakness in both his legs. After working in hot weather, he was unable to stand or walk. He had experienced similar symptoms twice before. He had no fever, trauma, back pain or autonomic involvement. His initial vital signs were normal and his examination revealed muscle power, three out of five and all limbs with hypotonia. Hyporeflexia which means the tone was low and the reflexes were low. There were no significant findings in the other systems. His initial investigation showed elevated creatinine and a low potassium of 2.1.
1:23:09 His TSH was elevated. The patient was admitted as a case of hypoclimic pre erotic paralysis due to type 1 RTA and I did not look that up. I'm not sure what that means, I'm sorry. And acute kidney injury from heat exhaustion and was started on levothyroxine and IV potassium replacement IV fluids for management. After two days of treatment, the potassium was corrected and the weakness improved significantly along with the creatinine level. Due to these improvements, the patient was discharged on the third day and referred to the outpatient department for follow up and genetic testing was not done due to the patient's financial issues. So this was a little bit different because he was the one out in the heat doing all of this stuff. Again, he was 46, so he was older, but he also had known hypothyroidism, right?
1:23:59 So he was at risk already. The power, muscle power that they're talking about is fairly significant. Three out of five, four out of five, two out of five. This is pretty, pretty strong weakness. The final case I'm going to bring up, although there are a bunch more, I tried to limit it. A 27 year old male patient with no prior medical history presented with progressive weakness in his upper and lower limbs that persisted for two days. He didn't have a fever, headache, trauma, recent infection, or incontinence. There was no significant family history. His vitals were normal and he was alert and oriented. However, he had a power of three out of five in both upper and lower limbs and his cranial nerves were normal, while other systems showed no abnormalities. So there was just nothing coming up. They did lab investigations and it reviewed potassium of 2.5, but there were no other significant findings. The patient was admitted to ICU for monitoring, management and further investigation as a case of hypokalemic paralysis. He received 20 milligrams of IV potassium and magnesium sulfate, and the next day his potassium levels had improved to 5.2 and his weakness has decided.
1:25:05 Consequently, he was transported to the medical ward for the lab test showed an aldosterone level. Do you remember that from before it was 119? I don't have the averages on that but they did measure it and TSH of 3.38. After two days the patient was discharged, referred to the outpatient department for follow up and genetic testing was not done due to patient financial issues. I never did see one that said it was done. Nope. But the fact that they bring it up every time means to me that it was significant to them and to me, but it was. It was really interesting. Those are the cases. There were a lot more, but those are the ones I grabbed because they showed some very variation in the process.
1:25:48 And again, this is a fairly rare condition. I'm very impressed that these providers discovered it apparently quite quickly. I don't know how many times a hospital or ER will come up with this, but I think it is fairly common to check potassium levels and other electrolytes, calcium and all of that, just to see where people are, blood sugars and all that. I'm pretty sure all of that is quite common. This is just such an interesting condition and scary. So scary. Very scary. To be weak, I can understand having weakness for a couple of days and not really go rushing out, but I certainly cannot understand having a paralysis and waiting four days for it to progress.
1:26:31 Like licorice toxicity. I'm like, Oh my gosh, I would be rushing right in. And like I said, this is one of those things that people I think do tend to get treatment faster then they might with other types of symptoms. So I'm glad about that. It's this condition I've never heard of before and it was really fun to research. That's awesome. Thank you. You're welcome. All right, If you've made it this far, clearly you like a little mayhem with your medicine. Or you just enjoy hearing us ramble about weird medical mysteries. Either way, we'd love you for it. So hit that follow or subscribe button wherever you're listening. And toss us a rating or review while you're at it. Helps other curious weirdos find the show. And we know there are a lot of you out there. Oh so many.
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