Pediatric neurologist Jenn Tu, MD, PhD, breaks down movement types in this guide to determining whether a patient needs referral and how to sum up observations in meaningful ways. With video examples to illustrate, she clarifies tics, tremors and other commonly seen motions; provides a straightforward vocabulary; and clarifies when something that looks alarming is actually cause for concern.
Hello everyone and thank you for joining us today. Um My talk today hopefully will be interesting for most of you. Um or at least you'll learn something as far as who needs to come to neurology and who doesn't. Um But as always we're happy to see anyone. So if you have questions you're unsure of something please send them along. So without further ado we're going to talk about admiral movements today and um this picture on the side it just sort of I think encapsulates my my idea of movement and patients that are affected by abnormal movements. It's by an artist called named Leonid Avramov. It's called a whirlwind of feelings. And that's really true for a lot of patients that have abnormal movements because they sort of can be quite buy it and you know, providing the right diagnosis can be really liberating. So I like this uh picture sort of just to introduce the topic of movements. So we're going to do three key things today. We're gonna talk about movements in main categories of volitional movements or intentional movements. The second category is going to be functional abnormal movements and these are ones that um they sometimes get mistaken as volitional or they're doing it on purpose, but really they should be thought of more as non volitional movements, but they fall into the functional category. And then lastly, we'll touch on a couple conditions with non volitional movements. Alright, when I am teaching the medical exam to residents and fellows, I usually highlight the fact that vocabulary is not so important. If you can convey yourself by describing what you see, then that usually is more important and more valuable than using the right terminology. Um I will caveat that for movements that sometimes it's really helpful to know the distinction and use the right terms because it really helps the neurologist who you're referring to to really understand what you're seeing. And so there's really only four terms that I'm going to bring up today. Very straightforward but very important. So amplitude is really, you can think about the size of the movement that they're doing. Um I'm sure you guys remember physics from forever ago, but it's just like our waves, right? So they have an amplitude and they have a frequency amplitude can be um it can be large, it can be small. Sometimes people call this coarse or fine, um they're big or small basically and then frequency is going to be how fast it happens. The frequency of movement really helps us distinguish several types, especially with tremors. Um We can really try and parse out what type of tremor it is based on how fast it is And then the last two I think um I don't really expect you to know the difference or as the neuro anatomy, why they're different. But it does help us differentiate the types of movements if you can distinguish what you see with a taxi and Demetria. So ataxia I like to say um if you think of a taxi cab driver uh sort of a memory trick here, they can get you to the same destination going hundreds of different routes. Like they learned the whole city. They have the map in their head and they drive all around but they always end up at the right spot. That's how you can think of a taxi to. And then for dismay tria. It really just comes from the greek word for a wrong measurement. So they try to get to the end point but they miss it a little bit right. So they're they're straight and direct in their course. But they just don't end up at the right spot. And I think those four terms when you're trying to describe some of the movements that you see in your clinic can really help differentiate. Does this patient need to go to a general neurologist or does this patient need to go or specific movement disorder or a neuro muscular doctor? And that's not for you guys to decide. But when the referral comes in, we do have an internal process to help us make sure the patient gets to the right place. All right. This is a daunting graph for a lot of people, but it's really just to help um set the stage for what's going on. Um This chart is really only pertinent to volitional movements. That first part of the chat for today is going to be about volitional movements and this is really your dopamine pathways in the brain. You can think about it in a very simple context, increased dopamine, usually increased movements. And that that's because the direct pathway is activated by dopamine. And the direct pathway is what turns on the thalamus to turn on the motor cortex. If you don't have dopamine than the indirect pathway is active, it's on and it's inhibiting the thalamus, you don't have to remember any of this except when we're talking about some of the volitional movement, it helps understand why certain medications are helpful and why others are not. Okay, so I won't spend too much time in it. I just if we're talking about movements, it would be silly of me not to bring this up. All right, so we're gonna go through about six cases today and I've got videos to help demonstrate um what this looks like. Um I'm gonna have to change my screen a little bit so I can see everything. Here we go. All right. So first case it's a 12 year old girl with difficulty focusing during school. Her parents note that the movements get worse when she's watching tv or when she gets excited. Thanks. All right, let's head to our videos. So here is case number one and I'm just going to play the video for you. Well, just watch a few seconds. And what you'll notice is that the movements are rapid. They're pretty course they're generally stereotyped. They kind of look the same each time. But there's there's many different types, right? Some of them are simple. Some of them are kind of complex movements. Joint together. And this is probably something you guys have all seen in your clinic before because it's very common. All right. So volitional movements. The first thing we're going to talk about is ticks. This is because it is so common. Like I said, these are sudden, their brief, they're repetitive and they're stereotyped. So it looks the same pretty much each time it happens over and over again and they're very quick. This can either be movement or it can be vocalizations. Most of the movements we saw in the video were very simple. There was an eye blink or a facial grimace, but she did also have some complex ones where her arm would come up or should have a whole side of her body would sort of spasm. Um A unique thing about ticks, sort of similar to conditions like obsessive compulsive disorder. There is a ritualistic component to this. Um So you might see it happen more often during their routines throughout the day, like to brush their teeth when they're getting ready to do that, they sort of have the same um explosion of these ticks that happen and and its predictive of when it's gonna happen throughout the day. These are voluntary meaning the patient can suppress them, but it's important to remember that this is aged dependent. Um It's rare to see a patient before the age of around seven or eight that can actually describe the compulsion or the need, the urge to do the tick. Um But that that degree of insight, It's actually really helpful for treatment as well, but it doesn't typically happen until like I said around age eight. Um An important thing to remember with families when you're counseling parents is that it often increases with anxiety and stimulation. But an important thing to remember is that stimulation and um stress can be positive or negative. So somebody can get excited about something positive. They're you know, they've got a party coming up this weekend that increases their sympathetic drive. They're they're more excited or they've got a big test coming up for. They're worried about it's the same underlying physiologic processes these that are happening and they both lead to an increase in the ticks. Common examples just like the ones we saw in the video or an eye blink uh facial grimace and gaze deviation. This one I see often referred because it looks funny and people have a hard time telling if it's tick if it's volitional or if it's a seizure of some type. But typically patients will divert their gaze usually to the side sometimes up. Um but it's very fast. Usually not persistent but it happens over and over again. Um And then the vocal ones we often see our throat clearing. Um These often start after somebody has a cold sort of a, it's reinforced like at one point they had a cold, there was something in their throat, they clear their throat and it felt good. And so that reinforces the behavior and then it sort of becomes a habit that's how most ticks start at some point Ticks happen. And about 1 350 Children. So very common. You're going to see this in your clinic and um we were happy to see these in our clinic as well, But they're very common and often benign. They usually start around age four and usually decrease after puberty. There's a simple rule of thirds that's easy to follow here. A third of patients are gonna be transient, meaning it's there for a month or so, a third or chronic, meaning it's more than six months and a third of persistent through puberty. And it's really that last third of patients that were more concerned about because they are more likely to go on and developed Tourette's syndrome. So Tourette's syndrome um Less common but still common enough that we've got a lot of aggregate data um to really understand this condition. It has a lot of stigma surrounding it which is probably the hardest part about treatment. Um But it really is very common and it is treatable. This is basically somebody who has a motorin the year and there's no more than four tick three months. So meeting criteria for Tourette's syndrome is actually a lot simpler um than I think a lot of people recognize. Um So a lot of patients that have ticks that I see are actually more likely to have Tourette's syndrome and they're just underdiagnosed. Um Not necessarily that it means it changes their management, but it often makes it easier to get supports in school because uh Tourette's syndrome can be quite disruptive to the education process and getting an I. P. Or a 504 is often easier for Tourette's syndrome than for ticks alone. Just like with ticks, we know that with Tourette's syndrome um you're going to have these remit and riker episodes over weeks to months and there's also a rule of thirds with Tourette's syndrome. So a third of patients will have no ticks in adulthood, A third will have some ticks. And the third of patients um have really significant ticks that still bother them. Um And it's those patients that we have to target um With usually with pharmacology so that they can continue with their meaningful life going to work and things like that without having that social stigma. I do want to point out that corporal alia. This is probably part of why the stigma is so significant for Tourette's syndrome. This is where people are. You know when when you see kids saying cuss words and and just being really um Of course and out of context and inappropriate in social situations. It really only happens in about 8% of patients. Um and it's it's another thing that for the patient themselves they don't want to do this and it's quite distressing for them as well. Um And so it's not common but it's a it's a good reason to treat Tourette's syndrome because of that social stigma. All right. Um Ticks in general there's a lot of comorbidities. The reasons it's important is not because of the movement itself but because of the things associated with it. And this is similar with Tourette's syndrome. So there's a higher prevalence of A. D. H. D. Obsessive compulsive disorder and learning disorders in these patients. And so most patients with ticks should have an evaluation through the school system. If they're showing any signs of struggling academically, we wanna make sure we're not missing any reading disorders, dyslexia, specific difficulty with math. They're all more common and they should certainly be evaluated for. And like I said with Tourette's syndrome and takes at the beginning it's not the movement so much that needs treatment. Honestly, I have a very clear recommendations for families that the only reason we recommend intervention as if the tick is so severe that it's causing injury if it's so disruptive that they can't stay in the mainstream classroom Or if they're overly distressing for the child. So outside of those three categories we rarely treat the movement itself and we focus more on the associated conditions, the anxiety and the 80. HD. The one thing about ticks that can make it challenging, I think for a lot of pediatricians is that because they often look irregular um it's hard to say that that's not some other movement disorder. And so Korea is one that comes up quite frequently when people have complex tics because they because they just look kind of funny. And so that's a nice thing to remember about Korea is that they're not really repetitive, they're not quite stereotype, They are pretty irregular. And the thing about ticks is that they're often um I'm sorry, they're not often incorporated. So Korea people recognize that they're doing it and they try to hide it so they incorporate it into their other movements. And the classic example is somebody who has an arm jerk with their Korea and they learn to turn it into a scratch of the head or something simple like that. Um to incorporate it into sort of hide or mask the movement and that doesn't happen with ticks. Like we saw in the first video, the arm jerk comes up and she doesn't do anything to hide the movement. Alright. The last piece with volitional movements before we move on to the next category is steri oddity and I think this is one that might be helpful to review for for for the pediatricians because this is one that um it often it really scares parents to see a lot of these behaviors because they're they have um a negative connotation to them most often. So these are repetitive, their stereotypes just like the name suggests, but these are slower movements and typically purposeful in their design. The classic example. Um we see is hand waving and hand flapping. So a lot of patients with autism when they get over stimulated their hands wave in front of them like this. That's a classic law on, we also see with Rhett syndrome, um, is a finger ringing, so doing movements like this with their hands, but the ones that I see more often as a referral for concern, our head banging, it's probably the one that I see the most where there's concern and this could be a, you know, a typically developing child, but when they're trying to go to sleep, they bang their head and it really worries their parents. You can think about most of these behaviors as a form of self soothing. And if you remind parents that the most common one is thumb sucking, then it becomes a bit more obvious that these are not dangerous. There's nothing wrong with a child. They've just learned how to do something to sort of self regulate and help calm themselves. Um We do see a higher prevalence with these with autism and self violation disorders, like a PhD, but it is very common to see these and typically developing Children. So stereo Tapie's remember are slower, purposeful and they're a form of self soothing. All right, moving on to the next case. So we have a 17 year old girl who has unusual hand and arm tremor. It started suddenly and it has persisted for several weeks. So I apologize. The video that we're going to see is not a 17 year old, but it's the best example I could find to demonstrate this. So I'm going to fast forward it to about 30 seconds and then I'm pretty so you can see what we're looking at. So her hands are going pronation, super nation sort of side to side. It's not really in her fingers, it's more in her wrist in her hand. Okay then when she puts her hand flat where she's resting it and now changes and becomes an up and down sort of flapping movement again. Not really in her fingers, more so in her wrist. And then when we move on, let me fast forward to a part where we're actually doing the exam right here. So the tremors in her right hand and the examiner has asked her to do large movements with her other hand and you can see that the tremor in her right hand changes and it pauses and it goes between the flapping and the pronation. Super Nation. Okay so we'll stop that one and go back to our lecture. So this is an example of functional movement of functional tremor. So this is the second category of our abnormal movements here. It's really important and I don't think can stress this enough. These are not volitional. Okay, you can think of this like software malfunction. So some abnormal movie like in Parkinson's disease comes because there's a hardware malfunction, right? The substantia nigra, you're you're missing your dopamine. There's an actual pathology to the brain tissue itself. This is software. We don't have the functional imaging the functional MRI studies to exactly train where this pathway problem exists. But best guess is that it's from the thalamus and it's related to the limbic connections there, but this is not under patient control. Um and and really applying the correct diagnosis can be quite liberating for patients with functional urologic disorders. So for functional tremor, as you saw in the video, the amplitude of the free. It's very okay. It was first it was the culmination pronation. And it was when she was holding a posture and then when she brings her hand down to rest on her leg, it changes. And now it's a resting tremor that's sort of a flapping quality. The other thing that we saw was distraction or entrainment is the term that we use. So when she was doing the big movements with her other hand, it's really hard to to create an abnormal movement unless you're, you know, uh pianist and you've got great control independently with both hands. It's really hard to continue a functional tremor with the same amplitude and frequency when you're doing purposeful movements that are repetitive. On the other hand, okay. Um I mentioned that it changed between rest and posture. That's also another feature. We see a lot with functional tremors and the onset is also very classic. Here it happens abruptly. We don't always are aren't always able to identify a stressful situation or something that happened in their life that precipitated it, but we think that it's you know, it's related to anxiety, it's related to stress, could there have been a life event that led to this? Often the answer is yes, but we don't always identify it, but they happen abruptly and then they stay the same. They aren't typically progressive, they don't get worse, they're just explosive at onset and then it's there. Um Usually the disability is out of proportion with what we're seeing. So um the patient that we saw in the video, it was really just her hand and she could still use her right hand well and it really shouldn't impair her. Um But other parts of the video patient specifically, it was starting to affect the way she was walking in the social stigma that she felt like she she stopped going outside. Um Tremor and that's classic to see with functional neurologic disorders is that the disability is just out of proportion. They usually happen anywhere in the body. But classically again the fingers are not involved like they are in other tremors. So we saw in her in this patient it was really the risk that was doing the movement and then another form of functional movements that we see quite frequently as a Stasia Basia. Um Oh goodness! I don't know if I have that video. So West asia Basia really is just inconsistent. Uncoordinated walking. Um I think the the way I like to describe this is that it's harder not to fall doing the movements at the page and just doing then normally and patients with West Asia and AsIA rarely fall. It's very inconsistent. Sometimes the legs give out like they sort of have this noodle look to them. Um And then they have usually have an up and down body movement that comes with it, it looks really irregular and it looks like they should fall if they really didn't have control of their body, but they usually don't um for both of these for functional movements. The treatment is physical therapy, There are a lot of tricks that can be learned. Um One of the classic ones they use for East Asia of Asia is to help the patient um get a rhythm in their brain. So if they snap their fingers or tap their hand on their leg as they're walking, really paying attention to that rhythm tends to help them. Another trick that's often tried is having them watch other people while they're walking in. This one sounds a little creepy, but really if they watch other people's feet and focus on, then they sort of are not really paying attention to what their own body is doing and it tends to lead to a more normal gate. Um, The same happens with functional tremor is having a good physical therapist or occupational therapist work with them on tricks and ways to help their brain not think so much about what they're doing often really helps them to gain more control and really move beyond beyond what's happening. Okay. All right, so our next case, This is a 13 year old boy with anxiety and he presents with twitching, not much more description than that, but the parents say that it is really stressing him out. He can't think about anything else. He's so worried that there's something wrong with him because his body keeps twitching. I'll pull up the video here and I'm sure most people in the audience at one point in their life have had an episode where just they feel these muscle twitches and when they look they're like, oh, that's funny. There's just little parts that are flicking there. Um This one's a little harder to see, but it happens in the feet as well in this patient right down here. Yeah. All right, so this move is on to non volitional movement. There's no control over this. It just happens to the person. This is part of what makes it so distressing, especially if you have a patient with underlying anxiety because they have no control over it. They don't know why it's happening. The classic one we see it's gesticulations, that's what the video was. These are spontaneous contractions, but it's not the whole muscle, it's not like a muscle cramp, it's just individual muscle fibers. And so you see this flicker of movement. Sometimes it's described as a bag of worms, sort of a rippling effect. And it can happen anywhere in the body. For some people, it always happens in the same place that's focal. For other people, it could be anywhere in their body. It happens in about 70% of people very well described. Very common. The thing that makes people worry and if they've done any reading about it, especially when they're older is that the stipulations are a presenting feature for L. S. Or Lou Gehrig's disease. This happens because the muscle gets renovated and so it fires on its own without input from the nerve to control it. All right. Um I will just go back and say for for for stipulations. If you have a patient with the stipulations, one of the easiest tricks and sort of helps the patient recognize that it's benign is if they're having for stipulations, if they simply change position, move the arm or the leg readjust themselves, then the physical ations will typically stop. And that's one easy way to sort of counsel the patient that you know, we can look for some basic labs. We can do electrolytes studies just to make sure that you're not getting dehydrated because that can be a trigger. Um But really if you can move your body and it stops then that's very reassuring. Moving on to case # four I hope I'm not going too fast. But I've got two more cases to get through and I want to make sure we have time for questions. So case # four. This is a 15 year old boy with progressive arm movements over the last it's asymmetric and it's more in his right hand. Unfortunately he happens to be right handed. So he's noticed that the handwriting has significantly changed. All right, So we'll go, oh goodness, I'm gonna have to just this so I can actually get to the right piece. So give me a second guys and then we'll bring it back big so everyone can see it. All right. So for this patient I'm going to fast forward to when they actually turn off his device so you can see what it looks like. Hey, no, if you look at his hands, you'll notice when he holds them up. They start to shake first it was in the left hand. Now it's more noticeable in the right hand. And when he tries to do an intentional task, like pretending he's drinking a glass of water, the sheikh becomes worse. The closer he gets to his target here he is trying to write. And the more he tries to write in a coordinated fashion, it becomes more difficult. And you see sort of a scalloped pattern to the shell he's trying to draw, he can't really do the concentric circles. Okay, I know you guys have seen this before. Um This is sort of a classic description but non volitional movement is the most common thing we see is Tremor and tremor can be broken down into a couple broad categories. Um And then within those categories we can get even more specific. But for you guys, I think it's moved to know um just the broader categories. So a tremor is basically an involuntary rhythmic osceola. Tory movement. So there's a fulcrum point and the movements go around that fulcrum. Okay, it's rhythmic typically can be described in the frequency or hurts of that rhythm and it is truly involuntary. It's not something that the patient has any control over. We generally think about tremors as either arrest tremor or an action tremor and then the action tremors get broken down more specifically. Um I will only point out isometric because this is one that I think I've seen a couple referrals for this. Typically in a T. Change patient who's worried about the fact that when you know they're doing something specific, they noticed a tremor. So an isometric tremor, the best example is um you're sitting in a chair and you've got your toes on the ground and you lift your heel. And let's say you just ran or exercise, you might notice that when you try and hold that position and you put tension on your quadriceps muscles you start to shape in your leg. That's an isometric tremor. Where you're putting trends are just like isometric exercises where you're holding tension in the muscle. Um That is a typically benign tremor but we see it quite frequently because people were like I exercise I'm healthy and then I start shaking. Very common one to see. All right. So the patient that we saw in that video has an essential tremor um His was so severe that he was actually implanted with a deep brain stimulator to control his control his tremor. The two here on this slide, the physiologic tremor and essential tremor are very similar. They looked very similar and can often get mistaken. The physiologic tremor is one that um everyone has. You just can't always see it and it's like that. You know you want your surgeon to have a steady hand. You don't want them to have a noticeable physiologic tremor really doesn't matter. It's kind of a funny adage. But you know, a steady hand doesn't matter with physiologic tremor because it doesn't get worse with intention whereas the essential tremor does. So a physiologic tremor is low amplitude so it's very small very fine high frequency. It's very fast. Usually you don't see it but with increased sympathetic tone when you're scared or excited or you've had too much coffee. Then you're going to see a very fine tremor just in the fingertips. Um It's also unmasked by some medications and one that we see very commonly especially growing in our patients as the antidepressants very common to see with the S. S. R. I. S. Um And unmasking of a physiologic tremor for essential tremor. It's slightly different. Um A little bit slower than a physiologic tremor a little bit larger amplitude but still small. Um The patient in the video you noticed it wasn't big movements but a little bit a little bit bigger than a than a physiologic tremor in a little bit slower. It's most often in the hands and arms, but we can see it in the head. Um And sometimes we hear it in a voice sort of a a wavering voice. This is a common complaint for people that have anxiety when they get up to talk in front of people. Their voice quivers. Um That's sort of um sort of physiologic sort of essential, more commonly an essential tremor that's being unmasked though by by sympathetic tone. Um as I mentioned intention with essential tremor. So at the end of a goal directed movement it gets worse. So the patient with an essential tremor might be able to hold a glass of water and have a little shake. But the closer they get to, the bigger it gets and then they end up spilling. That's one of the main reasons why patients get implanted with DBS or deep brain stimulator because they can't functionally use their arm. They always have to use a straw and it's really debilitating. This happens with pretty significant family history and a lot of patients. So there's some thought that there's an autism, a dominant gene. We just don't have it really identified it yet. Um And there's just reduced penetrates. So not every family member has it, but we know it does run in families. And classically the description is that it's improved with alcohol. That's not always true. And it's certainly not something that we're going to recommend. But sometimes during the history taking, especially if it's an adult, they might share that piece of information. Alright. Other action tremors that we sometimes see as a primary handwriting tremor. Um This can often be seen in combination with dystonia. So this one um Usually the patient describes that their hand gets tight and they have this super nation pronation quality to the tremor and it just makes it hard to the right, usually similar to essential tremor. This improves with propranolol so thankfully it's relatively easy to treat the other one that we don't see very often, but has happened a couple times in in pediatrics but more often than adults is the Ortho static tremor. This is what a lot of people have speculated we're seeing with Angela Merkel um and videos over the last couple of years when she stands up, her legs shake. It's a very broad oscillation. Um low frequency, but it's a big movement. Um And then sometimes um the way it starts is that only the patient can feel it right? It can be so small that nobody sees it but the patient. And then with time as it becomes worse, you see that the amplitude gets bigger. All right, so more um non volitional movement with tremor and those were action ones. We just discussed resting tremors. Um, I don't want to spend too much time on because really in our pediatric population we don't see them. This is more like Parkinson's disease. Um, her isolated rest tremor When we don't see any brain changes, these tend to be slower frequency, um, and generally not seen in the pediatric population, but I did want to show you a few videos because if you see something like this, this is definitely a patient that we want to see in neurology. So let me pull up the first one that we're going to do. This is called a ruble tremor or a wing beating trump. It's broad, irregular and asymmetric. So he can reset himself by putting his hands up on his head. But when he tries to maintain the posture, his hands start going and then he'll reset by doing a movement of purpose and then we see it again while he's holding a posture. So a ruble tremor um is typically because there's been um injury to your your ruble system, which is part of the cerebellum. It's a descending pathway that helps to coordinate movement based on where your head position is. So it suggests that there's an injury either from a toxic toxin or a stroke or something or an infection to that degree. And then I don't think we need to do the Parkinson's tremor, but I will show you a dis tonic tremor. So this patient has focal cervical dystonia. You can see her shoulders are asymmetric and the head is caught to the side and while she's just at rest during the dystonia, you see this sort of erratic jerky quality movement in her head. This is related to um the competing actions of the two muscles that are fighting against each other. So she has um If you if you think about it this way, like your your biceps bends the arm, your triceps extends the arm and when those two muscles are fighting against each other and both contracting at the same time when one of them wins over the other, you sort of have a drop And movements or a sway one way or the other. And that's basically what happens with a dis tonic tremor. Alright, five. This is one that I think all pediatricians should see, so you can learn to recognize it um in any of your patients. So this is an otherwise healthy eight year old boy who over the last few weeks has had progressive movements. They started off pretty small. Um And he noticed them before he started before his parents did, he just didn't say anything because he was eight, didn't really know what they were. You can see it in his body and as well as his face. Okay, so this video starts off um he's resting on a bed and as we pan out, you can see that the movements are in his face, His mouth is sort of pulled to the side and his arms are moving sort of erratic, flowing, not stereotyped, they just kind of go where they want to, it's on both sides. And then later when he tries to move his legs, you can see that they're also involved that the face is sort of contorted asymmetric, but all limbs are involved. Right? So that's an example of Korea. Korea comes from the greek Korea's which is dancing and it's described that way because it's really a regular unpredictable. Um They tend to be rapid and it happens throughout the body so it really can look like the patient is sort of dancing on the bed. They're very quick movements, they're irregular and unpredictable and they vary in basically everything timing speed and direction um Usually more often further down the extremity, not so much in the core, but more the distal limbs and usually low amplitude and tell the disease progresses and then you see much larger movements. Um But this is classically described initially as like the han driving sort of like this movement initially. Um and then if there's other belisa mus um involved in that it gets bigger. This usually happens because there's an injury or an imbalance in the direct and indirect pathways in the base. So there's excessive dopamine. This is one of the few times where that pathway really um really impacts non story non volitional movements where normally it's related to volitional movements and there are many causes of Korea. Most of them are not anything that you want in your patients. They're often neurodegenerative or autoimmune. Um But there certainly is a very common one that people have heard about, which is synonyms Korea. Um synonyms Korea can be delayed for several weeks after strep and that's important to know because getting threat throat cultures or an A. S. O. Tighter is usually not helpful. Um It could be, I've even seen up to eight months reported for having sitting in Korea. And there's no way that the families remember that he had this mild infection, you know, over six months ago that you know, clearly didn't get treated or he wouldn't have developed the Korea. So Korea in general it's president rest. But when they fall asleep, the movements stop. Usually they start and and patients don't notice them, but then they kind of build up and they're small and then they become more involved with other parts of the body. The longer they go on, especially in older patients, they can learn to make the movements purposeful. So like I um mentioned earlier when we're talking about ticks, patients often learn to take the Korea and turn it into something that looks like they were doing it on purpose. Um, that's a classic thing that happens with patients with Huntington's disease with their Korea. So it's often hard to even appreciate it for, for months for some patients that they're having these abnormal movements. One exam maneuver that you can do with patients if you think it's ticks, but it looks funny and you're trying to differentiate between Korea and ticks is something called motor and persistence. So if you have a patient squeeze your fingers, a patient with ticks will squeeze and squeeze and squeeze and you may see overflow where like they start to contort their face because they're trying so hard to squeeze your hands but they don't change their grip. They keep squeezing. Patients with Korea often will lessen and sort of sort of have a milking um movement with their fingers are trying to maintain the grip, but they have this in persistence and so some parts lesson and then it contracts again. So that's one good exam maneuver to try and differentiate between them. The other thing we see in older patients as post pump Korea, So patients that had an M. I. Or they had heart surgery and they had to be on a pump. Um Then they often end up with damage or um ischemia to the basil ganglia. Alright synonyms, Korea is an autoimmune disorder. Um It's a late manifestation of rheumatic fever and can be quite delayed usually. What's more um more pressing is the changes in behavior. The movements are sometimes not noticed until late until they become larger and more dramatic. But usually they see emotional changes. They're more label they've got more anxiety. Um I think this is where um there's a lot of uh contention with pandas and pans and where that comes in because you know that's classically described with O. C. D. And behavioral changes and I'm not going to get into the politics there for whether or not that's something that we treat. But for this condition for sitting homes it's well described that there's changes in anxiety and emotional ability. The important thing is that the Korea self resolves doesn't usually need to be treated. Um it's you know, difficult because the family that the patient often loses ability to do a TLS when they have the Korea but typically goes away on its own. There are some patients that have recurrences but generally itself resolved and treatment is not necessary other than supportive care. Of course there's other features that need to be treated. But the Korea itself doesn't usually need treatment. And then the other things that we often see with um Korea or a ketosis and and Bill is um um these are Korea. Apoptosis is usually what people describe. These are the more writhing um quality movement. So the Korea is the dance like. And then the apoptosis is slower and finger movements, things like that. And the bull is um heh metabolism is the gigantic movements that people are doing All right. Our last case, if we have time, Okay, we'll do this one very quickly and then we'll have time for questions. So this is a 12 year old girl who's having trouble writing because your fingers keep getting stuck. Um so this one is an older patient, you can see they're writing. And as the longer they go on with their writing, the hand starts to have a sort of um pronation. Oh quality movement to the wrist. The fingers start to get tighter and curled down for the base of the palm and the hand becomes more contorted and you can see the handwriting changes and it becomes harder and harder for that patient to actually functionally right. So the writer's cramp is the probably the most common example, but this is the type of dystonia. Um Dystonia is um contraction of competitive competing muscles. Um It's often repetitive, so it happens the same time, just like with writing every time they try and write, they see it happen. Um And it usually um leads to a sort of twisting or tremulous movement. So we saw that this tonic tremor that the other patient had in one of the other videos Because the muscles are competing against each other anytime one wins, you see a little bit of tremor with it, um It usually has a trigger, so a voluntary movement is usually what leads to it happening and with Children, what we worry about is our neuro degenerative degenerative disorders. The most one the one that people most people have heard about is pecan, which is your Panasonic kinds associated neuro degeneration. This is progressive injury of the basal ganglia. This is the tiger's eye that you see on MRI from your, you know um studying way back when from your all your medical school exams. But this is the neurodegenerative in the basal ganglia. So when we see dystonia and an otherwise healthy child, we definitely do some work up. It's commonly seen in patients with cerebral palsy. So we don't get too concerned if we know the patient has cerebral palsy. It's usually because they have high tone and then they have a volitional movement that leads to this happening. Um Other ones that we see Sandifer syndrome and protocols, those are considered studio dystonia. They're not true forms of dystonia but those are more like um sort of like a muscle cramp that happens. Other forms of dystonia the one week see um a lot in the emergency room is an acute this tonic reaction. Um And neurology especially we use a lot of medicines that have dopamine ergic blockade. Uh We treat migraines with things like prochlorperazine. uh and that can lead to adjust tonic reaction where that basically the neck gets stiff, the head extends and then the eyes often look up and the patient just feel stuck. The good news is that anti coal energy mix been a drill is a treatment for it and it works all right. Um I think this is a good stopping point. Um The last one is Dyskinesia and these are just ones I don't have videos from because they all look different. If you see a movement that doesn't fit with the ones that I showed videos for earlier, then I would expect a referral to neurology and that's what the dyskinesia is. Are they really are abnormal funny looking, sometimes triggered by movement? Sometimes not, but if you see movements like that we would like to see them in neurology. Usually they're benign, but it's good to rule out other other things. Alright, so key points here um abnormal movements other than simple tips. I know you guys are great at treating simple ticks. I see it. Um Those don't need to come to neurology, but we're always there for a second opinion. Or if you just want us to rule out anything else, we're happy to see them. But any movements other than simple ticks. Um It's good to have a neurology evaluation because there are so many mimics out there. A nice thing for the referral is that we don't usually need brain imaging. Um Most of this is diagnosed on the exam alone. Um And so don't worry with the referral that you need to get any imaging beforehand, unless there's other features that make you worry about a brain tumor. You know, the kids vomiting things like that. Usually you can place the referral and we can take it from there and then with ones that are severe onset um or you feel like they've gotten bad really fast. Those are the kind of patients that we want you to call because there are some conditions with these mimics that are severe and really need treatment. And so if it's progressing in the outpatient setting, we can often expedite the work up. So please call us for any patients like that. And then for our neurology team at UCSF, we are cross bay. So we've got Oakland and san Francisco and then we have several outreach satellite clinics that we're seeing patients that as well. And then for our Oakland contingent were a growing team. Um We're actually planning on bringing on two more neurologists over the next year, so We will continue to grow, but we in the East Bay side are seeing patients in Walnut Creek San Ramon, um and Brentwood. Um and in addition to Oakland, so we're happy to see patients, we want you to refer to us and to place those referrals. It's pretty easy. We have an access center, it's not an easy one to remember one. It's 877 you see child. Um Just call and the specialists on the line will certainly help you place the referral.
