Chapters Transcript Tics and Tourette Syndrome Sheel Pathak, MD, explains how to distinguish between primary and secondary tic disorders as well as functional movement disorder. All right. Well, good morning, everybody. Thanks for the introduction and um recognize uh many of your names. And so, uh for those of you I haven't met or uh shared patients with uh I'm Chil Patick. I'm one of the pediatric neurologists and uh I'm out here in the West County CS CC uh building. So, thank you for all your uh referrals. And um we see a lot of patients with uh Tick and Tourette and I know you guys do as well. Um And so I thought this would be a, a good topic to uh to share on. So the first thing here is uh click through this thing. Um I uh I do not have any uh financial interest in any of uh the treatments we're gonna talk about. Uh no non-financial conflict of interest. Um Either uh we will be discussing some uh medication treatments which are off label as you'll find out uh or probably already know many of the treatments that we use for uh uh tick and Tourette and associated conditions, especially for Children are off uh label. But um yeah, but no financial interest with any of those. So uh today, we're, I want to be able to distinguish between primary and secondary tick disorders um as well as functional movement disorder, which has been a topic of great interest in recent years. And I know, um probably everyone here in the uh in the presentation has, has come across patients who have a suspected functional movement disorder rather than a primary T disorder. So it's good to be able to distinguish those um like everybody to gain confidence in making a diagnosis of Tourette and initiate some initial management for patients that need it. Um And then also give a little bit of education on the natural history and the prognosis of uh thick disorders. Hello, as in many of these presentations start with a little case. Let's look at that. And so um probably I met a child very similar to this. So eight year old boy presents to the pediatrician who is coming for a well child exam and his mother says that uh he, he gets in trouble for a variety of things at school is making some noises getting out of his seat a whole lot. Um Maybe he got some detentions or in school suspensions, things like that. Um uh for rolling his eyes at the teacher as well. Um and making these noises. And so, um talk a little further and say he has a recent diagnosis of allergic rhinitis, maybe, maybe not in a chronic cough. Um and they haven't improved this by treatment with, uh, uh, steroids and other treatments. And, um, when you meet this, uh, child, he shakes your right hand and then he, he gets very, uh fixated on shaking your left hand as well. Um, before he can continue and you notice that he's hyperkinetic guy, his throat following directions, hes a lot of redirection and then has a flurry of cough during the exam. And he asked him, oh, are you? ok, why are you coughing so much? And he said he had to do it over again until, uh, it felt right or felt perfect. And so this is, uh, maybe an extreme example of somebody with all of these, uh, different, uh, points that we'll, we'll touch on. But I think we've met someone with a little bit of these features. Um, and then when leaving the visit, he takes, uh, two steps forward and then stoops down to touch the ground. And, um, he, he says he has to do this. And, um, his mother says that he always freaks out about everything and she asked him to, to stop doing that and stop touching the ground and, and says, you know, something bad is gonna happen if he, if he doesn't do this. And so, you know, some things that we may ask, uh, his family about, um, obviously this, uh, raises concern for some ticks, maybe the coughing sniffing or, or eye blinking. Some kids get, uh, diagnosed with, uh, allergic rhinitis. Um before they end up in the neurologist's office, he also had some uh sound like some compulsive behaviors and some maybe obsession with symmetry. Um These are all things that we see in our patients and maybe he has uh a DH D as well. We probably tease that out a little bit more if we talk to them. And so, um these are some of the things that we, we may talk about. So he, he had throat clearing, sniffing, eye rolling and vocalizations. Um And then maybe a fear of causing harm, he says, if he doesn't touch the ground in that uh special order, he might uh you know, something bad is gonna happen. Um And then he had some symmetry, had some rituals and then, you know, there are some concerns about uh his school performance and then we have funny comic because I like funny comics. Um And so that's, that's a pretty uh illustrative case. But um before we talk about the, the nuts and bolts of ticks and, and tourettes, I always like a little bit of uh history and stuff just because it's fun. Um uh ticks and Tourette we think have been that are described in popular literature since a long, long time ago. And so in uh the late 14 hundreds, um a couple of German uh clergymen wrote this um Melius Malefic Carum, which uh translates to the Hammer of the witches. And it was a treatise on witchcraft and demons. And um I think that's where they, the first written description of uh somebody who had uh possible t was when we went back and looked at that. And so it was a, a little excerpt from that where the, the priest in question, that I cannot help myself at all for, he still uses all my limbs and organs, my neck, tongue, lungs whenever he pleases causing me to speak or cry out. And I hear the words uh if they were spoken by myself, but I'm altogether unable to restrain them. When I try to engage in prayer, he attacks me more violently, thrusting out my tongue. So, yeah, maybe this was a, a tick. It's certainly interesting. Uh perhaps he was possessed by demons. I guess that's for a different type of lecture. I don't know. Um But uh that was the, the first uh described thing. Um Fast forward a few 100 years later. Um There's a famous case, uh There is a physician Jeanmarc Gaspard Guard and he came in contact with this woman, the Marquis De Dampier and she's a noble woman and she frequently uh shouted, you know, the things that she shouldn't uh shout and some curse words and things. And um her doing this was uh in stark contrast to her background and her station in life. And so they took uh interest in this and said, why is uh why is she doing this? Um The famous neurologist, Doctor Trese described uh similar cases uh several years later. And so this was one of the first uh cases of uh vocal tics that was uh described in recent times. Then. Uh a little bit more of history. This um the uh Pit Sal Petrie Hospital I think is how you pronounce it. I have no idea how to speak French. But um this opened in 1670 it's actually uh still a functioning hospital in, in France. Uh It was the workplace of several neurologists uh including doctor uh Charcot and uh and doctor Tot who is his resident. Um And so painting with, with these guys, they don't make paintings of us anymore. It's, it's a shame um and would look less uh luxurious than this. But uh I think they're all examining a patient who they potentially diagnosed with uh hysteria and we don't use that term anymore. Um But there's doctor Tot who wrote the first uh uh case uh series of uh patients with his name disorder. Um and he wrote this in 1885. He uh wrote a case series of nine patients who had uh ticks and uh they had these repetitive movements and there was some um evidence that these were heritable because he had some um parent child pairs in there. Um All the patients who had uh been reported on in this uh had a waxing and waning phenomenon with their ticks and they all described a premonitory sensation prior to the ticks. And uh that was one of the most famous things that uh that he did. He, he studied ataxia, he studied hysteria, he studied hypnosis as well. Um But uh kind of a sad story. Yeah, he was shot by an angry patient. Uh but actually, he didn't die from this. And then uh several years later developed a neurodegenerative condition and was sent away to an asylum. So, you know, a uh unglamorous end for Doctor Tot. But uh we still have his paper and his teachings, but he also attributed the tick symptoms to the sin of previous generations. So that's it, I guess antiquated way to think about it. But um we don't do that anymore, but it didn't advance much because over the next 80 years, um we had a lot to uh attribute to this guy, Doctor Freud who uh felt that psychosexual conflict was the center of Tourette and other tick disorders. Um and thought that people who had ticks were left-handed or sinful, et cetera, et cetera. And, you know, we did a lot of uh psychotherapy for this and fast forward to several years later when there was the uh epidemic encephalitis in the uh 19 hundreds and that uh patients developed uh tick disorder. Many patients developed a tick disorder after this uh um uh flu epidemic and it pointed to a potential as a organic cause of uh of ticks rather than uh spirits and, and such and fast forward even more uh medication haloperidol was uh synthesized at Jansen labs. And uh it was used for patients with uh psychotic disorders and schizophrenia. And many of those patients who had comorbid movement disorders, ticks uh had improvement of those things also pointing to a uh neurobiological basis of uh of the disorder. And uh this doctor Zain and Arthur Shapiro actually wrote a case report on this in uh 1968 showing that uh there was a benefit in T disorders uh with uh haloperidol. They studied uh 34 patients with this. Many of them had to stop the uh medication due to uh side effects and weight gain and, and um abnormal movements, extra Permal symptoms, things like that. But there was a reduction in their ticks and then fast forward. Um in 1972 the Tourette Association of America was called the Tourette's Syndrome Association of America. Now, it's called the Tourette Association of America. Um Removing the word uh syndrome to further destigmatize the condition. And uh Washington University has actually been named a Tourette Center of Excellence, meaning that uh we have um a good uh clinical treatment team as well as our research team to uh study T disorders. And so, while I'm using the words tick and to um I was like to, you know, cycle back just a little bit and say, well, what is a movement disorder? You know, people refer to our Movement Disorder Clinic all the time. And so what is that? Um So uh when I think of movement disorder, I think that uh it's a movement that's happening wrong basically. So it's inaccurate. So, you know, if you have um some uh dysmetria or something like that, so incorrect postures, the other thing or movements at the wrong time. So if I have a tick, it doesn't serve a purpose. It's happening for no reason. Um It's not uh functional. And so um yeah, wrong time, incorrect posture and accurate movement uh encompasses a lot of the uh the movement disorders, uh inaccurate speed or I'm sorry, in incorrect speed would be another one. And we separate movement disorders into two big categories. So you have, we'll start with hypokinetic movement disorders, um Bradykinesia, kinesia. So the biggest one there is parkinsonism and then you have hyperkinetic movement disorders where you're moving too much at the wrong time in the wrong way. So, um compared about aosis kind of slow riding movements of beus uh fast, uh uh big movements of proximal muscles, corea is fast, uh uh movements uh that are uh nonfunctional or nonpurposefully in the distal extremities. And then dystonia kind of sustained uh postures um because of uh abnormal co contraction of uh muscles and ataxia falls in that category and then ticks and stereos uh as well as tremors. So, those are kind of how I think about movement disorders when uh when I see a patient. And so when we are evaluating these patients with uh movement disorders, we try to uh obtain information from the parent and the child just like we would do for any condition. Um, but uh we really wanna know these specific things. And so when I speak to our residents who are seeing a patient with ticks for the first time, um I really want them to ask uh these specific things. So, is the child aware that they're doing this? You know, maybe they're completely unaware. I've certainly picked up a couple uh uh instances of absence epilepsy where they came in for an evaluation of blinking tick, but it was really absence epilepsy. Uh He was completely unaware that he was doing it. It was a complete surprise to him. But uh you know, it was uh that was an important point if they have an urge. So in early childhood Children are, are um not really able to verbalize this, but we can still get a, a feeling that they might be experiencing some uh premonitory urge prior to a tick. Um Some people will be able to articulate this and say, oh yeah, I get this itch feeling. I have to blink my eyes and if I don't do it, I'll feel weird. Um Whereas uh younger kids, I try to get that out of them, you know, if I can, by first asking if they know that they're doing it. And then I ask them, especially if they're doing this a lot. So, what if I tell you to not do it for five minutes? Uh, would you be able to stop and say no, I'll feel weird. It'll just happen. And so you can get a lot of information, um, out of even kids who are not fully aware that, uh, this is happening, like to know whether or not it's suppressible. If it's completely involuntary, you still have to entertain the possibility of a focal seizure or something. But they say, yeah, I can try to stop it for a little while, but then it happens that, you know, lends more credence to the idea of a tick. Um I really like to know what the exacerbating factors are like, oh when, when somebody talks about it or, you know, certain functional neurologic disorder type ticks might be triggered. Oh If you say the word banana, I'm gonna have my tick that, you know, I'd really like to know that sort of thing. What makes it better? Um If it causes pain or functional impairment. Um And then whether or not it's disruptive on other people and social interactions. So those are the very important tick points. And then obviously you wanna know just like any other condition, what past treatments they've had for it and how it affect school performance and comorbid conditions, uh things like anxiety O CD A DH D. Um you want to know their developmental history concerns for autism spectrum disorder, for example. And then I really like to ask about family. There's been, you know, several times when, you know, I'll ask a, uh, a parent and say, well, what about your ticks? They've been blinking the whole time or making a sniffing noise or something? I said, what are you talking about? Your, your t and so, um, a lot of parents come in and I highlight that because say, gosh, they've gone through life and have been successful and, and have Children and, and all these things and, um, didn't really notice their ticks. It was just something that they did. And so it kind of helps to destigmatize the, the problem a little bit. Um But a lot of parents have it and then obviously like to know the regular social history of parental education level and then when I examine, uh, Children, these are the important things. Um, but one thing that's a little bit different when we pull our patients from the waiting room, uh a little TV in there that's playing Disney channel. And I really like to see what the child is doing just uh while watching television. A lot of times people have an increase in ticks that you can observe while they're passively consuming media. And, um, I see a lot of it in there and it kind of goes away when they come into the office. And so the waiting room exam is important. Um I like to know about their general level of development as well as uh you know, their speech and language. Um And then level of hyperkinesis, sometimes kids, you know, are drawing on my walls and touching all the equipment and running around the room and trying to lope and things like that. Um or need a lot of redirection kind of gives you uh information on things like a DH D and then the rest of the neurologic exam is important to rule out any comorbid, uh, movement disorders. Sometimes it's not Tourette or primary tick disorder. Sometimes it's, you know, Huntington's disease or sometimes it's, you know, some drug induced thing or, you know, what have you. And so we'll go through some of those. But if you have other movement disorders that, um, you know, kind of develop at the same time as the tick disorder makes you think not necessarily a primary movement disorder and something else may be going on. So, what is a tick, you know, type of hyperkinetic movement disorder? We know that, um, but they're stereotype, intermittent, sudden, discrete and repetitive movements. Um, motor ticks typically involve skeletal muscle. It can be kind of a quick clonic tick, a shoulder shrug, an eye blink, nose twitch, a facial grimace. It typically involve the head and neck shoulders. Uh, not to say that they can't involve other parts of the body, but typically they, there is a uh, an abundance of, uh, of ticks in, in that area. Um, if somebody has most of their ticks in their feet or legs or some other part of their body, um, I think that's a little bit atypical. I know. I, uh, ask about other things. Um, you can also have phonic or vocal tics. They involve a diaphragm, laryngeal pharyngeal muscles. We think about the, you know, words that people say are grunting, throat clearing, sounds things like that, but sniffing also uh counts, it makes a noise. Um physiologically, there's probably not a huge difference between these things. And I think they're classified as such because of the uh the social problems that the phonic tics may cause um in relation to the motor tics. And whenever anybody mentions tick or Tourette, we think of, you know, the, the Marquis de D Pierre and Cerelia or potty mouth because it's the most recognizable manifestation. I call it that the Hollywood version of uh Tick and Tourette. And I like to emphasize to uh to families that, you know, in large scale studies, it's only present in 10 to 15% of people um in primary tick disorders and functional tick disorders. It's, it's much more and we'll talk about that. But um it's not something that we expect to happen. We don't expect their child to be in the middle of the store screaming obscenities, things like that. And so hopefully that's a bit of a reassurance to the families that present for the first time. But ticks may be important and gets you into the door of the neurologist's office. But oftentimes I find that it's other comorbidities that are far more important than the ticks themselves. Um, and we'll get into those in a minute. One thing I said about the ticks also was that they are, um, they're discrete short stereotyped movements, but that's not to say that you cannot have a complex tick. Sometimes people have clusters of movements. Um I saw a patient uh recently who had a, a dystonic tick, meaning that they had a facial grimace and kind of scrunch their body up in this way and they did it in the same way each time they could reproduce the movement. Um and kind of a funny dystonic posture and uh that can be a tick. Sometimes you can have a stereotype sequence of ticks. Um When you have very complex things, I think more about uh motor stere, which is slightly different um versus a, a tick, but you can't have ticks that are complex like that. Uh Sometimes you have ticks that are caused by other ticks like uh I've had patients who have a air swallowing kind of compulsion and that leads to a burping tick. Um You can have movement or uh speech blocking, meaning that you're in the middle of doing some activity and then have to pause or, you know, freeze for a minute. Um, so there's, there's a lot of, uh, complex ticks. Um, but it's important to distinguish it from other movement disorders. Stereo, for example, that would be associated with autism spectrum disorder or, um, you know, a tick that is actually, uh, you know, dystonic spasm or something like that and things that would tell me it's a tick versus one of these other things if it's waxing and waning, oh, basketball tryouts are coming up and he's doing this movement again. And oh, school's starting, we're doing the movement again. Our Disney world is coming up next week and we started doing the movement again and oh, we're totally relaxed and it's gone away a little bit. So if we have that pattern, you know, think about tips. I said earlier when we're passively consuming media ticks occur a lot. So occurs when bored. Um, oftentimes they worsen acutely during times of high emotion or physical stress. Um, premonitory urges, we spoke about earlier and then possibly, uh they can be suppressible and by and large they go away and sleep. Um But there is evidence that people can have during uh light sleep, some ticks uh still. So it's not 100% type of thing, but movement disorders in general tend to get better during sleep. And one thing about the premonitory urge, um it's important to distinguish that from what we call. Um I guess uh for monetary in a compulsion or O CD in Tourette and ticks. The premonitory urge is often vague. It's, uh, it's a feeling of just on discomfort or, you know, what I've described to people is it's a feeling that, that you get, if you're in a staring contest with somebody and you have to blink your eyes, why? Just because you do not necessarily because your eyes are dry, but just because you have to do it for some reason. Whereas in a compulsion like an O CD type of urge, you have to do the thing or some specific type of thing will happen. You know, uh I need to wash my hands 50,000 times because otherwise I'll get an infection and spread it to everybody and we'll get the plague or something like that. And so, um, it's a more formed premonition rather than a vague thing that we see in ticks. And, uh, one interesting quote, I conclude from some patient, this was, uh, I read this somewhere. Um The patient said the ticks aren't my Tourette. Uh, Tourette's the itch, it's the urge. And, uh, the tick is the way that I make that feel better. And in the same way with O CD, you have that feeling, oh, my hands are dirty or I'm gonna spread disease. And the thing that you do the compulsion is what you do to relieve that feeling in your head and make you more comfortable again. So, the criteria for Tourette, according to DS M five, presence of at least two motor and one vocal tick. They don't need to happen concurrently. The DS M four had a provision where, um, you couldn't have a three month break between the ticks and stuff that wasn't really based on anything. And so they took that away. Um, but just two motor, one vocal tick, not concurrent needs to last longer than one year. If it's less than one year, we call it a provisional tick disorder kind of thing that it may go away. Um, and then onset prior to age 18, certainly, you know, I suppose somebody could develop a tick disorder after 18, but it would be unusual and we look at other things and, um, we have to make sure it's not attributable to another medical disorder. So, medication toxicity. Oh, I took, you know, Benadryl and I had to start having these movements or it's not because of, you know, encephalitis or a stroke or something like that. And so, um, we can attribute it to something else and it was a primary tick disorder. And, um, a lot of times the most, the common eye blink or a common tick is the eye blink. And that's what gets a lot of people into the office and takes typically around 67 is when we start seeing, I've seen them younger as well and, um, they tend to wax and wane until maybe the middle school years where they might peak and then a lot of times they improve and t severity typically gets, uh, better as, as you get older. And besides Tourette Syndrome, we also have chronic motor tick disorder. If you just have those chronic vocal tick disorder, if you have those, um, there's something called other specified tick disorder, which is similar to Tourette, but they don't necessarily meet criteria because of something like you think they're probably gonna develop Tourette, but it's only been going on, uh, nine months, but you really think it's gonna be going in that direction or it happened when they were 19 years old or something like that. Um, and then there's unspecified tick disorder. Um, maybe there's not enough information as to what's going on that you're just presuming things, but those are the primary tick disorders. Um, we also have transient T disorder meaning you had a tick, it went away and never came back. Um, you have to watch somebody for a very long time to truly diagnose that provisional tick disorder is a diagnosis that we give a lot of times because, because, hey, you have these ticks and it's been going on for less than a year. We don't know exactly how it's gonna go. But, um, we're gonna keep watching you and obviously you can have a secondary tick disorder from a neuro neurodegenerative conditions TV. I, uh, drugs, stroke, things like that. Um, ticks pretty prevalent actually, you know, depending on the study that you look at, I think 6 to 12% of people might have some lifetime tick. I've seen estimates up to 25% which is huge number. Um, but a lot of ticks are underrecognizing, which is probably why we have such a spread. Um, I think if you walk around the grocery store, you can see people with ticks all the time. Um, but the thought of the prevalence of tot is, uh, about 0.1 to, to 3% of the population, maybe a little bit less than that. But um I think if we're thinking that it's unrecognized and they overestimate a little bit. Um Primary disorders have a uh male to female ratio of about 3 to 1 in terms of uh prevalence. And um it's more common in Children with various neurodevelopmental disorders. So, a DH D Autism Spectrum disorder and what's interesting is they did a survey of children's health. This is from the CDC. And um the red lines indicate patients who are diagnosed with uh Tourette and the blue is uh patients without uh Tourette. And what's interesting is there are conditions which are potentially unrelated where um people who are diagnosed with Tourette have a higher uh predisposition to these uh diagnoses in including asthma, which is interesting. But uh there's much more anxiety, depression, behavioral problems, A DH D developmental problems, intellectual disability and um and so on and so forth. And one important thing I keep talking about the comorbidities um is the most important thing. This is a famous Venn diagram for from doctor uh Vanko, famous movement disorder specialist. And um the ticks are important. Of course, it's at the top, but many patients have a DH D, many patients have O CD and many patients have uh other behavioral problems that are not neatly classified in these other boxes. And at the intersection of these is where we put Tourette and I tell families and I draw this all the time for them. And I say that um everybody has a different size of each of these circles. And what we focus on really has to do with which is the biggest, most bothersome circle. So O CD occurs in a large amount of patients with uh with Tourette. Um People will have uh rituals of symmetry like the child in the case at the beginning. Um And it, it can be really distressing and it's treated differently than primary things. Um Here's some common uh obsessions and compulsions. So everybody knows about the hand washing because of the obsessive thought about uh contamination. Um I've had uh people with uh ticks especially will do a symmetry thing. I do a tick on one side of my body and I have to do it on the other side. Um When I feel there's a compulsive uh component to the ticks, I'll ask a lot more about it. Anxieties and other O CD type things. Um But I've had people uh compulsively confess their sins because they had a bad thought. Um This happens in uh middle school kids a whole lot. Um I've seen it a handful of times. Um But yeah, those are some common obsessions and compulsions, other things. A DH d everyone in this talk has encountered this um occurs in about 50% of patients with tick disorders. The hyperactive phenotype is a little bit more common and I always like to ask about learning disability, emotional problems. Um and, and people can have uh big emotional dysregulation and what we call rage attacks with uh with tick disorders as well and anxiety disorders um kind of in uh I'd say O CD may be a subset or, or similar to anxiety disorders, but very uh prevalent in patients with ticks and often times it can drive the ticks. I'm feeling more anxious and thereby I have more ticks during that time and uh school problems as well. Um So uh patients with uh to and learning disability actually have more mood disorders. And so we always wanna make sure that we are addressing the school problems and th this is kind of a a hard slide to look at. But um you know, have copies of this, maybe you can look at it, but basically, this is um just a closer look at all the comorbidities that can occur. This is a large cover of 3500 patients with a diagnosis of uh Tourette and seeing what other types of things happen to them. We know that uh the mean age of onset of picks here was 6.5, about um 60% of the patients in this cohort had a DH D 37% had anger issues and then the Cooperia they addressed that as well. So 14% in this. So other things that we think about, you know, how do we address these comorbidities? Um I referred to our friendly neighborhood psychologists and psychiatrists and friendly neighborhood neurologists um to address some of these things. Um And it can also start treatment for a DH D um and uh initiate psychometric testing or learning disabilities at children's hospital as a neuropsychology department. Um There's uh many folks in town that uh are also very capable and, and great in seeing our uh Tourette patients. Um many of the scales that we use are not really uh don't lend themselves to use in a clinic setting, but some of the things Vander o'connor's, you know, people take home and, and send back to you and can be useful. But for O CD, I tend to just ask without using scales, but some of the psychologists will use scales in their visits. Nice thing I show families this slide a lot actually. And so it's a natural history uh study from uh 2009 and they had a scale of uh t severity and um this was over their age, it starts around 56 and then peaks around uh you know, age nine to, you know, 1011 and then tends to get better after that. And a similar thing here is that um this uh red represents uh moderate or greater pi severity. Um And then uh blue is mild, yellow is minimal and white is, is no takes. And what they did was they took a sample of patients, let's say, 20 years ago, who were all in the red category and they interviewed them again 20 years later and they said, ok, where are we at right now? Most of them, 80% of them were better. And I'm sure if we took this moderate or greater tick severity and, and subclass, moderate and severe or worsened ticks, it would be even more, you know, hopeful that uh many patients improved. However, um things like hyperkinetic disorder, um it gets more subtle as you get older. You might be more fidgety rather than, you know, overtly hyperkinetic. Um T tend to get better but things like O CD and I would say anxiety as well tends to get maybe a little bit worse um and not much better as you get older. I tell folks there's more things to be anxious about when you are older. Um And this just talks about t severity and O CD sever echoes the last slide in that uh uh in follow up a lot of patients with their t got better after several years. But uh time to follow up for O CD got worse. So it's something that they need to keep track of and we won't spend too much time on this. But uh when we think about the localization of ticks, we think about the, the neurobiological basis of ticks, um we think about the basal ganglia and there's plenty of evidence of basal ganglia dysfunction in ticks. But it's the large deep structure in the brain. Um a lot of interconnected uh nuclei that interact with the motor system and the limbic system and the motor planning system. And so that's why there's a heavy connection between emotions, anxiety and, and ticks. Um And we think that dysfunction in any of the circuits in the basal ganglia can result in unintended um movements and movement disorders. And so the coronal view of uh the brain, this is actually a patient with Huntington's disease that shows uh some atrophy in the basal ganglia. And then um vacuo dilation of the, of the ventricles. Um And so, um that's the area of the brain that we think is implicated in ticks as well. And there's evidence of basal ganglia dysfunction in F MRI studies of patients with Tourette. And uh there might be uh receptor abnormalities in D two dopamine receptors which are important in movement disorders. Um Think about primary versus secondary ticks. Ticks are thought to be polygenic. Um We don't have a tick gene. Um, there is some, a region on chromosome two P that's associated with chronic ticks and they actually, uh found a, a gene, his decarboxylase is implicated in a family, um, who had uh a lot of ticks and there's a variety other uh genes that were thought to contribute. But, um, there's no as of now tick panel or anything like that, but I tend to see uh a lot of inherit ability in this. And so we asked about family members and stuff with ticks. And uh oftentimes things like a DH D and anxiety will run in the families as well. We talked about secondary ticks or touret is and here's some other uh causes. So, infections, we talked about phal encephalitis. So, drugs toxins, uh various developmental disorders, genetic conditions, stroke. Um there's a variety of um neurodegenerative diseases. So, Huntington's disease, neuro campy tosis, um uh iron accumulation in the brain, um or pecan um Wilson disease and a variety of neuro cutaneous disorders can be associated with ticks as well. Um And then I think a really important thing that um everybody will come across is probably the most important thing of now is uh functional neurologic disorder. And so, um you know, previously termed conversion disorder, a variety of things. Um We've seen a lot more of this since the beginning of the COVID-19 pandemic um clinicians and many institutions actually reported an increase in patients. And I'm sure everybody here has uh encountered something like this rapid onset tick like movements, characteristic vocalizations. Um More frequently, they're complex movements. They're not as much short stereotype movements, but um uh throwing things, I've had patients uh uh hitting a whole lot. Uh Copper Lelia is very prominent in this and then copper praxia. So making a little gestures and things. Um these ticks that I see in these uh slightly older kids frequently interfere with focused activity where primary takes if you're focused in on something, the ticks might abate for a little while. Whereas in functional neurologic disorder ticks, uh they tend to get worse. Um And um many patients in the studied registry in the UK, I think uh were tot influencers on social media. Um and had characteristic vocalizations. There was a, a girl that said the word beans over and over again or a lot of people that will say uh a variety of uh obscenities that change in the course of like one minute. Um So it's very unusual for the patients that we see with the standard trajectory as described earlier. And um they've actually gone through and found some important distinctions. Um whereas primary ticks might start in early childhood, the functional neurologic disorder takes more affect adolescents and teens um affect uh females more than males. And there's a rapid dramatic onset like, oh in the course of, you know, this one Tik Tok video that I make I'll go through eight different ticks. Whereas, uh, ticks in uh primary t disorders tend to wax and wane and kind of have a stuttering start over the years. Um, the functional neurologic disorder ticks are complex from the start and don't have that typical face. Next, shoulder predisposition and more trunk arms, uh, voice. There's leg ticks, things like that, that are just a little bit more unusual, um, copper phenomena like, uh, you know, these are much more common um hitting self, things like that are more common. And a premonitory urge does not just between the two because people will describe it for mandatory urge in in FND type of tick, but it's, it's different. Um They'll a lot of patients to me have described like, oh before I do my ticks, I feel like energy pulse or I feel whole body pressure. Um but I, I don't hear that as much in people with primary ticks. They just say I get a feeling that I have to. Um a lot of times I see triggered ticks as well. I mentioned the a patient I had that said, oh if you say the word banana, I'll have these three ticks or something like that. That's unusual in patients who have a primary tick disorder. Um a lot of times a DH do CD anxiety are common in primary t disorders, but we see a lot more anxiety, depression, suicidal ideation actually in patients uh with functional ticks. And so it is a, a big concern. Something to pay good attention to. Um, and there's health disparities over here as well. Um, focus on, uh, public insurance versus private insurance will have a later diagnosis and tend to be diagnosed more with mood disorders or behavior disorders rather than, uh, focus on their tick and what's bothering them. Um, nonwhite patients, uh, tend to be, uh, diagnosed less, uh, with, uh, tick disorders and, uh, might have more unmet uh mental health needs and uh increased suicidality compared to controls. And in general, uh patients with tick disorders and to, um, might have a higher, increased risk of suicidality as well are the things that we have to pay attention to? And in the last part of the talk, we talk about the treatment and I think this is the most important slide. Um, the patients will come in and the parents say you got to stop this t and I always determine whether the treatment is warranted first. Is it bothersome to the patient? So, not bothersome to the mom and dad, not bothersome to Johnny down the street or whoever, but bothersome to the patient. If I, you know, I always folks, if I give someone a medicine for something that primarily bothers someone else that's not really a good message to, to send, you know, unless it's disruptive or, or harmful. Um, if it's uh violent or potentially injurious, there is a condition called the malignant tick disorder where you think that you're gonna, you know, dissect the vertebral artery from a neck t or something like that. Um And we have to treat those or if it's very socially inappropriate, then we always give reassurance and plenty of education regarding the natural history. And I tell people about resources that are available in the area. We'll go through that uh in a minute and if treatment is needed, we think about the comorbidities. Do you have anxiety or is it just the ticks or the A DH D as well? The side effect profile and plenty of medications. This is more of a reference uh thing. But we of often start with not the uh D two antagonists like Haldol and Heide and risperiDONE. So those are the old school kind of medications that can work very well. But obviously, antipsychotic or neuroleptic medications have their big side effects. Oftentimes we start with Alpha agonist pretty well tolerated, safe uh Quon and guon um and help with uh comorbid conditions like hyper uh kinetic disorders. Once in a while, we're using uh other things that uh deplete dopamine, tetrabenazine, uh valbenazine do tetrabenazine um that are used for a variety of hyperkinetic conditions and then botulinum toxin has been used as well. Um A lot of patients have ticks and A DH D together. Um There's black box warnings that don't use stimulants in patients with ticks. That's, that's false. Um is actually um core with alpha agonist and a stimulant such as methylphenidate would, uh, synergistically improve ticks and, uh, a DH D symptoms over time. Um, in the short term period, you might have an increase in ticks. But that's true actually, of any treatment. And so, uh, we don't need to be afraid of stimulant medications in patients that need it. Um, here's again, some of the common ones that we use quantity and guanine, we use toy make the anti seizure medication, um, which is thought to help ticks. Um, it helps a little bit sometimes use baclofen, especially if I have somebody who's having a shoulder neck bothersome ticks. And once again, Berlin is not gonna make things worse. Um, maybe in the short term, other non pharmacologic treatments. Um, CV, it or comprehensive behavioral intervention for ticks evidence based, it's protocol driven and it combines figuring out situations where the ticks might get worse and, you know, avoiding or modifying those situations. Um, and doing some habit reversal, which means that if you feel the premonitory urge, um, you're gonna do some competing exercise and after practicing that over and over again, the urge actually goes down. There's evidence for CBIT working. It's almost on par with, uh, uh, medications. And, um, I like to start with that when somebody has one or two bothersome ticks rather than a ton of different ones. It can be really useful and empowering. Um, it's better than just supportive behavioral therapy. A few people in town that uh that do C VIP that we refer to. It's also unusual in kids. So we won't spend much time on this at all. But deep brain stimulation has been studied for the treatment in Tourette's syndrome. Um There's plenty of trials to figure out with the, the right spot to put the leaves in. But I will say that not a lot of kids, you know, parents might ask about it, but for things that we know that are gonna get better as you get older, it's uh a hard cell to put a uh implantable device in somebody's brain. Uh They're also studying transcranial magnetic stimulation, but um it's not ready for prime time yet and we will all be asked about this. Um You know, can my child have medical marijuana for uh ticks? I think there's a lot more negatives than there are positives. There's not a lot of study here. But what's interesting is that um the two randomized controlled childs that I so cannabinoids for ticks is actually looking at THC as a therapeutic chemical, not CBD as in many other conditions. Um So we don't want kids to necessarily have THC for a variety of reasons. And so there are other better treatments and the studies were not super uh rigorous for some other things that are coming down the pipe, uh newer dopamine depleting medications, there's a medication called OP which is uh D one and D five receptor antagonist and um it's showing some promise and an ayl cysteine for repetitive behaviors, people study that it's over the counter as well, uh thought to reduce glutamate release. Um And so um those are some things that are coming down the pipe um that are useful. Biggest thing. So remember to treat the comorbidities, don't just treat the ticks, look for what's contributing to the ticks, uh treat anxiety O CD um support the learning disabilities and the attention deficit as well. That's the biggest takeaway. And one last thing just because we'll all be asked this as well. Um This is probably could take up a whole hour on its own, but let's spend one minute. Um There is Panda's pediatric autoimmune neuropsychiatric disorder associated with strep and its associated conditions, pans um and cans. Um which we think, ok, we have ticks that develop after getting a strep infection or some viral infection. Um It's very hard to make a temporal relation between a strep infection and behavior problems. Ticks and things like that. Ticks are actually thought of as a minor criteria. And there's a lot of controversy here because uh strep is very common. Some people are colonized with it. It's difficult to establish a real temporal relationship between the infection and the uh development of ticks. And there's similar neuropsychiatric manifestations observed in rheumatic fever in Sydenham S corea, like people will have ticks with that. And so I always tell folks if, if somebody's coming in and they're being evaluated for candas. You wanna make sure that we're not missing Sydenham S corea and missing treating them with antibiotics to protect their heart. Um, you know, from endocarditis. Um, and there's other viruses that can cause this, but we know in general in neurologic conditions, uh infection, you know, viruses strep whatever can worsen the underlying neurologic condition. And so I really like to ask about underlying, you know, did they have ticks before? And oftentimes you can get a history. But yeah, they were eye blinking before. Yeah, they were a little anxious before where we think, ok, this is just uh the strep or virus worsening their underlying condition rather than this completely new thing and the potential treatment is unproven, you know, right now, Ivig or, or exchange um sorry, um riTUXimab and other immune suppressants um are expensive unproven or potentially dangerous as well. And so the tough one and I'd say referred to neurologists of uh considering those are referred to infectious disease. Um treating with chronic antibiotics is um not really recommended in this type of condition. Um We don't in rheumatic fever, don't give antibiotics for prevention of the neurologic sequela of this. We do it to prevent endocarditis. Um and we use neuro mes to treat the neuro symptoms. So a couple of other takeaways here, um threat is common cause a lot of stress in families and, and Children and identification of the manifestations leads to the right treatments. You guys can be advocates for your patients and there are plenty of resources available both to uh patients and physicians. Uh The Tourette Association of America has a wealth of information. And the Missouri branch of the Tourette Association is uh is great as well. They sometimes have get togethers um a contact for the Washington University Movement Disorder Center. There's a variety of a um movies and things including the Story of Brad Cohen, which was like a lifetime movie and he was, uh from Parkway uh graduate, uh, way back when he's a big advocate for Tourette. Um, and then was, you has the t website for people that are interested in participating in research? Um, I think that they are, uh recruiting for a, uh, a study right now, a natural history study for uh patients who have a diagnosis of uh Tourette. And so that's on their website. So that being said, obviously there's a lot more to say. Um, I went a little bit over time. I apologize. But, um, what, uh, questions can I answer? Created by Presenters Sheel Pathak, MD Pediatric Neurology View full profile
