Hello. Welcome to our session on revamping musculoskeletal care for adults with cerebral palsy. My name is Hyekyong Kim. I'm a course director for this session, and I'd like to introduce my team. So let me share the screen. Can you guys see the screen? So we are all from Columbia University Irving Medical Center in New York City, and my first speaker is Dr. David Roy. He is an emeritus professor of pediatric orthopedic surgery. Currently, he is working in China, and he is doing amazing work for people with cerebral palsy in China. And next speaker will be Dr. Amy Tenelia, who is currently a senior pediatric rehabilitation medicine fellow in my program, and she will discuss muscle and function changes in adults with CP. And next speaker will be Dr. Hana Azizi. She is currently holding assistant professor of rehabilitation and also associate program director of pediatric rehabilitation medicine at Columbia University, and she will discuss pain in adults with cerebral palsy. Then last speaker, me, and then I'd like to discuss trajectory of asbestos in people with cerebral palsy. So learning objectives follows. We'll discuss the issues facing the aging patients with cerebral palsy. We'll try to understand the spastic muscles with aging, delineate the need for mobilization in adults with cerebral palsy, and describe causes for increasing spasticity in adults with CP. Understand pain in adults with CP. Learn orthopedic perspective on care for adults with CP. Now I'd like to invite Dr. David Roy, and he will discuss orthopedic perspective. Dr. Roy, please. I'm going to start share, and you can put your top. Thank you very much, Haki. I'm so happy to be here. I'm in Guangzhou, and it's 1230 at night, so if you see a yawn, it has nothing to do with being bored with my topic, about which I'm very passionate. There is, I want to share my screen, and get to the slide view. Give me one moment, there we go. All right, and again, good evening from Guangzhou, and I'm so happy to be here with my Columbia colleagues, talking about a subject that I'm very passionate about, which is the treatment of adults with cerebral palsy. These are my disclosures, none of which are cogent to tonight's talk. What are we talking about tonight? Adult providers are not taught to care for adults with childhood onset disabilities like CP. We talk about transition, but there are precious few human or logistical assets to whom we can transfer our aging patients. In addition to being concerned about how to treat, and how to organize, we have to advocate for the principles of skillful treatment of childhood onset and non-motor disabilities. This is not just a pediatric issue, but generally only pediatric providers are taught to deal with the childhood onset problems. There are half a million adults with CP in the US, and 90% of CP patients reach adult life, and the incidence of CP is increasing, and adults with CP are living longer. Adults with CP experience a continuation of the childhood health care issues, and impairments in mobility, and dexterity, spasticity, and dystonia, communication, and swallowing difficulties. Adults with CP are experiencing pain. A majority, 80%, report pain. There's an increased risk of osteoarthritis in a number of studies, including one from Boulding et al. in 2005 reporting that 59% of 160 patients had signs of osteoarthritis. In another study, 27% between the ages of 15 and 25 were found to have arthritis in an age group where only 4% should have signs of osteoarthritis. This is just one hospital's experience with the current adult boom in CP. This is New York Presbyterian Hospital where we are based, and you can see that over a period of a decade, there was 168% increase in the 18 to 34 age group admissions to our hospital. My experience in China has been since 2020, and I'm working full-time to build CP venues for children and adults, and the problems are amazingly similar to the care problems that we experience in the U.S. This is a patient of mine, preterm, 29 weeks, normal intelligence. It's a graduate student, fluently bilingual, no treatment, no surgery, no hip surveillance, and since age 8, has not been able to stand. He's in a wheelchair for daily life and study and requires an aid, and the big issue is he can't transfer. So he's a GMFCS4, spastic quadriplegic. Mainly it's pain, though, that affects his life. Pain in his hip and his knees and muscle pain. So how do we approach this? Multidisciplinary, baclofen, gabapentin, diazepam, physical therapy, and then the surgeries, including hip surgery and other orthopedic procedures, to produce a result that we were desiring. Muscle tone was moderated, pain controlled, the struggle response modulated, and performing standing transfers at the end of this treatment. The temporal spectrum is an important context for this discussion. Chronic diseases like CP are not episodic. Rather, it's a continuum of care that should continue across all ages. This is important not only for medical care, but for research. CP does not end at 18. It is a lifetime condition. More collaborative efforts between adult and pediatric providers is really needed. In the U.S., pediatric physiatrists and pediatric orthopedists are trained in adult care. Should be obvious. So we need more courses like this. Drink your coffee. Convince colleagues that treating CP is a lifetime endeavor. Offer students and young physicians opportunities to experience the care of CP patients. Find the adult providers in your system who don't know CP, but who would be interested in developing that expertise. And engage in local advocacy that can build the capacity in your colleagues in your department and in your hospital. This is a 32-year-old woman with this preterm at 28 weeks. She stood with support at age 2, had surgeries as a youngster, and walked with minimal support until about age 14, when she started losing ability. At age 28, she underwent five mesenchymal stem cell injections, which did not help her motor function. No medications, no Botox injections. She's at home, well supported by the family. She was seen by physicians and had hip x-rays. Hip x-rays from 2091 were relatively normal, and in 2000 as well, but despite the normal x-ray, surgery was performed. Acute deterioration occurred over the last few years. She lost the ability to ambulate with assistance, with pain in her right hip and thigh. So now we're in this situation. Fixed pelvic obliquity, adduction of the right hip, fixed external rotation of the right hip. She can no longer stand without assistance. Adult patients deserve the same evaluation that we give our pediatric patients. This evaluation revealed limited hip motion was on the right, was likely the cause of her deterioration. This video clearly demonstrates the problem. She can no longer bear weight at all on the right hip. This video documentation adds immeasurably to the visual assessment. She's not a candidate for 3D data analysis, but the video helps us to determine the problems and allows us to review it repeatedly. It turns out that her ethogenic extra-articular hip congestion that occurred in that surgery 20 years ago is the likely cause. The hip joint itself looks okay on MRI and CT. Pelvic obliquity is secondary to the hip pathology, and the kyphosis is worrisome, but that's not going to be treated right now. So surgery helped. Surgery on this 32-year-old adult. Better position, better motion. One month post-op, she's walking with assistance and is able for the first time in years to get from the floor to a chair on her own. It's the best function she's enjoyed in a number of years. So what can be done? We can correct contractures to improve range and function. We can identify and correct spinal stenosis. We can address spinal deformity. We can correct the subluxed arthritic and painful hip. We can address alignment problems and abnormalities in the lower extremity. Knee, coronal and sagittal alignment and patellar instability can be helped surgically, as can foot and ankle deformities. The adult CP algorithm needs to be changed, and it includes the fact that CP patients can and will respond to treatment. Every complaint is not necessarily due to CP. Treat the adult CP patient as holistically as you do the child. The evaluation of this 32-year-old woman with CP is routine for our center. Every patient is presented at a multidisciplinary conference. At the first evaluation, don't assume that routine medical evaluations have been performed. At the first visit, question if the diagnosis is correct with genetic screening done, even if it's an adult. Consider if adult lumbar stenosis or cervical spinal stenosis may be responsible for the deterioration in the function that you see. Even in patients in mean surgery, see how medications, real rehab, and equipment can impact tone and function. Time and effort need to be expended to convince patients and caretakers that regular monitoring and surveillance are essential in adult CP, just as they are in children's care. We need to use the existing system to provide the care that adults need. The capacity is there. The knowledge and the expertise are there. It's just not harnessed. It's not focused. The principle of continuum of care can help us answer these questions. A corollary to this concept of continuum is that, like all people with chronic diseases, people with CP deserve coherent, comprehensive, expert continuum care of the underlying chronic condition and all of their other health care conditions for their lifetime. Thank you very much for listening. My best wishes to you in the U.S. from China. And I want to introduce my colleague, Amy Kanaglia, PGY-6 Pediatric Rehabilitation Fellow at Columbia, who is going to talk to you about muscle and function changes in adults with CP. Take it, Amy. Thank you, Dr. LeRoy. I'm just going to share my screen. All right, so yes, I'm Amy, second year fellow at Columbia, and today I'll be giving a brief review of normal skeletal muscle structure and physiology and how these things can be different in individuals with cerebral palsy. So as we all know, CP is a disorder of movement, posture, and or tone resulting from some sort of lesion to an immature brain, and focusing really on just skeletal muscle very broadly, you'll see a reduction in voluntary activation, a failure of appropriate movement inhibition that can lead to the various forms of hypertonia and movement disorders that we see, and importantly to this talk, changes to the skeletal muscle structure itself. So before we tackle that last point, I just want to briefly review the structure of unaffected or healthy skeletal muscle. So the fundamental unit is the sarcomere, and we're starting on the bottom left of that picture, and this produces force through the interaction and specifically the overlap of the proteins it contains, actin and myosin. Sarcomeres are joined end-to-end in series, and they form myofibrils, which is now the picture on the bottom right, highlighted here, and these bundles of myofibrils form myofibers. These are the multinucleated muscle cells grouped together in that bottom right picture. These bundles of myofibers are joined to form muscle fiber bundles known as fascicles, pictured in the top right, and then finally you have the collagen-based noncontractile connective tissue, and this surrounds the muscle at many levels. So it's organized into the endomysium, which surrounds the myofibers, the perimysium surrounding the fascicles, and finally the epimysium surrounding the whole muscle. So an important feature of muscle is its extensive growth and regeneration capacity, and this is due to the intrinsic stem cell population known as satellite cells. So they're normally quiet, except when activated during times of muscle disease or injury, and their numbers, in fact, decrease with age or diseases characterized by extensive muscle regeneration, and they can change in response to growth or limb immobilization. The picture on the right is a cross-section of healthy skeletal muscle bundle, it has about a dozen or so muscle fibers, pink in color, you see their nuclei at the periphery, and then that little cutout is a close-up of some of the important muscle interstitial cells, the satellite cells being the ones we're talking about, pictured in gray, and they're located beneath the basal lamina of the muscle fibers near the vasculature. So now that we've reviewed the structure and components of healthy muscle, what does muscle look like in a person with CP? So first of all, we see abnormalities in skeletal muscle, including decreased muscle thickness, decreased volume, decreased muscle belly length and cross-sectional area, and a decrease in those important stem cells, those satellite stem cell numbers. In early development, the volume of calf muscle in children with cerebral palsy can be up to 22% smaller by preschool age, increasing to a deficit of 45% in young adults. In terms of increases, we see an increase in collagen and lipid content, and an increase in sarcomere length. So to demonstrate this, on the right, is a healthy myofibril, all the way on the right, and you see the sarcomeres, you see that they're normal in length. Picture on the left, E, is a myofibril in a person with CP, which has the same number of sarcomeres, but they're increased in length, and that leads to less overlap between the actin and the myosin, and therefore less force generation. To look more at the satellite cells, the studies, there were a few studies reviewed by Dianiti et al., who looked at satellite cell populations and muscle biopsies from children with typical development, and those with CP, they looked both at two different methods, both of which demonstrated a decrease in the number of satellite cells in CP muscle compared to their age-appropriate controls. The left was using flow cytometry, and they found a 60% reduced satellite cell population. On the right, they used immunohistochemistry, and found about a 70% decrease. And they used immunohistochemistry just briefly because, since children with CP have this extracellular matrix abnormality, the flow cytometry can actually be a little bit off, and you might not be able to extract as many satellite cells, so they did both and found similar results. So what do all of these changes translate to? So they're the clinical features of CP that we all know and are familiar with, decreased movement generating capacity, meaning decreased force, meaning weakness, spasticity, and even really muscle stiffness from a lot of that collagen and the changes that are happening in the noncontractile proteins, limited range of motion and contractures. And all of these, of course, we see in children, but they can become even worsened and even more apparent in adults with CP affecting their function. And this just demonstrates just more of the clinical, what we see clinically happens, the decrease in range of motion, the contractures, just a schematic of normal postnatal, normal and abnormal postnatal muscle growth. So A on the left, you have unimpaired muscle growth resulting from bone growth, both are growing at the same rate. You have an increase in myofibral length associated with adding sarcomeres, myonuclei, adding girth to the myofibrils, and that's that little insert on the left. And then you have normal range of motion shown on the bottom. B on the right is more typical of developmental disorders, such as CP, where you still have that bone growth, but the muscle does not match it. And now you have impaired sarcomere growth and they get very overstretched, seen in the picture there. And you also have this decreased cross-sectional area also compared to the picture on the left. So now you have a decrease in range of motion and eventual contracture formation. So for adults with CP, what does this mean? So children with CP reach adult life with substantial impairments in volume and functional capacity of these muscles, and they're at greater risk of developing age-related sarcopenia than typically developing individuals, translating to a loss of function at an earlier age. A 2014 systematic review by Morgan and McGinley found deterioration in gait for adults with CP begins early, relative to their unimpaired peers, and many studies reported decline as early as patients' 20s and 30s. And then overall, their results suggest mobility decline occurs in 25% or more of adults with CP, and these observations, among others, have led to the adoption of the term advanced musculoskeletal aging as a hallmark of the phenotype of individuals with CP over their lifespan. There are, of course, additional health problems to take into account when caring with adults with CP that Dr. Roy has mentioned and my co-speakers will talk about as well, but we won't go into detail. Similarly with treatment strategies, there are a number of them, many of which Dr. Roy touched on, and my co-presenters will soon speak about as well. These are my references. I'd like to introduce Dr. Azizi to talk about pain in adults with CP. So Dr. Azizi has a little difficulty with her slides, so I tried to share her screen. Let's see if I can help out. Dr. Azizi, you can see it, right? Yes. Just tell me the next, then I'll move your slide. Thank you, Amy, and thank you, Dr. Kemp. So my topic will be spasticity management for pain control in adults with CP, so mainly the focus is on pain. Next slide. I have no disclosures. Next one. This study, Health, Mortality, and Wellness Issues in Adults with CP by Dr. Turk, showed that health and functional status do not stabilize by early adulthood, and unfortunately, most clinicians are not that good in recognizing or managing these health issues, and that health outcomes in CP, they are related to the severity of CP and also to increasing age, and the most commonly reported problems are pain, fatigue, physical performance, and the musculoskeletal system, and again, few adults with CP engage in typical screening and health promotion activities. Next. This study, Aging with Cerebral Palsy and Other Disabilities, Personal Reflections and Recommendations, it's written by a patient with cerebral palsy, Dr. Gwen Jones. She says that all through high school and college, I experienced periods of massive fatigue and some muscle pain, but it never crossed my mind to see a doctor about it. During my pregnancies, neither of my doctors never ever discussed my disabilities or their possible influence on the course of the pregnancies and deliveries. Although I lived most of my early years with persistent fatigue, I had only occasional intermittent musculoskeletal pain. I thought I was home free until I turned 36. My pain became intense, pervasive, and persistent. For years afterward, I lived in a sea of pain. Every joint and muscle hurt. Doctors said, you can stand it, live with it. Turning 60 brought more high-intensity pain and severe muscle spasms that refused to abate with home remedies. Tests revealed severe degenerative arthritis in my spine. Sleep eluded me every night because of constant pain. Next. Different studies have shown that the most consistent health condition in a patient with CP is pain, and most of the patients report symptoms in the joint which is closest to the pain. And if providers are able to take a good history and do a good clinical exam, they will be able to identify the source of pain and also provide appropriate treatment for these patients. And spasticity, weakness, falls, or progression of contractures or deformities, they could be cause or effect, meaning that they can cause pain or pain can make them worse. Next slide. Also, it's important to remember that patients with CP, they have difficulty in reporting and describing pain due to different problems such as communication difficulties or intellectual disability. And that also applies to our pediatric patients. And the common locations for pain in CP patients in adults includes back, leg, hip, and cervical pain. Next. Pain has a greater incidence with spasticity and also in those with higher function. Women tend to report pain more than men. And overall, there's poor recognition and treatment of pain in adults with CP. And adults with CP tend to self-manage their pain. And also, it's been shown that traditional cognitive behavioral approaches could be helpful in treating their pain. Other symptoms associated with pain in adults with CP are fatigue and also sleep problems. And it's been shown that exercise relieves pain in adults with CP. And pain management, focused therapies, behavioral treatments, good sleep hygiene, medication, and exercise, they all could be helpful in treating their pain and also sleep. Next. This study by Flanagan and Dr. Gebler in 2020 showed in their study, which was from age 16 to 89, their inclusion criteria. It showed that pain was not associated with GMFCS level. So it showed that adults with CP, they have pain, but it didn't interfere with their activities. Pain increased with age and also was a predictor of lower quality of life. They used different scales, such as a brief pain inventory promise scale. And also, they used the Penn spasm frequency scale in their study and showed that self-reported spasm scores based on the Penn spasm was associated more with increased pain in contrast to clinical examination scales, which was modified Ashworth and Tardo. So this also, they showed that the summed summary of modified Ashworth after being adjusted for other factors, such as pain medication, was associated only with brief pain inventory. Next. I present two cases here. The first one, 55-year-old woman with spastic quad CP GMFCS4. So she's using power wheelchair for outside and long distances, even at home, but very short distances, she's using love strand crutches. She was referred to me in 2018 by Dr. David Roy. In that time, she had severe pain and had a history of bilateral hip replacement one year before. And she had run some recent imaging after her pain started and there was no concern on imaging. Next. So she reported sharp, pulling pain in groin areas and also in proximal thighs and quads and behind the knees for around two months. She had a fall episode before that. She just, that's what she remembered. There was no other triggering factors. She had muscle spasms and right leg crossed over the left leg at night. And she had contraction, which was interfering with her sleep. Her exam was very limited. I, as soon as I touched her leg, she was screaming in pain. So I couldn't do basically any exam. So I just was sure that there was no concern on hip imaging based on what they told me. So she received, before that, for her exam, the most of these patients, when they come, they're basically up in the air because of the spasticity and contracture they have. The hips are inflexed and very limited abduction. And it's interesting that our pediatric patients, they have tone mostly in knee flexors, but in adults, they have more tone in knee extensors than flexors and they have pain also in their quads. So if you see here, the MAS was four in knee extensors compared, which was more than the hamstrings and knee were extended in wheelchair and ankles were fused and gait, feet externally rotated, dragged feet on floor, very slow to take just two steps to bed. She received botulinum toxin injection, bilateral hip ID doctors, iliopsoas, hamstring and rectus because of pain, low dose. She still needs to stand and do transfers. And when she came back for follow-up pain and spasms decreased significantly, more than 75% after injection. And she reported that her legs do not cross and she's able to sleep on right side. This is something that to keep in mind that our CP patients have different goals, like for them, just being able to sleep on different sides at night or legs not crossing, it's an achievement. And she still comes back. She receives twice a year injection around those 200 to 300 units. And she's happy with that. Next one. The second patient is again referred by Dr. David Roy in 2018. And I just started here. 43 year old woman with spastic co-op CP GMF CS4. She had worsening of pain spasticity and function over time. She's using a walker at home and independent for transfers using a post. And in the past, she referred several times for ITP pump because of severe spasticity in lower extremity. But she was, she didn't do that. She was scared. And something here, I put medication baclofen and sertraline. Also important to remember that most of our adult patients with CP, they have depression, anxiety, and they take these medications. She has significant tightness spasticity. Spasticity and dystonia in legs, affecting hygiene care and pain in lower back and legs. Next. So physical exam, the same as the other patient, hip inflection, abduction, very, very limited. And knee flexion contracture, sorry. And MAS3 in hip adductors and four in quads. The same again, the quads are, they become more like kind of dead on MAS4. It's like rigid. It's very rigid and severe spasticity and also hamstrings. The hygiene was very poor due to limited hip abduction. And she has severe lumbar hyperextension walking with a hyperlordotic gait and crouch. She received injection botulinum toxin to hip flexors, adductor hamstrings, and also paraspinal muscles referred to neurosurgery for ITP pump. Next slide. Uh, when she came back, she had less pain in lower back and legs by 90% improved hygiene care. She's able to wipe after toileting. She walks and take steps. It's easier. It's not that there someone is holding them back when they want to take a step. And she, her own observation, not mine. She noticed significant decrease in lumbar hyperextension. I don't think so. More bed mobility and sleeps better. She receives PT at home, find it very helpful. She consulted several neurosurgeons and finally now she has an ITP pump. Next. So before that, I forgot to say for that patient, the hip abduction was so limited that basically I couldn't even use ultrasound probe. I barely could place my own hand. And I was thinking when it's so difficult for me, how difficult it is for a patient with CP to do their own ADLs and hygiene care. So in summary, pain is associated with lower quality of life. We should discuss the goals of spasticity and dystonia management with our patients. Sometimes they like some tone for function and consider the effect of decreased tone on function. I had a patient who wanted the tone to be able to carry a bag on her elbow and consider ITP pump for cases with severe spasticity dystonia who are in many muscle groups and also adults with CP, they need greater knowledge and understanding to be able to enhance decision-making processes about their own health. Next. These are my references. Next one. Next. And next. So the next speaker will be Dr. Hyekyong Kim. She's the professor of Rehab and Regenerative Medicine at Columbia. She's Director of Pediatric Rehab Medicine and Fellowship Program Director. And thank you, Dr. Kim, for playing my slides also. I have many different hats. So I have to share my own slide. I don't know where because I opened so many. I think I'm the problem maker now. Where is my... Oh, it ended up disappearing. Forgive me. I'm going to find out mine. Did I stop sharing? Right. Okay. Now I'm going to... Oh, so that was good. I'm sorry. I apologize. So I like to approach the spasticity management in different ways. I hear my colleagues' presentation outstanding. But meanwhile, I was having some different thought. And I think I want to change the topic title because I think we have to really think out of box when we treat the patient who we never experienced before. Well, we didn't have that much information. So I'd like to share some information through this talk. So trajectory of spasticity in people with cerebral palsy, lifespan care. This is my disclosure. And then we all know spasticity is velocity-dependent resistance. But the faster the passive movement, the greater the resistance of the muscles. So if you look at this video, you can see when I move slowly, the joint less resistance. When I stretch fast, I feel more resistance. So when the children with cerebral palsy, especially with the spasticity, we are focusing on a lot of spasticity management. And if the non-invasive spasticity management with the toxin injection, phenol injections, or oral medication braces and therapy, then we ask a surgeon to do SDR, ITV. And orthopedic surgery will address a lot of bone alignment problems. And then we absolutely want to work on the weakness. So basically, we are working on function and prevent deterioration of a function. No matter what, our patients just start to deteriorate at the teenager. So this little girl, I cannot see my, it's very interesting. So this little girl is two years old. She is unable to walk. But after we did the injections to left arm, left leg, she start to walk. Then six months later, she still is able to walk, but we did not repeat the injection yet. So we all know we are pediatrician. Children are growing. So we, I insisted that she needs regularly repeated injections. So she was coming every six months to get the botulinum toxin and or phenol injection together. So it's very interesting. I lost my, I have the same problem with Dr. Hurwicz earlier. So, this one is after the third time repeated injection. She looks like a normal child if you don't see the AFO. So, after then, I was very happy to provide all the services continuously. Now, I start to see adults with cerebral palsy. And then, this gentleman used to be GMS1 with hemiplegic condition, but he came to see me a couple of years ago when he was 62 years old. Now, I am really having trouble with my cause. But now, he is GMS3. So, what's wrong with this picture? Is his spasticity getting worse? So, I also learned from this patient who is referred to me when he was 18 years old. He was just about to graduate his high school. So, he was able to walk. He's left the triplegic CP, but he was walking with very severe crotch gait. But he was able to walk without difficulties. He graduated now. For 10 months, he was home without any participation, any therapy. And then, look at him. Now, he's falling into GMF. He's going to be 3 or 4 very soon. So, is his spasticity increasing as they get older? So, I was paying attention to ultrasound findings. So, GMS5, spastic quad, 3 years old. You can see muscle pattern, quality of the muscle on the left side of the picture. It looks great. Right side, 23 years old, spastic quad, GMS5. It's very fibrotic, very white. I cannot really see each different muscle pattern at all. So, there's some problems. Is spasticity really progressive with aging? Then, my question was, or are there any other causes for increasing spasticity in adults with CP? We all know pain increases more spasticity. We know that they have early onset arthritis, muscle stiffness, neuropathy. But if you are dystonic CP, 8 times higher frequency of cervical myelopathy. And then, we may underestimate it or undertreat the dystonia or rigidity, which I found a lot nowadays. Dr. Aziz mentioned that the quadriceps or other muscles are very rigid, she said, but she was treating spasticity. In new onset stroke, they have more than twice high frequency of stroke compared to the people with non-CP. And medication also can increase spasticity or increase stiffness. I learned this one very hard way from Dr. David Roy, even though he's an orthopedic surgeon, he's so wise and smart. So, he was able to work with psychiatrists and we started to learn. A lot of adults with cerebral palsy, showing increasing use of psychotropic medications. Among SSRI, Prozac increases the motor side effect. So, you can switch to the other type of SSRI. Then, let's take a look at spastic muscles. Acute, which means when they were young, we took a look at the ultrasound findings. We do see reflex-induced resistance, which means there is still active contractile tension. So, we also see exaggerated tendon jerk, we see clonus. But when you take a look at 60 years old spastic quadriplegic CP, chronic condition of spasticity, you don't see that much reflex-induced resistance. And I like to explain that with rheologic properties, rheologic phenomenon. So, for example, when you pour the starch in the water, you start the water with your chopstick. I'm Asian, so I use chopstick all the time. Let's say I start the water with chopstick, then starch is not going to become hard. But you forgot, Dr. Kim is calling me, let me go. You disappeared five minutes, you came back and you look at the water, starch is there and very white and very hard. You cannot start anymore. That happening to the muscles. So, if you don't move your muscles, there is rheologic phenomenon is going to happen. Therefore, patients start to complain more stiffness, actually. If you ask adult patient, they don't really say, I have spasticity. They say, my muscles are very stiff. So, that I can explain with the rheologic phenomenon I learned from Dr. Meyer at Moss Rehabilitation, as you see the reference here. So, they have a stiffness, contractures, fibrosis, and atrophy. And then I studied a little more about the immobility. So, immobility increases the hyaluronic acid, which is a high molecular weight, glycosaminoglycan, which is the lubricant for the muscles. And then increase the hyaluronic acid actually decrease the lubrication ability. Therefore, muscle stiffness gets increased, contracture increases, fibrosis, and atrophy increases. So, we have to think a little different ways, how we can prevent their stiffness based upon what we learned today. So, we also learned from Amy Tenelia that they have all the psychopenia. And then the psychopenia happens as early as the 15 months old. So, then I shared this information. We have a 78 adult with the CP. When I have a referral from orthopedic surgery mostly, they are asking us to do spasticity management. If I ask patient, they said, please treat my pain. So, pain is number one, spasticity number two. And so, I was reviewing my dose of toxin. Surprisingly, I was using lower than recommended dose or compared to children because they start to experience weakness. So, I actually decreased the dose of the botulinum toxin. Meanwhile, I was reviewing goal achievement. Actually, goal achievement was very high even though I was giving lower dose of botulinum toxin as you see on this graph. So, and then there are very handful of botulinum toxin in adult with the CP, very few. And then I was able to find only two. There's a prospective study was done to see any gait pattern is increasing. There's no significant difference in kinematics and health-related quality of life. And then when they did multilevel botulinum toxin injections, there's no improvement in GDI. So, why we are doing botulinum toxin injection to the adult with the CP? So, proposed chronic spasticity versus stiffness management for adult with cerebral palsy. So, before you decide to do chemoneuralysis or botulinum toxin injections, you have to have your checklist. Check any causes for increasing spasticity as we discussed. And you should be able to differentiate spasticity from stiffness. And you should check, is there any undertreated rigidity or dystonia? I start to use a hypertonia assessment to more than before. And then I witnessed that adult with cerebral palsy has much more rigidity and dystonia than spasticity. So, chronic spasticity management, we have to think out of the box. If they have spasticity, you have to treat with the chemoneuralysis and botulinum toxin injections. If they need a bony malalignment, causes a lot of functional problems, you have to recommend orthopedic surgeries. If they need to have a diffused dystonia and spasticity, absolutely, we have to recommend SDR or ITV. But what if because of immobility, they have severe stiffness, increase muscle mobilization, you may consider to use a higher hyaluronic base to decrease hyaluronic acid. And then if they have rigidity, you should treat with dopamine. I use amantadine to improve their attention span. Several patients I had to cancel the botulinum toxin injection after I started amantadine to control their rigidity. And then I'm even considering some intrathecal bacterial pump placement. Actually, some patients show more rigidity, even though we place the pump, their stiffness didn't get better than much. And I'm assuming that based upon my examination, it was rigidity rather than spasticity. I start to actually confess to my patient that my assessment was not correct. And then if they have dystonia, you should treat dystonia. So this is my summary. Lifespan care for people with spasticity. Initially, you are treating a lot of real spasticity with all kinds of tools. But when they get old, you know, they have a depression because they have nothing to do. They become very fatigued. So you start to use psychotropic medication, but make sure to check the side effect. And if they have spasticity, treat the spasticity. If they have pain or increased spasticity due to arthritis, myelopathy, whatever they have, you have to treat the origin of the pain source. But if they have a stiffness from the rheologic properties, you have to have them move around, have them participate in all kinds of activities. And then you may consider hyaluronidase. And this is my last slide. But this girl, R.M., is this video actually when she was four years old. And then because of pandemic, I was doing injection twice a year. Oh, my strength is crazy. But anyway, I was doing injection twice a year. And during pandemic, she now she's 12 years old. So mom was asking me, what can I do for her? So I said, you know, what can we do? Let's do dance. So then she said, no way I'm going to dance, right? I'm looking for my cursor. It's very interesting that I cannot see my cousin. I don't know how I can get to this stuff. Maybe I have to just escape or forgive me. I am unable to find my maybe go to the actual YouTube video. No, no, I can't. I have to stop sharing because I cannot. Oh, my goodness. OK, good. Somebody did it for me. So I'm going to if you don't mind, I'm going to share actual video. So she was having actually orthopedic surgery, recommended the surgery, atlas tendon lengthening or soft tissue release. But I said, why don't we do gait analysis? Actually, gait analysis didn't show that much problem, bony malalignment. So and they said, why don't you repeat the botulinum toxin injections? But meanwhile, she started to dance. So let's take a look her dancing. Are you guys seeing my? Yes. OK, good. So she started to dance. So that was my last slide. So you can see the mobilization was extremely important. So I my prescription is a dancing number one, even though they don't walk. I said, let's start to dance. So this is my last comment on how we're going to take care of adult or people with cerebral palsy. So I think we'd like to bring the audience to the floor with the questions. Amy, would you moderate the questions? Yes, absolutely. So we've had a lot of good comments and questions in the chat. Thank you, Dr. Roy, who's been answering them as we go. So the first question we have, which Dr. Roy answered, but I'll read. Dr. Roy, what advances in genetic testing do you find yourself revisiting the initial CP diagnosis for adult patients often with advances? Excuse me. I don't know if you want to comment or I'll just read your answer. Just that it's not often, but it's enough to be for him to want to be precise in every patient that he treats. He wants to know whether there is a generative or progressive component. And at this point in our development of understanding of neuromuscular conditions. OK, the second question we have, any role of CoQ10 in adult CP? If any of our panelists know. All right. One person did comment that in their practice, they did start CoQ10 in four patients with CP admitted for central stenosis and it was effective, but an NF4. Someone else was asking about the indication or mechanism for CoQ10. And the same person had commented no medical literature in CP, more neuromuscular medicine. But in the four patients that this person was talking about, they had complained of muscle weakness and pain. No critical illness, myopathy or polyneuropathy on EMG. So decided to start to help with muscle pain. I did a very brief literature search. This is not something I was familiar with and did find it used in more cerebellar ataxia. There was a paper that I'd be happy to share. One study said ataxic CP was the most common neurologic phenotype associated with CoQ10 deficiency, but there wasn't too much there. Anyone have any other thoughts or comments or I'll move to the next question? No. OK. Dr. Roy emphasizing Dr. Azizi's points that adults can be treated, helped and become more functional. A few comments on the challenges in systemic barriers of transitioning these patients from the PEDS to the adult world. And perhaps the solution is PEDS rehab docs running the adult CP clinics in the adult world. And then someone posted great resources. There was a CNS community session in October on transition of care of these patients and how adult physiatrists can be well positioned to care for these patients. Another model of care. So there's a lot of resources in that talk for anyone interested. That was by Kristen Caldera. Thank you. Let's see, skimming on down. Dr. Roy emphasizing that the rehab world may be better equipped to deal with the holistic care of the adult CP patient, but having a close relationship with the specialty surgeon is very important. Another question. What should I tell my patients are the long-term effects on muscle properties due to repeat botulinum toxin injections? Do we have enough info research for an answer at this point? I will answer for that. I don't know. We do not have. So that was my questions all along while I'm preparing this talk. So actually when I use botulinum toxin for adult with the CP, number one, absolutely. If there is a significant spasticity, I'm treating the spasticity, but because of the muscle properties, you can imagine that there's, because I'm using ultrasound, I see the quality of muscle. Then I'm sometimes I'm hesitant to do botulinum toxin injections, but the other side, botulinum toxin works as a pain medication for those patient population. Whenever we do injections, they get better in the other way. They can relax their muscle, but at the same time, absolutely it controls pain. So my answer is, I don't know what's going to happen to the spastic muscle or fibrotic muscle at this time. That's why we needed to do more research on muscle. I have a comment. So my comment is that there's the natural history of CP is pretty terrible when it comes to muscle function and the microscopy or pathology of muscle. Sarcopenia, the replacement of the interstitial substance of muscle with fibrosis is part of the natural history of CP. And so it's so hard to determine, and in studies that have been done so far, what is natural history and what is related to our attempts to treat spasticity with botulinum or similar medications? The answer is not there, but right now for me, I have the patient in front of me. I need to relieve pain. I need to relieve spasticity. I need to improve function. And I know that Dr. Kim can do that with Boson and Doxan. I don't actually do the treatment. So as an orthopedic surgeon and as someone who does treat adults holistically, it should absolutely be part of your thought process. And maybe not every 12 weeks, but every six months or 12 months, honestly, I can't even imagine we're doing harm to a patient when we can see the therapeutic result. Anyway, that's my opinion. And I add something. These adult patients, if the question was that how we, if we should worry about their muscles, sometimes they just, they don't have any muscles. Basically they have had a doctor's tendon release, and then they come back with hip abduction of 10 degrees of 20 degrees, very limited. And then when you look at the muscles, it's as someone mentioned, it's all fat. And then the muscle is like kind of hazy. It's not a very healthy, good muscle. So basically the muscle is not healthy for us to also worry about losing the muscle. And still as Dr. Roy mentioned, I did Botox for the same patients that who's was very, they don't have much muscle left, but still it helps with them. It helps for the pain. Thank you guys. We may only have time for one last question. A couple of people asked about hyaluronidase and how is that administered and how are you able to get it covered? We, I never done it. So Dr. you guys know, Dr. Raghavan who moved to Johns Hopkins, she was doing research with the hyaluronidase and Dr. what's her name? She's in Hawaii. Oh my God. Tamara. She does use the hyaluronidase for people with the cerebral palsy, but I don't know how she does that. I think we have to do more study, but this is going to be very, very helpful for adults with CP who is not, who are not responding to toxin or baclofen, anything. Great. One more minute. Maybe, maybe one more. I've sent so many of my patients to dance or martial arts, but the families do not believe me. What do you tell them to do? I think you just learned today what you have to say. If they don't move, they're going to be on wheelchair. That's the way I said. Martial arts is like fantastic, fantastic exercise program for people with disabilities. I totally support it. Great. Any comments on how to partner with communities to build these programs to be accessible? Oh my God, that is my question. I think we have to go to the to the government to achieve this goal. That has been my dream. Jessica, I think if you could mute, thank you. Yep. I think we're almost done. I think we're at time. I don't, but thank you everyone for these amazing questions. They're such great chats and people answering questions in the chat. Thank you. Thank you so much, everybody. Hopefully, yeah, I think we have to leave because time is up, but we appreciate your participation and hopefully you got something and we can help our patient population and we fight for our people with the cerebral palsy to the end so they can have the best quality of life. Thanks a lot. Please enjoy your rest of conference. Thank you so much, Dr. Roy, and you can go to bed now and then you influence us so much and because of you, we are able to provide this conference. Thank you so much. Bye bye. Bye.

musculoskeletal care

cerebral palsy

aging patients

spasticity

pain management

early intervention

treatment

research

holistic approach

challenges