Kade Sigford, MD, who specializes in pediatric physical medicine and rehabilitation, offers keys to understanding CP in infants and toddlers, including common misconceptions about the condition and a look at how the current definition is evolving. They present diagnostic steps and features of classification, such as the type of abnormal movements seen and using scales of functionality. They also share points to make when educating worried parents, ranging from the child's disability remaining fairly stable for life to the availability of pain-relieving treatments to the importance of treating kids with CP like other kids.
I I would like to talk to you today about one of the diagnoses that is core to what I do as a pediatric rehabilitation physician, and that is to take care of kids with cerebral palsy. I actually I'm going to start back up just a tiny bit to start out this talk, uh, even before cerebral palsy. Because I know that a Physical medicine and rehabilitation actually is a Actually is a specialty that is missed in some medical schools and even occasionally in residencies and so there may be folks out there who are not terribly familiar with what we do, especially the pediatric physiiatry end of it and Physical medicine and rehabilitation is a specialty where we focus on function more than any single body part. We are largely a musculoskeletal discipline, a musculoskeletal neurologic discipline. We really don't own a particular organ system. We help people after injuries, after illnesses, get back to their previous level of functioning from PM&R come come some specialists in sports medicine, in traumatic brain injury, in spinal cord injury, in pain. Um, and in pediatric rehabilitation where we do a little bit of all of that, um, and where I'm coming and where I'm coming from today, um. One of the core things that we take care of in pediatric physitry is cerebral palsy. That is a very common diagnosis. We also see a number of kids with spina bifida, some congenital and acquired amputations, kids after various trauma or severe illnesses. a random aside, I'm about to have 3 people admitted to inpatient rehab right now after electro scooter incidents, so Like, tell everybody to wear a helmet and be careful, and I'm really down on those right now. Um. I have no, uh, for disclosures, I have no conflicts of interest, though I will discuss off-label uses of medication during this presentation, um. By definition, cerebral palsy is a group of disorders of the development of movement and posture causing activity limitation that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain. This is a diagnosis or this is a definition that was agreed on that was proposed initially in 2005 and has been in use ever since. There is actually a working group right now working on revisions to the definition. One of the major things that has happened since 2005 is a lot of advances in genetic testing, and so there are questions about So there's questions about can it be cerebral palsy if there's a genetic cause. Now, this definition has a lot of big words that sometimes can be hard to That sometimes can be hard to understand, um, particularly when I'm trying to talk about it with a family who may be not listening as well as they could because they're worried about what, about what their child is getting diagnosed with, um. I do start with a version of this definition, but then try to talk about the the pieces of individually. Uh, first, uh, one of the key points is that it is that it is a non-progressive. Condition. The differences in the brain that have led to the movement limitations are stable. They do not change. Perhaps the brain had perhaps the brain formed with polymicrogyrhea or schizophaly, or perhaps there was an injury during development. There was an early stroke or a very early traumatic brain injury. And we can now see the results of those on the MRI, but those changes are not going to evolve. There's a small caveat to cerebral palsy being a non-progressive. Condition and that is that the brain itself does not change or does not change rapidly, but if the musculoskeletal components are not adequately managed, then function can be lost over the course of time. Lifespan work does suggest that There may be some, there there is probably some accelerated aging both cognitively, mentally and physically in folks with cerebral palsy, though the full ideology of that is not yet clear. The second part is that this is something that happens while the brain is still developing, which is generally felt to be up until about the age of 2, so kids can be born with these differences in their brain. And be diagnosed with cerebral palsy, but also something have something happen to them before about the age of 2, and then their development is more parallel to somebody who was born with a difference in their brain than somebody who has a traumatic brain injury at a later. At a later time A then then the last thing that we talk about is the difficulty with movement and posture. That's actually probably something the parents have already noticed and maybe why they're seeking and is usually why they're seeking care. And so in some ways it is the easiest part of the whole discussion for the parents to accept because you can, because you can say this is what you've been seeing in your child and When I say they have cerebral palsy, I'm naming what you have already seen. I'm not giving them a new problem. I'm just naming what you've already seen, um. That's kind of the simple version. I add the next slide is. All the footnotes, or at least a selection of the footnotes, because I had to snippet some on that. Definition which I don't expect any of you to read, but Goes on, but goes to show exactly how complicated it is, how complicated it is, um. What's not anywhere in the definition and what tends to and what tends to cause some A concern or confusion is that there is no There is not necessarily intellectual disability. Some people have intellectual disability and developmental delay. Some people do not. Some people have seizures, some do not. Some people have emotional problems and some don't. And I think the thing that most parents worry about is some way their child is not going to have a good or a fulfilling life, and that's not there anywhere, um. And so these are the things that Do not show up in the definition and that parents need to understand are not going to are not necessarily going to be part of their child's development. One of the things that Used to be much more common than it is today is the diagnosis of CPR CP. Cerebral palsy and mental retardation. Used essentially as a single diagnosis because they were felt to be so closely intertwined and I still see that I still see that occasionally in charting. Those two things need to be discussed, addressed, considered separately. Um, folks with CP can have normal intelligence, and those folks can get overlooked. Um The differential diagnosis is wide and starts with any other neuromuscular disorder, and this is especially true since infants with cerebral palsy, infants with cerebral palsy are often hypotonic no matter what the final tone is. Many, many will be hypotonic for the first few months of life, and So it is easy to see how you might see. How you might consider a neuromuscular disorder another. Other things on the differential diagnosis are a dopa responsive dystonia. This is worth. This is maybe worth knowing about. It's rare, but it is very easy to treat. You give carbidopa, levodopa, and essentially resolve the issue. It's a genetic condition. Otherwise also there's hereditary spastic paraplegia. Maybe the child's family will have some history. If you particularly if you hear about changes, consider spinal cord tumor, cyst compression, though there are infants who are born with. Who are born with spinal cord tumors have taken care of a A child with paraplegia secondary to a spinal cord tumor in utero that had actually involuted and resolved by the time she was born but had left its had left the damage behind. Um Genetic disorders. Including trisomy 21, Angelman syndrome, um. And Metabolic disorders like a mitochondrial or a storage disorder, um. What So what do we think about in terms of working on diagnosing cerebral palsy? The first thing to think, the first thing to check is to confirm that the history doesn't suggest a progressive or degenerative central nervous system disorder. Remember, I said cerebral palsy is a non-progressive condition and so any condition that has progressive characteristics, something else needs to be something else needs to be considered. Um, we have one person, I have one person I'm following now who is, who's, uh, diagnostic workup has essentially restarted. She was diagnosed with CP, has now made it to her early teens. And it is very clear that she is now showing some degenerate some characteristics of a degenerative disease and so the workup has restarted again and it looks like she may actually have a brain iron accumulation disorder. Then Uh, then you can classify the type of CP. And screen for associated conditions. If the child had previous neuroimaging or laboratory studies that determined the etiology of CP, then you're set. If not, then you attain a neuroimaging study in which an MRI is preferred to CP. This is a practice parameter. For older kids, there is new work in, uh, there's new work in early diagnosis of CT using a number of different tools, including, uh, the Hammersmith neurological exam and the general movements. Assessment that allow Diagnosis or at least suspicion of cerebral palsy down into into infancy with in some cases a fair degree of accuracy. It used to be that we said, well, cerebral palsy can't be diagnosed until at least age 2. That's not true anymore, and The earlier we can get kids in, we can get kids diagnosed or and in for services, the better. If it can't be diagnosed conclusively because we're still waiting on things, but the child has signs, then they can be then they still need to get into services. Common comorbid conditions that you might look for include sensory impairments, cognitive impairments, psychological impairments, and epilepsy. You can see there that epilepsy is much, much more common in folks with cerebral palsy than in the general population. visual impairments and musculoskeletal disorders. Most of the musculoskeletal disorders are acquired as a condition as a consequence of the abnormal tone. Um, so now if we want to talk about Cerebral palsy. How do we classify it? There are a few different ways that you can classify it. The first is by area of the body affected. The second is by type of tone, and last is by functional status. Most folks are probably quite familiar with the different terms we use for the areas of the body affected a hemiplegia, uh, a diplegia, or a quadriplegia. Um, monopplegia is a rare, but you occasionally see it, um. One arm or one leg. You can classify by type of abnormal movement, uh, whether it's spastic, which is a pyramidal abnormal movement, or dystonic, ataxic or hypotonic. To define those a little bit further, um, spasticity is defined as resistance to externally imposed movement. That increases with increasing speed of stretch and varies with the direction of joint movement. OK, so again we have a lot of very fancy words there. I usually, I usually summarize it as velocity dependent resistant to resistance to stretch. Parents will often talk about their child seeming stiff or rigid, plasticity actually is a different characteristic than rigidity or maybe tight, and This is one of the places that I think language actually that language is important because it's easy to talk about tightness for spasticity, but also for contractures where the muscle is just too short and therefore the joint can't be stretched beyond a certain point. The treatments for both of those things are different and So it's important to figure out what is the bigger functional problem, what is the bigger concern before embarking on a treatment course. The Next type of abnormal movement is dystonia, which is involuntary sustained or intermittent muscle contractions causing twisting and repetitive movements, abnormal postures, or both. This can be, this is often triggered by a tempting voluntary movement or by emotion. It varies in presence and severity over time to a much greater extent than spasticity. So here you might hear the parents coming in and might hear the parents telling you that their child seems really seems loose at night. And is easy to move and is still fairly loose and easy to easy to move and doesn't have too hard a time when they're calm and quiet, but as soon as they try to do something, their muscles tighten up or When they get mad, they get really, really stiff, and it's almost always mad you hear it's almost always mad you hear first, uh. Any strong emotion can trigger it, but it seems like kids are, uh, especially young kids are more inclined to get incredibly mad easily than incredibly, uh, happy and excited, um. Another one is ataxia. This is a lack of voluntary coordination of muscle movements. Things that you can see are, you know, obviously the clear in coordination of voluntary movement. Also dysciatic cokinesia, which is impaired rapid alternating movements, intention tremors, reeling or shaking of the trunk or head, staggering or stumbling or a broad-based gait. I actually took this set of descriptors of ataxia from the A from the definition that California Children's Services uses to Determine eligibility for medical therapy program services. It's not. So it's not necessarily authoritative in a huge sense, but it is this set of descriptors that they use, and it's a pretty decent one, particularly considering that much of the time. We Talking about these things, we are also looking into whether we can get kids' services through California Children's Services medical therapy program. Um The Next way that you can talk about classification of cerebral palsy is by function, um. There are a couple different ways. There are a couple of different scales. One is the gross motor functional classification system, which talks about the child's ability to perform gross motor functions. This is fairly stable throughout a person's life. There are age band. There is an expanded and revised version that gives you classification by age. You can see I selected for this particular slide illustrations between the 6th and the 12th birthday. Um, at which point kids are large, they're not quite up to their necessarily up to their full adult gross motor function, but getting real close, um. So somebody who is a GMF CS1 can run and jump, can climb stairs without a railing. Uh, down through somebody who's a GMFCS level 5 who is who uses a wheelchair, who will use a wheelchair for mobility in all settings. They might be able to manage a power wheelchair, but their motor control might be too limited even even for that. They need significant support in their wheelchair to remain upright and in a good position. A GMFCS is relatively stable during life, so Folks generally don't change a whole lot. The the biggest exception is in is in very early childhood. The earliest age band is from 0 to 2 years, and if you just think about how much motor development occurs between birth and 2 years of age, you can see why it's real difficult around you know 6 months, 8 months to figure out exactly where someone is going to go, and they might very well jump levels. Just because they learned to walk. Um, So while this is not intended as a prognostic tool, the fact that it is relatively stable throughout a lifetime is a way to have some idea of where a child that you're seeing around the age of 4345. will end up Um, the other exceptions to this being stable are if function is lost due to contractures. Sometimes growth can be a real problem because kids who are just barely getting by because their muscles are a little weak and hard to develop. Get too tall and too heavy and those weaker muscles that don't gain strength well can't keep up. Sometimes some of our surgical interventions. Can help move people maybe one level, uh, maybe one level, but more commonly just makes it easier within the same level. Um, parents are always coming and asking, how can we how can we get this person to not need their assistive devices, to not need their braces, to not need to be able to run and jump and keep up with their peers. And unfortunately, the answer for a lot of cases is that we can't. We can make things easier. But we can't make huge functional changes right now um. Another major classification system is the manual ability classification system. This is defined motor the same way that the as a GMFCS is the gross motor. So how So how are the child's fine motor skills? Can they do Ah, can they do pretty much anything? Maybe a little bit tougher but can do pretty much anything or require total assistance or anywhere in between. Both of these have Self-guided helps for classifying children. In fact, the GMFCS even has a, has sheets you can give to the parents to ask them, and they circle what they notice their child can do, and that helps you to figure out what their what the level is. A So that's a lot of background. What else is important, um. For Community providers and primary care providers, particularly, I know in some areas it's hard to get access to specialty care because we're just a few people in a major medical center a really long way away. Kids with cerebral palsy, especially non-ambulatory ones, have a very high risk of hip hip subluxation. Uh, kids who are ambulatory at GMF CS1, so the ones who can run and jump. Really very seldom have problems with their hips and GMFCS2, uh, who can walk but may need a rail on stairs and may not run well, uh, also generally do well on their hips, but anyone, uh, Anyone with less gross motor function than that has a very high risk of Hip subluxation. Things that should raise concern that something is going wrong is a passive hip A deduction of less than 35 degrees, a hip flexion contracture of greater than 20 degrees, um, hip pain. There are multiple monitoring schemes published. All are fairly similar and reasonable. A great place to start looking at this if you want to, if you want to know about some more about it is at hipscreen.org. Shriners in Sacramento, their orthopedists put this together and it also, it actually has an app that I spend a lot of time recommending to people. Because the app contains the Monitoring schemes, so frequency of monitoring it contains the guideline to how to figure out what what uh GMFCS level someone is because your monitoring is going to depend on that. It includes the information on what to tell on exactly how to tell the radiologist to get the X-ray that you need, so the view is exactly right. Oh sorry, uh, the radiology tech, um, and it includes a really easy to use tool to measure migration percentage on, on your own, um, and it's good enough that I periodically catch our pediatric orthopedists here using it instead of using fancier tools to do the measurements, uh. In General, if you're going to start looking at this, uh, a referral to ortho is absolutely indicated if you have any concerns, and certainly if you catch a migration percentage that exceeds 30% or that is changing or that is changing rapidly, um. This next slide is one of the folks that I, is one of the folks that I follow and you can see that the right, just as an example, and you can see that the right hip is doing fairly well. We've got a We've got a decent, a decent though not great angle on that, uh. Femur femoral neck to shaft angle, and the head of the and the head of the femur is really pretty well seated in the acetabulum. On this other side, this angle is much It is much shallower which directs the head of the femur. Up and then leaves it much less well covered. So those are a couple of things that you Might that you might notice. Um. And if we had the, if we had the hip screen app up and running, uh, it would be the work of a minute or two to actually measure out these migration percentages, um. Next, here is a sample of one of the sets of hip surveillance guidelines, the Australian hip surveillance guidelines, um. The American Academy of Cerebral Palsy and Developmental Medicine has also put out one. There's a set of guidelines. There's a set of guidelines from Sweden. They are all really pretty similar. I have a tendency to use the Australian hip surveillance guidelines partly because they were around before the American ones and so they are the ones I memorized. And partly because their table is just easier to read, but it doesn't really matter a whole lot. Another thing that is Another musculoskeletal condition that is Very prevalent in folks with cerebral palsy is scoliosis, and So that's something else to keep in mind to watch for, to screen for and potentially do X-rays for if you have any concerns. Again, this is ultimately something that you're going to need help from a pediatric orthopedist to manage in the long term. Different orthopedic practices have different preferences on exactly when they like to get people referred, but somewhere between 20 and 30 degrees for sure. If not, if not earlier. Um No, that's all, that's all that's all orthopedic stuff. Where do, where do we come in as, where do we come in as physiatrists? Well, a few other places. One is, uh, in terms of managing tone, um, there are a lot of ways to manage tone, but I guess the first question is, is why, um, spasticity, dystonia can lead to first pain, in which case it's absolutely imperative that we figure out how to manage it so the child doesn't have pain. Second, can lead to difficulty in function, and 3 can lead to difficulty in caregiving. It might be relatively easy to take care of a small an infant or a small child who has very high tone, and the parent is easy, is able to gradually get their legs apart to change the diaper or stretch their arms out to put the shirt on. But once that child gets up to 1516, 17, It takes a lot, it takes a lot of hard work and a lot of strength to do some of these things. So those are kind of the three things I think about when I'm asking the question of whether or not to treat tone. Um, spasticity is probably the easiest of the of the abnormal tones to manage. There are multiple options. Um, baclofen is available orally and is first line for generalized tone management. It's been real nice over the course of the last couple of years that they finally come up with a commercially available liquid product as opposed to having to have it compounded all the time. It's also now available. As granules that can be kind of like Depakote sprinkles can be used the same way as that. Um, Other medications that are used are the benzodiazepines, uh, predominantly Valium or uh diazepam and lorazepam. A So, uh, diazepam and clonazepam. Since a lot of kids have a comorbid, uh, epilepsy, you'll also see a lot of folks with CP on clobazam, given for their seizures. But it's always you always have to keep in the back of your mind that this clobam is a benzodiazepine and probably treating their tone. Every so often I have a parent come in and say, God, My kids' tone is worse all of a sudden and I can't figure out what's going on. chart review then shows that their neurologist has changed seizure medications and they're off their clobazam. And it turned out it was controlling the tone, and nobody thought about that. Clonidine and tizanidine can be used for tone. Tazanidine is the traditional medication. It is the more traditional of the two, the alpha agonist, it has a little bit, uh, Fewer cardiac effects than clonidine. Clonidine has the advantage of being much, much more commonly used for other things in pediatrics, so it's a little easier to consider pediatric dosing, to consider how it's going to affect kids. And then dantrolene is the other major medication that is used. That one is unique in that it that it works at the level of the sarcoplasmic reticulum as opposed to the central nervous system as all the others and worth remembering about that one is that there is a risk of a phytotoxicity. That never goes away. So as long as somebody's on dantrolene, this might be a problem, um. Once you get outside of the enteral medicines, the botulinum toxins are injectable, and first line for local tone management. This is less and less an off label use as they get more and more approval in pediatrics and. When I initially put this this talk together. None of them had pediatric approval. Since then. Many of them have gained pediatric approval for certain muscles or certain parts of the body. Um, then you can give, then surgically, there are a couple of different things you can do a baclofein pump to give baclofen into the intrathecal space if oral administration doesn't allow for high enough doses, or uh the neurosurgical procedure called a Selective dorsal rhizotomy is the main one to permanently reduce or eliminate spasticity in that the dorsal nerve roots are. Some selection of the dorsal nerve rolets are sectioned. To to eliminate the aberrant connections that lead to spasticity, um. Then dystonia is sometimes Dystonia can be harder to manage. It doesn't often respond to medications quite as well, but You'd use the same, you use the same things as spasticity, but then also trihexaphanidyl and carbidopa Levodopa are also commonly used. There are a number of less common options, but at this point, even I move on to consulting a movement disorder neurologist, um. You know, but And then finally, the last thing that I think gets that I think gets missed. Of and and I see it periodically in families of the kids I take care of is that kids with CP are kids. Um, they need their chance to have fun. They need love, they need chores, they need rules, just like any other child, um. Parents will sometimes want to Treat their child with CP differently because they feel bad, um. And I've met a number of, I've met a small number of Really difficult teenagers because they've gotten away with everything in their life and up until that point. I've also met a relatively large number. Of kids who are more physically impacted than cognitively impacted and so it didn't take them very long to learn how to look as pitiful as possible and try to use that to get there and try to use that to get their own way. So that's also something I talked with parents, I make sure to talk with parents about is that uh. Just like any other child, make sure that you have rules, make sure that you have limitations, make sure that you have chores. Maybe there's not, maybe it's hard to come up with things, uh. They are easy to do, but everybody can have, everybody can have some sort of job or some sort of way to participate in the family. Um And I think the last, not on this, not on this slide, but the last thing is that. As medical providers, we are not necessarily great judges of somebody's quality of life, um. The parents are general. I find parents are generally very good judges of their kids' quality of life and can tell me. When their child, when their child who is non-speaking, who doesn't have very much communication and ability. is still having fun in their life, is still enjoying more things than they're not enjoying. And they can tell me when that balance tips. So that we, so that we need to work on, uh, so that we need to work on how to improve that. Um These are the, these are the references, that's the That's the formal end of the talk, so we'll move over into questions at this point.
