University Place  Michael Ward Cerebral Palsy: An Integrated Approach  March 1, 2014 UP989   cc >> The topic I'm talking about today is really looking at a definition of cerebral palsy and discussing the complexities of this condition and how we then direct care. And I call it care through an integrated approach, meaning that with the issue with the phases of cerebral palsy it's sometimes challenging to get a perspective of what the whole person is about. And to do this, we have a lot of clinical work in teams at the Waisman Center so that we can integrate the team together to address many different aspects of the questions and challenges that people with CP may have to approach the problems then again from a more integrated and holistic direction. So we're starting, and what I'm doing today is complementary with a lot of the other topics that people are talking about, but my focus is on the definition of cerebral palsy and why we choose to address care for cerebral palsy at the Waisman Center with multidisciplinary teams. This is a little schematic of the teams we have actively today. The dark red are disciplines that are present in our cerebral palsy clinics. We have nursing and physician either in rehabilitation medicine, which is what I do, or developmental pediatrics, which is the other specialty that works with the developmental disabilities. And we have PT, OT, and speech evaluations generally in the cerebral palsy clinics. We have other services at the clinics that may be available to individuals at the Waisman Center but not every time in the clinic. And then we utilize a variety of other specialists at the American Family Children's Hospital as needed. 

And then, of course, community resources and we try to integrate that as possible, again, to get information and develop treatment and life plans for the people at the center of the multidisciplinary team. So the first issue is the question of what is cerebral palsy. And that actually is a very complicated answer, and I'm going to spend about 15 minutes now talking about that. And hopefully you will come away with a fairly fleshed out awareness. We interviewed a candidate who is a gentleman, a physician with cerebral palsy who had congenital left hemiplegia, and his answer on this obviously was I don't really know what cerebral palsy is. It's a person who's lived 30 years with what we would call cerebral palsy. So it's not a simple question. This is the modern working diagnosis of what we would call cerebral palsy. This is a consensus definition published in Developmental Medicine Child Neurology. But cerebral palsy is a group of disorders of movement and posture which have a non-progressive ideology, I'll talk about that, related to damage to the fetal or infant brain. 

And cerebral palsy is often accompanied by co-occurring problems with sensation, perception, communication, and/or behavior, and/or seizure disorder. I would only add on this that the often could easily be "usually." Cerebral palsy is a very complex condition. This is how I actualize this definition, if you're in clinic and we're talking through why do we call this cerebral palsy or why not when we're evaluating a disability issue. So I simplify it into this kind of four requirements. One is that it's a non-progressive impairment of the immature developing brain. It has to be brain, hence we call it cerebral palsy because that means brain. And with abnormal motor development, which is the palsy part. Palsy in literal translation would be weakness, but it defines the motor component. And so I'm going to talk about what all of those four categories mean. Non-progressive and exclusion for conditions which would degenerate over time, this, for example, could be some type of a genetic disorder which is toxic to the brain and gradually gets worse year by year that would not be called cerebral palsy that would be named by the defect in the gene or the chemical which was causing the brain damage. 

CP would also exclude conditions which resolve. There are many things that are risks in early development that don't have a lasting effect, and things that resolve would not be called cerebral palsy. Now, having said this, the ideology of cerebral palsy, the thing which caused some injury to the brain, this condition is non-progressive, but it doesn't mean that cerebral palsy looks the same when the baby's born as it does when the child is five or the adult is 20 or the adult is 75. The cerebral palsy, the brain part of it is non-progressive, but the manifestations are not unchanging. They do look different through the lifespan. And then when we look at the issue of development, anybody with an onset of a condition at any stage of development of the brain would have a developmental disability. But usually for CP we're talking about injuries that occur before or around the time of birth. So in this very early stage of development. If somebody had a period of typical development and then sustained a traumatic brain injury at age three, we would usually call that traumatic brain injury as the ideology for the disability, if there were disability issues. That would also be a developmental disability, but we wouldn't name it cerebral palsy. 

I met somebody this week who had a traumatic brain injury before birth, was a trauma, and so it was the same kind of injury, but since that occurred before birth we do call that cerebral palsy. It's a little bit arbitrary, but that's the way we do that. So in typical presentation of cerebral palsy, we're not quite sure for most people at the time of birth what disability issues might be there, but typically by six to 12 months we are moving toward making a diagnosis. My older cases may not be noticed until 12 to 18 months, especially if they involve primarily one side to a milder degree. The other challenge in early development is that infants can have some early developmental motor signs that are unusual for development, but they can also disappear and more typical movement patterns can emerge, and then we wouldn't call that CP. So the next step has to be an abnormality of the brain. The main point being here that there are other conditions which can cause motor difficulties, like muscle diseases or diseases of the spinal cord, and these would not be called cerebral palsy because it doesn't originate from the brain. The brain impairment or the brain injury with cerebral palsy actually is very, there are many different kinds of brain injuries that can occur. I'm going to talk in more detail about the most common injury among children today who develop cerebral palsy. And this is related to a complicated sequence of events that get set in motion among newborns with very low birth weight who have prematurity. 

And this is the largest contributing risk factor to cerebral palsy. Talking about the details of this sequence of events would be a very long discussion. I'm not going to go into depth, but I want to talk about what happens to the brain and what the MRIs look like in the most typical cases for children with this condition. And it causes something called periventricular leukomalacia, which is a mouthful, but if you break it down, it's pretty easy to understand what that means. You just have to know all these components of this medical terminology. But peri means just around, like perimeter. Ventricular, and I'll show you a picture of this, but this is around. There's some plumbing structures in the brain where the spinal fluid circulates, the cerebral spinal fluid, and I'll show you a picture of that. So around these deep brain fluid spaces there's a change in the white matter. Leuko means white. 

So you have the gray matter on the surface of the brain and the white matter interconnecting the surface. And malacia means thinning. So periventricular leukomalacia is thinning of the white matter in the area around the deep brain fluid spaces. And here's the picture of what periventricular leukomalacia looks like. I put up two MRIs. This is a normal brain, and these are these ventricles, these fluids. The spinal fluid circulates in this complicated plumbing system. And this is a brain with very large ventricles. And this is the white matter, and what we have is this area of black, the fluid space is much larger because this white matter is much smaller. So it's receded. 

And here the white matter to the gray matter, there's hardly any difference compared to this side where we have this more robust white matter. So this is a periventricular leukomalacia, and I'm going to come back to this several times because this abnormality of the brain, which is the most common finding on MRI underlying cerebral palsy now, is a fairly broadly based abnormality of the brain. It goes from the front to the back. It involves a lot of the brain tissue. The point we're going to make is that although this does cause motor disability, it probably also interferes with other things that your brain does because all of the areas of the brain can be affected by this. So we'll come back to that. Now, although PVL is the most common cause of cerebral palsy, it is by no means the only thing that happens to brains. Birth hypoxia is like having some trouble at the time of birth with getting oxygen delivery. That definitely can damage the brain. It actually is the minority contributor to cerebral palsy in our current population. 

It's much less likely. Babies can have strokes during fetal development and can be born with after effects of a stroke. That's another major ideology. High bilirubin used to be a major ideology of people with cerebral palsy. A lot of older folks now in the 60-plus age have after effects from this problem, but there was a change in medical treatment that substantially reduced the bilirubin, jaundice is another word for bilirubin, the jaundice related cerebral palsy. Some people with cerebral palsy have two or three of these events happen. So the ideologies can be multiple. This is just a slide that points out the different ideologies, and this big space is the PVL. So you see that most people currently, this is 2006 publication, in our modern population, this periventricular leukomalacia is the largest contributor. It's the main thing that's wrong with the brain. 

There's a group here who actually don't have any abnormality with the brain on the MRI. That's a small group, but sometimes we don't know what was contributing to the CP. We don't really have an ideology. But that's a relatively small group. Usually we do find out something in the brain tissue that explains the condition. This gray matter group would be largely the group that have some oxygen delivery problems at the time of birth or before, but usually around the time of birth or shortly after. And this group largely as well. This is the one where the jaundice affects these these basal ganglia. It's another deep brain area. And some people have genetic conditions or other problems that happen as the cells of the brain are beginning to organize very early in fetal development, and that would cause a malformation or the tissue didn't develop in the normal structural way. 

So this is just a picture again of the many ways the brain can be different in people with cerebral palsy, with the PVL being by far the most common. I'm back to those four conditions. So we talked about brain injury in the early period. This is the disturbance of motor development. So all people with cerebral palsy have some thing different about motor development. Delayed motor milestones are very common. That isn't a requirement for cerebral palsy. People can be different without being delayed. Most people with cerebral palsy have spasticity. It's very common, but again not everybody has spasticity. 

Some people have more what we would call abnormal involuntary movements. Almost everybody has one of these, but one could also have decreased quality of motor control. And in fact, all of the people with different manifestations of cerebral palsy may have not only weakness but difficulties with the quality of how the muscles move. So people with cerebral palsy will have some disturbance of motor development. Traditionally, cerebral palsy has been mostly described with looking at how the motor development is different and then naming it by the distribution. The spastic types of cerebral palsy where we have spasticity increase muscle stiffness and overactive reflex activity. The spastic types are described largely by where they affect the body. So quadriplegic, quad means four, that would be arms and legs are involved. Hemiplegic is one side. That's more typical after a stroke-like event. 

So the left side may have motor abnormality, the right side may not. And then diplegia is a condition pretty unique to cerebral palsy where the legs are heavily involved with a motor abnormality but the arms are not. And I'll come back to that ideology. The other types can be some abnormalities of movement disorder. Dystonias are more fixed movement disorders that go into postures and they stay there. The dyskinetic movements are things that are moving around but have a lot of irregularity. So athetoid movements are more writhing or inconsistent in movement quality. And ataxia relates to coordination problems where things target with difficulty. So those are kind of the movement disorders of cerebral palsy. So we can look at CP and say, okay, how many people have which kind of movement problem. 

So this whole group, black, blue, and red, these would be the people with spasticity or issues with stiffness. And this diplegic group is the one most strongly correlated with periventricular leukomalacia. So most of the people, three-quarters if we look at by how does it affect the body, will have spastic, and fairly evenly divided between this diplegic-like pattern, the hemiplegic, left versus right pattern, and the quadriplegic, all four parts involved. The movement disorders, pure movement disorders are down here. Although a lot of people in this blue group, in the quadriplegic group also have movement disorders on top of the spasticity. And then the others are a very small group. So most of the people with CP have spasticity. Then we look at this correlation. Do the scans match up with how it affects the body movement? And there is a loose correlation. This periventricular leukomalacia, which you saw on the picture, really relates fairly strongly to this diplegic pattern which is the legs are involved and the arms not so much. 

But if we look at periventricular leukomalacia, it actually is the number one cause of hemiplegia as well. So you can have that PVL everywhere or you can have it in the front of your brain and not in the back or you can have it really heavily on the left and minimally on the right. It's a patchy variable thing. So it actually underlies a lot of the different forms of cerebral palsy. So PVL is related to all three. Oxygen deprivation at the time or shortly after delivery is a very challenging process for the brain, and that tends to affect the whole brain and we see more problems in that group of people with spastic quadriplegia. And the people who had some kind of a stroke-like event prior to birth are more likely to have hemiplegia or involvement of half of the body. So there's a partial but not total correlation between the motor types and the ideologies. And I had made the point, and we'll look again at periventricular leukomalacia, that it can be asymmetric and involve one side more than the other. It can involve arms and legs if it's extensive, or it can involve just legs. 

The reason it can involve just legs and the diplegic pattern comes is that the legs travel more in the middle when they come down. The arm tissues are located on the surface of the brain, a little more on the outside, and the legs start from the inside. And so the legs travel more closely, the fibers to the legs, the nerves to the legs travel more closely in the deep brain to this ventricular area. So they're more frequently touched by the periventricular leukomalacia. But I want to look at this PVL again. We're going to move onto another piece that the signals to the arms and the legs come out of the cortex of the brain, about here. About a third of the way back. And so to change the motor system, it's the PVL in this area that would do that. Up in this area where this brain demonstrates some PVL, we have a lot of what we call executive functions. That's attention, if you have deficits, there is a child that would be in the direction of attention deficit disorder, but it has more social/behavioral manifestations. 

And back here in the back two-thirds of the brain, we have a lot of processing of our sensory system. And if you really look at this PVL, the thinning is actually more significant under the areas where we perceive where our body is in space and where we use vision, which are the functions that are more in the back of the brain. So the most common lesion associated with cerebral palsy when you look at it really goes from the front to the back of the brain and is variable from person to person on where it affects them. So I'm moving on to the next point that although all people with cerebral palsy would have a disturbance of motor development, the definition of cerebral palsy really doesn't include specifically sensory cognitive or behavioral dysfunction. But it's really important when we look at the diagnosis that we're recognizing that it is by no means an exclusively motor condition. There's no such thing as a disease of the brain which affects only motor function and doesn't affect other aspects of how we think, see, feel. I'm going to move quickly through this part just to kind of review the wide range of associated conditions that are common with cerebral palsy. Changes in thought patterns are common. Up to half, it depends on who's entering the study when people look at this question, but cognitive impairment is common. Learning disability is probably more common than intellectual impairment. 

Attention deficit disorder is common in CP but also common in children with prematurity who don't have CP. And other language and behavioral problems. Sensory abnormalities are very common. I tried to show you that with the scan that the PVL is very prominent in the areas that organize our sensory systems, and in particular the visual system is very affected by white matter disease because vision is something that really depends on that knitting together from the white matter. So these visual difficulties are very common. It isn't necessarily, although 80% of children with CP have some acuity or visual problem, you can also have normal visual acuity. You don't need glasses, but you still have difficulty sometimes in using visual information efficiently. So the brain can have trouble processing visual information even though the images are clear. So it's fairly complicated People also have abnormalities in how they perceive where their body is. Seizures fairly common in association with cerebral palsy. 

I would also point out that a lot of people with cerebral palsy have changes in these autonomic function. These are the unconscious things that your brain does. Regulating your temperature, regulating how things move through your stomach and colon, all of these other functions of the body are in part regulated by the brain. And so they can be affected in people with cerebral palsy. This next set of lists are things that happen not just because of the abnormality in the brain but because of how this affects the rest of the body. So people with motor difficulties can have challenges with eating, digestion, the oral cavity controlling saliva. It changes the development of how the teeth and the palate are arranged if you have motor changes in the mouth. Obviously motor difficulties from birth are going to affect how your bones and joints develop. So orthopedic difficulties can be prominent for people with cerebral palsy, also extremely variable. And then various skin problems can occur because of pressure points or difficulty with cleansing or sweating problems. 

So that was a little less of the many manifestations we can see with CP.  Other speakers today are going to get more into questions on prognosis, and I'm just going to introduce something that other people will talk more about. But we've been developing much more specific information to understand what's the future holding for somebody with cerebral palsy. And we have these classification systems, and I'm only talking about this from reference point of how do we describe what cerebral palsy is for an individual. We have different classification systems for motor function, which is mobility skills, walking, manual abilities for hand function, or communication skills for speech. And they all have five different ability levels, and one is very typical and five is very dependent. This describes how does somebody function overall. And that's just an example of the motor skill, but we'll come back to that and we'll introduce these curves. But we can, using information like this about the motor skills, we can project fairly early what adult life will be like. And we based a lot of interventions on what we think we can help people do. 

One of the points was the many things that we do for people with cerebral palsy to help with the motor disability are very helpful to people, but they don't change the MRI. So the people still have, even with lots of therapy and bracing and baclofen pumps, we still have a piece of cerebral palsy which is not changeable. And we don't help people get from level five to level two, but if you're functioning at level four, we certainly can help you a lot to be able to do more things with assistive technology or training so that you can do a lot more even though your body wants to function at this level of disability or impairment. We'll talk more about that. So where I led on this is to combine this all together, provide a more complete description of cerebral palsy. And if I had all the information, this is what I'd like to put in my little summary like I met somebody today and this is the issue that we're dealing with. An example may be somebody with a spastic quadriplegic type of cerebral palsy with an ideology of low birth weight and prematurity causing the MRI finding of periventricular leukomalacia functioning at these various functional levels, it's how the person does stuff, these are the abilities, and then the list of associated major concerns that we would have with cognitive visual development. This is a description of somebody with cerebral palsy. It's complex, it's multidimensional, and again, it matches in with this modern consensus definition of what we're calling CP. So I'm going to make one little point. 

When we look at a really, from a clinical standpoint because we're in clinic several times a week for people with cerebral palsy, and anybody who has met a lot of children with developmental disabilities will know the answer to this question. But what is the medical problem which is most common among children with cerebral palsy? And the answer is something that's really mundane. But anybody who deals a lot with disability populations knows this is the correct answer, that we're dealing with constipation a lot. And it isn't walking. It's really some really basics. And this problem has some really complicated underlying components. The number one issue is how much is somebody drinking. There are many reasons that people with cerebral palsy may not drink. Some people don't want to go to the bathroom a lot because there's some barriers there. So if you drink less, you don't have to go to the bathroom, but that's going to be very constipating. 

Other people have difficulties swallowing, and they have trouble getting enough hydration. So the issue there is the physical ability to get fluids in. But nutritional factors go in. We talked about the autonomic piece, that the brain controls how the stomach and colon work so people with CP can have difficulties there. And then we have some behavioral and physical and even sensory issues. If we look at the sensory development, what I it I can't like really feel where my body is and then you put me on the toilet and I fell like I'm going to sink. That's very uncomfortable. I wouldn't want to do that. Do we feel safe? Can we understand how our body interacts with that toilet seat? That's really important. So when we look at then what are we going to do about this, you can kind of get my point. 

Even a really simple thing like going to the bathroom can benefit from a team approach. So we look at the fluids and improving diet. This may involve swallowing and feeding abilities with speech and language pathology. We might look at medications for constipation or maybe not, but this would be the doctor and nurse part. OT and PT can look at these bathroom access issues, and does this person need a different kind of support to use the toilet. And then we may have psychological or cognitive issues on how is this person proceeding with the process of using the bathroom. So I picked this on purpose as being a really common problem but to illustrate that even this is not really a simple issue. And so I'm again back to the multidisciplinary team, and I'm just going to explain briefly the teams we have at the Waisman Center and kind of how people would flow through care through the life span. We have for those children, because prematurity is really the big risk factor now, there are children that had an event at the time of birth which would bring them in for newborn followup. These are children at risk, and we want to see whether there are developmental concerns or not. 

If there are developmental concerns and we have delays, we have a neuromotor development clinic, and this typically is seeing children six months to three years of age. And we're looking at the motor milestones and health problems and evaluating whether there are emerging disability issues and what are they like. Because it's hard to know is this something that might be like cerebral palsy or something that may be more like autism as we move forward. In the CP clinic where rehabilitation works, we have children with disability, and we work on the management of the motor disability issues and the whole rest of the package that comes with that person. And we work with people until we can transition to adult providers. So the CP clinic runs to early 20s. This is really crowded, but you have the kind of transition but along the way people may have other needs. So in the child at risk and early, they maybe would need help specifically more with feeding and nutrition. And so my point is that we refer along the way to other services at the Waisman Center or American Family Children's Hospital to kind of weave through the lifespan. As we look at evaluating a delay, orthopedic surgery, genetics may by involved here to find out why do you have CP, what's going on with this disability. 

We have people from the spasticity movement disorder clinic coming. And CASC is the communication systems clinic, which these clinics are running at the Waisman Center but on different days, and people may come from the CP clinic and be followed for a couple of years for some more intensive treatment for spasticity and then go back to the cerebral palsy clinic. So people may move through different specialty areas of care to address particular problems and then back into one of these base clinics to look at long-term guidance. And we end with dealing with the transition issues to look at getting you ready for adult life and the support you would need there. So this is my attempt at doing a brief introduction to a very complicated situation for the person with cerebral palsy, with really every person being very different with a unique set of needs for themselves and unique needs with who they need to access for healthcare and for system care in life support.