Individualizing Cerebral Palsy Care
Dr. Stephanie Acord dives into the importance of early identification of cerebral palsy, common misconceptions of the diagnoses as well as new diagnostic methods and therapies that help improve life-long outcomes for children with mild to severe cerebral palsy.
Meet the speaker
Host: Welcome to Doc Talk, today we're talking with pediatric neurologist Dr. Stephanie Acord. Dr. Acord sees and treats children with cerebral palsy and is a member of Cook Children's Movement Disorders Program. She also works closely with the neurosurgery eileptology, physiatry, and stroke teams here at Cook Children's to provide patients and their families with the most up to date information and treatment options. Welcome Dr. Acord.
Dr. Acord: Thank you. Glad to be here.
Host: So I'd like to start off with a little background about you how did you choose neurology and in particular movement disorders and cerebral palsy?
Dr. Acord: I don't think there is actually a patient that I can recall that steered me in the direction of neurology it's more of that type of field of medicine that neurology is a lot of times people think of it more as that kind of like putting together pieces of a puzzle, and I think that's why many people actually shy away from neurology. But to me that's the challenge that is intriguing, and very interesting to me.
Dr. Acord: My first exposure with movement disorders and cerebral palsy actually came here at Cook Children's. I was in the middle of doing a child neurology rotation at the university of Kentucky and I became very interested in reached out actually to Cook Children's and got a rotation set up here in between my second and third year of medical school.
Dr. Acord: During that rotation I spent a good portion of time with doctor Warren Marks and Fernando Acosta who really showed me the ropes as maybe disorders.
Dr. Acord: I think one of the unique things about Cook Children's is that here we have several movement disorder specialist whereas many other places in the country that actually don't have any let alone several.
Host: Can you tell us a little about the Cerebral Palsy Program here at Cook Children's?
Dr. Acord: Cerebral Palsy clinic here at Cook Children's is a multidisciplinary team approach in which includes the neurologist, the physiatrist, the orthopedic surgeons, neurosurgeons the therapists that are inovolved as well as the orthotics and prosthetics team. The nice thing about having a multidisciplinary team clinic is that you can have several providers looking at a patient at one particular time and all come together in terms of what do they feel like is the best treatment option for the patient that given time.
Dr. Acord: And then depending on the treatment plan that's laid out is whether or not do you need to come to that clinic every time verses do they come to that clinic every so often but they don't necessarily need the resources in that clinic for every visit.
Host: So according to the CDC cerebral palsy is the most common motor disability in children about one in 323 children being diagnosed with this condition and yet it seems like there are still a lot of misperceptions around cerebral palsy even in the medical world, can you talk about what those are and why?
Dr. Acord: I think that many people think that cerebral palsy is looks very similar but actually it looks very very different. Depending on the underlying etiology and severity of the injury patients can have mild symptoms to their more severe and are more wheelchair bound. About 50 percent or a little bit more than 50 percent as patients can walk independently or as about 30 percent has limited or no walking abilities.Most people kind of jump straight to the patient population with the more severe symptoms when that's the smaller percentage of the population. There's also more research that's going into cerebral palsy and early diagnosis which can also be helpful.
Host: So there's also a lot of stigma associated with cerebral palsy, why is that?
Dr. Acord: I think that kind of going back to what we're talking about earlier that most people feel like all cerebral palsy patients look similar, that people kind of let their minds wander to the most severe form when they hear the diagnosis. Everyone wants for their own child to have the best outcome and for them to be able to live independent lives but sometimes unfortunately that's not always the case. In some children may actually need lifelong care and diagnosis of cerebral palsy doesn't mean that it's just the child that's affected but many times it's the entire family as they're going to have to assist with care for their child sometimes lifelong.
Dr. Acord: I think another stigma associated with cerebral palsy is that all patients that have cerebral palsy have intellectual disability which is not the case. It's always important you should address each patient individually because even though they may not be able to speak verbally to you doesn't necessarily mean that they're not able to understand what's going on around them. They indeed are the the center of the treatment plan and so you should always make sure that they are heavily involved in the decision making to the extent that they are able.
Dr. Acord: There are many people that have normal intelligence that have cerebral palsy as well, even if they don't have normal intelligence doesn't mean that you should treat them any differently they may need assistance with daily care center activities but it's also important to still treat them as an individual.
Host: Cerebral palsy is kind of an umbrella term can you clarify what it is and what causes it?
Dr. Acord: So, cerebral palsy is a group of disorders that affects a person's motor movements, posture, and or balance. It's a very heterogeneous mixture as cations in which there it is some type of injury or insult that happens in the womb, during delivery, or after delivery that the injury or insults does not itself get progressively worse with time. These are referred to as congenital ideologies and can include things such as pre maturity, low birth weight, multiple births, intraventricular hemorrhage, or IVH, perinatal stroke, cortical malformations such as polymicrogyria or pachygyria - meaning too many or too few folds - of the brain, congenital infection such as CMB or zika virus, infections acquired from the placenta or even after birth which can include hsv encephalitis, E. coli meningitis or group B. strep meningitis, birth complications leading to compromise of the placenta or oxygen to the baby such as hypoxic events or less commonly these days kernicterus. Depending on the etiology and severity of the injury will also affect the different type of cerebral palsy that have as well as the constellation of symptoms that they display.
Host: So what symptoms should doctors be looking for?
Dr. Acord: I think the more common signs of cerebral palsy include asymmetric use or movement of a part of the body indicating an underlying weakness. So for instance if a baby prefers to grab with just one hand compared to the other or if they're cralling for instance it's that one leg they're dragging behind compared to the other. If they have things such as abnormal ton or posture, what we refer to as spasticity, or increased muscle tone or they can have low muscle tone which is known as hypotonia. They could also have ataxia, which is a problem with balancing and coordination, other things such as motor delay so if they didn't meet their gross motor milestones such as sitting, crawling, pulling to stand, cruising along furniture, walking, or even acquiring things such as a pincer grasp - if they have things such as cognitive or intellectual disabilities or even if they have a strong past medical history that is concerning that they could have cerebral palsy taking a deeper look into the pregnancy history as well as the birth history can sometimes shed light into if they have signs of cerebral palsy.
Dr. Acord: Many times though there's actually nothing in the history that makes this suspicious that there could have been a perinatal insult and as a result imaging can be very helpful in the situation.
Host: So are there symptoms that mimic cerebral palsy or mask it? For instance, children with complex conditions, can these kids have cerebral palsy that goes undetected or undiagnosed because it's hidden by the symptoms of their other issues or appear to have cerebral palsy when in fact they don't?
Dr. Acord: There can be many underlying genetic disorders that can mimic what looks like cerebral palsy as we start to learn more and more and there's more genetic testing that's being performed I think it's helping us to identify some conditions that either may be more likely to be associated with cerebral palsy, or, the diagnosis instead of cerebral palsy.
Host: So in May of this year the cerebral palsy foundation lowered the age of early detection from around 2 years of age to as early as 9.5 months how does this impact the diagnosis care and potentially the long term outlook for patients?
Dr. Acord: I think that the earlier that you can detect these patients a better it's going to be for them in the long run. Babies that are in the NICU are starting to be identified earlier and earlier. There is some research going on across the country in which they have different scales that they're watching some of the different movements that babies have and it's allowing them to be able to determine if certain babies are at risk for developing cerebral palsy.
Dr. Acord: Many times before the babies actually go home from the NICU some of them actually have been identified and or diagnosed with cerebral palsy based on what type of underlying etiology they may have or why they're actually in the NICU in the first place. The earlier that children can be diagnosed with cerebral palsy the sooner that they can actually have more hands on type of aggressive treatment options. Things that are most important at the beginning are going to be therapies but then as they get a little bit older other things such as time management can be helpful especially to help make additional progress from a developmental standpoint.
Host: So for babies who have a risk at birth, is there a time that obstetricians, primary care physicians, or neonatologists should discuss the possibility of a cerebral palsy diagnosis so that the parents of primary care doctor know what to watch for?
Dr. Acord: I don't think it's so much the obstetrician but mainly more the primary care provider or the neonatologist that should be able to help to identify patients that may be at risk or that actually have cerebral palsy. The obstetricians' information in terms of if it was a difficult delivery or what happened prior to the baby being born can be helpful but then the obstetricians usually kind of hand the baby off and then they usually kind of stay more with them on in the care for the mother. So it the baby ends up going to the NICU then depending on their reasoning for the baby going to NICU would be whether or not they need to continue to monitor and if they see any clinical symptoms that they're worried about that they need to do any type of imaging for their brain for instance, if the baby is not identified until they're a little bit older either because they have delays in their developmental milestones or maybe that there's some weakness that they have noticed that the baby is getting a little bit older than that for their primary care provider would be able to make a referral over for those concerns.
Host: So are all cerebral palsy cases congenital or are there other injuries or illnesses that can result in cerebral palsy, maybe even among older children?
Dr. Acord: Congenital cerebral palsy, meaning that something happens in the womb during delivery or after delivery, is by far more common and accounts for about 80 to 85 percent of patients that are diagnosed with cerebral palsy. There are however what we refer to as acquired cases in which that's about 15 to 20 percent of patients and that usually is consistent with patients That have had some type of traumatic brain injury such as child abuse which is referred to as non accidental trauma, it could be related to a motor vehicle accident, or it could be potentially related to some other type of fall.
Host: So the child is diagnosed with cerebral palsy, what are the treatment options and how are they determined?
Dr. Acord: It first depends on what type of cerebral palsy they actually have> Spastic cerebral palsy is by far the most common and accounts for about 75 to 85 percent of the different cases that we see. Depending on whether or not they are what we refer to as hemiplegic meaning one side of the body is affected and there's about 40 to 60 percent of patients that have this form. or diplegic meaning involvement of both legs is about 10 to 35 percent, or quadriplegic meaning both arms and legs are involved which account for about 25 to 30 percent. Depending on the type of cerebral palsy that they have, treatment options can look very different. There can also be things such as the dyskenetic, which means lots of abnormal movements such as involuntary ataxic meaning that if they have trouble with balance and coordination. hypotonic cerebral palsy meaning that they're kind of on the loose side or a mixed cerebral palsy which make up a much smaller percentage of the population. For pateints that we're looking at more of tone management especially from a spasticity standpoint it's always important to look at kind of the short term as well as the long term goals of the treatment plan.
Dr. Acord: Many times things such as therapies are going to be one of the first lines of treatment options for these children that started a young age. And then as they start to get a little bit older things such as bracing or what we refer to as orthotics as well as equipment may be something that they need in order to achieve the particular developmental milestones. If they start having a lot of trouble with their their tones such as spasticity, then sometimes we add things such as medications that you can take by mouth which include things such as baclofen, valium, or tizanidine that get absorbed through the intestinal system.
Dr. Acord: If we're trying to do more of a focal effect for patients for instance to have diaplesia or if they had hemiplegia then things like chemo denervation with botox could be helpful to target a particular area. Moivng up to things that are more invasive, these include things such as baclofen pumps, selective dorsal rhizotomies, or even deep brain stimulation may be potential options for some of these patients depending on the underlying etiology of their cerebral palsy as well as the location and the symptoms that are associated with it. As they get a little bit older other things to consider would be orthopedic surgeries as a treatment option as well.
Host: So you mention baclofen pump and selective dorsal rhizotomy, and deep brain stimulation, can you elaborate on what these treatments are?
Dr. Acord: I think we're lucky here at Cook Children's that we have the ability to do all of these different types of treatment options for children, the first one being baclofen pumps. Our children with more refractory spasticity that hasn't necessarily responded well to oral medications. Most of the time prior to actually implanting this device we do a trial of giving the medication like you would with a lumbar puncture and inject the medication and then have a therapist evaluate what they're tone did after the medication is on board. If they have a significant response to the medication then we usually discuss moving forward with this as an option. Sometimes baclofen pump can not only help with ease of care for patients especially if the families have difficulty with dressing or changing them but it can also help a lot with kind of pain discomfort that these patients may feel on a daily basis.
Dr. Acord: Selective dorsal rhizotomy, or SDR, are pretty extensive surgical options mainly for patients who have diaplegic cerebral palsy. Most of the time these patients are selected at a younger age usually you start thinking about the process in the age of anywhere from 3 to 5 to start the workup. Usually these patients do not walk independently or they can walk independently in the home setting but when they're out in the community need more equipment to assist them. Usually we have these patients go through the surgical workup process they undergo what we refer to as a gait or motion lab analysis to really look at the dynamics of their walking pattern and see whether or not does this seem to be strictly spasticity or if they're complaining of dystonia that lays over it as well. After this the gait lab is reviewed by the neurologist as well as the orthopedist, therapist and the orthotics and prosthetics team. Usually based on this as well as it sometimes having to do a baclofen trial we usually use this information to determine if the candidate is a good candidate. If we feel like they're a good candidate in usually we send them over to you our nurse surgeon, Dr. Richard Roberts, who's performed all of our surgeries, to go through the technical piece in terms of what does it look like from a surgery standpoint as well as the complications that can be associated with it. Afterwards usually it's a pretty intensive stay on the rehabilitation unit for usually about 4 to 6 weeks and then still afterwards when they go home it's also additional therapies that must continue in the home setting.
Dr. Acord: There are also some options for patients that have increased tone in arms and legs and may not be a good candidate from the baclofen pump standpoint that could be a candidate for more of what we refer to as a palliative rhizotomy in which this is used mainly to help with daily care as well as ease the pain and discomfort.
Dr. Acord: Deep brain stimulation or DBS can be beneficial to patients that have more of what we refer to as dystonic or dyskinetic cerebral palsy. These patients usually have a significant amount of dystonia and sometimes this can also affect their ability to speak clearly. Dystonia can look like spasticity however they are a distinct symptom and as a result the treatment options can be different. Depending on the etiology of the cerebral palsy as well as how extensively involved the patient's symptoms are the patient may be a good candidate for this as an option. However this is a permanent implantation as a device. As a result, there's an extensive work up that goes into this process and usually with patients that have cerebral palsy that were considering deep brain stimulation usually the movement disorders team will review the case in detail and make sure that we feel like they're a good candidate before proceeding.
Host: So we know that cerebral palsy doesn't get worse over time and with treatment patients can improve even for those with the most severe forms, but what about children who don't receive intervention what happens to them?
Dr. Acord: I think it's really important to remember that the patients that do get treatments are going to do better than the patients that don't get treatments initially. Many times as the brain continues to develop as babies brains mature with age they also start having developmental patterns that occur. So for instance, if you have weakness on the left side of the body and you try to use the right side of the body because it's easier and that's what everybody would want to do then that left side doesn't get to develop those muscles and that strength like their right side does and as a result more connections and pathways are going to develop towards the stronger side leaving the weaker side appearing even more weak. Over time the connections and pathways from the brain leading down to the spinal cord and out to the arm and leg are going to decrease over time and as a result sometimes you can see some differences not only in strength but also kind of differences in how the arm or the leg looks and sometimes it actually will appear smaller not only in the length of a foot or the size of a calf but also there can be a leg length discrepancy that would have to be addressed as they get older.
Dr. Acord: Things such as toe walking for instance that pattern that children develop that shouldn't usually be a heel to toe pattern if they walk on their toes because they have spasticity, then the brain starts to think that that's the normal type of pattern that should occur when they're walking and as a result if you try and correct the toe walking pattern when there are 2 or 3 for instance versus if you're trying to correct it when they're 8 it's going to be a lot more difficult when they're older to try and reverse some of those connections and pathways that have developed and to correct so many extra years as incorrect patterned walking.
Host: This diagnosis has to be a real challenge for families to hear so when a family is referred to Cook Children's how do you set expectations for outcomes in terms of care and long term outlooks?
Dr. Acord: Usually when the patient comes to Cook Children's for the first time they're usually not in the cerebral palsy clinic itself and they're usually just with the neurologist on a one on one visit. Usually at that visit I usually am pretty open and honest with the families and try and make sure that I explain to them what I'm looking for, for instance if we do any particular type of imaging of the brain or what I may be concerned about so that it's not a surprise to them when we get results back in terms of what explains the underlying cause for the symptoms. I think also laying out expectations as what does it look like right now, what are we kind of anticipating in probably the first couple of months to maybe the first couple of years and then kind of where the family is and and you got to meet them where they're at. Some families want a lot of information up front and they're going to go to the Internet and they're going to do a lot of Googling and some types of families are only gonna listen to exactly what you have to say, and so you've got to figure out where the family is and how much information that they may want to know at the beginning, you also have to remember that most of the information you may tell them at the beginning they may not actually hear because once you say the word cerebral palsy they may not actually hear the information afterwards and so on subsequent visits just remembering to kind of circle back to some of the information that you had told them prior to make sure that everybody's on the same page with the treatment plan and what do we expect as they get older.
Host: How is a child's treatment determined? Like who's involved in the decision making what does the care team look like?
Dr. Acord: So, depending again on the etiology of the cerebral palsy. what type of symptoms that they have, determines who all needs to be involved. Most of the time from a neurology standpoint it's going to be the neurologist that's making a bunch of different referrals and treatment plans. For instance if you have spastic cerebral palsy then depending on if it's just in the legs then they may start talking to you about things such as would oral medications be good options or more focal targeting of medication such as botulinum toxin or depending on the age of 30 a little bit older would they be a good candidate from a surgical option? And so some of these things take time in terms of planning to make sure that certain treatments they got early on aren't going to interfere with some of the results if they're going to be worked up for additional more invasive surgery such as selective dorsal rhizotomy or even baclofen pumps. But usually the neurologist is going to be the one that helps to guide the families of the different options and then whether or not they may need to go see the orthopedic doctors or they need to see the neurosurgeons and then again if they are in the cerebral palsy clinic then many times at therapist may be there as well as a orthotist could be there that can help in terms of making sure that they're getting all of the equipment that they need they're getting all the therapies that they need and they're getting the briefing that may be helpful for them.
Host: How is care coordinated for these children and their families?
Dr. Acord: Usually the primary care physicians are going to be the ones who are the main coordinators from a treatment standpoint. Usually the primary care providers are going to be aware of all the different subspecialists that they may be seeing as well as the comorbidities that can be associated with it. They can kind of be like their team or their home base and help to refer out to additional specialties that can assist and help with the treatment plans.
Host: So it sounds like the primary care physicians need to be very aware of what the comorbidities are and what kinds of comorbidities might there be?
Dr. Acord: From a neurologic standpoint, the most common comorbidity in patients that have cerebral palsy is epilepsy and that accounts for about 40 percent of patients. They could also have things such as intellectual disability or autism spectrum disorder. Things that have some relevance to the neurologic system but are things that we necessarily manage would be things such as swallowing dysfunction or dysplasia. Bowel and bladder dysfunction, visual impairment, hearing loss ,or even some pain.
Host: So what is the life expectancy of a cerebral palsy patient?
Dr. Acord: Depending on the severity of cerebral palsy can determine whether or not it would affect our lifespan. Patients that have more severe cerebral palsy symptoms are more at risk for having a shorter life expectancy usually secondary to either the seizures themselves and difficulty with controlling them. Things such as dysphasia or respiratory problems associated with it. Patients that have the less severe forms of cerebral palsy usually have a normal life expectancy.
Host: What does the future of cerebral palsy look like? What's on the horizon?
Dr. Acord: One of the up and coming fields in not only the neurology world but also the cerebral palsy world is the concept of robotics and the technologies associated with it. In the past few years the field has really started to blossom and it's allowing for a greater amount of independence for some of these patients. There are things such as wearable devices there are things that are helping with the arms as well as the legs, things like walking assistance devices as well as virtual reality equipment.
Dr. Acord: The thing to remember though is that nothing can replace standard therapies. However, some of these robotic devices can be helpful to further enhance the therapy that are already in place. There's no cure for cerebral palsy however there are lots of different treatment options. I think it's important to remember that no two patients are the same and a treatment plan for each individual is different. The patient should be the center of the process as well as the family and their expectations and goals for their child whether it's to improve that patient's independence to ease their daily care, and/or to eliminate pain trying to be mindful of the patients in the family to be able to gain the best results out of the options that are available.
Host: Thank you so much Dr. Acord for taking time out of your schedule to talk about cerebral palsy and the treatment and care available here at Cook Children's. The work you do is amazing and so important to the patients and families you care for.
Dr. Acord: It's been a pleasure thank you so much for having me.
Host: If you'd like to learn more about cerebral palsy and the Movement Disorders Program here at Cook Children's please visit Cook Children's dot org, and thanks for listening.