Dr. Mai Ngo presents "Rehab Management of Cerebral Palsy" at the UCSF Pediatric Musculoskeletal and Sports Medicine Conference 2023 in Berkeley, CA.
All right. Um To compliment Doctor Bra's talk. Um Our next uh speaker uh will be um talking about the rehab approach uh for C P. Our next speaker is Doctor May No uh doctor. No, is a pediatric uh physical medicine and rehab specialist. Uh here at U CS F uh Bank of Jones Hospitals. Um She completed her residency in pediatrics at U C F and then physical medicine and rehab residency at U C L A. Uh She has special interest in TB IC P and spina bifida. Um And at U CS F, she is the medical director of a pediatric physical medicine and rehab program at Hospital Oakland. Welcome doctor. Oh, great. They pulled up my slide pre uh pre presenter's view because I have a lot of slides. Um Thanks Selena for my introduction. So those who know me know that I really don't like public talking, but I think it's a little bit worth it because at least today in person, I get to see my extended work family and some of my um the crew from the CCS counties. My Alameda County people are here and then if you're from other counties And oh, um and we don't really know each other by face. Please come introduce yourself. So uh no disclosures. OK. So before I get straight into C P, I wanted to share this sort of mission statement about my specialty. One of the leaders in our field, Doctor Deb Gabler helped put together the statement for an envisioned future of pediatric rehabilitation medicine. So it reads in the future, the P D Pyschiatrist is recognized as the essential physician expert for Children and youth with acquired and congenital disabilities, with the main purpose to optimize function and life transitions across the care continuum and into the community. I just love how nice it makes my job sound. Um Here are my broad learning objectives. We're gonna discuss the definition and diagnosis of C P and then describe the clinical features and classification and then hone in on the current rehab management and treatment C P is easily the most common condition I see in my rehab clinic. So a typical referral would be a two year old male with frequent falling um on history. He took his first steps at 18 months but still has um poor balance and frequent falling. And most of the time I can just provide reassurance. Um however, sometimes it can be the first presentation of a true issue. So for this patient, there was a negative history of any prenatal events or post natal complications, neurology work up already included an MRI of the brain which showed Paraben Tri Leuco Mala. So that's a good hint. And then on exam, he had normal upper extremity tone, but then spastic lower extremities. So at um he was tied as his at his hip, abductors, knee flexors and planar flexors. And then his posture looks something like this picture which is consistent with a diagnosis of spastic diplegic C P. Uh More and more we strive for earlier diagnosis to help patients and families get plugged into appropriate resources as soon as possible. We rely on our pediatricians to refer early based on clinical clues such as poor feeding or growth positive of movement during the newborn period, delayed milestones such as not sitting by nine months, abnormal tone, which may reported be reported as a baby who rolls really early or is a good standard but is up on his toes. So these are the kids that sometimes the pa parents report as being really precocious, but then that raises a red flag for us. Sometimes you notice primitive reflexes or like really early handedness um way before two years of age or just general abnormal coordination. A lot of you in the audience will recognize this primitive reflex. It's the A T and R or asymmetric tonic neck reflex or fencing reflex. Um In typical development, this reflex goes away at around 4 to 6 months when the babies learn to roll. Because if you just imagine that arm keeps sticking out there and that gets in the way of them trying to roll. But in C pe we see this reflex um persist for a really long time. Like some of my more low functioning teenage patients will still have this fencing reflex. So back to our case, at this point, with our two year old boy, I can make the diagnosis of C P which is defined as a nonprogressive injury to an immature brain that impairs motor control. I feel like that's the fewest words that I can use to, to describe C P. Um Usually there's a correlate on history but not always. And I try to do some detective work during my history taking to figure out any risk factors that could explain someone having C P. So even though this is a nonprogressive injury, the clinical course is not static. It's an evolving picture because uh the child's function will change during growth and their development. And so when I take a history, I'm really trying to pay attention to the birth history. I want to know if there were any possible congenital infections. You remember our torch complex. Um these infections can go clinically unrecognized in the mother. Though sometimes if you're evaluating a baby who is born in an underdeveloped country, you worry about gestational toxins. So a bad iodine deficiency can lead to diplegia or lead or mercury poisoning can um lead to quadriplegia and then out of all the risk factors, you know, as you know, the most common is extreme prematurity these days for C P. So if I'm consulting on a new baby in the N U, I'm gonna comb through the chart and look to see like some of the um worrisome factors like a really low birth weight, less than 1000 g. A low apgar score less than three at 20 minutes out or a bad head bleed, grade three or four. And then also some of our babies can have prolonged seizures which can lead to uh encephalopathy. We know preterm infants have really fragile brain vasculature that can bleed easily. You have when you're grading I V H, it goes from 1 to 4. And then, so if you have the worst grade four, there's enlarged ventricles with blood in the actual brain tissue. And so these cases have a 90% risk of major neurologic sla bad I B H leads to paraventricular leuco mala or PB L, which I think more as of as scarring when you have a lack of perfusion to that paraventricular area that will result in like death or loss of brain tissue. And then this region is traversed by descending motor fibers from the cortex, fibers, supplying the lower extremities are more immediately located and therefore, are more affected in the mild to moderate lesions. But then when you, so you get, those are the kids where who look to have diplegia. And then when you have increasing severity of the bleed or hypoxic injury. The more lateral fibers get involved and um affect the upper extremities. And those are your kids with quadriplegia during my history taking if there's no report of extreme prematurity as a risk factor, then I go digging and then let's say the baby was born term. Then I go digging to see if there was some like bad hypoxic ischemic event or maybe a bad cars case. Let's say it was an emergency c section. There was placental abruption or um bad meconium aspiration nu cord that and the baby has a history of being intubated. Then that points me more in the direction of an hie or hypoxic is hemic encephalopathy or usually if I'm seeing a patient and they were coming from an underdeveloped country and there's a history of jaundice. Then um that alerts me more to maybe a, a case of bad Caris and the patient sometimes has more of a history of dystonia or a movement disorder. And if there was nothing obvious that went wrong during the actual birth, then I go digging for risk factors that occurred post natally affecting the immature brain in that 0 to 3 year. Um period. Sometimes there's a history of a serious bacterial infection or viral infection or the child suffered from a severe brain injury from non accidental trauma, a bad fall, a bad car accident, a near drowning event or even a stroke. Then there are the babies who are at risk of a heavy metal um or organo phosphate ingestion because their parents work in the agriculture industry. Once you know what risk factors to ask about, then you can try to get more out of your physical exam. So, Doctor Amita already already mentioned this in her talk. But um we know that most kids with C P end up getting diagnosed between one and two years of age. Um but there is, you know, a push to diagnose even earlier these days. So um most interventions, we know that most interventions to influence the cortical spinal tracts are most effective early, younger than two years of age in our N U. There is now a team of providers trying to diagnose as early as 20 weeks, five about five months, post term and they're trained on using the prac general movements assessment or G ma. Uh pre was a neurologist who published a lot on the neurologic exam of infants. So we know infants have typical and distinct spontaneous general movements from before birth to right, be right to the 20 weeks post term. If there's a pas of the movements or the movements are not complex enough, then you worry. I tried to find a good video for you guys on youtube, but that was not available. Um I'm not trained in it yet, but the idea is you take a 3 to 5 minute video of a baby who is nice and awake and calm, not necessarily engage in a toy or Binky or like their parent. And then later on somebody scores the video um in our N U, it's the P T who's in the N U Michelle and then our neurologist Dr Gardner, um a cramp. So if you see cramped synchronized general movements and there's an absence of fidgety movements, that's a good predictor of C P. Whereas just a poor repertoire of general movements and abnormal fidgety movements tend to be associated with just more minor neurological dysfunctions. Once they're a little older, you can use the hein or stands for the Hammersmith Infant Neurological exam providers who use this had to go through intense workshops to learn how to perform the assessment. Now, instead of just doing a visual assessment of the video, there's more of a hands on exam. It's used for patients 2 to 24 months. It assesses cranial nerve function, posture quality and quantity of movement, muscle tone reflexes and reactions and scores range from 0 to 78 with higher scores indicating better neural performance. In 2017, no V C based out of Australia at all, published a clinical practice guideline walking you through an algorithm for early diagnosis of C P. So before five months corrected age, the G MA is 95 to 98% predicted really high and the MRI is 80 to 90% predictive, a high score less than 57 at three months is 96 predictive still very high. But when you get a little older after five months, then the uh the values drop a little bit, the hen is now just 90% predictive MRI. And a different standardized motor assessment using the Ames or Alberta infant motor scale is 86% predictive. So once you make a diagnosis of C P, however early, it is the family invariably wants to know if their child will walk. The most common thing I tell them is that a positive prognosticator for walking without an assistive device is if their child can sit without support by two years. Typically, you know, this happens at six months. Another good sign is quadruped, crawling on hands and feet by 15 months. And normally this happens at 9 to 10 months. A bad sign is persistence of three or more primitive reflexes at 18 to 24 months. And then when they get older, if they're already walking, then families will ask you how long they will walk for like into adulthood. So there are several factors for that. So marginal ambulator lose function around their teenage years due to progressive orthopedic deformity that's pulling them closer to the floor. Um because they're getting more crouched, inadequate strength or control to carry their increased mass, we see that a lot and then other behavioral social emotional problems. So in now into the classification of C P, when I'm talking about a patient with C P I like to classify it based on the type of movement, the region involved and then their level of motor function. When classifying based on type of movement. There's four types. The most common is spastic, which occurs in greater than 80% of cases. It arises from damage to the cerebral cortex here in pink, resulting in stiff muscles. Dyskinetic C P is associated with uncontrollable movements, just involuntary movements. Um and that is associated with damage to the basal ganglia, deep brain in green. And that's when you hear terms like choo athetosis or dystonia. A toxic C P is associated with damage to the cerebellum and orange resulting in poor balance and coordination. So these patients have a very wide base git truncal sway makes them look drunk when they're walking. Mixed C P is due to damage to multiple areas of the brain and mostly it's a mix of spasticity and athetosis. In six C P, you can often see it further classified according to the limbs involved. So mono plegia, um it is usually a affecting a single limb. Usually the arm hemi is one side of the body. Arm, more than leg diplegia refers to legs, um usually more than the arms and then quadriplegia is all four limbs more severely affected. And then this is the G M F CS um system that doctor already spoke about. So, um I will say that knowing this, I would diagnose r to your patient with spastic diplegic C P G M F CS two. And just now that we went through the classification, you should have a better mental image of the of him. Even before meeting him, I mentioned um that in terms of the classification by type, more than 80% of the cases of C P are spastic. Um Here's a reminder of the definition most generally, it's an abnormal increase in muscle tone caused by injury at the upper motor neuron pathways regulating muscles. So, since we're talking about upper motor neurons, that's referring to our central nervous system, your brain and spinal cord symptoms include velocity dependent, increased resistance to passive stretch, hypertonia, hyperlexia, clonus, an exaggerated startle or primitive reflexes, pattern movements, loss of selective motor control or weakness. And so this is a kind of busy picture about the path of physiology. Um This is kind of going over normal physiology. So you first start at the level of your spinal cord. Um This picture kind of shows the interplay between your sensory and motor neurons. So when you stretch the propeller tendon, um and so normally you would stretch your patella tendon and get your knee jerk that way. In normal physiology, you hit the patella tendon with a hammer or in my case, I just use my fingers and then that activates your sensory pathways in the red um which then synapses and activates efferent motor neurons or the alpha motor neuron in blue. Um And then that goes to activate your agonous and antagonist muscles. Normally, you have an intact brain above to uh send down inhibitory impulses to the alpha motor neuron to balance out those excitatory sensory signals. But when you have a brain injury, like in C P, the spinal reflex can go haywire. You we talked about like this kind of general um pattern movements. So usually you see these kids have a flexion synergy in a patient with spastic quadriplegic C P. You see starting from above down, below shoulders are abducted and internally rotated. Elbows are flex and pronated. Wrists are flex, ulnar, deviated fingers are flexed and thumbs are fisted and then in the lower extremities, their hips are flex abducted, internally rotated, knees are flexed, ankles are plant, are flexed and inverted. Once you have a rehab diagnosis, we try our best to engage the parents in a plan, making sure that they know that the diagnosis is not just an end point that stops with this visit having rehab early on makes it a start and not a finalizing diagnosis. Rehab can provide the education, get the resources and do some follow up. We want to identify a plan and strategies for early intervention and treatment to empower the parents and the child and set the stage for function and activity success. One of the main things that rehab helps with is plugging patients and families into the appropriate outpatient therapy sources for early intervention in California, we're really lucky to have um some good outpatient resources. So usually if you're dealing with someone younger, I will try to refer them to Regional Center. And then when they get a little older, transition them into California children's services medical therapy program or CCS M T P. Um after they turn three, so I staff a lot of the CCS medical therapy units and I'm most familiar with that system for the Regional Center. There's a lot of um therapists contracted with them. So it's harder for me to um know whom to talk to. And in my opinion, the M T U therapists are really great in providing services to Children with chronic disabilities and determining appropriate D M E. The M T P has its own medical eligibility criteria separate from the general CCS program. It's more narrow in scope and then Children with C P make up the majority of the M T P eligible cli clients, but they open um there's other diagnoses that can um be open. So spina bifida muscular dystrophy, um various neuromuscular or muscular skeletal disorders and anyone can refer um including hos like hospital and clinic personnel, parents, teachers, most referrals are requesting for P T and O T. Um you can also get feeding therapy but there's not straight speech therapy. I mentioned earlier on how the clinical picture of C P evolves with age. So does the management during infancy and early childhood? Um The focus of management. Management is mostly on like motor and cognitive development. So, feeding and nutrition, contracture prevention, spasticity management with medications or injections contract your prevention, adaptive equipment and then hip and spine surveillance to curb or slow down the progression of um those complications. Then as the child enters school age, we encourage families to work with the school on formal cognitive testing and I E P development for the adolescent and young adult, We focus on social adaptation including disabled sports programs, vocational rehab, assessment, and impaired drivers evaluation. Locally, there's BP or Bay Area outreach and recreation program which is really popular for wheelchair basketball and adaptive cycling. Um This was a few years ago but I know one of the M T U supervisor I worked with would lead um a course in adaptive sailing in Half Moon Bay and then one of our senior doctors doctor, he used to take um his patients up for adaptive skiing into the mountains. Finally planning the transition to adulthood is critical and daunting. Um So we try to discuss and develop goals for education, independent living, medical care and conservatorship if appropriate. Oh, I forgot to mention earlier. Doctor Brah also runs the transition to adult clinic quarterly, which is very neat. Um OK, so the management of the child with C P should be carried out as a as a multidisciplinary team approach. I feel really lucky to get to work so closely with Ortho neurosurgeon neurology. Nutrition, all the therapy disciplines, social work and nursing. To address all of their physical medical and social needs. There are reasons to treat spasticity and these include trying to reduce pain from spasms or if there's functional impairment such as problems with ambulation, wheelchair propulsion, school activities, writing or computer interface, we also treat for positioning in standing or in the wheelchair or in the bed. And then if there are care issues with hygiene and dressing, um sometimes we can do it for cosmesis such as decreasing the guard positions or to maybe wean them out of races. Um or for deformity with hip subluxation, scoliosis, race tolerance or contracture. There's also reasons not to treat spasticity. You want to treat the patient, not the provider. Sometimes you need a new patient with spasticity and expect to get rid of it. But there can be side effects to treatment. Sometimes you can make them worse. You've heard of some people having maybe useful tone. So they are trying to compensate for weakness. There are some kids who have some standing balance using their knee extensor tone, some have functional arm positioning using their elbow or shoulder tone and then sometimes it can help with AD L S such as toilet transfers and dressing to help with those standing transfers. Um And then having some sort of tone can help with muscle bulk and prevent D V T S. When it comes to the actual treatment, you can think of a sending a ladder with therapy being less invasive and then each rung being more invasive and get until you get to neurosurgery, which is usually the option of last resort therapies is our default default intervention. The main players include P T O T and speech. How do you know which therapy is the best though? There was a systematic review by Morgan at all in 2016 that looked at 34 studies, 10 of which were randomized control trials. And it showed two types of intervention that had large size effect. Those were the ones that promoted child initiated movement enrichment and task specific training. Therapies can take place in the hospital or clinic in the classroom or in the home therapists use all sorts of modalities including casting compression, constraint, taping, e stem vibration, aquatherapy, hippotherapy, and adaptive sports. So here's a baby with right hemiplegic C P and who's undergoing constraint therapy of the stronger arm to try to get more spontaneous use of the more affected arm. If a patient needs bracing for stretching or support rehab can prescribe it for the orthotist to make most commonly used for the lower extremities is the S M O uh type with hyper to help with hyper pronation like you mean was talking about and then A F OS we use for toe walking or bad knee hyperextension for the upper extremity extremity. We commonly prescribe a who a W H O wrist han orthosis um and then you can also prescribe separately for the wrist and thumb flexion. You'll sometimes see patients with neuromuscular scoliosis in A T L S O. That's your thorac lumbar um Sarel orthosis, sometimes we'll just prescribe it for postural support, like for the kids who have truncal hypotonia and they slump too much in their wheelchair. Um that can sometimes help the CCS therapy team is the best with figuring out what durable medical equipment is, is most appropriate for the patients. So I usually fill out a generic prescription and then they work directly with the patient and the vendor. Um and the family of course, to find the exact exact brand and style that fits into their the patients uh function and space. Um Let's say if a patient is not able to stand by two years of age, we like to put them in a standard to give them some sort of weight bearing through the legs. Give them a different perspective of the world in a more vertical upright posture. Around three years, we most of the kids will have outgrown their commercial strollers. So they'll be assessed for a mobility device, usually either an adaptive stroller or a manual wheelchair. And then there's all types of bathing and toilet equipment to help families as well. Rehab does a lot of medication management in patients with overwhelming spasticity in all extremities that's causing pain, discomfort, caregiver, difficulty worsening contractures, then we'll start medications. Um you might recognize some of these names. So our go to medications are usually baleen Valium, other benzos like Klonopin tiZANidine clone, they all can cause um sedating effects. So we always start low and then titrate to effect. The only med on this list that does not work directly on the nerves is Dantrolene and that works um at the level of the muscle for kids who have certain problem areas there that are particularly tight. We target, we can target those muscles with the chemo denervation injections. So we don't have to oversedate them. Um, with higher doses of the other medications, the most common areas that we inject are the calves for the toe walkers or the hamstrings. Um For those who are getting more crouched at their knees and in our practice, we use Botox and Disport. Sometimes if an A F O or Botox alone is not enough to bring a toe walker's heels down, then we'll use serial casting to address that contracture. We have a casting clinic and then the doc will supervise and the Ortho tech places that hard cast and a rubber shoe for them to walk in and then we change that out every week. Um And we typically have them in the cast for three weeks total, um to try to get their heel down and get their Dorsey flexion above neutral. Um So this is a picture that nicely shows the progression of the ankle starting in plantar flexion and gradually getting to neutral. And then once the patient comes out of that last cast, he transitions into a F OS and then can do a burst of P T for more aggressive stretching and strengthening rehab also uses alcohol injections. We're typically targeting the operator nerves in the growing um in cases of severe scissoring. And when the family is reporting that it's really hard to do diapering or hygiene. Um the alcohol essentially burns the nerve and the pros include immediate onset. So, right, when they come out of the O R, they sh you should be able to see the effects, the low cost and longer duration. We say like it can last 9 to 12 months. However, it has to be done under general anesthesia. So that requires more coordination and risk from anesthesia and it sometimes can cause a burning sensation or hypersensitivity. So for these reasons, a lot of families choose the more popular Botox because of its minimal side effect profile. This is an overlap slide um of working closely with our orthopedic surgeons on opti optimizing the care of our patients with uh soft tissue or bony bony surgeries. And then the focus on like hip surveillance and uh surveillance for scoliosis. This is another plug for using the hip screen app. Again, I use it every day to check out migration percentage when I'm out at the M T U. I'll pull it up to kind of remind myself like based on G M F C G M F CS level. How often am I supposed to be recommending x-rays? So, back to our patient, in his case, his rehab management included bracing and equipment with hinged A F Os, a reverse walker. We didn't end up using Bain since her, his arms were not that affected. He did get Botox to his hamstrings gas drops and push to be to help straighten his legs out and bring his heels down to the floor. Serial casting, helped with contractures and his ankles and then he just was getting regular monitoring for hip and spine um to see if he, when he would need Ortho finally, on patients whom you've tried multiple rungs of that treatment ladder without success. Then neurosurgery can step in by either placing an intrathecal baclofen pump or doing a select dorsal rhizotomy. We're gonna have a whole panel about the rhizotomy later. So you all know that oral bali can cause side effects, that might limit its usefulness such as excessive drowsiness for patients with overwhelming spasticity and likely some bad dystonia as well. We offer the pump placement, neurosurgery places these pumps, but then rehab manages the pumps forever after um for dose adjustments and refills. It's a huge commitment for families and you pretty much have to be close enough to a medical center that you can come in to get it refilled every few weeks to months depending on um how high your dose is how quickly you're burning through the medication. And then after seven years you have to get the battery changed out too. So, it's a lifelong commitment there. So, for patients who are emulators with higher function, like a G M F CS one or two, and maybe they're having worsening gate issues, they're getting more crouched as they get older. Then we offer them the selective dorsal rhizotomy. It's a neurosurgical procedure that selectively divides parts of the dorsal lumbo saro roots of the spinal cord. Here's a picture of the exposed back and the nerve roots before it's dissected into the smaller rootlets and then by testing and snipping the rootlets, you get significant reduction in spasticity without causing paralysis. We screen everyone through our multidisciplinary spasticity clinic over in Oakland. The best candidates for a rhizotomy typically have spastic diplegic C P from prematurity and we aim for kids ages 4 to 11, but we definitely can do it for older Children. They should be cognitively able to follow directions, sequence tasks because they're expected to stay on the um our inpatient rehab unit for 46 weeks, sometimes eight weeks. Um Following the surgery, it's a big hardship for families obviously to stay that long. I think if you were to research other centers in the country who do it, the inpatient rehab stay is not as long, but it's because some of those other centers have access to more frequent outpatient therapies for the kids to transition to like um they can get maybe five days a week. Whereas with what we have available, usually we do the 4 to 8 weeks of inpatient rehab, then they switch to outpatient for six months, expecting to get 2 to 3, most of the time, it's two times a week of therapy. So outside of the standard management, I've talked about there are some cutting edge or emerging interventions. There's robotic assisted therapy for various functional goals um that are emerging such as the robotic trunk support, gate, uh support trainer for independent sitting and then robot assisted gate train for motor control. Our rehab department in Oakland has um invested a lot of money for the state of the art robotics, including the exoskeletal robotic ambulation device put out by this company called Exo Bionics in the Bay area. But you have to be at least five ft tall to use it. So for the smaller population, we purchase this Treo robotic gate trainer and then for the upper extremities you can use. We bought the robotic suite from Tyro Motion which our O T S will use to work with their patients. Um I think that's my last one. I think questions will be later on then. Oh, is it now? Ok. Thank you.