Definition
Cerebral palsy (CP) describes a group of permanent disorders of the development of movement and posture, causing activity limitation, which are attributed to nonprogressive disturbances that occurred in the developing fetal or infant brain.
The motor disorders are often accompanied by disturbances of sensation, perception, cognition, communication, and behavior, by epilepsy, and by secondary musculoskeletal problems.
Incidence
Survival following extreme premature birth has dramatically improved (60% to 90% depending on gestational age and birthweight), but survival without disability remains an elusive challenge for the field.
Cerebral palsy is one of the most frequent major neurodevelopmental impairments following prematurity. The earlier the baby is born, the greater the risk for CP. As a result, the incidence of CP among premature infants is most accurately reported as a range, which is conventionally described as 3-80/1000 neonatal survivors.
Cerebral palsy is in fact the most common physical disability in childhood, occurring in 2-2.5/1000 births. The overall rate has remained remarkably stable for the last 60 years.
When thinking about incidence, it is important to recognize that CP occurs in both preterms (40% of cases) and in term born infants (60% of cases), except the risk is much higher among premature infants.
The rate of CP among premature infants initially increased in the 1970s-1980s as younger and younger children survived extreme prematurity. Credit to advances in neonatal care, the rise in frequency of CP among extremely preterm infants appears to have stabilized and is encouragingly now dipping in some developed countries. Therefore, the rate and epidemiological profile of cerebral palsy is most accurate when consideration is given to the time and place of birth.
Pathophysiology
The cerebral insults that give rise to the motor impairments of cerebral palsy have wide ranging origins including vascular, traumatic, hypoxic-ischemic, metabolic, infectious, and genetic causal pathways.
Injuries can occur both within the white and gray matter of the brain and most often include:
White matter damage or periventricular leukomalacia (PVL) (40% to 60% of cases)
Cortical, subcortical, and deep gray matter lesions (10% to 20% of cases)
Brain malformations (10% of cases)
The brain injury patterns most commonly associated with prematurity include
Intraventricular hemorrhage
Dilated ventricles (ventriculomegaly)
Periventricular leukomalacia (PVL) or white matter injury
A correlation usually exists between the location and size of the injury and the severity of the child’s motor impairment, but this is not always the case. Advanced serial neuroimaging can now provide important prognostic insights about developmental motor outcomes.
Children with brain malformations and cortical/subcortical and basal ganglia lesions generally exhibit more severe motor impairments and have the highest risks for being nonambulatory.
Children with periventricular white-matter lesions generally exhibit milder motor impairments (ie, are usually ambulatory) and also contend with less-associated impairments. However, there is a subgroup of preterm infants with more severe white matter injury who may fair worse than those born at term age, with respect to long-term motor and developmental outcomes.
Three major classifications are used to further demarcate CP
Motor-type
Topography
Function
All of these are more reliable and informative within the follow-up period. In the intensive care period, the risk for developing CP can be broadly detected but the severity and motor-type cannot be fully determined.
Spasticity, dyskinesia, and ataxia will not yet be fully manifested during the birth hospitalization, owing to incomplete axon myelination, basal ganglia neuronal immaturity, and motor control immaturity. Symptoms of spasticity and dyskinesia can continue to evolve up until 2 years of age, with many infants initially classified as hypotonic evolving to become hypertonic. As a result, cerebral palsy registers reconfirm the diagnosis, motor-type, and severity of functional limitations at age five.
If cerebral palsy is detected in the intensive care period, topography cannot be easily determined from physical examination if hypertonia is not yet present, but brain MRI can provide valuable predictive information.
As a guideline for those with CP, unilateral abnormal MRI findings almost always translate into unilateral cerebral palsy (ie, higher likelihood of mild physical impairment), whereas bilateral abnormal MRI findings almost always translate into bilateral CP (ie, higher likelihood of moderate-severe physical impairment).
The diagnostic caveat here is that as many as 25% of children with an abnormal MRI such as PVL will transpire to not having CP. In addition there are individuals with unilateral lesions who do not demonstrate signs of neurological asymmetric dysfunction.
Risk factors
There is very rarely one specific cause of CP and hence the cause of the condition is best described by the phrase: causal pathways to cerebral palsy. Much research has been done to identify the risk factors for CP; however, little is understood about how these risk factors act together to produce a causal pathway to CP.
There are no biomarkers that accurately predict which infants will have CP, and most problematically, clinical risk factors only partially identify subpopulations of infants at risk of CP.
In fact, multiple risks occur across numerous time points in the continuum of conception, pregnancy, birth, and the neonatal period.
The known etiological risk factors for CP among premature infants bare a sizeable overlap with the risk factors for CP in term infants, and have been summarized in Figure 24-1.
In the intensive care period, if any of the following clinical indicators are present, the child’s risk for CP is elevated and further diagnostic investigation is warranted.
Brain injury including intraventricular hemorrhage (IVH) (grade 3 or 4), ventriculomegaly, or cystic PVL
Postnatal sepsis, necrotizing enterocolitis (NEC), or meningitis
Chronic lung disease (CLD) (ie, supplemental oxygen at 36 weeks’ gestation)
Severe retinopathy of prematurity (ROP) (stage 4 or 5)
Multiple births
Clinical presentation
Signs and symptoms
The most common and early recognizable developmental risk factor indicative of one of the cerebral palsy syndromes is difficulty with oral feeding skills.
Oral feeding is an infant’s first voluntary motor skill, and also involves an element of cause and effect.
Poor oral feeding is manifested as sucking and swallowing difficulty, taking longer to feed (eg, more than 15 minutes) for a small volume, and respiratory distress during feeding with desaturations, cough, gagging, or choking.
A key indicator in the developmental progress of feeding skills is the ability to consume an appropriate volume of feeds in a reasonable time so as to promote weight gain and linear growth.
Additional developmental risk factors, which may provide useful information for identifying cerebral palsy early, include
Uncoordinated eye movements or poor visual tracking
Head lag, limited head clearance off the floor in the prone position
Presence of abnormal postures
Many children with CP at 4 to 6 months will demonstrate a constellation of difficulty feeding, difficulty with head control, and an abnormal neurological examination.
For more information on identification of key developmental milestones, refer to http://pathways.org/awareness/healthcare-professionals/early-motor-surveillance/.
Condition variability
Cerebral palsy, as a diagnosis, has considerable variability in severity ranging from a mild impairment in motor skills and posture to a severe disability with global developmental impairments and associated medical and sensory issues.
How to reliably assess the infant
Physically examine a child in a relaxed state, paying special attention to head lag in pull to sit.
Important additional observations include both the infant’s state and their demonstration of an array of neurological skills, including
Tracking 360 degrees
Demonstrating strong sucks
Symmetric facies
Being able to lift head and chest and support weight on wrists or forearms in prone
Desegment roll with avoidance of flipping (early rolling in a nongraded fashion is a red flag)
Become unfisted and demonstrate midline and manipulative hand play and bat at objects, obtain objects, and transfer
The key strategy is to set up an assessment framework for demonstrating an array of gross motor, visual motor, oral motor, and fine motor skills over the first 6 months of life.
The combination of motor delay and a clustering of indicators of abnormalities of tone, reflexes, and postural motor control will help establish the diagnosis of cerebral palsy.
If one does not have voluntary rolling and has delayed motor milestones with an abnormal neurological examination reflecting central nervous system dysfunction, then a diagnosis of cerebral palsy and appropriate family supports and interventions can take place by 6 months for many term infants and by 9 months for preterm infants.
There are three major paths for identifying children with cerebral palsy.
First, children born premature with risk factors, abnormal imaging (including cranial sonograms or MRI), and abnormal quality of movement (eg, difficulty feeding), where lower gestational age elevates the risk for cerebral palsy.
Second, children born at term with neonatal seizures, encephalopathy, and significant motor delay, where increased severity of encephalopathy increases the risk for cerebral palsy.
Third, children with no obvious risk factors and an uneventful maternal and neonatal history but with motor delay identified by their parents or pediatrician at home, constituting 45% of all cases of cerebral palsy.
Diagnosis
For a child to be diagnosed with cerebral palsy, by definition they must have a disorder of posture and movement that limits their ability to perform activities. Activity limitations are of course difficult to observe in newborns, because by nature of their developmental age they have a very limited voluntary motor repertoire and thus it is unclear if they cannot perform an activity or have not yet learned to perform the activity.
Pronouncing the diagnosis of CP has traditionally been a conservative and protracted practice. The average age of diagnosis of CP varies but is usually between 18 and 24 months of age. However, 86% of parents know that their child has CP before the diagnosis is given.
In the intensive care period, it is commonplace for diagnosticians to outline the risks for CP, but rarely do they give the diagnosis at this time unless the child’s condition is so severe that the diagnostician is certain and/or choices about compassionate care are presented.
The reasons for delayed diagnosis are multifactorial.
First, there is no definitive diagnostic test or biomarker that can validate the diagnosis of cerebral palsy.
Most children with CP will have abnormal findings visible on a brain magnetic resonance imaging (MRI), but 12% to 20% of children with CP will have normal brain MRI findings.
Since neuroimaging is not yet foolproof, diagnosticians are lured into a conservative “wait-and-see” approach.
Second, CP is an umbrella description (not truly a diagnosis) for a group of heterogeneous motor impairments arising from multiple causal pathways. So in clinical actuality, the diagnosis of CP should not be given until every differential tested is ruled out, and nothing else biologically explains the persistent symptoms observed in the child (such as neurodegenerative, neuromuscular, metabolic, and genetic conditions).
Third, there is a long history of trusting “quick-and-dirty” neurological observations, such as reflex testing, even though hypertonia may not yet be present and the findings are not a good predictor of functional limitations.
Fourth, teams tend to initially delay the delivery of bad news to spare parents and provide for a possibility that the child will “grow out of it.” Transient dystonias do occasionally occur, and the hope and motivation for a positive outcome may help the family cope with the situation and assist in allowing the child to maximize their potential. However, early motor delays are often a marker that more complex cortical challenges will occur in communication, learning, and adaptive behaviors.
The problem with the conservative “wait-and-see” approach to diagnosis
Infants have an extremely limited voluntary motor repertoire at birth, and it can, therefore, take an inordinate amount of time (when relying on missed motor milestones or the detection of hypertonia) to be convinced that the motor delay is genuine and permanent.
The absence of rehabilitative interventions in the time closest to the brain injury contravenes what is known about promoting neuroplasticity.
The “wait and see” period before diagnosis is stressful and anxiety provoking for families, and has been attributed to parents experiencing higher, not lower, rates of depression.
With pioneering efforts in neuroprotection and regenerative medicine burgeoning, the push is for diagnosis to be made earlier to enable these children to benefit from emergent treatments that may prevent or lessen the severity of the condition or help manage proactively comorbidities of safe feeding, positioning, motor exploration, gentle handling, enriched experiences to promote communicative, social, and regulatory behaviors.
Making the early diagnosis
Since children born premature with abnormal imaging (such as IVH grade 3-4, PVL, ventriculomegaly) are at high risk for cerebral palsy, it is our responsibility to carefully follow and monitor these children for the first 3 to 9 months postdischarge, systematically observing their feeding and voluntary movement skills such as head control. Our task is to sensitively make the diagnosis as early as possible.
Early diagnosis at 12 weeks postterm equivalent age can now be made reliably using a combination of evidence-based assessment techniques; these are described in Figure 24-2. When seeking to make an early diagnosis, identify the following:
History of risk factors for CP (refer to Figure 24-1).
Presence of a brain injury detected using MRI at term equivalent age.
Abnormal quality of movement at 12 to 20 weeks postterm equivalent age, detected using the general movements assessment, where the child lacks “fidgety movements.”
Presence of abnormal neurological signs, bearing in mind that while hypertonia is a highly confirmatory sign it may not be present until later in childhood and may not be consistently demonstrated before the first birthday.
There are clustering of indicators of severe spasticity such as persistent fisting or scissoring when vertically suspended or elicitation of sustained clonus, or persistent equinus during supported lower extremity weight bearing, then spastic cerebral palsy is confirmed.
If there are difficulties with poor feeding skills, difficulty with tracking, and opisthotonic postures in supine or strong and obligatory primitive reflexes that are not being integrated into voluntary motor patterns then extrapyramidal cerebral palsy with combinations of dystonia, rigidity, and multiple neurological system concerns for gross, fine, and oral motor skills is occurring.
The overall goal when providing the early diagnoses to families is to be supportive and link families to informed management resources that get set achievable goals. Our goal is to be proactive and give all children with evolving neurodisability quality supports while not being too pessimistic that we overwhelm them. We should be supportive, positive, and not overwhelming and link families to resources so that they feel empowered.
Discussing the diagnosis with families
Informing a parent that their child has cerebral palsy will probably be a difficult conversation, but it is important that it is done well.
Many parents of children with CP report being dissatisfied with the amount of information they receive at diagnosis and the lack of discussion about the likely impact of the condition on their family.
Parents dissatisfied with the diagnostic process are more likely to experience depression and lasting anger.
Chief criticisms in delivering the news, as reported by parents, are unclear information and conveying a pessimistic future outlook.
When parents are learning a diagnosis, they are in essence, learning from a foreign textbook, containing more information than they can possibly assimilate. Parents’ recall about diagnostic information may not match what professionals attempted to communicate, as parents’ actively block the recall of bad news in order to cope.
Therefore, when communicating news with families, diagnosis needs to be a process, rather than a one-time event. Diagnosticians must recognize that parental acceptance of the CP diagnosis will be a shifting and ongoing process of resolution with continuing dialog needed.
Figure 24-3 is a summary of best-available evidence when giving the diagnosis of CP, about what to share, how to share, and when to share.
Management
Medical management
The early medical management of cerebral palsy requires attention to growth and nutrition and optimizing the child’s physical state (eg, pain management) for learning. More specifically this will involve monitoring weight gain; detecting and managing reflux and constipation; screening for silent aspiration where a child is having recurrent pneumonia; managing reactive airway disease especially in preterms and survivors of persistent pulmonary hypertension; measuring head growth among children requiring shunts using shunt protocol; and monitoring hearing and vision. It is important to recognize that children require a medical home base with access as needed to appropriate pediatric neurology, gastroenterology, ophthalmology, neurodevelopmental medicine, and rehabilitation subspecialists.
Early developmental/therapeutic interventions
In the neonatal period, a number of developmental and therapeutic interventions are offered with the aim of optimizing the infant’s comfort, parental attachment, and developmental outcome. None of these interventions are cerebral palsy specific but it is possible that the combined contribution of these interventions coupled with the medical management strategies previously outlined are helping to contribute to the reduced rate of CP following prematurity observed in recent years. These interventions are, therefore, an integral part of standard care for infants with cerebral palsy. The effectiveness of each of these interventions has been appraised using the GRADE system.
Pain management and environmental enrichment interventions
Intensive care often involves painful life-saving procedures and since sustained pain and stressful environments can have negative consequences for the infant’s neurological and behavioral development, pain management is vitally important. A number of interventions are used and include
Facilitated tucking: Facilitated tucking is where the parent holds the child in a calming flexed position to help reduce pain via a reduction in heart rate and reduction in crying time. Facilitated tucking is supported by high-quality evidence.
Infant massage: Massage seeks to reduce pain via helping the infant to become calmer and more settled. It has been shown to improve growth and reduce cardiorespiratory events in preterm infants. Massage is partially supported by low-moderate quality evidence.
Music: Music played for up to 15 minutes is provided to help the infant relax and better regulate their breathing, leading to pain reduction. Music is supported by very low-quality evidence.
Nonnutritive sucking: Nonnutritive sucking involves providing the infant with a pacifier to suck without breast or formula milk to provide nutrition. Nonnutritive sucking seeks to reduce pain via helping infants becoming calmer and more attentive coupled with a reduction in crying. Nonnutritive sucking is supported by high-quality evidence.
Prone positioning: Positioning the infant in prone is thought to provide abdominal counter pressure that relieves pain. Prone positioning is supported by high-quality evidence but must be monitored carefully as it can elevate the risk for sudden infant death syndrome (SIDS) postdischarge.
Swaddling: Swaddling involves wrapping the infant in a fabric cloth following a painful intervention to reduce pain via a reduction in pulse rate and also to promote self-soothing. Swaddling is supported by high-quality evidence but must be monitored carefully as it can cause hyperthermia and if used in combination with the prone position can elevate the risk for SIDS postdischarge.
Parent-infant attachment and social skills interventions
The physical environment of the intensive care is known to induce stress and disrupt parent-infant attachment. Interventions that seek to foster parent-infant attachments and infant socialization are, therefore, a high priority and include.
Kangaroo care:Kangaroo care is where the preterm infant is held, skin-to-skin, with an adult (preferably the parent) to encourage regular breathing via synchronization with the adult’s cardiac rhythm, socialization, and parent-infant attachment. Kangaroo care is supported by moderate quality evidence.
Massage: Massage seeks to foster the parent-infant attachment via positive touch experiences. Massage is partially supported by low-moderate quality evidence.
NIDCAP (Newborn Individualized Developmental Care and Assessment Program): NIDCAP is a family-centered developmental care program that includes parent training (both within the NICU and home environment) to improve parent-infant interaction quality and foster child development aiming to avoid developmental delays, especially in the cognitive domain. NIDCAP is supported by high-quality evidence but the gains are short term.
Though these interventions are not curative for those with extensive brain injury, they help promote developmental resiliency and appropriate inputs for neuroregulation and neuroplasticity as well as decrease child stressors and adverse consequences of a revved up hypothalamic pituitary axis.
Prognosis
Early predictors
At the time of diagnosis, parents of children with cerebral palsy will almost always ask, “Will my child walk?” and “Will my child talk?” in effort to understand how “bad” it is. Accurate individualized answers will depend on the severity of infant’s physical disability, the type of motor impairment, and the presence of comorbid conditions, most of which may well be very difficult to determine during the neonatal period.
Despite this, parents can and should be given a general evidence-based overview of how children with cerebral palsy usually progress. Keep in mind most parents will know very little about CP, and the general community attitude is that CP is a very unacceptable condition. Many parents are likely to assume and fear that their child with CP will be “wheelchair bound” with an intellectual disability. In addition, they may worry about life expectancy.
All children with cerebral palsy will have a motor impairment, but rarely does the motor impairment exist in isolation.
Cerebral palsy is frequently accompanied by co-occurring impairments, diseases, and functional limitations, all of which affect a child’s prognosis and independence.
The co-occurrence of epilepsy and intellectual disability in combination with severe physical disability affects prognosis and life expectancy the most.
Some of the most underrecognized but treatable functional limitations co-occurring with cerebral palsy include
Chronic pain (affecting 75%)
Behavior disorders (affecting 25%)
Sleep disorders (affecting 20%)
Physicians should routinely screen for these clinical problems and arrange for specific treatments, as this can substantially improve the child’s outcome, coping, and quality of life.
A meta-analysis was conducted across international cerebral palsy registers to summarize the rates of co-occurring impairments, diseases, and functional limitations and translated into parent-friendly clinical prognostic messages for communicating to parents. A summary of the clinical recommendations stemming from the meta-analysis is depicted in Figure 24-4.
Outcomes
It is natural that parents will want to do everything they possibly can to ameliorate the child’s condition, but it is important for parents to understand that cerebral palsy currently has no cure. We can comfort parents in the difficult task of coming to terms with having a child with a life-long disability by:
Helping to maintain hope: Contrary to popular belief, mild cerebral palsy is more common than severe cerebral palsy. Knowing this fact can offer parents hope. In addition, inform parents that substantial neuroprotection and neuroregenerative research is underway investigating the potential cures.
Informing parents that all children learn: Good parenting and environmental enrichment such as reading, all help to improve outcomes. Prompt parents to promote literacy and academic development.
Teaching parents to focus on function, adaption, and quality of life: Adults with cerebral palsy prioritize development of communication, self-care, independence, education, friendships, and community participation. Therefore, coach parents to be mindful of their child’s function long term rather than pursuing overly ambitious physical feats. Encourage parents to incorporate key therapeutic techniques into children’s activities of daily living, to build the child’s strengths, and to have fun. Parents can be reassured that severity of physical disability does not predetermine quality of life.
Discharge
Since children with cerebral palsy by definition have a life-long disability rather than an illness, the term “convalescent care” is not on the whole applicable. If the infant has progressed from intensive care but is still hospitalized, this is more likely to be due to a coexisting condition such as CLD, poor feeding, ROP, etc, than the motor disorder per say.
Teaching
Parents should be educated about their child’s current condition and comorbidities acquired during their NICU stay, as well as expected and possible outcomes.
Parents should be instructed how to give medications prescribed at discharge and told what condition they are treating. They should understand that if the symptoms worsen (muscle tone, reflux, seizures, etc) postdischarge that the medicine may need to be weight adjusted.
Parents should meet with a physical therapist and learn stretching/strengthening exercises applicable to their child. Also, consider teaching the parents infant massage if the child is hypertonic or very irritable.
Monitoring
Consider a home apnea monitor for infants with persistent apnea and bradycardia spells related to severe GERD or poorly controlled seizure disorder.
Infants with severe brain injury should have their hypothalamic-pituitary-adrenal axis assessed prior to discharge, including thyroid function tests (TSH, FT4), fasting cortisol and ACTH, and electrolytes to assess for diabetes insipidus.
Infants with severe brain injury should demonstrate stable temperature regulation, without an external heat source, for several days prior to discharge.
Safety
Caregivers should be instructed in infant CPR.
Infants should have a car seat safety test for at least 90 minutes prior to discharge. Infants with poor head control at discharge may need to use the manufacturer’s insert to provide lateral head support.
An emergency plan should be established, instructing what to do (give antiepileptic, call PCP, call neurologist, call 911, etc) if the child has symptoms of shunt infection/malfunction or has a seizure and what medical facility to bring them to.
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