Cerebral palsy: Overview of management and prognosis

INTRODUCTION — Cerebral palsy (CP) refers to a heterogeneous group of conditions involving permanent motor dysfunction that affects muscle tone, posture, and/or movement. These conditions are due to abnormalities of the developing fetal or infant brain resulting from a variety of non-progressive causes. Although the disorder itself is not neurodegenerative, the clinical expression may change over time as the central nervous system matures. The motor impairment results in limitations in functional abilities and activity, which can vary in severity. Multiple additional symptoms often accompany the primary motor abnormalities, including altered sensation or perception, intellectual disability, communication and behavioral difficulties, seizures, and musculoskeletal complications [1].

An overview of the management and prognosis of CP is reviewed here. Detailed discussion of the treatment of spasticity and dystonia and orthopedic interventions in children with CP is provided in a separate topic review. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues" and "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues", section on 'Physical and occupational therapy'.)

The epidemiology, etiology, prevention, classification, clinical features, evaluation, and diagnosis of CP are also discussed separately:

●(See "Cerebral palsy: Epidemiology, etiology, and prevention".)

●(See "Cerebral palsy: Classification and clinical features".)

●(See "Cerebral palsy: Evaluation and diagnosis".)

TREATMENT GOALS — Interventions for CP should be started early, at the time of suspected diagnosis, to maximize the child's functional abilities and independence while reducing the extent of disability [2,3]. Management includes interventions that specifically address the child's motor impairment (eg, spasticity treatments, orthotics, physical and occupational therapies, orthopedic surgery) and optimal treatment of medical comorbidities [4]. The care plan should promote the child's participation, communication, education, community engagement, and social and emotional development. Therapeutic and educational supports and family engagement play a significant role in promoting development.

Holistic management of the child with CP requires a multidisciplinary team to address the multiple medical, social, psychologic, educational, and therapeutic needs. Team members should have a goal-oriented approach that is based upon an understanding and appreciation of contributions from all the disciplines. A multidisciplinary clinic that allows the child to be seen by different providers together at the same visit is ideal; however, if such a clinic is not available, a team can be put together "virtually" with communication between key members. The team should set functional goals for the child that are realistic and family centered. Goals should be periodically reevaluated. Participation of the principal caregivers, including community-based support such as teachers, therapists, and home nurses, in treatment plan development is essential [3].

FUNCTIONAL EVALUATION — Assessment of motor and other impairments for individuals with CP helps to specify, communicate, and monitor symptoms and response to interventions.

Functional classification systems — Standardized measurement of an individual's overall functional status can help guide treatment selection and allows for monitoring of change over time. The Gross Motor Function Classification System (GMFCS) is the most widely used for assessing and monitoring overall functional status. Several other classification systems are also available to help assess specific functional domains for children with CP. (See "Cerebral palsy: Classification and clinical features", section on 'Functional classification systems'.)

Assessment of tone — Impairment of muscle tone is a key feature and therapeutic target of CP. Clinical scales that describe impairments of muscle tone include:

●Spasticity – The Modified Ashworth Scale (table 1), which is used for patients with spasticity [5], is useful for assessing specific muscle groups before and after an intervention, such as botulinum neurotoxin or systemic tone medications, although the reliability characteristics of the test are imperfect [6].

●Dystonia/dyskinesia – The Barry Albright Dystonia scale was designed specifically to measure dystonia for individuals with CP [7,8]. Other tools commonly used for dystonia include the Burke-Fahn-Marsden dystonia rating scale (BFM), which assesses the severity and burden of dystonia, and the Dyskinesia Impairment Scale, which assesses dystonia and choreoathetosis [8].

●General hypertonia – The Hypertonia Assessment Tool can help to differentiate among spasticity, rigidity, and dystonia in children [9].

Scales to categorize overall functional effects of impaired muscle tone are described separately. (See "Cerebral palsy: Classification and clinical features", section on 'Functional classification systems'.)

Monitoring function over time — While GMFCS levels do not typically change over time after age 5, motor function can change. This reflects interplay among a number of factors, such as evolving contractures, effect of treatments, and the impact of weight gain or change in body habitus as an individual reaches adult stature. Interventions such as physical therapies, bracing, and surgeries can improve a child's motor functioning but usually do not improve their GMFCS level. An unanticipated decline in GMFCS level should generally prompt further medical evaluation.

TECHNOLOGIC SUPPORT — Technologic aids provide important support for patients with CP, especially those with severe motor impairment [4]. Equipment such as motorized wheelchairs, switch-based devices used to activate communication systems, voice-activated computers, and other environmental control systems can improve quality of life by enhancing functional abilities and social interactions [10]. Many centers offer specialized clinics for evaluation of equipment and/or augmentative communication needs. These can be invaluable resources to the primary care provider in identifying the most appropriate piece of equipment for the child and can also assist the family in navigating the complex insurance regulations that can impact the timing and range of equipment available. (See 'Speech and communication' below.)

FAMILY HEALTH, SOCIAL, AND PSYCHOLOGIC SUPPORT — When a child has CP, social and emotional support for the entire family is essential, as it is for any family with a child with a chronic medical condition [3]. Parents or caregivers of a child with CP may experience chronic grief, as well as guilt, frustration, denial, anger, resentment, and embarrassment [11,12]. They may limit themselves socially, and relationships between spouses can become strained. They may find it difficult to devote as much time to their other children as they do to their child with CP. Siblings of children with CP may also need direct support.

Information about their child's condition should be provided to parents and caregivers with honesty and sensitivity as soon as the diagnosis is suspected. The process of evaluation and management should be described with the understanding that repetition may be necessary because they may not assimilate all the information during the first visit [13,14]. A second opinion should be offered if appropriate.

Clinicians should avoid being dogmatic or providing specific long-term prognoses, particularly around a child's functional abilities, early on. Parents and caregivers should be given the opportunity to ask questions and express their feelings. Coping strategies can vary widely. Some may feel a need to attribute CP to a specific cause, even when the medical evaluation has not revealed one. Others may feel a misplaced sense of guilt because they fear something they did may have caused the child's CP. It is important to attempt to relieve feelings of guilt when they are encountered. Counseling should be an ongoing process. All caregivers should be informed of available counseling services for their child with CP, their other children, and/or themselves.

Resources for children with CP and other disabilities are available in many communities, including support groups for families and siblings. In addition, specialized recreational programs are often available to permit participation in sports or other activities. For many children with CP, some form of sports participation is often feasible and has important benefits, including minimizing the deconditioning that can be caused by impaired mobility, optimizing physical functioning, promoting socialization, and improving self-esteem [15,16]. Even for the child with more profound motor involvement, there are many opportunities for recreational involvement, such as adaptive skiing, adaptive baseball (eg, Challenger baseball), and bowling. These activities can be extremely important in terms of enhancing a child's social participation.

MANAGEMENT OF ALTERED MOTOR FUNCTION — By definition, all children with CP have altered motor function to some extent, affecting posture, coordination, and balance. Treatment interventions should be offered from least to most invasive.

●Physical and occupational therapy – Physical therapy is an established part of treatment programs for CP and is an integral and vital part of management. Occupational therapy is often equally important and integrated into the treatment plan, with a focus on self-help skills and fine motor tasks that are important for school success. Physical and occupational therapy for children with CP is discussed in detail separately. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues" and "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues", section on 'Physical and occupational therapy'.)

●Orthotics and other devices – Numerous devices are available to help promote function, mobility, and participation for children with CP. These include orthotics, standers, seating systems, and mobility devices. This is discussed in greater detail separately.

●Spasticity – Treatment of spasticity in patients with CP may be systemic through the use of oral antispasticity medications (eg, baclofen, benzodiazepines) and/or through focal chemodenervation with targeted botulinum neurotoxin (BoNT) and/or phenol injections. Selective dorsal rhizotomy, which targets spasticity in the lower extremities, is an option for some patients depending on the overall functional goals. Placement of an intrathecal baclofen pump is generally reserved for patients with severe spasticity refractory to medical management. These interventions are discussed in greater detail separately. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues", section on 'Spasticity'.)

●Dystonia – Many oral agents have been used to treat dystonia, but they have not been extensively studied in rigorous controlled trials [17]. The available evidence suggests that several drugs may be beneficial in some patients with dystonia (table 2). However, the therapeutic window for most of the oral agents is narrow, and side effects frequently limit clinical benefit. BoNT injection is an option for patients with focal dystonia. Deep brain stimulation is rarely used. The approach to treating dystonia is reviewed in detail separately. (See "Treatment of dystonia in children and adults".)

ORTHOPEDIC INTERVENTIONS — Common orthopedic problems in children with CP include contractures; hip dysplasia, subluxation, and dislocation; foot and hand deformities; and progressive neuromuscular scoliosis. Assessment and management of these issues are discussed separately. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues", section on 'Orthopedic assessment and interventions'.)

ASSOCIATED CONDITIONS

Intellectual disability — Children with CP are at risk for global developmental delay, intellectual disability, and learning disabilities. Longitudinal assessment of cognitive and adaptive skills should be coupled with individualized interventions directed at advancing and strengthening functional progress. Assessment and management of developmental delay and intellectual disability are reviewed in detail separately. (See "Intellectual disability (ID) in children: Clinical features, evaluation, and diagnosis" and "Intellectual disability (ID) in children: Management, outcomes, and prevention".)

Dysarthria and other motor impairments of bulbar function can complicate assessment of cognitive abilities in individuals with CP. Evaluation for intellectual disability in patients with CP is distinct from evaluation of speech and bulbar dysfunction. (See 'Speech and communication' below.)

Epilepsy — Epilepsy occurs in one-quarter to one-half of patients with CP [18-20]. The risk of epilepsy may be higher in patients with CP due to chromosomal abnormalities and brain malformations than other causes [21]. Seizures may be more common in patients with spastic quadriplegia and acquired hemiplegia than those with symmetric spastic diplegia and CP that is mainly athetoid [22,23]. Management of epilepsy is discussed in detail separately. (See "Seizures and epilepsy in children: Initial treatment and monitoring".)

Vision problems — Vision problems occur in approximately one-third to one-half of children with CP, and approximately 10 percent are blind [24,25]. Visual impairment has been associated with CP due to neonatal stroke, hydrocephalus, and infection [21]. Visual problems may include strabismus, refractive errors, amblyopia, visual field defects, and cerebral visual impairment (CVI).

All children with CP should have baseline ophthalmologic assessments and longitudinal monitoring. Children with complex visual problems, including those with CVI, should be assessed by a neuro-ophthalmologist and functional visual assessment. Functional visual assessment can be extremely helpful in delineating a child's functional skills and providing guidance for classrooms and therapeutic treatments. Management of these issues is discussed separately. (See "Vision screening and assessment in infants and children" and "Refractive errors in children" and "Evaluation and management of strabismus in children" and "Amblyopia in children: Management and outcome".)

Hearing loss — Hearing loss occurs in approximately 5 to 15 percent of children with CP [26]. Early diagnosis and treatment of hearing loss in infants at risk may improve learning and language development. Management of hearing loss in children is discussed in detail separately. (See "Hearing loss in children: Treatment".)

Speech and communication — Disorders of speech and language, including impairments in expressive and written language as well as dysarthria, occur in up to two-thirds of children with CP; approximately one-quarter are nonverbal [27].

The use of augmentative communication strategies for individuals who are nonverbal or have diminished language skills can be helpful in promoting communication and decreasing frustration related to communication deficits. There is good evidence that the use of such technologies does not interfere with the development of language skills [28]. A range of strategies with varying technical complexity can be used, ranging from picture symbols to voice output devices [29]. Strategies must be individualized and appropriate for the individual's cognitive and motor capacities.

Evaluation and treatment of speech and language disorders in children is discussed in greater detail separately. (See "Evaluation and treatment of speech and language disorders in children".)

Growth and nutrition — In general, children with CP follow a different growth trajectory than typically developing peers and tend to be shorter in stature.

●Routine assessment – Children with CP require regular assessments of feeding skills and nutritional status. Weight and stature should be monitored longitudinally, although stature may be inaccurate due to contractures and may give rise to inaccurate body mass index [30]. Triceps folds can be monitored for a more accurate assessment of nutritional status. Standardized growth charts for children with CP are recommended for accurate longitudinal monitoring. It is important to note that they do not necessarily reflect optimal growth of children with CP but rather reflect population norms within each Gross Motor Function Classification System (GMFCS) subset height measurements. (See "Cerebral palsy: Evaluation and diagnosis", section on 'Nutrition and growth'.)

●Feeding difficulties – Feeding difficulties are very common in children with CP since many affected children have some degree of pseudobulbar palsy, which affects oromotor function and can, in turn, affect overall nutrition.

Risk for aspiration should be screened at all well visits. This includes questions about choking, coughing, gagging, or color change associated with eating. Extended length of time required for meals can be an indicator of risk for suboptimal nutritional intake. (See "Aspiration due to swallowing dysfunction in children".)

Problems with feeding influence morbidity and mortality because of poor nutrition and/or chronic aspiration and lung disease [31,32]. In patients with growth failure or chronic aspiration, alternative feeding methods may be needed.

●Enteral feeding – Feeding via enteral tube (gastrostomy, gastrojejunostomy) can improve nutritional status in children with CP and growth failure [33-37]. This intervention can also reduce aspiration risk and provides a reliable route for medication. It improves the quality of life for both the child and family in the majority of cases [38]. The optimal timing for this intervention is uncertain. Early gastrostomy tube placement (ie, prior to the development of malnutrition or aspiration pneumonia) might prevent these complications; however, it is unclear if this would improve long-term outcomes [31].

Successful enteral feeding can lead to excessive accumulation of adipose tissue in children with CP [39]. Caloric goals should initially be modest and subsequently adjusted to maintain an adequate bodyweight (eg, for children with GMFCS level V, maintaining weight above the 20^th percentile for the GMFCS level V-specific growth chart) [40]. Children with severe CP often have reduced energy needs because of inactivity, but some children have increased energy needs because of spasticity or dystonia. (See "Overview of enteral nutrition in infants and children".)

Gastrointestinal disorders — Disorders of dysmotility and other gastrointestinal disorders are common in children with CP. Management of these issues is discussed in separate topic reviews:

●Dysmotility and delayed gastric emptying (see "Treatment of gastroparesis")

●Constipation (see "Chronic functional constipation and fecal incontinence in infants, children, and adolescents: Treatment")

●Gastroesophageal reflux (see "Management of gastroesophageal reflux disease in children and adolescents")

Respiratory disorders — Chronic pulmonary disease is a leading cause of morbidity and mortality among patients with severe CP (see 'Survival' below). Management of these issues is discussed in separate topic reviews:

●Recurrent aspiration (see "Aspiration due to swallowing dysfunction in children")

●Inflammatory lung disease/reactive airways (see "An overview of asthma management")

●Restrictive lung disease due to hypoventilation, scoliosis, and chest wall deformity (see "Chest wall diseases and restrictive physiology")

●Hypoventilation (see "Disorders of ventilatory control", section on 'Neuromuscular disease' and "Respiratory muscle weakness due to neuromuscular disease: Clinical manifestations and evaluation")

●Obstructive sleep apnea (see "Management of obstructive sleep apnea in children")

●Laryngomalacia (see "Congenital anomalies of the larynx", section on 'Laryngomalacia')

Sialorrhea — Oromotor dysfunction in children with CP often leads to drooling. Drooling may be anterior, which is characterized by saliva spilling out of the mouth, or posterior, which is not visible and results in pooling of secretions in the posterior oropharynx. Patients with anterior drooling may experience skin breakdown and social challenges. Those with posterior drooling are at risk of aspiration. Many children with CP have both anterior and posterior drooling.

Approaches to improve drooling most commonly include behavior therapy, medication, salivary gland botulinum neurotoxin (BoNT) injections, and surgery. Drooling improves with age in some patients. Behavioral and pharmacologic management (usually with an anticholinergic agent such as glycopyrrolate) are generally trialed before considering more invasive treatments. A care pathway for sialorrhea management can be found on the American Academy of Cerebral Palsy and Developmental Medicine (AACPDM) website [41].

●Behavior therapy – A trial of behavioral therapy is appropriate for children who are able to understand commands and cooperate with training. This consists of oral awareness and oral motor skills training, designed to improve lip and jaw closure and tongue movements with positive reinforcement for swallowing [42-45].

The oral motor skills program is often initially taught by a specialist in speech and language pathology and implemented at home by the child's caretakers. Measures to improve the child's posture also may reduce drooling.

The evidence supporting behavioral therapy for drooling consists of controlled trials with important methodologic limitations, several small case series, and case reports, which have reported on a variety of oral motor exercises [44-46]. Most studies reported improvements in drooling frequency or severity and/or quality of life.

●Pharmacologic therapy – Medications for drooling include anticholinergic agents and BoNT injection into the salivary glands:

•Anticholinergic agents, such as glycopyrrolate, scopolamine (hyoscine), benztropine, and trihexyphenidyl, act by decreasing the flow of saliva [47,48]. Glycopyrrolate and scopolamine are the agents used most commonly [49,50]. Potential side effects of anticholinergic agents include swallowing difficulties due to sticky secretions (xerostomia), constipation, urinary retention, hyperactivity, and blurred vision.

In a randomized trial comparing glycopyrrolate and scopolamine (hyoscine) patch in 90 children with CP-related sialorrhea, both medications reduced drooling severity compared with baseline and the degree of improvement was similar with both agents [51]. Scopolamine was associated with more problematic side effects such that only 55 percent of patients were able to continue on treatment to the end of the 12-week study period compared with 82 percent of those in the glycopyrrolate group.

•BoNT injection into the salivary glands also decreases saliva flow. In an open-label clinical trial, drooling severity was reduced in approximately one-half of patients after both scopolamine and BoNT; however, fewer and less severe adverse effects were noted with BoNT [52]. In another study, the effect of BoNT waned over time, lasting a median of 22 weeks [53]. Approximately 25 percent of initial responders still had a clinically significant response at 33 weeks after BoNT injection.

A 2022 systematic review of 27 studies (including 5 clinical trials) found efficacy of several anticholinergic medications for sialorrhea in children including glycopyrrolate, scopolamine, trihexyphenidyl, benztropine, and atropine [54]. Another systematic review of six clinical trials on sialorrhea in children with CP reported efficacy and safety for glycopyrrolate, scopolamine, benztropine, and BoNT injection [55]. Both systematic reviews showed measurable reductions in various measures of salivary flow and/or severity of drooling; however, the included studies had important limitations, including small size, lack of blinding, and limited follow-up.

●Surgery – For patients with symptoms refractory to other measures, surgical options include repositioning of the submandibular ducts and unilateral ligation of a parotid duct and/or excision of the sublingual gland [56-59]. Surgery reduces drooling frequency and severity in 75 to 90 percent of patients, but complications such as ranula (pseudocyst) formation, dry mouth, difficulty with swallowing, and changes in the consistency of oral secretions are common and need to be considered in decision-making [56,58,60].

•Repositioning the salivary glands may be associated with longer benefit than BoNT treatment [60]. However, it may increase the risk of aspiration among patients with a high risk for aspiration (ie, those with poor swallowing function).

•Submandibular duct ligation appears to be more effective than BoNT injection therapy, but it is associated with greater risk of adverse events [61,62]. In a randomized trial involving 53 children and adolescents with sialorrhea secondary to CP or other nonprogressive neurodevelopmental disorders, more patients assigned to submandibular duct ligation achieved ≥50 percent reduction in drooling symptoms at 32 weeks compared with those assigned to BoNT injection (63 versus 27 percent, respectively); however, adverse events were more common with duct ligation (41 versus 19 percent) [61]. A follow-up report from the same trial reported that both interventions had positive effects on daily care, social interactions, and self-esteem, but patients assigned to duct ligation reported greater improvements over a longer duration [62].

•Excision of submandibular gland has been associated with favorable effects compared with duct ligation or BoNT treatment. In a retrospective cohort of children at risk for aspiration due to posterior drooling, excision of the submandibular gland was more effective for improving the severity of drooling than either duct ligation or BoNT injection at 32-week follow-up [63]. Recurrent respiratory infections were reduced in all groups and adverse events were uncommon but included bleeding in the surgical groups and thickened saliva in the BoNT group.

Bone health — Children with CP who are non-weightbearing are at risk for low bone density. The risk is further increased by factors such as poor nutrition or certain medications, particularly some antiseizure medications. In the setting of bone fragility, children with CP are at increased risk for fractures (those that occur with minimal trauma). All nonambulatory children with CP and those on chronic antiseizure medications should be screened regularly with 25-hydroxyvitamin D levels and should be supplemented if a deficiency is identified. Calcium intake should be reviewed to ensure dietary guidelines are being met. Weightbearing activity, such as using a stander, should be incorporated into the daily routine of all nonambulatory children with CP. Dual-energy radiography absorptiometry (DXA scan) should be obtained if there has been a fragility fracture to help further inform management.

Treatment with bisphosphonates is generally reserved for pediatric patients with severe reductions in bone mineral density and pathologic extremity fractures or vertebral compression. In a small randomized trial, treatment with high doses of intravenous pamidronate for 12 months increased bone mineral density in children with quadriplegic CP [64]. Lower doses also appeared to be effective in an open-label trial [65]. Systematic reviews concluded that there is low-quality evidence that bisphosphonates may improve bone mineral density or reduce fracture rates; however, there are some concerns that pamidronate and other bisphosphonates may make bones more brittle, and information about long-term risks of these drugs is lacking [66,67]. Weightbearing regimens and supplementation of calcium and vitamin D may improve bone mineral density, but the evidence is limited [66,68-72].

Additional information on management of osteoporosis in children with CP is available through the AACPDM website.

Skin integrity — Patients with CP, particularly those with severe CP (GMFCS levels IV and V), are at risk for skin breakdown and pressure ulcers, which can negatively impact their overall health and quality of life. Preventive measures include proper skin care, positioning, pressure support devices, and careful monitoring and adjustment of braces and other equipment with regard to size and fit. Caregivers of children at high risk for pressure injury should be instructed to conduct daily skin assessments to promptly identify and treat areas of skin breakdown. These issues are discussed in greater detail separately. (See "Prevention of pressure-induced skin and soft tissue injury" and "Clinical staging and general management of pressure-induced skin and soft tissue injury".)

Urinary control — Though urinary incontinence is common among patients with CP, many achieve functional bladder control. Children with CP generally take longer to achieve daytime bladder control compared with typically developing children. Parents and caregivers should be counseled that toilet training may need to be provided over a longer period of time than is usual. (See "Toilet training".)

The approach to managing urinary incontinence in patients with CP is similar to that of patients without CP. The treatment of choice for detrusor overactivity is anticholinergic medication. However, this must be done judiciously, with careful monitoring of residual urine to prevent the development of retention. (See "Management of bladder dysfunction in children", section on 'Anticholinergic agents'.)

For children with CP who have features of a neurogenic bladder, management depends on whether the bladder is underactive or overactive and whether the child has had recurrent urinary tract infections. Management may include anticholinergic medication, alterations of the toileting schedule or environment, and/or clean intermittent catheterization [73]. The medical management of neurogenic bladder in the setting of CP is similar to that in patients with myelomeningocele, which is discussed in greater detail separately. (See "Myelomeningocele (spina bifida): Urinary tract complications".)

Sleep disorders — Children with CP have high rates of disordered sleep. This may be due to behavioral issues, as is common in typically developing children. However, children with CP often have medical contributors to the disrupted sleep pattern, including dysfunctional circadian rhythms (eg, caused by low vision), obstructive sleep apnea, hypoventilation from either a central etiology or muscle weakness, gastrointestinal discomfort from gastroesophageal reflux or constipation, spasticity, and/or sleep disruption from chronic pain of a variety of etiologies.

Treatment generally is targeted to address the underlying concern and may include interventions to improve in sleep hygiene, parental education, spasticity management, a range of pharmacologic interventions (eg, melatonin, omeprazole), and/or noninvasive ventilatory support (eg, for treatment of hypoventilation or obstructive sleep apnea) [2,74,75]. Evaluation of contributing factors and treatment of a child's disturbed sleep patterns should be initiated as soon as the problem is identified, ideally before behavioral changes and scholastic difficulties arise. Referral to a sleep specialist may be warranted in some cases. Management of these issues is discussed in detail separately. (See "Overview of circadian rhythm sleep-wake disorders", section on 'Management' and "Management of obstructive sleep apnea in children" and "Congenital central hypoventilation syndrome and other causes of sleep-related hypoventilation in children", section on 'Management'.)

Psychiatric disorders — Anxiety, depression, and attention deficit hyperactivity disorder occur at higher rates in children with CP compared with the general population [76]. (See "Anxiety disorders in children and adolescents: Epidemiology, pathogenesis, clinical manifestations, and course" and "Pediatric unipolar depression: Epidemiology, clinical features, assessment, and diagnosis" and "Attention deficit hyperactivity disorder in children and adolescents: Clinical features and diagnosis".)

Chronic pain — Many children with CP experience chronic pain that limits their activities [77]. Pain is strongly associated with poor quality of life. Pain in children with CP may go unrecognized due to communication difficulties, and therefore it is important for clinicians to have a high index of suspicion in nonverbal children, including asking caregivers about their concern for chronic discomfort. Hip dislocation/subluxation, dystonia, gastroesophageal reflux, and constipation are commonly reported causes of pain.

Management of pain in children with neurodevelopmental disorders is discussed in greater detail separately. (See "Pain in children: Approach to pain assessment and overview of management principles", section on 'Neurologic impairment'.)

PROGNOSIS

Survival — The majority of children with CP survive to adulthood [78-80]. Life expectancy for individuals with CP is generally lower than for the general population, though this depends on the severity of impairment. Respiratory disease, often aspiration pneumonia, is the most common cause of death [81].

In a report of life expectancy and predictors of mortality in individuals with CP who received services in California from 1983 to 2010, survival in high-functioning individuals was similar to the general population [82]. However, life expectancy was substantially reduced in the most severely affected children. Among children who could not lift their heads when lying in the prone position, the median age at death in 1983 was 10.9 years, which improved to 17.1 years in 2010.

Similar results were noted in a population study from Sweden, in which survival to age 19 years was 100 percent for those with mild gross motor deficits (Gross Motor Function Classification System [GMFCS] levels I and II) compared with 60 percent among children with severe CP (GMFCS level V) [80].

In a report from France that included >3000 individuals with CP who died between 2000 and 2008, the median age of death was 45 to 54 years [81].

In a regional analysis of children with CP who were born in various regions of the United Kingdom between 1980 and 1996, 20-year survival ranged from 85 to 94 percent [79]. In multivariate analysis, survival was related to severity of impairment, birth weight, and socioeconomic status, with the number of severe impairments having the greatest effect.

Motor impairment — The extent of motor impairment is difficult to predict in young children, except at the extremes of mild or severe involvement. In general, prognosis for motor function depends upon the type of CP, rate of motor development, presence of developmental reflexes, and intellectual ability. In a meta-analysis of observational studies of children with CP, several factors were associated with good prognosis for achieving independent walking [83]. These included:

●Sitting independently by two years

●Absence of visual impairment

●Absence of intellectual disability

●Absence of history of seizure or epilepsy

Early predictors of walking were illustrated in a retrospective study of 5366 children with CP who were nonambulatory at two years of age [84]. By six years of age, 18 percent of children could walk with support and 13 percent could walk independently. Predictors of ambulation included the ability to sit and pull to standing by two years of age, cerebral palsy type other than spastic quadriparesis, and preserved visual function.

In a prospective longitudinal study in which 233 children with CP who were followed from 12 months to 3 to 11 years of age, some degree of functional walking was eventually attained by 79 percent [85]. The ability to sit by age 2 years was a highly reliable predictor of walking. However, more than one-half of those who eventually walked did not sit by two years. Among the latter, suppression of primitive reflex activity between 18 and 24 months predicted eventual walking.

Motor development curves can be used to predict the rate and extent of motor development based on the child's age and current level of function [86]. These tools are based on a longitudinal study in which 657 children with CP, aged 1 to 13 years, were followed serially for four years using the Gross Motor Function Measure-66 (GMFM-66), a validated measure of motor function. These curves can be used to guide interventions and monitor progress over time, similar to growth curves for weight and height.

When discussing motor ability with parents or caregivers of a child with CP, it is important to stress that every child is different and that the most important predictor is the child's progress over time. Although predictions of motor development provide important prognostic information, they should understand that considerable variability occurs within each severity group.

Quality of life — Quality of life is subjective and difficult to measure in children who are not able to communicate. Parent-reported child quality of life is lower in children with low motor function, low intellectual and adaptive ability, and high parental stress [87]. In addition, chronic pain is strongly associated with poor quality of life [87,88].

Importantly, among 8- to 12-year-old children who can self-report, quality of life is similar to that of children in the general population [89]. However, adolescents with CP report significantly lower quality of life in the social support and peers domain than the general population [88]. Additionally, among young adults with CP, general health-related quality of life may be similar to the population as a whole, but physical and mental health variables are lower in some individuals [90]. Emotional and social support may improve quality of life among children and adults with CP who report impairment in variables such as pain, fatigue, or depression [91,92].

SOCIETY GUIDELINE LINKS — Links to society and government-sponsored guidelines from selected countries and regions around the world are provided separately. (See "Society guideline links: Cerebral palsy".)

INFORMATION FOR PATIENTS — UpToDate offers two types of patient education materials, "The Basics" and "Beyond the Basics." The Basics patient education pieces are written in plain language, at the 5^th to 6^th grade reading level, and they answer the four or five key questions a patient might have about a given condition. These articles are best for patients who want a general overview and who prefer short, easy-to-read materials. Beyond the Basics patient education pieces are longer, more sophisticated, and more detailed. These articles are written at the 10^th to 12^th grade reading level and are best for patients who want in-depth information and are comfortable with some medical jargon.

Here are the patient education articles that are relevant to this topic. We encourage you to print or email these topics to your patients. (You can also locate patient education articles on a variety of subjects by searching on "patient education" and the keyword[s] of interest.)

●Basics topic (see "Patient education: Cerebral palsy (The Basics)")

SUMMARY AND RECOMMENDATIONS

●Treatment goals – Interventions for cerebral palsy (CP) should start at the time of suspected diagnosis to maximize the child's independence in daily functional activities and reduce the extent of disability. The care plan should promote the child's participation, communication, education, community engagement, and social and emotional development. (See 'Treatment goals' above.)

●Functional evaluation – Standardized measurement of motor function and assessment of tone can help to guide treatment selection and allow for monitoring of change over time. (See 'Functional evaluation' above.)

●Management of motor impairment – All children with CP have altered motor function to some extent, affecting posture, coordination, and balance. Treatment interventions should be offered from least to most invasive. Management of the child's motor impairment may include physical and occupational therapy, orthotics, medications to reduce spasticity and/or dystonia, and orthopedic interventions that are directed at relief and prevention of deformity and maximizing function. (See 'Management of altered motor function' above and "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues".)

●Common comorbidities – Individuals with CP often have associated comorbidities that may impact quality of life. Screening for and management of comorbidities includes (see 'Associated conditions' above):

•Developmental delays and intellectual disability (see 'Intellectual disability' above and "Intellectual disability (ID) in children: Management, outcomes, and prevention")

•Epilepsy and seizures (see 'Epilepsy' above and "Seizures and epilepsy in children: Initial treatment and monitoring")

•Vision and/or hearing impairment (see 'Vision problems' above and 'Hearing loss' above and "Vision screening and assessment in infants and children" and "Hearing loss in children: Treatment")

•Disorders of speech and language, including dysarthria and impairments with expressive and receptive communication (see 'Speech and communication' above and "Evaluation and treatment of speech and language disorders in children")

•Growth failure due to poor nutrition caused by feeding difficulties from oromotor dysfunction (see 'Growth and nutrition' above)

•Gastrointestinal problems such as dysmotility and delayed gastric emptying, constipation, and gastroesophageal reflux (see 'Gastrointestinal disorders' above and "Treatment of gastroparesis" and "Chronic functional constipation and fecal incontinence in infants, children, and adolescents: Treatment" and "Management of gastroesophageal reflux disease in children and adolescents")

•Respiratory disorders (eg, recurrent aspiration, inflammatory lung disease, restrictive lung disease due to scoliosis and chest wall deformity, hypoventilation, obstructive sleep apnea, laryngomalacia) (see 'Respiratory disorders' above and "Aspiration due to swallowing dysfunction in children" and "An overview of asthma management" and "Chest wall diseases and restrictive physiology" and "Management of obstructive sleep apnea in children" and "Congenital anomalies of the larynx", section on 'Laryngomalacia')

•Sialorrhea (see 'Sialorrhea' above)

•Low bone density and fractures (see 'Bone health' above)

•Skin breakdown and pressure injury (see 'Skin integrity' above and "Prevention of pressure-induced skin and soft tissue injury")

•Urinary incontinence (see 'Urinary control' above and "Management of bladder dysfunction in children")

•Sleep problems (eg, abnormal circadian rhythms, obstructive sleep apnea, hypoventilation from either a central etiology or muscle weakness, gastrointestinal discomfort, and/or sleep disruption from chronic pain) (see 'Sleep disorders' above and "Overview of circadian rhythm sleep-wake disorders", section on 'Management' and "Management of obstructive sleep apnea in children" and "Congenital central hypoventilation syndrome and other causes of sleep-related hypoventilation in children", section on 'Management')

•Psychiatric disorders (see 'Psychiatric disorders' above and "Anxiety disorders in children and adolescents: Epidemiology, pathogenesis, clinical manifestations, and course" and "Pediatric unipolar depression: Epidemiology, clinical features, assessment, and diagnosis" and "Attention deficit hyperactivity disorder in children and adolescents: Clinical features and diagnosis")

•Chronic pain frequently due to hip dislocation/subluxation, dystonia, gastroesophageal reflux, or constipation (see 'Chronic pain' above and "Pain in children: Approach to pain assessment and overview of management principles", section on 'Neurologic impairment')

●Prognosis – The prognosis for survival and motor function in children with CP is highly variable. Most children with CP survive to adulthood. Life expectancy for individuals with CP is generally lower than the general population, though this depends on the severity of impairment. The extent of motor impairment is difficult to predict in young children, except at the extremes of mild or severe involvement. In general, prognosis for motor function depends upon the type of CP, rate of motor development, presence of developmental reflexes, and intellectual ability. (See 'Prognosis' above.)

ACKNOWLEDGMENTS — The UpToDate editorial staff acknowledges Geoffrey Miller, MD and Laurie Glader, MD, who contributed to earlier versions of this topic review.