Cerebral palsy: Overview of management and prognosis

INTRODUCTION — 

Cerebral palsy (CP) refers to a heterogeneous group of conditions involving lifelong motor dysfunction that affects muscle tone, posture, and/or movement. These conditions are due to impairments in the developing fetal or infant brain. Although CP is not a neurodegenerative condition, the clinical expression may change over time as the child grows and the central nervous system matures into adulthood. Motor dysfunction can manifest as limitations in functional abilities and activity, which can vary in severity. Multiple additional symptoms often accompany the primary motor symptoms, 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".)

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".)

APPROACH TO MANAGEMENT — 

The management of CP begins with a clinical assessment of the nature and severity of symptoms as well as the overall functional status to help identify available therapeutic interventions. Individuals with CP often have symptoms in multiple domains; patients and their families/caregivers should be involved in multidisciplinary discussions of treatment priorities and decisions to optimally guide care. This can include motor goals related to ambulation and overall mobility as well as functional goals such as independence with toileting, the ability to assist with transfers in and out of a wheelchair, and optimizing strategies for effective communication and social development. Each of these goals may require different pharmacological, surgical, bracing, equipment, and therapy-focused approaches.

Assess motor impairments and functional status — Assessing and classifying motor and other impairments for individuals with CP helps to identify the extent of clinical needs as well as to objectively monitor and communicate both symptoms and response to interventions across disciplines.

●Determine functional status — Standardized measurement of an individual's overall functional status is an established way to gauge the severity of impairment in CP, guide treatment selections, and allow for monitoring of changes over time. The Gross Motor Function Classification System (GMFCS) is the most widely used system for assessing and monitoring overall gross motor functional status. Several other classification systems are also available to help assess other important functional domains for children with CP including manual ability, communication, eating and drinking, and vision. (See "Cerebral palsy: Classification and clinical features", section on 'Functional classification systems'.)

Gait analysis, using physical examination, videotaping, force plates, electromyography, and computerized analysis of limb motion, can also be used as an assessment tool to identify abnormalities in muscle function and limb alignment and to evaluate both the indications for and the effects of surgery [2-4]. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues", section on 'Surgery for contractures and bony deformities'.)

●Identify altered tone — Altered muscle tone is a key clinical feature and therapeutic target of CP. This may manifest as spasticity and/or dystonia. Clinical scales that describe the severity of altered tone include the Modified Ashworth Scale (table 1) for patients with spasticity and the Barry Albright Dystonia Scale or Dyskinetic Cerebral Palsy Functional Impact Scale (D-FIS) for patients with dystonia or choreoathetosis [5-9]. Scales that describe patterns and severity of motor impairments are discussed in greater detail separately. (See "Cerebral palsy: Classification and clinical features", section on 'Patterns of motor impairment'.)

●Monitor function over time — CP is a nonprogressive disorder, but clinical features may change over time as the child grows and the nervous system matures. Standardized examinations and clinical assessments should be performed as part of routine management of a growing child with CP. (See "Cerebral palsy: Evaluation and diagnosis", section on 'Examination and developmental assessment'.)

While gross motor functional status levels typically remain stable throughout childhood, motor function can change as the child grows [10]. Individuals with CP may have their peak motor function in late adolescence and many lose some gross motor function in adulthood [11]. A decline in gross motor functional status in adulthood may occur in individuals with more severe CP (eg, GMFCS III, IV, and V) [12]. 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. Treatment strategies to optimize motor function should be tailored to the evolving needs of the child.

However, a sudden or unanticipated decline in GMFCS level should prompt further medical evaluation [13]. Untreated or undertreated CP-related hypertonia and other treatable conditions (such as spinal stenosis) can significantly reduce functional status and mobility.

Assess multidisciplinary needs — Management strategies for individuals with CP differ according to the severity and complexity of both motor and nonmotor symptoms. Motor symptoms may require physical and occupational therapies, medications for spasticity, orthotics, and/or orthopedic surgery. Individuals may also require interventions that address any associated comorbidities such as intellectual disability, epilepsy, and orolingual disorders such as dysphagia and sialorrhea. Evaluation for nonmotor conditions associated with CP are discussed separately. (See "Cerebral palsy: Evaluation and diagnosis", section on 'Assessing for associated conditions'.)

Comprehensive 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 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. Participation of the principal caregivers, including community-based support such as teachers, therapists, and home nurses, in treatment plan development is essential [14].

Interventions for children with CP should be tailored to specifically address both motor and nonmotor symptoms [15]. (See 'Management of altered motor tone and function' below and 'Emotional and social support' below and 'Management of associated conditions' below.)

Establish treatment priorities and preferences — The overall care plan should promote the child's participation, communication, education, community engagement, and social and emotional development. Therapeutic and educational support and family engagement play a significant role in promoting development.

Conceptualizing comprehensive multidisciplinary management should be guided by the 2001 World Health Organization's International Classification of Functioning, Health, and Disability (commonly called the ICF framework) which have been further refined in the context of neurodevelopmental disabilities as the “F-words” principles to guide CP management [16]:

●Function

●Family

●Friends

●Fitness

●Fun

●Future

This framework suggests that management of CP symptoms should be guided by optimizing function, using family-centered approaches (eg, using shared decision-making strategies and identifying ways to reduce families’ stress), optimizing social interaction and development of friendships, identifying recreational activities to promote fitness, tailoring management to support activities that the person with CP finds to be fun, and focusing therapies to ensure optimal functioning from childhood through adulthood.

EARLY INTERVENTION — 

Therapeutic interventions should start at the time of the suspected diagnosis of CP to help maximize functional improvement related to neuromuscular plasticity of the developing brain. Interventions for motor and cognitive impairments in CP may be most effective when started early [17].

MANAGEMENT OF ALTERED MOTOR TONE AND FUNCTION — 

By definition, all children with CP have altered motor function to some extent, affecting posture, coordination, and balance. Generally, treatment interventions should be offered from least to most invasive.

Strategies to treat altered motor function include rehabilitation therapies, oral and parenteral medications, as well as surgical intervention. These issues are discussed in detail separately. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues".)

EMOTIONAL AND SOCIAL SUPPORT — 

When a child has CP, social and emotional support for the patient as well as family/caregivers is essential, as it is for any family with a child with a chronic medical condition [14].

●Initial support during evaluation and diagnosis – Information about their child's condition should be provided to parents and caregivers with honesty and sensitivity as soon as the diagnosis is suspected [18,19]. 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 [20,21]. A second opinion should be offered if appropriate. It is also important to establish and share the cause of a person’s CP, including whether that cause is unknown. This can provide some closure for families and can guide further diagnostic workup, including genetic testing [22,23]. (See "Cerebral palsy: Evaluation and diagnosis", section on 'Metabolic and genetic testing'.)

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. 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.

●Social support for children with CP – Resources for children with CP and other disabilities are available in many communities. 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 social benefits such as promoting socialization and improving self-esteem, as well as physical benefits including minimizing the deconditioning that can be caused by impaired mobility and optimizing physical functioning [24,25]. 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.

●Parents and caregivers – Parents or caregivers of a child with CP may experience chronic grief, as well as guilt, frustration, denial, anger, resentment, and embarrassment [26,27]. 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.

Mental health management for children with CP who have associated psychiatric conditions is discussed below. (See 'Psychiatric disorders' below.)

MANAGEMENT OF ASSOCIATED CONDITIONS

Intellectual disability — Children with CP are at risk for a spectrum of cognitive manifestations including global developmental delay, learning disabilities, and intellectual disability. In an Australian population-based study of more than 1100 children with CP, intellectual disability was reported in 45 percent overall [28]. Rates varied according to Gross Motor Function Classification System (GMFCS) functional status, from 30 percent for those at GMFCS I/II and 48 percent at GMFCS III, to 83 percent at GMFCS IV/V. 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 along with visual and/or auditory impairments can all complicate assessment of cognitive abilities in individuals with CP. Evaluation for intellectual disability in patients with CP is distinct from evaluation of speech, feeding, and bulbar dysfunction. (See 'Speech and communication' below.)

Psychiatric disorders — Anxiety, depression, autism spectrum disorder, and attention deficit hyperactivity disorder (ADHD) occur at higher rates in children with CP compared with the general population [29]. In a survey-based study that included 458 children with CP, the prevalence of any psychiatric disorder was higher in those with CP than matched controls (76 versus 54 percent) [30]. These conditions may impact learning, socialization, and engagement with rehabilitation therapies for motor impairments. Screening for psychiatric disorders should accompany routine screening and examinations. Physical activity and participation in sports may reduce the risk and burden of mental health disorders in children with CP [30-33]. Pharmacologic and other specific management of these conditions are discussed separately. (See "Pharmacotherapy for anxiety disorders in children and adolescents" and "Overview of prevention and treatment for pediatric depression" and "Autism spectrum disorder in children and adolescents: Overview of management and prognosis" and "Attention deficit hyperactivity disorder in children and adolescents: Overview of treatment and prognosis".)

Epilepsy — Epilepsy occurs in one-quarter to one-half of patients with CP [34-36]. The risk of epilepsy may be higher in patients with CP due to chromosomal abnormalities and brain malformations than other causes [37]. Seizures also appear to be more common in patients with more severe functional impairments and those with either spastic quadriplegia or hemiplegia than those with symmetric spastic diplegia and CP which is mainly athetoid [38-40]. In addition, seizures occur in up to 9 percent of adults with CP who did not have seizures in childhood [41].

The management of epilepsy is discussed in detail separately. (See "Seizures and epilepsy in children: Initial treatment and monitoring".)

Vision problems — Vision problems are reported in approximately one-third to one-half of children with CP, and approximately 10 percent are blind [42,43]. Visual problems can affect afferent visual function and may include ocular conditions such as strabismus, refractive errors, and amblyopia, as well as efferent visual function, including central conditions such as disorders of oculomotor control, visual field defects, and cerebral visual impairment (CVI). Visual impairment has been associated with CP due to neonatal stroke, hydrocephalus, and infection [37].

All children with CP should have baseline ophthalmologic assessments and longitudinal monitoring. Children with complex visual problems, including those with CVI, would benefit from a neuro-ophthalmology and/or 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 [44]. Early screening, 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 bulbar function or in expressive and written language, occur in up to two-thirds of children with CP; approximately one-quarter are nonverbal [45].

The use of augmentative communication strategies for individuals who have impaired language skills or are nonverbal can be helpful in promoting communication, decreasing frustration related to communication deficits, and fostering social interactions. The use of such technologies does not appear to interfere with the development of speech development [46]. Early implementation of augmentative communications strategies (eg, by one to two years old) can help to foster the development of communication and language skills and optimize overall function. Strategies must be individualized and appropriate for the individual’s cognitive and motor capacities. A range of strategies with varying technical complexity can be used, ranging from simple buttons or switches, and picture symbols, up to voice output devices [47,48].

Many centers offer specialized clinics for the evaluation of equipment and/or augmentative communication needs. These can be invaluable resources for the primary care provider to identify the most appropriate piece of equipment for the child and assist the family in navigating the complex insurance regulations that can impact the timing and range of equipment available.

The evaluation and treatment of speech and language disorders in children is discussed in greater detail separately. (See "Speech and language impairment in children: Evaluation, treatment, and prognosis".)

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

●Serial assessments – 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 thus result in an inaccurate calculation of body mass index [49]. Triceps folds can be monitored to assess 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 GMFCS subset height measurement. (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 oromotor dysfunction from pseudobulbar palsy that can, in turn, affect overall nutrition.

The 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 feeding difficulties and 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 [50,51]. 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 [52-56]. This intervention can also reduce aspiration risk and provide a reliable route for medication. It improves the quality of life for both the child and family in most cases [57]. 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 [50].

Successful enteral feeding can lead to excessive accumulation of adipose tissue in children with CP [58]. Caloric goals should initially be modest and subsequently adjusted to maintain an adequate body weight (eg, for children with GMFCS level V, maintaining weight above the 20^th percentile for the GMFCS level V-specific growth chart) [59]. 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".)

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 the 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, medications, salivary gland botulinum neurotoxin (BoNT) injections, and surgery. Drooling improves with age in some patients. We typically pursue behavioral and pharmacologic management (usually with an anticholinergic agent such as glycopyrrolate) before considering more invasive treatments, in general agreement with an American Academy of Cerebral Palsy and Developmental Medicine (AACPDM) care pathway established for sialorrhea management [60].

●Behavior therapy – A trial of behavioral therapy is appropriate for children who can understand instructions 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 [61-64].

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 caregivers. 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 [63-65]. Most studies reported improvements in drooling frequency or severity and/or quality of life.

●Pharmacologic therapy – Medications for drooling are used when behavioral therapy is ineffective or insufficient. Common options include anticholinergic agents, sublingual atropine, and BoNT injection into the salivary glands. Oral therapies are frequently tried first with BoNT used as an adjunctive agent or alternative when the response to oral agents is unsatisfactory.

•Anticholinergic agents for sialorrhea include:

-Glycopyrrolate 20 to 100 mcg/kg three times daily

-Scopolamine (hyoscine) one-quarter to one transdermal patch every three days

-Benztropine 0.02 to 0.05 mg/kg once daily

-Trihexyphenidyl 0.1 to 0.5 mg/kg two times daily

These medications act by decreasing the flow of saliva [66,67]. Glycopyrrolate and scopolamine are the agents used most commonly [68,69]. 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) patches 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 [70]. 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.

•Sublingual atropine inhibits saliva secretion by binding to muscarinic receptors in the submucosal glands. Atropine is used to reduce secretions in anesthesia and has shown promise in the treatment of sialorrhea [71]. In a retrospective chart review of 178 children and young adults with sialorrhea due to cerebral palsy or other neurologic or developmental disorders, benefit with sublingual atropine was reported with doses ranging from 0.01 to 0.49 mg/kg [72]. Atropine may be used either as a first-line medication or along with an anticholinergic agent.

•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 [73]. In another study, the effect of BoNT waned over time, lasting a median of 22 weeks [74]. Approximately 25 percent of initial responders still had a clinically significant response at 33 weeks after BoNT injection.

BoNT is generally well tolerated, but transient injection site pain or bruising my occur. Other adverse effects include fever, fatigue, and transient muscle weakness [75,76]. Serious adverse effects from systemic spread of BoNT are rare. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues", section on 'Botulinum toxin'.)

A 2022 systematic review of 27 studies (including five clinical trials) found the efficacy of several anticholinergic medications for sialorrhea in children including glycopyrrolate, scopolamine, trihexyphenidyl, and benztropine, as well as sublingual atropine [77]. Another systematic review of six clinical trials on sialorrhea in children with CP reported efficacy and safety for glycopyrrolate, scopolamine, benztropine, and BoNT injection [78]. 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 behavioral and pharmacologic measures, surgical options include repositioning of the submandibular ducts and unilateral ligation of a parotid duct and/or excision of the sublingual gland [79-82]. 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 [79,81,83].

•Repositioning the salivary glands may be associated with longer benefits than BoNT treatment [83]. 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 a greater risk of adverse events [84,85]. 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) [84]. 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 [85].

•Excision of the submandibular gland has been associated with favorable effects compared with duct ligation or BoNT treatment but is a more invasive technique. 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 [86]. 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.

Gastrointestinal disorders — Gastrointestinal disorders including dysmotility dysfunction, chronic constipation, and gastroesophageal reflux are common in children with CP. They can contribute to feeding and nutritional difficulties and cause discomfort leading to disordered sleep.

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 "Gastroesophageal reflux disease in children and adolescents: Management")

Respiratory disorders — Respiratory conditions among children with CP may be due to several factors. Sialorrhea can lead to chronic aspiration (see 'Sialorrhea' above), while joint contractures or neuromuscular scoliosis can contribute to hypoventilation or restrictive lung disease.

The management of common respiratory disorders in CP are 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 in children and adults")

●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 laryngomalacia")

Chronic pulmonary disease is a leading cause of morbidity and mortality among patients with severe CP (see 'Life expectancy' below).

Bone health — Many children with CP are at risk for low bone density, including those who are nonweightbearing and those who take chronic medications that can impair bone metabolism (eg, some antiseizure medications). The risk is further increased by poor feeding or nutrition. Low bone density can put children with CP at increased risk for fractures (eg, those that occur with minimal trauma).

●Vitamin D and calcium supplementation – All nonambulatory children with CP and those on chronic antiseizure medications should be screened at least annually 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. Limited data suggest vitamin D and calcium supplementation may help restore bone density [87-89]. (See "Vitamin D insufficiency and deficiency in children and adolescents", section on 'Treatment'.)

●Physical activity – Weight-bearing activity, such as using a stander, should be incorporated into the daily routine of all nonambulatory children with CP if possible. Weightbearing regimens may improve bone mineral density but the evidence is limited [87,89-92].

●Bisphosphonates for children with pathologic fractures – 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 [93]. Lower doses also appeared to be effective in an open-label trial [94]. 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 [87,95].

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

Skin integrity — Patients with CP, particularly those who are nonambulatory or have significant gross motor functional limitations, 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 people 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 — Urinary dysfunction is common in children with CP.

●Toilet training – Although 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 usual. (See "Toilet training".)

●Managing incontinence – For children with CP who are unable to achieve bladder control, medications may reduce incontinence. The approach to pharmacotherapy for urinary incontinence in children with CP is similar to that of others 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'.)

●Neurogenic bladder treatment – For children with CP who have 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 [96]. 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 due to several potential factors including:

●Behavioral issues

●Impaired circadian rhythm (eg, caused by low vision)

●Obstructive sleep apnea

●Hypoventilation from either a central etiology or muscle weakness

●Discomfort or pain from gastroesophageal reflux or constipation, dystonia, or spasticity

Treatment generally targets the underlying concern and may include interventions to improve sleep hygiene, parental education, tone management, a range of pharmacologic interventions (eg, melatonin, omeprazole), and/or noninvasive ventilatory support (eg, for treatment of hypoventilation or obstructive sleep apnea) [17,97,98].

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'.)

Chronic pain — Many children with CP experience chronic pain that limits their activities [99]. Pain is strongly associated with poor quality of life. Pain in children with CP may go unrecognized due to communication difficulties, 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. Spasticity and/or dystonia, orthopedic issues such as hip dislocation/subluxation, gastroesophageal reflux, or constipation are commonly reported causes of pain.

Management of pain in children is discussed in greater detail separately. (See "Pain in children: Approach to pain assessment and overview of management principles".)

PROGNOSIS

Motor function — Motor impairment prognostication is difficult to predict in young children, except at the extremes of mild or severe involvement. In general, the prognosis for various aspects of motor function depends upon the type of CP, rate of motor development, presence of developmental reflexes, and intellectual ability.

●Rate of motor development – 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 [10]. 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.

●Learning to walk – Several factors in young children have been identified to help predict the ability to walk independently. In a meta-analysis of observational studies of children with CP, factors associated with good prognosis for achieving independent walking included [100]:

•Sitting independently by two years

•Absence of visual impairment

•Absence of intellectual disability

•Absence of a history of seizure or epilepsy

Similar results were reported in a retrospective study of 5366 children with CP who were nonambulatory at two years of age [101]. 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, CP type other than spastic quadriparesis, and preserved visual function.

However, other data suggests some children with CP who do not have good prognostic factors for walking may eventually become ambulatory. In a prospective longitudinal study in which 233 children with CP were followed from 12 months to 3 to 11 years of age, some degree of functional walking was eventually attained by 79 percent [102]. While the ability to sit by age two years was a highly reliable predictor of walking, more than one-half of those who did not sit by two years eventually walked. Among these children, suppression of primitive reflex activity between 18 and 24 months predicted eventual walking.

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 can be difficult to measure in children, especially those 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 [103]. In addition, chronic pain is strongly associated with poor quality of life [103,104].

Among 8- to 12-year-old children with CP who can self-report, the quality of life is similar to that of children in the general population [105]. However, adolescents with CP report significantly lower quality of life in the social support and peers domain than the general population [104]. 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 [106]. Emotional and social support may improve the quality of life among children and adults with CP who report impairment in variables such as pain, fatigue, or depression [107,108].

Life expectancy — The majority of people with CP alive today are adults [109], highlighting that the majority of children with CP will live to experience adulthood [110-112]. 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 [111].

However, life expectancy for individuals with CP is generally lower than for the general population, driven in part by outcomes in those with severe impairment. 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 [109]. 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) [112].

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 [113]. Respiratory disease, often aspiration pneumonia, is the most common cause of death among individuals with CP [113].

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 approach – The management of cerebral palsy (CP) begins with a clinical assessment of the nature and severity of symptoms as well as the overall functional status to help identify available therapeutic interventions. Individuals with CP often have symptoms in multiple domains; patients and their families/caregivers should be involved in multidisciplinary discussions of treatment priorities and decisions to optimally guide care. Each of these goals requires different pharmacological, surgical, bracing, equipment, and therapy-focused approaches. (See 'Approach to management' above.)

●Early intervention – Therapeutic interventions should start at the time of the suspected diagnosis of CP to help maximize functional improvement related to neuromuscular plasticity of the developing brain. (See 'Early intervention' above.)

●Management of motor impairment – All children with CP have altered motor function to some extent, affecting posture, coordination, and balance. Strategies to treat altered motor function include:

•Rehabilitation therapies

•Oral and parenteral medications

•Surgical interventions

Generally, treatment interventions should be offered from least to most invasive. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues".)

●Emotional and social support – Comprehensive management of CP includes social and emotional support for the patient as well as family/caregivers. (See 'Emotional and social support' above.)

●Comorbid conditions – For patients with sialorrhea who can participate in behavioral therapy, we suggest oral motor skills therapy (Grade 2C). This is generally administered by speech or language pathologists and can be helpful. (See 'Sialorrhea' above.)

For patients in whom behavioral therapy is not feasible or is ineffective, we suggest a trial of an anticholinergic medication (eg, glycopyrrolate 20 to 100 mcg/kg three times daily or scopolamine one-quarter to one transdermal patch every three days) (Grade 2C). Use of these drugs may be limited by anticholinergic side effects. BONT may be a useful adjunct or alternative. Surgical treatment is an option for severe and refractory drooling.

Other comorbidities and complications are also common in individuals with CP. These may include intellectual disability, epilepsy, speech disorders, and growth/nutritional impairment. Screening and management of these are reviewed briefly in the sections above; specific treatment recommendations are provided in separate topic reviews. (See 'Management of associated conditions' above.)

●Prognosis – The prognosis for survival and motor function in children with CP is highly variable. (See 'Prognosis' above.)

•Motor impairment prognostication is difficult to predict in young children, except at the extremes of mild or severe involvement. In general, the prognosis for various aspects of motor function depends upon the type of CP, rate of motor development, presence of developmental reflexes, and intellectual ability. (See 'Motor function' above.)

•Life expectancy for individuals with CP is generally lower than the general population, though this depends on the severity of impairment. Most children with CP live to adulthood. Respiratory disease, often aspiration pneumonia, is the most common cause of death among individuals with CP. (See 'Life expectancy' above.)

ACKNOWLEDGMENTS — 

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