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 due to any nonprogressive cause. 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].
The clinical features and classification of CP are reviewed here. The epidemiology, etiologies, prevention, evaluation, diagnosis, management, and prognosis of CP are discussed separately:
●(See "Cerebral palsy: Epidemiology, etiology, and prevention".)
●(See "Cerebral palsy: Evaluation and diagnosis".)
●(See "Cerebral palsy: Overview of management and prognosis".)
CLASSIFICATION — CP is most commonly classified by the patterns of motor impairment (table 1) and/or extent of functional impairment (eg, Gross Motor Function Classification System [GMFCS]). Other schemas include classification based upon the body regions involved or underlying etiology.
Classification systems may be used to communicate the extent and pattern of symptoms to help provide optimal care for individuals with CP. They should be used at the time of diagnosis and revisited periodically to account for evolving motor symptoms in a growing child, track function, and reevaluate an evolving understanding of the etiology. (See "Cerebral palsy: Overview of management and prognosis", section on 'Monitoring function over time'.)
The use of multiple classification systems to accurately describe symptoms can help promote function-focused and comprehensive care of individuals with CP.
Patterns of motor impairment — Motor impairment is a necessary clinical feature of CP, but motor impairment phenotype can vary in severity and character [2]. The phenotypic categories include the following (table 1):
●Spastic types (includes diplegia, hemiplegia, and quadriplegia)
●Dyskinetic types (includes dystonic and choreoathetotic)
●Ataxic type
The clinical patterns associated with motor phenotypes are discussed in detail below. (See 'Motor phenotypes' below.)
Characterization of CP by distinct motor phenotype remains common but has certain limitations. The classification scheme defines subtypes, but, in reality, there is substantial overlap in the clinical features of these subtypes, with frequent coexistence of multiple motor abnormalities that can lead to inconsistency between providers. As examples, mild dyskinetic signs (eg, dystonia) may occur in the majority of people with the spastic subtype, and many with dyskinetic and ataxic forms of CP also have spasticity [3]. In addition, the clinical features may change as a child grows. A child may have predominant spasticity during early childhood that evolves to prominent dystonia in adolescence with greater limits on functionality.
Motor phenotype was thought to correlate with the pattern and mechanism of brain injury, however the association between injury pattern and motor phenotype is inconsistent. For example, although dystonic CP is typically associated with deep grey matter injury at term, many children without deep grey matter injury on brain magnetic resonance imaging (MRI) have dystonia-predominant CP [4,5]. (See "Cerebral palsy: Epidemiology, etiology, and prevention", section on 'Specific causes and risk factors'.)
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 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.
●Gross Motor Function Classification System (GMFCS) – The GMFCS categorizes motor function based on an individual’s age and usual performance in various settings: school, home, and community [6]. The GMFCS level is a standardized shorthand method of conveying information regarding the child's function, rehabilitative potential, and expected trajectory over time. It should be included in assessment of all children with CP.
The five GMFCS levels can be described as follows for children 6 to 12 years old [6]:
•Level I – Walks in all settings; climbs stairs without using a railing; runs and jumps, but speed, balance, and coordination may be limited.
•Level II – Walks in most settings, though may have difficulty walking long distances and balancing on uneven terrain; walks up and down stairs holding onto a railing; minimal ability to run and jump.
•Level III – Walks using a hand-held mobility device (canes, crutches, and anterior and posterior walkers that do not support the trunk); may use wheeled mobility for longer distances; when seated, may require a seatbelt for balance; may require physical assistance when transferring from sitting to standing; may walk up and down stairs holding onto a railing with supervision or assistance.
•Level IV – Generally dependent on wheeled mobility; may be able to use power mobility independently; may walk short distances with support in familiar environments; at home, may use floor mobility (roll, creep, or crawl); requires adaptive seating for trunk and pelvic control; requires physical assistance for most transfers.
•Level V – Transported in a manual wheelchair in all settings; limited ability to maintain antigravity head and trunk postures and control arm and leg movements; transfers require complete physical assistance.
Modified descriptions for other ages are available on the CanChild Centre website.
●Functional Mobility Scale (FMS) – The FMS has been constructed to classify functional mobility in children, taking into account the range of assistive devices a child might use [7]. This represents the child's mobility in the home, at school, and in the community setting.
●Canadian Occupational Performance Measure (COPM) – The COPM is an individualized tool that measures the child's self-perception of performance in everyday living over time and can be useful in tracking progress of defined goals [8].
●International Classification of Functioning, Disability and Health (ICF) – The ICF is a conceptual framework put forward by the World Health Organization (WHO) in 2001; a companion classification for children and youth (ICF-CY) was introduced in 2007 [9]. It outlines different components of functioning and disability (eg, body structure and function, activity, participation, and environmental and personal factors) that interact dynamically to describe an individual's overall health status [10]. Additional information about the ICF is available through the WHO website and the United States Centers for Disease Control and Prevention website.
●Manual Ability Classification System (MACS) – The MACS assesses fine motor function using a graded system from I to V, mirroring the structure of the GMFCS [11,12].
●Communication Function Classification System (CFCS) – The CFCS is used to specify a child's functional ability with communication skills [13,14]. It also uses a graded system from I to V.
●Visual Function Classification System (VFCS) – The VFCS is used for toddlers and children with CP to describe visual abilities either with or without the use of adaptive strategies [15].
●Eating and Drinking Ability Classification System (EDACS) – The EDACS is used to assess dysphagia in children with CP, grading the safety and efficiency of eating and drinking on a 1 to 5 scale [16].
Distribution — CP may be classified by body regions involved. The distribution classification specifies unilateral versus bilateral involvement and/or the number and distribution of limbs involved (monoplegic, hemiplegic, diplegic, triplegic, or quadriplegic) (figure 1) [1]. This can be particularly helpful when conceptualizing the impact that motor impairments might have on function.
Etiologies — Classifying CP by underlying etiology or etiologies, when identified, can be helpful to ensure appropriate diagnostic testing and is also often desired by the person with CP and their family [17]. Common etiologies include:
●Prematurity
●Perinatal hypoxic-ischemic injury
●Congenital abnormalities of the central nervous system
●Genetic factors
●Perinatal stroke
●Congenital infections
CP is frequently multifactorial. Etiologies of CP are discussed in greater detail separately. (See "Cerebral palsy: Epidemiology, etiology, and prevention", section on 'Specific causes and risk factors'.)
EARLY SIGNS OF CEREBRAL PALSY — Signs and symptoms suggestive of the diagnosis of CP may be present in infancy. This may include abnormalities in motor tone, significant delays in achieving motor milestones, abnormal developmental reflexes, or the presence of associated conditions such feeding difficulties, growth failure, or epilepsy [18-20].
Developmental reflex changes — In normal development, most motor reflexes related to posture (eg, tonic neck, trunk incurvation) disappear in infancy between three and six months of age (table 2). In infants and children with CP, these reflexes frequently either fail to develop or they persist beyond the expected age thresholds [21]. Delay in the disappearance or exaggeration of a developmental reflex may be an early indication of motor disability [22,23].
A developmental reflex that persists for as long as the stimulus is applied (obligatory reflex) is abnormal at any age [22]. (See "Detailed neurologic assessment of infants and children", section on 'Developmental reflexes'.)
Routine clinical evaluation of developmental reflexes may also identify abnormal responses, including the following:
●Exaggerated tonic labyrinthine response includes opisthotonic posturing or roll over at an age that is earlier than appropriate. The tonic labyrinthine response is tested in the supine position. Extension of the neck results in shoulder retraction, leg extension, and either elbow flexion or extension and pronation of the arms. Flexion of the neck results in flexion of the extremities.
●Persistent leg extension when the infant is held in vertical suspension is abnormal during the first few months of life. The infant may also display scissoring of the legs. The appropriate response is for the baby to assume a sitting position ("sit in the air").
●Persistent plantar flexion of the ankles is an abnormal response to positive support reaction. The anteromedial areas of the soles are placed on a firm surface, which should result in a few seconds of plantar flexion, then return of the feet to a neutral position. An abnormal response is plantar flexion that is obligate or maintained for more than 30 seconds, especially if accompanied by equinus posturing.
Motor tone and posture abnormalities — Tone may be increased, normal, or decreased in infants and young children with CP. Hypertonia usually tends to be the common form of tone. The most common patterns of abnormal tone and posture in infants and young children with CP are:
●Appendicular hypertonia (with some combination of spasticity and dystonia in the affected extremities)
●Axial hypotonia (with some difficulty supporting the trunk and head)
Other abnormalities of tone include:
●Persistent or asymmetric hand clenching or fisting
●Tongue retraction and thrust, tonic bite
●Oral hypersensitivity
●Grimacing
●Poor head control
However, increased neck extensor and axial tone may make head control appear better than it actually is.
An infant should be observed in the prone and supine positions to assess motor tone. Posture and tone may be assessed on pulling to sit, supported sitting, and vertical and ventral suspension. The infant should be able to self-support on arms and hands and rotate within the body axis at the appropriate ages. The emergence and quality of protective postural reactions should be noted.
Motor milestone delays — Serial examination using motor milestones can be an effective screening process for CP (table 3A-B). A young child’s motor development should be addressed at each pediatric preventive care visit. In addition, the American Academy of Pediatrics (AAP) recommends completion of a general developmental screening test (which includes assessment of motor skills) at the 9-, 18-, 30-, and 48-month visits [24]. (See "Developmental-behavioral surveillance and screening in primary care", section on 'Approach to screening'.)
International consensus guidelines suggest that any of the following motor milestone delays should prompt consideration of a CP diagnosis or referral to a specialist for CP diagnostic evaluation [25]:
●Hand preference develops before 12 months of age
●Sustained clenching or fisted posture of hands after four months of age
●Stiffness or tightness in the legs between 6 to 12 months of age (eg, unable to bring their toes to mouth when having their diaper/nappy changed)
●Head lag that persists beyond four months of age
●Inability to sit without support by nine months of age
●Consistent asymmetry of posture and movements after four months of age
Delays in multiple developmental motor milestones may better identify children with CP than delays in single milestones. In one study, six motor milestones (roll prone to supine, roll supine to prone, sit with support, sit without support, crawl, and cruise) were sequentially evaluated for at least 18 months in 173 infants born at <33 weeks gestational age (GA), of whom 31 (18 percent) developed CP [26]. Serial screening of these milestones predicted the development of CP better than any individual milestone. Delays in more than four milestones were especially worrisome. In another report, signs present at four months of age that indicated increased risk of CP were failure to support weight on the forearms in a prone position, sit supported with head erect, or show interest in surroundings or respond socially [22].
COMMON MOTOR FEATURES — Common motor abnormalities in CP include spasticity, dyskinesia, and ataxia. Weakness, hypotonia, and sensory abnormalities may also accompany these features in some individuals.
In affected individuals, voluntary movements that should be complex, coordinated, and varied can instead be uncoordinated, stereotypic, and limited. Simple actions that are performed unconsciously by unaffected individuals can require marked effort and concentration and may not be possible for some individuals with CP. In severely affected individuals, an attempted voluntary movement may evoke a primitive reflex, cocontraction of agonist and antagonist muscles, and mass movements [27]. As examples, attempts at isolated joint flexion may engage all segments of a limb, and extension of all the fingers may accompany extension of the wrist. Discrete, isolated movements, such as that of an individual finger, may be near impossible for some individuals with CP.
Spasticity — Spasticity is an upper motor neuron syndrome that includes positive and negative signs [28,29]:
●Positive signs − Positive upper motor neuron signs are abnormalities that lead to involuntarily increased muscle activity or movement patterns.
•Muscle spasticity − Muscle spasticity refers to the velocity-dependent increased resistance that occurs in response to passive muscle stretch. Examination may demonstrate classic "clasp-knife" response where the maximum resistance occurs after a few degrees of passive movement of a joint, which then relaxes after continued effort by the examiner. More rapid movement of the joint increases the degree of resistance and decreases its time of onset. (See "Detailed neurologic assessment of infants and children", section on 'Tone and strength'.)
Spasticity is present during both sleep and wakefulness and tends to be consistently present with minimal variability in severity within the same individual.
•Hyperreflexia – Hyperreflexia refers to a hyperactive involuntary response to percussive examination (movie 1). It is caused by hyperexcitability of the peripheral nerve response to mechanical muscle (and tendon) stretch. (See "Detailed neurologic assessment of infants and children", section on 'Tendon reflexes'.)
•Extensor plantar responses – Extension of the great hallux and fanning of the toes in response to stroking the lateral plantar surface of the foot (ie, "positive Babinski reflex") may indicate upper motor neuron dysfunction (movie 2). (See "Detailed neurologic assessment of infants and children", section on 'Superficial reflexes'.)
An extensor plantar response is a physiologic response in infants <12 months old.
•Clonus – Clonus refers to sustained involuntary muscle contractions and relaxations in the setting of muscle stretching. Clonus at the ankle may be elicited by dorsiflexing the ankle. (See "Detailed neurologic assessment of infants and children", section on 'Tendon reflexes'.)
●Negative signs − Negative signs are motor abnormalities that lead to reduced muscle activity or movement patterns and interfere with function. They may be due to insufficient control of muscle activity or disinhibition of central and/or peripheral neural circuits [30]. Negative upper motor neuron signs associated with spasticity may occur due to chronic dysfunction of an affected limb. These include:
•Weakness, with associated atrophy of weak muscles over time.
•Loss of dexterity.
•Fatigability.
•Axial hypotonia.
Dyskinesia — Dyskinesia refers to abnormal involuntary movements. Dyskinesia in individuals with CP includes dystonia, chorea, and athetosis. Dystonia is present in most individuals with CP to some degree.
●Dystonia refers to muscle activation that causes repetitive movements or postures. It is frequently worsened by voluntary movement, tactile stimulation, and/or heightened arousal. It may involve one or more body regions. Often, the severity varies in the same person over the course of a day and it commonly disappears with sleep.
●Chorea describes an irregular sequence of involuntary movements. Chorea may be observed in healthy infants <8 months old ("physiologic chorea"). In older infants, children, and adults, chorea is an abnormal finding.
●Athetosis refers to continuous involuntary writhing movements. Athetosis frequently occurs with chorea.
Dyskinesia and other hyperkinetic movement disorders in children are discussed in greater detail separately. (See "Hyperkinetic movement disorders in children".)
Ataxia — Discoordination of muscle contraction manifests as ataxia due to cerebellar dysfunction. Individuals with truncal ataxia may have poor head control, difficulty sitting upright, or trouble standing without support. Gait may be hesitant and wide-based. Limb ataxia features unsteady movements of arms and/or legs; some individuals may have an intention tremor. Dysarthria or scanning speech may indicate bulbar dysfunction. (See "Detailed neurologic assessment of infants and children", section on 'Coordination'.)
MOTOR PHENOTYPES — CP is commonly classified by characteristic patterns of abnormal motor activity, tone, and/or posture. After age 18 to 24 months, signs and symptoms generally point to a specific pattern that corresponds to a motor phenotype of CP (table 1) [31].
Spastic subtypes — Spastic-predominating CP is the most common pattern of motor impairment, accounting for up to 85 percent of individuals with CP [32]. Individuals with spastic-predominating CP have clinical features of an upper motor neuron syndrome. The spastic subtypes of CP have variable expression. The signs in children may differ from adults because the developing nervous system responds differently to insults [33]. Hypotonia may be more prominent in infants and younger children with spastic CP. Spasticity or other symptoms may become more prominent as the child grows and nervous system develops. Spastic CP may be symmetric or asymmetric and may involve one or more extremities (figure 1).
Dyskinesia may also be present in some individuals with spastic CP. Sensory disturbances of central nervous system origin, such as poor two-point discrimination and astereognosis, are common in all of the spastic syndromes [34]. Vasomotor abnormalities also occur.
Spastic diplegia — Spastic diplegia describes symptoms that are more prominent in the legs than the arms. In mild forms, individuals may have relatively good hand function and few associated disabilities. In those more severely affected, spasticity and contractures may cause functional impairment of the lower limbs, and upper limb function also may be compromised. Asymmetric spastic diplegia may occur such as those with periventricular leukomalacia and unilateral hemorrhagic infarction. The presence of prominent unilateral impairment of an arm with bilateral lower extremity impairment may be subclassified as spastic triplegia and is usually associated with more severe disability than symmetric spastic diplegia.
The primary muscle groups affected are the hamstrings and gastrocnemius muscles in the legs (resulting in knee flexion and foot plantar flexion) with some degree of foot pronation. Involvement of hip adductors leads to impaired gait. It is common to see inward rotation at the hip reflective of weak hip external rotators. Atrophy below the waist occurs in many individuals.
Associated involuntary movements and intellectual disability or learning disorders also may be present, but individuals with spastic diplegia tend to have fewer cognitive or other systemic impairments than individuals with spastic quadriplegia.
Spastic hemiplegia — Spastic hemiplegia refers to asymmetric symptoms that predominate on one side of the body. The arm is typically more affected than the leg, although the presentation is variable. The affected arm is usually held close to the body with spasticity primarily affecting the biceps and forearm pronators. The hand can be fisted and have an adducted thumb ("cortical thumb"). In the leg, the primary problem tends to be gastrocnemius spasticity resulting in foot plantar flexion. The affected arm may also display dystonia, which can be a significant functional limitation. This may be most commonly apparent as the arm is being involuntary raised, flexed, and tightened when the individual is doing more challenging tasks like a fine motor task with the less affected hand or running.
Most individuals with spastic hemiplegia also have sensory deficits. These are correlated with poor growth of the affected side, although not with the severity of the motor deficit [35].
Spastic quadriplegia — Spastic quadriplegia indicates bilateral motor impairments that involve both arms and legs. Individuals with this phenotype are more likely to have other nonmotor manifestations of CP such as intellectual disability, communication impairment, visual impairment, epilepsy, feeding difficulties, and/or pulmonary disease when compared with children with hemiplegia or diplegia [36,37]. Spastic quadriplegic CP is often regarded as the most severe form of CP because it affects numerous areas of the body and is more often associated with comorbidities given the extensive central nervous system involvement. However, the degree of functional impairment in any individual is variable. (See 'Associated conditions' below.)
Dyskinetic subtypes — Dyskinetic CP indicates that a dyskinesia such as dystonia or choreoathetosis is the predominant pattern of abnormal tone. However, individuals with dyskinetic CP often have more than one form of involuntary movement, and the types may overlap. Dyskinetic CP accounts for 15 percent of individuals with CP [38].
The presence of other motor impairments such as spasticity and ataxia (eg, dysarthria) and intellectual disability are variable.
Dystonia — Dystonia-predominating CP appears as sustained muscle contractions resulting in twisting and repetitive movements or postures [39]. Dystonia is frequently present in all body regions but is typically more prominent in the arms [38]. Limbs significantly affected by dystonia may even be intermittently hypotonic. Dystonia frequently interferes with daily activities and social participation for individuals with CP [40].
Chorea and athetosis — Chorea and athetosis frequently occur together but may occur separately in some individuals with CP. Symptoms typically include brief irregular, involuntary movements and slow twitching, wriggling movements. These hyperkinetic movements can give the appearance that the person is always moving when attempting to engage muscles.
Ataxic subtype — Ataxic CP is characterized by discoordinated movements and speech, usually associated with widespread disorder of motor function. However, the clinical presentation of ataxic CP is variable, and the etiology is frequently elusive [41]. Motor milestones and language skills typically are delayed. Ataxia may improve with time and therapy, and up to 70 percent of children with ataxic CP are able to walk [42]. However, intellectual disability accompanies motor deficits in nearly 70 percent of cases [42]. In general, associated disabilities are worse with increasing severity of motor impairment. Speech is typically slow, jerky, and explosive in cadence.
Ataxic CP is rare, accounting for 4 to 13 percent of individuals with CP [42], and should always be distinguished from genetic and progressive neurodegenerative disorders, which may present with some of the same features [43]. (See "Cerebral palsy: Evaluation and diagnosis", section on 'Differential diagnosis'.)
Others — A separate category of "hypotonic CP" has been described historically and is variably considered to be a phenotype of CP [44]. European CP registries do not include hypotonic CP as a classification, though Australian registries do. Individuals with spastic or dyskinetic CP may demonstrate predominantly hypotonia at birth and would not be considered to have "hypotonic CP". However, individuals with pure hypotonia as the cause of motor disability following a nonprogressive disturbance to the fetal or developing brain may be given a CP diagnosis by some practitioners.
"Mixed form of CP" is a term sometimes used when no pattern of motor impairment predominates [32]. Careful evaluation and monitoring for alternative diagnoses is warranted. Serial examinations may subsequently reveal changing features that identify a predominating CP subtype.
ASSOCIATED CONDITIONS — The motor impairments of CP are often accompanied by other disorders of cerebral function and associated systemic conditions. Associated cerebral disorders may affect cognition, vision, hearing, language, cortical sensation, attention, vigilance, and behavior. Dyspraxias and agnosias may interfere with skilled tasks and contribute to learning disorders regardless of the severity of motor deficit. Some individuals have epilepsy, and many have growth impairment or disturbed gastrointestinal function.
Based upon systematic reviews of published literature, estimated frequencies of other conditions are as follows [45,46]:
●Chronic pain (50 to 75 percent)
●Intellectual disability (50 percent)
●Speech-language disorders (40 to 60 percent)
●Bladder control problems (30 to 60 percent)
●Visual impairment (30 to 50 percent)
●Epilepsy (25 to 45 percent)
●Behavior disorder (25 to 40 percent)
●Hip displacement (30 percent)
●Sleep disorder (20 percent)
●Drooling (20 percent)
●Hearing impairment (10 to 20 percent)
●Gastrostomy tube dependence (7 percent)
In general, children with more severe motor disabilities are more likely to have comorbid conditions.
Because of these risks, all children with CP should undergo routine screening for intellectual, visual, auditory, and speech impairment [46]. Abnormal screening results call for more detailed evaluation. (See "Cerebral palsy: Evaluation and diagnosis", section on 'Assessing for associated conditions'.)
Learning disorders and intellectual disability — Intellectual disability occurs in approximately 50 percent of individuals with CP [45-47]. In addition, some children with mild motor symptoms without intellectual disability may have learning disorders and other academic challenges [48-50].
The severity of learning disorders or intellectual disability often correlates with the extent of motor impairment, particularly in children with spastic CP [47,51]. However, there is substantial variability in cognitive ability among affected individuals. Children with spastic quadriplegia are typically the most severely affected, while cognitive function usually is better with dyskinetic CP that is mainly athetoid. (See "Intellectual disability (ID) in children: Clinical features, evaluation, and diagnosis" and "Specific learning disorders in children: Clinical features".)
Behavioral and psychiatric conditions — Individuals with CP commonly have behavioral, emotional, and/or psychiatric disorders, including emotional lability, poor attention and vigilance, anxiety, depression, and obsessive-compulsive traits [52]. Attention deficit hyperactivity disorder (ADHD) is among the most commonly diagnosed psychiatric comorbidities, with a reported prevalence of 25 to 40 percent of children with CP [52,53]. Features of autism spectrum disorder occur in up to 7 percent of children with CP, but up to 18 percent in those with nonspastic CP [54]. (See "Attention deficit hyperactivity disorder in children and adolescents: Clinical features and diagnosis" and "Autism spectrum disorder in children and adolescents: Clinical features".)
Epilepsy — Epilepsy occurs in 25 to 45 percent of individuals with CP [45,46,54]. Seizures are most common in those with spastic quadriplegic and hemiplegic subtypes and less common in mild symmetric spastic diplegic and athetoid subtypes. (See "Seizures and epilepsy in children: Classification, etiology, and clinical features".)
The onset of seizures is typically during the first two years of life but can occur at any age. Due to focal brain injury, focal seizures are the most common seizure type in children with CP. At times, these seizures may develop secondary generalization [55].
Intellectual disability is more common in people with CP who have seizures than in those without seizures, and severe intellectual disability is more likely in those with multiple seizure types. Epilepsy can impose an additional cognitive burden when it is difficult to control or if anticonvulsant therapy causes sedation that further impairs learning and socialization.
Vision problems — Vision problems occur in approximately one-third to one-half of people with CP, and approximately 10 percent are blind [45,56-58]. Visual problems are varied and include strabismus, refractive errors, amblyopia, visual field defects, and cerebral visual impairment (CVI). Visual impairment is more common in individuals with severe motor impairment [59]. (See "Refractive errors in children" and "Amblyopia in children: Classification, screening, and evaluation".)
Children with CVI may have visual skills that seem to wax and wane, often within a single day or in association with fatigue. Severe dyskinetic eye movements can result in slow, variable, and highly inefficient visual function [60,61]. CVI is a phenomenon seen among children with more severe CP.
Preterm birth appears to be a risk factor for vision problems in children with CP. In one report, 558 children born before 32 weeks gestation (54 with CP) were examined at age two years [62]. Children with CP were significantly more likely to have retinopathy of prematurity (15 versus 2 percent), strabismus (52 versus 8 percent), and CVI (11 versus 0.2 percent). The rate of refractive error without other ocular abnormalities was similar in the two groups. (See "Retinopathy of prematurity (ROP): Risk factors, classification, and screening".)
Speech impairment — Disorders of speech and language, including aphasia and dysarthria, occur in approximately 40 to 60 percent of children with CP, and approximately 25 percent are nonverbal [45,46,63-65]. Abnormal function of oropharyngeal muscles (pseudobulbar palsy) and lack of coordination of breathing patterns contribute to speech disorders in some people. (See "Etiology of speech and language disorders in children" and "Evaluation and treatment of speech and language disorders in children".)
Coexisting intellectual disability and hearing impairment may also contribute to impairments of speech.
Hearing impairment — Hearing impairment occurs in 10 to 20 percent of children with CP, and approximately 5 percent are deaf [45,46]. Hearing impairment is most common in individuals with CP born at very low birth weight or with severe hypoxic-ischemic insults. Early diagnosis and treatment of hearing loss in infants at risk may improve learning and language development. (See "Screening the newborn for hearing loss" and "Hearing loss in children: Screening and evaluation".)
Feeding difficulties and growth failure — Individuals with CP often have growth failure, which generally correlates with severity of Gross Motor Function Classification System (GMFCS) level. Obtaining reliable and meaningful anthropometric measures is challenging. Growth charts specific to CP have been developed [66]. (See "Poor weight gain in children younger than two years in resource-abundant settings: Etiology and evaluation" and "Poor weight gain in children older than two years in resource-abundant settings".)
Feeding difficulties are common in children with CP and impact growth, nutrition, social interaction, and behavior [67-70]. Infants frequently have sucking and swallowing difficulties [68]. In school-aged children, common problems include needing help with feeding, prolonged feeding time (>30 minutes per meal), choking, and frequent vomiting [71]. These difficulties require caregivers to devote substantial time to oral feeding. Some children may not achieve sufficient intake if the time available for feeding is limited.
Poor nutritional status may be caused by inadequate intake and/or gastrointestinal problems [72-77]. Following a child's weight trajectory can be the most reliable measure of assessing the adequacy of nutrition. Depending on the degree of poor nutrition, gastrostomy tube placement may be warranted to promote increased caloric intake. (See "Cerebral palsy: Overview of management and prognosis", section on 'Growth and nutrition'.)
Gastrointestinal disorders — As many as 90 percent of people with CP have clinically significant gastrointestinal symptoms, which may include [76,78,79]:
●Chronic constipation (60 to 70 percent) [73,80] (see "Constipation in infants and children: Evaluation")
●Gastroesophageal reflux and/or vomiting (approximately 50 percent) [81] (see "Clinical manifestations and diagnosis of gastroesophageal reflux disease in children and adolescents")
●Swallowing disorders (approximately 20 percent) [78,82] (see "Aspiration due to swallowing dysfunction in children")
●Abdominal pain (10 to 30 percent) (see "Chronic abdominal pain in children and adolescents: Approach to the evaluation")
Gastrointestinal disorders may contribute to impaired nutrition and growth. (See 'Feeding difficulties and growth failure' above.)
Sialorrhea — Sialorrhea in individuals with CP generally results from difficulty managing secretions rather than overproduction of saliva. The etiology is multifactorial and may relate to oropharyngeal dysphagia (discoordination of the swallowing mechanism), poor head control, and/or facial diplegia with poor lip closure.
Anterior drooling is characterized by saliva spilling out of the mouth; posterior drooling is not visible and results in pooling of secretions in the posterior oropharynx. Individuals with posterior drooling are at risk of aspiration. (See "Cerebral palsy: Overview of management and prognosis", section on 'Sialorrhea' and "Aspiration due to swallowing dysfunction in children".)
Pulmonary disease — Chronic pulmonary diseases are common in people with CP and are a leading cause of morbidity and mortality among those with severe CP [83]. Pulmonary disease in individuals with CP results from different mechanisms that often occur together [84]:
●Recurrent aspiration − Recurrent aspiration can occur as a consequence of chronic oropharyngeal dysphagia and/or gastroesophageal reflux [85]. Gastrostomy tube feeding provides direct enteral access and reduces aspiration during swallowing but does not prevent aspiration of oral secretions or stomach contents from gastroesophageal reflux and may exacerbate gastroesophageal reflux. (See "Aspiration due to swallowing dysfunction in children".)
●Scoliosis − Scoliosis and chest wall deformity are common in children with CP and may contribute to restrictive lung disease (image 1). (See "Chest wall diseases and restrictive physiology", section on 'Pulmonary function' and "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues", section on 'Neuromuscular scoliosis'.)
●Respiratory muscle discoordination − Weakness and/or poor coordination of the respiratory muscles may result in hypoventilation, ineffective cough, and poor clearance of pulmonary secretions.
Orthopedic disorders — Orthopedic conditions are common in children with CP, including [86,87]:
●Contractures
●Hip dysplasia, subluxation, and dislocation
●Foot and hand deformities
●Progressive neuromuscular scoliosis
Orthopedic conditions may require orthotics, postural management, longitudinal physical therapy, and/or surgical intervention. (See "Cerebral palsy: Treatment of spasticity, dystonia, and associated orthopedic issues", section on 'Orthopedic assessment and interventions'.)
Osteopenia — Osteopenia can result in frequent fractures in people with CP. Multiple factors contribute to the development of osteopenia, including lack of mobility, feeding dysfunction, nutritional deficiency, and chronic antiseizure medication use [88-91]. A systematic review of five studies found that the prevalence of reduced bone mineral density among children with moderate to severe CP was 77 percent; the incidence of fractures was 4 percent per year [91]. Osteopenia in children with CP appears to be related to a diminished rate of bone growth rather than bone loss [92].
Urinary disorders — Urinary conditions occur in 30 to 60 percent of children with CP including enuresis, frequency, urgency, and stress incontinence [93-95]. Symptomatic neurogenic bladder and incontinence are most common in individuals with more severe (GMFCS IV and V) bilateral CP [95]. Continence often improves with age but may relapse as neurogenic bladder dysfunction progresses [94-96].
Children with incontinence have difficulty sensing bladder fullness and tend to have lower bladder capacity [97]. The dysfunction is caused by urodynamic abnormalities, such as bladder hyperreflexia, detrusor sphincter dyssynergia, bladder hypertonia with leakage, and periodic relaxation of the distal sphincter during filling [93]. Other contributing factors leading to incontinence can include reduced mobility and communication, poor cognition, low expectations of caregivers, and neurogenic dysfunction [98]. (See "Evaluation and diagnosis of bladder dysfunction in children".)
Children with milder forms of CP (ie, without significant limb spasticity) are also prone to detrusor overactivity, leading to urgency and/or daytime and nighttime incontinence [99].
Urinary infection and vesicoureteral reflux are uncommon but can occur in children with CP, generally in those with more severe GMFCS levels. Most children have normal storage pressures as measured by urodynamic testing, and the kidneys and bladder wall thickness are usually normal on ultrasonographic imaging [94]. However, in the setting of neurogenic bladder resulting in urinary retention, urinary tract infections and pain may be a recurrent problem. Some children with CP require a regular clean intermittent catheterization routine to reduce infection and improve comfort. (See "Cerebral palsy: Overview of management and prognosis", section on 'Urinary control'.)
Chronic pain — Pain is reported by 50 to 75 percent of children with CP, and approximately 25 percent experience pain that limits activities [45,100]. Pain is strongly associated with poor quality of life [101-103]. 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 the most frequently reported causes of pain [100]. Other less common etiologies, though still more common than in the general population, include kidney stones, gallstones, and pancreatitis.
Sleep disorders — Children with CP have high rates of disordered sleep [104]. This may be due to behavioral issues, as is common in typically developing children. However, there are often medical contributors to the disrupted sleep pattern in children with CP. These may include (see "Mechanisms and predisposing factors for sleep-related breathing disorders in children"):
●Abnormal circadian rhythms (ie, associated with low vision) (see "Overview of circadian rhythm sleep-wake disorders")
●Pain from various sources, such as muscle spasm or gastroesophageal reflux disease (see 'Chronic pain' above)
●Obstruction secondary to laryngomalacia, abnormal airway tone, or anatomic abnormalities (see "Evaluation of suspected obstructive sleep apnea in children")
●Hypoventilation from either a central etiology or muscle weakness (see "Congenital central hypoventilation syndrome and other causes of sleep-related hypoventilation in children")
Given the high prevalence of sleep disturbances in children with CP, the clinician should include screening for concerns in each well-child encounter, particularly for children with more severe CP. (See "Assessment of sleep disorders in children".)
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 5th to 6th 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 10th to 12th 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 info" and the keyword(s) of interest.)
●Basics topic (see "Patient education: Cerebral palsy (The Basics)")
SUMMARY
●Classifying symptoms – CP is characterized by abnormalities of tone, movement, and posture. CP is most commonly classified by the patterns of motor impairment but may also be classified by the extent of functional impairment, the body regions involved, or the underlying etiologies. Classification systems may be used to communicate the extent and pattern of symptoms to help provide optimal care for individuals with CP. (See 'Classification' above.)
●Early clinical signs – Signs of CP in infancy and early childhood include abnormalities in motor tone or significant delays in achieving motor milestones (table 3A-B) as well as abnormal developmental reflexes or the presence of associated conditions such feeding difficulties, growth failure, or epilepsy. (See 'Early signs of cerebral palsy' above.)
●Motor impairments – Common motor abnormalities in CP include spasticity, dyskinesia, and ataxia. Weakness, hypotonia, and sensory abnormalities may also accompany these features. (See 'Common motor features' above.)
●Common motor phenotypes – CP is commonly classified by characteristic patterns of abnormal motor activity, tone, and/or posture. After age 18 to 24 months, signs and symptoms generally point to a specific pattern that corresponds to a motor phenotype of CP (table 1). (See 'Motor phenotypes' above.)
•Spastic CP – Spastic-predominating CP is the most common pattern of motor impairment, accounting for up to 85 percent of individuals with CP. Individuals with spastic-predominating CP have clinical features of an upper motor neuron syndrome including muscle hypertonia, hyperreflexia, extensor plantar responses, and clonus. Spastic CP may be further classified as diplegic, hemiplegic, or quadriplegic based on the distribution of abnormal tone (figure 1). (See 'Spastic subtypes' above.)
•Dyskinetic CP – Dyskinetic CP indicates that a dyskinesia such as dystonia or choreoathetosis is the predominant pattern of abnormal tone. Dyskinetic CP accounts for 15 percent of individuals with CP. (See 'Dyskinetic subtypes' above.)
•Ataxic CP – Ataxic CP is characterized by discoordinated movements and speech, usually associated with widespread disorder of motor function. Ataxic CP is rare, and children who present with these findings should be evaluated for other potential causes of ataxia. (See 'Ataxic subtype' above.)
●Associated conditions – The motor impairments of CP are often accompanied by other disorders of cerebral function that may affect cognition, vision, hearing, language, cortical sensation, attention, vigilance, and behavior. In addition, many children have associated systemic conditions including growth failure, gastrointestinal disorders, sialorrhea, pulmonary disease, orthopedic disorders, osteopenia, urinary disorders, chronic pain, and sleep disorders. (See 'Associated conditions' above.)
In general, children with more severe motor disabilities are more likely to have comorbid conditions.
ACKNOWLEDGMENTS — The UpToDate editorial staff acknowledges Geoffrey Miller, MD and Laurie Glader, MD, who contributed to earlier versions of this topic review.
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