Basics
Description
Cerebral palsy (CP) describes a group of disorders of movement and posture, limiting activity, attributed to nonprogressive underlying brain pathology. The motor disorders of CP are often accompanied by disturbances of sensation, cognition, communication, perception, and/or behavior or by a seizure disorder:
- Spastic (pyramidal; 75%): increased deep tendon reflexes, sustained clonus, hypertonia, and the clasp-knife response:
- Spastic diplegia: lower extremity involvement
- Spastic hemiplegia: 1 side of the body involved
- Spastic quadriplegia: total body involvement; usually associated with dystonia
- Dyskinetic (10%): fluctuating tone, rigid total body involvement by definition. Persistent primitive reflex patterns (asymmetric tonic neck reflex, labyrinthine)
- Athetoid: slow writhing movements (or chorea; rapid, random, jerky movements)
- Dystonic: posturing of the head, trunk, and extremities
- Ataxic (<10%): characterized by cerebellar signs (ataxia, dysmetria, past pointing, tremor, nystagmus) and abnormalities of voluntary movement
- Mixed (10%): 2 or more types codominant, most often spastic and dyskinetic
- Other (10%): Criteria for CP met, but specific subtype cannot be defined.
- Extrapyramidal: sometimes applied to nonspastic types of CP as a group
Epidemiology
- ~50% of cases are associated with prematurity.
- Increased concordance among monozygotic versus dizygotic twins in some studies (not in others)
- Intrauterine growth retardation (IUGR) more common in CP than controls, especially for full-term infants in whom CP develops.
- Male > female (1.3:1)
- Inconsistent relationship to maternal age, socioeconomic status, and parity
- Prenatal factors are more strongly associated with subsequent CP than are perinatal or postnatal factors; however, individual risk factors are poorly predictive of subsequent CP in the individual child.
- Perinatal asphyxia accounts for only ~9% of CP; diagnosis requires evidence of hypoxic-ischemic insult, severe encephalopathy (e.g., neonatal seizures, severe hypotonia), and consistent laboratory/radiologic findings.
- Increased with multiple gestation (10% were twins in 1 study)
- Prevalence ~2-3/1,000
Etiology
- Not apparent in most cases. A more recently recognized perinatal factor is the presence of chorioamnionitis; mild or even subclinical infection may have increased association with CP.
- Epidemiologic studies indicate 2 types of associated vulnerability to CP:
- Prematurity: Vulnerability of the periventricular white matter between 28 and 32 weeks of gestation results in periventricular leukomalacia.
- IUGR: Fetal growth retardation associated with CNS dysgenesis, non-CNS malformation, teratogens, growth retardation, evidence of hypoxic-ischemic encephalopathy.
Commonly Associated Conditions
- Sensory
- Sensorineural and conductive hearing loss
- Impaired visual acuity
- Oculomotor dysfunction
- Strabismus
- Cortical visual impairment
- Somatosensory impairments
- Cognitive/developmental
- Intellectual disability in ~50%, especially in spastic quadriparesis
- Autism, ADHD
- Language and learning disabilities
- Dysarthria
- Sleep and behavioral disturbances
- Neurologic
- Musculoskeletal
- Contractures
- Hip subluxation/dislocation
- Scoliosis
- Cardiorespiratory
- Upper airway obstruction
- Aspiration pneumonitis
- Restrictive lung disease/thoracic deformity
- Reactive airway disease
- GI/nutritional
- Poor growth
- Gastroesophageal reflux
- Constipation
- Oral motor dysfunction/dysphagia
- Urinary: neurogenic bladder
- Skin: decubitus ulcers
- Dental
- Malocclusions
- Caries
- Gingival hyperplasia
- Abnormalities of enamel (congenital)
Diagnosis
History
- Prenatal
- Exposure to toxins/drugs
- Infections or fever
- HIV/STD risk
- Vaginal bleeding
- Abnormal fetal movement
- Preeclampsia (especially proteinuria)
- Breech position
- Poor maternal weight gain
- Premature labor
- Fetal distress
- IUGR
- Prenatal testing
- Placental disorders
- Perinatal
- Premature delivery
- Neonatal resuscitation
- Low Apgar scores (<5 at 5 minutes)
- Birth trauma
- Evidence of neonatal encephalopathy (seizures, lethargy, hypotonia)
- Complicated neonatal course (intraventricular hemorrhage, prolonged respiratory support, meningitis, sepsis, hyperbilirubinemia)
- Postnatal
- Hospitalization for severe infection or trauma
- Periodic or persistent deterioration in function (suggests neurodegenerative/metabolic disease)
- Development
- Significant delay in motor milestones/motor quotient (age of typical skill attainment/age of attainment <0.5) (e.g., not rolling at 10 months, not sitting at 12 months, not walking at 24 months)
- Associated with persistent primitive reflexes (e.g., prominent tonic neck and labyrinthine responses at 1 year of age) and delayed or absent development of protective reactions (e.g., lateral prop at 7 months, parachute at 13 months)
- Associated delays in language, play, social, and adaptive behavior
Physical Exam
- General observation: evidence of dysmorphism/pigmentary skin changes and growth abnormalities
- Head circumference: to evaluate for microcephaly/macrocephaly/hydrocephaly; growth velocity points to timing of brain pathology
- Strabismus/cataracts/iris or retinal abnormalities: eye exam: cranial nerve damage, muscle imbalance, metabolic disease, or congenital infection
- Musculoskeletal
- Decreased range with contractures
- Leg length discrepancy: hip dislocation
- Spinal curvature/scoliosis
- Neurologic
- Documentation of best level of visual motor/manipulative skills (transfer, hold a cup): to follow course of motor impairment
- Cranial nerves: strabismus, speech and swallowing, vision and hearing
- Tone: spasticity versus rigidity versus hypotonia
- Strength: often decreased
- Hyperactive deep tendon reflexes and clonus in spasticity; Babinski reflex (extensor response to plantar stimulation)
- Persistent primitive reflexes
- Protective reactions: head and trunk righting, prop reactions, parachute; cerebellar signs
- Balance, stability
Alert
Pitfalls
- Overdiagnosis of CP in premature infants with spastic hypertonia; normalization of tone/function may take up to 2 years.
- False or premature assumption of cognitive deficit in children with severe dysarthria. May take years of augmentative communication supports to determine true potential
- Slowly progressive neurodegenerative diseases and pediatric neurotransmitter disorders may masquerade as CP.
- Cervical cord lesions may masquerade as quadriparetic spastic CP.
- Determination of ideal body weight/caloric requirements may be complex in CP; skinfold measuring <10th percentile is the best indicator of poor nutrition.
- Pain is a common problem, with more than half of adults and children with CP reporting pain as an ongoing health concern.
Diagnostic Tests & Interpretation
Lab
- Genetic and metabolic studies: if history or physical suggests a progressive or hereditary disorder
- Blood chemistries, liver function studies, cell counts: evaluate nutritional/metabolic status, anticonvulsant levels
Imaging
- Brain imaging: perform when hydrocephalus is suspected; can help determine etiology
- Radiography: should be done routinely in spastic diparesis for hip dislocation; consider scoliosis films.
- Radionuclide studies to evaluate gastroesophageal reflux, gastric emptying, aspiration
Diagnostic Procedures/Other
- Hearing and vision: all in 1st year, with regular follow-up exams
- Audiologic evaluation required per guidelines
- Urodynamic studies: spastic bladder in those with recurrent UTIs or voiding dysfunction
- Sleep study: may disclose treatable obstructive sleep apnea in those with somnolence or abnormal sleep-wake cycles
- Pulmonary function studies: document progressive restrictive pulmonary dysfunction (e.g., in severe scoliosis)
- Consider bone density: liability to fracture
- Brain wave (EEG): if seizure suspected
Differential Diagnosis
- Motor syndromes related to spinal cord, lower motor neuron, peripheral nerve, primary muscular disease, or progressive disorders of the basal ganglia (dopa-responsive dystonia)
- Connective tissue disorders (primary and secondary) resulting in musculoskeletal abnormalities (e.g., arthrogryposis multiplex, skeletal dysplasias)
- Inborn errors of metabolism and CP: protean manifestations, dyskinesia, ataxia, postnatal growth failure, neurologic deterioration, recurrent vomiting
Treatment
Additional Treatment
General Measures
- Family-centered care is directed toward optimizing activity and participation.
- Interdisciplinary clinics: services (medical, surgical, therapy) coordinated with primary physician
- More frequent health maintenance visits and coordination meetings from a medical home practice may assist in managing multiple chronic associated heath conditions.
- Spasticity reduction with IM injections of botulinum toxin is well supported. Oral or intrathecal baclofen used increasingly, although consensus on functional improvement long term is variable.
- Orthopedic management with directed procedures to reduce contractures and improve posture has more evidence on improving functional outcomes long term.
- Education services: recent emphasis on inclusion/mainstreaming; for many, special education services are still required.
- Augmentative communication supports especially for nonverbal/dysarthric children.
- Physical, occupational, speech/language therapy, other allied health professionals: Therapy provided in home, school, and hospital settings; directed primarily at improved mobility, self-care, and communication; orthodontists for braces
- Counseling support for children coping with chronic disability
- Social services: provided in a variety of contexts to aid in the coordination of care
- Vocational counseling and employment options, assistance with transition to adulthood, self-advocacy, self-determination
- Transition to adult health care system
Ongoing Care
Follow-up Recommendations
- Requirements for follow-up vary greatly with the degree of disability and impairment. An interdisciplinary clinic setting may be more appropriate for a child with severe CP.
- Early referral to a pediatric orthopedist is indicated, especially for monitoring of the hip.
- Early referral for developmental assessment: need for early intervention to optimize development and promote family coping
Diet
Nutritional assessment and support for those with dysphagia or poor growth (especially calcium, vitamin D intake)
Additional Reading
- Delgado MR, Hirtz D, Aisen M, et al; Quality Standards Subcommittee of the American Academy of Neurology, Practice Committee of the Child Neurology Society. Practice parameter: pharmacologic treatment of spasticity in children and adolescents with cerebral palsy (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society. Neurology. 2010;74(4):336-343. [View Abstract]
- Liptak GS, Murphy NA. Providing a primary care medical home for children and youth with cerebral palsy. Pediatrics. 2011;128(5):e1321-e1329. doi:10.1542/peds.2011-1468. [View Abstract]
- Pakula AT, Van Naarden Braun K, Yeargin-Allsopp M. Cerebral palsy: classification and epidemiology. Phys Med Rehabil Clin N Am. 2009;20(3):425-452. [View Abstract]
- Rosenbaum P, Paneth N, Leviton A, et al. A report: the definition and classification of cerebral palsy April 2006. Dev Med Child Neurol Suppl. 2007;109:8-14. doi:10.1111/j.1469-8749.2007.tb12610.x. [View Abstract]
- Strauss D, Brooks J, Rosenbloom L, et al. Life expectancy in cerebral palsy: an update. Dev Med Child Neurol. 2008;50(7):487-493. [View Abstract]
Codes
ICD09
- 343.9 Infantile cerebral palsy, unspecified
- 343.0 Congenital diplegia
- 343.1 Congenital hemiplegia
- 343.2 Congenital quadriplegia
- 343.4 Infantile hemiplegia
- 343.3 Congenital monoplegia
- 343.8 Other specified infantile cerebral palsy
ICD10
- G80.9 Cerebral palsy, unspecified
- G80.1 Spastic diplegic cerebral palsy
- G80.2 Spastic hemiplegic cerebral palsy
- G80.0 Spastic quadriplegic cerebral palsy
- G80.4 Ataxic cerebral palsy
- G80.3 Athetoid cerebral palsy
- G80.8 Other cerebral palsy
SNOMED
- 128188000 Cerebral palsy (disorder)
- 275469001 Congenital diplegia
- 43486001 Hemiplegic cerebral palsy
- 371119007 Quadriplegic spastic cerebral palsy (disorder)
- 56409008 Monoplegic cerebral palsy
- 230773005 Spastic cerebral palsy
- 278512001 Ataxic cerebral palsy (disorder)
- 75019001 Athetoid cerebral palsy (disorder)
FAQ
- Q: Is severe clumsiness a form of CP?
- A: Mild spastic diplegia or hemiplegia may present this way, but tone abnormalities and significant functional impairments distinguish CP from milder developmental coordination disorders.
- Q: Do all children with CP also have intellectual disability?
- A: Only ~50% have intellectual disability.