• Cerebral palsy (CP) encompasses non-progressive motor impairment syndromes that result from damage to or dysfunction of the developing brain.
• Subtypes are defined by type of neurological impairment and anatomic distribution:
  • Spastic CP (pyramidal CP) (40%): increased deep tendon reflexes, sustained clonus, hypertonia, and the clasp-knife response; spastic diplegia—lower extremity involvement; spastic hemiplegia—one side of the body involved; and spastic quadriplegia—total-body involvement.
  • Dyskinetic CP (30%): fluctuating tone, rigidity (coactivated antagonists). Total-body involvement by definition. Persistent primitive reflex patterns [e.g., asymmetric tonic neck reflex (ATNR), labyrinthine] often seen. Subtypes—athetoid CP: slow writhing movements (or chorea: rapid, random, jerky movements), and dystonic CP: posturing of the head, trunk, and extremities.
  • Ataxic CP (10%): characterized by cerebellar signs (ataxia, dysmetria, past-pointing, tremor, nystagmus) and abnormalities of voluntary movement
  • Mixed CP (10%): two or more types codominant, most often spastic and dyskinetic
• Other CP (10%): criteria for CP met but specific subtype cannot be defined
• Extrapyramidal CP: sometimes applied to non-spastic types of CP as a group

• Prevalence approximately 2 per 1000
• Approximately 50% of cases are associated with prematurity.
• Increased incidence with multiple gestation (10% were twins in one study)
• 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 ratio, 1.3:1
• Inconsistent correlation to maternal age, socioeconomic status, and parity
• Epidemiological studies suggest that prenatal factors are more strongly associated with subsequent CP than perinatal or postnatal factors; however, individual risk factors are poorly predictive of subsequent CP in the individual child.
• Diagnosis of perinatal asphyxia requires evidence of multiorgan system hypoxic-ischemic insult and severe encephalopathy (e.g., neonatal seizures, severe hypotonia), and accounts for only about 9% of cases of CP.

• In majority of cases, etiology not apparent
• Epidemiological studies indicate two types of vulnerability to CP: prematurity-related and IUGR-related.
  • Prematurity-related: unique vulnerability of the periventricular white matter between 28 and 32 weeks of gestation results in periventricular leukomalacia
  • IUGR-related: in full-term infants, fetal abnormalities associated with CNS dysgenesis, non-CNS malformation, teratogens, growth retardation, evidence of hypoxic-ischemic encephalopathy more often seen

• Sensory
  • Sensorineural and conductive hearing loss
  • Impairments of visual acuity
  • Oculomotor dysfunction
    • Strabismus
  • Somatosensory impairments
• Cognitive
  • Mental retardation (MR) in about 50%, especially in spastic quadriparesis
  • High incidence of learning disabilities
    • Attention deficit/hyperactivity disorder
    • Sleep and behavioral disturbances
• Neurological
  • Seizures
  • Hydrocephalus
• Musculoskeletal
  • Contractures
  • Hip subluxation/dislocation
  • Scoliosis
• Cardiorespiratory
  • Upper airway obstruction
  • Aspiration pneumonitis
  • Restrictive lung disease
    • Secondary to thoracic deformity
  • Reactive airway disease
• Gastrointestinal tract/nutrition
  • Failure to thrive
  • Gastroesophageal reflux
  • Constipation
  • Oral motor dysfunction/dysphagia
• Gastrourinary tract
  • Neurogenic bladder
• Skin
  • Decubitus ulcers
• Dental
  • Caries
  • Gingival hyperplasia
  • Abnormalities of enamel (congenital)

• Motor impairment syndromes related to spinal cord, lower motor neuron, peripheral nerve, or primary muscular disease
• 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 (especially associated with recurrent vomiting) are clues, but presentation can be insidious.

Question: Prenatal history
Significance: Exposure to toxins/drugs, infections or fever, HIV/STD risk, vaginal bleeding, abnormal fetal movement, pre-eclampsia (especially proteinuria), breech position, poor maternal weight gain, premature labor, fetal distress, IUGR, prenatal testing, placental disorders

Question: Perinatal history
Significance: Premature delivery, neonatal resuscitation, low Apgar scores (7 at 5 and 10 minutes), birth trauma, evidence of neonatal encephalopathy (seizures, severe hypotonia), complicated neonatal course (intraventricular hemorrhage, prolonged respiratory support, meningitis, sepsis, hyper-bilirubinemia)

Question: Post-natal history
Significance: Hospitalization for severe infection or trauma, periodic or persistent deterioration in function (suggests neurodegenerative/metabolic disease)

Question: Development
Significance: Severe delay in motor milestones (e.g., not rolling at 7 months, not sitting at 8 months, not walking at 15 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.

Finding: Respiratory pattern
Significance: Obstruction, aspiration risk, evidence of dysmorphism/pigmentary skin changes and growth abnormalities contribute to assessment of etiology.

Finding: Head circumference
Significance: To evaluate for microcephaly/macrocephaly/hydrocephaly

Finding: Strabismus/cataracts/iris or retinal abnormalities
Significance: Either cranial nerve damage, muscle imbalance, metabolic disease, or congenital infection.

Finding: Range of motion
Significance: Decreased with contractures

Finding: Leg-length discrepancy
Significance: Hip dislocation

Finding: Spinal curvature
Significance: Neuromuscular imbalance

Finding: In addition to formal examination, documentation of best level of visual motor/manipulative skills (i.e., able to run, transfer, hold a cup, etc.)
Significance: Helpful in following the course of the motor impairment.

Finding: Cranial nerves
Significance: Especially strabismus, speech and swallowing, vision and hearing

Finding: Tone
Significance: Spasticity versus rigidity versus hypotonia

Finding: Strength
Significance: Often decreased

Finding: Clasp-knife response
Significance: Hyperactive deep tendon reflexes, and clonus in spasticity; Babinski reflex (extensor response to plantar stimulation)

Finding: Persistent primitive reflexes
Significance: CNS damage

Finding: Protective reactions
Significance: Head and trunk righting, prop reactions, parachute; cerebellar signs

Finding: Postural stability
Significance: Neuromuscular imbalance

Finding: Gait abnormalities, subtle seizures
Significance: Motor or CNS damage

Test: Hearing and vision
Significance: All in first year with regular follow-up examinations (identification of sensory deficits and clues to etiology)

Test: Brain imaging
Significance: Should be performed when hydrocephalus is suspected; frequently useful in determining etiology

Test: Genetic and metabolic studies
Significance: Indicated when the history and physical examination suggest a progressive or hereditary disorder.

Test: X-ray studies
Significance: Should be done routinely in spastic diparesis for hip dislocation; usefulness of scoliosis films depends on physical findings.

Test: Audiological evaluation
Significance: Required for those with language delay or those who manifest hearing impairment.

Test: Radionucleotide studies or pH probe
Significance: “Milk scan”; evaluate gastroesophageal reflux (GER), gastric emptying, aspiration

Test: Blood tests—chemistries, liver-function studies, cell counts
Significance: Evaluate nutritional/metabolic status, anticonvulsant levels

Test: Urodynamic studies
Significance: Spastic bladder, indicated in those with recurrent urinary tract infections or voiding dysfunction.

Test: Sleep study
Significance: May disclose treatable obstructive sleep apnea in those with excessive somnolence or abnormal sleep-wake cycles.

Test: Pulmonary-function studies
Significance: Helpful in documenting progressive restrictive pulmonary dysfunction (e.g., in severe scoliosis).

Test: EEG
Significance: Seizure disorder is suspected

• Family-centered care is directed toward optimizing function/minimizing handicap (habilitation).
• Interdisciplinary clinics: to provide multiple services (medical, surgical, therapy, etc.) coordinated with primary care physician.
• Education services: recent emphasis on inclusion/mainstreaming; for many children, special education services are still required and appropriate.
• Physical, occupational, speech/language therapy, other allied health professionals: therapy provided in home, school, and hospital settings, directed primarily at improved functioning in the areas of mobility, self-care, and communication; orthodontists for braces.
• Social services: provided in a variety of contexts to aid in the coordination of care

• Requirements for follow-up vary greatly with the degree of disability and the scope of impairments. 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 early hip subluxation, which is best managed before progression to frank dislocation.
• Early referral for developmental assessment to establish need for early intervention, to optimize development, and to promote family coping

• Overdiagnosis of CP in infants with spastic hypertonia (especially those born prematurely): normalization of tone/function may take up to 2 years.
• Slowly progressive neurogenerative disease may masquerade as CP.
• Cervical cord lesions may masquerade as quadriparetic spastic CP. Swallowing reflexes may be preserved, cognitive development is normal.
• Determination of ideal body weight may be complex in CP; growth standards according to CP type are under development.

Q: Is severe clumsiness a form of CP?
A: Mild spastic diplegia or hemiplegia may present this way, but spasticity and contractures distinguish these from developmental coordination disorders.

Q: Do children with CP also have mental retardation?
A: Not necessarily; although 50% have mental retardation, it is not a part of the definition of CP.

Q: What about surgery for CP?
A: Spasticity in the lower extremities may be addressed directly with selective dorsal rhizotomy (interruption of afferent limb of stretch reflex arch). Otherwise, surgical therapy in CP is directed at associated conditions; many children with CP undergo orthopedic procedures for hip dislocation, release of contractures, and scoliosis. Surgery for correction of strabismus or placement of a gastrostomy tube is commonly performed.

Badawi N, Watson L, Petterson B, et al. What constitutes cerebral palsy? Dev Med Child Neurol 1998;40(8):520–527.

Freeman JM, Nelson KB. Intrapartum asphyxia and cerebral palsy. Pediatrics 1988;82:240–249.

Golden CG. Apgar scores as predictors of chronic neurologic disability, by Karin B. Nelson, MD, and Jonas H. Ellenberg, PhD, Pediatrics, 1981;68:36–44. Pediatrics 1998;102(1 Pt 2):262–264.

Kuban KC, Leviton A. Cerebral palsy. N Engl J Med 1994;330:188–195.

Nelson KB, Ellenberg JH. Antecedents of cerebral palsy: multivariate analysis of risk. N Engl J Med 1986;315:81–86.