History and exam
Key diagnostic factors
common
presence of risk factors
Strong risk factors include prematurity, multiple births, maternal illnesses (e.g., chorioamnionitis, TORCH [toxoplasmosis, rubella, cytomegalovirus, herpes simplex] infections, thyroid disease, iodine deficiency, thrombotic disorders), birth asphyxia (due to placental abruption, rupture of the uterus, prolonged/obstructed labour, instrumental delivery), brain malformation, metabolic/genetic disorders, severe hyperbilirubinaemia, periventricular haemorrhage, neonatal sepsis, respiratory distress, early meningitis, neonatal seizures, teratogen exposure, low socioeconomic status.
early general movement abnormalities
Abnormal general movements include persistent cramped synchronous movements prior to age 3 months (corrected age for children born preterm) and absence of fidgety movements at 3 to 4 months corrected age.[69]
abnormal HINE scores
The Hammersmith Infant Neurological Examination (HINE) is a neurological examination tool for evaluating children aged 2 to 24 months (corrected age for children born preterm). Abnormal scores are indicative of CP. [78]
delay in motor development
Delayed motor milestones are often the key diagnostic factor. Assessment of potential delay of developmental milestones should use corrected age for children born preterm, aged up to 2 years.[56][73]
Typically, children sit by age 6 months, crawl with reciprocal locomotion by 9 months, walk between 12 and 18 months, and climb stairs in an adult fashion (step over step) by 3 years.
Gross motor function classification system (GMFCS) levels I-V range from mild impairment of advanced motor skills to total dependence on assistance for all mobility and daily living skills.
The upper extremity in CP requires classification schemes of its own. Reviews support the use of the manual ability classification system (MACS)[9] Manual Ability Classification System Opens in new window and House methods.[103]
delay in speech development
Typically, children talk in short sentences by age 2 years. A delay in speech development may reflect motor delay or an intellectual disability.
Assessment of potential delay of developmental milestones should use corrected age for children born preterm, aged up to 2 years.[56][73]
Speech delay is more prevalent in children with total body involvement.
delay in cognitive/intellectual development
Cognitive impairment is observed in 40% of patients with CP. Intellectual disability is more common in patients with more severe motor involvement (gross motor function classification system [GMFCS] levels IV and V).
retention of primitive reflexes
Reflexes and reactions that are poor prognostic factors for development of independent walking include retention of asymmetric and symmetric tonic neck reflexes, retention of Moro (startle) reflex, retention of neck righting reflex, and presence of lower-extremity extensor thrust response.
Diagnosis of CP can be made as early as age 6 months (corrected age for children born preterm) with up to 98% sensitivity using Prechtl's Assessment of General Movements (GMs), the Hammersmith Infant Neurological Examination (HINE), and neuroimaging.[77][78][79]
lack of age-appropriate reflexes
Lack of parachute reaction and foot placement reaction are poor prognostic factors for development of independent walking.[82]
Diagnosis of CP can be made as early as age 6 months (corrected age for children born preterm) with up to 98% sensitivity using Prechtl's Assessment of General Movements (GMs), the Hammersmith Infant Neurological Examination (HINE), and neuroimaging.[77][78][79]
spasticity/clonus
Spasticity typically develops after the second year of life and manifests when the child attempts activities. It is confirmed by velocity-dependent resistance to passive motion, abnormally increased deep tendon reflexes, and clonus.
Spasticity may be accompanied by a 'clasp knife' phenomenon in which resistance to passive motion abruptly decreases.
selective voluntary motor control impairment
An inability to perform isolated motion of joints without obligatory movement of non-agonist joints can be assessed by tests such as the selective control assessment of the lower extremity (SCALE).[84]
Common for spastic CP.
toe walking/knee hyperextension
Excessive plantar flexion in patients with spastic hemiplegia may manifest as unilateral toe-walking in the young child or knee hyperextension in the older child or adult.
In the child with spastic diplegia, bilateral toe-walking may occur.
scissoring
Hip adductor or medial hamstring spasticity may manifest itself as 'scissoring' (crossing of the legs) during upright activities. Internal rotation of the femora or tibiae may also mimic scissoring.
crouched gait
Excessive dorsiflexion caused by weak plantar flexors, hip or knee flexion contractures, tight hamstrings, or a combination of these factors contributes to a crouched gait in patients with spastic diplegia.
contractures
Progressive contractures or deformities occur during periods of rapid growth and can develop by age 5 years. Fixed contractures are not altered by sleep or anaesthesia.
The severity of contracture and deformity tends to be less in patients with hemiplegia than in those with more global involvement; hip dysplasia and deformity are rare in hemiplegia, but should not be ignored.[85]
Patients with spastic diplegia have bilateral involvement, with the lower extremities being more involved than the upper extremities.
Other diagnostic factors
common
muscle weakness
Common with all subtypes of CP.
joint instability/dislocation
More common as severity of spasticity increases.
uncommon
dystonia
Involuntary, sustained contractions resulting in twisting and abnormal postures.
chorea
Rapid, involuntary, jerky, and fragmented motions. Tone is usually decreased but fluctuating.
athetosis
Slower, constantly changing, writhing, or contorting movements.
ataxia
Involves loss of muscular coordination with abnormal force and rhythm, and impairment of accuracy, resulting in gait and truncal ataxia, poor balance, past pointing, terminal intention tremor, scanning speech, nystagmus and other abnormal eye movements, and hypotonia.
neonatal hypotonia
Early postnatal period is characterised by diminished muscle tone, which becomes progressively hypertonic at ages 16 to 18 months.
scoliosis
More common as severity of spasticity increases.
Risk factors
strong
prematurity
Preterm birth is associated with an increased risk of CP, and the risk increases with decreasing gestational age.[42][43]
In one meta-analysis, prevalence of CP was reported to be 14.6% in children born at 22 to 27 weeks' gestation, 6.2% at 28 to 31 weeks, 0.7% at 32 to 36 weeks, and 0.1% in term infants.[43]
Selective vulnerability of the periventricular white matter occurs between 26 and 34 weeks' gestation, so fetal insults at this time may result in CP.
low birth weight
fetal birth asphyxia
A severe compromise in oxygen and/or cerebral perfusion leads to hypoxic-ischaemic encephalopathy and results in fetal distress during labour.[45]
Possible causes include birth trauma, placental abruption, rupture of the uterus, prolonged/obstructed labour, and instrumental delivery. However, less than 10% of cases of CP are believed to be related to birth asphyxia, and use of electronic fetal monitoring has not been shown to be a factor in preventing CP.[33][34]
Amplitude-integrated electroencephalogram predicts long-term neurodevelopmental outcome in term infants with hypoxic-ischaemic encephalopathy.[46][47]
multiple births
Multiple births are at an increased risk of CP; in one UK study, the reported prevalence of CP per 1000 live births was 2.3 for singletons, 12.6 for twins, and 44.8 for triplets.[16]
maternal illness
Term and near-term infants are at particular risk if the mother develops chorioamnionitis and/or fever.
TORCH (toxoplasmosis, rubella, cytomegalovirus, herpes simplex) infections during pregnancy can affect the developing brain.[22][27]
Maternal thyroid disease and iodine deficiency require antenatal screening, as maternal thyroid levels affect fetal brain development.
Thrombotic disorders including factor V Leiden mutations are greater in mothers of children with CP than in those without.[25][26]
fetal brain malformation
Known risk factor for CP. Hemiplegia may be due to focal lesions in utero.
major birth defects
In one case-control study of term singletons, birth defects in combination with growth restriction contributed substantially to the risk for CP.[30][31] Birth defects were recognised in 5.5% of neonates in the control group compared with more than half of neonates with CP (without hypoxic-ischaemic encephalopathy).[30]
While birth defects are more commonly detected in preterm infants in the general population, there is an increase in major birth defects (by a factor of 9) in full-term children with CP compared with preterm babies with CP.[32]
familial metabolic/genetic disorder
Genetic dysfunction may be a predisposing factor in developing early brain damage. For example, single nucleotide polymorphisms of the excitatory amino acid transporter 2, with resultant impairment of glutamate uptake, has been related to increased susceptibility to brain injury in very premature infants.[48]
Research suggests that mutations disrupting neuritogenesis genes confer risk for CP.[49]
Familial metabolic and/or genetic disorders may masquerade as cerebral until a more definitive diagnosis is established.[48]
neonatal complications
Severe hyperbilirubinaemia (now largely preventable) leads to damage to basal ganglia due to deposition of bilirubin byproducts, causing dyskinesia.
Significant periventricular haemorrhage (grade III and IV), especially in premature infants, may result in CP.[50]
Neonatal sepsis, especially in very low birth weight infants, is a particular risk factor.[51] Around 25% of infants who survive neonatal seizures develop CP.[35][50]
meningitis
Meningitis during early development is a strong risk factor for CP.[52]
maternal teratogen exposure
low socioeconomic status
Low income levels were associated with a twofold increase in CP in one study.[17]
weak
non-vertex presentation
The association of CP with abnormal presentation and difficult birth may be an effect of pre-existing difficulties rather than a cause.[23]
postmaturity
May be a risk factor for CP.
head injury
Injuries to the developing brain (prior to age 3 years) result in a CP-like syndrome, as opposed to injuries to the developed brain, which result in a stroke-like presentation. Includes non-accidental head injury (child abuse) and injury associated with shaken baby syndrome.
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