Paediatrics chapters
Cerebral palsy
A permanent, non-progressive disorder of movement and posture from injury to the developing brain: its causes, the spastic, dyskinetic and ataxic types, early warning signs, associated problems and team management.
- Cerebral palsy is a permanent, non-progressive disorder of movement and posture from injury to the immature brain (before birth up to about 2 years); the lesion is static but the clinical picture changes with age.
- Prematurity is the commonest antecedent (periventricular leukomalacia gives spastic diplegia); others are birth asphyxia, kernicterus (athetoid CP), infections and malformations; about half have no clear cause.
- Spastic types are the commonest (diplegia, hemiplegia, quadriplegia); associated problems include epilepsy, intellectual disability, squint, deafness and speech difficulty; care is multidisciplinary physiotherapy, spasticity drugs, orthopaedic surgery and family support.
On this page
Overview
Cerebral palsy (CP) is a group of permanent, non-progressive disorders of movement and posture caused by a lesion in the developing brain. It is a static encephalopathy: the brain injury does not get worse, but the child's signs change as the nervous system matures. The term is used for injuries before birth and in early life; it is rarely applied to lesions after 2 years.
Epidemiology
- Nelson gives a prevalence of about 3.6 per 1000 children; it is much higher in preterm and twin births.
- The lower the birth weight, the higher the risk.
Aetiology
No definite cause is found in about half of cases. Prematurity is the commonest antecedent, yet most affected children are born at term. Fewer than 10% have evidence of intrapartum asphyxia.
| Timing | Causes |
|---|---|
| Prenatal | Intrauterine infections (rubella, CMV, toxoplasmosis), bleeding, thromboembolism, brain malformations (neuronal migration defects, schizencephaly), irradiation, maternal seizures; multiple pregnancy, eclampsia, polyhydramnios, abnormal presentation |
| Perinatal | Prematurity, birth trauma, intraventricular and intracerebral haemorrhage, birth asphyxia (HIE), hypoglycaemia, kernicterus, acidosis |
| Postnatal (about 10%) | Meningitis and head injury (the commonest acquired causes), encephalitis, near-drowning, stroke, child abuse |
In preterm infants, periventricular leukomalacia (PVL) and intraventricular haemorrhage are the main lesions. Periventricular cysts on ultrasound are the best predictor of CP and usually mean spastic diplegia, because fibres to the legs run closest to the ventricles.
Pathology
Widespread cortical atrophy with cavities in severe cases; basal ganglia damage with rigidity and dyskinesia; atrophy of the opposite hemisphere (middle cerebral artery territory) in hemiplegia.
Classification
By type of motor disorder
| Type | Frequency (Nelson) | Lesion and features |
|---|---|---|
| Spastic | 70-80% (commonest) | Pyramidal (upper motor neuron): increased tone (clasp-knife), brisk reflexes, clonus, Babinski |
| Dyskinetic (extrapyramidal) | 10-15% | Basal ganglia: involuntary movements (athetosis, chorea, dystonia) |
| Ataxic | Under 5% | Cerebellum: poor coordination, intention tremor, broad gait |
| Mixed | 10-15% | More than one pattern; more complications |
Spastic types by distribution
| Type | Features |
|---|---|
| Diplegia (largest group) | Legs far more than arms; scissoring from adductor spasm, toe walking, delayed walking; legs may be thin and short; intellect usually normal, seizures uncommon; linked to prematurity and PVL |
| Hemiplegia | One side, arm more than leg; early hand preference (before 1 year is abnormal); walking delayed to 18-24 months with a circumducting gait; affected limbs may be smaller; cognition often spared; seizures common; from vascular insults |
| Quadriplegia | All four limbs; the most severe; linked to severe asphyxia; seizures, intellectual disability, swallowing problems (pseudobulbar palsy), speech and vision problems, flexion contractures, often microcephaly |
| Monoplegia | One limb (rare) |
Dyskinetic (athetoid) CP
- Classically from kernicterus; now mostly from hypoxic-ischaemic injury to the basal ganglia.
- Infants are floppy with poor head control; the involuntary movements appear after the first year.
- Feeding difficulty, drooling, tongue thrust, speech badly affected.
- Upper motor neuron signs and seizures are uncommon; intelligence is often preserved.
- High-tone (sensorineural) deafness is common after kernicterus.
Ataxic and atonic CP
Hypotonia, incoordination and intention tremor; may be genetic. Atonic diplegia combines marked hypotonia with brisk reflexes and severe cognitive delay.
Athetoid CP = kernicterus and the child often has normal intelligence despite severe motor disability; spastic quadriplegia has the highest rates of seizures and intellectual disability.
Clinical features
Early signs in infancy
- Delayed motor milestones (any infant with delayed motor development should be assessed for CP).
- Early hypotonia (floppy for the first months) that gives way to hypertonia.
- Persistent primitive reflexes: Moro beyond about 3 months, stepping reflex beyond about 6 weeks; absent Landau reflex.
- Persistently clenched fists with adducted thumbs, poverty of movement and facial expression.
- Legs extended and crossed (scissoring) when held up under the arms.
- Early hand preference, asymmetry, feeding problems.
- Diagnosis is hard before 6 months, because tone and reflex abnormalities are not obvious until the cortex matures; most are diagnosed by 18 months.
Associated problems
| Problem | Note |
|---|---|
| Epilepsy | Common, especially in quadriplegia and hemiplegia |
| Intellectual disability | About half; not an essential feature |
| Vision | Squint, visual impairment |
| Hearing | Deafness (especially after kernicterus) |
| Speech and swallowing | Dysarthria, drooling, aspiration |
| Growth and nutrition | Failure to thrive, microcephaly in severe forms |
| Orthopaedic | Contractures, hip dislocation, scoliosis, tight Achilles tendon |
| Behaviour and learning | Learning difficulties, behaviour problems |
"Diplegia = Down-low legs, Preterm, PVL"; "Hemiplegia = Hand early, one Half."
Spastic CP is the commonest type (about 70-80%), and diplegia is the largest spastic group as more preterm babies survive.
Persistence of the Moro reflex beyond about 3 months and early hand preference are classic early clues.
A floppy baby can still have CP: tone is often low in the first months and becomes high later.
Diagnosis
- Clinical: careful history (pregnancy, birth, neonatal illness) and examination; document that the problem is not progressive.
- MRI (or CT, cranial ultrasound in infants) shows the lesion (PVL, infarct, malformation).
- EEG if seizures; hearing and vision tests for everyone.
- Suspect another diagnosis if there is regression (loss of skills), consanguinity, an affected sibling, skeletal anomalies (pes cavus, scoliosis) or neurocutaneous signs.
Differential diagnosis
| Condition | Points of difference |
|---|---|
| Degenerative brain disease, leukodystrophy | Progressive, with regression; CSF protein raised in leukodystrophy |
| Hydrocephalus, subdural effusion | Large head, raised ICP signs |
| Brain or spinal cord tumour | Progressive; spinal lesions spare the arms |
| Muscular dystrophy | No spasticity, reflexes reduced, high CK |
| Spinal muscular atrophy, benign hypotonia | Floppy but reflexes absent or normal, normal intellect |
Management
There is no cure; the aim is the best possible function and independence.
- Multidisciplinary team: paediatrician, physiotherapist, occupational and speech therapist, orthopaedic surgeon, teachers and family.
- Physiotherapy and stretching to improve posture and movement and prevent contractures; teach parents how to handle and position the child.
- Spasticity: oral baclofen, diazepam, dantrolene (limited by drowsiness and drooling); botulinum toxin injections; intrathecal baclofen pumps.
- Dyskinesia: trihexyphenidyl; levodopa for athetosis; carbamazepine for dystonia (Akbar).
- Orthopaedic surgery: tendon lengthening, adductor tenotomy, psoas release for fixed contractures; splints and braces.
- Treat associated problems: seizures, feeding and nutrition, vision, hearing, speech, dental care, nutrition.
- Special education and family support.
Prevention
Good antenatal and perinatal care, careful care of low birth weight babies, prompt treatment of apnoea, and prevention of kernicterus.
Prognosis
Outcome depends on the extent of the disability and on the type and intensity of rehabilitation. Physical and intellectual abilities decide how independent the child can become.
Expect: definition (non-progressive, immature brain); prematurity and PVL leading to spastic diplegia; spastic type as the commonest; diplegia versus hemiplegia versus quadriplegia features; kernicterus and athetoid CP with deafness; early signs (persistent Moro, scissoring, early hand preference, delayed milestones); associated epilepsy, intellectual disability and squint; regression pointing to a degenerative disorder; baclofen and botulinum toxin; multidisciplinary care.
Quick revision
- Non-progressive motor disorder from injury to the immature brain.
- Half no cause; prematurity the commonest antecedent; under 10% birth asphyxia.
- PVL in preterms causes spastic diplegia (legs, scissoring, normal intellect).
- Hemiplegia: arm more than leg, early handedness.
- Quadriplegia: most severe, seizures, intellectual disability, bulbar problems.
- Kernicterus: athetoid CP and high-tone deafness.
- Early signs: floppy then stiff, persistent Moro, clenched fists, scissoring.
- Regression means not CP.
- Physiotherapy, spasticity drugs, botulinum toxin, surgery, family support.
Written by The House Job from standard references (Basis of Paediatrics by Pervez Akbar; Nelson Essentials of Paediatrics).
FAQ
Why is cerebral palsy called non-progressive if the child's signs change?
The brain lesion itself does not worsen, but the way it shows changes as the nervous system matures, so a floppy baby may become stiff and involuntary movements may appear later.
Which type of CP is linked with prematurity?
Spastic diplegia, from periventricular leukomalacia damaging the leg fibres that run close to the lateral ventricles.
Is intellectual disability part of cerebral palsy?
Not always. About half of children have it, mostly with spastic quadriplegia, while many with diplegia, hemiplegia or athetoid CP have normal intelligence.
What features suggest a diagnosis other than CP?
Loss of skills already gained, a similar illness in a sibling, consanguinity, skeletal deformities or skin stigmata point to a progressive or genetic disorder.