Key Facts

Cerebral palsy (CP) is an umbrella term for a group of conditions that affect motor function. Although the brain lesion itself is static, the clinical manifestations evolve as the child develops. Comprehensive multidisciplinary management significantly improves function, participation, and quality of life.

Epidemiology

Prevalence is approximately 2-2.5 per 1,000 live births. Incidence has remained relatively stable despite advances in neonatal care, as improved survival of very preterm infants offsets reduced rates of birth asphyxia. CP is more common in boys (1.3:1). Higher incidence in preterm infants (~7% in those born <28 weeks vs ~0.1% in term infants).

Aetiology

Prenatal (70-80%): Cerebral malformations, congenital infections (CMV, toxoplasmosis, rubella), placental insufficiency, maternal factors (pre-eclampsia, chorioamnionitis), genetic factors, stroke in utero.

Perinatal (10-15%): Hypoxic-ischaemic encephalopathy (HIE), preterm birth (periventricular leucomalacia, intraventricular haemorrhage), neonatal stroke.

Postnatal (5-10%): Meningitis/encephalitis, non-accidental head injury, accidental head trauma, near-drowning, kernicterus.

Pathophysiology

The nature and location of brain injury determine the clinical pattern:

• Periventricular leucomalacia (PVL): Damage to white matter adjacent to lateral ventricles → spastic diplegia (legs > arms, as leg motor fibres are most medial)
• Middle cerebral artery territory infarct: → Spastic hemiplegia (contralateral)
• Basal ganglia damage (e.g., acute severe HIE, kernicterus): → Dyskinetic/athetoid CP
• Cerebellar damage: → Ataxic CP
• Widespread cortical damage: → Spastic quadriplegia

Motor Features by Type

• Spastic CP (~80%): Increased tone (velocity-dependent), brisk reflexes, clonus, positive Babinski
  • Unilateral (hemiplegia): One side affected, arm usually > leg, hand preference before 12 months, toe-walking
  • Bilateral diplegia: Legs > arms, scissoring gait, toe-walking
  • Bilateral quadriplegia: All limbs severely affected, poor head control, often with bulbar dysfunction
• Dyskinetic CP (~10-15%): Involuntary movements — athetosis (writhing), dystonia (sustained postures), chorea; variable tone; basal ganglia injury
• Ataxic CP (~5%): Cerebellar — intention tremor, broad-based gait, dysmetria, hypotonia
• Mixed: Features of more than one type

Early Signs (First Year)

• Abnormal tone (hypotonia progressing to spasticity)
• Persistence of primitive reflexes beyond expected age
• Delayed motor milestones (not sitting by 9 months, not walking by 18 months)
• Hand preference before 12 months (suggests hemiplegia)
• Feeding difficulties

Comorbidities

• Intellectual disability (~50%)
• Epilepsy (~25-35%)
• Visual impairment (~40%) — cortical visual impairment, strabismus
• Communication difficulties (~50-60%)
• Hearing impairment (~10%)
• Feeding/swallowing difficulties, GORD, drooling
• Pain (musculoskeletal, spasticity-related)
• Hip displacement/subluxation (~30% of GMFCS IV-V)
• Behavioural/emotional difficulties
• Osteoporosis

Red Flags

• Progressive neurological deterioration — reconsider diagnosis; CP is non-progressive
• New onset of focal neurological signs — consider new pathology
• Severe hip pain — hip surveillance, X-ray for displacement
• Respiratory deterioration — aspiration, scoliosis-related restriction

Bedside

• Neurological examination: Tone, reflexes, posture, movement patterns, primitive reflexes
• GMFCS assessment: Classify gross motor function level (I-V)
• Developmental assessment: Cognitive, language, adaptive, motor domains
• Growth: Use CP-specific growth charts for GMFCS IV-V
• Hip surveillance: Clinical examination ± X-ray (essential for GMFCS III-V)

Bloods

• Not diagnostic for CP; may help exclude other conditions:
  • CK: Exclude muscular dystrophy
  • Metabolic screen: Amino acids, organic acids, lactate — if atypical or progressive features
  • TFTs: Exclude thyroid disease
  • Genetic testing: Chromosomal microarray, exome sequencing — increasingly performed if no clear aetiology

Imaging

• MRI brain: Recommended for all children with CP (NICE NG62) — abnormal in ~80-90%
  • PVL: High signal in periventricular white matter
  • Basal ganglia injury: High signal in basal ganglia/thalami (HIE)
  • Cortical/subcortical infarction: Territory-specific changes
  • Malformations: Schizencephaly, lissencephaly, polymicrogyria

Special Tests

• EEG: If epilepsy suspected
• Video swallow/FEES: If aspiration or feeding difficulties
• Hearing assessment: Formal audiological evaluation
• Visual assessment: Ophthalmology review
• Hip X-ray (migration percentage): Per hip surveillance programme — baseline at 12-18 months if GMFCS III-V

Non-pharmacological

• Physiotherapy: Core intervention — maintaining range of motion, strengthening, functional mobility, postural management
• Occupational therapy: Adaptive equipment, seating, hand function, activities of daily living
• Speech and language therapy: Communication (including AAC — communication aids), swallowing management
• Orthotics: AFOs (ankle-foot orthoses), standing frames, spinal bracing
• 24-hour postural management: Seating, sleep systems, standing programmes
• Education: EHCP, mainstream or specialist school as appropriate
• Psychological support: For child and family

Pharmacological

• Spasticity management:
  • Oral baclofen: 2.5mg TDS initially, titrate to max 60mg/day (adults); sedation, withdrawal risk
  • Diazepam: 0.5-1mg/kg/day in divided doses — short-term use; sedation, tolerance
  • Tizanidine: Alpha-2 agonist; off-licence in children
  • Botulinum toxin A (Botox): Focal spasticity — inject into affected muscles; onset 2-3 days, duration 3-6 months; commonly gastrocnemius/soleus for equinus
• Intrathecal baclofen pump: For severe generalised spasticity (GMFCS IV-V); surgically implanted pump delivers baclofen directly to spinal cord
• Pain management: Paracetamol, NSAIDs, gabapentin for neuropathic pain
• Epilepsy treatment: Standard anti-seizure medications as indicated
• GORD: Omeprazole, dietary modifications
• Drooling: Glycopyrronium 20-40mcg/kg TDS; botulinum toxin to salivary glands

Surgical/Interventional

• Orthopaedic: Soft tissue releases, tendon transfers, hip reconstruction (salvage or containment), spinal fusion for scoliosis
• Selective dorsal rhizotomy (SDR): Selective cutting of dorsal nerve rootlets — permanent spasticity reduction; best outcomes in spastic diplegia GMFCS II-III, age 3-9 (NICE IPG373)
• Gastrostomy (PEG): For children with unsafe swallow or inadequate oral intake
• Tracheostomy: Rarely, for severe upper airway obstruction

Referral Criteria

• Suspected CP — refer early to community paediatrics/child development team
• NICE NG62: All children with suspected CP should be assessed by a specialist before age 2
• MDT approach: Regular review by physiotherapy, OT, SALT, orthotics, orthopaedics, neurology as needed

• Life expectancy: Highly variable by GMFCS level — GMFCS I-III: near-normal life expectancy; GMFCS V: significantly reduced (50% survive to age 30)
• Motor: GMFCS level at age 5 is the best predictor of adult motor function; most motor development occurs by age 5-7
• Walking: GMFCS I-III typically achieve independent or aided walking; GMFCS IV-V are wheelchair-dependent
• Cognitive: ~50% have intellectual disability; GMFCS level does not predict cognitive ability (dyskinetic CP often preserves cognition)
• Pain: ~75% of adults with CP report chronic pain
• Hip displacement: Occurs in ~30% of GMFCS IV-V; surveillance prevents late painful dislocation
• Quality of life: Self-reported quality of life in children with CP is comparable to peers in many domains with appropriate support

GMFCS Classification

CP Classification Summary