Cerebral palsy

Question 1

Definition of Cerebral palsy:

Answer 1

• A spectrum of permanent motor impairment caused by non-progressive CNS lesions sustained before 2yrs of age (developing brain)
• MRI changes MUST be present in order to confirm the diagnosis

Question 2

Aetiology / Risk factors of Cerebral palsy

• Antenatal
• Perinatal
• Postnatal

Answer 2

• Antenatal (80%)
  
  • Prematurity
  • Multiple birth
  • Maternal factors
    
    • TORCH infections
      
      • Toxoplasmosis
      • Rubella
      • Cytomegalovirus
      • Herpes simplex
    • Thyroid/iodine deficiency
    • Thrombotic disorders (factor V Leiden) - Neonatal stroke
  • Teratogen exposure
• Perinatal
  
  • Birth asphyxia (10%)
  • Birth trauma
• Postnatal
  
  • Meningitis/encephalitis
  • Head trauma/intraventricular haemorrhage
  • Hyperbilirubinaemia (Kernicterus 2º to Rh incompatability)

Question 3

Classification of Cerebral palsy

• Spastic
• Dyskinetic
• Ataxic

Answer 3

• Spastic
  
  • Upper motor neuron (pyramidal) lesion
  • ↑ muscle tone (spasticity)
    
    • Velocity-dependent (↑ speed of stretch = ↑ resistance) → Dynamic ‘catch’ (hallmark)
  • Hyperreflexia
  • Extensor plantar reflex (Babinski sign)
    
    • Hemiplegia
      
      • Unilateral involvement of arm & leg (arm > leg) → Asymmetrical Moro reflex
      • Early development of hand preference
      • Antenatal aetiology
    • Diplegia
      
      • Legs affected > arms
      • Child may appear normal until picked up (legs ‘scissor’)
      • Primarily premature infants, associated with periventricular leukomalacia
    • Quadriplegia
      
      • Most severe form
      • All limbs and trunk affected
      • Associated with:
        
        • Seizures
        • ↓ IQ
        • Dysphagia (→ aspiration pneumonia)
• Dyskinetic
  
  • Basal ganglia lesion (extrapyramidal)
  • Involuntary movements
  • Causes:
    
    • Hyperbilirunaemia (kernicterus) due to Rh disease of newborn
    • Hypoxic-ischaemic encephalopathy (HIE) at term
    • Hypoglycaemia
      
      • Dystonia: characterised by 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
• Ataxic (hypotonic)
  
  • Cerebellar lesion → uncoordinated movements
  • Early hypotonia (trunk and limb), ataxic gait, poor balance, delayed motor development, intention tremor

Question 4

History, Signs & Symptoms for Cerebral palsy

Answer 4

• Abnormal tone and posture
• Delayed motor milestones
• Feeding difficulties
• Retention of primitive reflexes (beyond 4 months)
• Spasticity (velocity-dependent), brisk reflexes, clonus
• Early hand preference (Hemiplegia)
• Toe-walking
• Scissoring (Diplegia)
• Contractures (deformity/rigidity of joints)
• Delay in motor, speech, cognitive/intellectual development

Question 5

Investigations for Cerebral palsy

Answer 5

• Brain MRI
  
  • Periventricular leukomalacia: necrosis of white matter around the lateral ventricles due to hypoxic-ischaemic injury
  • Stroke
  • Haemorrhage
  • Congenital malformation
• Other
  
  • Coagulation studies
  • Genetic analysis
  • Metabolic screen (inborn errors of metabolism)
  • X-ray (affected joints)

Question 6

Management of Cerebral palsy

Answer 6

• Multidisciplinary
• Pharmacological (↓ spasticity)
  
  • Botulinum toxin (IM)
  • Baclofen (intrathecal infusion)
• Neurosurgery
  
  • Selective posterior rhizotomy (selective nerve dissection)