What is Cerebral Palsy (CP) and how is it managed from diagnosis to treatment?

Cerebral Palsy: From Diagnosis to Management

Cerebral palsy (CP) is a group of permanent disorders affecting movement and posture caused by non-progressive disturbances in the developing fetal or infant brain, requiring early diagnosis and prompt intervention to optimize neuroplasticity, prevent secondary complications, and enhance quality of life. 1, 2

Definition and Epidemiology

• CP is the most common physical disability in childhood, with a prevalence of 2.1 cases per 1000 live births in high-income countries 2
• The prevalence appears to be declining in Australia and Europe but remains higher in low to middle-income countries due to greater infectious disease burden and differences in prenatal and perinatal care 2
• CP encompasses multiple etiologies resulting in brain injury affecting movement, posture, and balance 3

Etiology and Risk Factors

• Genetic factors: Recent evidence suggests genetic components in approximately 31.1% of CP cases, with higher diagnostic yield in pediatric populations (34.8%) than adult populations (26.9%) 1
• Maternal conditions: Thyroid disease, preeclampsia, infections 2
• Pregnancy complications: Intrauterine growth restriction, multiple gestation 2
• Preconception risks: History of stillbirths, miscarriages, low socioeconomic status, assisted reproduction 2
• Birth complications: Contrary to common belief, birth asphyxia accounts for less than 10% of CP cases 1
• Prematurity and low birth weight are significant risk factors 4

Clinical Classification

• Spasticity (85%-91%): Most common motor type, characterized by increased muscle tone 1, 2
• Dyskinesia (4%-7%): Includes dystonia and athetosis 1, 2
• Ataxia (4%-6%): Characterized by problems with coordination and balance 1, 2
• Hypotonia (2%): Not classified in all countries 1, 2

Topographical Classification

• Unilateral (hemiplegia): Affects one side of the body (38%) 1, 2
• Bilateral diplegia: Lower limbs affected more than upper limbs (37%) 1, 2
• Quadriplegia: All four limbs and trunk affected (24%) 1, 2

Diagnosis

Early Diagnostic Tools

• Before 5 months' corrected age:

  • Term-age magnetic resonance imaging (86%-89% sensitivity) 1, 2
  • Prechtl Qualitative Assessment of General Movements (98% sensitivity) 1, 2
  • Hammersmith Infant Neurological Examination (90% sensitivity) 1, 2

• After 5 months' corrected age:

  • Magnetic resonance imaging (86%-89% sensitivity) 1
  • Hammersmith Infant Neurological Examination (90% sensitivity) 1
  • Developmental Assessment of Young Children (83% C index) 1

Neuroimaging Findings

• White matter injury (cystic periventricular leukomalacia or periventricular hemorrhagic infarctions) (56%) 2
• Cortical and deep gray matter lesions (18%) 2
• Brain maldevelopments (9%) 2

Common Comorbidities

• Chronic pain (75%) 1, 2
• Epilepsy (35%) 1, 2
• Intellectual disability (49%) 1, 2
• Musculoskeletal problems (e.g., hip displacement) (28%) 1, 2
• Behavioral disorders (26%) 1, 2
• Sleep disorders (23%) 1, 2
• Visual impairment (11%) 1, 2
• Hearing impairment (4%) 1, 2

Management Approach

Early Intervention

• Early diagnosis and prompt referral to CP-specific intervention is essential to optimize infant motor and cognitive plasticity, prevent secondary complications, and enhance caregiver well-being 1, 2
• Intervention should begin as soon as CP is suspected rather than waiting for definitive diagnosis 2
• Early intervention maximizes neuroplasticity and minimizes deleterious modifications to muscle and bone growth 1

Motor Interventions

• For unilateral CP (hemiplegia): Constraint-induced movement therapy (CIMT) or bimanual therapy 1, 2
• For bilateral CP (diplegia): Learning Games Curriculum 1
• For all subtypes: Goals-Activity-Motor Enrichment (GAME) 1
• Physical and occupational therapy using child-initiated movement, task-specific practice, and environmental adaptations 1

Spasticity Management

• Intramuscular onabotulinumtoxinA injections 5, 3
• Oral muscle relaxants 5, 3
• Intrathecal baclofen 5, 3
• Selective dorsal rhizotomy 5, 3
• Regular surveillance and intervention for children with bilateral CP to reduce rates of hip displacement, contracture, and scoliosis 1

Communication Interventions

• Speech language pathology interventions to foster parent-infant transactions 1
• Augmentative communication when speech is not possible or inadequate 1

Prognosis

• In high-income countries:
  • 2 in 3 individuals with CP will walk 1, 2
  • 3 in 4 will talk 1, 2
  • 1 in 2 will have normal intelligence 1, 2

Emerging Treatments

• Regenerative agents to induce brain repair are being studied:
  • Erythropoietin plus hypothermia 1
  • Umbilical cord blood plus hypothermia 1
• Exome sequencing is recommended for diagnostic evaluation of individuals with CP, similar to other neurodevelopmental disorders 1

Prevention of Secondary Complications

• Early intervention helps prevent secondary impairments that worsen function or interfere with learning 1
• Successful and early prevention of contracture may reduce the need for later corrective surgery 5
• Support for parents/caregivers to reduce stress, anxiety, and depression 1