Cerebral Palsy Causes and Management
Definition and Epidemiology
- Cerebral palsy (CP) is defined as "a group of permanent disorders of the development of movement and posture, causing activity limitation, that are attributed to non-progressive disturbances that occurred in the developing fetal or infant brain" 1, 2
- It is the most common physical disability in childhood, with a prevalence of 2.1 cases per 1000 in high-income countries 1
- Prevalence is declining in Australia and Europe but appears higher in low to middle-income countries due to greater infectious disease burden and differences in prenatal and perinatal care 1
Causes and Risk Factors
- Genetic factors: Recent evidence suggests 14% of cases have a genetic component 1, 2
- Maternal conditions: Thyroid disease, preeclampsia, infections 3
- Pregnancy complications: Intrauterine growth restriction, multiple gestation 3
- Preconception risks: History of stillbirths, miscarriages, low socioeconomic status, assisted reproduction, and abnormal genetic copy number variations 3
- Infections, trauma, stroke, and hypoxic events occurring in early infancy 3
Neuroimaging Findings
- The most predictive MRI patterns for CP include white matter injury (cystic periventricular leukomalacia or periventricular hemorrhagic infarctions) (56%) 3
- Cortical and deep gray matter lesions (basal ganglia or thalamus lesions, watershed injury, multicystic encephalomalacia, or stroke) (18%) 3
- Brain maldevelopments (lissencephaly, pachygyria, cortical dysplasia, polymicrogyria, or schizencephaly) (9%) 3
Clinical Presentation and Classification
- Spasticity (85%-91%) 1
- Dyskinesia (4%-7%), including dystonia and athetosis 1
- Ataxia (4%-6%) 1
- Hypotonia (2%) 1
- Unilateral (hemiplegia) (38%) 1
- Bilateral, including diplegia (lower limbs affected more than upper limbs) (37%) 1
- Quadriplegia (all 4 limbs and trunk affected) (24%) 1
Common Comorbidities
- Chronic pain (75%) 1
- Epilepsy (35%) 1
- Intellectual disability (49%) 1
- Musculoskeletal problems (e.g., hip displacement) (28%) 1
- Behavioral disorders (26%) 1
- Sleep disorders (23%) 1
- Visual impairment (11%) 1
- Hearing impairment (4%) 1
Early Diagnosis
- Term-age MRI (86%-89% sensitivity) 2, 4
- Prechtl Qualitative Assessment of General Movements (98% sensitivity) 2, 4
- Hammersmith Infant Neurological Examination (90% sensitivity) 2, 4
Management Approach
- Early diagnosis and prompt referral to diagnostic-specific intervention is essential to optimize infant motor and cognitive plasticity, prevent secondary complications, and enhance caregiver well-being 2, 5
- Early intervention should begin as soon as CP is suspected rather than waiting for definitive diagnosis 4
- Loss of motor milestones (suggestive of neurodegenerative process) 6
- Respiratory insufficiency with generalized weakness 6
- Abnormalities on brain MRI 6
- Motor delays present during minor acute illness 6
Prognosis
- 2 in 3 individuals with CP will walk 2, 5
- 3 in 4 will talk 2, 5
- 1 in 2 will have normal intelligence 2, 5
Importance of Neurologist Referral for Patients with Cerebral Palsy, Seizures, and Microcephaly
Benefits of Early Neurologist Referral
- Early, accurate diagnosis and early intervention in cerebral palsy improves outcomes through specialized neurological expertise that can maximize neuroplasticity and minimize deleterious modifications to muscle and bone growth and development, as recommended by the American Academy of Pediatrics 7
- Early referral to a neurologist allows for prompt diagnosis and intervention, which helps foster acceptance and leads to increased confidence in the medical team, and implementation of cerebral palsy-specific interventions that can maximize neuroplasticity, as supported by evidence from JAMA Pediatrics 7
- Delaying diagnosis and appropriate neurological management can be harmful to parent and caregiver well-being, potentially leading to depression and lasting anger, highlighting the importance of timely referral to a neurologist 7
- Early detection and referral allows improved access to early intervention and efficient use of resources, which is crucial for optimal management of cerebral palsy, seizures, and microcephaly 7
Life Expectancy in Cerebral Palsy
Key Determinants of Life Expectancy
- Respiratory diseases and aspiration pneumonia are the leading causes of death, particularly in individuals with swallowing dysfunction, according to the American Academy of Pediatrics 8
Common Causes of Early Death
- Aspiration pneumonia is a major contributor to mortality risk, especially in those with feeding difficulties, as reported by the JAMA Pediatrics guideline 8
Impact of Modern Management on Survival
- Early intervention programs that optimize neuroplasticity and prevent secondary complications can improve life expectancy, as recommended by the American Academy of Pediatrics 9
- Better prevention and treatment of respiratory complications through early swallowing assessments and appropriate tube feeding when indicated can also improve survival, according to the JAMA Pediatrics guideline 8