Cerebral Palsy (CP)

Background

• Cerebral palsy describes a complex group of persistent nonprogressive disorders of central origin involving movement or posture and motor function including spasticity, dyskinesia, hypotonia, and ataxia that occur due to damaged brain tissue from various perinatal or neonatal causes.
• Motor impairments are frequently accompanied by multiple comorbid conditions including vision and hearing deficits, cognitive disabilities, and seizure disorders.
• The prevalence of cerebral palsy has been stable at about 2 cases per 1,000 newborns for more than 30 years.
• Risk factors for cerebral palsy include:
  • prenatal exposure to toxins or inflammation
  • placental disorders
  • fetal growth restriction
  • birth defects and congenital malformations
  • genetic disorders
  • premature or postdates delivery and low birth weight/small for gestational age
  • neonatal complications such as intraventricular hemorrhage, kernicterus, encephalopathy, necrotizing enterocolitis, seizures, hypoglycemia, or infection
  • perinatal complications such as acute intrapartum hypoxic event, perinatal asphyxia, and chorioamnionitis
• While neurologic deficits associated with cerebral palsy are nonprogressive, the prognosis varies depending on the severity, the effect on functional level attained, and associated health conditions.

Evaluation

• Cerebral palsy is diagnosed clinically based on history and physical exam findings.
  • The first clinical signs of cerebral palsy are usually delays in motor development and may present any time from 1 week to 36 months, with a mean age of 19 months.
  • Physical examination findings may include hyperreflexia, persistent primitive reflexes (Babinski, startle, atonic neck reflex), tonal abnormalities (hypo/hypertonicity).
  • The diagnosis can be made in infants and children with persistent signs of nonprogressive disorders of movement such as spasticity, dyskinesia, hypotonia, or ataxia.
  • Neuroimaging (magnetic resonance imaging [MRI] is preferred) may establish an etiology but it is not required for diagnosis.
  • Mild cerebral palsy is sometimes difficult to distinguish from other neurodevelopmental problems and may present as late as age 5 years.
• Functional assessment is the most important part of an evaluation for children with cerebral palsy.
  • The Gross Motor Function Classification System (GMFCS) and Pediatric Evaluation of Disability Inventory (PEDI) are often used at diagnosis and throughout treatment to assess overall function, ambulatory capabilities, and the level of independence.
  • Other functional scales may be used to evaluate specific impairments or outcomes.
• Perform neuroimaging as part of the evaluation of a child with cerebral palsy if the underlying cause has not been established (Strong recommendation).
  • Cranial ultrasound may be used in the special care nursery setting.
  • MRI is preferred to computed tomography (Strong recommendation).
• Evaluation of infants and children with newly diagnosed cerebral palsy:
  • Assess for growth and nutritional status and signs of pain.
  • Assess for signs of pain.
  • Obtain initial hip and pelvic x-rays, which may be repeated over time depending on the severity of involvement.
  • Consider testing for a coagulation disorder in children with hemiplegic cerebral palsy (Weak recommendation).
  • Test for developmental delay/intellectual disability, ophthalmologic and hearing impairments, speech and language disorders, and learning problems (Strong recommendation).
  • Consider not obtaining routine metabolic and genetic studies in the evaluation of children with cerebral palsy (Weak recommendation).

Management

• The overall goals of management and treatment are to improve function, participation, and mobility while reducing secondary complications and pain.
• Conventional therapies such as physical, occupational, and speech and language therapy are appropriate for a majority of patients with cerebral palsy.
• Specific treatments to improve gross motor function, mobility, spasticity, and involuntary movements may improve functional outcomes.
  • Surgical treatments may include multilevel surgery (including tendon or muscle lengthening, tendon transfers, tendon release), surgery for positional deformities or joint abnormalities, and selective dorsal rhizotomy.
  • Other orthopedic interventions may include casting, bracing, and orthotics.
  • Medications such as botulinum toxin A, baclofen, and diazepam may be considered for certain associated features of cerebral palsy.
• Complications such as chronic pain, oromotor impairment/feeding difficulties (leading to drooling, aspiration), reflux, depression, seizures, scoliosis, malnutrition/poor growth, and bowel and bladder dysfunction contribute significantly to an overall reduction in health and functioning and are generally managed as they would be in patients without cerebral palsy.