Cerebral palsy

Cerebral palsy (CP) is a heterogenous group of disorders affecting the muscle tone and the development of movement and posture. CP results from a non-progressive damage to the brain in utero or during infantile development up to the age of 3 years. Depending on the affected brain area, spastic, ataxic, or dyskinetic cerebral palsy develops. While, in many cases, there is no identifiable cause, risk factors for cerebral palsy are prematurity, perinatal complications such as chorioamnionitis, birth trauma with intracerebral hemorrhage, or postnatal infections such as meningitis. The diagnosis of CP is usually not made until features become apparent as children fail to meet certain milestones during the postneonatal period (e.g., inability to roll over or sit independently). Physical indicators of spastic cerebral palsy include spastic paresis of multiple limbs and joint contractures, scissors gait, and persistence of primitive reflexes. Patients with non-spastic cerebral palsy present with dysarthria and abnormal involuntary movements (choreoathetoid, dystonic, or ataxic) that worsen with stress. Seizure disorders and intellectual disability are associated with all types. Diagnosis is mainly based on the clinical picture but cranial ultrasound or MRI can help identify the causative lesion (e.g., hemorrhage, brain malformation). Since there is no cure, management follows a multidisciplinary approach with a focus on treating contractures (e.g., bracing, antispasmodics, physical therapy, surgery) and ensuring social participation (e.g., speech therapy, social support). Depending on the severity and type of cerebral palsy, patients may be slightly restricted or severely disabled and unable to walk.

• Most common motor disability in children
• Approx. 2/1000 live births in developed countries

References:^[1][2]

Epidemiological data refers to the US, unless otherwise specified.

• Idiopathic (most cases)
• Risk factors:
  • Preterm birth and low birth weight (most important risk factors)
  • TORCH infection
  • Perinatal asphyxia
  • Intracranial hemorrhage
  • Structural abnormality of the brain
  • Neonatal seizures
  • Kernicterus
  • Postnatal infection (e.g., meningitis, encephalitis)

References:^[2][3][4]

• Spastic cerebral palsy: spastic paresis of one or more limbs (75% of cases)
• Non-spastic cerebral palsy :
  • Dyskinetic: abnormal involuntary movements (choreoathetoid, dystonic)
  • Ataxic: intention tremor, lack of balance and coordination

References:^[2][3][4]

Patients may present with mixed types of cerebral palsy (e.g., combination of spastic and athetoid CP).

• All types
  • Patients do not reach certain milestones
  • Intellectual disability (50%)
  • Seizure disorder (35%–50%)
  • Joint contractures
  • Attention deficit hyperactivity disorder
• Spastic type:
  • ↑ Muscle tone in one or more limbs
  • ↑ DTRs
  • Persistence of primitive reflexes (e.g., positive Babinski sign)
  • Toe walking or equinus deformity
  • Muscle weakness and/or atrophy
  • Scissor gait (as a result of spastic paraplegia of the hip adductors)
  • Hip dislocation
  • Scoliosis
  • Hearing or vision impairment
• Non-spastic type:
  • Abnormal involuntary movements that worsen with stress and disappear with sleep
    • Chorea
    • Athetosis
    • Dystonia
    • Ataxia
  • Dysarthria and dysphagia

Definite hand preference before 1 year of age, suggests a one-sided muscle weakness and is a red flag for hemiplegia!

References:^[2][3][4][5]

Diagnosis is mainly based on the clinical picture.

• Cranial ultrasonography (early neonatal period): e.g., intracerebral hemorrhage and/or hypoxic-ischemic injury, structural abnormalities
• MRI (in older infants): to detect causative lesion (e.g., periventricular leukomalacia, congenital malformation, intracranial hemorrhage)

References:^[2][3]

• Hypothyroid myopathy
• Mitochondrial myopathy
• Progressive muscular dystrophies
• Myelomeningocele
• Traumatic peripheral nerve lesions
• Genetic syndromes

The differential diagnoses listed here are not exhaustive.

There is no curative therapy for cerebral palsy. Management is dependent on the severity and nature of symptoms. A multidisciplinary approach is employed in management to improve function and quality of life.

• Nonpharmacological management
  • Physical therapy: prevent muscle contractures
  • Occupational therapy: motor skill development
  • Speech therapy: communication skills and dysphagia
  • Orthotic devices (e.g., braces, splints, and casts) and assistive devices (e.g., wheelchairs, walkers)
  • Educational support for intellectual disability
  • Nutritional support for dysphagia
  • Social and psychological support
• Pharmacological management
  • Antispasmodics; (e.g., botulinum toxin, baclofen, dantrolene, benzodiazepines)
  • Anticonvulsants
  • Anticholinergics for rigidity and sialorrhea
• Surgery: orthopedic surgery to treat scoliosis, relieve contractures, and neurosurgical management of spasms