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Cerebral Palsy

  • Introduction

    Cerebral palsy is a persistent but not unchanging disorder of movement and posture due to a defect or lesion of the developing brain. It is accepted that children up to five years, who acquire permanent motor impairment due to non-progressive neurological insults, have cerebral palsy. There are many causes, a wide range of manifestations of the motor disorder and various associated problems.

    *Cerebral palsy is not a single disorder but a group of disorders with diverse implications for children and their families.

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    Presentation

    • Follow up of "at risk" infants, such as those born prematurely
    • Delayed motor milestones, particularly learning to sit, stand and walk
    • Asymmetric movement patterns, for example, strong hand preference early in life
    • Abnormalities of muscle tone particularly spasticity or hypotonia
    • Management problems, for example, severe feeding difficulties and unexplained irritability. Many other conditions present with these features.

    *Observation of the child often provides more information than 'hands on' examination. Look for the presence or absence of age appropriate motor skills and their quality.

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    Associated disorders

    • Visual problems (approx 40%) eg strabismus, refractive errors, visual field defects and cortical visual impairment
    • Hearing deficits (approx 3 - 10%)
    • Speech and language problems
    • Epilepsy (approx 50%)
    • Cognitive impairments. Intellectual disability, learning problems and perceptual difficulties are common. There is a wide range of intellectual ability and children with severe physical disabilities may have normal intelligence

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    Management

    Management involves a team approach with health professionals and teachers. Input from the family is important.

    1. Accurate diagnosis and genetic counselling.

    Establish the cause of cerebral palsy if possible.

    History of pregnancy, birth and neonatal period, along with physical examination.
    • Cause clear,
      for example, massive antepartum haemorrhage followed by neonatal encephalopathy:
    No further investigation
    • Cause not clear:
    Urine / plasma metabolic screen
    Consider congenital infections
    Chromosomal analysis
    Radiological investigation - MRI
          ? vascular lesion
          ? malformation
          ? periventricular leucomalacia

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    2. Management of the associated disabilities, health problems and consequences of the motor disorder

    Associated disabilities

    • All children require hearing and visual assessments
    • Assess and review anticonvulsants for epilepsy
    • Formal cognitive assessment is beneficial. Children often need help with their educational program

    Health problems

    • Monitor growth and provide dietary advice. Failure to thrive is frequent. Consider nasogastric or gastrostomy feeds if there is difficulty in achieving satisfactory weight gains, or there are major feeding problems. Obesity interferes with progress in motor skills
    • Gastro-oesophageal reflux can result in oesophagitis or gastritis, causing pain, poor appetite and aspiration.
    • Constipation
    • Some children with severe cerebral palsy develop chronic lung disease, due to aspiration from oromotor dysfunction or severe gastro-oesophageal reflux. Coughing or choking during meal times, or wheeze during or after meals, may signal aspiration. It can also occur silently. Barium videofluroscopy may be helpful.
    • Monitor ventriculo-peritoneal shunts
    • Osteoporosis and pathological fractures occur in severe cerebral palsy
    • Monitor dental health
    • Emotional problems can be responsible for suboptimal performance either with academic tasks or self care.

    Consequences of the motor disorder

    • Drooling (poor saliva control). Speech pathologists assist with behavioural approaches. Medication (anticholinergics) and surgery may be helpful

    • Incontinence. Children may be late in achieving bowel and bladder control because of cognitive deficits or lack of opportunity to access toileting facilities because of physical disability or inability to communicate. Some children have detrusor overactivity causing urgency, frequency and incontinence.

    • Orthopaedic problems. Contractures may develop and require orthopaedic intervention. Surgery is more commonly undertaken on the lower limb.
      • The hip. Non-walkers and those partially ambulant are at risk for hip subluxation and dislocation. Perform hip Xrays and refer children to an orthopaedic surgeon according to  consensus guidelines Ambulant children occasionally develop hip problems.
      • The knee. Flexion contractures at the knee may require hamstring surgery.
      • The ankle. Equinus deformity is the commonest orthopaedic problem in children with cerebral palsy. Toe walking is treated conservatively in young children with orthoses, inhibitory casts, and Botulinum toxin A therapy. Older children benefit from surgery.
      • Children may require multi-level surgery (for example, hip, knee and ankle), usually between 8 and 12 years. The aims of surgery are to correct deformities and to improve both the appearance and efficiency of walking.
      • A number of procedures are available for the upper limb.
      • Correction of scoliosis is sometimes necessary.

    • Spasticity management aims to improve function, comfort and care and requires a team approach. Options include:
      • Oral medications, for example, diazepam, dantrolene sodium and baclofen.
      • Inhibitory casts, for example, below knee casts increase joint range and facilitate improved quality of movement.
      • Botulinum toxin A reduces localised spasticity.
      • Intrathecal baclofen is suitable for a small number of children with severe spasticity and may enhance quality of life.
      • Selective dorsal rhizotomy is a neurosurgical procedure whereby anterior spinal roots are sectioned to reduce spasticity.

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    3. Assessment of the child's capabilities and referral to appropriate services for the child and family

    • The role of the team
      Assessment by a multidisciplinary team is essential.
      • Physiotherapists give practical advice on positioning and handling to minimise the effects of abnormal muscle tone and encourage the development of movement skills. They also advise about the use of orthoses, wheelchairs and other mobility aids.
      • Occupational therapists help parents to develop their child's upper limb and self-care skills, and also recommend suitable toys, equipment and home adaptations.
      • Speech pathologists assist in the development of communication skills and advise about augmentative communication systems. They provide guidance with feeding difficulties and saliva control problems.
      • Othotists, medical social workers, psychologists, special education teachers and nurses are helpful.

    • Trends in service provision
      Services are best provided within local communities. Therapists and special education teachers work with children at home and later in child care centres, kindergartens and schools. Most children attend regular preschools and schools but others benefit from attendance at centre-based early intervention programs and special schools. It is essential that parents are made aware of all available options.

    • Working with families
      Care of the child with cerebral palsy involves developing a trusting and cooperative relationship with parents. As with all children, a supportive home environment builds self-esteem and confidence. Parents may need practical support such as provision of respite care and information about financial allowances, and are helped by meeting other families in similar circumstances.

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    4. Common presentations to the Emergency Department

    • Respiratory problems particularly pneumonia
    • Uncontrolled seizures / status epilepticus
    • Unexplained irritability - consider acute infections, oesophagitis, dental disease, hip subluxation, pathological fracture. Review medications.
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