CEREBRAL PALSY – non progressive mucsular disorder

 

 

Cerebral palsy is a non progressive neuromuscular disorder. It occurs as the result of the immature brain damage. The insult to the immature brain may occur prenatally (during pregnancy), perinatally (during labour) or postnatally (within first few years of life). The incidence is about 1 in 400 children of school age. In western countries, the incidence is 1 in 500 live births. About 1% of premature babies or small for dates babies have been diagnosed with cerebral palsy.

 

Some non motor features associated with cerebral palsy:

  • microcephaly

  • learning difficulties – intelligence is often normal

  • speech disorders

  • epilepsy (fits occur in 25% of cases)

  • disorders of vision and/or hearing

  • squint

  • sensory loss

  • impaired growth

  • short attention span

  • hyperactivity

  • contractures and deformities

  • neurodevelopmental delay

 

Aetiology of cerebral palsy can be divided by 3-prenatal, perinatal and postnatal

Prenatal factors:

 

Predisposing factors Specific causes
  • Low birth weight for gestational age ( dyskinetic, spastic quadriplegia)
  • Sex: male > female
  • Race: white > black
  • Multiple birth, twin death, prematurity
  • Complications: bleeding, infection, preeclampsia
  • Genetic predisposition (e.g.: Factor V Leiden)
  • Genetic (in diplegia and ataxic cerebral palsy)
  • Structural brain malformations (e.g.: microcephaly, agenesis corpus collosum, Sturge-Weber Syndrome)
  • Ischaemia (e.g.: porencephaly-often middle cerebral artery, ‘fetal stroke’, periventricular leukomalacia)
  • Teratogens (e.g.: alcohol – 8.3% of fetal alcohol syndrome have cerebral palsy)
  • Deficiencies (e.g.: iodine (in New Guinea) and magnesium)
  • Infections (e.g.: TORCHES infections – toxoplasmosis, rubella and cytomegalovirus)

 

Perinatal factors:

  • Prematurity. (Uncomplicated – diplegia; Complicated – severe, mixed)

  • Intraventricular and intracerebral haemorrhage. Periventricular leukomalacia.

  • Severe jaundice may lead to dyskinetic cerebral palsy however kernicterus is very rare now in term infants. However, hyperbilirubinaemia is a risk factor in premature infants.

  • Perinatal asphyxia (dystonic quadriparesis +/- deafness ; spastic quadriparesis +/- dystonia, +/- complications like epilepsy or learning difficulty.

Postnatal factors:

  • Infection like meningitis and encephalitis. Gastroenteritis with severe dehydration also may lead to this movement disorder.

  • Head injury – either accidental or non accidental injury

  • Hypoxic-ischaemic encephalopathy – near miss SIDS (sudden infants death syndrome), near drowning or postoperative (e.g.: cardiopulmonary bypass)

  • Status epilepticus – may be followed by hemiseizure/hemiplegia or epilepsy.

  • Cerebrovascular accident

 

Upon the diagnosis of cerebral palsy, advice from paediatric neurologist should be sought. The diagnosis is made after we exclude the metabolic disorders and cord tethering.

 

Classification:

 

Diagnosis with certainty is rarely made in infancy. Mainly the diagnosis is made at 2 years of life although there is some symptoms present like abnormality of the tone, reflexes and posture during developmental screening. If there is any suspicion of cerebral palsy, early referral to paediatrician is mandatory to optimize function.

As for the management, multidisciplinary approach is needed and ideally should be commence as early possible to optimized benefit to the patients when they get older. The team will involve physiotherapists, occupational therapists, speech therapists, social workers, experienced teachers, community paediatricians, primary healthcare team and last but not least parents or care takers. The goal is mainly to develop maximal independence of the child within limits of their handicap.

Surgical intervention is necessary to enhance function and posture of the patient. It may prevent disabling deformity that is very important to these patients. Generally orthopaedic interventions are resolved for those with spasctic cerebral palsy where the main aim is to control spinal deformity (by using bracing and instrumentation) and control the hip displacement (by using splintage, soft tissue and/or bony reconstruction). Other than that it is also useful to control the muscle balance or contractures. In this case, tendon lengthening and tendon transfers is needed. Appliances such as various orthoses, customized wheelchairs and communication aids is very helpful and contribute significantly.

The last thing (probably the most important) is counselling to the parents and care takers to help them in handling this difficult situation. Just like other chronic medical illness, the child and care takers need assistance in understanding the disability, setting the realistic goals and support the emotional aspect to help them deal and live with this disability.

 

Reference:

  1. The Great Ormond Street Colour Handbook of Paediatrics and child Health. Manson Publishing. 2007.

  2. Oxford Handbook of General Practice. Oxford University Press. 2005.

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