Cerebral palsy

Cerebral palsy (CP) is a non progressive central motor deficit of posture and movement due to defect in the developing brain, often associated with mental retardation, special sense organ defects and epilepsy. The condition was first described by Little as 'Little diplegia' in premature babies. It may be a sequela of perinatal asphyxia, perinatal infection, metabolic disorders, neonatal jaundice, neonatal seizures or developmental malformation of the brain. The incidence of CP, 2 in 1000 live births as noticed in olden days is remaining same even now with the best possible obstetric and neonatal care. Hence, the role of developmental malformation or vulnerability to asphyxia is postulated as an important cause of CP rather than iatrogenic causes. Around 10% of asphyxiated babies develop CP and 10% of CPs gives a definite history of asphyxia. In those with a low APGAR score persisting at 10 minutes, the incidence of CP is as much as 60%.

Classification of CP

Cerbral palsy can be grouped based on various classifications. The popular ones are added below.

Physiologic

  • Spastic
  • Hypotonic/atonic
  • Dystonic
  • Choreoathetoid
  • Ataxic
  • Mixed

Topographic

  • Monoplegia
  • Diplegia
  • Hemiplegia
  • Triplegia
  • Quadriplegia
  • Double hemiplegia

Etiologic

  • Prenatal
  • Natal
  • Postnatal
  • Perinatal

Functional

  • Class 1- no disability
  • Class 2- mild limitation
  • Class 3- significant limitation
  • Class 4- NO useful activity

Spastic

  • Spastic monoplegia is usually seen in preterm babies with mild disability and is usually not associated with seizure/ mental reatrdation/ special sense organ defects. Whereas spastic diplegia is the usual type in preterm babies with lower limb affection more than upperlimb and is usually not associated with other disabilities. In preterm babies, periventricular lower limb fibres are affected more during hypoxia as deeper vessels are less in number and superficial cortical vessels more, before maturity. Whereas in term babies, the insult occurs in the superficial cortex due to the presence of end arteries superficially, when the circle of willis becomes established.
  • Spastic hemiplegia is due to an insult occuring in one hemispherical cortex and the upper limb is more affected than the lower limb. Due to cortical involvment, there may be seizures and MR (mental reatrdation). There may be shortening of the affected side and associated focal or generalised seizures. The affected cerebral hemisphere ie., the hemisphere opposite to the disability will show atrophy or porencephalic cyst.
  • Spastic triplegia is similar, with 3 limbs affected and spastic quadriplegia usually occurs in full term babies with asphyxia. It is usually associated with seizure and mental reatrdation. In full term babies, deeper penetrating vessels are more compared to the superficial cortical end arteries without mutual communication.

Others

In dystonia/choreoathtoid CP, there is cogwheel rigidity and extrapyramidal signs. There may be an associated deafness. It is usually associated with bilirubin encephalopathy/kernicterus leading to status marmoratus and gliosis of the basal ganglia.

Atonic/hypotonic CP usually evolves into spasticity and there is more severe mental retardation.

In double hemiplegia, both upper limbs are more affected than the lower limbs. In mixed type, there are spasticity and choroathetoid movements.

In CP, there are no progressive deterioration of functions, but, there will be slow attainment of milestones. Antigravity muscles are weak leading to postural; abnormalty, foot drop, tendo achilles contracture etc.

Investigations

CNS imaging, EEG, vision and hearing evaluation tests are mandatory. In other selected cases the following tests may be useful too:
  • Thyroid function test
  • TORCH screen
  • Karyotyping

Differential diagnosis

Cerebral palsy being a slowly progressive degenerative disorder, may manifest in infancy and childhood and the rest in adulthood.

Treatment

This includes both multidisciplinary team approach with physiotherapy and occupational therapy, seizure control, special schooling, appliances etc are also beneficial. Tendon lengthening operation, rhizotomy for reducing severe hypotonia in spastic diplegia and botox (botulinum toxin) injections into the affected limb have also been tried. Benzodiazepenes or baclofen for spasticity, carbamazepine for dystonia and levodopa for athetosis are also beneficial.

The 'Spastic society' is a support group comprising of patients, their parents and wellwishers. Community based rehabilitation and home based rehabilitation are breakthrough approaches in this field.



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