Saturday, September 17, 2016

Cerebral Palsy



An 18 month old child attends the child development center for a thorough assessment with suspected diagnosis of cerebral palsy. Which of the following clinical features would be most likely to suggest an alternative diagnosis?
Select ONE answer Only

A. Dystonia
B. Family history of cerebral palsy
C. Hyperkinesia
D. Prematurity
E. Spasticity

Correct Answer is option B:  Family history of cerebral palsy

Cerebral Palsy:

Cerebral Palsy is a descriptive term which has been defined as a group of permanent disorders of movement and posture causing activity limitation that are attributed to non progressive disturbances that occurred in the developing fetal or infant brain.


Children with cerebral palsy are not always hypertonic and four different clinical types have been described:

  • Spasticity
  • Dystonia
  • Ataxia
  • Choreo-athetoid.
Children with cerebral palsy often have a mixed picture of movement. 

Conditions That Can  Mimic Cerebral Palsy: Cerebral palsy can be mimicked by several other conditions and before making a firm diagnosis the other differentials must be considered:
  • Spinal cord tumors
  • Ataxia telengectasia
  • Congenital dopa responsive disorders
  • GLUT1 deficiency
  • Glutaric aciduria type 1. 
Management Of Cerebral Palsy: Meeting the child's and the family's complex needs require a multi-disciplinary team. 

Specific Drug Management Of Spasticity:

1. Diazepam: is a GABA agonist. ( GABA is the principal neuroinhibitory neurotransmittor regulating neuronal excitability in the nervous system). Diazepam relieves muscle spasms and spasticity as well as helps in sedation and acts as an anxiolytic. Long term use can leasd to dependency but short term use is helpful for painful spasms and to break cycles of distress and spasticity.

2. Baclofen: is also a GABA agonist and inhibits neuronal transmission at a spinal level. Its main adverse effect is sedation, which often limits its use. When given orally large doses are needed as its passage to CSF is poor. Efficacy may be increased by giving smaller intrathecal doses via a surgically implanted continuous delivery pump.

3. Tizanidine: is an alpha-2-adrenergic receptor agonist and acts at the spinal level to help relieve spasms. It is particularly useful in children who are severely disabled by cerebral palsy and in those with night time spasms, as it commonly has a sedative effect.

4. Botulinum toxin A: it works by chemically denervating the muscle, allowing the muscle to relax and thus improving function.

5. Dantrolene: works on spasticity and spasms by affecting the calcium uptake into skeletal muscle and decreasing the free intracellular calcium concentration. .Sedation is less of a problem with dantrolene than with the other drugs mentioned, but it should be used with caution as it cause liver dysfunction and blood dyscrasias.






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