#1 cause of pediatric death & disability in the united States


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#1 cause of pediatric death & disability in the united States

    • #1 cause of pediatric death & disability in the united States
    • Boys : Girls = 2 : 1
    • Each Year, approximately...
      • 400,000 children go to Emergency Rooms for TBI
      • 29,000 are hospitalized
      • 3,000 die
    • Some common causes of pediatric TBI:
    • Non-accidental trauma (infants), Falls (toddlers)
    • Motor vehicle accidents & pedestrian accidents (school-age children) , Sports-related injuries (children & teens)


Most children with TBI have

  • Most children with TBI have

    • mild injury (81%)
    • Good long-term recovery
    • No lasting effects of their TBI
  • However, the remaining approx. 20%:







No “classic” outcome , but some more common effects are:

  • No “classic” outcome , but some more common effects are:

    • Slower processing speed
    • Difficulty learning new information
    • Difficulty with higher-level language/conversation
      • “reading between the lines”
      • Drawing inferences
    • Lower IQ






  • Children with greater attention problems before brain injury also had greater attention problems after a brain injury.



MYTH: Younger children have better outcomes from TBI, because the brain is more “plastic” or “resilient”

  • MYTH: Younger children have better outcomes from TBI, because the brain is more “plastic” or “resilient”

  • FACT: Early TBI interferes with critical phases of rapid brain development

  • But… negative outcomes may not be apparent until years later

    • when brain functions that required early development finally come ‘online’
    • Academic and social demands increase over time.




Children’s nervous system is still developing.

      • Children’s nervous system is still developing.
        • at the time of injury, is a given skill...
        • ...emerging, developing, established?
        • (will the child ‘grow into’ a new area of deficit?)
      • Plasticity: young brain ≠ better recovery
      • Acquired injury overlays ongoing brain development






Genetic disease, X-linked

    • Genetic disease, X-linked
    • Missing enzyme can’t break down “Very Long Chain Fatty Acids (VLCFA)”
    • VLCFA accumulation = breakdown in myelin sheath
    • Has a parietal-occipital pattern of demyelination
    • Severe (Cerebral) form = progressive, neurodegenerative, leads to death if untreated.




Behavioral changes

  • Behavioral changes

    • withdrawal, aggression, hyperactivity, learning problems
  • Sensory loss (vision, hearing)

  • Cognitive decline & dementia

  • Seizures

  • Motor decline

  • …ultimately, premature death







Bone marrow (blood stem cells) transplantation

    • Bone marrow (blood stem cells) transplantation
    • Is now an established treatment option for COCALD…
    • but is not successful for all patients
    • Must be offered before cerebral disease has advanced too far, or child will not benefit from the treatment.
    • How do doctors know which children will benefit from this treatment?




      • Children’s nervous system is still developing.
        • How does a progressive disease interact with brain development?






Does not involve ‘seeing’ letters/words backwards, up-side down, transposed, etc.; i.e., is not a primary visual processing disorder.

  • Does not involve ‘seeing’ letters/words backwards, up-side down, transposed, etc.; i.e., is not a primary visual processing disorder.

  • Is properly defined as, “an unexpected difficulty in reading”.

  • Involves core deficits in:

    • Phonemic processing
    • Sound recognition
    • Mapping sounds to the written code of letters and letter groups.
  • And also impacts

    • Reading fluency and reading rate
    • Reading comprehension


Dyslexia occurs in between 5-15% of the population.

  • Dyslexia occurs in between 5-15% of the population.

  • Is familial and heritable.

    • Up to 50% of parents (of dyslexic child) may also have dyslexia
    • Up to about 40% of siblings also have dyslexia
    • When looking at parents with dyslexia, up to 65% of children
    • also affected
    • Boys & girls are equally affected
    • Teachers over estimate
    • reading problems in boys, and
    • under estimate them in girls.
















Plasticity: sometimes, plasticity is beneficial, but we might have to help it along

      • Plasticity: sometimes, plasticity is beneficial, but we might have to help it along
      • Developmental (and neurobiological) basis for Specific Learning Disability in Reading (dyslexia)






  • Thank you

  • Please don’t hesitate to contact me with any questions:

  • heather_adams@urmc.rochester.edu



  • Extra slides





Pre-morbid (pre-injury) function

    • Pre-morbid (pre-injury) function
      • IQ (“cognitive reserve”)
      • Emotional / behavioral well being
      • Learning disability
      • ADHD or other neurodevelopmental problem
    • Age & Sex
    • Severity of injury
    • Psychosocial resources
      • Family function
      • Educational level (parent)
      • Economic resources




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