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Neuropsychological Implications Of Ucd

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The Neuropsychology of Urea Cycle Disorders M. Cullen Gibbs, Ph.D. Pediatric Neuropsychologist Children’s National Medical Center
Neuropsychological Implications of UCD ,[object Object],[object Object],[object Object]
 
The Importance of White Matter ,[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object]
Developmental status Chronological age Vector of normal development Vector of disease effect Hypothetical Effects of Disease and Normal Development on Growth Trajectory Courtesy of Dr. Elsa Shapiro; University of Minnesota Medial School. 0 2 4 6 8 10 3 4 5 6 7 8 9 10 Resultant of vectors of development and disease in affected child
Problems in the Assessing the Cognitive Impact of Disease Processes in Childhood ,[object Object],[object Object],[object Object]
Problems Assessing the Cognitive Impact of Disease Processes in Childhood ,[object Object],[object Object]
What is Neuropsychology? ,[object Object],[object Object]
What is a Neuropsychological Evaluation? ,[object Object],[object Object],[object Object]
What is the Purpose of a Neuropsychological Evaluation? ,[object Object],[object Object],[object Object]
What is the Purpose of a Neuropsychological Evaluation? ,[object Object],[object Object],[object Object]
What is the Purpose of a Neuropsychological Evaluation? ,[object Object],[object Object],[object Object],[object Object]
Left Right Anterior Posterior
Anterior - Posterior Axis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Lateral Axis ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],<-->
Domains of Neuropsychological Functioning ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
What are the Potential Neuropsychological Implications of Damage to the White Matter? ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
What are the Executive Functions? ,[object Object],[object Object]
Role of the Executive ,[object Object],[object Object],[object Object],[object Object]
Executive Functions as the “Conductor of the Orchestra”
Functions of the “Conductor” 1. Initiate goal-oriented action 2. Working Memory 3. Plan & Organize 4. Self-monitor & evaluate 5. Inhibit 6. Flexibility 7. Emotional regulation 8. Self-awareness
Functional Domains of The Executive ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Functions of the “Conductor” 1. Initiate goal-oriented action 2. Working Memory 3. Plan & Organize 4. Self-monitor & evaluate 5. Inhibit 6. Flexibility 7. Emotional regulation 8. Self-awareness ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Critical features of Executive Control Functions (Denckla 1995) ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Demand Situations for Executive Functions ,[object Object],[object Object],[object Object],[object Object],[object Object]
Outcome of “Good” Executive Function ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
The Frontal lobes: Strategic executive Conductor Orchestra
Neuroanatomic Organization: ,[object Object],[object Object],[object Object],[object Object],[object Object],[object Object]
Interventions: General Principles ,[object Object],[object Object],[object Object],[object Object]
Interventions: General Principles ,[object Object],[object Object],[object Object],[object Object],[object Object]
Interventions: General Principles ,[object Object],[object Object],[object Object],[object Object],[object Object]
Structuring an executive function intervention program ,[object Object],[object Object],[object Object]
What Do We Hope to Learn? ,[object Object],[object Object],[object Object],[object Object]
What Do We Hope to Learn? ,[object Object],[object Object],[object Object],[object Object]

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Neuropsychological Implications Of Ucd

  • 1. The Neuropsychology of Urea Cycle Disorders M. Cullen Gibbs, Ph.D. Pediatric Neuropsychologist Children’s National Medical Center
  • 2.
  • 3.  
  • 4.
  • 5.
  • 6. Developmental status Chronological age Vector of normal development Vector of disease effect Hypothetical Effects of Disease and Normal Development on Growth Trajectory Courtesy of Dr. Elsa Shapiro; University of Minnesota Medial School. 0 2 4 6 8 10 3 4 5 6 7 8 9 10 Resultant of vectors of development and disease in affected child
  • 7.
  • 8.
  • 9.
  • 10.
  • 11.
  • 12.
  • 13.
  • 14. Left Right Anterior Posterior
  • 15.
  • 16.
  • 17.
  • 18.
  • 19.
  • 20.
  • 21. Executive Functions as the “Conductor of the Orchestra”
  • 22. Functions of the “Conductor” 1. Initiate goal-oriented action 2. Working Memory 3. Plan & Organize 4. Self-monitor & evaluate 5. Inhibit 6. Flexibility 7. Emotional regulation 8. Self-awareness
  • 23.
  • 24.
  • 25.
  • 26.
  • 27.
  • 28.
  • 29. The Frontal lobes: Strategic executive Conductor Orchestra
  • 30.
  • 31.
  • 32.
  • 33.
  • 34.
  • 35.
  • 36.