Neuropsychological Implications Of Ucd

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]

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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],[object Object]

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]

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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]

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]

Neuropsychological Implications Of Ucd

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