Understanding and promoting executive functions in children
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Understanding and Promoting Executive Functions in Children. Stephen R. Hooper, Ph.D. Department of Allied Health Sciences University of North Carolina School of Medicine. Learning Objectives. Describe the various definitions of Executive Functions (EF).

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Understanding and promoting executive functions in children

Understanding and Promoting Executive Functions in Children

Stephen R. Hooper, Ph.D.

Department of Allied Health Sciences

University of North Carolina School of Medicine


Learning objectives

Learning Objectives

  • Describe the various definitions of Executive Functions (EF).

  • Describe the neurological underpinnings and associated models

  • Examples of EF assessment strategies

  • Identify the manifestation of EF deficits in selected pediatric disorders.

  • Overview specific strategies for managing executive dysfunction.


Case example

Case Example


Case example1

Case Example

Joshua was referred for a neuropsychological evaluation due to a reported decline in intellectual functioning, and to assist in identifying neurocognitive contributions to his ongoing learning and social-emotional difficulties.


Case example2

Case Example

Joshua is an 8 year, 8 month old male whose history is significant for aggression and exposure to family conflict and abuse.

At the age of 3 months, Joshua was removed from his biological parents’ care because his parents were physically abusive toward each other and were abusing a variety of substances.

Joshua was adopted at 3 months of age by his maternal grandparents.


Case example3

Case Example

  • Joshua is placed in a self-contained classroom for emotional/behavioral challenges where he has received instruction since Kindergarten due to destructive and aggressive behaviors in preschool.

  • With respect to specific skills, Joshua is behind his peers in all core areas and he is not yet able to read or count fluently.

    • His gross motor coordination is reported to be poor and he has not yet learned to ride a bike.

  • Concerns have arisen regarding Joshua’s language abilities as he can be difficult to understand and he struggles with sound-symbol associations.


Case example4

Case Example

  • Emotional/behavioral difficulties are long standing.

  • His adoptive parents noted that he can be aggressive, which includes hitting, biting, and spitting at others, and that he is impulsive, inattentive, and lacks self-control with few self-soothing strategies.

  • He has sensitivity to food textures and has difficulty sleeping through the night.


Case example5

Case Example

  • Socially, Joshua is teased by peers, does not play well in groups, prefers to organize his own play, is unwilling to share, and prefers to play with younger children.

  • His adoptive parents noted that it is not uncommon for peers to encourage him to become angry and act out.


Case example6

Case Example

  • Joshua carries the diagnoses of:

    • Bipolar Disorder

    • Post-traumatic Stress Disorder

    • Asperger’s Disorder

    • Attention Deficit/Hyperactivity Disorder


Case example7

Case Example

Joshua’s medical history is significant for in utero alcohol and cocaine exposure for the entire duration of his prenatal life, with alcohol amounts reaching a bottle of whiskey a day according to the DSS report, subsequent premature birth, asthma, constipation, and significantly delayed developmental milestones.


Case example8

Case Example

  • IQ = 77

  • Achievement:

    • Reading = 64

    • Math = 65

    • Spelling = 63

  • Motor: Right sided dominance, slow fine-motor speed bilaterally, fine-motor coordination was poor

  • Sensory: No major problems with tactile perception, discrimination, or sensory suppressions

  • Language – borderline to low average range

  • Visual Processing – Visual discrimination intact, but borderline higher-order visual-spatial and visuoconstructive functions.


Case example9

Case Example

  • Problem Solving:

    • Disproportionately low, with associated poor planning and significant cognitive rigidity;

    • Characterized by concrete thinking

  • Set-Maintenance/Shifting:

    • Highly rigid and prone to frequent perseverations


Case example10

Case Example

  • Inhibitory Control

    • Highly impulsive and poorly regulated

  • Working Memory

    • Disproportionately low verbal and visual working memory

  • Processing Speed and Efficiency

    • Overly quick in his responding, consistent with his high degree of impulsivity

  • Attention Regulation

    • Selective and sustained attention were poor and highly variable


Definitions

Definitions


Definitions1

Definitions

  • Of all of the neurocognitive constructs, this one is probably the most complex.

    • Is it taught as part of an assessment class sequence?

  • The complexity comes not only in its overall regulatory functions, but also in how executive functions interact with nearly every other neurocognitive function.

  • Executive processes have been deemed critical to the integrity of many learning and social-behavioral functions.

    • Not typically assessed in a routine psychoeducational evaluation.

  • There are a number of definitions of executive functions, some extending back nearly 50 years.


Executive functions luria 1966

Executive Functions(Luria, 1966)

  • Executive function is defined as the ability to maintain an appropriate problem-solving set for attainment of a future goal. This involves:

    • an intention to inhibit a response or to defer it to a later, more appropriate time;

    • a strategic plan of action sequences and/or;

    • a mental representation of the task, including the relevant stimulus information encoded in memory and the desired future goal-state.


Executive functions welsh pennington 1988

Executive Functions(Welsh & Pennington, 1988)

  • Executive function is primarily the set maintenance required to achieve a future goal.

    • This set maintenance includes the requisite skills of planning, organization, inhibition of maladaptive responses, self-monitoring, and flexibility of strategies contingent on feedback.

    • Goldman-Rakic (1990) would add to this definition the concept of working memory.


Executive functions zelazo mueller 2002

Executive Functions(Zelazo & Mueller, 2002)

  • Executive function (EF) describes the control processes that affect overall output. This collection of abilities involves:

    • Regulatory control over thoughts and behaviors during goal-directed or intentional actions

    • Problem solving

    • Flexible shifting between actions to meet task demands.


Executive functions stuss alexander 2000

Executive Functions(Stuss & Alexander, 2000)

  • Noted that one important contribution of EF is the regulation of affect and social behavior.

  • This notion is supported by the finding that abstract reasoning has been linked to vulnerability to social anxiety during adolescence in typically developing children.


Models

Models


Models of executive functioning

Models of Executive Functioning

  • Executive functions are generally viewed within a multidimensional framework of cognitive abilities that provide critical support for goal-directed, future-oriented behaviors.

  • These behaviors typically include attention regulation, inhibitory control, working memory, set shifting/cognitive flexibility, planning, and cognitive efficiency.

  • The multiple dimensions of executive functions have been embedded within conceptual and empirical models that comprise two, three, four, and six factors.


Executive functioning welsh et al 1991

Executive Functioning(Welsh et al., 1991)

  • Speeded responding

    • Visual search - achieved at age 6

    • Verbal fluency - > age 12

    • Motor sequencing - > age 12

  • Set maintenance

    • Wisconsin Card Sort - achieved at age 10

    • MFFT - achieved at age 10

  • Planning

    • Tower of Hanoi (3 disk) - achieved at age 6

    • Tower of Hanoi (4 disk) - > age 12


Executive functions pennington 1997

Executive Functions(Pennington, 1997)

  • Implicated 4 empirical subcomponents of EF using Typicals and children with Reading Disabilities. Then applied to different disorders:

    • Inhibition (ADHD< FXS, ASD; ADHD improves on medication)

    • Set Shifting (ADHD>FXS>ASD)

    • Working Memory (ADHD>ASD>FXS)

    • Planning (ADHD>FXS>ASD

  • Both the level and pattern of EF abilities has potential to discriminate across disorders.


Executive functioning denckla 1993

Executive Functioning (Denckla, 1993)

  • Delay between stimulus and response

  • Internal representation of schema

  • Internal representation of action plan

  • Response inhibition

  • Efficiency and consistency of response

  • Active strategies and deployment

  • Flexible strategies and deployment


Executive functioning denckla 19931

Executive Functioning (Denckla, 1993)

  • Denckla utilized this conceptual framework to propose a 4-factor model of executive functions:

    • Initiating

    • Sustaining

    • Set-Shifting

    • Inhibiting

  • Has been applied to 4th and 5th grade students with Written Language Disorder (Hooper et al., 2002).

    • Poor Writers were less proficient at initiation, set-shifting, and sustaining than Good Writers.


Executive function summary

Executive Function Summary

  • There are notable differences between these models, but there does appear to be a number of specific executive functions that appear across the models.

    • Planning and problem solving, inhibitory control, set shifting, and set maintenance usually are included in some fashion.

    • Speeded responding and working memory also have been discussed as subcomponents of executive function.

    • Don’t forget affective regulation.


Executive function summary1

Executive Function Summary

  • Most of these functions are linked to the dorsolateral prefrontal cortex and associated brain regions

    • It is important to remember that the emotional control functions associated with the ventromedial prefrontal cortex also should be included in a larger executive function model.

  • This will be especially important for increasing our understanding of the childhood neurobehavioral difficulties.

    • Applications for both behavioral and affective regulatory difficulties in children.


Neurodevelopmental underpinnings

Neurodevelopmental Underpinnings


Neurodevelopmental underpinnings1

Neurodevelopmental Underpinnings

  • Lesion, neuroimaging, and brain function (e.g., EEG) studies suggest that the prefrontal cortex (PFC) is primarily involved in executive processing.

  • Based upon anatomical connectivity, there are two major regions of the PFC:

    • Dorsolateral PFC (DlPFC)

    • Ventromedial PFC (VmPFC).


Neurodevelopmental underpinnings2

Neurodevelopmental Underpinnings

  • The so called “cool” functions of dorsolateral prefrontal cortex represent the executive functions of motor planning and regulation, integration of sensory and mnemonic information, working memory, and attention regulation

  • The ventromedial regions of the orbitofrontal cortex are involved in planning and decision-making, but they also mediate affective regulatory functions, or the so called “hot” functions.


Neurodevelopmental underpinnings3

Neurodevelopmental Underpinnings

Dorsolateral Prefrontal Cortex (DlPFC)

  • Regions within DlPFC influence:

    • The selection of behaviors

    • Recognition of context-dependent changes between stimuli and behavior

    • Potentiation of sets of stimulus-response contingencies related to behaviors in context

    • Flexible, goal-driven control of behavior


Neurodevelopmental underpinnings4

Neurodevelopmental Underpinnings

  • Varying levels of damage to the DlPFC are associated with:

    • Lack of motivation, creativity, or goal-following.

    • Difficulty in initiating or flexibly modifying actions, resulting in stereotyped responses.

    • Loss of affect.

    • Inability to assess others’ mental states – Theory of Mind.

    • Perseveration and more random-choice errors than age-matched controls.

    • Increased distractibility and problems with sustained attention.

    • Impaired working memory.

    • Understanding of complex task rules.


Neurodevelopmental underpinnings5

Neurodevelopmental Underpinnings

Ventromedial Prefrontal Cortex (VmPFC)

  • The VmPFC is critical for elucidating the relation between stimuli and reinforcers, and for explaining the inability of individuals with vmPFC damage to learn reward contingencies.

  • Important for affective regulation.


Neurodevelopmental underpinnings6

Neurodevelopmental Underpinnings

  • Varying levels of damage to the vmPFC are associated with:

    • Impulsivity

    • Sensitivity to immediate rewards

    • Lack of self-control

    • Disruption of both affective and nonaffective stimuli

    • Tendency to select behaviors with the highest perceived reward, not the highest perceived utility


Understanding and promoting executive functions in children

Neurodevelopmental Underpinnings

Dorsolateral Prefrontal Cortex

Frontal Pole

Ventromedial Prefrontal Cortex


Neurodevelopmental underpinnings7

Neurodevelopmental Underpinnings

  • All of the various executive functions have a developmental basis that will have differential effects on learning and behavior over time.

  • There is a sequential unfolding of various executive functions over the course of development.

  • Contemporary findings from typical developmental studies, lesion studies, and primate studies suggest that the PFC is operational, at a basic level, by the end of infancy (i.e., ~24 months), with some evidence of even earlier emergence.


Neurodevelopmental underpinnings8

Neurodevelopmental Underpinnings

  • While executive functions appear to develop from infancy into early adulthood, it appears that the period of most rapid development occurs:

    • Between the ages of 6 and 8 years of age

    • More modest gains between the ages of 9 and 12

  • Differential development continues through adolescence into early adulthood depending on the specific task or behavior.

  • An injury during any of these periods of development will have an effect on specific functions along with the developmental trajectories of these functions.


Neurodevelopmental underpinnings9

Neurodevelopmental Underpinnings

  • Disruption to the neurological underpinnings of any of these brain regions of the prefrontal cortex could trigger specific executive dysfunctions in cognitive and/or behavioral-emotional functioning.

  • Given the developmental trajectories of many of these functions, it also is possible for an earlier injury to “lie silent” until they are developmentally required, and a specific executive dysfunction could emerge at a later developmental epoch (e.g., during middle school).

  • This can create the apparent emergence of a “new” learning or behavior problem, and requires that evaluators routinely inquire about brain injuries in their developmental history for any given case.


Executive function assessment strategies

Executive Function Assessment Strategies


Assessment

Assessment

Most psychoeducational measures do not assess executive functions.

Intellectual measures have rarely assessed EF, although recent efforts have tried to extend into this area. For example:

WISC-IV and WISC-IV-I

K-ABC-2

Stanford-Binet V

WJ-III Cognitive Abilities Test


Assessment1

Assessment

  • Executive deficits can appear in a myriad of medical conditions and disorders.

    • Can impact both learning and social-behavioral functioning in these situations.

  • In this regard, the inclusion of executive functions measures should be considered in any assessment of neurocognitive abilities.

  • In the past, there were few instruments available to clinicians—even neuropsychologists—for the assessment of executive functions.

    • This was accentuated by the lack of training in these types of assessments.


Assessment2

Assessment

Over the past 20 years or so the assessment of EF has seen the development of a number of tools, many of which are normatively based, standardized, and span a wide age range—including the preschool years.

Assessment tools even have been developed to assess EF using qualitative approaches and rating scales in an effort to increase ecological validity.

The “value added” may be critical to treatment planning and developmental surveillance.


Assessment3

Assessment

There are a number of standardized measures that provide estimates of selected executive functions.

Executive function batteries and ratings scales also have been developed.

Regardless of the measurement, remember the “s” in executive functions; i.e., this is a highly multidimensional construct.


Assessment4

Assessment

  • When one considers the various subcomponents of executive functions, there are measures of:

    • Planning and problem solving

    • Set maintenance and set-shifting

    • Inhibitory control

    • Working memory

    • Speeded responding

    • Attention regulation


Assessment5

Assessment

  • Problem Solving and Planning

    • Wisconsin Card Sorting Test

    • Test of Problem Solving Skills-Revised

    • HRNB Category Test (Booklet version)

    • Tower of Hanoi

    • Tower of London

    • Porteus Mazes

    • WJ-III Planning Subtest


Assessment6

Assessment

  • Set Maintenance and Set-Shifting

    • Wisconsin Card Sorting Test

    • Stroop Color and Word Test

    • Ruff Figural Fluency Test

    • Verbal Fluency Test

    • Trail-Making Test (Part B)


Assessment7

Assessment

  • Inhibitory Control

    • Continuous Performance Tests

    • Matching Familiar Figures Test

    • Go No-Go tasks

    • Stop Signal Test

    • Stroop Color and Word Test


Assessment8

Assessment

  • Working Memory

    • Span Tasks

      • Digit Span

      • Word Span (words and pseudowords)

      • Spatial Span

      • Dane-Carpenter task

    • Delayed recall types of tasks

      • WRAML-2 Design Memory

    • Manipulation tasks

      • Mental arithmetic

      • WJ-III Auditory Working Memory Subtest


Assessment9

Assessment

  • Attention Regulation

    • Continuous Performance Tests

    • Visual search tasks

    • Auditory Sequencing tasks


Assessment10

Assessment

  • Speeded Processing

    • Rapid Naming tasks

    • Motor sequencing tasks

    • Verbal fluency (e.g., Controlled Oral Word Association Test)

    • Nonverbal fluency (e.g., Ruff Nonverbal Fluency Test)

    • WJ-III Processing Speed Cluster

    • Reaction Time


Assessment11

Assessment

Working Memory Battery for Children

9 subtests based around the Baddelley Working Memory model

Delis-Kaplan Executive Function System

9 subtests that adapt classic EF measures

Behavioural Assessment of the Dysexecutive Syndrome for Children


Understanding and promoting executive functions in children

Wisconsin Card Sorting Test


Understanding and promoting executive functions in children

Tower Task


Understanding and promoting executive functions in children

Tower Task


Understanding and promoting executive functions in children

WISV-IV Integrated Spatial Span


Understanding and promoting executive functions in children

Stroop Color and Word Test


Understanding and promoting executive functions in children

Stroop Color and Word Test


Understanding and promoting executive functions in children

Stroop Color and Word Test


Assessment12

Assessment

Additionally, there are relatively new measures becoming available to assess selected executive functions in the preschool population, with tasks going down to two years of age.

Several of these tasks include:

The Shape School, which assesses inhibitory control

The IS Task, which measures set-shifting

Working memory span tasks

The Tower Task, which assesses planning and problem solving

Continuous performance tests (e.g., Kiddie Conners CPT) are available for use for children ages 3 to 5


Assessment13

Assessment

To assess affective regulation, there are emergent tasks that extend from the laboratory into the clinical arena:

Reading the Mind in the Eyes Test

Benton Facial Recognition Test

The Awareness of Social Inference Test

NEPSY-II Social Perception Domain

Affect Recognition

Theory of Mind


Assessment14

Assessment

  • A number of the measures to assess affective regulation are now computerized.

  • The Penn Neuropsychological Battery

    • Emotional Recognition Task

  • The Affective Go No-Go Task from the Cambridge Neuropsychological Automated Battery

    • The individual is asked to inhibit responses to selected targets

  • The Diagnostic Assessment of Nonverbal Abilities (DANVA)

    • Provides estimates of emotional regulation and control via visual and auditory pathways.

    • Scores provide an indication of both receptive and expressive affective regulation.


Assessment15

Assessment

There are several ratings scales available to assess executive functioning in children and adolescents.

These measures are not direct assessment batteries per se, but rather comprehensive rating scales that provide estimates of both cognitive and affective regulatory functions in a multi-rater, multi-setting framework and in a time efficient manner.

Examples include:

The Dysexecutive Questionnaire for Children (DEX-C), which is part of the larger Behavioural Assessment of the Dysexecutive Syndrome for Children

The Behavior Rating Inventory of Executive Function (BRIEF)

The Delis Rating of Executive Functions – uses online administration, scoring, and reporting (ages 5 to 18 years)


Assessment16

Assessment

The BRIEF:

Three overall summary indices

Metacognitive Index

Behavioral Regulation Index

Global Executive Composite

Eight different empirically derived scales:

Inhibit

Shift

Emotional Control

Initiate

Working Memory

Plan/Organize

Organization of Materials

Self-Monitor


Assessment17

Assessment

Qualitative tasks that involve real-life simulations:

Video-taped vignettes of social situations

The Party Planning Task

The Child-Kitchen Task

The School Assessment of Motor and Process Skills

Provides an appraisal of skills that children need to function in the classroom setting.

The Children’s Cooking Task

An adaptation of an adult task that was designed to determine the child’s ability to follow a checklist without being distracted, evaluate the outcomes with respect to the initial goals for cooking, and make adjustments for any errors.

Comprised of making a chocolate cake and a fruit cocktail; performed in a kitchen.

Variables of interest include the number of errors and an overall qualitative analysis of the task.

Children with TBI made more errors in the cooking process as compared to aged matched controls.


Assessment summary

Assessment Summary

  • There are a variety of measures that currently exist that cross age boundaries from preschool through adulthood.

  • Employ one of the theoretical models of EF in your assessment approach.

    • Which constructs will you employ?

    • How does it align with known brain function?

  • Don’t hesitate to employ both quantitative and qualitative assessment methods.


Disorders showing executive dysfunction

Disorders Showing Executive Dysfunction


Disorders with executive dysfunction

Disorders with Executive Dysfunction

  • The presence of EF deficits can appear in a variety of disorders and conditions.

    • Chronic illness (e.g., diabetes, CKD)

    • Pediatric neurological conditions (e.g., traumatic brain injury, stroke)

    • Pediatric Genetic Disorders (e.g., Prader-Willi Syndrome, Fragile X Syndrome)

    • Psychiatric disorders (e.g., bipolar disorder, schizophrenia, substance abuse, childhood maltreatment)

    • Developmental disabilities (e.g., Autism Spectrum Disorder, Learning Disabilities, ADHD)


Pediatric traumatic brain injury

Pediatric Traumatic Brain Injury


Traumatic brain injury

Traumatic Brain Injury

  • Along with attention impairments, one of the most common findings in pediatric traumatic brain injury is the presence of various types of executive dysfunction.

  • This is not unexpected given that many cases of TBI will evidence some degree of frontal lobe involvement.


Traumatic brain injury1

Traumatic Brain Injury

  • Empirical studies have documented the presence of impairments in:

    • Planning and problem solving

    • Abstract thinking and analogical reasoning

    • Set-shifting and cognitive flexibility

    • Inhibitory control

    • Metacognitive capabilities

    • Processing speed

    • Working memory


Traumatic brain injury2

Traumatic Brain Injury

  • Given the regulatory role of the ventromedial prefrontal cortex in social and affective functions, a number of studies have documented the difficulties of self-regulation and social cognition in children following a traumatic brain injury.

    • These impairments have been documented in moderate to severe brain injuries up to five years post injury.

  • Self-regulatory abilities also can serve as mediators of the effects of traumatic brain injury and the emergence of social-behavioral difficulties in this population.


Traumatic brain injury3

Traumatic Brain Injury

  • Children sustaining a TBI also have evidenced problems in:

    • Identifying emotions

    • Labeling emotions

    • Extracting meaning from non-literal social communication

    • Evaluating problem solving outcomes

  • Higher-order language impairments have been reported such that both expressive and receptive language pragmatic skills (e.g., understanding proverbs, figurative sayings).


Traumatic brain injury4

Traumatic Brain Injury

  • In general, following a TBI, nearly all of these executive functioning components will evidence some degree of impairment that is dictated, in part, by the severity of the brain injury.

    • These findings have been reported for children with a mild TBI, although results were not as striking.

  • These deficits also have been reported to be persistent in some fashion 7 to 10 years post injury.


Traumatic brain injury5

Traumatic Brain Injury

  • Executive function ratings have been associated with:

    • Psychiatric status

    • Family burden

    • General family functioning

  • Adolescents with severe TBI tended to report fewer problems in executive functioning, when compared to their parents, particularly in their metacognitive abilities.

    • Assessment and treatment issues?


Autism spectrum disorder

Autism Spectrum Disorder


Autism spectrum disorder1

Autism Spectrum Disorder

Individuals with Autism—both low and high functioning—appear to manifest significant impairments when compared to various comparison groups.

These deficits included:

Planning and organization

Set-Shifting and perseverative tendencies

Abstract thinking

Executive dysfunction has been correlated with both the quality of joint attention and social interactions.


Autism spectrum disorder2

Autism Spectrum Disorder

In contrast, individuals with brain injuries to the frontal region are not “autistic.”

Neuroimaging studies have not documented clear frontal lobe brain injuries, but neurological abnormalities have been reported (e.g., early white matter tracts).

Neurocognitive findings have not been universally supportive of a global executive dysfunction in Autism.


Autism spectrum disorder3

Autism Spectrum Disorder

Studies of older children have converged to suggest that working memory is not a deficit in Autism, and this is the only EF domain with consistent results.

Several research groups reported deficits in problem solving and self-monitoring, perhaps secondary to a lack of verbal self-regulation, but his has been contested.

Deficits in cognitive flexibility were documented in some studies but, again, not in others.

Independent of verbal IQ or present even after controlling for verbal IQ.

Intact cognitive flexibility also has been linked to improvements in social competence.


Autism spectrum disorder4

Autism Spectrum Disorder

Several research groups have reported deficits in inhibition, while others reported no deficits.

Could they be related to poor verbal abilities or are they unrelated?

Regarding attention, children with Autism performed similarly to children with developmental language delays matched on nonverbal IQ on a sustained attention task, although their modulation of arousal levels has been implicated.

This was not consistent with the high prevalence of ADHD symptoms in individuals with Autism.

Individuals with Autism also seem to experience less encroachment on their functioning during divided attention tasks.


Autism spectrum disorder5

Autism Spectrum Disorder

A model of executive functioning may prove to be useful in linking selected executive functions to core and associated symptoms.

Working memory, cognitive flexibility, and response inhibition have been variably related to restrictive and repetitive symptoms, but planning and fluency were not.

Verbal fluency difficulties could be another contributor to communication impediments in individuals with Autism.

Organizational deficits have been noted, but not thoroughly studied.

Baron-Cohen Empathizing-Systemizing (ES) theory would suggest otherwise.


Autism spectrum disorder6

Autism Spectrum Disorder

While this theory can explain some aspect of each of the core problems with Autism, they are not universally supported and account for small amounts of the variance of the core symptoms.

Executive dysfunction also is not exclusive to Autism and, consequently, is not the sole cause of autistic behaviors.

These deficits also cannot explain some of the assets that many children with Autism can manifest (e.g., savant skills).


Autism spectrum disorder7

Autism Spectrum Disorder

Current theorizing indicates that while executive dysfunction can be present early in development, it also can surface as a secondary deficit as children with Autism increase in age.

There also may be environmental contributors to the executive dysfunctions, such as a caregiver unintentionally reinforcing cognitive rigidity in an interaction.

Further, given the prolonged developmental trajectory of many executive functions, might an extended window for neurorehabilitation be present?


Fragile x syndrome

Fragile X Syndrome


Fragile x syndrome1

Fragile X Syndrome

  • Most common known inherited cause of ID, and the second most frequent chromosomal cause of ID after Down Syndrome.

  • The Fragile X Mental Retardation 1 gene (FMR-1) was been isolated and identified in 1991.

  • Prevalence: 1/1250 males; 1/2500 females in the general population.

  • Fragile X is an X-linked disorder, named for a mutation or fragile site on the long arm of the X chromosome at locus Xq27.3.


Fragile x syndrome2

Fragile X Syndrome

  • This mutation is associated with an elongated sequence of trinucleotide repeats (CGG) within the FMR-1 gene.

    • Normal = 5 to 52 repeats

    • Premutation = 52 to 200 repeats (the person is usually unaffected)

    • Full mutation > 200 repeats (full expression)

  • A full mutation is associated with an abnormal methylation of the FMR-1 gene, and will affect cells where the FMR protein is expressed.


Fragile x syndrome3

Fragile X Syndrome

  • Because it is an X-linked disorder, its effects are more severe in males than females.

  • Fragile X gene affects physical features (e.g. macrocephaly, elongated face, prominent jaw, and long protruding ears), behaviors (e.g., ADHD, anxiety), and overall development.

  • Associated problems include mitral valve prolapse (80%), orthopedic problems (73%), recurrent otitis media (63%), and seizures (20%).


Fragile x syndrome4

Fragile X Syndrome

  • Many of the behavior problems in FXS can be traced to possible EF deficits.

  • The available literature has pointed to problems in:

    • Attention modulation

    • Impulsivity/Inhibition

    • Problems managing transitions

    • Perseveration

    • Sequencing problems

    • Working memory

  • EF in FXS has received moderate examination, but few studies directed to children.


Fragile x syndrome5

Fragile X Syndrome

  • Problem Solving/Cognitive Flexibility

    • NEPSY Tower

    • Contingency Naming

    • WJ-III Rapid Picture Naming

    • WJ-III Planning

  • Inhibition/Attention Regulation

    • Auditory CPT False Alarms

    • Auditory CPT d-prime

    • Visual CPT False Alarms

    • Visual CPT d-prime

    • Day-Night Task

  • Working Memory

    • Leiter-R Reverse Memory

    • WJ-III Auditory Working Memory

    • N-Back Task

  • Processing Speed

    • Auditory CPT Reaction Time (to hits)

    • Visual CPT Reaction Time (to hits)


Understanding and promoting executive functions in children

Tower Task

*

Hooper et al., 2008


Understanding and promoting executive functions in children

Auditory Continuous Performance Test

*

Hooper et al., 2008


Understanding and promoting executive functions in children

Auditory Continuous Performance Test

Hooper et al., 2008


What happens over time

What Happens Over Time?


Understanding and promoting executive functions in children

NEPSY Total Correct

12

10

Problem Solving

8

6

4

2

Typical

FXS

0

44

49

54

59

64

69

74

79

84

89

MA (Leiter BIQ, Age in Months)

Hooper et al., 2013


Understanding and promoting executive functions in children

WJ-III Planning

120

100

Planning

80

60

40

20

Typical

FXS

0

44

49

54

59

64

69

74

79

84

89

MA (Leiter BIQ, Age in Months)

Hooper et al., 2013


Understanding and promoting executive functions in children

Day/Night Task

16

14

12

Set-Shifting

10

8

6

4

Typical

FXS

2

0

44

49

54

59

64

69

74

79

84

89

MA (Leiter BIQ, Age in Months)

Hooper et al., 2013


Understanding and promoting executive functions in children

Leiter-R Reverse Memory

WJ-III Memory for Words

9

16

8

Working Memory

14

7

12

6

10

5

8

4

3

6

2

4

Typical

Typical

FXS

1

FXS

2

0

44

49

54

59

64

69

74

79

84

89

0

MA (Leiter BIQ, Age in Months)

44

49

54

59

64

69

74

79

84

89

MA (Leiter BIQ, Age in Months)

Hooper et al., 2013


Fragile x syndrome6

Fragile X Syndrome

  • These findings support the available literature identifying EF deficits in children and adults with FXS.

  • These deficits manifest primarily in:

    • Problem solving/Cognitive flexibility

    • Working memory

    • Inhibition/attention regulation

  • These functions are disproportionately low even when compared to MA matched typicals, and change more slowly over time.


Written language disorder

Written Language Disorder


Written language disorder1

Written Language Disorder

  • A number of studies have begun to document the importance of executive functions to the writing process.

  • Hooper et al. (2002) showed that fourth and fifth grade students with and without writing problems manifested specific differences in their executive functions, with the poor writers being less proficient at initiation, set-shifting, and sustaining.

  • From a developmental perspective, it would seem that executive functions will carry more variance with respect to written language production as students move into their middle school years, perhaps serving as moderators to specific types of writing interventions.


Understanding and promoting executive functions in children

Z-scores

Executive Functions of Good Writers vs. Poor Writers


Written language disorder2

Written Language Disorder

  • One key executive function that has been the target of scientific investigations is working memory.

  • The cognitive workspace, or working memory, is important to written expression because it is the function that underlies the active maintenance of multiple ideas, the retrieval of grammatical rules from long-term memory, and the recursive self-monitoring that is required during the act of writing.

  • Working memory contributes to the management of these simultaneous processes, and a breakdown may lead to problems with written output.


Written language disorder3

Written Language Disorder

  • Reduced working memory capacity has been reported in poor writers when compared to good writers, and it appears to have both general and domain-specific contributions to the writing process.

  • Recent work by our group with fourth and fifth grade students with and without writing problems has documented not only working memory deficits, particularly in verbal working memory and its development, but also broader memory problems that could undermine the entire writing process.


Written language disorder4

Written Language Disorder

  • A contemporary model of written expression, the Not-So-Simple View of Writing, incorporates three major components:

    • Transcription (handwriting or letter production and spelling or word production)

    • Executive functions (planning, monitoring, revising)

    • Text generation (i.e., the main writing goal of the beginning writer)

      • Text generation occurs at the word, sentence, and text levels; consequently, automatic production of letters is necessary, but not sufficient, as spelling words via the alphabetic principle and related orthographic elements are necessary for writing proficiency. Working memory provides a mechanism for linking all of the components.

  • The Not-So-Simple View of Writing Model postulates that neuropsychological, linguistic, and related cognitive functions will be recursive throughout the development of the writing process, but that each of these functions will exert relatively more influence at different points in the developmental process.


Written language disorder5

Written Language Disorder

  • According to this model, early elementary school students will be constrained by fine-motor output (e.g., letter formation), memory for letters and words, emergent working memory capacity, and linguistic capabilities.

  • By middle school, many but not all children have sufficient transcription skills, and their writing skills will progress with increased emphasis being placed on the development of their executive functions.

  • We have demonstrated the general utility of this model, particularly with respect to the stability of the cognitive components over time, with the impact of executive functions being noted even in first grade.


Neurocognitive components

Neurocognitive Components

Fine Motor

Attention/ Executive Function

Language

WIAT II

Written

Expression

and Spelling

Elision/PAL Phonemes

PAL

Letters

PAL Word Choice

PAL Finger Succession Dominant

PAL Finger Succession Non-Dominant

Verbal Working Memory

WJ-III Retrieval Fluency

WJ-III Planning

VIGIL Omissions

VIGIL

Commissions

Visual Working Memory


Executive function management strategies

Executive Function Management Strategies


Management strategies

Management Strategies

Problem Solving

  • Develop a guide to help the individual through the stages of problem solving

    • Identifying the problem

    • Acquire relevant information

    • Generate several possible solutions

    • List pros and cons for each solution

    • Identify best solution

    • Create a plan of action

    • Evaluate the effectiveness of the plan

    • Encourage generalization


Management strategies1

Management Strategies

Problem Solving

  • Raise questions about alternatives and consequences (Can they predict outcomes?)

  • Allow the child to bring up relevant real-life problems that are appropriate for group discussions, and promote brainstorming

  • Introduce roadblocks and complications to encourage flexibility

  • Provide ongoing, non-judgmental feedback


Management strategies2

Management Strategies

Problem Solving

  • Computerized intervention for problem solving have received some support

    • Teen Online Problem Solving Program for pediatric traumatic brain injury

  • Computerized interventions for attention regulation have been mixed.


Management strategies3

Management Strategies

Organizational Processes

  • Limit the number of steps in a task

  • Provide part of a sequence and have the student finish it

  • Give cues such as, “Correct, but what do you think will happen next?”

  • Structure thinking processes graphically (e.g., time lines, outlines, flow charts, graphs, etc.)


Management strategies4

Management Strategies

Organizational Processes

  • Use categories to focus on one topic at a time

  • Identify the main idea and supporting details, categorize them, and encourage student to do the same

  • Have the student practice organizational skills in other settings


Management strategies5

Management Strategies

Speed of Information Processing

  • Recognize as the student fatigues, speed of processing declines

  • Recognize speed of processing can be negatively affected by some medications

  • Eliminate timed measures to assess learning

  • Reduce the amount of work required


Management strategies6

Management Strategies

Speed of Information Processing

  • Provide a reader for text materials and tests

  • Permit the student the use of an aide or peer tutor to take notes for them, or a computer for written language

  • Lower difficulty level until speed of processing improves


Management strategies7

Management Strategies

Speed of Information Processing

  • Allow extra time for the completion of tests and assignments

  • Frequent breaks allow the student to “recharge”

  • When necessary, allow the student extra time to travel between classes


Management strategies8

Management Strategies

Working Memory

  • Making lists

  • Graphic organizers

  • Automatization of information

    • This frees up other cognitive resources (e.g., driving a car)

    • Overlearning and rehearsal training

  • Environmental manipulations

    • Chunking, repetition

    • Use of technology (e.g., iPads, iPhones)

  • Computerized training programs

    • Cogmed Working Memory Training Program


Management strategies9

Management Strategies

  • Social Skills Curricula

    • ICPS-I Can Problem Solve: Preschool, Kindergarten, and Elementary

    • Skillstreaming in Early Childhood, Elementary, and Adolescence

    • Second Step: Preschool through 9th grade

    • Positive Action: Elementary grades

    • The ACCEPTS Program: Elementary

    • The PREPARE Curriculum: Middle and High School

    • The ACCESS Program: Middle and High School

    • Teaching Social Skills to Youth: A curriculum for child-care providers

  • Innovative Programming addressing:

    • Affective Training

    • Family involvement

  • Field needs more evidence-based treatment approaches to the management of EF deficts.


Management strategies10

Management Strategies

  • Feeney & Ylvisaker (1995) developed an empirically-based approach to reduce maladaptive behaviors and increase on-task efforts:

    • Analysis and restructuring of the individual’s daily routine wherein the sequencing of the routine was negotiated (e.g., task analysis)

    • Liberal use of visual cues to facilitate the sequencing

    • Liberal rehearsal prior to each component, accompanied by a performance review


Management strategies11

Management Strategies

  • Feeney & Ylvisaker found that this program:

    • Promoted predictability and orderliness

    • Gave the individuals a sense of control

    • Promoted goal setting

    • Lessened oppositionality and impulsivity

    • Increased decision-making

    • Increased problem solving efforts


Management strategies12

Management Strategies

  • Self-Regulated Strategy Development (SRSD) is a model wherein individuals are taught an overarching strategy to learn specific strategies.

  • SRSD comprises three major areas:

    • Six stages of explicit instruction (i.e., Develop background knowledge; Discuss it; Model it; Memorize it; Support it; Independent performance)

    • Explicit instruction in self-regulation strategies including goal setting, self-monitoring, and self-instruction

    • Develop positive self-efficacy about strategy use


Management strategies13

Management Strategies

  • Written language is considered a problem solving process that involves planning, knowledge transfer, and various skills.

  • The SRSD model distinguishes itself from alphabetic principle-based intervention strategies (e.g., PAL) as the focus is on advancing higher-order executive functions versus developing phonological processes, vocabulary, or other language functions.

  • Developmentally appropriate for middle school.

  • Significantly positive data for students with writing disabilities and behavior disorders.

  • Holds promise for teaching other self-regulatory skills (e.g., behavior).


Management strategies14

Management Strategies

  • Math Curriculum: Solve It!

  • A research-based instructional program in which teachers explicitly teach the processes and strategies that underlie mathematical problem solving.

  • Incorporates the cognitive processes critical to mathematical problem solving, with a particular focus on strategies:

    • Reading the problem and developing math vocabulary

    • Paraphrasing

    • Visualizing

    • Hypothesizing about problem solutions and their order

    • Estimating and predicting the answer

    • Computing via procedures and calculations

    • Checking the problem

  • Students learn a metacognitive strategy that they apply at each step:

    • Say aloud or to themselves what the problem is asking them to do

    • Ask themselves if they understand the problem

    • Check their progress

  • Data on students with MDs are encouraging.


Conclusions

Conclusions


Conclusions1

Conclusions

  • There are numerous definitions and models of executive function to consider, but the available evidence clearly indicates that this a complex, multidimensional construct that evolves over the course of development.

  • There is a clear linkage to underlying neurological integrity and neurodevelopmental processes that is critical to understanding executive functions and dysfunctions.


Conclusions2

Conclusions

  • The assessment of executive functions has come a long way over the past two decades, with many measures moving out of the laboratory into the clinical setting

    • Affective regulatory measures

  • Even with these advances, the assessment of EFs is not conducted as part of a routine psychoeducational or psychological evaluation, or as part of other types of evaluation (e.g., speech/language, OT, etc.).


Conclusions3

Conclusions

  • Given the regulatory aspects of many of the EFs, it is not uncommon to see functions such as expressive and receptive language, sensory-motor functions, or academic achievement skills appearing to be unaffected by a brain injury, yet the child is not able to access the preserved information accurately and/or efficiently secondary to executive dysfunction.

  • Given the relatively high prevalence of frontal lobe involvement in children and adolescents with a wide variety of disorders, this oversight could leave a significant hole in the overall profile of the impact of a specific disorder or condition on cognitive functioning, adherence to care, response to treatment, and quality of life.


Conclusions4

Conclusions

  • Without the assessment of executive functions, those working with the child are left with other assumptions about the nature of the child’s capabilities (e.g., lazy, unmotivated, uninterested, behavioral problems, etc.).

  • Consequently, it is essential for the examiner to include executive function measures as part of any assessment of a child as part of a larger assessment.


Conclusions5

Conclusions

  • As a final note, it is suspected that the administration, scoring, and interpretation of executive function measures are rarely taught across the various professional training programs (e.g., clinical psychology, school psychology, speech and language, occupational therapy, etc.), and it is time for these types of assessment procedures to become part of training programs.

  • This is important not only from an assessment perspective, but also with respect to how executive dysfunctions can impact upon therapeutic activities of children and the understanding of how these deficits can emerge later in development.

  • Field needs more evidence-based treatments!


Questions

Questions


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