Download Effects on cognitive development and academic achievement

SPINA BIFIDA AND
COGNITIVE FUNCTION
Kara L. Leiser, Ph.D., LP
March 28th, 2015
OUTLINE
Spina Bifida and Hydrocephalus basics
 Effects on cognitive development and academic
achievement
 Summary
 Case example
 Strategies to enhance areas of vulnerability
 What to expect of neuropsychological assessment
 Resources and references
 Questions

SPINA BIFIDA AND
HYDROCEPHALUS
The Basics
SPINA BIFIDA

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Spina Bifida (SB) is a neural tube defect
associated with significant spine and brain
malformations
Creates a lesion, or opening, on the spinal column
Can occur at any level of the spine
 Evident in first weeks of gestation when the neural
tube is being formed
 Requires closure shortly after birth
 Lesion level matters
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SPINA BIFIDA AND HYDROCEPHALUS
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Between 60 and 95% of children with the most severe form
of SB, myelomeningocele, have hydrocephalus
Hydrocephalus is the buildup of fluid in the cavities
(ventricles) deep within the brain
Excess fluid increases the size of the ventricles and puts
pressure on the brain
Pressure of too much cerebrospinal fluid can damage brain
tissues and cause impairments in brain function
Surgical treatment for hydrocephalus can restore and
maintain normal cerebrospinal fluid levels in the brain
EFFECTS ON COGNITIVE
DEVELOPMENT AND ACADEMIC
ACHIEVEMENT
EDUCATIONAL ISSUES
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Children with SB/Hydrocephalus show unique
learning strengths and weaknesses
Special education assessment and
Neuropsychological evaluation are important
Parents and schools need to work together to
help young people meet their physical, social,
emotional, and academic goals
COGNITIVE FUNCTIONING IN CHILDREN
WITH SB/HYDROCEPHALUS
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General strengths
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Vocabulary

Word Reading/Decoding
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Learned Associations (Rote learning)
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Categorization
COGNITIVE FUNCTIONING IN CHILDREN
WITH SB/HYDROCEPHALUS
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General Weaknesses
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Spatial
Memory
Concept Formation
Language Discourse
Math
Reading Comprehension
Hand-eye coordination
COGNITIVE FUNCTIONING IN INFANTS AND
CHILDREN WITH SB

Core areas of developmental vulnerability that
impact learning
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Motor functioning: control/movement and timing
Children with SB have impacted upper and lower limb
control, and often eye movement difficulties
 Motor impairment restricts ability for infants to explore
environment, thereby reducing sensory experiences

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Attention orienting (i.e. turning towards a stimulus)
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Infants with SB have difficulty with attention orienting
(takes longer to shift attention than typically developing
infants), but once they attend to stimulus at a rate
comparable to peers
Impact an infant’s ability to learn from the
environment and impact functional skills
GENERAL INTELLIGENCE

Many children have overall IQ scores within the
broad average range, but every child is different
In SB, higher lesion level associated with lower IQ
scores
 Some studies have shown with SB + hydrocephalus
associated with lower IQ scores

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Discrepancy between verbal and non-verbal IQ
Word skills and IQs higher than performance skills
 Consistent evidence of visuomotor and
visuoperceptual deficits in children with
hydrocephalus

LANGUAGE
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Basic language often appears intact
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Language that depends on repetition and rote
memory is good
Tend to struggle with higher-order language
Metaphorical language
 Using language in a flexible manner
 Understanding complex grammatical structure
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Lack of efficiency in integration of information
and inferencing
LANGUAGE
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Challenge with language usage, rather than language
content
“Cocktail Party” phenomenon
Superficial content; use of overlearned social phrases
 Tendency to “talk too much”
 May use words out of context
 Generally becomes less evident with age
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Conversations may be tangential
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Inefficient in ability to share information concisely
MEMORY
Immediate recall appears intact
 Deficits in word-list, paired associates, and new
fact learning
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Problems with initial encoding, rate of learning, and
delayed free recall
Overreliance on rote recall
 Poorly developed organizational strategies
needed for effective storage and retrieval of
information
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VISUAL MOTOR AND VISUAL SPATIAL SKILLS
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Poor hand-eye coordination
and
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Ineffective motor skills
interfere with the ability to move, use tools, read, and
write
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Ocularmotor difficulties (i.e. nystagmus; strabismus)
result in difficulties with visual fixation, gazeshifting, tracking and scanning. Implications for:
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Spatial Learning
Rule-based learning
ATTENTION AND EXECUTIVE FUNCTIONING
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Attention refers to the ability to direct attention to
an object, maintain this attention, and then
redirect it to another object
Individuals with SB
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Generally intact simple attention
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Struggle with more complex attention demands
Shifting attention from one task to another
 Divided attention
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ATTENTION AND EXECUTIVE FUNCTIONING
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Executive functions are the skills necessary to regulate
cognition and behavior, including:
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Initiation
Planning/Organization
Generate new information or solutions
Inhibit impulses
Demonstrate cognitive flexibility
Hold and manipulate information in working memory
Self-Monitoring
Emotional Control
Individuals with SB and Hydrocephalus
Have trouble with organization
Have trouble with sequencing (keeping ideas or doing
activities in the proper order)
 Struggle with novel problem solving and abstract reasoning
 Have trouble with decision-making and using what was
learned in the past to solve a new problem now
 Have trouble regulating behavior to achieve a goal
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ACADEMIC ACHIEVEMENT
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SB affects academic achievement in reading and
math; math is greater affected than reading
Reading comprehension less skilled than
decoding
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May have adequate to above average word reading
and vocabulary knowledge
Reading and Discourse comprehension less
developed/inefficient, especially when comprehension
requires:
Integration of ideas
 Making inferences
 Suppressing information no longer relevant

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Greater challenge with greater chunks of text
ACADEMIC ACHIEVEMENT
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Struggle with Arithmetic
May have relatively intact simple calculation skills
 Deficits in applied math, such as word problems
 Struggle with algebra and geometry concepts
 Sequencing challenges
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Struggle with Written Expression
Poor spelling
 Difficulty generating/organizing/sequencing ideas
 Slow rate of writing
 Poor handwriting due to fine motor difficulties
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SOCIAL AND EMOTIONAL FUNCTIONING
Have been described as socially inappropriate or
immature
 Younger children may be indiscriminately
friendly or overly familiar
 Older children may be socially withdrawn
 Depressed mood
 Anxiety
 Somatic (physical) complaints
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NONVERBAL LEARNING DISABILITY
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Cognitive profile with the following characteristics:
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Social
Lack of ability to comprehend nonverbal communication
 Deficits in social judgment and social interaction
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Academic
Problems in math, reading comprehension, handwriting
 Problems with organization, problem-solving higher
reasoning
 Strengths include strong verbal and auditory attention and
memory
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NONVERBAL LEARNING DISABILITY
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Visual-Spatial
Lack of image, poor visual recall
 Faulty spatial perception and spatial relations
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Motor
Lack of coordination
 Severe balance problems
 Difficulties with fine motor skills
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Emotional
Frequent tantrums, difficulties soothing, easily
overwhelmed
 Fears of new places and changes in routines
 Prone to depression and anxiety as they get older
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SUMMARY
SUMMARY
Cognitive profile is on a continuum rather than
an “all or nothing” picture
 Hydrocephalus status plays a big role
 Strengths in basic and rote skills
 Weaknesses in visual-spatial skills, abstract
concept formation, complex language, complex
attention, and executive functioning
 Expect individual variability with regard to
cognitive development
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CASE EXAMPLE
Mary
CASE EXAMPLE – MARY
12-year-old left-handed female
 Spina Bifida
 Shunted hydrocephalus
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Numerous revisions
History of cognitive and academic difficulties
Struggles with concepts of money, time, and tasks
involving charts or graphs
 Slow processing speed
 Struggles to follow multi-step directions and needs
help with organization
 Social difficulties
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CASE EXAMPLE – MARY
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Test Results
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Below average IQ overall, but significantly better
verbal than nonverbal abilities
Mildly impaired processing speed
Impaired sustained attention
Basic vocabulary within average range
Difficulty following complex instructions
Impaired visual-spatial skills
Impaired novel problem solving skills
Below average math skills
CASE EXAMPLE – MARY
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Recommendations
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Academic support for math
Extended time for tests and assignments
Class notes and outlines
Use of keyboarding or voice recognition software
Address attention
Organizational skills
Participation in social groups and activities
STRATEGIES TO ENHANCE
AREAS OF VULNERABILITY
READING COMPREHENSION
DEVELOPMENT
Learn the meanings of new words
 Learn to ask/answer questions during
conversations, storytime
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Ask “Why?” questions throughout the day to create
cause and effect relationships (e.g. Why do we have
houses?)
Learn to predict/anticipate what might happen
next in a social situation or story
Give your child a chance to draw conclusions from different
pieces of information (e.g. It’s snowing outside and there is
a fire in the fireplace, what time of year is it?)
 Look at a picture and have your child describe what s/he
sees
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Learn to summarize or retell a story
MATH FUNDAMENTALS DEVELOPMENT
Practice counting throughout the day
 Help your child learn to sort items by category by
grouping according to shared characteristics
 Help develop concepts such as more and less by
comparing different groups of items
 Sharing pizza or other food can be a good
teaching tool
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Help child count the people, divide the food, and
distribute to each person one at a time
 Play fun counting games with math concepts
embedded in them (e.g. Candyland; Dominos)
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Help your child recognize and label simple
shapes
PROMOTING EXECUTIVE FUNCTION
DEVELOPMENT
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5 Key Principles
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Skills are taught and learned
Begins with environmental manipulation
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Repetition, repetition, and more repetition
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Rehearse, review, teach back
Success increases with investment
Process needs to be simple, streamlined, and individualized
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The “External” Brain – low tech and high tech
Cues
Rewards and natural consequences
Reduce multi-tasking
Keep steps short
Make steps meaningful, use more than words
What doesn’t work
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Punishment/Lectures/Doing for with hope of spontaneous
imitation
FAMILY FUNCTIONING
Parenting factors shown to affect well-being:
 Responsive parenting style at all developmental
levels
Accurate perception of child’s needs with contingent
responding
 Balance supportive learning and still providing child
control
 Emotional/affective support
 In adolescents, respond to needs for increasing
responsibility and decision-making
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Appropriate monitoring and clear behavioral
expectations
 Parental health, stress and coping
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WHAT TO EXPECT OF
NEUROPSYCHOLOGICAL
ASSESSMENT
NEUROPSYCHOLOGICAL ASSESSMENT
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Neuropsychology is a subspecialty of clinical
psychology that specializes in the assessment and
treatment of patients with brain injury or disease
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Knowledge of the brain, including an understanding
of areas such as neuroanatomy and neurological
disease
Involves the application of standardized
measures in the study of brain behavior
relationships
“Learn how a person learns”
WHAT TO EXPECT ON
“LEARN HOW YOU LEARN DAY”
Bring documentation of any previous testing
and/or educational records (e.g. IEP)
 Bring corrective lenses, hearing aids, etc. as
would be utilized in the school setting
 Participate in a clinical interview
 Patient will be observed and administered a
series of standardized measures that may include
oral responding, listening, pointing, paper and
pencil activities, computer game, small motor
manipulations, etc.
 Complete rating scales/questionnaires
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RESOURCES, REFERENCES, AND
QUESTIONS
RESOURCES
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Spina Bifida Association
http://www.spinabifidaassociation.org/
 Resources for families and educators
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“Executive Skills in Children and Adolescents: A
Practical Guide to Assessment and Intervention”
(2004) by P. Dawson and R. Guare
“Smart but Scattered: The Revolutionary ‘Executive
Skills’ Approach to Helping Kids Reach Their
Potential” (2009) by P. Dawson and R. Guare
“Late, Lost, & Unprepared: A Parents Guide to
Helping Children with Executive Functioning” (2008)
by J. Cooper-Kahn and L. Dietzel
PACER Center

www.pacer.org
SELECTED REFERENCES
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Dennis, M., & Barnes, M. (2010). The cognitive phenotype of spina bifida
meningomyelocele. Developmental Disabilities Research Reviews, 16, 31-39.
Dennis, M., Landry, S.H., Barnes, M., & Fletcher, J. (2006). A model of
neurocognitive function in spina bifida over the life span. Journal of the
International Neuropsychological Society, 12, 285-286.
Iddon, J. L., Morgan, D.J.R., Loveday, C., Sahakiam, B.J., & Pickard, J.D.
(2004). Neuropsychological profile of young adults with spina bifida with or
without hydrocephalus. Journal of Neurology Neurosurgery Psychiatry, 75,
1112-1118
Taylor, H.B., Landry, S.H., Engish, L., & Barnes, M. (2010). Infants and
children with spina bifida. In Donders, J., & Hunter, S. J. (Eds.). Principles and
practice of lifespan developmental neuropsychology (pp. 169-181). New York:
Cambridge University Press.
Zebracki, K., Zaccariello, M., Zelko, F., & Holmbeck, G. (2010). Adolescence and
emerging adulthood in individuals with spina bifida: a developmental
neuropsychological perspective. In Donders, J., & Hunter, S. J. (Eds.).
Principles and practice of lifespan developmental neuropsychology (pp. 183193). New York: Cambridge University Press.
QUESTIONS???
THANK YOU!!!