The Neuropsychology of Spina Bifida:

Research You Can Use!

• T. Andrew Zabel, Ph.D., ABPP• Kennedy Krieger Institute

• Johns Hopkins University School of Medicine

• zabela@kennedykrieger.org

Thanks to:

Eric Levy, M.D.

Scott Schultz, M.D.

Susan Demetrides, L.P.N.

E. Mark Mahone, Ph.D.

Lisa Jacobson, Ph.D.

Darcy Raches, Ph.D.

Reem Tarazi, Ph.D.

Srishti Rau, M.A.

Disclaimer

Spina Bifida is It’s Own Thing

Evolution of neuropsychological model

IQ: Mawdsley et al., 1967

Presence of HYD: Smith & Smith, 1973

Spinal lesion level: Hunt et al., 1973

Perceptual-Motor: Soare & Raimondi, 1977

Cocktail Party Syndrome: Tew & Laurence, 1979

VIQ>PIQ: Dennis et al., 1981

Reading: Barnes & Dennis, 1992

Subtle language deficits: Dennis et al., 1994

Nonverbal Learning Disability: Fletcher et al., 1995

Executive Dysfunction: Fletcher et al., 1995; Mahone et al., 2002

Math: Barnes et al., 2002

Phenotype of SB: Dennis et al., 2006

Nonverbal Learning Disability

Nonverbal Learning Disability (Rourke, 1995)

Nonverbal Learning Disability (Rourke, 1995)

Identification of a Cognitive Phenotype

Main points

It’s not all about hydrocephalus (or

the brain’s white matter)

The malformations that underlie the

hydrocephalus are important too

Brainstem malformations in SB are

associated with a unique set of

cognitive issues

Neural Phenotype: Primary CNS Insults

Timing

Attention

Movement

Core Deficits

Assembly Associations

Neural Phenotype:

Secondary CNS Insults Environment

Perception

Language

Literacy

Numeracy

Quick Reference Sheet for Educators

Cognitive Phenotype of SB

Strength: Associative Processing

Linking words with word meanings

Linking letters with letter sounds

Gradually learning math facts by memory

Navigating by landmarks

Weakness: Assembled Processing

Drawing inferences

Using multi-step math procedures

Navigating by coordinates

Associative and Assembled Processing

Literacy/Reading

Strength: Associative Processing

Read words and access word meanings

Vocabulary learned through association and repetition

Knowledge

Grammatical skills

(Barnes & Dennis, 1998; Barnes et al., 2004; Dennis, Jacennik, & Barnes, 1994).

Weakness: Assembled Processing

Constructing text understanding

Using the sentence context to suppress one word meaning in

favor of another

Bridging inferences (conclusions reached on the basis of

reasoning and evidence found at different points in text)

(Barnes et al., 2007; Clifton & Duffy, 2001

Bridging Inferences

Near condition:

It was the day of the big bake sale at Ryan’s school.

All of his friends had walked past the tables and picked

out their favorite snacks.

Ryan was very hungry that day, and was hoping to buy a

red velvet cupcake.

Ryan had forgotten to bring his money to school.

a) Ryan bought a delicious red velvet cupcake.

b) Ryan had money left over for an extra snack.

c) A friend of Ryan’s gave him a bite of his brownie

Bridging Inferences

Near condition:

It was the day of the big bake sale at Ryan’s school.

All of his friends had walked past the tables and picked

out their favorite snacks.

Ryan was very hungry that day, and was hoping to buy a

red velvet cupcake.

Ryan had forgotten to bring his money to school.

a) Ryan bought a delicious red velvet cupcake.

b) Ryan had money left over for an extra snack.

c) A friend of Ryan’s gave him a bite of his brownie

Bridging Inferences

Far condition:

Ryan had forgotten to bring his money to school.

It was the day of the big bake sale at Ryan’s school.

All of his friends had walked past the tables and picked

out their favorite snacks.

Ryan was very hungry that day, and was hoping to buy a

red velvet cupcake.

a) Ryan bought a delicious red velvet cupcake.

b) Ryan had money left over for an extra snack.

c) A friend of Ryan’s gave him a bite of his brownie

Literacy/Reading

What’s behind the problem with bridging

inferences?

Early (~36 months) cognitive abilities help support

later (~9.5 year) reading comprehension and

bridging inference skill

Working memory/inhibitory control

Oral language comprehension

Narrative recall

(Pike, Swank, Taylor, Landry, & Barnes, 2013)

Literacy/Reading

Weakness: Using the sentence context to suppress one word

meaning in favor of another

“John laughed when he picked up the spade”

Literacy/Reading

Using the sentence context to suppress one word meaning in

favor of another

“John laughed when he picked up the spade”

“John drew his last card, and laughed when he picked up the

spade”

Literacy/Reading

Using the sentence context to suppress one word meaning in

favor of another

“John laughed when he picked up the spade”

“John drew his last card, and laughed when he picked up the

spade”

“He wiped the sweat from his forehead in the hot sun, and then

John laughed when he picked up the spade”

Literacy/Reading

Using the sentence context to suppress one word meaning in

favor of another

“John laughed when he picked up the spade”

“John drew his last card, and laughed when he picked up the

spade”

“He wiped the sweat from his forehead in the hot sun, and then

John laughed when he picked up the spade”

The working memory and inhibition demands are higher when

the information necessary for inference is further away in the

text (ala “bridging inference”)

Literacy/Reading

Take home message

Word reading is an early strength shown by many

children with Spina Bifida

Be on the look-out for reading comprehension

difficulties

Advocate for early assessment and periodic

reassessment of reading comprehension in school, as

this is a common area of difficulty in the Spina

Bifida phenotype

Numeracy/Math

Strength: Associative Processing

Retrieval of math facts (e.g., 2 + 3 = 5) from memory

(Barnes et al., 2006)

Weakness: Assembled Processing

Performing math calculation of problems in which the

answers cannot be retrieved from memory

Numeracy/Math

Barnes, Wilkinson, Khemani, Boudesquie,

Dennis & Fletcher, 2006

Numeracy/Math

14

x 6

288/

48

Numeracy/Math

Take home message

Children with Spina Bifida typically learn their math

facts

Be on the look-out for math calculation/procedure

difficulties

Advocate for early assessment and periodic

reassessment of math calculation and procedures in

school, as this is a common area of difficulty in the

Spina Bifida phenotype

Tests of number sense given in kindergarten, such as the

Test of Early Mathematics Ability – Third Edition (TEMA-

3), can be predictive of third grade math deficits

Executive Functioning

Inhibition / Impulse Control

Working Memory

Initiation

Organization / Planning

Prospective Memory (Remembering to Remember)

Age (in years)

1816141210

BR

IEF

MC

I -

Me

an

Ite

m r

aw

Sco

re

3.0

2.5

2.0

1.5

1.0

.5

group

controls

Spina Bif ida

Tarazi, Zabel, & Mahone (2007), TCN

What rating did parents give most of the time?

Initiation, Working Memory, Planning, etc.

Often

Sometimes

Never

Executive Dysfunction

10 12 14 16 18

Controls

Spina Bifida

Executive Functioning

Extra adaptive and executive demands

The SB Double Whammy

Executive Functioning Difficulties

Extra self-care requirements (executive burden)

Executive Functioning

Self Management

Kennedy Krieger Independence Scales – Spina Bifida Version (KKIS-SB)

• Begin CIC on time

• Get out of bed on time

• Finish morning hygiene routine on time

• Take medication on time

• Keep room clean

• Finish chores