NF1 Learning Disorders Clinic
Prof Kathryn North – Head, Neurogenetics Research Unit
A/Prof Arthur Shores – Clinical Neuropsychologist,
Macquarie University
Sharon Watt
– Clinical Neuropsychologist
Jennifer Irle
– Intern Clinical Neuropsychologist
Dr Belinda Barton
– Psychologist, and Head, CHERI
Jennifer Lorenzo
– Educational and Developmental Psychologist
Neurogenetics Research Unit
The Children’s Hospital at Westmead
What do we do?
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Provide neuropsychological assessments
for children with NF1
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Provide developmental assessments for
very young children
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Assistance for children experiencing
behavioural difficulties
Cognitive Aspects of NF1
Jennifer Irle
Sharon Watt
A/Prof Arthur Shores
What does cognitive mean?
z Thinking
skills
z Problem-solving
What do we examine in a
neuropsychological assessment?
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Intelligence
Memory
Attention
Visual processing / fine motor skills
Language
Higher processes e.g. planning/organisation
Academic skills
How do you measure cognitive skills?
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Neuropsychological assessment:
Pencil and paper tasks
Puzzles
Question and answer
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Parent / teacher questionnaires
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Helps find strengths and weaknesses in
thinking
Intelligence
Children with NF1
Low
Average
Average
High
Average
What are some common cognitive
difficulties in NF1?
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Visual
Processing
What does this mean?
One of the most common cognitive
problems in NF1 is a difficulty
processing visual information
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Difficulties on neuropsychological tests:
Judging orientations of lines
Assembling puzzles
Copying shapes
Visual
Processing
What does this mean?
Copying from the whiteboard can
become extremely tricky because
there are so many lines to copy down
and figure out how they go together.
What are some common cognitive
difficulties in NF1?
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Attention
What does this mean?
z Concentrating
z Being
able to divide and switch your
attention between 2 or more things
Attention
What does this mean?
z Many
children with NF1 have difficulties
with attention
z Hyperactivity
z Impulsivity
z Inattention
What are some common cognitive
difficulties in NF1?
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Behaviour
What problems are seen?
z Generally
z Social
related to attention difficulties
skills may be affected
What are some common cognitive
problems in NF1?
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Executive
Functions
What does this mean?
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Complex problem-solving
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“Thinking outside the box”
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Also includes:
Solving problems in mind
Organising
Planning
Stopping an automatic response
Executive
Functions
What does this mean?
z Planning
and Organisation
z Working
memory
What are some common cognitive
difficulties in NF1?
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Language
What does this mean?
z Difficulties
understanding and expressing
language:
Sequencing ideas
Telling stories
Following instructions
Responding to questions
z Affects
academic skills, particularly
reading and spelling
Preliminary Results from our NF1
Reading Study
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high percentage of children with NF1
experience reading difficulties
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particular difficulty when sounding out
words
Reading Examples
“bick” → “brick”
“gurve” → “groove”
Reading: Our Next Challenge
z Intervention
for children with reading
difficulties
z Early
identification of children who are at
risk of reading difficulties (at preschool or
early Kindergarten age)
Is this the complete picture?
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Most research has been on school-aged
children
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One small study:
Are these problems present in preschool and
kindergarten kids?
Preliminary Results
z Within normal range
z Difficulties concentrating and ignoring
distractions
Is this the complete picture?
z One
very big study:
Can we identify children who are at risk of
cognitive difficulties?
Longitudinal Study of
Children with NF1
Dr Belinda Barton
Our longitudinal study
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Very little is known about the cognitive skills
of very young children (< 4 yrs) with NF1
How do the cognitive skills of very young
children with NF1 develop over time?
Does the development of their cognitive skills
skills differ to children without NF1?
When do learning problems start to occur?
Answer: Longitudinal - follow young children
with NF1 as they grow older.
Why is early identification important?
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The chance that a Year 1 reader will remain a
poor reader in Year 4 = 88%
Restricted access to curriculum and
implications for building knowledge
Increased risk for behavioural and emotional
problems
Impact on future life opportunities and
employment
Early identification = Early intervention!
At risk factors and predictors
In children without NF1
z Family history of learning difficulties
z Children with language and speech problems
z Research has shown that performance on
certain tasks are predictive of later reading
difficulties
Our longitudinal study
What we want to find out:
z How do very young children with NF1 develop
their cognitive skills?
z When do learning problems start to occur?
Who can participate?
z Children with NF1 aged between 5 months
and 40 months
z Children without NF1
Our longitudinal study
What happens in the study?
z The child is assessed at certain ages in their
development
5m
9m
15m
21m
30m
…
m = months
y = years
40m
5y
7y
Our longitudinal study
The story so far:
z Study commenced in 2004 so we have
6 years to go!!
z Still recruiting - 21 children with NF1 and
5 children without NF1 tested
z Based on scores, most children performing
within age-appropriate limits
z Children with NF1 having difficulty in
completing tasks that measure executive
functioning
Strategies to Help Minimise
Cognitive Difficulties in NF1
Jennifer Lorenzo
Strategies for visual processing
difficulties
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Visual
Processing
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Ways to help
Simplified presentation of visual
information:
Cover all the page except current activity
Present fewer problems on a page
Increase print size
Avoid cluttered worksheets
Visual
Processing
Example
Name 3 colours of the rainbow.
1. _______________
2. _______________
3. _______________
Complete the problems below.
a. 1 + 3 = ___
b. 4 – 2 = ___
c. 2 x 3 = ___
d. 5 – 2 = ___
e. 1 x 8 = ___
Visual
Processing
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Ways to help
Check accuracy of what the child has
copied
Provide verbal instructions
Additional time to complete tasks
Write in different colours to help keep the
child in place
Visual
Processing
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Ways to help
Use paper, ruler, or child’s finger to help
keep his/her place when reading
Use graph paper to keep numbers in line
Highlight important text
Trace shapes and copy pictures
2
Play sequencing activities
+
3
5
Strategies for attention difficulties
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Attention
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Ways to help
For concentration difficulties:
Minimise potential distractions
Provide high interest activities
Keep instructions brief
Have additional breaks if possible
Assign shorter tasks
Present activities in smaller units
Reward on-task behaviour
Reduce directions to steps
Attention
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Ways to help
For hyperactivity:
Lots of energy = frequent breaks
Ignore little motor behaviours
Token economy
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For impulsivity:
Provide feedback
Prompt ‘stop, think, do’
Strategies for behaviour problems
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Behaviour
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Ways to help
Be consistent in applying consequences for
behaviours
Reinforce demonstration of appropriate
behaviour
Keep a list of problem behaviours
Behaviour management (e.g. anger reduction,
social skills training)
Behaviour
Behaviour checklist
Date & Time
Monday, 1 January
Problem behaviour
Tim had a temper tantrum
What was your child
doing?
How intense was the
behaviour? (0-10)
How long did the
behaviour last?
What did you do? Did it
work?
Crying, throwing his toys all
over the room
8
10 minutes
Yelled, no – it made him
more upset
Strategies for higher level
(executive) thinking difficulties
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Executive
Functions
Ways to help
z Planning/organisation
difficulties
Give instructions in small steps
Set up a schedule for homework
Assist in beginning a task
Help plan out tasks, making each part clear
Break down large tasks into more smaller
tasks
Encourage practice of strategy use
Executive
Functions
Ways to help
z Planning/organisation
difficulties
Establish a daily routine at home
Keep a calendar for important events
z Immediate/working
memory problems
Avoid multi-step commands
Provide with visual and verbal instructions
Verbally repeat new information
Strategies for language problems
Attention
Visual
Processing
Behaviour
Difficulties
Language
Executive
Functions
Language
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Ways to help
Expressive language
For younger children, identify objects using
categories
Expand child’s “word bank”
Relate events to real-life experiences
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Receptive language
Paraphrase to assist comprehension
Highlight main points of text
Increase understanding of unfamiliar words
Language
Ways to help
z Reading
Use a phonics approach
Develop an awareness of hearing word
sounds
Provide practice by presenting text that
contains phonics rules
Have the child say the sound as s/he writes
it
Language
Phonics example
The letters st can be seen at the beginning or
the end of words…
“I stir a mixture and cast a spell!”
Let’s try!
be_ _
_ _and
pa _ _
What’s next for the NF1 Learning
Disorders Clinic?
z Develop
remediation programs that
target learning and behavioural
difficulties
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Your support is vital for our research
Finally…
If you would like to be involved with any of our
research projects, please contact:
Jennifer Lorenzo
Ph: 02 9845 3875