1. Cognitive and behavioural profile in NF1
Dr Christine Steiger
Consultant Community Paediatrician
Wirral

2. Wirral NF1 FU clinic Commonest parental concerns:
Developmental progress pre-school
Educational progress
Attention difficulties
Social skills
Clumsy, organisational difficulties
How to help? Who can help?

3. Nf1 and Learning Difficulties
Significant generalised LD unusual
3-8% IQ <70
Downward shift of IQ
High incidence of specific difficulties
Incidence 30-60%
Academic underachievement

4. Emotional and Behavioural concerns
Poor interpretation of social cues, difficulty forming friendships
Attention difficulties, hyperactivity, impulsivity
Low self esteem
Impact of chronic health condition

5. Literature review Kathryn North et al Dev Med and Child Neurology 2006
81 children NF1. 43m 38f mean age 11y6m. Range 8-16y9m no CNS pathology
49 comparison children (unaffected sibs)
Neuropsychological assessment

6. Of NF1 children SpLD 16 (15 male) 20% GLD 26 32% no LD 39 48%
31 38%, satisfied DSM-IV criteria for ADHD
7/10 children with NF1 and literacy disability also has ADHD

7. NF1-SpLD group had deficits of. attention. planning
NF1-SpLD group had deficits of attention planning visuospatial skills language (receptive and expressive)
Had strengths in visual and verbal memory verbal fluency fine motor co-ordination
High comorbidity of ADHD

8. No LD NF1
28% had ADHD
Difficulties planning visuospatial skills

9. ADHD in NF1
Behavioural phenotype in NF1 group with ADHD/ADD same as in control group of ADHD/ADD.
Behavioural management and medication led to improved academic achievement and reduced behavioural problems

10. Developmental Coordination disorder
Significant impairment motor skills for age and opportunity
Affecting academic performance and/or daily living
Present from early developmental period
Not explained by LD or neurological disorder

11. Autistic Spectrum Disorder
Persistent difficulties with social communication and social interaction
Restricted and repetitive patterns of behaviours, activities or interests
Present since early childhood
These limit and impair every day functioning
Includes sensory behaviours

12. Advice and Support
Consider the possibility of learning and behavioural difficulties in NF1
Readiness to assess for ADHD, DCD, ASD
Recognition of difficulties can help!
Process of SEN support
Sharing of information.
Medical needs can also affect progress

13. Interventions
Pathways to assess and manage concerns will vary between areas
National measures SEND Code of Practice (Education) NICE Guidelines (Health)
Educational advice and support
Health advice and support; SALT, OT, PT
Voluntary agencies

14. Address needs Child centred (PCP) May be a cluster of difficulties
Not all about a diagnosis!
Recognise and support needs
Services to work together
SEND process
Monitor progress, review. Outcomes
Things can get better!