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

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?

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

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

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

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

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

No LD NF1 28% had ADHD Difficulties planning visuospatial skills

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

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

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

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

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

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!