THE NEUROCOGNITIVE PROFILE OF SOUTH AFRICAN PRESCHOOL CHILDREN WITH NEUROFIBROMATOSIS 1

Objectives: Neurocognitive deficits are reported as the most frequent complication in children with Neurofibromatosis 1 (NF1), with poor attentional skills also described. 70% of school aged children attending our Neurofibromatosis clinic showed academic problems. This study aims to determine the neurocognitive profile of preschool aged children with NF1 in order to institute early intervention.

Methods: This forms part of an ongoing study. 11 preschool children aged 1 year to 8 years old, with NF1, were matched with 11 unaffected children with similar demographic profiles. Participants were tested using the Griffith's Scales of Mental Development. Parents completed the Aberrant Behaviour Checklist-Community (ABC).

Results: 2 control children were excluded. Data from 20 children was analysed (NF1=11; control=9). Mean age: NF1 vs. control was: 57.9 months vs. 56 months (SD 17.25 vs. 6.36). There was no significant difference in maternal age, education, housing, employment, or socio-economic status between the 2 groups. There were significant subtest differences in Eye-Hand Co-Ordination: mean NF1 vs. control: 80.3 vs. 101.4 (SD 11.04 vs. 20.43) p<0.017; Performance: mean NF1 vs. control: 92 vs. 104.1 (SD 12.01 vs. 10.3) p<0.027; and General quotient: mean NF1 vs. control: 90.7 vs. 103.7 (SD 10.72 vs. 12.92) p<0.028. The ABC questionnaire showed children with NF1 demonstrated significantly higher irritability: mean NF1 vs. control: 17 vs. 5.1, p<0.02; hyperactivity: mean NF1 vs. control: 8.2 vs. 9, p<0.008; and inappropriate speech: mean NF1 vs. control: 5.1 vs. 1.86, p<0.01.

Conclusion: These findings highlight general performance, specific visuospatial, executive function and behavioural deficits in young children with NF1, thus supporting the need for early intervention.