Motor problems in children with neurofibromatosis type 1

André B Rietman,Caspar W N Looman,Sanne Bongers,Rianne Oostenbrink,Pieter F A De Nijs,Sandra Van Abeelen,Eddy Gaukema,Marie-Claire De Wit,Jos G Hendriksen

doi:10.1186/s11689-017-9198-5

Abstract

BackgroundChildren with the neurogenetic disorder neurofibromatosis type 1 (NF1) often have problems with learning and behaviour. In both parent reports and neuropsychological assessment, motor problems are reported in approximately one third to one half of the children with NF1. Studies using broad motor performance test batteries with relatively large groups of children with NF1 are limited. The aim of this cross-sectional observational study was to describe the severity of motor problems in children with NF1 and to explore the predictive value of demographics, intelligence, and behavioural problems.MethodsFrom 2002 to 2014, 69 children with NF1, aged 4 to 16 years (age = 9.5 ± 2.8 years; 29 girls) had a motor, psychological, and neurological evaluation in an NF1 expertise centre. Data were collected about (1) motor performance (M-ABC: Movement Assessment Battery for Children), (2) intelligence, and (3) emotional and behavioural problems as rated by parents.ResultsSixty-one percent of these children scored within the clinical range of the M-ABC. In ordinal logistic regression analyses, motor problems were associated with symptoms of attention-deficit/hyperactivity disorder (ADHD), symptoms of autism spectrum disorder (ASD), and externalising behavioural problems. Motor outcome was not predicted by age, intelligence, scoliosis, hypotonia, nor hypermobility.ConclusionsMotor problems are among the most common comorbid developmental problems in children with NF1, and these problems do not diminish with age. Because of their impact on daily functioning, motor problems need to be specifically addressed in diagnosis, follow-up, and treatment of NF1.

Highlights

Children with the neurogenetic disorder neurofibromatosis type 1 (NF1) often have problems with learning and behaviour
Our study shows that motor problems frequently occur in our group of children with NF1: 61% of these 69 children have serious motor problems and another 17% score within the borderline range
Using a broad motor assessment in a large cohort of children with NF1, we showed a high prevalence of serious motor problems

Summary

Introduction

Children with the neurogenetic disorder neurofibromatosis type 1 (NF1) often have problems with learning and behaviour. In both parent reports and neuropsychological assessment, motor problems are reported in approximately one third to one half of the children with NF1. Studies using broad motor performance test batteries with relatively large groups of children with NF1 are limited. The aim of this cross-sectional observational study was to describe the severity of motor problems in children with NF1 and to explore the predictive value of demographics, intelligence, and behavioural problems. A shared abnormal neurodevelopmental process underlying cognitive and motor abilities in NF1 was hypothesised [9]

Methods

Results

Discussion

Conclusion