Psychological disturbance and sleep disorders in children with neurofibromatosis type 1
Hilary Johnson1, Luci Wiggs, Gregory Stores
1Park Hospital for Children, Old Road, Headington, Oxford OX3 7LQ, UK. hilaryjohnson@onetel.com
Insights
Children with neurofibromatosis type 1 (NF1) exhibit higher rates of behavioral issues and sleep disturbances, including parasomnias. Early screening for conditions like attention-deficit-hyperactivity disorder is recommended.
Area of Science:
- Pediatric Neurology
- Child Psychology
- Sleep Medicine
Background:
- Neurofibromatosis type 1 (NF1) is a genetic disorder with varied clinical manifestations.
- Behavioral and sleep problems are frequently reported in children, but specific data for NF1 is limited.
Purpose of the Study:
- To investigate the prevalence and nature of behavioral and sleep patterns in children diagnosed with NF1.
- To identify correlations between sleep disturbances and specific behavioral difficulties in the NF1 population.
Main Methods:
- A cross-sectional descriptive survey was conducted using postal questionnaires and telephone follow-ups.
- Validated instruments, including Simonds and Parraga's sleep questionnaire and the Strength and Difficulties Questionnaire, were employed.
- Data were collected from 64 children (39 male, mean age 10 years 7 months) with NF1.
Main Results:
- Children with NF1 showed significantly higher scores in borderline/abnormal ranges for peer problems, hyperactivity, emotional symptoms, and conduct disorder compared to general population norms.
- Parasomnias, such as sleepwalking and sleep terrors, were more frequent in the NF1 group.
- Within the NF1 cohort, conduct problems, hyperactivity, and emotional issues were significantly associated with frequent sleep disturbance.
Conclusions:
- NF1 is strongly associated with a high proportion of children experiencing sleep and behavioral problems.
- Conditions like attention-deficit-hyperactivity disorder may be underdiagnosed in children with NF1.
- Implementing simple screening tools in clinical practice could aid in identifying these issues early.
Abstract:
The objective of this study was to explore the behaviour, including sleep patterns, of children with neurofibromatosis type 1 (NF1). For this purpose we designed a cross-sectional descriptive survey conducted by postal enquiry with telephone follow-up. Simonds and Parraga's sleep questionnaire and the Strength and Difficulties Questionnaire were used to screen sleep patterns and behaviours respectively. Information was obtained for 64 children (39 male, mean age 10 y 7 mo, SD 4 y 1 mo, range 3 to 18 y; 57% response rate). There were increased numbers of children with NF1 achieving scores in the borderline and abnormal range for ratings of peer problems (p < 0.001), hyperactivity (p < 0.001), emotional symptoms (p < 0.001), and conduct disorder (p < 0.05). Total difficulties score was also high (p < 0.001) in the NF1 group. Only one group of sleep problems, parasomnias (in particular sleepwalking and sleep terrors), had a higher occurrence (p < or = 0.05) in the NF1 group than in the general population. Within the NF1 group, conduct problems (p < or = 0.05), hyperactivity (p < or = 0.01), emotional problems (p < or = 0.01), and the total difficulties score (p < or = 0.01) were all significantly higher in children with frequent sleep disturbance. We conclude that NF1 is associated with sleep and behavioural problems in a high proportion of children. Psychiatric conditions, e.g. attention-deficit-hyperactivity disorder, may be underdiagnosed on a large scale in children with NF1, and the use of simple screening tools in clinical settings may prove beneficial.
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