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Adaptive Behavior in Young Children with Neurofibromatosis 1

NF Midwest has been funding cognitive studies of young children with NF for the last two years. These studies have been lead by Bonnie Klein-Tasman at the University of Wisconsin-Milwaukee. This past fall we saw the results of these studies with the publications of two journal articles by Dr. Klein-Tasman and her team.

As an added benefit, these studies that NF Midwest funded also provided free neuropsychological testing to 65 children with neurofibromatosis type 1 in the Midwest.

Check out the journal articles in the International Journal of Pediatrics and in the Journal of International Neuropsychological Society. Also, below you will find a “lay” summary of these articles.

Adaptive Behavior in Young Children with Neurofibromatosis 1

International Journal of Pediatrics

Bonita P. Klein-Tasman, Alina M. Colon, Natalie Brei, Faye van der Fluit, Christina L. Casnar, Kelly M. Janke, Donald Basel, Dawn H. Siegel, and Jasmine A. Walker

 In this study, we looked at the everyday adaptive functioning of children ages 3 through 8 with NF1. Adaptive behavior includes children’s communication skills in everyday contexts, their ability to understand instructions from others, dressing, toothbrushing, and other self-care skills, skills in helping out around the house, understanding of time and money concepts, and everyday fine and gross motor skills. Fine motor skills include unwrapping wrappers, drawing, and writing. Gross motor skills include walking on a narrow surface, catching a ball, jumping rope. Parents are interviewed about what their children can do independently, without help or supervision, and without being reminded. Partial “credit” is given when the child needs to be reminded.

We found that young children with NF1 (as a group) showed less well-developed everyday adaptive behavior than did same-aged peers without NF1, and more children with NF1 than children without NF1 showed motor skill and language expression skill delays. We also found that overall everyday behavior described by parents was strongly related to structured assessment measures of verbal and nonverbal intellectual abilities. Children with stronger intellectual abilities showed stronger everyday adaptive functioning (and vice versa). Even once cognitive abilities were taken into account, however, motor skills were weaker for the children with NF1 than for the same-aged peers without NF1. It is important to keep in mind that, like in older children with NF1, there was a lot of variability in everyday adaptive behavior of children with NF1. While more children with NF1 showed delays, most young children with NF1 (around 75%) have adaptive behavior that falls in the solidly average range and is not delayed. We will need to do more research to see whether these early motor difficulties are related to challenges for children with NF1 later in life.

Klein-Tasman, B. P., Colon, A. M., Brei, N., van der Fluit, F., Casnar, C. L., Janke, K. M., Basel, D., Siegel, D. H. & Walker, J. A. (2013). Adaptive functioning in young children with neurofibromatosis – 1. International Journal of Pediatrics, Article ID 690432, 7 pages. doi:10.1155/2013/690432.

PDF Version

Cognitive and Psychosocial Phenotype of Young Children with Neurofibromatosis-1

Journal of the International Neuropsychological Society

Bonita P. Klein-Tasman, Kelly M. Janke, Wen Luo, Christy L. Casnar, Scott J. Hunter,

James Tonsgard, Pamela Trapane, Faye van der Fluit, & Lorri A. Kais

In this study we examined the cognitive and psychosocial functioning of children with NF1 ages 3 through 6. Cognitive functioning includes the child’s vocabulary, understanding of language, ability to copy simple line drawings, ability to put together visual patterns with blocks, understanding of similarities between concepts, and understanding of visual patterns and was assessed in individual assessments with the child. Psychosocial functioning includes attention, activity level, anxiety, social skills, and everyday communication, and was assessed based on a parent questionnaire. We wanted to see whether the cognitive and psychosocial difficulties that are seen in older children with NF1 can also be observed in the preschool years. We found that children with NF1 (as a group) showed more cognitive difficulties than did same-aged peers, which is a similar finding to that seen in older children. About half of the children with NF1 showed a cognitive difficulty in at least one area. However, there was no consistent pattern of strengths and weaknesses for children with NF1; different children had different areas of difficulty. Based on parents’ responses to the questionnaires, the young children with NF1 in this sample show similar psychosocial functioning to did same-aged peers in most areas, but showed more difficulties with communicating their needs (called “functional communication”). Children with more verbal difficulties also showed more communication difficulties, social difficulties, and attention problems, which also fits with research findings in older children with NF1. These findings suggest that it is likely useful for young children with NF1 to undergo cognitive assessments, as cognitive difficulties may be a sign of likely everyday challenges.

Klein-Tasman, B. P., Janke, K. M., Luo, W., Casnar, C. L., Hunter, S. J., Tonsgard, J., Trapane, P., van der Fluit, F. & Kais, L. A. (2014). Cognitive and psychosocial phenotype of young children with neurofibromatosis-1. Journal of the International Neuropsychological Society, 20, 88-98. doi:10.1017/S1355617713001227

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Comments (1)

  • Iam from the Newcastle in Uk I was diagnose at age 40 years old with

    can please send me so information regarding neurofibromatosis posts and blogs please.

    back home in Newcastle UK i attend the Gentic Centre of Medicine they are doing excellent job treating me following up treatment for me regarding my NF1 I had plexiform remove from back of my head recently at the local hospital RVI

    I may send you list of all my medical problems since this start to flare up back from 1992 up to current day

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