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. 2022 Feb;188(2):488-497.
doi: 10.1002/ajmg.a.62544. Epub 2021 Nov 3.

Adolescents and young adults with neurofibromatosis type 1: A descriptive study of adaptive functioning

Kari L Struemph  1 , Adreanna T M Watts  2 , Pamela L Wolters  2 , Mary Anne Tamula  1 , Andrea Baldwin  1 , Brigitte Widemann  2 , Staci Martin  2
Affiliations

Adolescents and young adults with neurofibromatosis type 1: A descriptive study of adaptive functioning

Kari L Struemph et al. Am J Med Genet A. 2022 Feb.

Abstract

Neurofibromatosis type 1 (NF1) is a genetic syndrome affecting about 1 in 3500 individuals; many of those affected have plexiform neurofibroma (pNF) tumors and associated symptoms and complications. Furthermore, learning and attention problems, as well as deficits in adaptive functioning, are common, often beginning in early childhood. This study aimed to describe adaptive functioning and to examine relationships between adaptive functioning and cognitive and academic variables and level of independence among adolescents and young adults (AYA) with NF1 and pNF tumors. Fifty-five AYA aged 16-31 years participated in a series of neuropsychological evaluations while parents completed the Vineland Adaptive Behavior Scales (VABS-II) as part of a larger natural history study. Over one-third (35%) of AYA were neither in school nor employed. Mean VABS-II daily living and socialization scores were low average while mean Verbal and Performance IQ scores were average. VABS-II scores were positively correlated with processing speed, executive functioning, and working memory scores. Verbal IQ was the only significant predictor of work/school status. Identification of the correlates and predictors of adaptive functioning and life achievement can help guide healthcare providers with the early identification of risk factors and possible areas for intervention.

Keywords: NF1; adaptive functioning; adolescents and young adults (AYA).

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References

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