Written language achievement in children and adolescents with neurofibromatosis type 1 and Plexiform Neurofibromas

ABSTRACT

Neurofibromatosis type 1 (NF1) is associated with below average writing achievement. However, little is known about specific aspects of written language impacted by NF1, changes in writing over time, and associations between cognitive aspects of the NF1 phenotype and writing. At three timepoints over six years, children with NF1 and plexiform neurofibromas (PNs) completed Woodcock-Johnson tests of writing mechanics (Spelling, Punctuation & Capitalization, handwriting), written expression of ideas (Writing Samples), writing speed (Writing Fluency), and tests of general cognitive ability, executive function, memory, and attention. Children (N = 76, mean age = 12.8 ± 3.4 years) completed at least one baseline writing subtest. Overall writing scores were in the Average range (M = 93.4, SD = 17.4), but lower than population norms (p = 0.002). Scores were highest on Writing Samples (M = 95.2, SD = 17.3), and lowest for Punctuation & Capitalization (M = 87.9, SD = 18.8, p = 0.034). Writing scores were mostly stable over time. Nonverbal reasoning was related to some tests of writing mechanics and written expression of ideas. Short-term memory and inattention explained additional variance in Writing Samples and Spelling. Poor handwriting was associated with writing content beyond the impact of cognitive factors. Children with NF1 and PNs may benefit from early screening and writing support. Interventions should address the contribution of both cognitive and handwriting difficulties in written language.

KEYWORDS:

• Academic achievement
• executive function
• neurofibromatosis type 1
• plexiform neurofibroma
• written language disability

Acknowledgments

This research was supported by the Intramural Research Program of the National Institutes of Health, National Cancer Institute. We thank Dr. Yang Hou (Florida State University) for her assistance with the statistical analysis plan. We would like to acknowledge the many staff members who helped to conduct this study, as well as thank all the parents and children who participated in the study.

Disclosure statement

No potential conflict of interest was reported by the author(s).

Additional information

Funding

The work was supported by the Intramural Research Program of the National Institutes of Health, National Cancer Institute, Center for Cancer Research, Pediatric Oncology Branch.