PTOTSLPRCTDevelopmental medicine and child neurology2020

Predictors of cognitive development in children with neurofibromatosis type 1 and plexiform neurofibromas.

Yang Hou, Taryn Allen, Pamela L Wolters and 7 others

PMID 32052421

WHAT IT FOUND

Children with NF1 and plexiform neurofibromas show widening gaps in math, writing, and executive function over time.

Those with parents who also have NF1 or lower parental education decline fastest. Regular school-based assessments and targeted support are critical.

Key findings

01Math, writing, inhibitory control, and working memory scores significantly declined over time relative to norms, widening the gap with peers.

02Children whose parents also had NF1 experienced significant declines in performance IQ, reading, writing, attention, and working memory, unlike children without familial NF1.

03Lower parental education predicted a faster decline in math scores and higher baseline deficits in IQ and academic achievement.

STILL TO COME

How it was doneWhat they foundWhat it means for PTsWhat it means for OTsWhat it means for SLPs

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What it does not show

The study focused only on children with NF1 who also had plexiform neurofibromas, which affects about half of the NF1 population, so results may not apply to all children with NF1. There was considerable missing data at later time points, with only 39% of participants assessed at the third time point. The sample size was small for a longitudinal study, limiting the power to detect subtle effects. Cognitive scores were compared against cross-sectional normative data rather than a matched typically developing control group followed longitudinally.

Declared interests

Research supported by the N.I.H. Intramural program. No specific commercial conflicts of interest were declared in the provided text.

The easy way to misread this

Do not assume that physical disease burden (number of NF1 complications) predicts cognitive decline. The study found no relationship between the total number of complications and cognitive outcomes. Instead, demographic and familial factors (parental NF1 status and education) were the significant predictors of worsening cognitive trajectories.

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