Academic achievement in Noonan Syndrome, spectrum, and related disorders

Abstract

Abstract

The present study examines (1) the prevalence of learning difficulties; (2) correlates and predictors of academic achievement; and (3) explores IQ minus academic achievement distributions across different Noonan Syndrome spectrum and related genetic variants (NSSD). IQ, academic achievement, executive function, and attention performance-based and/or caregiver-rated measures were completed across two samples of youth with Noonan Syndrome, spectrum, and related disorders (NSSD; n = 123) and unaffected youth (n = 172). Youth with NSSD had significantly lower scores on all academic achievement measures than the unaffected group with large effects. Executive skills (e.g., processing speed, working memory, inhibitory control) were significant predictors of academic skills in addition to whether a participant has NSSD, and together they accounted for ~1/3–1/2 of the variability in academic scores. Exploratory analyses revealed significantly skewed IQ-Achievement distributions in the SOS1 and Other Variant groups, but not PTPN11 group, as compared to the unaffected youth. Between one fifth and one half of youth with NSSD are estimated to meet criteria for Specific Learning Disorder. Clinicians and educational settings should conduct rigorous, frequent screening of academic difficulties of youth with NSSD to intervene as early as feasible with appropriate services. Executive skills, especially working memory, processing speed, and cognitive flexibility, play a contributory role to difficulties with academics in NSSD, and should also be supported with targeted interventions.