BACKGROUND: Fragile X syndrome (FXS) is the most common known heritable cause of intellectual disability. Prior studies in FXS have observed a plateau in cognitive and adaptive behavioral development in early adolescence, suggesting that brain development in FXS may diverge from typical development during this period. METHODS: In this study, we examined adolescent brain development using structural magnetic resonance imaging data acquired from 59 individuals with FXS and 83 typically developing control subjects aged 9 to 22, a subset of whom were followed up longitudinally (1-5 years; typically developing: 17, FXS: 19). Regional volumes were modeled to obtain estimates of age-related change. RESULTS: We found that while structures such as the caudate showed consistent volume differences from control subjects across adolescence, prefrontal cortex (PFC) gyri showed significantly aberrant maturation. Furthermore, we found that PFC-related measures of cognitive functioning followed a similarly aberrant developmental trajectory in FXS. CONCLUSIONS: Our findings suggest that aberrant maturation of the PFC during adolescence may contribute to persistent or increasing intellectual deficits in FXS.
BACKGROUND:Fragile X syndrome (FXS) is the most common known heritable cause of intellectual disability. Prior studies in FXS have observed a plateau in cognitive and adaptive behavioral development in early adolescence, suggesting that brain development in FXS may diverge from typical development during this period. METHODS: In this study, we examined adolescent brain development using structural magnetic resonance imaging data acquired from 59 individuals with FXS and 83 typically developing control subjects aged 9 to 22, a subset of whom were followed up longitudinally (1-5 years; typically developing: 17, FXS: 19). Regional volumes were modeled to obtain estimates of age-related change. RESULTS: We found that while structures such as the caudate showed consistent volume differences from control subjects across adolescence, prefrontal cortex (PFC) gyri showed significantly aberrant maturation. Furthermore, we found that PFC-related measures of cognitive functioning followed a similarly aberrant developmental trajectory in FXS. CONCLUSIONS: Our findings suggest that aberrant maturation of the PFC during adolescence may contribute to persistent or increasing intellectual deficits in FXS.
Authors: E M Dykens; R M Hodapp; S Ort; B Finucane; L R Shapiro; J F Leckman Journal: J Am Acad Child Adolesc Psychiatry Date: 1989-05 Impact factor: 8.829
Authors: Nitin Gogtay; Jay N Giedd; Leslie Lusk; Kiralee M Hayashi; Deanna Greenstein; A Catherine Vaituzis; Tom F Nugent; David H Herman; Liv S Clasen; Arthur W Toga; Judith L Rapoport; Paul M Thompson Journal: Proc Natl Acad Sci U S A Date: 2004-05-17 Impact factor: 11.205
Authors: F Xavier Castellanos; Patti P Lee; Wendy Sharp; Neal O Jeffries; Deanna K Greenstein; Liv S Clasen; Jonathan D Blumenthal; Regina S James; Christen L Ebens; James M Walter; Alex Zijdenbos; Alan C Evans; Jay N Giedd; Judith L Rapoport Journal: JAMA Date: 2002-10-09 Impact factor: 56.272
Authors: Jennifer L Bruno; David Romano; Paul Mazaika; Amy A Lightbody; Heather Cody Hazlett; Joseph Piven; Allan L Reiss Journal: Proc Natl Acad Sci U S A Date: 2017-09-18 Impact factor: 11.205
Authors: Elizabeth Berry-Kravis; David Hessl; Leonard Abbeduto; Allan L Reiss; Andrea Beckel-Mitchener; Tiina K Urv Journal: J Dev Behav Pediatr Date: 2013-09 Impact factor: 2.225
Authors: Jun Yi Wang; David Hessl; Christine Iwahashi; Katherine Cheung; Andrea Schneider; Randi J Hagerman; Paul J Hagerman; Susan M Rivera Journal: Neuroimage Date: 2012-10-12 Impact factor: 6.556
Authors: Da Shi; Su Xu; Jaylyn Waddell; Susanna Scafidi; Steven Roys; Rao P Gullapalli; Mary C McKenna Journal: J Neurochem Date: 2012-11-07 Impact factor: 5.372