Aberrant Frontal Lobe Maturation in Adolescents with Fragile X Syndrome is Related to Delayed Cognitive Maturation

作者：Bray Signe; Hirt Melissa; Jo Booil; Hall Scott S; Lightbody Amy A; Walter Elizabeth; Chen Kelly; Patnaik Swetapadma; Reiss Allan L^*

来源：Biological Psychiatry, 2011, 70(9): 852-858.

DOI：10.1016/j.biopsych.2011.05.038

• Adolescence
• brain development
• fragile X syndrome
• longitudinal
• MRI
• prefrontal cortex

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.

• 出版日期2011-11-1