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Biol Psychiatry. 2011 Nov 1;70(9):852-8. doi: 10.1016/j.biopsych.2011.05.038. Epub 2011 Jul 29.

Aberrant frontal lobe maturation in adolescents with fragile X syndrome is related to delayed cognitive maturation.

Author information

1
Department of Psychiatry, Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada.

Abstract

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.

PMID:
21802660
PMCID:
PMC3191299
DOI:
10.1016/j.biopsych.2011.05.038
[Indexed for MEDLINE]
Free PMC Article

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