Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP).

Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP). Research Abstract Details 

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Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP). Abstract Text:

Doron Gothelf,Joyce A Furfaro,Fumiko Hoeft,Mark A Eckert,Scott S Hall,Ruth O'Hara,Heather W Erba,Jessica Ringel,Kiralee M Hayashi,Swetapadma Patnaik,Brenda Golianu,Helena C Kraemer,Paul M Thompson,Joseph Piven,Allan L Reiss,Doron Gothelf,Joyce A Furfaro,Fumiko Hoeft,Mark A Eckert,Scott S Hall,Ruth O'Hara,Heather W Erba,Jessica Ringel,Kiralee M Hayashi,Swetapadma Patnaik,Brenda Golianu,Helena C Kraemer,Paul M Thompson,Joseph Piven,Allan L Reiss,

OBJECTIVE: To determine how neuroanatomic variation in children and adolescents with fragile X syndrome is linked to reduced levels of the fragile X mental retardation-1 protein and to aberrant cognition and behavior. METHODS: This study included 84 children and adolescents with the fragile X full mutation and 72 typically developing control subjects matched for age and sex. Brain morphology was assessed with volumetric, voxel-based, and surface-based modeling approaches. Intelligence quotient was evaluated with standard cognitive testing, whereas abnormal behaviors were measured with the Autism Behavior Checklist and the Aberrant Behavior Checklist. RESULTS: Significantly increased size of the caudate nucleus and decreased size of the posterior cerebellar vermis, amygdala, and superior temporal gyrus were present in the fragile X group. Subjects with fragile X also demonstrated an abnormal profile of cortical lobe volumes. A receiver operating characteristic analysis identified the combination of a large caudate with small posterior cerebellar vermis, amygdala, and superior temporal gyrus as distinguishing children with fragile X from control subjects with a high level of sensitivity and specificity. Large caudate and small posterior cerebellar vermis were associated with lower fragile X mental retardation protein levels and more pronounced cognitive deficits and aberrant behaviors. INTERPRETATION: Abnormal development of specific brain regions characterizes a neuroanatomic phenotype associated with fragile X syndrome and may mediate the effects of FMR1 gene mutations on the cognitive and behavioral features of the disorder. Fragile X syndrome provides a model for elucidating critical linkages among gene, brain, and cognition in children with serious neurodevelopmental disorders. Ann Neurol 2007.

Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP). Publishing Authors By Initials

D Gothelf,JA Furfaro,F Hoeft,MA Eckert,SS Hall,R O'Hara,HW Erba,J Ringel,KM Hayashi,S Patnaik,B Golianu,HC Kraemer,PM Thompson,J Piven,AL Reiss,D Gothelf,JA Furfaro,F Hoeft,MA Eckert,SS Hall,R O'Hara,HW Erba,J Ringel,KM Hayashi,S Patnaik,B Golianu,HC Kraemer,PM Thompson,J Piven,AL Reiss,

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Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP). Journal Published:

PUBLICATION TYPE: Journal Article

Journal: Annals of neurology

VOLUME: 63

Page Numbers: 40-51

Journal Abbreviation: Ann. Neurol.

ISSN: 1531-8249

DAY: 4

MONTH: Jan

YEAR: 2008

Neuroanatomy of fragile X syndrome is associated with aberrant behavior and the fragile X mental retardation protein (FMRP). Information

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LANGUAGE: eng

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AFFILIATION: Behavioral Neurogenetics Center, Child Psychiatry Department, Schneider Children's Medical Center of Israel, Petah Tiqwa.

Country: United States

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MEDLINETA: Ann Neurol

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