title: Anomalous sylvian fissure morphology in Williams syndrome.
abstract: The unusual sensitivity and attraction to auditory stimuli in people with Williams syndrome (WS) has been hypothesized to be the consequence of atypical development of brain regions surrounding the Sylvian fissure. Planum temporale surface area, which is determined in part by Sylvian fissure patterning, was examined in 42 WS and 40 control participants to determine if anomalous Sylvian fissure morphology is present in WS. WS participants had significantly reduced leftward asymmetry of the planum temporale compared to control participants, due to a significant expansion in the size of the right planum temporale. The increased right planum temporale size was largely due to WS participants (24%) who had a right hemisphere Sylvian fissure that coursed horizontally and failed to ascend into the parietal lobe. This sulcal pattern is unusual in the right hemisphere and is more commonly found in the left hemisphere of typically developing individuals. There were no control participants with this type of right hemisphere Sylvian fissure pattern. The right hemisphere Sylvian fissure sulcal patterns were also related to a measure of cortical complexity and the amount of right hemisphere occipital lobe volume, suggesting that intrinsic genetic influences leading to anomalous visual system development in WS have widespread influences on cortical morphology that are similar in manner to extrinsic embryonic visual system lesions.
authors.affiliation: Department of Otolaryngology-Head and Neck Surgery, Medical University of South Carolina, Charleston, 29425, USA. eckert@musc.edu
authors.forename: Mark A
authors.initials: MA
authors.lastname: Eckert
authors.forename: Albert M
authors.initials: AM
authors.lastname: Galaburda
authors.forename: Asya
authors.initials: A
authors.lastname: Karchemskiy
authors.forename: Alyssa
authors.initials: A
authors.lastname: Liang
authors.forename: Paul
authors.initials: P
authors.lastname: Thompson
authors.forename: Rebecca A
authors.initials: RA
authors.lastname: Dutton
authors.forename: Agatha D
authors.initials: AD
authors.lastname: Lee
authors.forename: Ursula
authors.initials: U
authors.lastname: Bellugi
authors.forename: Julie R
authors.initials: JR
authors.lastname: Korenberg
authors.forename: Debra
authors.initials: D
authors.lastname: Mills
authors.forename: Fredric E
authors.initials: FE
authors.lastname: Rose
authors.forename: Allan L
authors.initials: AL
authors.lastname: Reiss
mesh_terms: D000328:Adult; D002540:Cerebral Cortex / Q000473:pathology*; D005260:Female; D017709:Fractals; D007839:Functional Laterality / Q000502:physiology; D006801:Humans; D007091:Image Processing, Computer-Assisted; D017404:In Situ Hybridization, Fluorescence; D008279:Magnetic Resonance Imaging; D008297:Male; D009778:Occipital Lobe / Q000473:pathology; D013702:Temporal Lobe / Q000473:pathology; D018980:Williams Syndrome / Q000473:pathology*