Association between Cerebral
Shape and Social Use of Language
in Williams Syndrome
-
1
The Salk Institute for Biological Studies, Laboratory for Cognitive Neuroscience, United States
Williams syndrome is a neurogenetic disorder resulting from a hemizygous microdeletion at band 7q11.23. It is characterized by aberrant development of the brain and a unique profile of cognitive and behavioral features. We sought to identify the neuroanatomical abnormalities that are most strongly associated with Williams syndrome employing signal detection methodology. Once identified with a Quality Receiver Operating Characteristic Curve, we hypothesized that brain regions distinguishing subjects with Williams syndrome from controls would be linked to the social phenotype of individuals with this disorder. Forty-one adolescents and young adults with Williams syndrome and 40 typically developing controls matched for age and gender were studied. The Quality Receiver Operating Characteristic Curve identified a combination of an enlarged ventral anterior prefrontal cortex and large bending angle of the corpus callosum to distinguish between Williams syndrome and controls with a sensitivity of 85.4% and specificity of 75.0%. Within the Williams syndrome group, bending angle significantly correlated with ventral anterior prefrontal cortex size but not with other morphometric brain measures. Ventral anterior prefrontal size in subjects with Williams syndrome was positively associated with the use of social engagement devices in a narrative task assessing the use of social and affective language. Our findings suggest that aberrant morphology of the ventral anterior prefrontal cortex is a pivotal contributing factor to the abnormal size and shape of the cerebral cortex and to the sociallinguistic phenotype of individuals with Williams syndrome.
Conference:
12th International Professional Conference on Williams Syndrome, Garden Grove,CA, United States, 13 Jul - 14 Jul, 2008.
Presentation Type:
Poster Presentation
Topic:
Multidisciplinary Poster Session
Citation:
Searcy
Y,
Gothelf
D,
Reilly
J,
Bellugi
U,
Lanre-Amos
T,
Mills
DL,
Korenberg
JR,
Galaburda
A and
Reiss
AL
(2009). Association between Cerebral
Shape and Social Use of Language
in Williams Syndrome.
Conference Abstract:
12th International Professional Conference on Williams Syndrome.
doi: 10.3389/conf.neuro.09.2009.07.055
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Received:
04 May 2009;
Published Online:
04 May 2009.
*
Correspondence:
Y. Searcy, The Salk Institute for Biological Studies, Laboratory for Cognitive Neuroscience, La Jolla, United States, searcy@salk.edu