Association between cerebral shape and social use of language in Williams syndrome.
Association between cerebral shape and social use of language in Williams syndrome.
复制标题
威廉姆斯综合征大脑形状与语言社会使用之间的关联。
DOI：
10.1002/ajmg.a.32507
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发表时间：
2008-11-01
影响因子：
2
通讯作者：
Reiss, Allan L.
中科院分区：
文献类型：
--
作者：
Gothelf, Doron;Searcy, Yvonne M.;Reilly, Judy;Lai, Philip T.;Lanre-Amos, Tope;Mills, Debra;Korenberg, Julie R.;Galaburda, Albert;Bellugi, Ursula;Reiss, Allan L.
威廉姆斯综合征是一种由 7q11.23 区半合子微缺失引起的神经遗传性疾病。其特点是大脑发育异常以及独特的认知和行为特征。我们试图利用信号检测方法来识别与威廉姆斯综合征最密切相关的神经解剖学异常。一旦确定了质量接受者操作特征曲线，我们假设那些区分威廉姆斯综合症患者和对照组的大脑区域将与患有这种疾病的个体的社会表型相关。研究人员对 39 名患有威廉姆斯综合征的青少年和年轻人以及 40 名年龄和性别相匹配的典型发育对照进行了研究。质量接收者操作特征曲线确定了扩大的腹侧前前额叶皮层和胼胝体大弯曲角的组合，以区分威廉姆斯综合征和对照，敏感性为 85.4%，特异性为 75.0%。在威廉姆斯综合征组中，弯曲角度与腹侧前额叶皮层大小显着相关，但与其他大脑形态测量指标无关。威廉姆斯综合征受试者的腹前前额叶大小与评估社交和情感语言使用的叙事任务中社交参与设备的使用呈正相关。我们的研究结果表明，腹侧前额叶皮层的异常形态是导致大脑皮层异常大小和形状以及威廉姆斯综合症患者典型的社交情感语言使用的关键因素。
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 those brain regions distinguishing subjects with Williams syndrome from controls would be linked to the social phenotype of individuals with this disorder. Thirty-nine 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 social-affective language use typical of individuals with Williams Syndrome.
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影响因子：
2.6
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