Wechsler intelligence test and a whole-brain matter analysis in children with Turner syndrome of different karyotypes

Wechsler intelligence test and a whole-brain matter analysis in children with Turner syndrome of different karyotypes
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不同核型特纳综合征患儿韦克斯勒智力测试及全脑物质分析

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发表时间:
2012
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通讯作者:
Gong Gao
Gong Gao
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作者:
Gong Gao

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目的应用韦氏智力测验(Wechsler intelligence test)和基于体素的形态计量学(voxel-based morphometry,VBM)技术检测不同染色体核型的Turner综合征(Turner syndrome,TS)患儿的认知表型及脑灰质和白色结构的变化。对TS患儿和正常女童进行高分辨磁共振成像,收集全脑结构数据,用SPM 8软件进行VBM分析,对TS患儿和正常对照组的45 X和非45 X核型的灰质和白色体积进行协方差分析。经统计学分析,TS组智商差异有显著性45 X核型TS组在言语理解、知觉推理、工作记忆和加工速度方面差异有统计学意义(P0.05);非45 X核型TS组在知觉推理、加工速度上有显著性差异TS组与对照组在言语理解能力上差异有统计学意义(P0.05与正常对照组相比,45 X染色体核型TS儿童的体积明显减小(P0.05右侧上级顶叶、中央后回、楔前叶、距状核、楔叶皮质、左侧枕中叶和枕下叶灰质中,与对照组相比,TS患儿双侧补充运动区、内侧上级额叶、右侧扣带回中、左侧上级、颞中、颞下回体积增大,左侧梭状回、左侧海马旁、左侧海马、左侧小脑体积增大(P0.05,FWE校正);非45 X染色体核型的TS儿童表现出显著的体积减小(P0.05,FWE校正)在海马旁、梭形、齿状回、海马、壳核、上级颞回、杏仁核、尾状核、丘脑、在白色物质区,45 X染色体核型TS儿童的左侧中央后回和顶下小叶的体积显著减小,(P0.05,FWE校正)。在非45 X核型TS儿童中,(P0.05,结论TS儿童智商降低,并伴有非智力因素。语言障碍。2单体性TS女孩可能存在语言障碍。3某些脑区异常的灰色和白色物质体积可能与特纳综合征的神经病理学有关。
Objective To detect the cognitive phenotype and structural changes of cerebral gray and white matter in children with Turner syndrome(TS) of different chromosome karyotype by using Wechsler intelligence test and voxel-based morphometry(VBM).Methods Nine 45X karyotype and 11 non 45X karyotype TS girls and 20 age-matched control girls were recruited.Wechsler intelligence scale for children was used to obtain their intelligence quotients(IQ).High-resolution magnetic MR imaging was performed in TS children and control girls to collect the whole brain structural data.The analysis of VBM was performed with SPM 8 to compare the volume of gray and white matter between the 45X karyotype and non 45X karyotype of TS children and normal controls by covariance analysis.Results Compared with normal controls,the statistical analysis revealed significant difference of IQ in TS group(P0.05);significant difference was observed in 45X karyotype TS group in verbal comprehension,perceptual reasoning,working memory and processing speed;and there was significant difference in non 45X karyotype TS group in perceptual reasoning,processing speed(P0.05).There was significant difference in verbal comprehension between TS groups(P0.05).Compared with normal controls,45X karyotype TS children showed significantly decreased volume(P0.05) in the gray matter of the right superior parietal lobule,postcentral gyrus,precuneus lobule,calcarine,cuneus cortices,and the left middle and inferior occipital lobe.However,the volume of the bilateral supplemental motor area and the medial superior frontal lobes,the right middle cingulum,the left superior,middle,and inferior temporal gyri were increased in the TS children,compared to the controls.The left fusiform,the left parahippocampus,the left hippocampus and the left cerebellum were also significantly enlarged in TS children(P0.05,FWE-corrected);non 45X karyotype TS children showed significantly decreased volume(P0.05,FWE-corrected) in the gray matter of both parahippocampus,fusiform,insula,hippocampus,putamen,superior temporal gyrus,amygdala,caudate,thalamus,cerebellum and the left lingual.In the white matter regions there was significantly reduced volume in the left postcentral gyrus and inferior parietal lobule,in 45X karyotype TS children(P0.05,FWE-corrected).In non 45X karyotype TS children,significantly decreased volume(P0.05,FWE-corrected) was seen in the left superior parietal lobule and precuneus lobule and the superior occipital lobule.Conclusions The IQ of TS children is decreased and accompanied by non-verbal obstacles.There may be verbal obstacles with monosomy TS girls.Abnormal gray and white matter volumes in some brain regions may be involved in the neuropathology of Turner syndrome.