CAJ | 학술논문

目的利用基于体素的形态测量学(VBM)方法,探测单体型特纳综合征(TS)患儿脑灰、白质体积的异常.方法选取9例染色体核型为45XO的单体型TS患儿(TS组)和20名正常女童(对照组),进行了韦氏智力测量和3.0TMR扫描.TS组和对照组的全脑高分辨率T1WI采用统计参数图SPM 8软件包进行VBM分析,以全脑灰、白质总体积为协变量,以协方差分析比较单体型TS患儿和对照组之间全脑灰、白质体积的差别,采用功能神经图像分析软件包(AFNI)中的蒙特卡洛模拟方法(即AlphaSim方法)进行簇水平上的多重比较校正.结果 TS组患儿韦氏总智商值为81±13,而对照组儿童韦氏总智商值为109±16,两组儿童智商差异具有统计学意义(t=-4.70,P＜0.05).与对照组比较,单体型TS患儿右侧顶上小叶、中央后回、楔叶、楔前叶、距状皮层,左侧枕叶中下部灰质体积减小,差异有统计学意义(统计区体素数目分别为631、525、520个,t值分别为3.95、3.50、3.36,P值均＜0.05)；双侧辅助运动区、额上回内侧,右侧扣带回中部,左侧颞上、中、下回,左侧额上、中、下回,左侧小脑,左侧海马、海马旁回,左侧梭状回灰质体积增加,差异有统计学意义(统计区体素数目分别为2082、974、1708、588、579个,t值分别为5.45、4.59、4.40、4.29、3.55,P值均＜0.05).白质体积比较的结果表明,单体型TS患儿左侧中央后回及顶下小叶的白质体积较对照组减小,差异具有统计学意义(体素数目957,t=5.85,P＜0.05).结论 TS患儿存在脑灰、白质发育的异常,该异常改变可能是单体型TS患儿认知功能障碍的神经病理学基础.
목적 이용기우체소적형태측량학(VBM)방법,탐측단체형특납종합정(TS)환인뇌회、백질체적적이상.방법 선취9례염색체핵형위45XO적단체형TS환인(TS조)화20명정상녀동(대조조),진행료위씨지력측량화3.0TMR소묘.TS조화대조조적전뇌고분변솔T1WI채용통계삼수도SPM 8연건포진행VBM분석,이전뇌회、백질총체적위협변량,이협방차분석비교단체형TS환인화대조조지간전뇌회、백질체적적차별,채용공능신경도상분석연건포(AFNI)중적몽특잡락모의방법(즉AlphaSim방법)진행족수평상적다중비교교정.결과 TS조환인위씨총지상치위81±13,이대조조인동위씨총지상치위109±16,량조인동지상차이구유통계학의의(t=-4.70,P＜0.05).여대조조비교,단체형TS환인우측정상소협、중앙후회、설협、설전협、거상피층,좌측침협중하부회질체적감소,차이유통계학의의(통계구체소수목분별위631、525、520개,t치분별위3.95、3.50、3.36,P치균＜0.05)；쌍측보조운동구、액상회내측,우측구대회중부,좌측섭상、중、하회,좌측액상、중、하회,좌측소뇌,좌측해마、해마방회,좌측사상회회질체적증가,차이유통계학의의(통계구체소수목분별위2082、974、1708、588、579개,t치분별위5.45、4.59、4.40、4.29、3.55,P치균＜0.05).백질체적비교적결과표명,단체형TS환인좌측중앙후회급정하소협적백질체적교대조조감소,차이구유통계학의의(체소수목957,t=5.85,P＜0.05).결론 TS환인존재뇌회、백질발육적이상,해이상개변가능시단체형TS환인인지공능장애적신경병이학기출.
Objective To detect the structural changes of cerebral gray and white matter in children of monosomy Turner syndrome (TS) by using voxel-based morphometry (VBM).Methods Nine children 45XO karyotype TS and 20 age-matched control girls were recruited in this study.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 data were analyzed by VBM based on SPM 8 to compare the volume of gray and white matter between the TS children and normal controls by using covariance analysis.Results The IQ of TS children was 81 ± 13,and the IQ of the controls was 109 ± 16.Statistical analysis revealed significant difference of IQ between the two groups (t =-4.70,P ＜ 0.05).Compared with normal controls,TS children showed significantly decreased volume (numbers of voxel in clusters were 631,525,520,t =3.95,3.50,3.36,P ＜ 0.05,FWE-corrected) in the gray matter of the right superior parietal lobule,postcentral gyrus,precuneus lobule,calcarine,cuneus cortices,as well as 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 enlarged in TS children (numbers of voxel in clusters were 2082,974,1708,588,579,t =5.45,4.59,4.40,4.29,3.55,P ＜0.05,FWE-corrected).White matter regions in the left postcentral gyrus and inferior parietal lobule showed significantly reduced volume (voxel number 957,t =5.85,P ＜ 0.05,FWE-corrected).Conclusion Children with monosomy TS show abnormal gray and white matter volumes in some brain regions,which may be involved in the neuropathology of Turner syndrome.