Turner Syndrome In Patients With Clinical And Experimental Research Of Brain Function

Object ive1. To observe the abnormalities of brain gray matter volume and neural and cognitive function with different karyotype of Turner Syndrome in children2. To observe the changes of ovarian hormone levels wi th ovar iectornized model mouse and explore the changes of the morphological and the5-HT, BDNF neurons in brain.3. To Observe the protection of estrogen and scurfpea fruit in brain, and to observe the early use of low-dose estrogen on neurocognitive function damage and whether the protective effects of the application effect is better after pubertyMethods1. Twenty-one45X karyotype and twenty-four non45X karyotype TS girls and20age-matched control girls were recruited in this study. Wechsler intelligence scale and Behavioral tests for children was used to obtain their intelligence quotients (IQ) and abnormal behavior parameters. Highresolution magnetic MR imaging was performed in TS chi ldren and control girls tocollect the whole brain structural data. The ana lys is of VBM was performed wi th SPM8to compare the volume of gray and white matter between the45X karyotype and non45X karyotype of TS children and normal controls by covariance analysis.2. Set up the3weeks of age ovarian model, divided the rats into control group and ovarian group. Each group contains12rats, and all the animals are bred adaptability to ovarian surgery after2day. After9weeks, observe the changes of body weight of rats and mind the hippocampus and cortical pathology and the expression of choroid plexus’s5-HT and the hippocampus’s BDNF positive neurons3. Set up the3weeks of young age ovarian model, which were divided into control group, ovarian group, early estrogen group, late estrogen group and scurfpea fruit group. After12weeks feeding, observe the changes of body weight of rats and mind the hippocampus and cortical pathology and the expression of choroid plexus’s5-HT and the hippocampus’s BDNF positive neurons and SOD, nNOS, CHAT, CHE enzyme activity changing in the hippocampusResults1. Compared with normal controls, the statistical analysis revealed significant difference with TS group in the items of verbal comprehension, Perceptual Reasoning, working memory and Processing speed (P<0.05); There was no significant difference between TS group in the items of verbal comprehension, Perceptual Reasoning, working memory and Processing speed(P>0.05)and there was s ignif icant difference between TS group in the i tems of the accuracy and reaction time interval execution control test and attention tests of short, medium and long react ion t ime interva1and the accuracy at tent ion test of long t ime interva1time (P>0.05).Compared with normal controls,45X karyotype TS children showed significantly decreased volume (P<0.05, FWE-corrected) in the gray matter of the right precuneus, superior parietal lobule, lingual gyrus, as well as the left precentral gyrus, cingulate gray and wedge leaf, calcarine, posterior central gyrus. However, the volume of the bilateral dorsal midbrain, orbitofrontal, left insula, superior temporal gyrus, superior temporal gyrus, parahippocampal gyrus, cerebellum, the back of insula, the right side of the caudate nucleus and putamen, temporal pole gray were increased in the TS chi ldren compared to the controls; non45X karyotype TS children showed significantly decreased volume (P<0.05, FWE-corrected) in the gray matter of bilateral inferior frontal gyrus, left the precuneus, cingulate gyrus, right posterior central gyrus, supramarginal gyrus, angular convolution,cuneus; Bilateral cerebellum, left caudate nucleus, superior tempora1gyrus, right insular cortex, temporal pole size, were increased compared to the controls.45X group compared wi th other karyotype TS group, the left hippocampa1. left caudate nucleus gray matter volume reduction, the right side of the edge gray matter volume increased, the differences were statistically significant (P<0.05, FWE-corrected)2. After9weeks, there was significant differences between the two groups in weight. From HE staining and toluidine blue staining, Ovarian group’s hippocampal CA1region and cortex can slightly saw a disordered arrangement of pyramidal cells, not clear arrangement level, cell depigmentation, nuclear pyknosis and neuronophagia phenomenon (microglial cells into the damaged nerve cells). Immunohistochemical method of hippocampus and cortex to observe the expression of BDNF, the BDNF positive cells of two groups of neurons express both brown and tan. Compared wi th control group, Ovarian group’s BDNF pos i t ive number decreased, cell arranged disorderly, sparsly, contour blur, and the first spike number decreases. Immunohis to chemical of5-HT in choroid plexus, there were significantly difference between the two groups(P<0.05).3. There were no signi ficant ly differences between the five groups(P<0.05) after surgery,6weeks and12weeks after surgery to escape latent period and across platform number. From HE staining and toluidine blue staining.Estrogen and scurfpea fruit group rats hippocampal CA1area pyramidal cells arranged orderly, hierarchy clearly,celly form close to normal, structure completely. distribution of cortical neurons increased thanthe model group, the cellular structure is complete, form close to normal.5-HT positive expression of Five groups rats in choroid plexus has no significant differences (P>0.05). There was significantly differences of5-HT expression between Control and model group (P<0.05), Compared with model group, Estrogen and scurfpea fruit set group’s BDNF positive number decreased, cell arranged disorderly, sparsly, contour blur. After castration, SOD, nNOS, CHAT of Each group rats had no statistical difference (P were0.195,0.187,0.259>0.05). CHE activity had statistical differences between all groups (P=0.000<0.05), the normal group and drug group were statistical ly significant differences with the model group (P <0.05).Conclusion1. The IQ of TS Children was decreased, accompanying non-verbal obstacles.There may be verbal obstacles with monosomy TS girls. Abnormal gray and white matter volumes in some brain regions, which may be involved in the neuropathology of Turner Syndrome.2.Ovarian rats appear a certain degree of damage of brain in structure and function, with the passage of time, this damage could have been worse.3. To ovarian, estrogen and scurfpea fruit has a certain protective effect on brain function, but the effect is not obvious, There was no obvious differences in infancy and adolescence giving estrogen group, whether was relate to the medicine dose and for the length of medicine-giving time and during the draw ma terials time was the rat s’best in organs and tissues times is need for further research.