Volumetric Neuroimaging in Children with Neurodevelopmental Disorders-Mapping the Brain and Behavior

神經發展異常小孩之磁振造影腦部體積測量－腦與認知特爲之相關研究

張瑛玿(Ying-Chao Chang) ； 黃朝慶(Chao-Ching Huang) ； 黃嵩雀(Song-Chei Huang)

《Acta Paediatrica Sinica》 39卷5期 (1998/10) Pp. 285-292

https://doi.org/10.7097/APS.199810.0285

摘要

我們整理了近來有關脆弱X染色體症，唐氏症，雷特症，及多雷氏症候群之腦部體積測量研究。依據我們目前對這些疾病神經生理及病理之以解，來探討各個疾病在神經影像學上的變化及其所對應之認知及行爲的發展問題。在脆弱X染色體症中，常被影響的區域是小腦蚓部，顳葉，及尾狀核，此可能與其臨床上之自閉傾向，記憶與語言問題，及執行功能缺失有關。在唐氏症中，以額葉，小腦，內側顳葉爲主要區域，與其臨床上明顯語言發展問題可能有關。顯著地整個大腦萎縮，以灰質尤劇，與雷特症厲害的神經發展障礙若相符合。右側基底核較左側大，見於多雷氏症候群，亦可能與其臨床上執行功能缺失有關。我們並進一步討論對這些發障礙，將來神經影像學及神經心理學研究之方向。

關鍵字

磁振造影 ； 脆弱X染色體症 ； 唐氏症 ； 雷特證症 ； 多雷氏症候群

並列摘要

The recent volumetric neuroimaging studies in children with fragile X syndrome, Down syndrome (DS), Rett syndrome (RS), and Tourette syndrome (TS) were reviewed. Neuroimaging studies and cognitive and behavioral phenotypes pertinent to each disorder were reviewed within the context of current knowledge of their neurobiological mechanisms. The mainly involved brain regions in fragile X syndrome are the cerebellar vermis, temporal areas, and the caudate nucleus which may be related to autistic tendency, problems of memory and language and deficits in executive function, respectively. The preferential involvement of frontal lobe, cerebellum and mesial temporal areas may correspond to the selective language and speech deficits in DS children. There was significantly generalized cerebral volume reduction with greater loss of gray matter in comparison to white matter, consistent with the extensive clinical features of RS. Right side predominance of basal ganglia supported the suggestion that the basal ganglia is related to pathogenesis of TS, which may also be related to the impaired executive function and the absence of functional asymmetry. Further prospectives for integrating neuroanatomic findings and neurobehavioral studies also were discussed.

並列關鍵字

volumetric MRI ； fragile X syndrome ； Down syndrome ； Rett syndrome ； Tourette syndrome