以幼兒瓜胺酸血症之腦部磁振造影擴散張量影像神經纖維追蹤術

瓜胺酸血症為尿素循環異常的遺傳疾病，根據過去的文獻記載針對瓜胺酸血症的影像報告以成年人較為常見，針對幼兒患者的神經影像報告較為稀少，本文取材於一瓜胺酸血症的幼兒於其6個月大及一歲時分別施以常規的磁振造影像、磁振造影血管成像、擴散影像、擴散張量影像及神經纖維追蹤術，個案於6個月大時在雙側內囊(bilateral internal capsules)及胼胝體的壓部(splenium of corpus callosum)有低髓鞘化的現象，FA值為0.183±0.907，於1歲時在雙側內囊及胼胝體的壓部低髓鞘化的現象並沒有改善，胼胝體的膝部(genus of corpus callosum)亦有低髓鞘化之情形，FA值降低為0.158±0.0881，結果證明瓜胺酸血症會對幼兒患者腦部造成損傷，擴散張量影像及神經纖維追蹤術對於腦部損傷其診斷價值相當的顯著。

關鍵字

磁振造影；擴散張量影像；神經纖維追蹤術；瓜胺酸血症

並列摘要

Citrullinemia is the urea cycle abnormalities of genetic diseases, Reports on citrullinemia usually describe the imaging findings in the adult form of the disease, neuroimaging findings of neonatal cases are seldom reported, We report on the Conventional MR, MRA, diffusion weighted imaging, diffusion tensor imaging and fiber tractography were performed in a boy with citrullinemia when he was six months old and one years old. When the case was 6 months old, the image shows hypomyelination in bilateral internal capsules, splenium of corpus callosum with FA value 0.183±0.0907. The hypomyelination had not improved with further extension to the genus of corpus callosum when baby1 years old, FA value reduces to 0.158±0.0881. The result proves damage in the neonatal brain with citrullinemia. Diffusion tensor imaging and fiber tractography are valuable techniques for the detection of brain damage in the infant with citrullinemia.

並列關鍵字

MRI ； Diffusion Tensor ； fiber tractography ； citrullinemia

國際替代計量

以幼兒瓜胺酸血症之腦部磁振造影擴散張量影像神經纖維追蹤術

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