查詢結果分析
來源資料
頁籤選單縮合
題 名 | Isolated Complete Agenesis of Corpus Callosum=單獨性先天胼胝體無發育 |
---|---|
作 者 | 盧文憲; 陳珠瑾; 邱寶琴; 陳英堯; 林淑敏; 卓福男; 賴炳宏; 于慶功; 謝凱生; | 書刊名 | 臺灣兒科醫學會雜誌 |
卷 期 | 44:1 民92.01-02 |
頁 次 | 頁5-7 |
分類號 | 417.5 |
關鍵詞 | 先天胼胝體無發育; Agenesis of corpus callosum; Congenital heart disease; Neuronal migration disorders; |
語 文 | 英文(English) |
中文摘要 | 胼胝體缺乏是一種神經細胞移行不良的疾病,常伴隨有其他腦部神經細胞移行障礙,如:裂腦、空腦、平腦等,也常出現在其他異常症候群中,如:三染色體8、三染色體13、胎兒酒精症候群、Hurler症候群等。在腦部疾病中,單獨性先天胼胝體完全缺乏的情形是少見的,其預後是值得探討的。在此我們回溯地探討本院6位單獨性先天胼胝體完全缺乏的病例,發現6位中有四位(4/6)無合併心臟異常,此四位中有三位生長發育正常,預後良好,一位有抽搐智障的現象;另二位(2/6)病人合併心臟異常者,一位有嚴重發育遲緩,一位於一歲內死亡,預後極差。故我們建議在産前檢查發現可能有先天性胼胝體完全缺乏者,産前的腦部磁振造影與心臟超音波爲必要的檢查,其結果可做爲産前諮詢及討論懷孕是否終止一個客觀參考依據。 |
英文摘要 | Agenesis of corpus callosum (ACC) often accompanies other neural migration disorders and its prognosis must take these disorders and other associated abnormalities into account. Isolated complete ACC, i.e., agenesis of corpus callosum without other central nervous system anomaly, is rare. A total of six patients, aged nine months to 15 years, with isolated complete ACC diagnosed by brain image study in the past eight years of our hospital were evaluated for associated defects and prognosis. Isolated complete ACC without associated congenital heart disease (CHD) was found in four of the six patients (4/6), of whom three had normal development so far. The other two patients (2/6) were found to have associated CHD and both had poor prognosis. This result suggests that checking for any associated CHD may play an important prognostic role for isolated complete ACC patients. Therefore, it is recommendable that prenatal brain MRI and fetal echocardiography should be performed for evaluation and for genetic consultation whenever ACC is suspected. |
本系統中英文摘要資訊取自各篇刊載內容。