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頁籤選單縮合
| 題 名 | Early Cerebral Images of Alexander Disease: Report of One Case=亞歷山大症的早期腦部影像發現:一病例報告 |
|---|---|
| 作 者 | 黃淵德; 陳珠瑾; 邱寶琴; 賴炳宏; 謝凱生; | 書刊名 | 臺灣兒科醫學會雜誌 |
| 卷 期 | 45:1 民93.01-02 |
| 頁 次 | 頁52-55+64 |
| 分類號 | 417.5012 |
| 關鍵詞 | 亞歷山大症; 影像診斷; 腦室週圍環; 白質病變; Alexander disease; Image diagnosis; Periventricular rim; White matter disease; |
| 語 文 | 英文(English) |
| 中文摘要 | 亞歷山大症是一種罕見的神經退化性疾病,其特點是在腦部的星形細胞出現Rosenthal纖維質。臨床上依其症狀出現的年齡,有人將之分爲三種亞型:嬰兒型、青春型及成人型;其中以嬰兒型最常見:病患於一歲前出現大頭症和/或水腦、發展遲緩、僵直性四肢病變及抽搐,常導致生命的早期結束。亞歷山大症的診斷必須靠病理,沒有特殊生化檢查或酵素值來確定,但影像上往往出現典型的變化,即廣泛的白質病變,以額葉區爲主,且有顯影劑增强的特點。這裡我們描述一個四個月大的男嬰出現抽搐及發展遲緩,當時的腦部磁振造影有不正常的影像,但我們沒有辦法確定診斷,一直到他一歲大時再接受一次磁振造影,此時表現亞歷山大症典型的影像變化。在這篇文章裡,我們引用了van der Knaap提出的診斷亞歷山大症的腦部磁振造影要件,並提醒亞歷山大症在早期可能先出現特殊的、別於後期的變化。 |
| 英文摘要 | Alexander disease is a neurodegenerative disorder characterized pathologically by demyelination and accumulation of eosinophilic hyaline bodies (Rosenthal fibers) within astrocytes. Demonstration of Rosenthal fibers on histological examination is considered a prerequisite for a definitive diagnosis. However, the CT and MRI scans may be highly suggestive of the disorder. We describe a patient who presented with subtle seizures at the age of 4 months. On examination, he was floppy with evident head lag. There was no visual following or social smiling. At that time, the brain MRI showed abnormal findings, with more white matter involvement and a characteristic periventricular rim. A diagnosis of Alexander disease was not made until he was one year old, when a repeated MRI showed the full-blown pictures typically seen in Alexander disease. The images fulfilled the diagnostic criteria proposed by van der Knaap in 2001. The early brain MRI findings in Alexander disease can be very characteristic and greatly different from those in the late stage. |
本系統中英文摘要資訊取自各篇刊載內容。