頁籤選單縮合
題名 | Alexander's Disease in a 2-Month-Old Female Infant=亞歷山大症於一位二月大之女嬰 |
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作者 | 何慈育; 洪志興; 阮春榮; 盧建維; 許木嶺; 陳俊榮; 陳錫洲; | 書刊名 | Journal of Medical Sciences |
卷期 | 22:4 2002.08[民91.08] |
頁次 | 頁195-198 |
分類號 | 417.5012 |
關鍵詞 | 亞歷山大症; Alexander's disease; Leukodystrophy; Magnetic resonance image; Rosenthal fiber; |
語文 | 英文(English) |
英文摘要 | Alexander's disease (AD) is a rare progressive lethal leukodystrophy of brain, usually affecting infants. It is characterized by the progressive failure of central myelination and accumulation of Rosenthal fiber (RF) in the astrocytes. We reported a 2-month-old female infant had a history of brief focal clonic seizure over the left upper and lower limbs. During six months follow-up, markedly delayed neurodevelopment, progressive megalencephaly and refractory seizure were noted. The first magnetic resonance (MR) images performed at her age of 2 months and revealed bilateral frontal periventricular white matter T2 hyperintensity and enhancement of basal ganglia, tectum, and bilateral frontal periventricular white matter with the concurrent dilatation of bilateral lateral ventricles. A followed MR study at her age of 8 months showed interval progression of disease presenting as necrosis with marked frontal cystic encephalomalacia and loss of brain tissue, causing passive dilatation of bilateral lateral and the ventricles. According to the patient's progressive history, clinical manifestations, and the serial changes in the MR images of brain, Alexander's disease was diagnosed. |
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