Vázquez E, Macaya A, Mayolas N, Arévalo S, Poca M A, Enríquez G
Department of Pediatric Radiology, Area Materno-Infantil, Hospital Universitari Vall d'Hebron, Universidad Autónoma de Barcelona, Barcelona, Spain.
AJNR Am J Neuroradiol. 2008 Nov;29(10):1973-5. doi: 10.3174/ajnr.A1215. Epub 2008 Jul 24.
Alexander disease (AD) is a rare neurodegenerative disorder characterized by megalencephaly, leukoencephalopathy, and Rosenthal fibers within astrocytes. This report describes the case of a female patient with sonography-detected ventriculomegaly at 32 weeks' gestation and distinctive MR imaging features at 33 and 36 weeks' gestation, at birth, and at 2 months of age, which led to the suggested diagnosis of Alexander disease. Molecular analysis confirmed a missense mutation in the GFAP gene. The literature contains little information on the fetal MR imaging findings that may allow prenatal diagnosis of AD.
亚历山大病(AD)是一种罕见的神经退行性疾病,其特征为巨头畸形、白质脑病以及星形胶质细胞内的罗森塔尔纤维。本报告描述了一名女性患者的病例,该患者在妊娠32周时超声检查发现脑室扩大,在妊娠33周、36周、出生时及2个月大时具有独特的磁共振成像特征,这些特征提示诊断为亚历山大病。分子分析证实胶质纤维酸性蛋白(GFAP)基因存在错义突变。关于可能有助于产前诊断亚历山大病的胎儿磁共振成像结果,文献中报道较少。