Fetal neuropathology of proliferative vasculopathy and hydranencephaly-hydrocephaly with multiple limb pterygia.
Fetal neuropathology of proliferative vasculopathy and hydranencephaly-hydrocephaly with multiple limb pterygia.
复制标题
增殖性血管病和脑积水脑积水伴多肢翼状胬肉的胎儿神经病理学。
DOI：
10.1159/000120409
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发表时间：
1988
期刊：
影响因子：
--
通讯作者：
B. Mcgillivray
中科院分区：
文献类型：
--
作者：
M. Norman;B. Mcgillivray
我们描述了第三个家庭，其中胎儿大脑皮质外套膜非常狭窄，脑室扩大，大脑皮质动脉异常增生，肌肉发育不全。我们描述了迄今为止报道的最年轻的胎儿。以前这种情况被解释为破坏性的，可能是由于感染。我们认为这是神经外胚层细胞在脑室区形成的主要失败，大脑皮层中产生的细胞太少，异常血管增生是畸形的一部分。男女都有，提示常染色体隐性遗传。
We describe the third family in which fetuses have very narrow cerebral cortical mantles, enlarged ventricles, a peculiar proliferation of cerebral cortical arteries, and hypoplasia of muscle. We describe the youngest fetus yet reported. Previously this condition was interpreted as destructive, perhaps due to infection. We believe it is a primary failure of neuroectodermal cells to form in the ventricular zone with too few resulting cells in cerebral cortex and that the abnormal vascular proliferation is part of the malformation. The involvement of both sexes suggests autosomal recessive inheritance.