Proliferative Vasculopathy and an Hydranencephalic‐hydrocephalic Syndrome: a Neuropathological Study of Two Siblings
Proliferative Vasculopathy and an Hydranencephalic‐hydrocephalic Syndrome: a Neuropathological Study of Two Siblings
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增殖性血管病和脑积水-脑积水综合征：两个兄弟姐妹的神经病理学研究
DOI：
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发表时间：
1983
影响因子：
3.8
通讯作者：
A. Hockey
中科院分区：
文献类型：
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作者：
Clive Harper;A. Hockey
两名女性同胞出生时患有积水性无脑-脑积水综合征，妊娠并发羊水过多。没有环境因素，如感染，药物或代谢紊乱，注意到在任何怀孕。神经病理学研究显示，在每种情况下相同的变化。病理学的特征性特征是整个中枢神经系统的增殖性血管病变，这显然引起局灶性缺血性病变和CNS组织的进行性破坏。该缺陷可能是作为常染色体隐性遗传性状遗传的。
Two female siblings were born with an hydranencephalic‐hydrocephalic syndrome, following pregnancies complicated by hydramnios. No environmental factors such as infections, drugs or metabolic disorders were noted during either pregnancy. Neuropathological studies revealed identical changes in each case. The characteristic feature of the pathology was a proliferative vasculopathy throughout the central nervous system, which apparently caused focal ischaemic lesions and progressive destruction of CNS tissue. The defect probably is inherited as an autosomal recessive trait.