SUBEPENDYMAL GERMINOLYTIC CYSTS IN ZELLWEGER-SYNDROME
SUBEPENDYMAL GERMINOLYTIC CYSTS IN ZELLWEGER-SYNDROME
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DOI：
10.1007/bf02011090
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发表时间：
1995-06-01
影响因子：
2.3
通讯作者：
BARTH, PG
中科院分区：
文献类型：
--
作者：
RUSSEL, IMB;VANSONDEREN, L;BARTH, PG
超声显示脑室周围囊肿通常伴有脑室周围无髓鞘白色物质坏死引起的囊性脑室周围白质软化。具有类似外观的囊性间隙也可能来自胎儿室周基质组织的产前溶解，而不是不成熟的白色物质。由此产生的空的空间被称为“假囊肿"或溶胚囊肿。神经病理学研究表明，溶菌囊肿可能出现在齐薇格综合征（全身性过氧化物酶体疾病）。在这里，我们报告他们的超声表现在齐薇格综合征，强调这种迹象在诊断中的潜在价值。
Ultrasonographic demonstration of periventricular cysts is usually associated with cystic periventricular leucomalacia due to necrosis of periventricular unmyelinated white matter. Cystic spaces with a similar appearance may also arise from prenatal lysis of fetal periventricular matrix tissue, rather than immature white matter. The resultant empty spaces are called ''pseudocysts'' or germinolytic cysts. Neuropathological studies have shown that germinolytic cysts may arise in Zellweger syndrome (generalized peroxisomal disorder). Here, we report their ultrasonographic demonstration in Zellweger syndrome, emphasizing the potential value of this sign in the diagnosis.