Neurological complications of Grönblad-Strandberg syndrome
Neurological complications of Grönblad-Strandberg syndrome
复制标题
格伦布拉德-斯特兰伯格综合征的神经系统并发症
DOI：
10.1007/bf00313660
复制
发表时间：
1984
影响因子：
6
通讯作者：
C. Rossberg
中科院分区：
文献类型：
--
作者：
K. Fasshauer;C. Reimers;H. ;I. Strempel;C. Rossberg
在一名患有Grönblad-Strandberg综合征的59岁女性患者中观察到明显的脑血管变化导致的神经系统并发症。除了典型的皮肤变化的弹性假黄瘤和眼部改变（渗出性黄斑变性和血管样条纹），有一个不寻常的数量紊乱的内分泌功能。眼科检查结果显示特征性的布鲁赫膜破裂，脉络膜血管增生进入视网膜下腔，视网膜色素上皮增生和黄斑变性。脑的尸检显示，由于脑血液供应的反复紊乱以及许多脑动脉的弹性内动脉的特别明显的变化，导致多发性脑梗死。虽然在我们的患者中观察到的神经系统体征不是Grönblad-Strandberg综合征的特征，但组织病理学发现导致了以下假设：相同的基本机制导致弹性纤维和胶原蛋白的改变，主要是在眼-脑系统中。
Neurological complications resulting from pronounced cerebrovascular changes were observed in a 59-year-old female suffering from Grönblad-Strandberg syndrome. In addition to typical skin changes of the pseudoxanthoma elasticum and ocular alterations (exudative macular degeneration and angioid streaks) there were an unusual number of disturbed endocrinological functions. The ophthalmological findings revealed characteristic breaks in Bruch's membrane, proliferation of chorioidal vessels into the subretinal space, hyperplasia of retinal pigment epithelium and disciform macular degeneration. Postmortem examination of the brain showed multiple cerebral infarcts due to recurrent disturbances in cerebral blood supply and particularly pronounced changes in the elastica interna of many cerebral arteries. Although the observed neurological signs in our patient were not characteristic of Grönblad-Strandberg syndrome, the histopathological findings led to the assumption that the same basic mechanisms result in alternations of elastic fibres and collagen, mainly in the ocular-cerebral system.