Nikoskelainen E, Hoyt W F, Nummelin K, Schatz H
Arch Ophthalmol. 1984 Jul;102(7):981-9. doi: 10.1001/archopht.1984.01040030783017.
Fluorescein angiography was performed in nine members of families with Leber's disease. Serial studies were obtained in four men observed from asymptomatic to atrophic stages. Peripapillary microangiopathy was observed in six of nine asymptomatic eyes. Arteriovenous shunting occurred in the telangiectatic vascular bed. These changes remained stable in some eyes and progressed in others. In the acute stage, arteries and telangiectatic vessels were maximally dilated and flow was rapid. Angiography showed florid shunting in lower and upper vascular arcades and reduced filling of papillomacular capillaries. Vessels of the shunting vascular bed gradually narrowed irregularly. In the atrophic stage, disc vascularity diminished and arteriovenous circulation time increased markedly. Arterioles narrowed and peripapillary microangiopathy disappeared. These findings support our contention that Leber's disease is a hereditary vascular neuroretinopathy.
对患有莱伯病的家族中的九名成员进行了荧光素血管造影。对四名从无症状期观察到萎缩期的男性进行了系列研究。在九只无症状眼睛中的六只观察到视乳头周围微血管病变。在扩张的血管床中出现动静脉分流。这些变化在一些眼睛中保持稳定，而在另一些眼睛中则进展。在急性期，动脉和扩张的血管最大程度地扩张，血流迅速。血管造影显示上下血管弓有明显的分流，乳头黄斑毛细血管充盈减少。分流血管床的血管逐渐不规则变窄。在萎缩期，视盘血管减少，动静脉循环时间明显延长。小动脉变窄，视乳头周围微血管病变消失。这些发现支持了我们的观点，即莱伯病是一种遗传性血管性神经视网膜病变。
