Brandt Alexander U, Oberwahrenbrock Timm, Costello Fiona, Fielden Michael, Gertz Karen, Kleffner Ilka, Paul Friedemann, Bergholz Richard, Dörr Jan
*NeuroCure Clinical Research Center, Charité-Universitätsmedizin Berlin, Berlin, Germany; †Departments of Clinical Neurosciences, and ‡Surgery, University of Calgary, Calgary, Alberta, Canada; §Calgary Retina Consultants, University of Calgary, Calgary, Alberta, Canada; ¶Department of Neurology, Charité-Universitätsmedizin Berlin, Berlin, Germany; **Department of Neurology, University of Münster, Münster, Germany; and ††Department of Ophthalmology, Charité-Universitätsmedizin Berlin, Berlin, Germany.
Retina. 2016 Feb;36(2):366-74. doi: 10.1097/IAE.0000000000000700.
To describe retinal lesion development in Susac syndrome during acute, postacute, and late phases of the disease.
Cross-sectional study of four patients with Susac syndrome and longitudinal short-interval case study of one additional patient. Retinal changes were analyzed with high-resolution spectral domain optical coherence tomography and retinal fluorescein angiography.
Retinal Susac syndrome lesions comprise four different lesion sections, which can be distinguished in acute and postacute phases of the disease: a primary section at the site of branch retinal artery occlusion, which spans more layers than supplied by the affected vessel; hypoxic sections from superficial and deep capillary networks; and an axonal damage section with degenerating axons from perished ganglion cells in the main and hypoxic sections. In the later stages, main and hypoxic lesion sections can no longer be distinguished, and both show degeneration from outer plexiform to retinal nerve fiber layers.
The dynamics of lesion development and morphologically distinct lesion sections suggest more complex mechanisms of lesion evolution beyond an isolated endothelial immune reaction and subsequent hypoxic tissue damage. The characteristic lesion morphology assists in differentiating the diagnosis of acute visual loss in neuroinflammatory disease. Specificity of the identified changes has to be determined in future studies also including patients with other retinal vascular diseases.
描述Susac综合征在疾病急性期、急性后期和晚期的视网膜病变发展情况。
对4例Susac综合征患者进行横断面研究，并对另外1例患者进行纵向短间隔病例研究。采用高分辨率光谱域光学相干断层扫描和视网膜荧光血管造影分析视网膜变化。
视网膜Susac综合征病变包括四个不同的病变区域，在疾病的急性期和急性后期可加以区分：视网膜分支动脉阻塞部位的主要区域，其跨越的层次比受影响血管供应的层次更多；来自浅层和深层毛细血管网的缺氧区域；以及轴突损伤区域，主要区域和缺氧区域中死亡的神经节细胞的轴突发生退化。在后期，主要病变区域和缺氧病变区域无法再区分，两者均显示从外丛状层到视网膜神经纤维层的退化。
病变发展的动态过程和形态学上不同的病变区域表明，除了孤立的内皮免疫反应和随后的缺氧组织损伤外，病变演变机制更为复杂。特征性的病变形态有助于鉴别神经炎性疾病急性视力丧失的诊断。所确定变化的特异性还必须在未来的研究中加以确定，这些研究还应纳入其他视网膜血管疾病患者。
