Alexander U Brandt1, Timm Oberwahrenbrock, Fiona Costello, Michael Fielden, Karen Gertz, Ilka Kleffner, Friedemann Paul, Richard Bergholz, Jan Dörr. 1. *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.
Abstract
PURPOSE: To describe retinal lesion development in Susac syndrome during acute, postacute, and late phases of the disease. METHODS: 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. RESULTS: 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. CONCLUSION: 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.
PURPOSE: To describe retinal lesion development in Susac syndrome during acute, postacute, and late phases of the disease. METHODS: 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. RESULTS: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. CONCLUSION: 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.
Authors: Kay Gawlik; Frank Hausser; Friedemann Paul; Alexander U Brandt; Ella Maria Kadas Journal: Biomed Opt Express Date: 2018-11-28 Impact factor: 3.732
Authors: Simone Alex Bagaglia; Franco Passani; Giovanni William Oliverio; Leandro Inferrera; Feliciana Menna; Alessandro Meduri; Cosimo Mazzotta Journal: Int J Environ Res Public Health Date: 2021-03-26 Impact factor: 3.390
Authors: Timm Oberwahrenbrock; Maria Weinhold; Janine Mikolajczak; Hanna Zimmermann; Friedemann Paul; Ingeborg Beckers; Alexander U Brandt Journal: PLoS One Date: 2015-09-08 Impact factor: 3.240