title: RETINAL LESION EVOLUTION IN SUSAC SYNDROME.
abstract: OBJECTIVE
  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.
  
  
  CONCLUSIONS
  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.affiliation: *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.
authors.forename: Alexander U
authors.initials: AU
authors.lastname: Brandt
authors.forename: Timm
authors.initials: T
authors.lastname: Oberwahrenbrock
authors.forename: Fiona
authors.initials: F
authors.lastname: Costello
authors.forename: Michael
authors.initials: M
authors.lastname: Fielden
authors.forename: Karen
authors.initials: K
authors.lastname: Gertz
authors.forename: Ilka
authors.initials: I
authors.lastname: Kleffner
authors.forename: Friedemann
authors.initials: F
authors.lastname: Paul
authors.forename: Richard
authors.initials: R
authors.lastname: Bergholz
authors.forename: Jan
authors.initials: J
authors.lastname: Dörr
mesh_terms: D000208:Acute Disease; D000368:Aged; D002908:Chronic Disease; D003430:Cross-Sectional Studies; D005260:Female; D005451:Fluorescein Angiography; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D012161:Retinal Artery / Q000473:pathology*; D012164:Retinal Diseases / Q000175:diagnosis* / Q000503:physiopathology; D055955:Susac Syndrome / Q000175:diagnosis* / Q000503:physiopathology; D041623:Tomography, Optical Coherence; D014792:Visual Acuity