Retinal lesion evolution in Susac syndrome (P5.319)
Bibliographic record
Abstract
OBJECTIVE:To describe retinal lesion development in Susac syndrome. BACKGROUND:Susac syndrome is a rare disorder but an important differential diagnosis to other autoimmune, neuroinflammatory disorders. The clinical triad of acute visual loss, encephalopathy, and cochlear hearing loss characterizes the syndrome. Its etiology is unknown, but a microangiopathy with an autoimmune reaction against vascular structures in the respective organs is suspected. DESIGN/METHODS:Longitudinal evaluation of a 34 year-old female patient with active Susac syndrome undergoing immunosuppressive treatment at multiple time points. Confirmatory cross-sectional evaluation of four additional patients with Susac syndrome. Retinal morphology and disease activity was monitored with serial 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 post-acute 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 primary 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 lesions development and morphologically distinct lesion sections suggest more complex mechanisms of lesion evolution beyond an isolated endothelial immune reaction. The characteristic lesion morphology may assist in differentiating the diagnosis of acute visual loss in the context of neuroinflammatory disease.
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.001 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.000 | 0.000 |
| Bibliometrics | 0.001 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.001 | 0.000 |
| Insufficient payload (model declined to judge) | 0.001 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".