Revised Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorders (S63.001)
Bibliographic record
Abstract
Objective: To revise diagnostic criteria for neuromyelitis optica (NMO) and NMO spectrum disorders. Background: NMO is an inflammatory demyelinating CNS syndrome distinct from multiple sclerosis (MS) and associated with serum aquaporin-4 antibodies (AQP4-IgG). Current diagnostic criteria were developed in 2006 and require both optic nerve and spinal cord involvement. However, clinical and neuroimaging evidence, especially from AQP4-IgG seropositive patients, has revealed a wider disease spectrum. The International Panel for NMO Diagnosis (IPND) was convened to develop revised, evidence-based consensus diagnostic criteria. Design/Methods: The INPD met on 7 occasions between October, 2011 and November, 2013. Nineteen panel members participated in 6 working groups (clinical presentation, serology, neuroimaging, pediatrics, systemic autoimmunity, and opticospinal MS), each of which were charged with addressing focused questions to contribute to the revised diagnostic criteria. Each working group conducted systematic literature reviews related to their specific charges and summarized the results. Electronic surveys were then used to develop new criteria, which were iteratively refined through electronic scoring and face-to-face meetings. Results: The new diagnostic nomenclature defines the unified term “NMO spectrum disorders” (NMOSD), which is stratified by serological testing results (NMOSD with or without AQP4-IgG). Core characteristics of NMOSD with AQP4 antibodies include clinical syndromes and/or MRI findings related to optic nerve, spinal cord, brain stem, diencephalic, or cerebral presentations. The presence of enriched core characteristics, plus additional supportive criteria, are required for diagnosis of NMOSD without AQP4-IgG. The IPND also achieved consensus on pediatric NMOSD diagnosis, AQP4-IgG testing, monophasic NMOSD and opticospinal MS. Conclusions: The IPND achieved consensus on revised diagnostic criteria for adult and pediatric NMOSD, with or without AQP4-IgG, for clinical and research purposes and to distinguish NMOSD from competing diagnoses. These criteria require prospective validation. The proposed nomenclature allow for future revisions to account for new clinical, neuroimaging, laboratory, and antibody associations. Study Supported by: Guthy-Jackson Charitable Foundation.
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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 | 0.004 | 0.010 |
| Meta-epidemiology (narrow) | 0.001 | 0.000 |
| Meta-epidemiology (broad) | 0.001 | 0.002 |
| Bibliometrics | 0.005 | 0.002 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.001 | 0.002 |
| Open science | 0.002 | 0.002 |
| Research integrity | 0.002 | 0.002 |
| Insufficient payload (model declined to judge) | 0.014 | 0.009 |
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".