Suspect-and-test checklist
Neuromyelitis optica spectrum disorder (NMOSD)
Antibody-mediated CNS disease, often first labeled MS. Serum AQP4-IgG by cell-based assay is the key test, ideally drawn before treatment.
ICD-10-CMG36.0Trial Friend for Clinicians
1Red flags present
Eye
Spinal cord
Gut and brainstem
Other brain
Who
CSF and MRI against MS
2Already labeled as
3Family to test
4Testing, in the order the sources describe
- Serum AQP4-IgG, cell-based assayAny 1 of the 6 core characteristics. Draw before steroids, plasma exchange or immunosuppressants where possible.Result
- Area postrema criteriaSymptoms 48 hours or more, or less with a new area postrema lesion; other causes excluded.Result
- MRI of brain and spinal cordMandatory in the NEMOS workup. Orbital MRI in selected patients.Result
- Lumbar punctureOligoclonal bands, cell count and differential, protein, lactate, glucose.Result
- Serum MOG-IgG, cell-based assayAQP4-IgG negative patients who meet NMOSD criteria. Meeting MOGAD criteria replaces the NMOSD label, per NEMOS.Result
- Confirm or repeatSecond assay type for a low-titer ELISA positive or atypical picture. Retest seronegatives who relapse or before B-cell therapy.Result
- 2015 IPND criteriaAQP4-IgG positive: 1 core characteristic. Negative or unknown: 2 core characteristics plus MRI requirements. Exclude alternatives.Result
5Results the sources say do not rule it out
- A negative AQP4-IgG drawn after steroids, plasma exchange or immunosuppressants.
- A single negative AQP4-IgG. Later tests turned positive after years in some misdiagnosed patients.
- A cord lesion shorter than 3 segments. Seen in 7% to 14% of first myelitis attacks.
- MS-like white matter lesions on brain MRI. Up to 16% meet Barkhof MS criteria.
- An unrevealing gastroscopy, small bowel X-ray or abdominal ultrasound in intractable vomiting.
- A positive AQP4-IgG by ELISA alone in an atypical picture. Confirm by cell-based assay.
6Specialists the sources name
Diagnosis code: G36.0 Neuromyelitis optica [Devic]Codes patients often carry before diagnosis are listed in the online guide.
Full guide and sourcesEvery point here is sourced in the online guide, which also has a patient note to print.
Trial Friend, trialfriend.com/for-clinicians. Key sources: 2015 IPND diagnostic criteria; NEMOS 2023 recommendations, part I; NEMOS 2024 recommendations, part II; Shosha 2018 area postrema study; Apiwattanakul 2010 Mayo Clinic vomiting series; Iorio 2013 gastroenterology study; all 34 with links in the online guide. Checked November 11, 2026. A reference checklist for licensed clinicians that summarizes published sources. It is not a diagnostic tool, a score or a protocol, and it does not replace clinical judgment, local practice or the prescribing information. Not coding advice. Trial Friend never receives anything written or typed on this sheet.