Neuromyelitis optica spectrum disorder classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Julinka Auta Fernandes
Overview
- Neuromyelitis optica spectrum disorder (NMOSD) is classified primarily according to serum aquaporin-4 immunoglobulin G (AQP4-IgG) serostatus and may additionally be described according to its clinical course.
- The 2015 International Panel for Neuromyelitis Optica Diagnosis (IPND) consensus framework defines two formal serologic categories: NMOSD with AQP4-IgG and NMOSD without AQP4-IgG; the diagnostic pathway for patients with unknown AQP4-IgG status is the same as that for AQP4-IgG-negative patients.[1]
- The 2023 recommendations of the Neuromyelitis Optica Study Group (NEMOS), a European expert group, endorse the 2015 IPND framework, advise that truly negative and unknown AQP4-IgG status be documented separately, and discourage use of the superseded 1999 and 2006 criteria.[2]
- Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) is a distinct disorder. AQP4-IgG-negative patients who fulfill the international MOGAD criteria should be classified as MOGAD rather than NMOSD.[3][4]
Classification
- Neuromyelitis optica spectrum disorder is classified by AQP4-IgG serostatus; relapsing or monophasic course may be added as a descriptive clinical-course modifier.[1][2]
Classification according to AQP4-IgG serostatus
- Neuromyelitis optica spectrum disorder is classified according to AQP4-IgG serostatus as shown below.
| Category | Definition | Classification note |
|---|---|---|
| NMOSD with AQP4-IgG | Serum AQP4-IgG is detected with an appropriate assay in a patient who fulfills the 2015 IPND diagnostic requirements. | This is an antibody-defined autoimmune astrocytopathy. AQP4-IgG positivity does not by itself establish NMOSD; at least one compatible core clinical characteristic and exclusion of a better diagnosis are required. |
| NMOSD without AQP4-IgG | Serum AQP4-IgG is not detected with an appropriate assay, and the patient fulfills the more stringent 2015 IPND clinical and imaging requirements. | This is a heterogeneous category. MOGAD and other alternative diagnoses must be excluded before this label is assigned. |
| NMOSD with unknown AQP4-IgG status | AQP4-IgG testing has not been performed, is unavailable, or cannot be interpreted reliably. | The 2015 IPND criteria apply the same diagnostic requirements used for AQP4-IgG-negative disease. NEMOS recommends AQP4-IgG testing at the earliest opportunity and separate documentation of unknown status rather than treating it as a true negative result. |
Classification adapted from the 2015 IPND consensus criteria and the 2023 NEMOS recommendations.[1][2]
Serologic refinement of AQP4-IgG-negative cases
- Neuromyelitis optica spectrum disorder without AQP4-IgG should be further assessed according to serum myelin oligodendrocyte glycoprotein immunoglobulin G (MOG-IgG) status because MOGAD is no longer classified as a subtype of NMOSD.[2][3][4]
| Serologic finding | Classification |
|---|---|
| MOG-IgG positive | If the international diagnostic criteria for MOGAD are fulfilled, classify the disorder as MOGAD, not NMOSD. |
| AQP4-IgG and MOG-IgG negative | The descriptive term double-seronegative NMOSD may be used only when the stricter IPND clinical and imaging requirements are fulfilled and alternative diagnoses have been excluded. This remains a heterogeneous, non-biomarker-defined group and should not be assumed to represent one biological disease. |
| AQP4-IgG and MOG-IgG positive | True double positivity is exceptionally rare. Both results should be reconfirmed with appropriate assays and the case reviewed at a specialized center before a final classification is assigned. |
Adapted from the 2023 NEMOS recommendations and the 2023 International MOGAD Panel criteria.[2][3]
Descriptive classification according to clinical course
- Neuromyelitis optica spectrum disorder may be described as relapsing or monophasic; this course modifier does not replace classification according to AQP4-IgG serostatus.[1][2]
| Course | Definition and interpretation |
|---|---|
| Relapsing NMOSD | A new clinical attack occurs more than 4 weeks after onset of the initial attack. New symptoms arising within 4 weeks of attack onset are considered part of the same attack rather than a relapse. |
| Monophasic NMOSD | No further attack occurs after the index event or closely related index events. Because late relapse may occur, the IPND recommends at least 5 years—and preferably longer—of relapse-free observation before a monophasic course is assigned with confidence. The term should be used particularly cautiously in AQP4-IgG-positive disease. |
Adapted from the 2015 IPND consensus criteria and the 2023 NEMOS recommendations.[1][2]
Terminology
- Neuromyelitis optica spectrum disorder is the unifying contemporary term; classic neuromyelitis optica (NMO, historically called Devic disease) is included within NMOSD and is not a separate modern serologic subtype.[1]
- The terms AQP4-IgG negative and AQP4-IgG status unknown are not interchangeable and should be recorded separately.[2]
- The term MOG-IgG-positive NMOSD should be avoided when the patient fulfills MOGAD criteria; the appropriate classification is MOGAD.[2][3]
- NMOSD has no staging system.
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Wingerchuk DM, Banwell B, Bennett JL, et al. International consensus diagnostic criteria for neuromyelitis optica spectrum disorders. Neurology. 2015;85(2):177-189. doi:10.1212/WNL.0000000000001729. PMID: 26092914.
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 2.8 Jarius S, Paul F, Aktas O, et al. Update on the diagnosis and treatment of neuromyelitis optica spectrum disorders—revised recommendations of the Neuromyelitis Optica Study Group (NEMOS). Part I: Diagnosis and differential diagnosis. Journal of Neurology. 2023;270(7):3341-3368. doi:10.1007/s00415-023-11634-0. PMID: 37022481.
- ↑ 3.0 3.1 3.2 3.3 Banwell B, Bennett JL, Marignier R, et al. Diagnosis of myelin oligodendrocyte glycoprotein antibody-associated disease: International MOGAD Panel proposed criteria. The Lancet Neurology. 2023;22(3):268-282. doi:10.1016/S1474-4422(22)00431-8. PMID: 36706773.
- ↑ 4.0 4.1 Cortese R, Hacohen Y, Arrambide G, et al. Use of MRI in neuromyelitis optica spectrum disorder and myelin oligodendrocyte glycoprotein antibody-associated disease—MAGNIMS consensus recommendations. Nature Reviews Neurology. Published September 28, 2026. doi:10.1038/s41582-026-01271-2.