Abstract
Neuromyelitis optica (NMO) is an inflammatory CNS syndrome distinct from multiple sclerosis (MS) that is associated with serum aquaporin-4 immunoglobulin G antibodies (AQP4-IgG). Prior NMO diagnostic criteria required optic nerve and spinal cord involvement but more restricted or more extensive CNS involvement may occur. The International Panel for NMO Diagnosis (IPND) was convened to develop revised diagnostic criteria using systematic literature reviews and electronic surveys to facilitate consensus. The new nomenclature defines the unifying term NMO spectrum disorders (NMOSD), which is stratified further by serologic testing (NMOSD with or without AQP4-IgG). The core clinical characteristics required for patients with NMOSD with AQP4-IgG include clinical syndromes or MRI findings related to optic nerve, spinal cord, area postrema, other brainstem, diencephalic, or cerebral presentations. More stringent clinical criteria, with additional neuroimaging findings, are required for diagnosis of NMOSD without AQP4-IgG or when serologic testing is unavailable. The IPND also proposed validation strategies and achieved consensus on pediatric NMOSD diagnosis and the concepts of monophasic NMOSD and opticospinal MS.
GLOSSARY
- ADEM=
- acute disseminated encephalomyelitis;
- AQP4=
- aquaporin-4;
- IgG=
- immunoglobulin G;
- IPND=
- International Panel for NMO Diagnosis;
- LETM=
- longitudinally extensive transverse myelitis lesions;
- MOG=
- myelin oligodendrocyte glycoprotein;
- MS=
- multiple sclerosis;
- NMO=
- neuromyelitis optica;
- NMOSD=
- neuromyelitis optica spectrum disorders;
- SLE=
- systemic lupus erythematosus;
- SS=
- Sjögren syndrome
Footnotes
Go to Neurology.org for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article. The Article Processing Charge was paid by the Guthy-Jackson Charitable Foundation.
Editorial, page 118
Supplemental data at Neurology.org
- Received September 30, 2014.
- Accepted in final form January 28, 2015.
- © 2015 American Academy of Neurology
This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially.
Letters: Rapid online correspondence
- Age and NMOSD Diagnosis
- Late-onset NMOSD: another diagnostic challenge
- Definitive diagnosis of NMOSD without AQP4-IgG is preferable to a "probable" category
- Patients with AQP4-IgG negative or unknown should be classified as "probable NMO"
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