requirements by permitting unilateral optic neuritis or asymptomatic brain MRI lesions

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 AQP4IgG 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. Neurology® 2015;85:1–13 GLOSSARY ADEM 5 acute disseminated encephalomyelitis; AQP4 5 aquaporin-4; IgG 5 immunoglobulin G; IPND 5 International Panel for NMO Diagnosis; LETM 5 longitudinally extensive transverse myelitis lesions; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMO 5 neuromyelitis optica; NMOSD 5 neuromyelitis optica spectrum disorders; SLE 5 systemic lupus erythematosus; SS 5 Sjögren syndrome. Neuromyelitis optica (NMO) is an inflammatory CNS disorder distinct from multiple sclerosis (MS). It became known as Devic disease following a seminal 1894 report. Traditionally, NMO was considered a monophasic disorder consisting of simultaneous bilateral optic neuritis and transverse myelitis but relapsing cases were described in the 20th century. MRI revealed normal brain scans and$3 vertebral segment longitudinally extensive transverse myelitis lesions (LETM) in NMO. The nosology of NMO, especially whether it represented a topographically restricted form of MS, remained controversial. A major advance was the discovery that most patients with NMO have detectable serum antibodies that target the water channel aquaporin-4 (AQP4–immunoglobulin G [IgG]), are highly specific for clinically diagnosed NMO, and have pathogenic potential. In 2006, AQP4-IgG serology was incorporated into revised NMO diagnostic criteria that relaxed clinical From the Departments of Neurology (D.M.W.) and Library Services (K.E.W.), Mayo Clinic, Scottsdale, AZ; the Children’s Hospital of Philadelphia (B.B.), PA; the Departments of Neurology and Ophthalmology (J.L.B.), University of Colorado Denver, Aurora; the Service de Neurologie (P.C.), Centre Hospitalier Universitaire de Fort de France, Fort-de-France, Martinique; Department of Neurology (W.C.), Sir Charles Gairdner Hospital, Perth, Australia; the Department of Neurology (T.C.), Massachusetts General Hospital, Boston; the Department of Neurology (J.d.S.), Strasbourg University, France; the Department of Multiple Sclerosis Therapeutics (K.F.), Tohoku University Graduate School of Medicine, Sendai, Japan; the Departments of Neurology and Neurotherapeutics (B.G.), University of Texas Southwestern Medical Center, Dallas; The Walton Centre NHS Trust (A.J.), Liverpool, UK; the Molecular Neuroimmunology Group, Department of Neurology (S.J.), University Hospital Heidelberg, Germany; the Center for Multiple Sclerosis Investigation (M.L.-P.), Federal University of Minas Gerais Medical School, Belo Horizonte, Brazil; the Department of Neurology (M.L.), Johns Hopkins University, Baltimore, MD; Portland VA Medical Center and Oregon Health and Sciences University (J.H.S.), Portland; the Department of Neurology (S.T.), National Pediatric Hospital Dr. Juan P. Garrahan, Buenos Aires, Argentina; the Department of Medicine (A.L.T.), University of British Columbia, Vancouver, Canada; Nuffield Department of Clinical Neurosciences (P.W.), University of Oxford, UK; and the Department of Neurology (B.G.W.), Mayo Clinic, Rochester, MN. 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. 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. © 2015 American Academy of Neurology 1 a 2015 American Academy of Neurology. Unauthorized reproduction of this article is prohibited. Published Ahead of Print on June 19, 2015 as 10.1212/WNL.0000000000001729