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Neurology. 2015 Jul 14;85(2):177-89. doi: 10.1212/WNL.0000000000001729. Epub 2015 Jun 19.

International consensus diagnostic criteria for neuromyelitis optica spectrum disorders.

Author information

1
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. wingerchuk.dean@mayo.edu weinb@mayo.edu.
2
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.

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.

PMID:
26092914
PMCID:
PMC4515040
DOI:
10.1212/WNL.0000000000001729
[Indexed for MEDLINE]
Free PMC Article
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