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Revised Diagnostic Criteria for Neuromyelitis Optica Spectrum Disorders (S63.001)

2014; Lippincott Williams & Wilkins; Volume: 82; Issue: 10_supplement Linguagem: Inglês

10.1212/wnl.82.10_supplement.s63.001

1526-632X

Dean M. Wingerchuk, Brenda Banwell, Jeffrey L. Bennett, Philippe Cabre, William M. Carroll, Tanuja Chitnis, de Sèze, Kazuo Fujihara, Benjamin Greenberg, Anu Jacob, Sven Jarius, Marco Aurélio Lana–Peixoto, Michael Levy, Jack H. Simon, Sílvia Tenembaum, Anthony Traboulsee, Patrick Waters, Kay E. Wellik, Brian G. Weinshenker,

Sinusitis and nasal conditions

Objective: To revise diagnostic criteria for neuromyelitis optica (NMO) and NMO spectrum disorders. Background: NMO is an inflammatory demyelinating CNS syndrome distinct from multiple sclerosis (MS) and associated with serum aquaporin-4 antibodies (AQP4-IgG). Current diagnostic criteria were developed in 2006 and require both optic nerve and spinal cord involvement. However, clinical and neuroimaging evidence, especially from AQP4-IgG seropositive patients, has revealed a wider disease spectrum. The International Panel for NMO Diagnosis (IPND) was convened to develop revised, evidence-based consensus diagnostic criteria. Design/Methods: The INPD met on 7 occasions between October, 2011 and November, 2013. Nineteen panel members participated in 6 working groups (clinical presentation, serology, neuroimaging, pediatrics, systemic autoimmunity, and opticospinal MS), each of which were charged with addressing focused questions to contribute to the revised diagnostic criteria. Each working group conducted systematic literature reviews related to their specific charges and summarized the results. Electronic surveys were then used to develop new criteria, which were iteratively refined through electronic scoring and face-to-face meetings. Results: The new diagnostic nomenclature defines the unified term "NMO spectrum disorders" (NMOSD), which is stratified by serological testing results (NMOSD with or without AQP4-IgG). Core characteristics of NMOSD with AQP4 antibodies include clinical syndromes and/or MRI findings related to optic nerve, spinal cord, brain stem, diencephalic, or cerebral presentations. The presence of enriched core characteristics, plus additional supportive criteria, are required for diagnosis of NMOSD without AQP4-IgG. The IPND also achieved consensus on pediatric NMOSD diagnosis, AQP4-IgG testing, monophasic NMOSD and opticospinal MS. Conclusions: The IPND achieved consensus on revised diagnostic criteria for adult and pediatric NMOSD, with or without AQP4-IgG, for clinical and research purposes and to distinguish NMOSD from competing diagnoses. These criteria require prospective validation. The proposed nomenclature allow for future revisions to account for new clinical, neuroimaging, laboratory, and antibody associations. Study Supported by: Guthy-Jackson Charitable Foundation.