title: Evaluation of the 2015 diagnostic criteria for neuromyelitis optica spectrum disorder.
abstract: OBJECTIVE
  To evaluate the application of the 2015 International Panel for NMO Diagnosis (IPND) criteria to consecutive cases of neuromyelitis optica spectrum disorder (NMOSD) in a large cohort of individuals with CNS inflammatory diseases.
  
  
  METHODS
  In total, 594 patients with CNS inflammatory diseases were included. Rigorous confirmation of the patients' aquaporin-4 immunoglobulin G antibodies (AQP4-IgG) status throughout the disease duration (mean 9.2 ± 5.7 years) using repeated assays, including ELISA and cell-based assay, was performed.
  
  
  RESULTS
  A total of 252 patients fulfilled the IPND criteria (AQP4-IgG positive: 226 [90%], AQP4-IgG negative: 26 [10%]). Of these, 136 (54%) patients met the 2006 neuromyelitis optica criteria. When we assumed an unknown AQP4-IgG status in the confirmed NMOSD group with AQP4-IgG, 162 of 226 (72%) patients with AQP4-IgG were classified as having NMOSD by the IPND criteria. The majority of patients were diagnosed with NMOSD within 2 years of onset (73%) or after a second attack (72%). Acute myelitis (83%) and optic neuritis (65%) were the most common clinical features throughout the disease duration. Optic neuritis (42%) was the most common initial manifestation, followed by acute myelitis (38%) and area postrema syndrome (14%).
  
  
  CONCLUSIONS
  The IPND criteria well-reflected the broader clinical spectrum of NMOSD and markedly improved the diagnostic yield compared to the previous criteria, even in patients with an unknown AQP4-IgG status.
authors.affiliation: From the Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: Jae-Won
authors.initials: JW
authors.lastname: Hyun
authors.affiliation: From the Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: In Hye
authors.initials: IH
authors.lastname: Jeong
authors.affiliation: From the Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: AeRan
authors.initials: A
authors.lastname: Joung
authors.affiliation: From the Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea.
authors.forename: Su-Hyun
authors.initials: SH
authors.lastname: Kim
authors.affiliation: From the Department of Neurology, Research Institute and Hospital of National Cancer Center, Goyang, Korea. hojinkim@ncc.re.kr.
authors.forename: Ho Jin
authors.initials: HJ
authors.lastname: Kim
mesh_terms: D000328:Adult; D051401:Aquaporin 4 / Q000276:immunology; D003937:Diagnosis, Differential; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007074:Immunoglobulin G / Q000378:metabolism; D008297:Male; D009471:Neuromyelitis Optica / Q000175:diagnosis* / Q000503:physiopathology; D012189:Retrospective Studies
chemical_list: C496030:AQP4 protein, human; D051401:Aquaporin 4; D007074:Immunoglobulin G